VOL. LIX • NO. 5 • 2018

VOLUME LIX • ISSUE NO. 5

Celebrating Years

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House of Delegates MSMA ANNUAL SESSION 2018

FRIDAY AUGUST 17, 2018 8:00

MSMA Board of Trustees meeting [Time for specialty society meetings]

10:00 House of Delegates

SATURDAY, AUGUST 18, 2018 7:00 8:00

SPECIAL COMMEMORATIVE CELEBRATION KICK OFF BREAKFAST Emcee J. Edward Hill, MD CME: Arthur P. Guyton, MD. John Hall, MD

11:30

Candidate Forum (box lunch available @ 11:30)

8:30

CME: Civil War Medicine in Mississippi.

Sid Bondurant, MD

12:30

Reference Committee Hearings

9:00

CME: Mississippi Medicine and the Civil Rights Struggle. Loretta ClaudeJackson-Williams, D. Brunson, MDMD

Caucuses

9:30

CME: Yellow Fever in Mississippi Deanne S. Nuwer, PhD

3:30

Shuttle from the Westin to museum & Old Capitol Inn back to Westin afterward.

Reception 6:00 Commemorative at Museum of Mississippi History Enjoy hors d’oeuvres and beverages while exploring the new Mississippi History Museum. Then, walk one block to the Old Capitol Inn for the Gala.

7:30

Inaugural Gala, Old Capitol Inn

Gala Dinner Dance includes the official inauguration of 151st President Michael Mansour, MD; Leadership Awards program and dancing.

10:00

Break

10:15

CME: Images in Mississippi Medicine Luke Lampton, MD

10:45

CME: MSMA from Education to Advocacy Michael Trotter, MD

11:15

CME: William Faulkner Nobel Laureate or Town Drunk? Randy Easterling, MD

11:45

CME: James D. Hardy, MD. Marc Mitchell, MD

12:15

KEYNOTE LUNCHEON The Honorable Haley Barbour

2:00

House of Delegates

4:00

MSMA Board of Trustees Meeting

VOL. LIX • NO. 5 • MAY 2018

EDITOR Lucius M. Lampton, MD ASSOCIATE EDITORS D. Stanley Hartness, MD Richard D. deShazo, MD

THE ASSOCIATION President William M. Grantham, MD President-Elect Michael Mansour, MD

MANAGING EDITOR Karen A. Evers

Secretary-Treasurer W. Mark Horne, MD

PUBLICATIONS COMMITTEE Dwalia S. South, MD Chair Philip T. Merideth, MD, JD Martin M. Pomphrey, MD and the Editors

Speaker Geri Lee Weiland, MD Vice Speaker Jeffrey A. Morris, MD Executive Director Charmain Kanosky

JOURNAL OF THE MISSISSIPPI STATE MEDICAL ASSOCIATION (ISSN 0026-6396) is owned and published monthly by the Mississippi State Medical Association, founded 1856, located at 408 West Parkway Place, Ridgeland, Mississippi 39158-2548. (ISSN# 0026-6396 as mandated by section E211.10, Domestic Mail Manual). Periodicals postage paid at Jackson, MS and at additional mailing offices.

CORRESPONDENCE: Journal MSMA, Managing Editor, Karen A. Evers, P.O. Box 2548, Ridgeland, MS 39158-2548, Ph.: 601-853-6733, Fax: 601-853-6746, www.MSMAonline.com.

SUBSCRIPTION RATE: $83.00 per annum; $96.00 per annum for foreign subscriptions; $7.00 per copy, $10.00 per foreign copy, as available.

ADVERTISING RATES: furnished on request. Jill Gordon, MSMA Director of Marketing. Ph. 601-853-6733, ext. 324, Email: JGordon@MSMAonline.com POSTMASTER: send address changes to Journal of the Mississippi State Medical Association, P.O. Box 2548, Ridgeland, MS 39158-2548.

The views expressed in this publication reflect the opinions of the authors and do not necessarily state the opinions or policies of the Mississippi State Medical Association.

Copyright © 2018 Mississippi State Medical Association.

SCIENCE OF MEDICINE A Patient-Centered Approach to the Opioid Overdose Crisis Jennifer P. Schneider, MD, PhD Percutaneous Mitral Valve Repair in Mississippi Jason Fisher, DO; Brittain Heindl, MD; Christopher Douglas, MD; Thad Waites, MD Prevalence Estimate and Cost of Hemoglobin Disorders among African-Americans in Mississippi LaQuita Cooper, MPH; Thia Walker, MPH; Beryl Polk, PhD; Larry Smith, PhD; Cynthia Karlson, PhD; Melissa McNaull, MD; Suvankar Majumdar, MD Disposable Versus Conventional Bronchoscope Cost for Percutaneous Dilatational Tracheostomy Andrew Robichaux, MD; Andrew Wilhelm, DO; Cindy Moore, MD; Lana Jackson, MD Familial Gigantiform Cementoma in Twins: A Case Report Anna Jade Hartzog, MD; David Kowalczyk, MD; Jeffrey D. Carron, MD General Anesthesia without the Use of Muscle Relaxant for a Patient with Myasthenia Gravis and Multiple Co-morbidities: A Case Report and Literature Review Rachel Roberts, MD; Natesan Manimekalai, MD Top 10 Facts You Should Know About Bullous Skin Disorders: Dermatopathologic Approach Bonnie D. Hodge; Robert T. Brodell, MD DEPARTMENTS From the Editor – Wisdom in Tillerson’s Farewell Lucius M. Lampton, MD Commentary – Five Inadvertent HIPAA Violations by Physicians Tracey Haas, DO, MPH, Co-Founder, DocBookMD Personals President’s Page – If We Are Not Part of the Solution, We Will Be Part of the Problem William M. Grantham, MD Mississippi State Department of Health Reportable Disease Statistics MSMA 2017-18 Legislative Session Recap R. David Roberts, III, MSMA Director of Government Affairs Legalese – Decipher the Code: Keep Your Patients Safe Jon Corey Jackson, MD; Chasity Lynne Torrence, MD Images in Medicine – King’s Daughters Hospital, Greenville, 1927 Lucius M. Lampton, MD Poetry and Medicine – Just Call It Something, Doc!!

John D. McEachin, MD New Members

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ABOUT THE COVER Photo by Robert Brahan, MD, Hattiesburg

Official Publication

MSMA • Since 1959

Sunset over Moon Lake – Located across the river from Helena, Arkansas, and about halfway between Tunica and Clarksdale, Moon Lake is an old oxbow lake formed by the Mississippi River and until the 1850’s was connected to the River. There was a low levee, which was blown up by U. S. Grant in an attempt to find a route to the Yazoo River and come in behind the city of Vicksburg. He brought his flotilla into the north end of Moon Lake and entered the Yazoo Pass, into the Coldwater River and then into the Yazoo. However, he was turned back at Fort Pemberton at Greenwood and had to retrace his route back to the Mississippi River.

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Wisdom in Tillerson’s Farewell

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utgoing U. S. Secretary of State Rex Tillerson’s farewell address in mid-March to several hundred diplomats and civil servants who gathered in the State Department lobby to see him off possessed wisdom not only applicable to politics but also to the world of medicine. The 65-year-old Texas oilman reflected on his bruising 414-day term as secretary of state for the Trump administration. “This can be a Lucius M. Lampton, MD very mean-spirited town, but you don’t Editor have to choose to participate in that,” he commented to warm applause. “Each of us gets to choose the person we want to be, and the way we want to be treated, and the way we will treat others.”

Medicine, too, can be a mean-spirited town. The culture of medicine is too quick to judge harshly our peers and our patients, forgetting too frequently human frailty which dominates our fellow physicians, nurses, and our patients. Our nurses and staff often don’t treat our patients with the kindness and dignity they should. None of us is

perfect, and none of us should be expected to be. We make mistakes daily as we try to do our best for our patients. Hospitals, insurance companies, and the federal government too often dehumanize our patients and enforce senseless rules and regulations which aren’t consistent with the practice of medicine or even reality. Understanding and empathy should be emphasized over criticism and judgment in most of our interactions.

Tillerson urged his staff “to treat each other with respect, regardless of the job title, the station in life, or your role.” He closed his farewell address by encouraging his personnel to “undertake to ensure one act of kindness each day towards another person.” The culture of medicine needs to undertake this as well. Back in the 4th Century BC, Hippocrates in his Oath stressed not only the ethics of our profession but also our treatment of fellow physicians. He wrote for us “to hold” other physicians as “equal to” our parents and to consider “his family as my own brothers.” Physicians need to undertake acts of kindness daily in our treatment not only of our patients but also of our brothers and sisters. r Contact me at LukeLampton@cableone.net.

— Lucius M. Lampton, MD, Editor

JOURNAL EDITORIAL ADVISORY BOARD ADDICTION MEDICINE Scott L. Hambleton, MD

EMERGENCY MEDICINE Philip Levin, MD

MEDICAL STUDENT John F. G. Bobo, M2

ALLERGY/IMMUNOLOGY Stephen B. LeBlanc, MD Patricia H. Stewart, MD

EPIDEMIOLOGY/PUBLIC HEALTH Mary Margaret Currier, MD, MPH Thomas E. Dobbs, MD, MPH

NEPHROLOGY Jorge Castaneda, MD Harvey A. Gersh, MD

ANESTHESIOLOGY Douglas R. Bacon, MD John W. Bethea, Jr., MD

FAMILY MEDICINE Tim J. Alford, MD Diane K. Beebe, MD Jennifer J. Bryan, MD J. Edward Hill, MD Ben Earl Kitchens, MD James J. Withers, MD

OBSTETRICS & GYNECOLOGY Sidney W. Bondurant, MD Darden H. North, MD

GENERAL SURGERY Andrew C. Mallette, MD

OTOLARYNGOLOGY Bradford J. Dye, III, MD

HEMATOLOGY Vincent E Herrin, MD

PEDIATRIC OTOLARYNGOLOGY Jeffrey D. Carron, MD

INTERNAL MEDICINE Daniel P. Edney, MD W. Mark Horne, MD Daniel W. Jones, MD Brett C. Lampton, MD Jimmy Lee Stewart, Jr., MD

PEDIATRICS Michael Artigues, MD Owen B. Evans, MD

CARDIOVASCULAR DISEASE Cameron Guild, MD Thad F. Waites, MD CHILD & ADOLESCENT PSYCHIATRY John Elgin Wilkaitis, MD Nisha S. Withane, MD, Fellow CLINICAL NEUROPHYSIOLOGY Alan R. Moore, MD DERMATOLOGY Robert T. Brodell, MD Adam C. Byrd, MD

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ORTHOPEDIC SURGERY Chris E. Wiggins, MD

PLASTIC SURGERY William C. Lineaweaver, MD Chair, Journal Editorial Advisory Board PSYCHIATRY Beverly J. Bryant, MD June A. Powell, MD PULMONARY DISEASE Sharon P. Douglas, MD John R. Spurzem, MD RADIOLOGY P. H. (Hal) Moore, Jr., MD RHEUMATOLOGY Shweta Kishore, MD C. Ann Myers, MD UROLOGY W. Lamar Weems, MD

Do Not Enter the Courtroom Without the Protection of MACM Regardless of merit, thousands of medical malpractice claims are filed each year. Some say it is not a question of IF you will be sued; it is a question of WHEN. Call MACM today for your professional liability needs and to join so many others who have placed their trust with a company that has been physician owned and physician governed for over 40 years.

601-605-4882 | macm.net JOURNAL MSMA

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A Patient-Centered Approach to the Opioid Overdose Crisis JENNIFER P. SCHNEIDER, MD, PHD [Editor’s Note: Jennifer Schneider, MD, PhD, who specializes in Internal Medicine, Addiction Medicine, and Pain Management, is an internationally recognized expert in two addiction-related fields: addictive sexual disorders and the management of chronic pain with opioids. She has extensive clinical experience in treating patients with chronic non-cancer pain of all types and is recognized as a national expert in the appropriate use of opioids for chronic pain. She is the author of 15 books and numerous articles in professional journals, including the landmark book, “The Wounded Healer,” which she co-authored with Richard Irons. She is a brilliant and compassionate professional committed to educating others in her fields of specialty. She resides in Tucson, Arizona, and can be reached at jennifer@jenniferschneider.com. This essay (special to the JMSMA) provides an informed and insightful patient-centered approach to the current opioid overdose crisis.] — Ed.

T

he assumption that decreasing doctors’ ability to prescribe opioids is the most effective (and only) solution to the “opioid crisis” is a mistaken approach. One problem is that statistics on opioid deaths combine several groups of people: (1) Usually – compliant patients who accidentally took too many prescribed pills and overdosed. (2) People trying to commit suicide. (3) People who purchased prescription drugs on the street, either to treat their anxiety, depression, etc. or to feed their addiction with a high. (4) People who purchased illegal drugs on the street, such as heroin and fentanyl. (5) Young people (and older ones as well) whose source was a bottle of opioids in a relative or friend’s home. The type of user who is the main source of the increasing “opioid deaths” is #4, and their numbers are increasing. But this isn’t obvious if all that people hear about is the total number of deaths.

The real solution to opioid-related overdose deaths requires getting to the basics. The essential problem is poisoning due to overdosing on legitimately prescribed or illegally obtained opioids. Why do people take opioids in the first place? Some do because of pain, and they may accidentally take too much. Others are treating emotional problems such as anxiety, depression, and PTSD (post-traumatic stress disorder) related to childhood or adult trauma.1 Some of these persons are seeking to feel normal (opioids can be effective antidepressants or anxiolytics).2 In many cases members of this group were initially prescribed opioids for pain, but when they found that it was very effective for their psychological problems, they became committed to continuing to take the opioids. Then there are addicts seeking euphoria. Finally, still others are teens who began by experimenting with opioids.

Why do people prefer to buy prescription drugs (rather than illegal drugs) on the street? One common reason is that with prescription drugs, they know exactly what dose they’re getting. This knowledge makes them safer than heroin/fentanyl/etc., which are not regulated.

With the illegal drugs you don’t know what dose (or even what drug) you’re actually getting, and the risk of accidental overdose deaths is much greater than with the prescription drugs. After 2010, when the first extended-release oxycodone (OxyContin) was made into an abuse-deterrent formulation, the street value of OxyContin dropped

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dramatically (because it could no longer be crushed and inhaled or injected, and what gives a person a high is not the concentration of the drug in the blood stream but rather how quickly it gets into the brain).3 Instead, people switched to heroin,4 resulting in a massive increase in overdose deaths due to heroin; from 2010 to 2014, the age-adjusted rate of drug overdose deaths involving heroin more than tripled;5 and now the availability of fentanyl (and its similar versions) is worsening the situation. Because nearly every extended-release opioid is now in an abuse-deterrent formulation, their use too is decreasing and there is more of substitution of heroin and illegally obtained fentanyl. So, when we look at it, the cause of the increase in overdose deaths “due to opioids” was the decreased availability of prescription opioids on the street. This already shows us that decreasing the availability of prescription drugs on the street did not decrease the number of opioid deaths but rather increased it!

As Joshua Sharfstein, former Principal Deputy Commissioner of the FDA wrote in February 2018, “At a time when most insurers still do not provide adequate reimbursement for nonpharmaceutical approaches to pain or treatment for opioid use disorder, overly restrictive prescribing policies risk pushing patients with pain or addiction to illicit drugs, a transition many have made. A few distraught patients have even committed suicide.”6 The more frequently new rules appear and result in fewer opioid prescriptions, the more people will be using unregulated drugs such as heroin and illegally produced fentanyl, with their increased risk of accidental overdose deaths. You may have already heard, as we have in my state, Arizona, how HAPPY the producers and sellers in Latin America and other places are with the currently increasing regulations in the U. S. regarding opioid prescribing. What they see is an INCREASED market for their heroin and fentanyl! Their business is booming!

A patient-centered approach for opioid prescribing requires learning about the patient’s life, including a thorough risk assessment. What is a day in the patient’s life like? Are they employed? Do they have a support system of friends and family? How active physically are they? Personal or family history of drug or alcohol abuse or addiction? There are several simple tools available for risk assessment, such as the Opioid Risk Tool7

or SOAPP. Obtaining old records is important, as is consulting the state’s Prescription Drug Monitoring Website. Obtaining a urine drug screen will provide information about what relevant drugs the patient has recently taken. Childhood trauma is another risk factor for abuse or misuse;8 it can be assessed with the 1-page Adverse Childhood Experiences (ACE) questionnaire.9 Patients with vulnerabilities should not be denied pain treatment, but must be followed carefully.

Patients whose function and quality of life is improved by opioids need to continue getting them (usually not in isolation but along with other modalities); many of them are able to continue taking the same dose for years.10 Those patients who are “treating” their psychological problems with opioids, and those at risk of addiction, definitely need to have other modalities, including behavioral health assessment and treatment [including addiction treatment if that is an issue], and the necessity for them to become engaged in their chronic pain treatment: This means they are willing to go to physical therapy and do home exercises; go to an interventional pain specialist if warranted; see a behavioral health specialist if they are resistant to becoming engaged in their treatment and/or have unresolved childhood [or other] trauma issues; quit smoking if they smoke; work on losing weight if they’re obese; lower thier blood sugar if they’re diabetic, etc. Pills should not be the only treatment. Physicians, patients, and government officials need more understanding of the nature of addiction and the role of behavior health disorders. There is also a need for public education efforts to dispose of unused opioids, which are the main source of opioid overdose deaths of high-school age teens.

The real solution must make these modalities accessible to pain patients, who often can’t afford them, or their insurance won’t pay. That’s the big problem. Patients need to be able realistically to benefit from a “team effort.” Recently what was probably the best pain clinic in my city closed. Why? The deciding factor was that the insurance companies wouldn’t pay for treatment by the pain psychologist who worked there full time and focused on treatment of the root psychological causes of opioid overuse and high-risk use. Unfortunately, insurance companies often prefer to pay only for pills and procedures. They may be willing to pay for naloxone, a medication which will prevent an imminent opioid overdose death, but naloxone can’t change someone’s lifestyle and stop them from further overdoses. What is also needed is addiction treatment, and insurance companies often will not pay for inpatient or outpatient treatment.

The widespread belief (as recently stated by an “expert” consultant to the Arizona legislature) that “80% of patients on chronic opioids are addicted” needs to be corrected with an understanding of the difference between addiction and physical dependence.11 There is no quality evidence to support the 80%; on the contrary, the evidence suggests that a only a small minority of pain patients on chronic opioids develop an Opioid Use Disorder (the current name for addiction).12,13 Nora Volkow of the NIH has written, “Addiction occurs in only a small percentage of persons who are exposed to opioids – even among those with preexisting vulnerabilities.”14 Instead, what we

have now is a widespread (erroneous) belief that doctors are turning most of their chronic pain patients into addicts, who are then at a high risk of overdose deaths. If you believe that, then no wonder you believe that the solution is to stop prescribing opioids. If you understand what addiction is and the role of psychological issues in many patients, you’re in a better position to determine if a patient with chronic pain is also addicted or has a psychological disorder and should not be treated with opioids unless an appropriate specialist is also on the team, there are appropriate safeguards in place, etc. And of course, patients who are diverting their prescribed opioids should definitely not be treated with opioids.

Yes, correcting the misconceptions of prescribers and the public about the nature of addiction versus physical dependence, the actual (relatively small) likelihood of de novo addiction among chronic pain patients, the diverse causes of opioid overdoses, etc. is an important first step. But the ultimate solution to the opioid crisis is to shift the focus from a drastic reduction in opioid prescribing (an approach which penalizes a large number of chronic pain patients whose pain level and functioning have benefited from treatment with opioids) to a patientcentered approach15 that involves additional information gathering, a team approach, and consideration of a broader set of medication and non-medication treatment modalities including behavioral health. r

References 1. Davis B. Are opioids the next antidepressant? New York Times. July 4, 2016.

2. V olkow ND, McLellan AT. Opioid abuse in chronic pain – misconceptions and mitigation strategies. N Engl J Med. 2016;374:1253-1263.

3. American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition 2013;486.

4. Cicero TJ, EllisMS, Kasper ZA. Increased use of heroin as an initiating opioid of abuse. Addict Behav. 2017;74:63-66.

5. Warner M, Bastian BA, Minino AM, Hedegaard H. Drugs most frequently involved in drug overdose deaths: United States, 2010-2014. National Vital Statistics Reports 2016;65(10), CDC website.

6. Sharfstein J. A New Year’s wish on opioids. J Amer Med Assoc. 2018;319(6):537-538.

7. Webster LR, Webster RM. Predicting aberrant behaviors in opioid-treated patients: preliminary validation of the Opioid Risk Tool. Pain Med 2005;6(6):432-442.

8. Felitti VJ, Anda RF et al. Relationship of childhood abuse and household dysfunction to many of the leading causes of death in adults: The Adverse Childhood Experiences (ACE) study. Amer J of Preventive Med 1998;10:245-258.

9. U.S. Centers for Disease Control and Prevention (CDC) Adverse Childhood Experiences (ACEs) http://www.cdc.gov/violenceprevention/acestudy/ Accessed March 29, 2018.

10. Schneider JP, Kirsh KL. Defining clinical issues around tolerance, hyperalgesia, and addiction: a quantitative and qualitative outcome study of long-term opioid dosing in a chronic pain practice. J. Opioid Manage 2010;6(6):385-395.

11. Schneider JP. Ask the expert: Dependence versus addiction Pract Pain Manag.

2015;14(7).

12. Minozzi S, Amato L, Davoli M. Development of dependence following treatment with opioid analgesics for pain relief: a systematic review. [Cochrane Review] Addiction 2011; doi:10.1111/j.1360-0443.2012.04005.x.

13. Fishbain DA, Cole B, Lewis J, Rosomoff HL, Rosomoff RS.W hat percentage of chronic nonmalignant pain patients exposed to chronic opioid analgesic therapy develop abuse/addiction and/or aberrant drug-related behaviors? A structured evidence-based review. Pain Med. 2008;9(4):444-459.

14. Volkow ND, McLellan AT. Opioid abuse in chronic pain – misconceptions and mitigation strategies. N Engl J Med. 2016;374:1253-1263.

15. Schneider JP, Davis B. How well do you know your patient? Practical Pain Manage 2017;16(2).

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Percutaneous Mitral Valve Repair in Mississippi JASON FISHER, DO; BRITTAIN HEINDL, MD; CHRISTOPHER DOUGLAS, MD; THAD WAITES, MD Introduction Mitral regurgitation is among the most common valvular heart diseases in the industrialized world, but many patients are not candidates for open heart repair or replacement of the mitral valve. The main contraindications are age and significant comorbidities such as chronic renal failure or advanced pulmonary disease. This has since led to the development of percutaneous interventions to repair and potentially replace the mitral valve. Only one percutaneous intervention has been approved by the FDA for the treatment of mitral regurgitation. Known as the MitraClip system, this percutaneous repair procedure was performed for the first time in Mississippi in January of last year and subsequently the structural heart team at the Hattiesburg Clinic/Forrest General Hospital has performed over 30 cases. This article presents the case of this first patient to undergo a percutaneous mitral valve repair in Mississippi. It subsequently describes the development of the procedure, the technique, and the indications for its use.

Key Words: Percutaneous mitral valve repair, mitral regurgitation, MitraClip, mitral valve insufficiency Case Description A 62-year-old man was evaluated in the structural heart disease clinic at this hospital in December 2016. His symptoms began in August when he presented to the emergency department with progressive bilateral lower extremity edema, dyspnea, and orthopnea. Prior to this admission he had a history of coronary artery disease (with distant two-vessel coronary bypass grafting), hyperlipidemia, type 2 diabetes mellitus, hypertension, peripheral vascular disease, and diffuse parenchymal lung disease. He had a 75-pack-year smoking history but stopped several months prior to this evaluation.

An echocardiogram was performed on admission and described an ejection fraction of 28%, grade 2 diastolic dysfunction, and mild mitral regurgitation. An anteroapical wall motion abnormality was also noted.

Left heart catheterization revealed patent bypass grafts but severe left ventricular dysfunction. He was diagnosed with NYHA class III-IV congestive heart failure and treated with intravenous furosemide. He was discharged on an appropriate medication regimen, along with an external defibrillator vest.

Within four weeks he experienced two subsequent hospitalizations, first with a transient ischemic attack, and then with a subsequent ischemic stroke resulting in left-sided hemiplegia. During the evaluation of this second event, a transesophageal echocardiogram was performed and revealed no thrombus but worsening of his mitral regurgitation from mild to moderate. Apixaban was started for secondary stroke prevention and the patient was discharged.

Over the following three months he was hospitalized on three separate occasions with congestive heart failure exacerbations. During his second hospitalization the patient developed acute renal failure thought to be a sequelae of cardiorenal syndrome. Another transthoracic echocardiogram revealed worsening of his mitral regurgitation into the severe range.

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He was started on milrinone with improvement in his renal function, though his estimated glomerular filtration rate remained less than 30 mls/ min/1.73m2 after discharge. Before discharge, cardiac transplantation was discussed, but the patient declined referral.

He was agreeable to evaluation in the multidisciplinary structural heart disease clinic, and in December a second transesophageal echocardiogram was performed, revealing severe mitral regurgitation of mixed etiology. An abnormal mitral leaflet morphology was noted, suggesting that the valve itself was incompetent. In the setting of multiple comorbidities he was not a candidate for surgical mitral valve replacement. After discussions between the patient and the structural heart team, the decision was made to proceed with percutaneous mitral valve repair using the Mitraclip system.

Traditional Management of Mitral Regurgitation Mitral valve regurgitation (MR) is the second most common valvular heart disease in the industrialized world, after aortic stenosis.1 Mitral regurgitation can be separated into two types based on etiology: primary and secondary. Primary MR is caused by defects in the valve leaflets and chords, and any ventricular dysfunction is thought to be caused by the underlying regurgitant flow. In secondary MR the valvular apparatus is normal, but progressive left ventricular dilation from ischemic or intrinsic myocardial disease causes papillary muscle and annular displacement, preventing adequate leaflet coaptation.2 In primary MR, surgery is the definitive treatment. Surgery could mean either repair or replacement; both would require cardiopulmonary bypass and sternotomy or thoracotomy. The decision to pursue surgery is based on the combination of heart failure symptoms, MR severity, and estimated ejection fraction. With secondary MR the benefit of surgery is not clear, and often medical management of the underlying cardiomyopathy is the most appropriate therapy. The criteria for pursuing surgery in both situations is clearly defined in guidelines jointly offered by the American College of Cardiology and American Heart Association.2 Despite current guidelines a large percentage of patients with severe MR do not undergo surgery. Data suggests that only half of the patients that qualify for surgical intervention actually undergo surgery.1 The most common reasons are age, advanced valve degeneration, advanced left ventricular dysfunction, or a combination of comorbidities – especially pulmonary disease and diabetes.1 The irony is that these patients are more likely to benefit from intervention as standard medical therapy will be less effective.

Percutaneous Mitral Valve Repair In 1991, cardiovascular surgeons developed what they described as an edge-to-edge technique to repair mitral valves. One edge of the anterior mitral leaflet was simply sutured to the posterior leaflet. The mitral valve then had two openings and the volume of regurgitation decreased with the physiology of the valve maintained. Initially, the surgeons were concerned that stenosis would develop, but fortunately this was not the case and the procedure was proven effective. This technique was a clear target for a less

invasive percutaneous approach. The initial procedure involved a suturing technique, but the durability was poor and it was abandoned for a clipping technique. In 2003 the first animal trials were reported for the MitraClip system (Abbott Vascular, Santa Clara, California), which would soon become the first successful percutaneous repair device.3 Standardized steps for MitraClip implantation were described in the Endovascular Valve Edge-to-Edge Study (EVEREST I) study. A guide catheter is inserted into the groin through the common femoral vein. It is advanced through the inferior vena cava and into the right atrium. The catheter punctures the atrial septum and is advanced into the left atrium.

The clip delivery system is guided by transesophageal echo into the peak MR jet and the clip is closed onto both mitral valve leaflets as they are naturally pushed back into the jaws of the clip. The resultant mitral regurgitation and stenosis are assessed, and if not appropriately reduced, the clip can be opened and repositioned. Additional clips can be used if one is not sufficient.4 EVEREST I established the safety and efficacy of the procedure, as the MitraClip system was compared with standard surgical repair or replacement in a similar patient population. The rates of death and recurrence of severe MR at 12 months were similar, but, the rate of surgery for mitral valve dysfunction was 20% in the MitraClip arm and 2.2% in the surgery arm. Surgery was more efficacious, but rates of adverse events within 30 days after the procedure were lower in the MitraClip group.

Both groups had an equal reduction in symptoms and improvement in left ventricular function.4 A second and much larger EVEREST study group also proved that traditional surgery was more efficacious if the patient could tolerate bypass and sternotomy.5 The EVEREST II High Risk Registry (HRS) investigated the role of percutaneous repair in those not suitable for surgical intervention and proved that in patients with a prohibitive surgical risk percutaneous repair had significant benefit.6 At 5 years the durability, safety, and efficacy of percutaneous repair has been well validated.5 Based on these studies, the current guidelines recommend percutaneous repair in severely symptomatic patients (NYHA Class III to IV) with severe primary (degenerative) MR (3-4+) who have a reasonable life expectancy but have a prohibitive surgical risk and remain severely symptomatic despite optimal goal directed medical therapy (i.e. an appropriate heart failure regimen).2 The goals of treatment are MR ≤ 2+ resulting in reduced hospitalizations, improved quality of life, reverse left ventricular remodeling and symptomatic relief in patients with no other therapeutic options.6 The multidisciplinary structural heart team, including cardiovascular surgeons and cardiologists, has been established as a Class I indication for the evaluation of complex patients with valvular heart disease to determine whether surgery or percutaneous repair is the best option for each patient.7 An STS score is most often used to determine surgical risk (≥8% is considered prohibitive), though many other factors may preclude surgery.6,8 With the success of transcatheter aortic valve replacement (TAVR) and the MitraClip system we are likely to see additional devices studied for percutaneous valvular interventions. The first study to document the feasibility of percutaneous mitral valve replacement was performed in January of this year, and the outcomes were very encouraging.7 Along with the application of the TAVR system to lower risk patients, this system is being used for broader indications, including the treatment of pulmonary valve stenosis.9 The future of valvular heart disease management certainly

appears to lie in the percutaneous approach.

Case Outcome The patient underwent MitraClip percutaneous repair in January. One clip was placed and his mitral regurgitation grade was reduced from 4+ to 1+.

He developed hypertensive emergency after the procedure with associated flash pulmonary edema. This resolved with noninvasive ventilation and IV furosemide. He spent one evening in the cardiac intensive care unit and was transferred to the floor the following day. He was discharged home three days after the procedure. He was readmitted January 17 with generalized functional decline and volume overload –milrinone was restarted. After IV diuresis he was discharged on hospital day 5.

Repeat transesophageal echocardiography performed in February revealed mild to moderate mitral regurgitation with a functioning clip. His ejection fraction was estimated at 38%. Over the next several months he has continued to improve, and to the date of this writing – 8 months postprocedure – he has not required hospital readmission.

Since this first case, 23 more percutaneous mitral valve repairs have been performed by the structural heart team at Forrest General Hospital. Thus far, the team has had a 96% procedural success rate with a reduction in mitral regurgitation from 4+ to 1-2+. Outcomes in terms of quality of life, symptom relief, and reduced hospitalizations have been very positive. r

References 1. Mirabel M, Lung B, Baron G, et al. What are the characteristics of patients with severe, symptomatic mitral regurgitation who are denied surgery? Eur Heart J. 2007;28(11):1258-1265.

2. Nishimura RA, Otto CM, Bonow RO, et al. 2014 AHA/ACC guideline for the management of patients with valvular heart disease: executive Summary: a report of the American College of Cardiology/American Heart Association task force on practice guidelines. J Am Coll Cardiol. 2014;63(22):2455-2488.

3. Maisano F, La Canna G, Colombo A, et. al. The evolution from surgery to percutaneous mitral valve interventions. J Am Coll Cardiol. 2011;58(21):2174-2182.

4. Feldman T, Foster E, Glower D, et al. Percutaneous repair or surgery for mitral regurgitation. N Engl J Med. 2011(15);364:1395-1406.

5. Feldman T, Kar S, Elmariah S, et al. Randomized comparison of percutaneous repair and surgery for mitral regurgitation: 5-year results of EVEREST II. J Am Coll Cardiol.

2015;66(25):2844-2854.

6. Whitlow P, Feldman T, Pedersen W, et al. Acute and 12-month results with catheterbased mitral valve leaflet repair: the Everest II (endovascular edge-to-edge repair) high risk study. J Am Coll Cardiol. 2012;59(2):130-139.

7. Muller D, Farivar R, Jansz P, et al. Transcatheter mitral valve replacement for patients with symptomatic mitral regurgitation: a global feasibility trial. J Am Coll Cardiol.

2017;69(4):381-391.

8. Shroyer A, Coombs L, Paterson E, et al. The Society of Thoracic Surgeons: 30-day operative mortality and morbidity risk model. Ann Thorac Surg. 2003;75(6):1856-1864.

9. Wilson W, Benson L, Osten M, et al. Transcatheter pulmonary valve replacement with the Edwards Sapien system: the Toronto experience. JACC Cardiovasc Interv.

2015;8(14):1819-1827.

Author Information PGY-3 resident, Forrest General Hospital Family Medicine Residency, Hattiesburg (Fisher). Internist and faculty member, Forrest General Hospital Family Medicine Residency (Heindl). Interventional cardiologist, Hattiesburg Clinic and Forrest General Hospital. He leads the structural heart team that has been performing the first MitraClip procedures in Mississippi. (Douglas). Interventional cardiologist, Hattiesburg Clinic and Forrest General Hospital. Among many other positions he served as past Trustee of the American College of Cardiology, and Chair of the Board of Governors. He was recently selected as a Master of the ACC. (Waites).

Corresponding Author: Brittain Heindl, MD; Forrest General Hospital Family Residency, 415 South 28th Avenue, Hattiesburg, MS 39401.

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Prevalence Estimate and Cost of Hemoglobin Disorders among African Americans in Mississippi LAQUITA COOPER, MPH; THIA WALKER, MPH; BERYL POLK, PHD; LARRY SMITH, PHD; CYNTHIA KARLSON, PHD; MELISSA McNAULL, MD; SUVANKAR MAJUMDAR, MD Introduction Hemoglobinopathies or hemoglobin disorders are a group of inherited red blood cell disorders that include Sickle Cell Disease and thalassemia. SCD is an autosomal recessive condition that occurs from a single base mutation in the globin gene of the hemoglobin molecule (hemoglobin S). In the United States, SCD primarily affects African Americans with four genotypes: sickle cell anemia (hemoglobin SS), sickle cell-hemoglobin C disease (hemoglobin SC), hemoglobin S/β0 thalassemia, and hemoglobin S/β+ thalassemia. Due to the abnormal hemoglobin, red blood cells assume a sickle shape resulting in pain from vaso-occlusion and multiple organ damage, including stroke, kidney disease and lung complications that can require frequent hospitalization and blood transfusions.1 Thalassemia is an inherited blood disorder that occurs when the body does not make enough hemoglobin, the protein that is responsible for carrying oxygen within the red blood cell.2

Mississippi law mandates all newborns in the state are screened for genetic disorders including hemoglobinopathies. The MSDH Newborn Screening Program supports and manages the state’s newborn screening process to ensure that proper procedures are followed, such as newborn screening specimens being collected between 24 and 48 hours of age and specimens being sent to PerkinElmer Genetictm where results are electronically downloaded to the MSDH Newborn Screening Program database.3,4 As a result, thousands of newborns with various genetic disorders have been identified and treated. The UMMC and MSDH Newborn Screening Program collect information on incidence and prevalence of all genetic disorders. The collection of newborn screening data has provided a more comprehensive and detailed understanding of hemoglobin disorder trends. Researchers such as Brousseau et al. estimate that there are 2,292 Mississippians with the hemoglobin disorder known as SCD, while Hassell et al

Table 1. Hemoglobin 114,490 African-American newborns screened Table 1. Hemoglobin prevalence ofprevalence 114,490 African of Americans screened Disorder Hemoglobin SS Hemoglobin SC Hemoglobin S/Beta+ Thalassemia Hemoglobin S/Beta0 Thalassemia Hemoglobin C disease Hemoglobin C/Beta+ Thalassemia Hemoglobin C/Beta0 Thalassemia Hemoglobin Beta+ Thalassemia Hemoglobin E Disease Total

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# Confirmed 189 137

Percent 46.32% 33.58%

37

9.07%

11 17

2.70% 4.17%

14

3.43%

1

0.25%

2 0 408

0.49% 0.0% 100%

African-American Detection Prevalence per 1,000 births Rate 1.65 1:606 1.20 1:836 0.32 1:3,094 0.10 1:10,408 0.15 1:6,735 0.12 1:8,178 0.01 1:114,490 0.02 1:57,245 0 0 1:281

Figure 1. Hemoglobin Disorder Mortality Rate for Mississippi African Americans by Age Group

Figure 1. Hemoglobin Disorder Mortality Rate for Mississippi African-Americans by Age Group 4.50

Mortality Rate/100,000

4.00

3.73

3.50

3.81 3.42 3.02

3.00

2.48

2.50

1.94 1.98

2.00

2.28

2.18 1.81

1.98 1.34

1.33

1.50 1.00 0.50

2.97

0.55

0.89

0.88

0.18

85+

80-84

75-79

70-74

65-69

60-64

55-59

50-54

45-49

40-44

35-39

30-34

25-29

20-24

15-19

10-14

5-9

<5

0.00

Age Group Source: Mississippi State Department of Health (MSDH), Public Health Statistics (Vital Records)

report SCD population estimates for Mississippi in the range from 2,761 to 4,347.5,6 According to the MSDOM, 2,626 beneficiaries with hemoglobin disorders were provided with healthcare annually.7 The purpose of this study was to provide an accurate estimate of the number of individuals in the state of Mississippi with hemoglobin disorders that include sickle cell disease and thalassemia. Birth-cohort data and US Census data were adjusted for early mortality and to estimate the financial burden of the condition in Mississippi.

Key Words: SCD, sickle cell disease; MSDH, Mississippi State Department of Health; UMMC, University of Mississippi Medical Center; MSDOM, Mississippi Division of Medicaid; CDC, Centers for Disease Control and Prevention; NNSIS, National Newborn Screening Information System Methods Prevalence of Hemoglobin Disorders Using Birth-Cohort Data The prevalence of hemoglobin disorders was derived using the total detected hemoglobinopathy births and the total number of newborns screened. The confirmed hemoglobin disorders were retrieved from the MSDH Newborn Screening Report 2003-2008 and Newborn Screening Report 2009-2014.3,4 Additionally, the total number of newborns screened was obtained from the Mississippi National Bureau

of Statisticsâ&#x20AC;&#x2122; database. For the analysis, the detection rate was used to estimate the prevalence of hemoglobin disorders among the newborns screened and calculated by dividing the number of African American newborns with confirmed hemoglobin disorders into the total number of newborns screened from 2005-2010. Numbers were further broken down by the different forms of hemoglobin disorders within the Mississippi African American population.

Hemoglobin Disorder Mortality Adjustment The CDC collects mortality data from death certificates in an online database (https://wonder.cdc.gov/ucd-icd10.html).8 For this analysis, the ages at death for individuals with hemoglobin disorders were tabulated in 5-year age brackets. Estimates of hemoglobin mortality rates, at each 5-year age bracket, were calculated by taking the number of hemoglobin disorder deaths between 2005 through 2010 and dividing by the total of the six Mississippi population estimates from 2005-2010 US Census data. The mortality-adjusted hemoglobin disorder rate was calculated in each age-bracket by subtracting mortality rates from the birth-cohort based population prevalence estimate. The adjusted rate was multiplied by the 2010 U.S. Census population count to arrive at an estimate of the number hemoglobin disorders. Newborns considered non-permanent Mississippi residents were excluded from the above estimate.

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Medicaid Cost for Hemoglobin Disorder

Figure 2. Distribution Hemoglobin Disorders among African Americans in Mississippi Public Figure 2. of Distribution of Hemoglobin Disorders among African by Americans Health District

Data collected from the MSDOM provided the expenditures related to beneficiaries by gender in two age categories; 0 to 19 and 20 and older.

Data were collected using the ICD-9 codes for hemoglobin disorders: sickle cell disease 282.41, 282.42, 282.60, 282.61, 282.62, 282.63, 282.64, 282.68, 282.69, and thalassemia 282.49. The annual cost of medical treatment was calculated for each age category by gender and divided by 12 to determine the cost per patient-month.

in Mississippi by Public Health District

Population Projections by Public Health District There are 82 counties in the state of Mississippi. To provide adequate public health services Mississippi is divided into nine public health districts, each overseen by a District Medical Officer and a District Administrator. The age-adjusted mortality prevalence for the different hemoglobin disorders was calculated for each Mississippi public health district. ArcGIS ArcMap 10 software was used to show the age adjusted prevalence of hemoglobin disorders for African Americans by public health district.9 Results During the period from 2005 to 2010, a total of 253,347 newborns of all races and ethnicities were screened, of which 114,490 were African Americans. The total number of newborns with confirmed hemoglobinopathies were 418. Among those newborns, 51.0% (213) were female and 49.0% (205) were male. African Americans accounted for 408 (97.6%) of these cases, while Non-African Americans accounted for only 10 (2.4%) cases. Table 1 shows the number and empirical distribution of confirmed hemoglobin disorders identified in Mississippi from 2005-2010 among African American newborns that were screened.

There were 13 types of hemoglobin disorders detected in African American newborns from 2005-2010 as shown in Table 2. In practicality, hemoglobin Beta thalassemia intermedia is considered to be hemoglobin Beta+ thalassemia, and any SCD with hemoglobin Barts was included in their respective SCD category; for example, hemoglobin Sickle C Disease + Barts was considered to fall under hemoglobin SC disease. The hemoglobin disorders with the highest prevalence among African American newborns were Sickle Cell Anemia, Sickle Cell C Disease, Hemoglobin S/Beta + Thalassemia, Hemoglobin C Disease, and Hemoglobin C/Beta + Thalassemia.

The overall prevalence of any hemoglobin disorder was 1 in 281 among African American births compared to 1 in 13,886 in non-African American births. We estimate that there are a total of 4,874 people of all races in Mississippi suffering with hemoglobin disorders, most of which are African Americans (3,893).

Table 2. The different hemoglobinopathy genotypes detected by newborn screen2. The different hemoglobinopathy genotypes detected by newborn screen Table Sickle Cell Anemia (hemoglobin SS) Sickle C Disease (hemoglobin SC) Hemoglobin S/Beta+ Thalassemia Hemoglobin S/Beta0 Thalassemia Hemoglobin C Disease Hemoglobin C/Beta+ Thalassemia Sickle Cell Anemia + Hemoglobin Barts (hemoglobin SS with alpha thalassemia) Hemoglobin C/Beta 0 Thalassemia Hemoglobin Beta + Thalassemia Sickle C disease + Barts (hemoglobin SC with alpha thalassemia) Hemoglobin Beta Thalassemia Intermedia Hemoglobin E Disease Hemoglobin E Disease + Hemoglobin Barts (hemoglobin E with alpha thalassemia)

99.2% (n=127) of the deaths and approximately 83% of the hemoglobin disorder deaths occurred between the ages of 15 and 59 years.

Population Projections by Public Health District

Hemoglobin Disorder Mortality Adjustment There were a total of 128 hemoglobin disorder deaths that occurred during this time period (Figure 1). African Americans accounted for 238 VOL. 59 â&#x20AC;˘ NO. 5 â&#x20AC;˘ 2018

Figure 2 shows the distribution of the projections for African Americans with hemoglobin disorders in Mississippi. From the projection estimates, the majority (51.7%) of the hemoglobin disorder

patients are in public health districts I, III, and V (Figure 2). Over half of the counties within districts I, III, and V are a part of the impoverished Mississippi Delta region.

Medicaid Cost for Hemoglobin Disorder From 2005-2010, the MSDOM provided healthcare for approximately 2,626 beneficiaries with hemoglobin disorders annually. During that period of time, over 93 million dollars was spent by the MSDOM for hemoglobin disorders expenditures, which is approximately 15.5 million annually. Most (59.6%) of the Medicaid recipients were between the ages of 0-19, and 55.4% of the beneficiaries were female.

The hemoglobin disorder-related costs were lower for the pediatric beneficiaries compared to adult beneficiaries.

Costs per patient-month were slightly higher for pediatric males ($1169.10) compared to pediatric females ($1043.51) as shown in Table 3. The costs per patient-month were significantly higher for adult males ($1977.50) compared to adult females ($1569.75).

Discussion In the United States, SCD affects about 70,000 to 100,000 individuals primarily of African descent.6 According to the 2010 US Census, Mississippi has the highest percentage (37.6) of African Americans in the nation.10 The Centers for Disease Control and Prevention estimates SCD occurs nationally in about one out of every 365 Black or African American births and about one out of every 16,300 HispanicAmerican births.1 Our analysis shows Mississippi to have a higher prevalence rate for SCD (hemoglobin SS, SC, S/beta0 thalassemia and S Beta+ thalassemia) than the national average with one out of every 306 African American newborns screening positive for SCD. Specific to genotype, an estimate from California showed a prevalence rate of hemoglobin SS of one out of 700, while data from the NNSIS found a prevalence rate of 1:601, which was similar to our findings for Mississippi (1:606).11 A recent study evaluated the birth prevalence of SCD in Shelby County, Tennessee, over a 10-year period, there were 322 cases of SCD, with a birth prevalence that varied from 2.8 to 4.7 per 1000 African American live births.12 In Hasselâ&#x20AC;&#x2122;s landmark paper evaluating population estimates of SCD in the United States which include state-specific prevalence rates corrected for early mortality,

utilizing CDC data and NNSIS cohort data, there are 2,761 and 3,092 individuals with SCD in Mississippi, respectively. However, based on our mortality-adjusted analysis, there are approximately 3,503 people who suffer from SCD in Mississippi, and approximately 1,775 have the severe form of sickle cell disease (hemoglobin SS).

The annual expense attributed to a SCD patient is $9,360 for Medicaid recipients and $13,469 for private insurance recipients on average.13 Our data shows that adult males with a hemoglobin disorder utilize greater health care expenditures than female adults with hemoglobinopathies. Fewer routine physician visits may lead to less optimal medical management for a chronic disorder like SCD and, thus, may lead to greater expenditures through Emergency Department visits or hospitalizations for vaso-occlusive episodes and medical complications. Research is needed in this area to determine the reasons for greater health care expenditures in adult males with hemoglobinopathies and interventions developed to reduce these monetary and likely psychosocial costs.

The estimated population projections are presented to make decision makers, healthcare providers, and the larger community aware of the burden of hemoglobin disorders. The available evidence indicates that Mississippi likely has the highest prevalence of hemoglobin disorders in the US, with 97.6% of hemoglobin disorders being in African Americans. A large portion of these individuals with hemoglobin disorders live in the western-central (Hinds, Madison, Rankin, Simpson, Copiah, Claiborne, and Warren Counties) and Mississippi Delta regions (Yazoo, Issaquena, Sharkey Counties). Disparities related to low income, low education levels, and lack of access to and utilization of quality healthcare are particular concerns for African Americans in these areas. These social factors can contribute to the health and wellbeing of people with hemoglobin disorders as early detection of hemoglobin disorders permits families to receive counseling on proper medical management and future reproductive decisions.

Additionally, from a public health perspective, there is a need to focus on strengthening surveillance and monitoring of disease occurrence and health outcomes, as well as increase education and awareness among those African American communities who are most affected.

The current research attempts to serve as a foundation to support the gaps in surveillance and monitoring efforts, policy development, and

Table 3. Mississippi Division of Medicaid SCD Disorders Related Cost, 2005-2010 Table 3. Mississippi Division of Medicaid SCD Disorders Related Cost, 2005-2010

Gender

Female Male Female Male

Age

0 to 19 0 to 19 20 & over 20 & over

Distinct Beneficiary Count

Percentage

757 808 698 363

28.80% 30.80% 26.60% 13.80%

Annual Average Per Beneficiary $12522.15 $14029.24 $18836.96 $23730.02

Average per Beneficiary (Patient-Month)

$1043.51 $1169.10 $1569.75 $1977.50

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population-based programs to reduce morbidity and mortality among people in Mississippi with hemoglobin disorders. With the progress of public health intervention, universal newborn screening has improved public health efforts of early detection for hemoglobin disorders.

However, these efforts can be enhanced to provide standardized newborn screening follow-up procedures to ensure that families receive early and on-going counseling in disease management, genetic counseling related to future reproductive decisions and appropriate psychosocial support services for chronic hemoglobin disorders. A comprehensive approach to specialized treatment and services can improve health outcomes of patients with hemoglobinopathy. r References 1. Centers for Disease Control. What you should know about Sickle Cell Disease. http://www.cdc.gov/ncbddd/sicklecell/documents/SCD%20factsheet_ What%20is%20SCD.pdf. Accessed February 15, 2012.

2. Centers for Disease Control and Prevention: Thalassemia. https://www.cdc.gov/ ncbddd/thalassemia/index.html. Accessed February 15, 2012.

3. Mississippi State Department of Health: Newborn Screening Report 2003-2008 http://msdh.ms.gov/msdhsite/_static/resources/4367.pdf. Accessed January 31, 2017.

4. Mississippi State Department of Health: Newborn Screening Report 2009-2014 http://msdh.ms.gov/msdhsite/_static/resources/6444.pdf. Accessed January 31, 2017 5. Brousseau DC, Panepinto JA, Nimmer M et al. The number of people with sickle-

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cell disease in the United Sates: national and state estimates. Am J of Hematol. 2010; 85(1):77-78.

6. Hassell KL. Population estimates of sickle cell disease in the U.S. Am J of Prev Med. 2010; 38(4): S512-S521. Mississippi Division of Medicaid. https://medicaid. ms.gov/ Accessed February 15, 2012 7. Centers for Disease Control and Prevention, National Center for Health Statistics.

Compressed mortality fıle, 1999-2015 request. https://wonder.cdc.gov/cmfICD10.html Assessed January 10, 2012 8.

Esri, ArcGIS Pro http://www.esri.com/en/arcgis/products/arcgis-pro/ Overview. Assessed January 31, 2017 9. U.S. Census Briefs. The Black Population: 2010 Census Briefs. http://www. census.gov/prod/cen2010/briefs/c2010br-06.pdf. Accessed January 10, 2012.

10. Lorey FW, Arnopp J, Cunningham GC. Distribution of hemoglobinopathy variants by ethnicity in a multiethnic state. Genet Epidemiol. 1996; 13(5):501–512.

11. Smeltzer MP, Nolan VG, Yu X et al. Birth prevalence of sickle cell trait and sickle cell disease in shelby county, tn. Pediatr Blood Cancer. 2016; 63(6): 1054-1059.

12. Amendah DD, Mvundura M, Kavanagh PL et al. Sickle cell disease-related pediatric medical expenditures in the U.S. Am J of Prev Med. 2010; 38(4): S550-S556.

Author Information Walden University Sciences (Cooper), Jackson State University (Walker), Mississippi Department of Health (Cooper, Walker, Polk, Smith), Mississippi Division of Medicaid (Smith), University of Mississippi Medical Center (Karlson, McNaull, Majumdar).

Corresponding Author: Suvankar Majumdar, MD; Associate Professor of Pediatrics and Medicine, 2500 North State Street, Jackson, MS 39216. Ph: (601)984-5220; (smajumdar@umc.edu).

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Disposable Versus Conventional Bronchoscope Cost for Percutaneous Dilatational Tracheostomy ANDREW ROBICHAUX, MD; ANDREW WILHELM, DO; CINDY MOORE, MD; LANA JACKSON, MD

Abstract Objective: A cost comparison of single-use, disposable bronchoscopes (SDB) to conventional bronchoscopes (CB) in a 20-bed Medical Intensive Care Unit (ICU) for the purpose of percutaneous, dilatational tracheostomy (PDT).

Study Design: Cost Comparison Methods: We reviewed our database for all bronchoscopies performed in the Medical ICU over the interval August 2015 to August 2016.

PDTs performed with SDB and all cases for which CB were used were collected and entered into a Microsoft Excel database. Cost analysis was performed. Our model makes the assumption that an institution is considering purchasing an additional CB or the SDB system for use performing PDTs. Results Bronchoscopies performed with SDB and CB in the ICU totaled 34 and 94, respectively. SDB unit variable costs were $274/unit with a fixed cost of $1,495 for an associated monitor. Attributable CB value was 26% of an upfront cost of $30,000 per scope or $7,968.75, as determined by the ratio procedures using SDB to total bronchoscopies performed in the ICU over one year. Service contracts for a CB were $2394-2889/scope/year. Other associated CB costs per procedure were $6 for supplies and $13.53 for technician wage. The use of SDB during PDT was cost efficient until thirty-five PDTs were performed or until $11,085 was spent.

Conclusion As medicine strives to improve quality of care, institutions must thoroughly weigh the cost versus benefit of new technology with quality and efficiency of care. Institutions must examine the multiple factors included in our model in performing there own cost analysis for purchasing a SDB system.

Key Words: Cost comparison, bronchoscopy, dilatational tracheostomy

Background Tracheostomy has been utilized as an airway management technique for millennia; however, the technique and indications have evolved 242 VOL. 59 • NO. 5 • 2018

dramatically. The earliest evidence of tracheostomy dates back to 2000 BCE in the Hindu Book of Medicine, the Rig Veda. Although described BCE, tracheostomy was rarely used until the 1800s, and even then, it was used only for emergency airway access. Modern era intensive care medicine and prolonged mechanical ventilation have necessitated and popularized another application of the tracheostomy.1 Tracheostomy within 5-10 days of anticipated prolonged endotracheal intubation has been shown to decrease pulmonary morbidity, decrease critical care resource utilization, and lower overall mortality rates.2,3 Open surgical tracheostomy (OST) requires operating room time and costs as well as associated risks with patient transfers. Percutaneous dilatational tracheostomy (PDT) allows for tracheostomy placement at the bedside, thus avoiding the aforementioned issues. When comparing PDT to OST, there is no significant difference in overall mortality, intraoperative hemorrhage, and postoperative hemorrhage.4 Wound infection rates were found to favor PDT compared to OST. These findings support PDT, performed electively at the bedside, as the recommended procedure of choice in critically ill adult patients.5 Flexible fiberoptic bronchoscopy-guided PDT is performed to ensure appropriate endotracheal cannulation in an otherwise blind, bedside technique.6 Commonly cited reasons for bronchoscopy guidance include use by a less experienced technician, as a tool to enrich the educational experience in a teaching environment, and as an adjuvant modality to confirm airway cannulation in patients with co-morbidities such as obesity, cervical spine injuries, or difficult anatomy.6,7 The routine use of flexible fiberoptic bronchoscopy during PDT lacks support by the most recent scientific evidence, and it is not recommended for or against by the latest critical care guidelines regarding tracheostomy.7 Despite the lack of scientific evidence, flexible fiberoptic bronchoscopy use during PDT is prevalent with rates as high as 97.7% as shown in a nationwide survey in German Intensive Care Units (ICU).8 Bronchoscopy guidance is traditionally performed using conventional bronchoscopes (CB). Following the development of the singleuse, disposable bronchoscope (SDB), questions have been raised regarding the quality, efficiency of use, and cost between these two bronchoscopy systems. SDB image quality is inferior to CB when

performing complex procedures such as visualization of bronchial hemorrhage; however, it is adequate for airway visualization in basic procedures such as bronchoalveolar lavage and PDT. 9,10 SDB allow for simultaneous or rapid succession bronchoscopies thus improving efficiency between procedures as well as prompt availability to accommodate the PDT surgeon’s schedule. Conversely, CBs provide excellent visualization but burden the hospital with turnover and costs associated with cleaning, procedure delays, and repair.

With advances in medical technology come increases in cost of care. The Centers for Medicaid and Medicare Services has projected that healthcare spending will be 19.9% of the United States Gross Domestic Product in 2022.11 According to the Healthcare Cost and Utilization project, in 2011 ICU charges accounted for half of total aggregate charges for hospitalizations that involved the ICU. Ninetythree percent of ICU necessity was related to respiratory disease with ventilator support.12 The looming financial burden has implored the medical community to provide cost-effective care. We performed a cost comparison between SDB and CB for our 20 bed Medical ICU over a 12-month time period.

Materials and Methods At our institution, the Otolaryngology and Pulmonary/Critical Care services assist one another during PDT. The surgeon enters the airway with a needle and, using Seldinger technique, inserts a wire and subsequent dilators to eventually place a tracheostomy tube.

Bronchoscopy performed simultaneously affords visualization of each of these steps. Our institution formerly used CB, specifically the Olympus® EVIS EXERA IIITM BF190, for all ICU bronchoscopy procedures, including PDT. In August of 2015, our 20 bed Medical ICU began using SDB by Ambu® with a reusable Ambu monitor for PDT. Our institution’s ICU Tower Endoscopy Log book was used to identify all bronchoscopies performed on the Medical ICU service from August 2015 to August 2016. All bronchoscopy-guided PDTs were performed using a SDB, while the CB was used for other diagnostic and therapeutic procedures. The two system variables were identified, compiled, and analyzed using Microsoft Excel (Table 1).

Concerning the SDB arm, the cost to the hospital for the Ambu® aScopeTM 3 was $274 per unit and the Ambu® aViewTM (portable monitor) was a fixed cost of $1495. The Olympus® EVIS EXERA IIITM BF190 bronchoscope costs were more complex to calculate, given the multiple use function. The upfront cost of a CB is approximately $30,000, but the allocation of cost is spread between multiple departments with most of the value allocated to the endoscopy lab and operating room for procedures that require CB for therapeutic interventions. The cost can also be spread out over the lifetime of the scope. For the purpose of this analysis, we decided to compare the SDB system to therapy provided by a single CB in the Medical

Table. Materials Number SDB PDT

34

CB

94

Total Bronchoscopies

128

SDB

Cost (Dollars)

Ambu® aScopeTM 3 (per unit)

274

Ambu® aViewTM (One time fixed cost)

1495

CB

Cost (Dollars)

Olympus® EVIS EXERA IIITM BF190 Bronchoscopes (per unit)

30,000

Usage ratio of scope (34/128)

7,968.75

Technician wage (hourly, one hour per scope)

13.53

Supplies per cleaning

6

Lease for service (yearly per scope)

2394 Time

Time for repair (days)

14-21

Time for sterilization (minutes)

60

SDB – Single-use, disposable bronchoscope, PDT – Percutaneous, dilatational tracheostomy, CB – Conventional bronchoscope

ICU. In other words, we set about comparing these two systems as if an institution were investigating adopting the SDB system versus purchasing an additional CB solely for ICU usage. Therefore, the ratio of SDB to total Medical ICU bronchoscopies was used as marker for the percentage of bronchoscopies that were amenable usage of SDB. The CB cleaning procedure time was 40 minutes once in the Steris® system. This time was rounded up to one hour given transit time and the human experience. The average hourly technician wage for cleaning a CB was $13.53 per hour. An additional $6 cost (previously determined by an institutional cost analysis) was applied to each individual use of CB to account for equipment service and cleaning fluids. Additionally, our hospital employs service contracts for the CB ranging from $2394-2889/scope/year. This contract provides full repair and preventative maintenance for all covered contracted scopes with no cap on expense of repair. Thus, it became unnecessary for us

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to compute the rate or type of damage to scopes, but we did include the fixed cost of the agreement in the cost of a CB. For the purposes of break-even analysis we used the lower cost lease in order to avoid bias. Repair times were estimated between 14-21 days. We made the assumption that an institution would have already incurred the cost of the portable endoscopy tower and computer software; thus, it was not included in the cost.

Results There were 34 bronchoscopies performed using SDB for PDT and 94 using CB for other purposes. The ratio of SDB use to total Medical ICU bronchoscopies was (34/128 or 26%). This definition allowed us to assign 26% of the upfront CB value ($7,968.75) as fixed costs in the CB arm. Break-even analysis demonstrates that the SDB is cost efficient until thirty-five PDT are performed (Figure). Based on our contracts and service agreements, the SDB arm appears cost-effective until more than $11,085 is spent at which point it becomes more economical to use CB. If we considered the higher cost repair lease, the break-even point shifts to thirty-seven PDT. This break-even point would be different for other institutions; however, a similar model may be applied with different ICU bronchoscope utilization rates, costs, contracts, etc.

Discussion For the purposes of the study, we used the ratio of SDB to total bronchoscopies in order to assign value to the fixed cost of CB. This was a close marker to approximate the cost of total bronchoscopies that could be attributed to and replaced by SDB at our institution on a one-time purchase basis. Another way of viewing this model is that 74% of CBs cannot be replaced by SDBs secondary to the need for complex diagnostic and therapeutic interventions. The calculated break-even point of 35 PDTs is highly dependent on the volume of

PDT to total ICU bronchoscopies as well as the fixed institutional costs such service contracts. In addition, we did not include the upfront cost of an endoscopy tower, cart, portable supplies, and computer software required for CB because we make the assumption an institution would already have these in place for current CB procedures.

We cannot overlook the reasonable and expected shortcomings of a physical logbook from which we determined the number and type of bronchoscopies. Errors, absent information, and altogether missing reports are an unfortunate reality with self-reporting. Most importantly, and perhaps most difficult to measure, are the differences of an SDB that make it more efficient to use for certain procedures.

They are readily available, easy to transport to bedside, require set up time less than 1 minute, and are operationally similar to CB; afterhours they increase the availability of the CB for emergent therapeutic necessities. The major disadvantages of SDB are associated with the economy of disposable technology. These scopes are diagnostic only due to limitations in screen size, suction strength, and excessive malleability of scope with prolonged procedures.

There are many limitations on our analysis, but finance necessitates pro forma analysis based on assumptions in order to determine return on investment. One such consideration is that identified real, capturable costs and overlooked missed opportunity costs associated with time lost during CB repair. CB damages during PDT are related to bronchoscope perforation by the PDT needle. CB damage may be as common as 14% during bronchoscopy-guided PDTs.6 Ideally, institutions would have service contracts that do not allow lapse in CB availability. Institutions must assess the associated financial burden related to cost of repairs (without a service contract) or missed opportunity costs when purchasing SDB versus an additional CB for ICU usage.

Figure. Bronchoscopy Procedure Cost

Cost (dollars)

Figure. Bronchoscopy Procedure Cost

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CB cost

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Conclusion As medicine strives to improve quality of care, institutions must thoroughly weigh the cost versus benefit of new technology with quality and efficiency of care. Just because we can use new technology does not mean that we should use new technology. Institutions must examine the multiple factors included in our model in performing their own cost analysis for purchasing a SDB system. Overcoming the financial burden of medicine is dependent on adequate and thorough value analysis in the context of quality, efficient care.

Acknowledgements Special thanks to Renee Brown Lee, RN, BSN, Daynelle H. Lee, RRT, Ashley Moore Hardy, BSN, RN, and Michelle Spera, MSN for their help and assistance gathering the financial data for this project.

This abstract was selected for poster presentation at the American Broncho-Esophagological Association meeting at the Combined Otolaryngology Spring Meetings on April 26-30, 2017 in San Diego, California. n References 1. Pratt LW, Ferlito A, Rinaldo A. Tracheotomy: historical review. Laryngoscope.

2008;118(9):1597-1606.

2. Hyde GA, Savage SA, Zarzaur BL, et al. Early tracheostomy in trauma patients saves time and money. Injury. 2015;46(1):110-114.

3.

Keeping A. Early versus late tracheostomy for critically ill patients: A clinical evidence synopsis of a recent Cochrane Review. Can J Respir Ther.

2016;52(1):27-28.

4. Johnson-Obaseki S, Veljkovic A, Javidnia H. Complication rates of open surgical versus percutaneous tracheostomy in critically ill patients. Laryngoscope.

2016;126(11):2459-2467.

5. Delaney A, Bagshaw SM, Nalos M. Percutaneous dilatational tracheostomy versus surgical tracheostomy in critically ill patients: a systematic review and meta-analysis. Crit Care. 2006;10(2):R55.

6. Jackson LS, Davis JW, Kaups KL, et al. Percutaneous tracheostomy: to bronch or not to bronch--that is the question. J Trauma. 2011;71(6):1553-1556.

7. Raimondi N, Vial MR, Calleja J, et al. Evidence-based guidelines for the use of tracheostomy in critically ill patients. J Crit Care. 2017;38:304-318.

8. Kluge S, Baumann HJ, Maier C, et al. Tracheostomy in the intensive care unit: a nationwide survey. Anesth Analg. 2008;107(5):1639-1643.

9. Marshall DC, Dagaonkar RS, Yeow C, et al. Experience With the Use of SingleUse Disposable Bronchoscope in the ICU in a Tertiary Referral Center of Singapore. J Bronchology Interv Pulmonol. 2017;24(2):136-143.

10. Zaidi SR, Collins AM, Mitsi E, et al. Single use and conventional bronchoscopes for Broncho alveolar lavage (BAL) in research: a comparative study (NCT 02515591). BMC Pulm Med. 2017;17(1):83.

11. Actuary Oot. National Health Expenditure Projections 2012-2022. In: Services Cf MaM, ed. cms.gov2012.

12. Barrett ML SM, Elixhauser A, Honigman LS, Pines JM. Statistical Brief #185: Utilization of Intensive Care Services, 2011. Healthcare Cost and Utilization Project: Agency for Healthcare Research and Quality; 2014.

Helping you build a more secure future.

We invest our own money alongside yours, so we are invested in your success.

Author Information Resident, Otolaryngology/Head and Neck Surgery Department (Robichaux).

Associate Professor. Pulmonology and Critical Care Department (Wilhelm).

Resident, Otolaryngology/Head and Neck Surgery Department (Moore). Associate Professor, Otolaryngology/Head and Neck Surgery Department (Jackson).

University of Mississippi Medical Center, Jackson. Conflict of Interest: The authors report no conflict of interest.

Corresponding Author: Cindy Moore, MD; 2500 N. State St., Jackson, MS 39211 Ph: (601) 815-3037. (cmmoore4@umc.edu).

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Familial Gigantiform Cementoma in Twins: A Case Report ANNA JADE HARTZOG, MD; DAVID KOWALCZYK, MD; JEFFREY D. CARRON, MD

Abstract Gigantiform cementoma is a rare form of fibro-cemento-osseous lesion that is usually inherited in an autosomal dominant fashion with variable penetrance; when occurring in families, it is known as familial gigantiform cementoma (FGC). The treatment for these lesions is individualized and ranges from conservative management to radical surgery. The literature supports complete removal whenever possible due to the high risk of recurrence. We present a case of a de novo mutation resulting in familial gigantiform cementoma in identical twin sisters that presented to our clinic secondary to massive mandibular and maxillary tumors. These patients have been managed with serial debulking, staged tooth extractions, orthodontics, and continued follow-up with conservative management.

Figure 1a, b. Preoperative CT Sinus and Postoperative CT Face without Contrast of Twin A.

Key Words: fibro-osseous lesion, familial gigantiform cementoma, de novo mutation, maxillary sinus infiltration, sinonasal tumor, twins Introduction Gigantiform cementoma is a rare, benign type of fibro-cementoosseous lesion affecting the mandible and maxilla that is often slow growing, multifocal, and expansile in nature, resulting in disruption of dentition and possible invasion local structures including the sinuses.1,2 There have been reports of gigantiform cementomas occurring in families known as familial gigantiform cementoma (FGC) that often present with an autosomal dominant inheritance with variable phenotypic expression.3, 4 The prevalence of these lesions is more common in Caucasians but equal between sexes 3, 4 We present a case of de novo mutation FGC in twin girls that resulted in mandibular tumors and nasal and maxillary sinus obstruction. In each patient, serial surgeries, continued follow-up, and orthodontics have helped minimize morbidity while preserving function.

Case Presentations Twin sisters with history of recurrent cementifying fibromas of the jaw requiring a total of 16 prior surgeries at 6-month intervals since age 8 years were referred by an oral surgeon to our Pediatric Otolaryngology clinic at age 12.

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Admission CT Sinus showing coronal view of Gigantiform Cementoma filling maxillary sinuses in twin A (a); Postoperative CT Face without contrast showing coronal view of postsurgical changes from debulking as well as residual lesions involving the bilateral maxillae and mandible with extension into the alveolar ridges. Osteogenesis is present along the sinus walls.

Figure 2a, b. Preoperative CT Face with Contrast and Postoperative CT Face without Contrast of Twin B.

Preoperative CT Face with contrast of Twin B showing coronal view of right and left maxillary sinus lesions (a); Postoperative CT Face without contrast of Twin B showing residual maxillary and mandibular lesions with postoperative debulking changes (b).

Twin A originally presented for evaluation of nasal congestion, reporting worse congestion on the right, chronic mouth breathing, difficulty sleeping with witnessed apnea, intolerance of harsh chemicals such as bleach or paint, heat intolerance, and shortness of breath regardless of activity. In addition, she reported clear drainage and inability to blow her nose and denied relief with antibiotics or steroid treatments. Of note, she underwent dental surgery a month prior to presentation for what was believe to be recurrent cementifying fibroma with possible tumor infiltration into sinus cavities. On physical exam, the nares and mucosa were normal without drainage but with occlusion of the right nostril by a bulging inferior turbinate. Flexible nasopharyngoscopy was notable for a significant, deforming mass occupying the majority of the right nasal cavity with compressive effects on the septum.

Preoperative CT sinus of twin A showed large, expansile masses of mixed density containing moderate calcifications as well as soft tissue extending from the hard palate and filling bilateral maxillary antra with elevation of the orbital floors (Figure 1a). Imaging also showed a large partially visualized lytic lesion of the angle and body of the left mandible. Preoperative CT Face with contrast of twin B showed bilateral 4.5 cm maxillary sinus masses and additional smaller fibromas of the mandible (Figure 2a). The differential for these masses included cemento-ossifying fibromas or other fibro-osseous lesions. Surgical excision of the masses for both twins was performed via a CaldwellLuc incision combined with endoscopic technique, and both were discharged home with antibiotics, pain medication, and saline nasal irrigations.

Twin B presented 1 month after twin A with complaints of cheek swelling without sinus symptoms. She also reported a recent ear infection diagnosed at an outside center where she was told that the cheek pain and swelling were referred from this infection. Physical exam was benign except for left inferior turbinate impingement on the septal wall without drainage.

Twin A continued to have right maxillary and eye pain and copious green nasal discharge post-surgery, requiring extended antibiotic treatment and eventual endoscopic debridement of the right maxillary and ethmoid cavities, left turbinectomy, and left nasal debridement.

Post-surgical imaging showed post-surgical debulking changes, lesions involving bilateral maxillae and mandibles with extension into the

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Figure 3a,b. Histological Photomicrographs of Twin A’s lesions.

H&E stains of Twin A’s lesions showing whorls of spindle cells (arrowhead) in islands of concentrically laminated basophilic cementum (arrow) at 100x (a) and 200x (b).

alveolar ridges, and bilateral enlargement of the lesions in the lateral maxillary sinus walls (Figure 1b).

Twin B underwent excision curettage of the right-sided mandibular tumor via endoscopic approach, extraction of 5 molars, bilateral maxillary antrostomy, and excision of 3 pigmented nevi on the central back in addition to procedure discussed above. She also required endoscopic debridement of bilateral maxillary cavities and right mandibular cavity after her initial surgery. Post-operative imaging showed postsurgical changes with residual lesions in bilateral maxillae unchanged in size but with minor changes in density as well as osseous healing of the right mandibular surgical site (Figure 2b).

She complained of a chipped tooth at a follow-up appointment, but otherwise, both twins only had minor complaints at each visit. Of note, each girl has had unilateral nasolacrimal duct stents placed at surgery due to epiphora from mechanical blockage.

Both patients were presented at our multidisciplinary Head and Neck Tumor conference. Histology from both patients revealed whorls of spindle cells in islands of concentrically laminated basophilic cementum and fragments of bone marrow spaces containing fibrous tissue (Figures 3a, b), and our oral pathologist confirmed the diagnosis as FGC. It was recommended that the patients receive further genetic testing and continue conservative debridement and teeth extraction as needed without proceeding with aggressive surgical resection.

Genetics evaluation revealed a balanced 1;13 translocation in the tumors from both sisters; the mother and father tested negative for

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this mutation, and a younger sister is unaffected. This mutation was deemed a de novo mutation, and further genetic testing is in progress.

Both patients continue to be followed via a multi-specialty approach including Pediatric Otolaryngology, Facial Plastic Surgery, Oral and Maxillofacial Surgery, Genetics, and an Orthodontist. All treatment teams agree on close follow-up with continued serial debulking, staged dental extractions, and orthodontics as needed, as radical resection would require removal of most of the hard palate and mandible.

Discussion Our twin patients presented at age 12 with highly advanced disease resulting from a de novo mutation causing a rare form of osseous dysplasia known as FGC that involved the mandible, maxilla, and maxillary sinuses. To our knowledge, this is the only case of de novo FGC reported in twin siblings. However, there have been reports of FGC in other families in the literature with discussion of the varying characteristics of this disease.2, 4-8 One such characteristic is that of 3 different growth phases of lesions, including initial onset, rapid expansion, and suppression of growth.2, 6 Our patients were treated by an outside provider during the initial onset and rapid expansion phases that required multiple, serial surgeries.

After aggressive treatment following erroneous diagnosis of ossifying fibroma, the tumor growth has significantly slowed. Due to this slowed growth and timely patient follow-up, all treatment teams involved agreed upon close follow-up with serial debulking, staged dental extractions, and orthodontics. With this plan, the patients’ functional

concerns are addressed without extensive measures and, in turn, can help prevent the need for a radical surgery.

Radiologically and histologically, the lesions in FGC have distinctive features. In the early stages of growth, FGC radiologically resembles other osseous dysplasias by demonstrating a mixture of radiolucent and radiopaque appearance. However, as the lesions mature, they become more opacified in appearance and can cause disruption of dentition and invade local structures. Once mature, the growth of these lesions stabilizes and the radiology demonstrates central radiopaque lesions with a radiolucent rim.9 Our patients demonstrated these radiological findings as well as the invasive nature of this disease as described in the literature. Histologically, these lesions have been described as having whorls of spindle cells with basophilic acellular lamellated masses of calcified tissue resembling cementum, which our patients also demonstrated.10 In addition, there have been reports of pathological bone fractures in patients with FGC.3, 11 Because of the disruption of dentition, our patients have continued to see an orthodontist and oral surgeon with efforts taken to preserve dentition if possible and perform staged dental extractions when necessary. It is likely that all of their molars will eventually be lost and one girl may need segmental mandibulectomy with reconstruction due to excessive cortical thinning.

Fibro-osseous lesions, including FGC, vary clinically, and therefore, the treatment for such lesions is individualized.12 In many case reports and studies within the literature, the treatment options range from conservative measures including observation and follow-up to complete radical resection of lesions with possible use of bone flap for reconstruction.1,2,9,10,12 In a study by Finical, et al., the average number of surgeries to treat the studied patients was seven with multiple surgeries required regardless of symptomatic versus end-stage treatment, and the authors recommend against simple recontouring of the tumors due to recurrence.2 In another study by Noffke, et al., recontouring is also rejected as sufficient treatment, and complete surgical resection of progressive expansile FGC lesions to limit multiple surgeries and further deformity is supported.9 Kumar, et al. presents a patient who underwent continued follow-up for 3 years without need for radical surgery, posing this as an acceptable option.10 With surgical removal of these lesions, recurrence is almost inevitable if incomplete removal is performed, and the benefits versus risks of surgical treatment compared to conservative management must be considered.1,2,9,10,12 In conclusion, we present a rare case of de novo FGC in a set of twin girls. The lesions in these girls began around the age of 8 years of age and required multiple debulking surgeries and eventual referral to our clinic for extensive disease of the upper and lower jaws causing maxillary sinus obstruction, sleep apnea, and nasolacrimal duct obstruction. Both patients’ disease has been controlled through serial debulking measures, staged tooth extractions, orthodontics, and close

follow-up. Further genetic studies are in process. r References 1. Abdelsayed RA, Eversole LR, Singh BS, Scarbrough FE. Gigantiform cementoma: clinicopathologic presentation of 3 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2001 Apr;91(4):438-44.

2. Finical SJ, Kane WJ, Clay RP, Bite U. Familial Gigantiform Cementoma. Plast Reconstr Surg. 1999 Mar;103(3):949-54.

3. Şakar O, Aren G, Mumcu Z, Ünalan F, Aksakallı N, Tolgay CG. Familial gigantiform cementoma with Ehlers - Danlos syndrome: A report of 2 cases. J Adv Prosthodont.

2015 Apr;7(2):178-82. doi: 10.4047/jap.2015.7.2.178. Epub 2015 Apr 23.

4. Young SK, Markowitz NR, Sullivan S, Seale TW, Hirschi R. Familial gigantiform cementoma: classification and presentation of a large pedigree. Oral Surg Oral Med Oral Pathol. 1989;68:740–7.

5. Şakar O, Aren G, Mumcu Z, Ünalan F, Aksakallı N, Tolgay CG. Familial gigantiform cementoma with Ehlers - Danlos syndrome: A report of 2 cases. J Adv Prosthodont.

2015 Apr;7(2):178-82. doi: 10.4047/jap.2015.7.2.178. Epub 2015 Apr 23.

6. Wang HW, Yu M, Qin XJ, Zhang CP. Familial gigantiform cementoma: distinctive clinical features of a large Chinese pedigree. Br J Oral Maxillofac Surg. 2015 Jan;53(1):83-5. doi: 10.1016/j.bjoms.2014.09.013. Epub 2014 Oct 3.

7. Oikarinen K, Altonen M, Happonen RP. Gigantiform cementoma affecting a Caucasian family. Br J Oral Maxillofac Surg. 1991 Jun;29(3):194-7.

8. Rossbach HC, Letson D, Lacson A, Ruas E, Salazar P. Familial gigantiform cementoma with brittle bone disease, pathologic fractures, and osteosarcoma: a possible explanation of an ancient mystery. Pediatr Blood Cancer. 2005 Apr;44(4):390-6.

9. Noffke CE, Ngwenya SP, Nzima N, Raubenheimer EJ, Rakgwale NB. Gigantiform cementoma in a child. Dentomaxillofac Radiol. 2012 Mar;41(3):264-6. doi: 10.1259/dmfr/13435626.

10. Kumar VV, Ebenezer S, Narayan TV, Wagner W. Clinicopathologic conference: multiquadrant expansile fibro-osseous lesion in a juvenile. Oral Surg Oral Med Oral Pathol Oral Radiol. 2012 Mar;113(3):286-92. doi: 10.1016/j.tripleo.2011.08.021.

11. Moshref M, Khojasteh A, Kazemi B, Roudsari MV, Varshowsaz M, Eslami B. Autosomal dominant gigantiform cementoma associated with bone fractures. Am J Med Genet A. 2008 Mar 1;146A(5):644-8. doi: 10.1002/ajmg.a.32171.

12. Kumar KA, Kishore PK, Mohan AP, Venkatesh V, Kumar BP, Gandla D. Management and treatment outcomes of maxillofacial fibro-osseous lesions: A retrospective study. J Maxillofac Oral Surg. 2015 Sep;14(3):728-34. doi: 10.1007/ s12663-014-0726-5. Epub 2014 Nov 16.

Author Information University of Mississippi School of Medicine graduate (May 2016), Anesthesiology resident, Vanderbilt University Medical Center, Nashville, Tennessee (Hartzog).

Fifth year Otolaryngology resident, University of Mississippi Medical Center, Jackson (Kowalczyk). Professor of Pediatric Otolaryngology, University of Mississippi Medical Center, Jackson (Carron). The authors have no financial disclosures or conflicts of interest to disclose.

Corresponding Author: Anna Jade Hartzog, MD; Department of Anesthesiology, Vanderbilt University Medical Center, 1211 Medical Center Dr., Nashville, TN 37232.

Ph: (662) 295-5233. (anna.j.hartzog@vanderbilt.edu).

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General Anesthesia without the Use of Muscle Relaxant for a Patient with Myasthenia Gravis and Multiple Co-morbidities: A Case Report and Literature Review RACHEL ROBERTS, MD; NATESAN MANIMEKALAI, MD

Abstract Myasthenia gravis is an autoimmune disorder caused by autoantibodies to nicotinic acetylcholine receptors. Myasthenia gravis patients have weakness in skeletal muscles which improve with rest. The decrease in muscle strength can create a unique difficulty during anesthesia, particularly general anesthesia. We present a patient with myasthenia and multiple comorbidities who tolerated a general anesthetic without the use of muscle relaxants and, thus, was able to be extubated at the end of the surgical case.

Keywords: Myasthenia gravis, autoimmune disorders, general anesthesia Introduction Myasthenia gravis is an autoimmune disorder characterized by symptoms of weakness and fatigability of skeletal muscles with improvement at rest. The disorder is caused by antibodies to postsynaptic nicotinic acetylcholine receptors; these antibodies cause a reduction in the absolute number of functional receptors.1 Muscle weakness can be confined to the external ocular muscles or it can be widespread including problems with limb muscle weakness, bulbar symptoms, and respiratory muscle weakness leading to respiratory failure.1 Despite advancing diagnostic and treatment techniques, myasthenia gravis remains a challenge to anesthesiologists. Concerns include a range of issues from mild weakness to severe post-operative respiratory failure. Many intraoperative medications, including muscle relaxants, worsen skeletal muscle weakness in these patients. In the present article, we discuss avoiding muscle relaxants during a general anesthetic to decrease muscle weakness exacerbation in a myasthenia gravis patient.

Case Presentation A 42-year-old African American female was scheduled for laparoscopic sacrocolpopexy, a surgery performed for pelvic organ prolapse.

She had a past medical history of myasthenia gravis, systemic lupus

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Table 1. Types of Myasthenia Gravis Depending upon the Skeletal Muscles Affected

Table 1. Types of Myasthenia Gravis Depending upon the Skeletal Muscles Affected Type of Myasthenia Gravis

Symptoms

Ocular Myasthenia Gravis

Extra ocular movements are affected but the pupils remain intact. Patients have rapidly progressing double vision and/or ptosis which is usually symmetric and bilateral. It is worse in the evening and improves with rest.

Bulbar Myasthenia Gravis

This type of myasthenia affects the muscles of mastication. These patients have trouble swallowing and have frequent episodes of choking. The dysphagia is worse for liquids than solids. Anorexia may be the presenting symptom.

Generalized Myasthenia Gravis

Generalized myasthenia starts with ocular symptoms then affects proximal limb muscles.

Table 2. Types of Myasthenia Gravis Depending on Age of Onset Table 2. Types of Myasthenia Gravis Depending on Age of Onset Type of Myasthenia Gravis Neonatal Myasthenia Gravis

Congenital Myasthenia Gravis

Myasthenia Gravis in Children Adult Myasthenia Gravis Myasthenia Gravis in the elderly

Symptoms Neonatal myasthenia gravis is due to placental transfer of antibodies from an affected mother. If this occurs, the baby is born with the inability to suck.

Congenital myasthenia gravis is an autosomal recessive inherited disease. It occurs in children aged 0 to 2 years of age.

Myasthenia gravis in children is rare and can be difficult to diagnose. It has a female predominance and occurs in children aged 0 to 20 years of age.

Adult myasthenia occurs during the ages 20 to 40.

It is the most severe type of myasthenia.

The ocular and bulbar musculature are affected and this type of myasthenia tends to respond well to steroids and immunosuppression.

erythematosus, hypertension, inferolateral myocardial infarction four years prior, asthma, obstructive sleep apnea, obesity, gastroesophageal reflux, thyroid goiter, multiple strokes in 2003 and 2006 with residual right sided weakness, deep vein thrombosis in 2012 to the right lower extremity, and anxiety. Her present symptoms included dyspareunia, urinary incontinence, and pelvic pain secondary to pelvic organ

prolapse. She was scheduled for surgical correction with vaginal graft excision combined with pelvic reconstruction with laparoscopic sacrocolpopexy, cystoscopy, and perineorrhaphy.

Table 3. Drugs that Worsen Weakness in Patients with Myasthenia Gravis Table 3. Drugs that Worsen Weakness in Patients with Myasthenia Gravis Neuromuscular blocking agents

Lab values were within normal limits. Her EKG showed normal sinus rhythm. Recent stress echocardiogram showed no evidence of exercise induced ischemia.

On physical examination, the patient was 5’5” and weighed 83kg. She had residual weakness on her right side secondary to her previous stroke. Her airway exam showed a Mallampati score of II, thyromental distance of >3 finger breaths, and good range of motion of her neck.

She had partial dentures, which were removed prior to surgery.

Current medications included pyridostigmine 60mg four times daily, mycophenolate 500mg twice a day, atorvastatin, ranitidine, losartan, hydroxychloroquine, cyclobenzaprine, clopidogrel, carvedilol, aspirin, and albuterol. She was previously on steroids but stopped one week prior to surgery.

Although regional anesthesia is considered to be an excellent option in patients with myasthenia, we were unable to perform regional anesthesia because of the surgical procedure. General anesthesia without the use of neuromuscular blocking drugs both for induction and maintenance was planned and the surgeon agreed to that. The anesthetic plan was discussed in detail with the patient along with the possibility of remaining intubated and staying in intensive care unit post-operatively. The patient understood the risks and agreed to proceed with surgery.

On the day of surgery, the patient took her daily dose of pyridostigmine 60mg at 0600, which is recommended for myasthenic patients undergoing surgery. Preoperatively, she received 4mg of midazolam for anxiety. She was then induced with lidocaine 50mg, fentanyl 200mcg, propofol 200mg and intubated without any muscle relaxant. Induction and intubation were uneventful. 100mg of hydrocortisone was given shortly after induction as stress dose steroid, since the patient had been on steroid intermittently prior to surgery. Antibiotics included 500mg metronidazole and 2g cefazolin, which were requested by the surgical team. No neuromuscular blocking drug was used throughout the case.

The surgery proceeded without complications and the patient remained stable throughout the intra-operative period. The total length of surgery was four hours. During emergence, the patient resumed spontaneous respirations, sustained head lift, and obeyed verbal commands. She was extubated in the operating room and transferred to the post-anesthesia care unit. She was breathing spontaneously with normal vitals signs.

She received her next scheduled dose of pyridostigmine in the PACU. She did not require any ventilator support/intubation or transfer to

Antibiotics

Aminoglycosides, particularly gentamycin Macrolides, particularly erythromycin and azithromycin

Cardiovascular Agents

Beta blockers Calcium channel blockers Procainamide Quinidine Quinine

Other Medications

Magnesium Antacids, laxatives, tocolytics Iodinated contrast agents D-penicillamine

the intensive care unit. She was sent to the post-operative floor and was discharged home on the second post –operative day in stable condition.

Discussion Myasthenia gravis is an autoimmune disorder caused by auto-antibodies to nicotinic acetylcholine receptors characterized by symptoms of weakness and fatigability of skeletal muscles with improvement after rest. Muscle weakness can be confined to the external ocular muscles or it can be widespread including limb weakness, bulbar symptoms, and respiratory weakness. This skeletal muscle weakness can create difficulty during extubation under general anesthesia, especially when combined with neuromuscular blocking drugs.1 Types of Myasthenia Gravis. The classification of Myasthenia Gravis is dependent on age of onset, skeletal muscles affected and thymic abnormalities. There are three main types of myasthenia gravis (Table 1). 2 The onset of myasthenia gravis is typically in young adults, especially women. It can, however, occur in neonates, children, and the elderly (Table 2).2 Myasthenic crisis vs. Cholinergic crisis. Myasthenic patients are at risk for developing two different types of crises: myasthenic crisis and cholinergic crisis.3 Myasthenic crisis is an exacerbation of the disease; it can be precipitated by poor control of myasthenia gravis, stress, hyperthermia, and infections. It is characterized by severe muscle weakness which can eventually lead to respiratory failure.

Major predictors for death in these patients are the patient’s age, time to recognition of crisis, and the need for endotracheal intubation.4

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The management for this crisis is additional doses of intravenous cholinesterase inhibitors and possibly intravenous immunoglobulins or plasmapheresis.

Cholinergic crisis occurs when the patient is overdosed with cholinesterase inhibitors. Symptoms include excessive salivation, sweating, abdominal cramps, bradycardia, and muscle weakness.3 Treatment includes atropine, cessation of cholinesterase inhibitors, and intubation. Distinguishing between myasthenic and cholinergic crisis is done by giving a single dose of edrophonium. If the patient has myasthenic crisis, the symptoms will improve, but symptoms will worsen if the patient is having a cholinergic crisis.3 Treatment of Myasthenia Gravis. Medical treatment includes cholinesterase inhibitors such as pyridostigmine, steroids, immunosuppressants such as azathioprine, intravenous immunoglobulin and plasmapheresis in severe cases. Myasthenia gravis is associated with enlarged thymus gland, and thymectomy may improve symptoms. Although the mechanism is not completely clear, antibodies decrease after surgery and patients have clinical improvement.

Challenges in Anesthesia in a Patient with Myasthenia Gravis.

Preoperatively, patients with myasthenia gravis should be assessed for any exacerbation of signs or symptoms and specific attention should be given to respiratory muscle strength, which may include pulmonary function tests. These can be used as a reference, to determine the optimal condition for extubation, although bulbar signs and symptoms are considered primary risk for respiratory failure postoperatively.1 These patient should be advised to continue pyridostigmine and steroid medications until the morning of surgery; it should then be continued intra-operatively either via intravenous or through nasogastric tube.

The four pre-operative criteria that correlate with the need for postoperative ventilatory support include disease duration of greater than 6 years, presence of respiratory issues such as chronic obstructive pulmonary disease, pyridostigmine dose of greater than 750mg per day, and preoperative vital capacity of less than 2.9L.5 These four criteria should be assessed carefully and the risk of post-operative complications explained to the patient. Elective surgeries should be performed during a stable phase of myasthenia gravis, while the patient is on a lower dose of pyridostigmine and minimal or no steroids.1 Patients undergoing emergent surgery can be optimized with plasma exchange or intravenous immunoglobulin.

Intra-operatively it is advisable to omit or reduce the use of muscle relaxants and drugs that can reduce neuromuscular transmission and increase weakness (Table 3).6 With general anesthesia, potent inhalational anesthetics such as isoflurane and sevofluorane can lead to increased muscle relaxation.1 Myasthenic patients are sensitive to non-depolarizing neuromuscular blockers and resistant to depolarizing agents, requiring up to 2.6x the dose of non-myasthenic patients.7 If intra-operative muscle relaxation is necessary, only small doses of short acting non-depolarizing

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muscle relaxants should be used. Reversal of paralytics with acetylcholinesterase inhibitors such as neostigmine can be less effective in myasthenia gravis patients taking pyridostigmine due to chronic inhibition of this enzyme. In our case, muscle relaxants were avoided altogether, which allowed us to extubate the patient in the operating room and decrease the risk of prolonged ICU stay.

Regional or local anesthesia may be a good alternative to general anesthesia in patients with myasthenia gravis. However, if ester group local anesthetics are used the dose may need to be reduced as esters are degraded by plasma cholinesterases and patients with MG are likely receiving anti-cholinesterase drugs.5 When epidural and spinal anesthesia are used, the anesthetic level must be monitored closely to prevent a high block level that could weaken accessory respiratory muscles.5 As stated previously, hyperthermia and stress can increase the risk for myasthenic crisis and should be avoided. Post-operative management includes adequate pain control, pulmonary hygiene, and avoiding drugs that cause muscle weakness.1 Conclusion Myasthenia gravis is an autoimmune disease with many implications for physicians, especially anesthesiologists. There is high potential for skeletal muscle weakness and respiratory failure, especially with general anesthesia. Our case report showed that a patient with myasthenia gravis with multiple co-morbidities was successfully managed by avoiding the use of neuromuscular blocking drugs. This allowed us to extubate at the end of surgery and avoid postoperative intubation and intensive care stay. In addition to the multidisciplinary team effort, proper pre-operative and intra-operative planning helped us to manage this high-risk patient. r References 1. Jamal BT, Herb K. Perioperative management of patients with myasthenia gravis: prevention, recognition, and treatment. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2009; 107:612.

2. Oger J, Frykman H. An update on laboratory diagnosis in myasthenia gravis. Clin Chim Acta. 2015; 449:43-8.

3. Blichfeldt-Lauridsen L, Hansen BD. Anesthesia and myasthenia gravis. Acta Anaesthesiol Scand. 2012; 56(1):17-22.

4. Alshekhlee A, Miles JD, Katirji B, Preston C, Kaminski HJK. Incidence and mortality rates of myasthenia gravis and myasthenic crisis in US hospitals.

Neurology 2009; 72: 1548–1554.

5. Ceremuga MAJ TE, Yao XL, McCabe JT. Etiology, mechanisms, and anesthesia implications of autoimmune myasthenia gravis. AANA Journal. 2002; 70:301-310.

6. Jeul VC. Myasthenia Gravis: Management of myasthenic crisis and perioperative care. Semin Neurol. 2004; 24(1):75-81.

7. Abel M, Eisenkraft JB. Anesthetic implication of MG. Mt Sinai J Med. 2002;69:31–37.

Author Information Anesthesiology Resident, PGY-4/CA-3 (Roberts). Associate Professor of Anesthesiology (Manimekalai). University of Mississippi Medical Center.

Authors report no conflicts of interest.

Corresponding Author: Dr. Natesan Manimekalai, University of Mississippi Medical Center, Department of Anesthesiology, 2500 N State Street, Jackson MS 39216. Ph: (601)984-5900 Fax: (601)984-5939 (nmanimekalai@umc.edu).

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TOPICS:

Antibiotic Stewardship | Alzheimer’s | Hypertension | Pre-Diabetes | Osteoporosis | Chronic Kidney Disease | Cervical & Upper Extremity Sports Injuries | Post Care of Transplant Patients | Pediatrics | Atrial Fibrillation | MACRA Update | MSMA Update | Anti-Stigma | MSBML New Rules & Regulations | Trauma and Prescribing | Suboxone Prescribing | Safe Prescribing for Chronic Pain

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Top 10 Facts You Should Know About Bullous Skin Disorders: Dermatopathologic Approach BONNIE D. HODGE; ROBERT T. BRODELL, MD The vesicobullous diseases are characterized by the size of their primary lesions. “Bullae” (adjective “bullous”) are large blisters and “vesicles” (adjective vesicular) are smaller fluid-filled lesions. These Figure 1. primary lesions are further characterized by the depth of the blister within the skin. Some blisters occur at various levels within the epidermis and others at the dermal-epidermal junction.1 Finally, these lesions are characterized by their time of onset as either congenital or acquired. Thus, blisters may occur in a wide variety of clinical settings including drug reactions, autoimmune disorders, arthropod bites, genetic disorders, and physical injury.2

1

Diagnostic tests are essential in determining the etiology of most blistering diseases.

The most commonly employed tests are skin biopsy with light microscopy and direct immunofluorescence (DIF), antigen-specific serologic testing, and microbiologic studies.2 The selection of appropriate tests is patient-specific and should be based on the differential diagnosis generated by the clinical presentation.

The epidermis overlying this dermalIn patients with vesiculobullous eruptions, young blisters should be chosen for biopsy. epidermal blister is entirely necrotic and Regenerative and necrotic changes over time may alter the histological appearance of the blister.1 there is little underlying inflammation First, as fluid accumulates in the vesicle and lifts the blister roof from the floor, the roof may become in this patient with toxic epidermal necrotic and lead to diagnostic confusion (Figure 1). Secondly, in subepidermal blistering processes, necrolysis (hematoxylin & eosin, 100x).

2

re-epithelialization of the blister floor may occur as early as 48 hours after blister formation.3 Therefore, in older subepidermal lesions the histopathologic appearance can mimic an intraepidermal blistering process (Figure 2). This leads to the inappropriate consideration of an entirely separate subset of diseases. Figure 2.

3

In suspected primary blistering diseases, two biopsy specimens should be obtained for hematoxylin & eosin staining and DIF studies.4 Alternatively, a 6 mm punch biopsy at the edge of the blister can be bisected at the bedside to reveal the take-off point of the blister.5 Light microscopy of H&E stained formalin fixed tissue will demonstrate the level of the split, the presence of a predominant inflammatory infiltrate, markers of infection, and presence of dyskeratotic or acantholytic cells.2 DIF allows the visualization of immune reactants deposited in primary blistering processes (Figures 3 and 4).6

Tables:

Table1.1.Differential Differential Diagnoses Diseases Table DiagnosesforforBullous Bullous Diseases

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Re-epithelialization at the base of a dermal-epidermal blister in a biopsy from a long-standing blister in a patient with pemphigoid. This leads to the appearance of an intraepidermal blistering process. The roof is also necrotic owing to the long-standing nature of this blister, not the primary pathophysiologic basis of pemphigoid (hematoxylin & eosin, 100x).

Figure 3.

Table 2.

2. Differential Differential Diagnoses Blistering Processes Table DiagnosesforforSubepidermal Subepidermal Blistering Processes

Direct immunofluorescence demonstrates a linear band of IgG at the dermal epidermal junction typical of bullous pemphigoid in this elderly patient with widespread tense blisters.

Figure 4.

Direct immunofluorescence demonstrates a “chicken-wire” appearance in this middle-aged patient with pemphigus vulgaris who presented with widespread flaccid bullae.

Figure 5.

This intraepidermal blister demonstrates acantholysis, dyskeratosis (necrotic keratinocytes) and multinucleated giant cells typical of Herpes infections (hematoxylin & eosin, 100x).

4

When taking a biopsy for DIF, sampling nonbullous, perilesional skin within 1-2 cm of the lesion Figure 6. yields the best results.7,8,9 Biopsies taken from the bulla or non-bullous skin farther away from the lesion are associated with higher rates of false negatives.9 If a biopsy is taken at the “take-off ” point of the blister, 70-80% of the specimen should be perilesional to prevent the loss of the epidermis when the lesion is bisected at the bedside.

5

Determining the anatomical level of the split provides important diagnostic information in vesiculobullous diseases.

There are four critical levels of blistering: 1) subcorneal; 2) within the spinous or malpighian layers; 3) suprabasilar; and, 4) subepidermal. A subcorneal split occurs within the stratum corneum or just beneath it. A split within the malpighian layer is termed an intraepidermal blister and most often occurs as a result of spongiosis or ballooning degeneration (Figure 5).

On the other hand, a suprabasilar split occurs as a result of acantholysis (Figure 6). Finally, a subepidermal split occurs at the dermal-epidermal junction; diagnosis is generally based on the predominant inflammatory cell type in the vesicle fluid.1 Table 1 demonstrates the differential diagnosis for each respective split level.

6

There are several mechanisms of action that can lead to splitting within the epidermis: spongiosis, acantholysis, and ballooning degeneration of the keratinocytes. Intercellular edema is the defining characteristic of spongiosis. In some cases, the edema is so pronounced that it leads to breakdown of intercellular connections and subsequent vesicle formation. Acantholysis is defined by the loss of attachments between keratinocytes as a result of either autoimmune destruction or hereditary dysfunction. The

Suprabasal acantholysis without dyskeratosis typical of pemphigus vulgaris (hematoxylin & eosin, 100x)

Figure 7.

The stratum corneum has fallen away leaving a denuded surface in this biopsy of a flaccid bulla. Note the acantholytic cells “floating” at the surface secondary to dissolution of the cement substance holding cells together in pemphigus foliaceus (hematoxylin & eosin, 100x).

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disconnected cells subsequently appear rounded and float within the blister fluid (Figure 7). Ballooning degeneration refers to the swelling of keratinocytes observed most often in the context of viral infection of the cells. The swelling causes loss of desmosomal attachments and resulting vesicle formation.1

Figure 8.

7

Consideration of the predominant inflammatory cell type observed in subepidermal blisters can help the clinician form a differential diagnosis. Depending on the etiology of a subepidermal blister, several inflammatory cell types will predominate in the underlying dermis.

Table 2 shows the differential diagnosis for each subcategory of inflammatory cell type. A 6 or 8 mm punch biopsy at the blister edge, oriented and bisected at the bedside will reveal the anatomical level of the split and the inflammatory infiltrate from the blister edge.9

8

Bullous Pemphigoid (BP) is the most common autoimmune bullous disease. It presents with widespread tense, fluid-filled blisters most commonly in the elderly (Figure 8). The histological hallmark is a subepidermal blister with eosinophils (Figure 9). BP sometimes presents with urticarial or eczematous eruptions in a prodromal phase without any bullous lesions,10 or it may present with both (Figure 10). Consider BP as a diagnosis in any elderly patient with a chronic, pruritic eruption.

This patient demonstrates the classic tense, fluid-filled blisters seen in bullous pemphigoid.

9

Figure 9.

Nikolsky sign is a useful clinical test to differentiate pemphigus vulgaris from bullous pemphigoid. This sign is elicited by rubbing non-blistered skin. Minor friction results in blister formation in patients with a positive Nikolsky sign providing evidence of an intraepidermal blistering process such as pemphigus or a subepidermal blistering process in the case of toxic epidermal necrolysis. A negative result is typical of subepidermal blistering diseases such as bullous pemphigoid, linear IgA bullous dermatosis, or blisters in porphyria cutanea tarda. When the sign is elicited in normal skin in the vicinity of the blister, it is called marginal Nikolsky. When elicited away from the blister, it is called direct Nikolsky.11

10

Corticosteroids are the mainstay for the acute treatment of most bullous diseases, especially those of autoimmune etiology. Both topical and systemic steroids are used in these patients. Steroids are known to improve the clinical symptoms of bullous diseases by inhibiting the secondary inflammatory effects. Potential benefits include relief of blistering and pruritus.12 Of course, the use of long-term systemic steroids is problematic and a variety of adjunctive treatments are used to wean patients from their steroids depending upon the specific disease process. r References

1. Patterson JW. The vesiculobullous reaction pattern. In: Weedon’s Skin Pathology. Vol Fourth. Edinburgh: Elsevier Churchill Livingston; 2015:50-57.

2. Hull C, Zone, J. Approach to the patient with cutaneous blisters. Post TW, ed. UpToDate. Waltham, MA: UpToDate Inc. http:// www.uptodate.com (Accessed on November 8, 2017.)

3. Zappi E, Zappi E. Inflammatory Cutaneous Lesions. In: Dermatopathology. Vol Springer London; 2013:59-114.

4. Rastogi V, Sharma R, Misra SR, Yadav L. Diagnostic procedures for autoimmune vesiculobullous diseases: A review. J Oral Maxillofac Pathol. 2014;18:390-7 5. Braswell M, N Mc Cowan, J Schulmeier, RT Brodell. High Yield Biopsy Technique for Subepidermal Blisters. Cutis. 2015,95:237-40.

6. Mascaró J, JoseM. Histological and Immunofluorescence Diagnosis of Autoimmune Blistering Diseases. In: Murrell DF, ed.

Blistering Diseases. Vol Springer Berlin Heidelberg; 2015:161-191.

7. Seishima M, Izumi T, Kitajima Y. Antibody to bullous pemphigoid antigen 1 binds to the antigen at perilesional but not uninvolved skin, in localized bullous pemphigoid. Eur J Dermatol. 1999;9:39-42.

8. Sladden C, Kirchhof MG, Crawford RI. Biopsy location for direct immunofluorescence in patients with suspected bullous pemphigoid impacts probability of a positive test result. J Cutan Med Surg. 2014;18:1-5.

9. Elston DM, Stratman EJ, Miller SJ. Skin biopsy: Biopsy specific issues in specific diseases. J Am Acad Dermatol. 2016;74(1):1-16.

10. Di Zenzo G, Della Torre R, Zambruno G, Borradori L. Bullous pemphigoid: From the clinic to the bench. - Clin Dermatol. 2012 Jan-Feb;30(1):3-16.

11. Ganapati S. Eponymous dermatological signs in bullous dermatoses. Indian J Dermatol. 2014;59:21-3.

12. Kubin ME, Hellberg L, Palatsi R. Glucocorticoids: The mode of action in bullous pemphigoid. Exp Dermatol. 2017;00:1–8.

Author Information University of Mississippi School of Medicine (Hodge). University of Mississippi School of Medicine, Department of Dermatology (Brodell).

Corresponding Author: Bonnie D. Hodge, 1607 Piedmont Street, Jackson, MS 39202. Ph: (601) 692-6193 (bhodge2@umc.edu).

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This dermal-epidermal blister is associated with an underlying mixed inflammatory infiltrate including eosinophils in this patient with bullous pemphigoid (hematoxylin & eosin, 100x).

Figure 10.

This patient with bullous pemphigoid demonstrates both urticarial, eczematous plaques and tense, fluid-filled blisters.

Pemphigoid can present with urticarial and eczematous plaques with no blisters at all or a mixed phenotype including both.

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Five Inadvertent HIPAA Violations by Physicians TRACEY HAAS, DO, MPH AND CO-FOUNDER, DOCBOOKMD

P

hysicians do not plan ahead to violate HIPAA, but in this digital age you may be doing it because you did not plan ahead. The recent final rule of the HITECH Act outlines that even if the physician is unaware of the violation, he may be fined a civil penalty of $100 - $50,000 per violation. It is time for even the most resistant physician to pay attention to how he handles protected health information (PHI).

To assist MSMA members the Association offers DocBookMD free to all actively practicing member physicians. Here we will outline five common ways you could be breaking HIPAA/HITECH privacy and security rules – and not even know it.

1. Texting PHI to members of your care team It’s a simple scenario: you’ve left the office and your nurse texts you that Mr. Smith is having a reaction to the medication you’ve just prescribed. She has included his name and phone number in the text.

You may know that texting PHI is not legal but feel justified because it is a serious medical issue. Perhaps you even believe that deleting the text right away will protect you – and Mr. Smith. In reality, this text message with PHI has just passed from your nurse’s phone, through her phone carrier, to your phone carrier, and then to you – four vulnerable points where this unprotected message could be intercepted or breached. A secure messaging app like DocBookMD encrypts this type of message as it passes through all four points of contact. Ideally, both sender and recipient should be verified and have signed a business associate agreement (BAA).

2. Taking a photo of a patient on your mobile phone To some this sounds silly; to others, it is as common as verifying a rash with a colleague or following the margins of a cellulitis day by day. When these photos are viewed by eyes for which they are not intended, you may be in violation of your patient’s privacy. It’s important to be aware of where and how patient information and images are stored. Apps that allow you to take a secure photo are just as important as sending the message securely. DocBookMD allows photos to be taken within the secure messaging app itself – never stored on your phone or within your phone’s photo album. Always use this type of feature when taking any photo of a patient or patient information.

3. Receiving text messages from your answering service Many physicians believe a text message from a third party, like an answering service, shifts the responsibility for a violation of HIPAA – this is simply not true. Many services do send a patient’s name, phone number and chief complaint via SMS text. The answering service may verify it is encrypted on their end, but if PHI pops onto the physician’s screen, it is certainly not secure on her end – and this is where the physician’s responsibility lies. Talk with your answering service today to see how they protect you at both ends of the communication.

4. Allowing your child to borrow your phone that contains PHI Many folks allow their kids to use their phones to play games on apps while in the car. If your phone has an app that can access PHI, then you may be guilty of a HIPAA breach if the information is viewed by or sent to someone for whom it is not intended. The simple fix is to utilize the pin-lock feature on your messaging app; and, for doubleprotection, always password protect your phone.

5. Not reporting a lost or stolen device that contains PHI Losing your smartphone or tablet is a pain for many reasons.

But if you have patient information on that device, you could be held responsible for a HIPAA breach if you do not report the loss right away. The ability to remotely disable an app that contains or handles PHI is an absolute must for technology that handles communications in the medical space. Be sure to ask for this feature from any company claiming to help you be HIPAA-compliant in the mobile world.

Remember: Being HIPAA-compliant is an active process. A device can claim to be HIPAA secure, but it is a person who must ensure compliance. n

DocBookMD partners with to bring MSMA members a free, HIPAA-secure messaging app that uniquely provides extra security to avoid each of these potential pitfalls. DocBookMD is available in Mississippi only to actively practicing MSMA members. For more information on the mobile app, visit www.DocBookMD.com or call toll-free 888-930-2048. To join MSMA, visit MSMAonline.com or call 601-8536733. Membership is open to any MD or DO licensed to practice in Mississippi.

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P E R S O N A L S

Thad Waites, MD, FACC, a physician at Hattiesburg Clinic Heart & Vascular, has been awarded the 2018 Master of the ACC Award by the American College of Cardiology in honor of contributions to the cardiovascular profession.

Dr. Waites was recognized for these achievements along with all 2018 Distinguished Award winners at the Convocation Ceremony Thad Waites, MD on March 12 during the ACC’s 67th Annual Scientific Session in Orlando, Florida. He is one of only three people in the nation to be selected this year and the first physician from Mississippi to ever receive the ACC’s highest award.

“Thad Waites’ dedication to improving cardiovascular health and enhancing patient care is a testament to his expertise and professionalism,” said ACC President Mary Norine Walsh, MD, FACC. “Waites’ hard work truly shows his determination to make a lasting positive impact in the cardiovascular field. I am proud to present him with the Master of the ACC Award.”

The Master of the ACC Award recognizes and honors Fellows of the American College of Cardiology who have consistently contributed to the goals and programs of the College and who have provided leadership in important College activities. MACCs have been members of the College for at least 15 years and have served with distinction and provided leadership on various College programs and committees.

Dr. Waites, a native of Waynesboro, has been practicing medicine for nearly 50 years and has been with Hattiesburg Clinic since 1987.

Various service positions throughout his professional career include chair for the ACC’S Board of Governors, member of the ACC’s Board of Trustees, member of Hattiesburg Clinic’s Board of Directors and many others. Dr. Waites currently serves as chair for the ACC’s Health Affairs Committee and vice-chair for the Mississippi State Board of Health.

“Hattiesburg Clinic is incredibly proud of Dr. Waites on his most recent honor of Master of the ACC Award. Dr. Waites is a longtime member of the Hattiesburg Clinic family and has vastly contributed to the medical community through his work as a physician and a leader.

We are grateful for his service and honored to call him one of our physicians,” said Tommy Thornton, executive director for Hattiesburg Clinic.

Dr. Waites said receiving this top honor from the American College of Cardiology, of which he’s been a member for 38 years, is the pinnacle of his career. He said he is humbled by the award.

“This is recognition of a lifetime of effort on my part and this effort is deemed by my colleagues as something to be awarded for. When you get this, it’s saying, ‘you’ve done well.’ And I greatly, greatly appreciate it,” said Dr. Waites.

Nineteen Distinguished Awards will be presented at the Annual Scientific Session this year, each recognizing an individual who has made outstanding contributions to the field of cardiovascular medicine. Recipients are nominated by their peers and then selected by the American College of Cardiology Awards Committee.

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Lucius M. “Luke” Lampton, MD, of Magnolia, has contributed a chapter to America’s leading Family Medicine textbook, Conn's Current Therapy 2018.

The publication, designed for busy primary care specialists and other first-line care providers, focuses on the most current diagnosis and treatment protocols for Luke Lampton, MD common complaints, acute diseases, and chronic illnesses. Chapters are written by nationally recognized leaders in the field who provide their personal approaches and evidence-based clinical management options for the conditions physicians are most likely to see in their everyday practice.

Dr. Lampton’s chapter was “Yellow Fever” and focused on the re-emergence of this tropical disease in modern medicine.

Serving as a Mississippi Board of Health member since 2006, Dr. Lampton served as Board chairman from 2007 to 2017. He has served as Editor of this Journal for two decades, written thousands of articles on medicine and health care, and received national awards for excellence in writing and journalism. He has also served as Medical Editor of the recently published Mississippi Encyclopedia, authoring multiple entries on the history of medicine in Mississippi.

Dr. Lampton serves as Clinical Associate Professor of Family and Community Medicine at Tulane University School of Medicine, Clinical Instructor in Family Medicine at the University of Mississippi School of Medicine, and as Adjunct Clinical Professor of Family Medicine at William Carey University College of Osteopathic Medicine in Hattiesburg.

He has been recognized as Mississippi’s “family physician” of the year by his peers, alumnus of the year by his medical school, professor of the year nominee by his Tulane medical students, and has received national citation for his hospice work.

Dr. Lampton specializes in Family Medicine in multiple settings: clinic, hospital, nursing homes, geri-psych, long-term acute care, and hospice. Dr. Lampton also serves as President of the Foundation of Mississippi History and helped oversee the creation of the 2017 opening of the Museum of Mississippi History and the Mississippi Civil Rights Museum.

J. Edward Hill, MD, of Tupelo retired at the end of December from the faculty at the North Mississippi Medical Center Family Residency Center. In recognition of Dr. Hill’s decades of service, including serving as the founding director and a fixture on the faculty for 22 years, the facility’s South Green Street center has been renamed the J. Edward Hill Family Medicine Residency Center.

J. Edward Hill, MD

P E R S O N A L S

Avani Patel, M3, UMC SOM, is the recipient of a Mentor Program Grant given by the American Society of Anesthesiology (AMA) Committee on Professional Diversity. Her grant proposal was for an advocacy event she plans to pilot at UMMC, which can then be used for all medical schools across the country.

She presented the project at the ASA’s annual Avani Patel, M3 meeting this year in Boston and will serve as UMMC medical student delegate to the ASA next year in San Francisco. She serves as the UMMC Anesthesiology Interest Group President.

In addition to serving as a student Board of Trustees member for both MSMA and Central Medical, Patel serves as the AMA-MSS Region III Membership Chair and on the 2017-18 Membership, Engagement, & Recruitment Standing Committee along with the Hospitality Convention Committee for Interim 2017. She serves as president of the student AMA/MSMA chapter at UMMC and has been a Delegate to the MSMA Annual Session and Alternate Delegate to the AMA for the past two years. She was awarded the Dr. Virginia Stansel Tolbert Award during her second year of medical school and was awarded STAT Scholar which she completed in less than six months during her first year of medical school.

Patel is the first Mississippian and UMMC medical student to blog

for Merck Manuals, having published over 50 articles since July 2016.

She published two posts specifically about AMA/MSMA titled “2 for 1: AMA & MSMA 2017 Annual Session” and “House of Delegates: MS Edition.” She also published “Prescription for Effective Lobbying” utilized to train the new M1 class in lobbying for Capitol Day. Her articles are available at: http://medstudentstories.merckmanuals.com/ author/apatel/ Additionally, she is Southern Region Chair for the Organization of Student Representatives for the Association of American Medical Colleges.

William C. Lineaweaver, MD, FACS, has been named President and Chief Research Officer of the Joseph M. Still Research Foundation in Augusta, Georgia. Active for over 20 years, this foundation has been active in clinical trials and translational research in burns, wound healing and surgical infections. Dr. Lineaweaver continues as medical director of the Joseph M. William Still Burn & Reconstruction Center at Merit Lineaweaver, MD Health Central, the state’s only designated burn center. He also continues as editor of Annals of Plastic Surgery and chair of the JMSMA Editorial Advisory Board.

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If We Are Not Part of the Solution, We Will Be Part of the Problem WILLIAM GRANTHAM, MD

E

verywhere you turn physicians, patients and the public are talking about the opioid crisis. I want to talk about how doctors are part of the solution. Between the new regulations passed by the State Board of Medical Licensure (SBML) and bills introduced at the Capitol, many ideas have surfaced.

Use of the Pharmacy Board’s Prescription Monitoring Program (PMP) is at the top of the list. Virtually all practicing physicians in Mississippi are signed up with the PMP and many use the information available on the PMP website to make better prescribing choices. That’s because it is a requirement that every physician registered with the DEA to prescribe controlled substances must also register with the PMP. Every state now has a PMP and some require prescribers to check the prescription history of patients, although the specific requirements vary from state to state.

The variations range from requiring physicians to check (1) when prescribing more than 30 morphine milligram equivalents (MMEs) per day, (2) when prescribing Schedule II and III controlled substances; (3) when prescribing Schedules II-IV; or (4) if prescribing certain scheduled drugs to new patients. MSMA recently recommended that the SBML adopt a provision requiring physicians to check the PMP only at a patient encounter in which a Schedule II medication is prescribed for acute pain or chronic non-cancer pain. However, the medical board chose to require PMP use in more instances.

While there are many opinions as to when prescribers should check the PMP, there is a severe lack of guidance telling physicians what is expected of them when they actually run a PMP report on a patient.

The PMP is a great tool, but, like any tool, it must come with directions for use. I have heard physicians say, “I checked the PMP. Now what?”

No one seems to agree on exactly what action a PMP report should prompt a physician to do or not do.

This isn’t just a Mississippi problem. Doctors across the country are talking about the ethical and legal dilemmas they face once they run PMP report and discover “something.” Some patients will need counseling and treatment options to deal with an addiction. Some may be diverting the drugs for a family member with an addiction. Others may be diverting to sell the drugs.

Certainly it is possible my patient just has two overlapping prescriptions and doesn’t have a drug problem at all. These are all

262 VOL. 59 • NO. 5 • 2018

William M. Grantham, MD MSMA President 2017-2018 questions a physician will have to consider while evaluating the patient, reviewing the PMP report and deciding on a course of treatment. This doesn’t address what the physician is expected to do when the PMP report indicates that another prescriber appears to be less attentive.

Tennessee state law requires prescribers to report to law enforcement within 5 days suspected doctor shopping (defined as when a patient tries to hide that he/she has received the same or similar controlled substance from another prescriber in the past 30 days). This is just one more example of non-physicians directing the practice of medicine.

The PMP is a great tool and physicians should drive the conversation about its use. Let me know what you want to see addressed in guidelines. Write me at President@MSMAonline.com. r

William M. Grantham, MD MSMA President 2017-2018

M I S S I S S I P P I

S T A T E

D E P A R T M E N T

O F

H E A L T H

Mississippi Mississippi Mississippi

ProvisionalProvisional Reportable Disease Statistics* Reportable Disease Statistics*

Provisional Reportable Disease Statistics February 2018 February 2018 February 2018

*Monthly statistics are provisional. Disease totals may change depending on additional reporting

healthcare providers publicmay healthchange investigation.

These numbers do not reflect the final case *Monthly statistics arefrom provisional.

Diseaseand totals depending on additional reporting counts. from healthcare providers and public health investigation. These numbers do not reflect the final case Public State counts.

Health District

I

69

66

57

0

175 139

155

0

126 0

0

1

1

61

Mycobacterial Diseases

Pulmonary Tuberculosis (TB) Extrapulmonary TB

IX

Feb 2018

2

1

1

5

1

0

13

3

0

4

57

2

131

56

21

84

2

0 0

VI

1126

VII

5 362

3

2

03

0

131

0

0

0

56

21

362 1 133 1

68 6

1 0

9

VIII

IX

Feb 4 2018

0

60

1331 68

0175

0

3

46

1

60

1

0

0

0 0

5 0

527

1

0

1274

70

37 0

0

0

0

01

0

0

0

10

11

0

0

0

20

0

0

0

0

9

1

0

0

0

Hepatitis B (acute)

Diphtheria

Mumps

0

0

0

01 00

0

0

0

0

0

0

0

00

0

0

0

0

0

0

0

0

0

0

1

Invasive H. influenzae disease

Tetanus

Invasive Meningococcal disease

Poliomyelitis

Hepatitis A (acute)

0

Salmonellosis Shigellosis

Campylobacteriosis

Hepatitis B (acute)

E. coli O157:H7/STEC/HUS

Invasive H. influenzae disease

Animal Rabies (bats)

1

2

1

0

0

0

0

0

0

Lyme disease

Rocky Mountain spotted fever West Nile virus

0

6

0

0

0

1

2

0

0

1

0

0

0

0

00

0

00

1

00

00

0

0

00

2

3

0

1

0

1

0

3

6 0 0

0

0

0

0

0

00

0

1 1 0 4

0

0

0

01

0 2

1

00

4

1

1

00

0

00

0 7

0

40

0

0

3

1

02

0

0

4

2

01

0

20

0 0

0

0

0 0

0

0

0

0

0

0

00

0

0

0

0

0

1

1

0

0

0

0

0

00

0

0

00 00

0 0

0

0

0

0

0

00

0

0

0

00

0

00

0

0

00

0

0

2 2

0 2 2 0 0

910

83

44

0

0

5

0

10

00

0

0

1

5

2

3

0

0

00

4

3

16

02

2

5 40

0

10

13

0

00

0 10

0 1

0 0 0

0

0

0

0

05

0

0 0

0

0

04

00

1

0

0

0

Totals include reports from Department of Corrections and those not reported from a specific District.

0

859 44 2,842 1,633 45 81 3

0

2

3

954

1

0

00

0

77 72

77 0

0

0

9

954

43 1,633

950

0

0

31 2,842

YTD 72 2017

0

5

5

0

1274

34

0

24 0

4

527

0

4

30

0

24

0

0 0

2018

2

603

82

0

Poliomyelitis

2017

5095

6

0

16 Feb 39 YTD50

34

175 0 1964 1,529 3

0

43

39

0

0

31

37

0

0

20

7 16

03

0

YTD 2017

13 1,52920 859 196

20

0

YTD 2018

603

0

0

Feb

State 2017 Totals*

82

8

Measles

Enteric Diseases

VIII

0

Mycobacteria Other Than TB

*

2 139 1 155 175

VII

03

Tetanus

Mumps

1 66

VI

2

Pertussis

Measles

V

V

1

Pulmonary Tuberculosis (TB) Extrapulmonary TB

IV

0

IV

5

Diphtheria

Zoonotic Diseases

Vaccine Preventable Diseases

1

3

HIV Disease

Hepatitis A (acute) Enteric Diseases

52

0

Mycobacteria Other Than TB

Invasive Meningococcal disease

Zoonotic Diseases

1

III

HIV Disease

Pertussis

*

1 69

II

Early Latent Syphilis

Chlamydia

3

1

I

Gonorrhea Chlamydia

Gonorrhea

0 61

Early Latent Syphilis

Primary & Secondary Syphilis

Vaccine Preventable Diseases

Mycobacterial Diseases

Sexually Transmitted Diseases

Sexually Transmitted Diseases

Primary & Secondary Syphilis

II Public III Health District

Totals*

0

0 0

0

0 0

0

450

1

00

6 5

0

0

0

81 0 10

6

10

21

14

10

8

1

00

0

0

07

00

0

33

38

0

78

20

075

24 57

1 6 5 1 0

7 31 2

61

0

210

0 3 0

0

0 1

70

10

0

1

10 4 0 14 0

1

7

00

1

2

1

3

3

1

0

2

3

16

33

38

78

Shigellosis

0

1

0

0

0

0

0

2

2

5

7

20

24

Campylobacteriosis

3

6

7

2

4

2

1

2

13

40

31

75

57

E. coli O157:H7/STEC/HUS

0

0

0

1

0

0

0

0

0

1

2

1

4

Animal Rabies (bats)

0

0

0

0

0

0

0

0

0

0

0

0

0

Lyme disease

0

0

0

0

0

0

0

0

0

0

0

0

0

Rocky Mountain spotted fever

0

0

0

0

0

0

0

0

0

0

3

1

7

West Nile virus

0

0

0

0

0

0

0

0

0

0

0

0

0

Salmonellosis

Totals include reports from Department of Corrections and those not reported from a specific District.

MAY â&#x20AC;˘ JOURNAL MSMA

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© 2018 Associated Press

Beauty is in the eye of the beholder Beauty is in the eye of the beholder. It’s an old adage that makes the point: we see everything from our own viewpoint. That said, the success of the 2018 legislative session will also be seen different ways by different viewers. For organized medicine, it wasn’t a bad year. In fact, medicine’s efforts were highly successful in the scope of practice arena.

It was a little foggier in the anti-tobacco barn but medicine’s efforts were significant.

Some would even say, “You’ve come a long way, baby,” to quote the 70’s era cigarette advertisements. Physicians also gained a little ground with Medicaid and medicine was definitely in the winner’s circle once again protecting the best immunization law in the country. The summary below outlines legislative efforts by subject.

– R. David Roberts, III, MSMA Director of Government Affairs

264 VOL. 59 • NO. 5 • 2018

T H E

M I S S I S S I P P I

S T A T E

L E G I S L A T U R E

Recap of the 2018 Legislative Session

T

he Mississippi State Legislature convened the 2018 session in Jackson on January 2, 2018, for the third session of the 2016-2019 term. The legislature adjourned Sine Die March 28th. MSMA staffed the Capitol Medical Unit during the legislative session with Apphia McCollough, RN, serving as the Capitol Nurse.

MSMA also continued efforts to encourage Mississippi physicians to volunteer as the Doctor of the Day. Forty MSMA physicians from around the state participated in the program. This program continues to provide members an exclusive opportunity to observe and participate in the legislative process firsthand.

Scope of Practice. Eight bills were designed to authorize independent practice for advanced practice nurses (APRNs). Both the Senate and House Public Health Committee chairs stood firm and contained those bills without allowing them to be considered by the committee. Physicians responded to MSMA’s calls to action and flooded the Capitol with emails and phone calls stressing the quality care provided by a physician led team. An important component of scope of practice is identification of one’s credentials and training. Truth in Advertising HB838, SB2900. Representative Bryant Clark (D-Pickens) sponsored HB 838 and Senator Charles Younger (R-Columbus) introduced SB2900 to strengthen signage requirements for APRNs who practice without a physician on site.

The bills were not reported out of committee.

Immunization Exemptions. MSMA worked hand-in-hand with the State Department of Health and the Mississippi Immunization Coalition to protect the nation’s most comprehensive law requiring pre-school vaccinations. Six bills, four in the House and two in the Senate, were all defeated in committee.

Medicaid Re-authorization SB2836. After contentious debate, lawmakers agreed in the final days of the 2018 legislative session on a reauthorization bill for the Division of Medicaid. Commonly called the Medicaid technical amendments bill, the reauthorization bill sets eligibility standards, provider reimbursement rates and the services that will be covered. A summary follows.

• Requires managed care companies to reimburse providers at or above Medicaid fee-for-service rates.

• Removes the physician office visit limit and allows the Division of Medicaid to increase the limit.

• Allows OB-GYNs to be paid 100% of the Medicare allowable rate for primary care services.

• Allows physician-administered drugs to be billed as either a medical claim or a pharmacy claim so the physician does not have to stock items like long acting reversible contraceptives

(LARCs) and injectable anti-psychotic medications.

• Requires the managed care organizations (MCOs) to accept credentialing of providers by the Division of Medicaid and prohibits separate credentialing by the MCO. • Removes the five-prescription drug limit and allows the Division of Medicaid to increase the limit.

• Clarifies existing authority of the Governor to address revenue deficits and removes the protection that previously capped the amount hospital reimbursement could be reduced.

• Requires more audits and evaluations of the MississippiCAN program.

Tobacco Tax HB1478, SB2230, SB2445, SB2701, SB3048.

Along with a coalition of 20 health related organizations, MSMA pushed a $1.50 per pack cigarette tax. Four bills were introduced to increase the cigarette tax although none was reported out of committee. However, the Senate amended a bond bill by inserting the tax code sections that made it possible to increase cigarette taxes.

SB 3048 quickly passed the Senate but then fizzled out in the House Ways and Means Committee. Physicians pummeled legislators with grassroots messages; yet the coalition effort fell short. Many believe that legislators were reluctant to go into the 2019 campaign cycle with a tax hike of any kind under their belt. Anti-Tobacco Capitol Day for Students (MSMA 2017 Resolution 18). MSMA organized a Capitol Day on February 15, 2018, for medical students to specifically push a higher tobacco tax. MSMA President Dr. Bill Grantham, Senators Brice Wiggins (R-Pascagoula) and Willie Simmons (D-Cleveland) held a press conference to spell out the positive health impact a cigarette tax would generate by reducing and preventing tobacco use. Medical students from University of Mississippi School of Medicine and William Carey College School of Osteopathic Medicine participated.

Licensure of Anesthesiologist Assistants HB907, SB2037.

MSMA closely worked with the Mississippi Society of Anesthesiologists to seek licensure of Anesthesiologist Assistants.

Senate Public Health Chair Dean Kirby (R-Pearl) sponsored SB2037 and Representative Nick Bain (D-Corinth) introduced companion bill HB907. Due to intense opposition on other scope of practice battles, both bills died without consideration.

Prescribing Rules SB2759. Senate Health Chair Dean Kirby introduced SB2759 to urge conversation and complementary action by state agencies that license prescribers. The measure sought to unify members of the Medical Board, Dental Board, Nursing Board and Pharmacy Board beside representatives from the addiction treatment/mental health community to identify uniform concepts

MAY • JOURNAL MSMA

265

Anti-Tobacco Capitol Day at the Mississippi State Capitol on February 15, 2018 – Student chapter representatives of the University of Mississippi School of Medicine and William Carey University College of Osteopathic Medicine pictured with (front row l. to r.) MSMA President William Grantham, MD; Sen. Willie Simmons (D-Cleveland) and Sen. Brice Wiggins (R-Pascagoula).

before each board independently implemented recommendations from the Governor’s Opioid Task. SB2759 passed unanimously out of the Senate and was not reported out of committee in the House.

Telemedicine HB799. MSMA worked with House Public Health Chairman Sam Mims (R-Brookhaven) who sought to close a loophole that some insurers have used to refuse reimbursement for telemedicine services. The bill was not reported out of committee.

Firearms in Public Hospitals HB1083, SB2889. MSMA continued efforts to amend Mississippi law to prohibit firearms in public hospitals. Laws governing concealed carry permits for firearms in 2016 extended this protection to private hospitals but failed to cover public facilities like county and municipal hospitals. Both bills died and Andy Gipson (R-Braxton) who was then chair of the House Judiciary B Committee committed to work on this issue. Rep. Gipson

266 VOL. 59 • NO. 5 • 2018

has subsequently been appointed to finish out the term as Agriculture Commissioner, replacing Cindy Hyde-Smith (R-Brookhaven) who has been tapped to finish out the US Senate seat held by Thad Cochran (R-Jackson).

Ban Tanning Beds by Minors HB1400, SB 2048. MSMA joined the Mississippi Dermatology Society and the American Cancer Society Cancer Action Network seeking to ban the use of tanning beds by minors. Although there was support in the Senate committee, the Chair chose not to bring the bill up after conferring with the House Public Health Chairman who remains opposed to the measure.

Ban Texting while Driving HB900. MSMA and the Mississippi Chapter of the American Academy of Pediatrics successfully deleted the re-authorization requirement for the texting ban law. The texting ban law prohibits any driver from using text messaging and other

HOUSE CONCURRENT RESOLUTION NO. 99 A CONCURRENT RESOLUTION DESIGNATING MARCH 30, 2018, AS DOCTOR'S DAY IN MISSISSIPPI AND RECOGNIZING THE SERVICE OF THE DOCTORS OF THE DAY WHO SERVE THE MISSISSIPPI LEGISLATURE DURING THE 2018 REGULAR SESSION. The Resolution recognizes our physicians who served this legislative session: WHEREAS, we are particularly proud of the exemplary public service shown by the Doctor of the Day program for the Mississippi Legislature, and we take this opportunity to honor the following physicians who donate their professional time and service during the 2018 Regular Session: Afifa Adiba, M.D., Brandon; Joseph D. Austin, M.D., Vicksburg; J. Stephen Beam, M.D., Hattiesburg; Sidney W. Bondurant, M.D., Madison; K. Page Branam, M.D., Ridgeland; Jennifer J. Bryan, M.D., Flowood; L. Susan Buttross, M.D., Jackson; Stephen Coachys, M.D., Jackson; Randy Easterling, M.D., Vicksburg; Jeremy Erwin, M.D., Jackson; Roderick C. Givens, M.D., Natchez; Tamara Glenn, M.D., Jackson; William M. Grantham, M.D., Madison; Scott L. Hambleton, M.D., Madison; J. Clay Hays, Jr., M.D., Jackson; W. Mark Horne, M.D., Laurel; Dedri M. Ivory, M.D., Vicksburg; Jon Corey Jackson, M.D., Whitfield; Marc E. Johnson, M.D., Brookhaven; Vern Antoine Keller, M.D., FACS, Jackson; Carlos Latorre, M.D., Vicksburg; Day S. Lennep, M.D., Madison; Philip Levin, M.D., Gulfport; Michael Mansour, M.D., Greenville; Lori Marshall, M.D., Jackson; Gail Martin, M.D., Jackson; Shawn A. McKinney, M.D., FACS, Jackson; Philip T. Merideth, M.D., J.D., Jackson; John R. Mitchell, M.D., Pontotoc; John F. Pappas, M.D., Gulfport; Katherine T. Patterson, M.D., lndianola; Frank N. Perkins, IV, M.D., Jackson; Sara B. Robertson, M.D., Jackson; Chasity L. Torrence, M.D., Whitfield; Justin M. Turner, M.D., Madison; John Vanderloo, M.D., Jackson; Christy Vowell, D.O., Eupora; Micah Walker, M.D., Flora; William L. Waller, M.D., Hattiesburg; and Myra A. Wheaton, M.D., Jackson; and The Resolution also recognized the nurses who served: Apphia McCollough, RN, Brandon; Ann Lincoln, RN, Brandon; and Stephanie Austin, RN, Vicksburg.

social media on a cell phone. The law became effective on July 1, 2015, and required a review of the law during the 2018 legislative session. The law is now permanent and will no longer need to be reviewed by the Mississippi legislature.

Mississippi Department of Health (DOH) Funding HB1599. MSMA followed the lead of the State Health Officer Dr. Mary Currier supporting the agency’s 2018 appropriation request. DOH received $2.5 million in state general fund above legislative budget office recommendation. Several key DOH initiatives were funded including $1.26 million for tuberculosis testing/surveillance and $650,000 for HIV/ STD program. Bonding authority of $3 million was allocated for local government rural water loans program. STEMI and Stroke System Funding HB1599. For the 2018-2019 fiscal year ST Elevated Myocardial Infarction Program (STEMI) was level funded at $382,500. The Stroke System of Care was level funded at $212,500.

Rural Physicians Scholarships and Graduate Medical Education SB2947. The Rural Physician Scholarship Program was funded at the fiscal year 2017-18 rate of $1.8 million for 60 medical school scholarships. The Office of Physician Workforce was level funded as well at $1.4 million for new fiscal year 2018-2019. r

TOP PHOTO: Rep. Greg Haney (R) from Gulfport visited with medical students during the Anti-Tobacco Capitol Day, February 15, 2018. AT LEFT: UMC Medical Student Mary Elizabeth Butts at Anti-Tobacco Capitol Day for Medical Students.

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L E G A L E S E

Decipher the Code: Keep Your Patients Safe JON COREY JACKSON, MD; CHASITY LYNNE TORRENCE, MD

“B

e familiar with the law in your state of practice.” For many, this was generally one of the biggest take home points of legal discussions during residency training. Emergency holds were likely such a discussion. This could potentially lead to some confusion especially if you chose to practice medicine in another state following residency. Learning the ins-and-outs of day-to-day operation in your post-training position probably did not leave much time for you to peruse your local laws. Before you knew it, being aware of the law was an afterthought. That is until you need to be familiar.

they relate to hospitalization. Many patients, if given time to process the importance of hospitalization, will eventually consent to voluntary treatment. This is a matter of taking time, showing the patient respect and compassion, and giving them the power to make an informed decision.

So, you have done all of that. The patient still refuses treatment and you as a provider do not feel comfortable with that decision.

What next? Unfortunately, even the best provider may be unable to convince a patient in this scenario that hospitalization is necessary.

Panic may set in again. Avoid this feeling and be prepared. The path of least resistance was blocked, and now you must enact an emergency hold in order to ensure the patient’s safety.6

One day, it happens. You have a severely depressed and suicidal patient in your clinic requiring hospitalization. They have a detailed plan with available means. A review of studies analyzing this clinical scenario ccording to Mississippi Code, estimated 45% of those dying by suicide any licensed physician who “has saw their primary care physician in the 1 month before their death. Panic takes reason to believe that a person poses over because this is a new situation. an immediate substantial likelihood of You are not sure what next step is best. physical harm to himself or others or is To further complicate the case, your patient (with emphasis on “your”) gravely disabled and unable to care for does not want to be hospitalized. An himself by virtue of mental illness” may emergency hold may be necessary to hold the person or admit the person ensure the patient’s safety.2 What do you do next? to and treat the person in a licensed

A

medical facility…

Although different clusters of presentations exist, this basic scenario is far too common. As a provider, you are expected to do no harm and also care for your patient.3 Many of us simply need to be familiar with the basics behind handling this situation until care can be handed off safely. Luckily, Mississippi’s law as it relates to this scenario is readily available to those who are interested and know how to find it. We will now discuss how this situation can be handled successfully and then take a look at the law itself.

When you find yourself in this scenario, it is important that you remain calm and collected. It seems easy enough, but this situation can cause anxiety especially within providers who have had little to no experience with it. Once you have collected yourself, remember the importance of establishing and/or maintaining the best therapeutic alliance. Studies agree this is arguably the most important aspect of such a scenario and will have potential consequences for further care if mishandled.4-5 Make every attempt to speak honestly about your treatment decision. Take the time to discuss the patient’s concerns as

268 VOL. 59 • NO. 5 • 2018

Statutes provide physicians with an emergency protocol for a person meeting specific criteria including (but not limited to) dangerousness to self.7 This provision is utilized when regular commitment procedures are unavailable. Under this provision, a physician with reasonable grounds to suspect a patient is likely to physically injure self or others, may place that patient on an emergency hold.8 Should this occur in your practice, involve AMR to transfer the patient to the nearest emergency department. Ideally, your patient will then be assessed by a psychiatrist to determine whether or not they can be safely discharged or if the process should continue, i.e. petition the court to hold a commitment hearing. It is generally this pre-hospitalization process that confuses providers. Since physicians are responsible for knowing their local laws, the Mississippi Code will now be explained.

According to Mississippi Code, any licensed physician who “has reason to believe that a person poses an immediate substantial likelihood of physical harm to himself or others or is gravely disabled and unable to care for himself by virtue of mental illness” may hold the person or admit the person to and treat the person in a licensed medical facility. The physician may do this without a civil order or warrant for a period not to exceed 72 hours. However, if the 72- hour period begins or ends when the chancery clerk’s office is closed, or within three hours of closing, and the chancery clerk’s office will be continuously closed for a time that exceeds 72 hours, then the 72hour period is extended until the end of the next business day that

the clerk’s office is open. The person may be held and treated as an emergency patient at any licensed medical facility, available regional mental health facility, or crisis intervention center. The physician who holds the person shall certify in writing the reasons for the need for holding. If a person being held and treated decides to continue treatment by voluntarily signing consent for admission and treatment, the 72-hour hold may be discontinued without filing an affidavit for commitment. In addition, Mississippi physicians are protected in that persons acting in good faith in connection with the detention and reporting of a person believed to be mentally ill shall incur no liability, civil or criminal, for those acts.8 In short, your safest option is to arrange transportation to the closest emergency department. Best practices for psychiatric emergency patients have been well researched, and there are several options available regarding the next steps.9 A psychiatric consultation will be requested on-site if available. If not, an emergency physician will begin searching for the closest facility where psychiatric consultation is available. Psychiatric consultation via telemedicine is an increasingly 1 popular option. Once examined by a psychiatrist, the patient will 2 either be safely discharged with an appropriate outpatient treatment3 plan or admitted. Steps beyond that would be subject to future 4 discussion. Now you have saved the day, and hopefully maintained5a positive rapport with your patient. The aforementioned steps will help 6 to ensure your patient is safe and coming back to you. Who knows?

7 Someday down the road, you may even receive heartfelt thanks from 8 your patient. r

9 10 11 1. Luoma, JB, Martin CE, and Pearson JL. Contact with mental health and primary 12 care providers before suicide: a review of the evidence. Am J Psychiatry. 159: 13 909–916. 2002.

2. Faulkner LR, McFarland BH, Bloom JD. An empirical study of emergency 14 commitment. Am J Psychiatry. 146:182–186, 1989.

15 3. Taylor R, Buchanan A. Ethical problems in forensic psychiatry. Curr Opin 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 References

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Psychiatry. 11:695–702, 1998.

4. McCabe R. The Therapeutic Relationship in the Treatment of Severe Mental Illness: A Review of Methods and Findings. International Journal of Social Psychiatry. 50 (2):115-128, 2004.

5. Fondacaro M, Frogner B, Moos R. Justice in health care decision-making: patients’ appraisals of healthcare providers and health plan representatives. Soc Justice Res. 18:63–81, 2005.

6. O’Connor v Donaldson. United States Reports: US. pp 563. 1975.

7. Miss. Code Ann. § 41-21-61(e).

8. Miss. Code Ann. § 41-21-67(5).

9. American College of Emergency Physicians Emergency Medicine Practice Committee. Care of the Psychiatric Patient in the Emergency Department – A Review of the Literature. 2014. https://www.acep.org/uploadedFiles/ACEP/Clinical_and_ Practice_Management/Resources/Mental_Health_and_Substance_Abuse/ Psychiatric%20Patient%20Care%20in%20the%20ED%202014.pdf. Accessed March 28, 2018.

Author Information Dr. Jackson and Dr. Torrence are clinical psychiatrists at the Mississippi State Hospital in Whitfield. They are both adjunct professors in the Department of Neural and Behavioral Science at William Carey University College of Osteopathic Medicine in Hattiesburg.

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ING’S DAUGHTERS HOSPITAL, GREENVILLE, 1927 – Situated on the 300 block of Arnold Avenue near its intersection with Starling Street, on the current site of the Delta Regional Medical Center West Campus, the King’s Daughters Hospital, seen above and erected in 1926-7, was the replacement structure for the King’s Daughters Home, the first hospital in Greenville and the first King’s Daughters hospital built in the state. The drive to build this larger modern hospital began in 1924 and its cornerstone was laid in May 1926. The $450,000 project would result in the opening by February 1927 of this 100-room Mediterranean-style hospital. This new hospital was equipped with all modern conveniences of the 1920s: two electric elevators, four operating rooms, a delivery room and nursery, a complete x-ray department, laboratory, and a dietetic department. Inset is a photo of the medical staff and nursing school faculty around 1927. From left to right are Drs. John Archer (Internal Medicine), D. C. Montgomery (EENT), Hugh A. Gamble (Surgery), Paul G. Gamble (Surgery), R. E. Wilson (Pediatrics) and John Lucas (Surgery). The sunporches on the left of the structure would be removed at the time of the addition of the Weinberg Annex in the 1960s. The hospital has evolved over the years and the historic structure still stands, with many additions, and is still used as a medical facility. See previous issues and future issues for related Images of Greenville hospitals. If you have an old or even somewhat recent photograph or image which would be of interest to Mississippi physicians, please send it to me at lukelampton@cableone.net or by snail mail to the Journal. r Lucius M. “Luke” Lampton, MD; JMSMA Editor

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P O E T R Y

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M E D I C I N E

Just Call It Something, Doc!!

[This month, we print a delightful poem by John D. McEachin, MD, FAAP, a Meridian pediatrician and the Journal’s unofficial poet laureate. Reflecting on what he calls “the Medical Winter of our Discontent,” John writes: “Sylvia was in bed two weeks ago with flu-like illness. She was on Tamiflu B.I.D. for five days. I took it for 10 days, once a day. Her nasal swab was negative for A. This has been a real test for all physicians on the front-line, and I sympathize. I had a few minutes’ break from harvesting fallen limbs in the yard, so I jotted this ditty down for my pleasure, and perhaps, a reader’s smile.” John is very right that this past winter was an especially difficult flu season for both patients and physicians! For more of Dr. McEachin’s poetry, see past JMSMAs. Any physician is invited to submit poems for publication in the Journal, attention: Dr. Lampton or email me at lukelampton@cableone.net.] — Ed.

“Doc, I am sick! Got fever and a cough.

Get me well! I don’t have many days off.

A shot ought to fix it! Know what I mean?

Tamiflu’s fair—so says this magazine.

You want to do what? A swab up my nose?

Now that’s just great; Gonna add to my woes!

What difference does it make—Type A or B? Does this check for that bug called RSV? Flu-like illness is what my neighbor had; Or that’s what he thought his doctor had said.

My swab is fine, but my fever is high!

I’m sixty-years-old! You know I might die!”

“Wish I could tell you more Mister Magoo.

Get rest, drink fluids, and take this Tamiflu!”

– John D. McEachin, MD Meridian * Dedicated to Dr. Luke Lampton and his colleagues for their endeavors during this recent “Medical Winter of our Discontent.”

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