When Neurologic Illness Hits Home — A Blessing and a Curse

18 | NEUROLOGY TODAY | JANUARY 7, 2010

B y O r l y A v i t z u r , M D

Neurologists don’t need to devel-op an illness to treat patientswith compassion. Yet, an illnessthat strikes us or a member of our familyelicits a unique perspective that can con-nect us more intimately with the humanside of medical care. And when that ill-ness is a neurologic condition, the addedknowledge can be both a blessing and acurse.

This was the case for Alan Pestronk,MD, professor in the departments of neu-rology, pathology, and immunology atthe Washington School University Schoolof Medicine, where he had moved to runthe neuromuscular group. In March1993, when his former wife, Ilene Edison— a psychotherapist he had met when hewas an intern — complained of beingtired after a day with her grandchildrenand had difficulty getting up from a chairand going up stairs, he became immedi-ately worried. Dr. Pestronk examined herat their kitchen table and the worst cameto mind. A colleague performed an EMGand muscle biopsy and confirmed her di-agnosis — a variant of amyotrophic lat-eral sclerosis (ALS) without upper motorneuron signs.

“I read biopsies myself every Saturdaymorning and after seeing Ilene’s on thatparticular morning, I asked her to cometo my office, took a deep breath, and gaveher the bad news,” he recalled.

Being a neuromuscular expert made ita great deal easier, Dr. Pestronk ac-knowledged. “The hardest thing is rec-ognizing the reality that there is no treat-ment that clearly alters the course of thedisease, and the sooner that one knowsthat it’s true, the better,” he said. What’sessential, he added, is that patients envi-sion their life three months ahead so thatthey have the equipment and resources

needed to make adjustments to the pro-gression of the disease. The first neu-rotrophic factor treatment trial was justunderway at Washington University, andDr. Pestronk was able to get his wife com-passionate use without actually enrollingher in the trial.

“An underappreciated value to clini-cal trials,” he explained, “is the excellentcare that is provided by a multidiscipli-nary team.” As Ilene became weaker, Dr.Pestronk also reconstructed their home,building an elevator so that his wife couldmove between floors. She died one yearafter the onset of symptoms.

Over the past 16 years since Ilene’sdeath, Dr. Pestronk has been seeing pa-tients with ALS and running clinical tri-als. His wife’s illness gave him a keen ap-preciation of the subtleties of treating pa-tients with diseases whose underlyingcauses cannot be modified. “It made meacutely aware of the role the neurologist

can play and the huge role other profes-sionals — social workers, occupationaltherapists, physical therapists, respiratorytherapists, pulmonologists — can play inmaking patients comfortable,” he added.His experience also taught him how im-portant it is to treat the caregiver. “Manyterrible things can happen,” he said, re-calling a time when his wife was aloneand fell in the bathroom, unable to move.

The second question Dr. Pestronk asksalmost every time a patient comes in is‘How’s the caregiver?’ “I try to make surethat someone comes in to the house to re-lieve the caregiver for periods duringevery week,” he said, conceding howfrustrated and exhausted he had becomeat times.

FRUSTRATIONS WITHPHYSICIANSJay H. Rosenberg, MD, was also angry fora long time after his wife, Judy, died. Shehad been diagnosed with multiple scle-rosis (MS) soon after they got marriedand later developed breast cancer and aprimary brain tumor. Dr. Rosenberg wasfrustrated with the physicians who wereunable to help her when she developedan autonomic syndrome: with her oncol-ogist whose approach to the recurrenceof breast cancer seemed arbitrary, hersurgeon for delaying the placement of afeeding tube when her weight plum-meted to eighty pounds, and the medicalsystem in general, which he at timesfound uncaring.

Like Dr. Pestronk, the experience madehim more aware not only of the patient’sstruggles, but also of the impact on thefamily, the feelings of the spouse and chil-dren, and the powerful influence physi-cians can have if they are willing to listenand share with them. “We’re taught thatthe white coat is the boundary, but maybe

it’s not the right boundary,” he said. In-stead, he discovered that when he beganto reveal to his patients that his wife hadMS, it made a difference in their willing-ness to address the social and emotionalissues related to the medical problems.

Dealing with illness and death hastransformed the way Dr. Rosenberg prac-tices neurology and cares for the largenumber of MS patients in his practice. “Iunderstand the human trauma and lookdifferently at the whole dying process. I’mwilling to talk about death now, and listento what patients are saying,” he explained.“My whole practice has changed.”

A TRANFORMATIVE CAREER SHIFTAt times, a family member’s illness cannot only profoundly affect the way aphysician practices, but can also chart anew course for a medical career. So it waswith John A. Kessler, MD, chair of the De-partment of Neurology at the North-western University Feinberg School ofMedicine, when in 2001, his then 15-year-old daughter suffered a spinal cordinjury from a skiing accident. He spokeabout the transformation in the Peabodyaward-winning documentary “MappingStem Cell Research: Terra Incognita.”

“I really knew nothing about spinalcord injury except what I read in pass-ing,” he said. “I really knew nothing aboutthe problem of regenerating axons. Theday of the accident I decided that’s whatI’m going to do,” and thus the SpinalCord Injury Project was born. [Dr.Kessler describes the project as an effortto use nanoengineered materials to facil-itate regeneration after spinal cord injuryand examine the combined use of thismaterial with various types of stem cells.]

“This becomes an obsession, it be-

tients’ decisions to have home ventilatortherapy. But it would be wrong for physi-cians to wield the costs of care as a blud-geon to intimidate or coerce patients andfamily members into agreeing with thephysician’s decision.

Some physicians and nurses find itpsychologically difficult to order extu-

bation even after tracheal positive-pres-sure ventilation has been validly refusedby an ALS patient. Other physicians alsomay be uncertain or fearful of medico-legal consequences when a patient diesafter refusing life-sustaining therapy orbelieve that this activity violates theirpersonal moral code. Some of these ob-stacles are amenable to education aboutclinical practice guidelines and othersresolve with more experience in con-ducting this activity. But if a physician

remains unwilling to order extubationafter further tracheal positive-pressureventilation has been validly refused byan ALS patient — on the grounds that itviolates the physician’s personal moralcode — the physician should attempt totransfer the patient to another physicianwho is willing to carry out the patient’swishes. While it is true that physiciansare not required to perform medicalpractices that they regard as unethical,their conscientious opting out of stop-

ping therapy that a capacitated patienthas validly refused should not deprive apatient of the right to refuse life-sus-taining therapy. •

Dr. Bernat is professor of neurology andmedicine at Dartmouth Medical School.He was the former chair of the AANEthics Law & Humanities Committee andis the author of Ethical Issues in Neu-rology, 3rd ed. (Lippincott Williams &Wilkins, 2008).

Ask the NeuroethicistContinued from page 17

Continued on page 19

DR. ORLY AVITZUR

In Practice

JANUARY 7, 2010 | NEUROLOGY TODAY | 19

comes the driving force to life. There isno clinical dissociation from this… thisis a very personal issue now,” Dr. Kesslerexplained in the documentary.

EMPATHY FOR THE PATIENTIt becomes even more personal when theneurologist is affected by a neurologicalcondition, even temporarily. About fiveyears ago, Roy Katzin, MD was wrestlingwith his 15-year-old son, who acciden-tally broke his nose. A few days later, dur-ing the nasal repair, Dr. Katzin aspiratedand developed pneumonia. He was ad-mitted to one of his hospitals in Boca Ra-ton, FL, where he had been in privatepractice for 23 years, and treatment in-cluded a course of intravenous steroids.Dr. Katzin, who developed severe delir-ium, recalls crying for his mother and hiswife and calling his internist to insist thathe be transferred to a different hospitalfor no apparent reason. Once he im-proved, he became aware of medicationerrors made by staff — some of which hecould correct. For example, he inter-

rupted the intravenous administration ofthe wrong antibiotic in the wrong doseby reading the IV bag label while it wasbeing hung.

“I survived the experience and devel-oped a heightened sense of empathy forthose confused and disoriented in thehospital setting,” Dr. Katzin said. He nowwrites orders to orient every delirium pa-tient each shift and to keep each of thosepatients near an open window 24/7 sothey can tell day from night.

One of the most common neurologi-cal conditions affecting neurologists ismigraine. According to a 2003 paper inNeurology, the one-year-prevalence ofmigraine in American headache special-ists is 66 percent and in neurologists40.9 percent, compared with 12 percentof adults with migraines per year in theUS. The high prevalence in neurologistshas been confirmed in other countries in-cluding Canada, Germany, Spain, andTaiwan. Thirty percent of the headachespecialists who were migraineurs saidthat their own migraine was one factor,among others, that had led them to be-come headache specialists.

Mark Stillman, MD, director of theSection for Headache and Pain in theNeurological Institute Cleveland Clinic

Department of Neurology, has sufferedfrom migraine since he was a child. Likeother migraine specialists, he under-stands the concerns of his patients be-cause he has experienced them as well.His examination room is dark, and heuses incandescent light in his office be-cause fluorescent lighting triggers aheadache.

“When a patient relates that to you,historically there is a camaraderie, a con-federacy of dunces,” he said. “We all swapstories.”

Dr. Stillman’s experience has alsohelped him understand that regardless ofhow subjective pain can be, it’s importantto take patients at face value. “Many clin-icians tend to discount patients and say‘that’s nothing’; they don’t realize that it’svery disabling,” he said, adding, “Youhave to believe what the patient tellsyou.”•

Dr. Avitzur, a neurologist in private prac-tice in Tarrytown, NY, holds academic ap-pointments at Yale University School ofMedicine and New York Medical College.She is also the editor-in-chief of the AANWeb site, AAN.com, and chair of the AANPractice Management and Technology Sub-committee.

In PracticeContinued from page 18

DR. JAY H. ROSENBERG saiddealing with his wife’s illnessand death has transformed theway he practices neurology andcares for the large number ofMS patients in his practice,adding: “I understand the hu-man trauma and look differentlyat the whole dying process; I’mwilling to talk about death now,and listen to what patients aresaying.”