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Editors’ Note: In this article, Nancy Drinan acknowledges the encouragement from her sisters’ neurologist to write about her experience as a health care advocate for her sisters. The neurologist, Dr. Dan Hoch, has in turn written about how his experiences with the Drinan sisters provide excellent examples of participatory medicine in action. –JG & TG

Abstract

Summary: This is the story of a family in crisis and how a unique model of participatory medicine enabled three sisters (two patients and their advocate) to achieve vastly improved medical management of a congenital brain disease and comorbid conditions.
Keywords: Advocacy, developmental disability, guardianship, brain disease, epilepsy, frontal lobe impairment, metabolic damage, behavioral challenges, mental health, shared decision making, collaborative decision making, participatory medicine.
Citation: Drinan NL. Three sisters, one voice. J Participat Med. 2011 Dec 14; 3:e50.
Published: December 14, 2011.
Competing Interests: Nancy Drinan is on the staff of a hospital affiliated with Harvard Medical School, an institution referenced in this narrative.

Background

When my sisters’ neurologist recently suggested I write about my personal experience in the setting of participatory medicine, I felt somewhat overwhelmed at the thought of having to tell a story that has taken 16 years to unfold. While it has been punctuated by great successes, some failures, numerous crises, and considerable heartache, this is a story that will never end. I like to think of it as a “love story;” certainly not of the romantic type, but a love story nevertheless, for only great love could have compelled me over much of my adult life to advocate so relentlessly for my developmentally disabled sisters, the patients featured in this piece. I hope what I have learned and continue to learn as their advocate will inspire others to persevere and believe that positive outcomes are possible, even in the face of what may appear to be insurmountable odds.

This is actually the story of three sisters, currently ages 47, 54, and 56. I am the middle sister and the author of this narrative of my experience in participatory medicine, first as an advocate for my now-deceased father, and second as an advocate for my sisters, both of whom have been developmentally disabled from infancy. For most of their lives, they suffered from intractable epilepsy; with the metabolic damage that accrued from many years of taking powerful anti-epileptic medications, they became morbidly obese and acquired many associated illnesses such as polycystic ovarian syndrome, type 2 diabetes, and dangerously high lipids. Because of significant frontal lobe impairment (the part of the brain that governs insight, social judgment, and self-control), they also have serious behavioral issues that can include violent and aggressive outbursts, the inability to follow instructions and stay on task, and failure to follow generally accepted social norms such as not hugging or touching strangers. Owing to these mental and physical challenges, they are not able to work and must have 24/7 supervision in their residence and in the community. There is no clear diagnosis. However, the fact that they are siblings (eight years apart), and experienced their first seizure at precisely the same age (16 months), suggests this disorder is congenital. We will most likely never know the cause.

As long as there is no cure for this brain disease, my sisters will always need an advocate. Theirs is a dynamic story that will continue indefinitely and is sure to produce new twists on the basic themes of advocacy. It is especially unusual because it involves advocating for two patients at the same time — sisters who share a similar syndrome, but who, nevertheless, are unique persons with distinct personalities and discrete medical, behavioral, and social challenges that require separate treatment plans and medical management.

This essay then, is an attempt to describe (1) the rather special family circumstances that drove the urgent need for advocacy; (2) the world-class medical system that ultimately brought my sisters from grave illness to robust health; and (3) the multidisciplinary team of specialists who worked (and continue to work) tirelessly and in a participatory way with me to achieve these remarkable outcomes.

Thrust into Advocacy: A Family Affair

After 19 harrowing months of nearly continuous hospitalization, my father died of congestive heart failure. The year was 1995. In the 16 years since his death, my life has been indelibly changed by this event. Without my intensive exposure to the workings of a major urban hospital and intimate involvement in the day-to-day details of my father’s care as his primary advocate, I would never have been able to handle the challenge yet to come.

In retrospect, I believe Dad hung on to life as tenaciously as he did because he feared what would become of his two mentally and physically challenged daughters — my only sisters. He and my mother cared for my sisters with love and devotion for more than 40 years at great personal sacrifice and financial hardship. My mother was also consumed with fear, mostly because she knew she was about to lose her husband, but also because he had always been the major decision maker in the family. He was better able to deal with my sisters’ complex physical and behavioral challenges than she was. Without Dad to “steer the ship,” what would become of them?

Further complicating matters was my mother’s poor health. After months of caring for a very sick husband, she was left completely exhausted and with few resources when he died. Though the family was battered and frayed, it remained intact. Within a year of Dad’s passing, Mom sold our family’s rural home and moved to the state capital so she could be closer to health care providers and basic services. Though she continued to care for my sisters, each day became more difficult as Mom was less and less able to control and manage their behaviors. My sisters were eligible for state aid as “permanently disabled persons,” but the assistance was minimal and delivered only when a crisis hit, which was often. Clearly, my mother and sisters could not continue in this way. Mom was at risk for collapse, and without adequate supervision and almost no meaningful program, my sisters were at risk for being taken advantage of in the community. These were very dark days.

I lived quite a distance from my mother and sisters, but visited as often as I could and increasingly became the advocate and spokesperson for them as I dealt with an ever-changing stream of social workers, case managers, and respite providers. Each seemed worse than the last. I struggled to understand complex state regulations regarding the provision of services to the developmentally disabled so I could argue more forcefully for resources that would stabilize the family. This was truly a “crash course.” Meanwhile, conditions at home continued to deteriorate, particularly with regard to the younger of my two siblings. She had grown increasingly belligerent and begun to wander all over their small city, associating with homeless persons, mentally ill persons, and substance abusers. My mother was at her wit’s end, understandably living in a constant state of fear and anxiety. The toll this exacted on her health was shocking. However, there was not much she (or I) could do about the situation, since both sisters were adults and neither had a guardian appointed. Between 1996 and 1998, I fought through the Probate Court for guardianship of both sisters. In the end I prevailed, though my sisters did not understand why guardianship was needed or why it would be beneficial to them.

At last, I was legally able to advocate for my sisters. I could push for an increased level of services that would ensure their safety and promote their independence. I also arranged for a fresh look at their medical management. Having seen first-hand the array of resources available at a major medical center during my father’s extended hospitalization, I understood clearly that my sisters had never received adequate medical care, and not because my parents were neglectful. They were, to a large extent, hamstrung by Medicaid regulations that would not authorize out-of-state care. My sisters received care locally, typically no more than an annual physical. In spite of their longstanding epilepsy, neither had ever had a brain MRI; their morbid obesity was never addressed; neither sister had ever experienced regular menstrual cycles; they were never tested for diabetes; nor were alternative, more current epilepsy medications ever suggested. We can only speculate as to the reasons for this apparent failure on the part of local health care providers. Suffice it to say, my sisters never benefited from basic diagnostic testing until I transferred their care to a major teaching hospital in Boston. I did this in defiance of their former locally appointed case manager, so convinced was I that help was available. I was correct. This is where the heart of my experience in participatory medicine begins.

The Real Journey Begins: Good Medicine; Great Doctors; Comprehensive, Coordinated Care

Since I was under great pressure at this time to stabilize conditions in the home, my first priority was to justify an increase in services based on level of need. In order to accomplish this, I needed a comprehensive evaluation of my sisters’ respective cognitive impairments and learning disabilities. I began with a psychoeducational assessment, a difficult process that took place over the course of two days. Once equipped with these results, I was able to argue for an expansion in services. Fortunately, the specialist who performed the evaluations quickly understood that this was a family in crisis. With our consent, she contacted her colleagues in neurology; shortly thereafter, my sisters underwent a full battery of neurological tests including magnetic resonance imaging of the brain. At the end, these tests did not lead to a more definitive diagnosis, but we did embark on the delicate and time-consuming task of transitioning to new antiepileptic therapies, which proved much more effective in controlling their seizures and also far less damaging metabolically, especially with respect to weight gain. This process was made all the more complicated because the course differed for each patient: different drugs, different titration schedules, and different responses. After some trial and error over the course of at least two years, we finally hit upon effective therapies and optimal doses for each. Today my sisters are largely seizure-free.

I will be forever grateful to the neurologist who has directed my sisters’ care from the outset and who continues to manage their care. From the beginning, he did not look just at the brain. Rather, he saw the whole person and quickly identified the cumulative damage that had taken place over the decades from poor medical management and therapies that were less than optimal. To his enormous credit, he wasted no time in arranging for new primary care through the Women’s Health department at his institution. For the first time in their lives, my sisters were not just viewed as “mentally retarded” persons, but as women with special challenges and medical needs. It was in this exceptional primary care setting that my sisters, then 35 and 43, had their first gynecological exams, pap smears, and mammograms. They were being treated as full persons with every right to comprehensive medical and preventive care. This was an extraordinary step up in their level of care, and these exams are now a standard part of their annual physicals.

Based on clinical exam, the neurologist also suspected polycystic ovarian syndrome (PCOS); in part because my sisters had not had normal menstrual cycles for many years; and in part because of their obesity, acne, and excessive facial hair. PCOS was confirmed on ultrasound. Based on this finding, he next referred us to a neuroendrocrinologist, who quickly ordered a fasting glucose test. Diabetes type II was confirmed. I recall well the day he delivered this difficult diagnosis to my mother and me. I had suspected this would be the result; nevertheless, it was painful to hear, and we cried. We will never know how much metabolic damage was done because the condition went undiagnosed and untreated for years. My sisters were immediately placed on a regimen of metformin to outstanding effect. By introducing more effective anticonvulsants as well as proper medication for the diabetes, by adopting better nutrition and diet, and by increasing their physical activity through a program of regular exercise, my sisters have each lost over 100 pounds. Their weights and BMI are now within normal limits; likewise, their lipids have dropped into optimal ranges. More remarkably, their glucose intolerance has vanished and the metformin has been discontinued.

In parallel with being followed by neuroendocrinology for diabetes, I also arranged for a consult with the (then-newly established) Weight Center, where the director worked closely with me and other team members to coordinate nutrition and medications that affect the metabolic profile. Now well into our second decade of care, I continue to work with the neurologist, their respective PCPs, and a neuropsychiatrist who has been an integral part of this multidisciplinary effort. His knowledge of the most current psychotropic drugs and his expertise in treating patients with developmental disabilities has enabled us to manage my sisters’ behavioral challenges far more effectively than ever before.

The efforts of this remarkable team have produced nothing short of a medical miracle. Each of my sisters has lost over 100 pounds and no longer has diabetes or dangerously elevated lipid levels. Both have had menstrual cycles restored and their intractable seizure disorders controlled with optimal medications. In addition, their explosive, disruptive behaviors have been reduced with the latest psychotropic drugs — all with minimal side effects. None of this could have been accomplished without a team of specialists who (1) place the highest priority on respect for the patient and her advocate; (2) listen intently to individual patient needs and concerns; (3) work flexibly and in a coordinated manner to achieve the best outcomes; (4) emphasize open communication and access to patient information; and (5) understand that the patient (and in this case, the patient’s advocate) must take ownership over his or her own health in order to achieve meaningful and lasting change.

Continuity of Care: The Team Behind the Team

While I was working with the medical team to better understand and treat my sisters’ very complex brain disorder and associated conditions, I was, in parallel, working with local case managers to put a full array of residential and community services in place. The key to our current programmatic success has been a very stable residential placement (a small equestrian boarding and riding facility) where they are lovingly cared for by a highly experienced couple with three daughters of their own. Today, my sisters enthusiastically engage in all aspects of farm life. They are learning how to ride and care for horses; with assistance from their residential providers, they bake, package, and sell homemade horse biscuits to local businesses and individual customers; and they participate in numerous community and volunteer activities in their small town. Fortunately, our mother, who died in 2009, lived to see my sisters happily and securely placed with this wonderful family. She was greatly relieved.

The patients are now so stable medically that hospital visits are rare, with the exception of an annual physical with the PCP. I email the team directly or make contact through the hospital’s excellent web-based patient portal that enables the patient (or designee) to request appointments, email questions or concerns to providers, and review medical history and test results. This streamlined system works very well, especially for patients who live at a great distance from the hospital. The full array of services — both medical and programmatic — now works synergistically to achieve outcomes that I never could have envisioned when I embarked on this incredible journey 16 years ago. It has truly been a team-based effort that has taken the commitment, perseverance, and input of all stakeholders. My sisters themselves embody the very best of participatory medicine and are clear examples of what is possible when strong patient advocacy and best medical practice converge.

Bibliography

Lotan G., Ells C. Adults with intellectual and developmental disabilities and participation in decision making: ethical considerations for professional-client practice. Intellect Dev Disabil. 2010 Apr;48(2):112-25.

Copyright: © 2011 Nancy Lisa Drinan. Published here under license by The Journal of Participatory Medicine. Copyright for this article is retained by the author, with first publication rights granted to the Journal of Participatory Medicine. All journal content, except where otherwise noted, is licensed under a Creative Commons Attribution 3.0 License. By virtue of their appearance in this open-access journal, articles are free to use, with proper attribution, in educational and other non-commercial settings.

 

 

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