Med School Sorta


I feel inspired to write a few words about Johns Hopkins, where Colleen continues to occupy a hospital bed. She definitely will not be getting her wings back tomorrow; maybe Wednesday afternoon, maybe Thursday.  She most definitely wants to go home.

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John Hopkins is home to one of the largest and most active Scleroderma Research Centers in the world. It was founded in 1992 by physician-scientist Fredrick Wigley, MD, who remains its Director and who, as of a year or so ago, became Colleen's primary scleroderma doctor. Those of us who've met Dr. Wigley like him enormously; when he's with Colleen, his focus is unblinkingly on her, his ego nowhere in sight. 

Dr. Wigley

(who reminds camille of actor geoffrey rush)

Patients with scleroderma from all over the world come to Baltimore to see Dr. Wigley. He has put together a strong team of Johns Hopkins doctors--that Wigley is a team builder as well as a team player also speaks volumes about this man's character--doctors who specialize in every aspect and area of the body affected by this uncommon disease. That's how pulmonologist Stephen Mathai, MD (pronounced ma-tai) has become involved in Colleen's case and, because of her need for new lungs, has taken the lead in her care for the time being
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Dr. Mathai

It was Dr. Mathai who made the telephone call from Baltimore to his colleague Dr. John McDyer in Pittsburgh, a call that succeeded in getting Colleen an expedited admission to Pittsburgh's week-long lung transplant evaluation. We don't exactly know whether Mathai and McDyer are friends-friends, but they are clearly professional friends whose paths have intertwined over the years in ways that have now turned out to be propitious for Colleen. We don't know the exact years, but they both grew up in Cherry Hill, both received their MD degrees from Jefferson Medical College, and both chose Pulmonology as their speciality. And in their photographs, they appear to be of similar age. McDyer is presently immersed in Pittsburgh's Lung Transplant Program. Mathai, by his own account, is a relative newcomer to lung transplantation. I think this has already been of benefit to Colleen. I believe she and her particular case have his full attention. On top of that is the tensile Mathai-McDyer link, a top-flight duo now working earnestly on her behalf.

Dr. McDyer

UPMC Lung Transplant Program

So there you have it. Wigley, Mathai, and McDyer--Colleen's main cast of characters in this pre-pre-transplant phase of her "long day's journey." 

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What is scleroderma? A rare illness of the immune system in which the immune system begins, for a reason or reasons yet unknown, to work against the body it is designed to protect. A normally functioning immune system defends the body by fighting off foreign invaders, such as, viruses and infectious bacteria. An immune system with an autoimmune dysfunction, such as scleroderma, on the other hand, mistakes the body's own tissues for foreign invaders and sets up a protective attack that backfires and wreaks havoc that ultimately becomes fatal. Scleroderma is a chronic and progressive autoimmune disease, its target the body's connective tissue. 

What is connective tissue? In simple terms, connective tissue is one of the body's primary tissues and resides, well, simply everywhere inside us. It's an "extensive extracellular matrix"--or cellular glue--that supports, protects, connects, and separates other tissues and every organ of the body. Even blood vessels travel through webs of connective tissue.  

A primary components of connective tissue is the protein collagen. In a body with scleroderma, cells start making collagen  as if responding to a cellular injury needing repair. Once begun, however, this process doesn't turn off and, as a result, far too much collagen is generated. Excess collagen in the tissues then causes a cascade of problems that can culminate, over time, in serious damage to vital organs and even impairment of the circulatory system. Inflammation plays a part in all of this, but I don't know whether inflammation triggers or follows the immune system's runaway collagen production.

In essence, scleroderma tightens and hardens the body's connective tissues. In fact, scleroderma literally means "hard skin." Colleen's lungs, for instance, are roughly 50% fibrotic--or hard. Envision a live sponge in ocean waters. Normal lungs are soft and elastic like this. Lungs rendered fibrotic from scleroderma, on the other hand, are hardened and inelastic. Thus, the increasing inability to breathe. Thus, the difficulty of the right side of the heart to pump blood through the lungs. Thus, the hypertension or bulging of the pulmonary artery that connects the right side of the heart to the lungs. And so on. 

The cause of scleroderma remains unknown and currently there is no cure. Autoimmune disorders appear, to me at least, to be among the most baffling diseases. Treatments for scleroderma exist to prevent or limit its damage, research continues, and remissions of various lengths are possible--Colleen experienced remission for a time. But, meanwhile, this quixotic disease pretty much has its way. 

Yours,
The Professor