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.

.....

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
.

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." 

.....

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

sunday night . baltimore via california

...tuesday or wednesday still looking good for Colleen to go home...chelsea made it to baltimore today, swinging by the University of Delaware to pick up her first cousin (Col's niece, our sister Kim's daughter) hayley on the way...so together they visited colleen at johns hopkins...colleen is in repossession of her chargers and also fresh underwear...whew...

just in case anyone is tripping to trader joe's

. . . of this particular bar, Colleen is fond

                . . .just sayin'

saturday night

today was a quiet day for Colleen...no tests...Dr. Mathai came by for a visit...the pain from Wednesday morning's surgery is still there but subsiding...Chelsea is driving down to Baltimore tomorrow and bringing Colleen's request for her gadget chargers and clean underwear...not until helping Colleen pack for Pittsburgh and then helping with the laundry when we got back to Somers Point did I learn with what care my sister attends to her undergarmentry...impressive...heads-up on her hospital discharge:   it's looking like the earliest would be Tuesday or Wednesday...

thanks for being my saturday night dates

santa monica ocean avenue @ sunset

just so you know, i've been reading your "Comments" to colleen over the phone & she's been lovin em.

PS: No cause for alarm at Colleen's delayed hospital discharge--Johns Hopkins is merely erring on the side of thorough. We like thorough. So when might she head home? We don't expect it to be much longer. You'll know as soon as we do. Colleen says the hospital has been "wonderful" to her.

this just in...dictated to me for you by colleen. . .

TWO LUNGS

How can an educated, professorial, Europhile, American male who's lived more than 60 years with all senses intact, who's rubbed elbows with presidents, the rich and famous, and the Dalai Lama, who's lived in Dublin and Bonn, who flew on the mission to drop emergency supplies at Wounded Knee on the Pine Ridge Indian Reservation in South Dakota, and who's my friend of thirty years and go-to catsitter--how can this person, who prefers to remain anonymous for purposes of this blog post and whom I shall call Larry, how can this worldy-wise individual not know that we, all homo sapiens, have not one but two lungs?

He called last night to ask about Colleen, which inevitably led to medical detail about her status and about lung transplants.

"We have two lungs?" he said, sounding astonished.

"Uh, yes, Larry."

"Two lungs?" he said again, incredulous. Sometimes it can take a while for Larry to grasp basic life information like this. 

"You mean you thought we only had one?" I said.

"Uh, I guess. Do we really have two lungs?" 



This vignette is dedicated to Colleen and our family members who've listened to my Larry stories for years now. Colleen, I know you've got a big smile on your face. I don't think Larry will mind a welcome laugh at his expense. He's a kind, thoughtful man. Just clueless about anatomy.

Larry, if you're reading this, see drawing below:

PS to Larry: I just read this post over the phone to Colleen at Johns Hopkins. She did, indeed, laugh! So thank you for that, my friend. "Did he really not know we have two lungs?" she said to me. "That's exactly what I said to him, Col!"

She asked me to tell you that she's been enjoying your "Irish Pics," the music you recorded for me some time ago and that she downloaded to her own ipod and has been listening to in her hospital bed at night. In the suddenness and flurry of having to leave quickly for Baltimore, she remembered to pack her ipod but forgot the charger. So she's conserving the battery by waiting 'til evening to use the ipod. It's been a godsend, she says.