Home Again

This past week at Johns Hopkins was a challenge and a learning experience.  As many of you know, once you enter the medical labyrinth, it all becomes completely....

Whether it's medical info and decisions or figuring out what is covered by insurances while feeling so lousy, you're completely undone.  I can honestly say that I'm not quite sure what the heart follow-up is going to be after Wednesday's procedure was aborted.  I think I heard two different things.  I received word today, however, that Dr. Mathai will be calling me to brief me again.  Although I have high quality medical insurance, it would not cover the supplemental feeding.  The nutritionists went to work to figure out an alternative in order to lower costs.  Then, I received a call today telling me insurance will pay for equipment supplies, which leaves me only paying out-of-pocket  for the food itself, not everything.  That saves a few hundred.  I learned you can't do anything about this insane maze that can take advantage of those of us who are compromised for some reason or another.  Be patient and keep inquiring until you find someone who takes the step to find the answer.  Secondly, have someone to back you up because you can't keep it all together yourself.  That's why we have each other.  I know I keep saying this but the support has kept me going.  Prayers, cards, notes, trader joe's chocolate, inspiring quotes, blog followers, and all.  If I could get points for all of this, I could fly around the world twice.  How lucky am I?

Colleen is home.

LASSIE COME HOME
Our lassie is coming home tomorrow. Yep. And she is unequivocably ready to leave now, just as she was at the end of her previous Baltimore stay, not all that long ago. 

A complication in this morning's heart procedure caused it to be called off during the first step of a two-step process. This outcome does not preclude Colleen going forward with the procedure again in the future--as early as two weeks time, if she wants. The cause of today's complication (apologies for vague medical jargon) should be a non-issue by then, we are told.

All the same, after today's aborted mission, I think it safe to say that Colleen, so willing to do all that's necessary, is nonetheless feeling beaten up right now and wants nothing else but to go home.

And tomorrow she will do just that. Yep.

I'm All A-Flutter 
(from Colleen, dictated close to midnight EST)

It seems as though my heart's flutter is fluttering more often that it used to. No, I didn't meet a debonair doctor.  Rather, it's a consequence of the increasing strain on my heart from the persistent difficulty of its trying to pump blood to my lungs. A procedure known as ablation, in the majority of cases, can correct an a-flutter like mine. An electrical cardiologist here at Johns Hopkins will perform this procedure for me sometime in the next few days. Normally, this is outpatient. Naturally, I have to complicate matters because, I was told, general anesthesia is required in my case. 

It was a day of decision-making as there were other options to consider. I consulted with "my cabinet," contemplated, and decided to move forward. The odds are good that the flutter will be eliminated, which will help give my heart some relief. So the decision was made by  Executive Order, needing no congressional approval. Thank God. After this, let's hope my next heart-flutter is caused by Doctor Debonair.

deja-vu 
loyal readers, have we been here before? colleen scheduled to depart Baltimore only to be instructed, at the 11th hour, to remain? is life repeating itself?

as it turns out, the snowstorm was only one glitch in Colleen's morning. The other was an unexpected visit to her hospital room by two doctors from Cardiology, whom Dr. Mathai had brought in for consultation regarding a known issue with Colleen's heart -- an "electrical" issue. The cardiologists said to Colleen, "Think of us as electricians." 


two different options for fixing her heart's electrical circuitry -- one temporary fix, one permanent -- were laid out for her today. after she discusses this with Dr. Mathai and gets his opinion, colleen will decide which course to take. her "a-flutter," as it's called, may underlie, or play a role in the extreme edema of late and also her ongoing, extreme breathlessness.

many pieces to this puzzle, everything connected.

.....

almost 8:30 PM in Baltimore now...colleen spoke on the phone with our brother Danny who lives in Ventnor, another South Jersey shore town near Ocean City...Dan has undergone the permanent heart procedure himself, with success...she's leaning in that direction...departure delayed...stay tuned...

.....

perhaps you've asked yourself, as I have, why is baltimore so reluctant to let colleen go?

are angels in residence like they were in pittsburgh?

then there's the irish connection i came upon...and we irish-americans don't exactly not believe in fairies -- the irish equivalent of angels...

so i leave you with this factoid:
  

the city of Baltimore is named after Lord Baltimore, who was a member of the Irish House of Lords and, ultimately, the founding Proprietary Governor of the of Maryland. He was the son of First Lord Baltimore of County Longford in Ireland in the 1600s. Maryland's Baltimore was one of the first American revolutionaries, playing a key part in the fight against the British...(Colleen, the historian, knows more about all this)...

the name Baltimore is an anglicization of the Irish Gaelic name Baile an Tí Mhóir" from which the village of Baltimore in western County Cork derives its name...

Baltimore, County Cork, Ireland

Plot Twist: Storm Slams South Jersey, Baltimore too

     Winter Storm Virgil is making its final stop 
     in the Mid-Atlantic after dumping heavy snow 
     in the Midwest. Possible robust snowfall in
     South Jersey. Inches expected as far south   
     as Washington DC and Baltimore.

Leslie made it as far as the Garden State Parkway this morning -- only a few miles inland from Ocean City -- and, not for lack of valor, made the wise decision in the face of heavy snow falling to turn back. 

Cliffhanger: Will Hopkins let Col stay the night? Will she make it home tomorrow?

Last Night in Baltimore
Another chapter at Johns Hopkins Hospital comes to an end. Colleen is going home tomorrow. Leslie is driving down to pick her up. End of.

       Colleen's Baltimore Campus

END-OF-ANOTHER-ANOTHER-ANOTHER-DAY UPDATE

Colleen feels better, and breathing is easier for her with each passing day. Last night, finally, she got real sleep, and this afternoon too, even though the day entailed moving from the 7th floor "Step Down Unit" -- the next rung down from ICU -- to a general medical floor. She had to say goodbye to the 7th floor nurses she'd grown close to, but told me that several familiar faces have already come by her new room -- a doctor from the 7th floor and the food tray guy.

Obviously, she wasn't discharged today. And, for the moment, Col is absolutely okay with that.

.....

Col's sleeping

End-of-Another-Another-Day Update
All quiet on the Baltimore front, where night has already settled in. Sweet dreams, Col.

End-of-Another-Day Update
Colleen continues to feel better. She tells me that a hospital team of doctors -- who are in and out of her room during the day -- is working to fine-tune her oxygen, the volume of her supplemental nutrition (reduce liquid while maintaining the necessary calories), and her various medications at the same time that they carefully eliminate the excess fluid that led to this emergency hospital stay. The team is purposely doing this slowly, in order to put as little stress on her body as possible.

So will she come home Saturday? (Sound familiar?) 

Col says it's doubtful. And right now, she's not minding that relatively small uncertainty. Rather, she would very much like to have a good night's sleep.

Day at a time.

Good Sleep?
Not. A bed didn't become available until 1 AM last night. Colleen wasn't fully settled in her room until 2:30 AM. She's "feeling better" but she's tired. Her daughter Chelsea is driving down to see Col today; Beth and Pat are planning to go down tomorrow.

End-of-Day Update

Col's Johns Hopkins pulmonologist, Dr. Mathai, wants Colleen, still stable, to stay in the hospital a few more days. Although her being in Baltimore means being 3 hours away from everybody, we feel the 24/7 nursing care, the proximity to her doctor and, hopefully, some good sleep, are the best things for Colleen right now.

Two hours ago -- 8 pm EST -- she was still in the ER waiting for a bed and feeling restless. (Pat & Beth had just headed back up to New Jersey.)  It's 10 o'clock there now, so too late to call to see if she's settled in her own room. Please be so.

Powerless.

Stabilized

The Johns Hopkins ER has stabilized Colleen.

No way to say this any easier: back to Baltimore

Colleen is, at this moment, on the road back to Johns Hopkins hospital, her friends Beth and Pat driving. Over the last two days, fluid has built up again to the point where only medical staff can intervene. The fluid buildup is related to the heart-lungs and also kidney symbiosis. Strained lungs cause a backup of blood from the heart which causes hypertension in the pulmonary artery between the heart and lungs and also means that the blood and oxygen flow throughout the entire body, including the brain, is insufficient which then, don't know how exactly, impacts the kidneys. It's at this point, I believe, fluid retention begins.

Prayers.

Postscript to Saint Patrick & Saint Brigit's Day

                                 Isabel Stenzel (left), double-lung recipient  

From the San Francisco Chronicle, March 18, 2013, via my friend Loy in Palo Alto:

Ten years ago, Isabel Stenzel couldn’t walk without getting short of breath. On Saturday, she’ll be playing the bagpipes in San Francisco’s St. Patrick’s Day parade — a job that’s taxing even for people who haven’t undergone a double lung transplant.

The 41-year-old social worker from Redwood City emerged from the operation in 2004 able to breathe normally after a lifetime with cystic fibrosis, a genetic and chronic disease that builds up heavy mucus in lungs and makes breathing almost impossible.

The sound of the bagpipes “is so powerful,” she says, calling it “my donor’s voice, my donor’s sound that would be heard by the world. What a beautiful way to honor his gift of lungs.”

She’d never even played the pipes before her operation.

.....

When Isabel Stenzel wails away on her bagpipes, playing "The Wearing of the Green" and marching in Saturday's St. Patrick's Day parade in San Francisco, she'll be relying on her own fingers, her own legs and someone else's lungs.

"I was so sick before," Stenzel said. "I couldn't even walk without being short of breath. At the end, I couldn't even brush my teeth without being short of breath. And here I am marching, da-da-da, playing 'Scotland the Brave'  in full uniform. I'm like, 'What?' It's more than a dream."

Playing wasn't even a pipe dream in the months following Stenzel's transplant at Stanford Hospital. She had to blow into a device to help reinflate the donated lungs after they shrank slightly in transit, then slowly built up her breathing capacity by jogging and swimming.

Bagpipe fever struck, however, at a donor-recipient event in Hayward a few months after her transplant, when a bagpipe troupe played.

Honoring donor

Stenzel had never played the pipes, but "when they came on stage, I was like, 'Wow. I want to do that,' " she said. "The sound is so powerful. It wouldn't be my sound, but it would be my donor's voice, my donor's sound that would be heard by the world. What a beautiful way to honor his gift of lungs."

She began taking lessons near her job in San Jose and joined the Stewart Tartan Pipes and Drums in 2007, practicing weekly at a church in Saratoga and drilling marching formations in the parking lot.

Stenzel has devoted her life to organ donation advocacy. She and her twin sister, Anabel, were both born with cystic fibrosis - her sister has received two double-lung transplants - and they wrote a memoir about living with the disease that inspired a documentary film in 2011. They're both active in helping others in the Bay Area with cystic fibrosis, and Stenzel has gotten to know the family of her donor, an 18-year-old Fresno man who died in a car crash.

But when Isabel Stenzel puts on her wool tartan plaid kilt, doublet, cape, spats and ostrich-feather bonnet Saturday to march up Market Street, she'll take off her advocacy hat. 

Just like anyone else

"What I love is that my identity my entire life has been dominated by my illness, and I've always been the CF patient, the sick girl, the transplant recipient," she said. "In my piping band, I'm just another piper."

Bagpipes require deep lung capacity, enough to fill the bag with air, which then leaves through three drone pipes that always play the same note and one chanter that plays a melody.

Stenzel, like other beginners, started playing with all three drones plugged to lessen the airflow demand. She had played piano and violin as a child but never a wind instrument, and had no idea how to direct her air. As she played more and conditioned her lungs enough to unplug all three drones, her breathing capacity grew from 95 percent of normal to 125 percent, she said.

"It amazes me that I have the lung power to march and play," Stenzel said. "In one of my earlier parades, I had this very surreal and out-of-body experience. I felt like I was living a different life. I was in a different body, and in a way I am."

This new life, while sweet, could also stop at any point, Stenzel knows. Lung recipients face a risky future even after a successful transplant, with odds slightly higher than 50 percent that they will survive more than five years after the operation.

Twin's second transplant

Stenzel's twin had a double-lung transplant in 2000 and another in 2007 after her body rejected the first set. Stenzel's lungs are vulnerable to infections, and while high airflow from playing the pipes can help, she also has to be vigilant about mold that can grow in the instrument.

She can't swap her pipes with other players, who pass them around at shows and festivals "like a cup of whiskey," she said. And even if she's careful, transplanted lungs can slowly lose function over time.

"Rejection is always a possibility," she said. "I even get very tearful thinking about how someday, I'm not going to be able to play the pipes. I guess that's the case for every human being - all of us have to give up something we love as we age. For me, it's much more imminent."

But while she's still able to play, she said, it's "life-affirming," for herself and for her donor's family. "I feel so alive."

Donating organs

To register to be an organ donor, go to www.donatelifecalifornia.org.

Hepititis B

Another doctor's visit today
First shot in the series of 3 over 6 month s.
No sweat, a prick and a burn.

UPMC wanted me to have this vaccine just in case a lung donor had had this virus.  I know Chelsea had to get it before college and I understand now they give it to the babies.  Most adults  recover in a short time. However, Sometimes the virus causes a long-term infection, called chronic hepatitis B. Over time, it can damage your liver. Babies and young children infected with the virus are more likely to get chronic hepatitis B. 

You can have hepatitis B and not know it. You may not have symptoms. If you do, they can make you feel like you have the flu. But as long as you have the virus, you can spread it to others through contact with the blood and bodily fluids.

Word to the Wise...You may get hepatitis B if you:

• Have sex with an infected person without using a condom.  
• Share needles (used for injecting drugs) with an infected person. 
• Get a tattoo or piercing with tools that were not cleaned well.
• Share personal items like razors or toothbrushes with an infected person. 

I think I'm safe in all these categories.

Helping Hands

I hope your spirits were lifted when you heard the sound of the pipes.  I settled for TV viewing.  It's not the  same but it was still great and it spurred me on to listen to my great Irish CDs, courtesy of Larry.  I missed a good cold Irish Beer and a sing-a-long.   My days have been unbelievably busy.  All of a sudden it's 2 0'clock and I haven't accomplished much.  Tasks take a long time.  Nellie and I are adjusting to one another.    I look like a mad scientist, gloves, swaps, water, .....but she doesn't wake me up anymore at night.  My stuffed up nose from high flow oxygen does.

I woke fatigued and more breathless so the monkey mind gets going. You think you're the only one going through this or that.  Then, for some uncanny reason, you connect with someone who is there to give you a "helping hand".  Thanks to the internet, more of us can find each other.  Ilene from Florida and Scott from right here in Atlantic County spent hours on the phone giving me advice and letting me know some of the logistics of  transplant.  Once again, it made me realize that I'm not in this alone and there are so many good people out there offering a helping hand.

Today, I will do the best that I can.

reporting in . on somers point via santa monica . gusting, high winds of the medical variety . holding steady

                                                

     Since returning from Pittsburgh a month ago on February 16, and since coming home from Johns Hopkins 10 days ago on March 10, Colleen has been fully engaged in reaching the goals set for her by Pittsburgh's Lung Transplant Committee--with Dr. Mathai & Dr. McDyer having her back, so to speak.

     To put it more graphically, my opinion as a manual transmission driver is that Colleen has shifted from 1st to 4th gear without the luxury of stops in between.

     There's a deadline now. Or a "kinda" deadline, since nothing's exact in this arena of infinite variables.

     May 13.

     This is the date of Colleen's next scheduled visit to the University of Pittsburgh Medical Center. Pittsburgh requires all of its lung transplant candidates--even deferred lung transplant candidates like Colleen--to have a very specific series of tests done every three months. No exceptions. And these tests have to be done in Pittsburgh. No exceptions. 

     Colleen's first 3-month checkup is now scheduled for May 13. And, thus, has become her personal deadline for reaching the goals necessary to be put on the transplant list. The Lung Transplant Committee believes that Colleen has a "small window" in which to get a new pair of lungs. Colleen and all of us heard that message loud and clear.

     So, with all that in mind, the following To-Do list might give you an idea of what daily life at 10 Village Drive is like for Colleen at the moment: 

   Put forth the effort to gain weight: do your   
   best, in spite of how difficult it is, to eat 600 
   extra calories a day, in addition to giving myself
   supplemental liquid nutrition at night through 
   NutriNellie; keep NutriNellie clean 
  
   Make sure to get between 50-60 grams of protein a 
   day.

   Put forth the effort to gain strength, especially 
   in the legs, the breathing muscles, and aerobic
   endurance: a Physical Therapist comes to the house
   2 times a week, an Occupational Therapist 2 times 
   a week; Colleen is doing as much indoor walking as
   she can manage. The specific goal here is to be 
   able to walk 400 feet in 6 minutes.

   Put forth the effort to stay in good, regular 
   communication both with Johns Hopkins and with 
   Pittsburgh.

   Be sure to do all required testing, which means 
   making and keeping all necessary doctor 
   appointments, including Colleen going back to 
   Baltimore the week after next to see her primary 
   scleroderma physician, Dr. Wigley. She hasn't seen
   the beloved Dr. Wigley since before going to 
   Pittsburgh. So I'm sure he's eager to see her 
   after all that's transpired since then.

   Welcome the RN, who now comes a few days a week,
   and also Mickey, a friend and home-health aide, 
   who comes for a few hours one or two times a week.
  
   Welcome visitors. Take phone calls.

   Get dressed, shower, wash hair, prepare meals, 
   drink water, manage groceries, manage oxygen 
   supplies and equipment, tend to nose rendered 
   quite vulnerable due to 24/7 oxygen canula, manage
   clean clothes,count pennies, manage sanity and, oh
   yeah, rest. 

   Aim for personal bests.

   Prepare to move to Pittsburgh at a moment's 
   notice.

And that's not all of the iceberg.

Do you think this is a full-time job? Do you think
this is a job for the faint-of-heart? Do you think
even a person with fully operational lungs would 
have the mettle to take this on?

Questions to ponder.


Reminder to all, including myself:  resist the temptation to talk too long on the phone with Colleen. To those who visit: know that, for right now, a short stay is far better for her than a long one. Suggestions only.


PS:  We checked in by phone today with Judy and Ed, whom we met at Family House in Pittsburgh. After being there since before Christmas for Ed's single-lung transplant surgery, they just returned home to New York yesterday. Ed is doing great and no longer psychologically attached to the oxygen canula. Hooray. I asked about the others we'd come to know well at Family House. They're all home now too.

5:30 pm pdt . san francisco bay

colleen . thought of you many times today . walking in Muir Woods where you and I walked . going back and forth across the Golden Gate Bridge, shrouded in fog on the way into the city, sunlit and shining on our way back to Mill Valley . perusing a Dutch exhibit at the de Young Museum in Golden Gate Park, seeing Vermeer's Girl With a Pearl Earring hanging in a room by itself

i fell in love with this 1654 painting called The Goldfinch by Dutch painter Carel Fabritius

missed you

Heave The Anchor Short

The fog of the week in Johns Hopkins hospital is slowly lifting and I am prepping to set sail.  It’s been rocky seas.  Chelsea is able to stay with me for a while and that is a major help, physically and emotionally.  The organization and assessment of home care is underway.  The health care service, Holy Redeemer will serve as my anchor for now.  Caring for Nellie, gaining strength and endurance and returning to being as independent as I possibly can are the big goals.  Specifically, I want to be able to walk 400 feet in 6 minutes when I return to UPMC on May 13^th.  Oh, and 5 additional pounds would be a bonus.  Training began today.  It’s depressing to see how quickly my body has weakened.  I have to admit that I let my fatigue and feelings of defeat win out over the more important need to move the body.  Now, the starting point is farther behind than it should be.  Yes, I’m going to start where I am but I wish I had reached out sooner for help getting me back to my “mat”…or as the ship’s captain might order, “head to the eye of the wind”.

This is a replica of the  fast sailing, Baltimore Clipper that first appeared after the Revolutionary War and remained a favorite until after the War of 1812. 

Home

This bird is finally home but I’m not singing a sweet song yet.  Once again, thanks to Mignon for keeping everyone up to date.  I have my feeding tube, aka Nutri-Nellie.  I’m awaiting a nurse to show me how to use this set up.  I remember how freaked I was when I got the pacemaker but I managed to get used to it.  People say it’s easy.  Yeah, sure!  It does not look it to me.  George, a friend of mine just arrived home with his and apparently he’s a happy camper.  Not me, I’m nervous as hell and dreading the Moo-Moo look.  Makes me wonder what style George got?  Or, maybe George has some cute nurse filling his.  Given this and my recent hospital visit, I question whether or not I’m up for the challenges that lie ahead.  That being said, Valerie Harper said in her book, I, Rhoda, “Life is amazing.  Stay as long as you can.” It’s the “tude”, right Suze.

Med School Anatomy, Sorta

Given the title of this post, I suspect absolutely no one is going to give it a read. No worries. Except for bleary-eyed med students, I'm probably the only creature in LA right now, at 9:30 pm, who's got the anatomy of the heart and lungs on her mind, and wants to write about it too. So feel free to ditch.

A few days ago I posted a black & white drawing that I really love of a bird atop a pair of lungs and the handwritten words "The Love Between Two Lungs." 

My primary focus was on the bird and the lungs, secondary the dangling heart that I figured must represent "the love between." The artist's positioning of the heart between the lungs struck me as odd. Recently, I chided my friend, alias Larry, about not knowing we each have two lungs. But now I find I have been similarly clueless about where the heart lies inside us. I kinda sorta thought it was somewhere close to the left lung but on the lung's outer side. Where the heart resides exactly I didn't know and didn't even realize I didn't know. 

Whenever Colleen and I talk about the pulmonary artery connecting the right side of the heart to the lungs, I have always assumed that the lungs have a single pulmonary artery only. No, I've just learned. The lungs have two pulmonary arteries, one for each lung. During those discussions, I have also vaguely imagined the heart beating somewhere north of the lungs. Wrong.

For the past few days the image of the bird, the lungs, and strangely positioned heart nagged at me. So I went Googling and found out not only where my heart is but also that it only weighs about 9 ounces. It makes up less than 0.5% of our total body weight.

Voila:

The heart isn't "outside" the lungs, nor is it located on the left of the left lung. Rather, it's almost dead center in between them. I say "almost" because 2/3 of the heart is left of the midline of the body (think sternum or spine) and 1/3 of the heart is on the right. 

How could I have not known this?

The Pledge of Allegiance comes to mind. From kindergarten on, we recited the Pledge of Allegiance at the start of every school day. We each put our right hand on our heart, or what we thought was our heart. Every kid's hand, including mine, was placed somewhere below the left clavicle and to the left of the sternum. As an adult, I somehow came to know that the heart was not located in that upper left quadrant of the torso, though I continued to think it was somewhere in that vicinity, only lower. I never, not once, thought about it's proximity or relationship to the lungs.

(The size of the lungs in this illustration surprised and stunned me. More on that to follow.)

This next, purposely distorted diagram nicely illustrates the fact there there are two pulmonary arteries (in blue):

This last illustration gives us an idea of what pulmonary hypertension and an enlarged right heart look like:

Colleen's pulmonary hypertension--all pulmonary hypertension--affects the left pulmonary artery only because the fibrosis or hardening in her lungs makes pumping blood into them more difficult for the right heart. As a consequence, the pulmonary artery swells under the pressure of blood unable to course through it swiftly and, also, the right heart swells or enlarges as well. The inelastic lungs become somewhat like a dam. Everything behind it backs up.

.....

If you're still reading, did you know that the lungs are as big as these images show them to be? Our lungs take up almost all of our chest space and, along with the heart, sit inside our ribs for protection. 

The size of adult human lungs vary greatly. But the average length is 10 to 14 inches and they weigh an average of 2 to 3 pounds! (Remember that the heart, by contrast, only weighs on average 9 ounces; the size of the heart is only, approximately, the size of a fist.)

.....

Now that Colleen is in full throttle exploring the possibility of a lung transplant, I want to be as un-vague as possible about all aspects. Until now, I had given little thought to the organs of the lungs themselves. If Colleen has lung transplant surgery, the donor lungs will likely be somewhere around 10 inches long. And in order for the surgeon to attach these new lungs into Colleen's body, to make them hers, he (and we're pretty sure it would be a "he") has to work around the heart situated right in the middle of things.

I'm glad to know this.

Yours,
The Professor

She's back home at the Point

Bye Bye Baltimore Tomorrow -- For Real

Colleen should be home by late afternoon tomorrow (Thursday). This probably goes without saying, and Colleen didn't ask me to say this, but let's all be cognizant of how much she needs to rest and get stronger. Colleen's eye is back on Pittsburgh now; on the Selection Committee saying an unqualified yes to putting her on the lung transplant list, sooner rather than later, because her ill-defined "small window" sounds a lot like "soon." So we all, myself included, need to exercise discretion regarding long phone calls and protracted visits. Unlimited amounts of love? Not a problem. Over and out. Mignon

Bye-Bye Baltimore?

If they don't let Colleen out tomorrow afternoon, Dawn & Sandi are going to have to go down and break her out of her 9th floor window. Otherwise, there's no telling what mayhem might ensue. 

.....

This just in: Colleen tells me that 6-10" of snow are predicted for Baltimore tomorrow; high winds and rain predicted for the entire Jersey coast. If and when Colleen learns she's free to go home, she'll phone Sean, who'll be ready and waiting to head down to Maryland to get her. What will all that snow do to the freeways? Stay tuned.

                          

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