Peter's Progress: July 2011

Thursday, July 7, 2011

Reciting the alphabet - forwards and backwards

Peter will be six on November first, so he is only five years and eight months old. Tonight he recited the entire alphabet BACKWARDS! He has long known the entire alphabet and the phonic sounds associated with each letter, but out of the blue he started with Z and went all the way to A consecutively, without missing a letter. Wow!

Peter lost his fifth baby tooth a few days ago. So far, all four bottom front permanent teeth are brown from tip to botton. I can't tell what color the new top front tooth will be, but had a glimpse of white when brushing his teeth tonight, a brief moment of excitement, and then realized that it was only toothpaste, not the new tooth. We continue to wait and prayerfully hope that the other permanent teeth will be white, or at the very least, not MORE discolored than the baby teeth are.

Peter can now swing on the monkeybars. He hangs on one bar and swings back and forth from that one bar a couple of times, but he is propelling himself, not screaming in fear, and is rightly proud of his new feat. One of these days, I'll post that he can finally jump... perhaps a video to go with that one!

Finding Omegaven

Omegaven is the lipid that saved my son from TPN-induced liver failure. I am posting links for anyone trying to obtain Omegaven or find out more about it. Dr. Mark Puder at Children's Hospital Boston is the one who has done the research on Omegaven, and he is a contact to remember if you look into getting this lipid for your child's TPN:

http://www.childrenshospital.org/doctors/mark-puder

http://grey.colorado.edu/shortgut/index.php/Omegaven#Omegaven
Good site for short-gut as well as anyone on TPN therapy, also has good info about Omegaven and kids' success stories

http://oley.org/documents/How_Physicians_Can_Obtain_Omegaven.pdf
How your doctor can obtain Omegaven

http://www.childrenshospital.org/newsroom/Site1339/mainpageS1339P1sublevel222.html
Info about the first child who was put on Omegaven and Dr. Mark Puder

http://eleanorbrogan.blogspot.com/2009/01/omegaven-welcome.html
Info on Omegaven, a success-story herself, and has links to MANY other children with short-gut... see the links for all of this on right-hand side of her blog page

Welcome to Holland

I was told about this short essay two years ago. I continue to appreciate this piece and wanted to post it to share with others.

WELCOME TO HOLLAND
byEmily Perl Kingsley.
c1987 by Emily Perl Kingsley. All rights reserved

I am often asked to describe the experience of raising a child with a disability - to try to help people who have not shared that unique experience to understand it, to imagine how it would feel. It's like this......

When you're going to have a baby, it's like planning a fabulous vacation trip - to Italy. You buy a bunch of guide books and make your wonderful plans. The Coliseum. The Michelangelo David. The gondolas in Venice. You may learn some handy phrases in Italian. It's all very exciting.

After months of eager anticipation, the day finally arrives. You pack your bags and off you go. Several hours later, the plane lands. The stewardess comes in and says, "Welcome to Holland."

"Holland?!?" you say. "What do you mean Holland?? I signed up for Italy! I'm supposed to be in Italy. All my life I've dreamed of going to Italy."

But there's been a change in the flight plan. They've landed in Holland and there you must stay.

The important thing is that they haven't taken you to a horrible, disgusting, filthy place, full of pestilence, famine and disease. It's just a different place.

So you must go out and buy new guide books. And you must learn a whole new language. And you will meet a whole new group of people you would never have met.

It's just a different place. It's slower-paced than Italy, less flashy than Italy. But after you've been there for a while and you catch your breath, you look around.... and you begin to notice that Holland has windmills....and Holland has tulips. Holland even has Rembrandts.

But everyone you know is busy coming and going from Italy... and they're all bragging about what a wonderful time they had there. And for the rest of your life, you will say "Yes, that's where I was supposed to go. That's what I had planned."

And the pain of that will never, ever, ever, ever go away... because the loss of that dream is a very very significant loss.

But... if you spend your life mourning the fact that you didn't get to Italy, you may never be free to enjoy the very special, the very lovely things ... about Holland.

__________________________________________________

Peter's history - condensed version

In March 2006, at four months of age, Peter began to stop gaining weight and was found to be quite anemic. This led to a lengthy period of testing at Children's Hospital of Philadelphia, many frustrating moments in the hospital, and ultimately the discovery of a vein and artery communication, called an arteriovenous malformation (AVM).

This AVM was in Peter's liver and was originally thought to be reparable through embolization, a non-surgical procedure via catheter in the femoral vein, at some point when Peter grew bigger with the help of TPN. Peter did begin to gain some weight on TPN, but also began to vomit blood and bile, developed high fevers and a white count of 120,000. None of the medical authorities caring for Peter believed that the AVM could be responsible for all of this until test after test came back negative for anything else. We had at this point come across the words "mesenteric ischemia" from two vascular surgeons, who said that it was possible the AVM was causing pain, but the elevated white count was still unexplained. We begged then that the AVM be removed, as Peter was obviously in pain, and we could try to decipher the white count later if it still remained elevated.

The day finally came for the AVM to be embolized and it was discovered that it was necessary to be removed surgically after all, due to its complex connections and size. Upon that surgery it was noted that the bowel was dusky and had two punctures in it, and that the gallbladder was necrotic (dead tissue). The punctures were dead areas of bowel that had occurred due to the AVM diverting blood away from areas of the bowel. The necrotic gallbladder occurred from this diversion of blood as well. The dead tissue and the bowel perforations releasing gut bacteria into the abdomen would explain the white count of 120,000!

Three days later Peter went down for more bowel surgery as his fevers returned and white count began to rise again. Peter came out of this surgery with his duodenum cut in half and to drainage, an ileostomy, and a G-tube. Dr. Flake, the surgeon who had been called upon now twice to save Peter's bowel, did this to try to salvage his bowel from the multiple intestinal punctures that he could now see during that surgery, which had also occurred from the lack of blood supply caused by the original AVM.

A week later, Peter developed abdominal compartment syndrome from all of the bowel swelling and had to have his abdomen opened and left open to relieve the pressure so the kidneys would not stop working. Peter's abdomen never could be closed back up, so he had to heal it with scar tissue, taking months to close.

Meanwhile, Peter developed another AVM, which apparently isn't all that uncommon when an AVM is removed and the blood circulates through channels that may have been closed or receiving less flow. No one caught that AVM. It ultimately closed on its own but left a clot in the portal vein of the liver, adding to the portal hypertension Peter already had. Portal hypertension can make a person prone to bleeding in the bowel, as can liver disease itself. Peter bled huge amounts and at one point was receiving transfusions every few days. He had huge clots of blood coming out of his ostomies, as well as diffuse bleeding from the intestines. There were several times that I had to push the nurse-call button to summon help as I held pressure over his ostomies to try to slow down the bleeds by trying to keep the clots inside the itestines to tamponnade the blood flow. (I have to thank my dad for that idea!) Peter would still end up requiring transfusions with those episodes, but his blood pressures at least stopped their free-fall if we could slow down the bleeding some.

Peter had numerous infections throughout his stay, to the point that Infectious Disease raised his temperature threshold for cultures just so we could stop the on/off antibiotic routine that goes with culturing in the hospital. Also during his stay, he also develped a fistula (hole) in the bowel going straight out of the scar tissue that he grew over the abdominal opening that had been created months ago to relieve the compression syndrome. An additional insult was that Peter's bones became so osteopenic from the TPN and bedrest (Peter had been in the ICU since the beginning of June 2006), that he had a hairline fracture on his right femur. You can see the splint on his leg in one of the photos.

I was told that Peter would need a liver transplant as the TPN was destroying what was left of his liver, and maybe an intestinal transplant as well. Fortunately, my father found an article in the Wall Street Journal just before Thanksgiving, telling us about Dr. Puder (in Children's Hospital Boston) and Omegaven. Children's Hospital Boston (CHB) also has the capability of doing multivisceral transplants, so we were to be evaluated for that as well. With blessings from Dr. Flake, but frank sarcasm from two other doctors, we transferred up to CHB very shortly after hearing about Omegaven. Philadelphia was not interested in using it. I was given a hospice brochure the day before we left the ICU to go up to Boston, the ICU where Peter had been for 6 months, as so few people believed that Omegaven could do what it was credited.

At the end of two weeks on Omegaven and in the constant care of Dr. Kim (transplant surgeon), Dr. Kamin (GI doctor), and Dr. Puder (Omegaven researcher and surgeon), Peter was off the ICU and on a regular surgical floor, and within two and a half months of being on Omegaven came home. He remains home now as we wait to see if he pulls out of the liver injury and bowel injury enough to avoid transplant. He remains still in the care of Dr. Kim, Dr. Kamin, and Dr. Puder, those very same doctors who greeted us when we arrived at Children's Boston.

-------------------------------

Update: July 2010:

I am revisiting this history and wanted to note another doctor from CHOP who also played a crucial part in saving Peter's life. John McCloskey was the attending anesthesiologist and PICU attending the week of Peter's first surgery. There was much debate about putting Peter under anesthesia due to his deteriorated condition, but Dr. McCloskey insisted that Peter could be safely intubated and anesthetized and personally handled Peter's anesthesia. Had he not taken personal interest and risk for Peter, it is quite doubtful that Peter would have ever made it to surgery.

ALSO, I need to add to Peter's list of doctors at Children's Boston, the name of Russell Jennings. He has been following Peter for the last two years or so and is another integral part of Peter's team of physicians (named above) at Children's Boston.

Thursday, July 7, 2011

Reciting the alphabet - forwards and backwards

Peter's Progress

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