We've heard the watchtower say that organ transplants can change your personality but this story suggests a transplant can cure you- twice over!
http://www2.bostonherald.com/lifestyle/health_fitness/live02112002.htm
Liver transplant from living donor cures hemophiliac
by Michael Lasalandra
Monday, February 11, 2002
In an apparent medical first, an Abington man who underwent a living donor liver transplant last summer got a huge bonus benefit - he was cured of his hemophilia.
``To this day, it's still hard to fathom,'' said Tom Wales, a husband and father of a 12-year-old boy. ``Hemophilia has been a part of my life for 39 years.''
As a result of the cure, Wales is now able to play catch with his son for the first time and is considering taking up skiing once he undergoes a back operation until now considered too risky.
Hemophilia, an inherited blood disorder, prevents blood from clotting normally, causing excessive bleeding.
``It's a whole new life,'' said the 39-year-old former welder. ``I feel like I'm reborn.''
It may be the first time that a living donor transplant has been performed on a hemophiliac, experts said.
Dr. Elizabeth Pomfret, one of four surgeons involved in performing the liver transplant at the Lahey Clinic, said hemophilia is caused by a deficiency of factor VIII, a protein made in the liver that allows blood to clot.
``He tells me all the time the most incredible part of this whole thing is that he is no longer a hemophiliac,'' she said. ``In the past, anytime he would suffer any kind of injury, even just a bruise from fooling around with his son, he would need a transfusion and (an infusion of) factor VIII.''
Like many hemophiliacs, Wales contracted hepatitis C from a blood transfusion and it eventually caused him to develop cirrhosis of the liver. That's why he needed the transplant.
Wales said a liver specialist had told him years ago that a transplant would cure his hemophilia, but he also told him ``they don't do transplants for that reason.''
He went on a waiting list for a cadaver transplant two years ago, but his doctors at Lahey Clinic told him he probably wouldn't make it, as it can take several years to get to the top of the list.
They said his best hope was a transplant from a relative or friend, where surgeons take a portion of the donor's healthy liver and transplant it into the sick patient after removing the diseased organ.
Wales' brother-in-law, Walter Moran of Hanover, offered and proved a suitable tissue match.
``I was blown away,'' Wales said. ``It takes a special person to consider something like that.''
Wales said he started feeling better immediately after receiving the transplant.
Before the operation, he said, ``I was sluggish all the time. I probably felt like I was 60 years old or older. I'd fall asleep during the day.''
Although Pomfret said it was known that a successful liver transplant from a cadaver would cause his hemophilia to go away, it was not so clear that a living donor transplant would do the same thing.
``We just weren't sure if just giving him a piece of a liver would do it,'' she said.
Blood tests taken soon after the transplant, however, showed the new liver was making plenty of factor VIII.
``It was really quite remarkable,'' she said.
Fewer than 50 hemophiliacs have received liver transplants, said Dr. Arthur Rabinowitz, hematologist at Lahey.
But because more and more hemophiliacs are developing end-stage liver disease, ``more and more are getting liver transplants,'' said Steven Humes of the National Hemophilia Foundation.
However, he said he had never heard of a living donor liver transplant performed on a hemophiliac.
At the University of Pittsburgh, where pioneering liver transplant surgeon Dr. Thomas Starzl discovered 13 years ago that a transplant could cure hemophilia, doctors have performed about 30 transplants on hemophiliacs, including seven with HIV.
But none has been a living donor transplant, said Dr. John Fung, chief of transplantation.
Nobody, however, is suggesting that liver transplantation be done just to cure hemophilia.
``That's not something we would recommend,'' Humes said, citing the traumatic surgery involved - for both donor and recipient.
``Liver transplantation can cure hemophilia but it wouldn't be appropriate in the absence of liver disease,'' said Rabinowitz. ``The operation has a high mortality potential.''
Science, instead, is looking to gene therapy to cure the inherited blood disorder. Researchers at a number of locations are studying inserting enough healthy clotting factor genes into patients' liver cells for their blood to clot normally.
One study, published last year by doctors at Beth Israel Deaconess Medical Center, raised hopes that the technique may work.
The technique was tested on patients with hemophilia A, the most common form of the disorder, which afflicts 15,000 in the United States. It is the form that had afflicted Wales.