In the fall of 2002, Scott was diagnosed with a rare liver disease called Primary Sclerosing Cholangitis (PSC). This is a disease where the bile ducts in the liver get constricted (partially or fully squeezed closed). No one knows what causes the disease, and it is not related to alcohol consumption. The disease slowly gets worse and eventually causes cirrhosis (hardening) of the liver. Although the symptoms can be treated for as much as 10 years, the only way to get rid of it is to undergo a liver transplant. Scott lived with the disease for more than 4 years, and his condition slowly got worse. He had several trips to the emergency room and was no longer able to function normally. He was working from home more and more often so he could rest. It was becoming more difficult to do the things he loved, like work in his workshop in the basement, play with kids, do maintenance on the house, or go camping with friends. In July of 2006, he was still able to go water skiing – something he has loved to do since he was about 6 years old. But he only skied for 5 minutes and it took him 4 days to recover.
Scott’s cousin Marcus volunteered to donate part of his liver to Scott during a living donor liver transplant, which occurred on September 5, 2006 at Johns Hopkins Hospital in Baltimore. To make matters more interesting, the BBC contacted us through Johns Hopkins. They were doing a medical documentary and wanted a section on living donor liver transplant – and thought we would be good candidates. For just under 2 months, we were followed around by our “British film crew”. Both guys seemed to be doing great after the surgery. Marcus was sent home one week after the surgery and was able to return to work part time about 1 month after the surgery. Scott went home in “near record breaking time” as well after only 2 1/2 weeks. The only complication was that Scott’s liver was leaking bile after the surgery, and a tube was inserted into his liver and out through his side to a bag that drained away the bile so the leak could heal. One reason for performing the transplant so “early” in the course of the disease was so that Scott could better recover after this major surgery – described by one surgeon as “the most complex surgery we know how to do”.
But, 3 days after Scott got home, he started to run a fever and we went back to Hopkins. As it turns out, a blood vessel in Scott’s liver had been nicked during the placement of the tube. The segment of the liver that was supplied by that vessel was not getting “perfused” well (inadequate blood supply) and was becoming infected. To make matters worse, because this was a living donor transplant, the bile duct that connects the liver to the large intestines had stayed with the donor – and Scott’s half of the liver had been tied directly to the large intestines. Every time they “messed” with the bile drain tube, they dragged nasty bacteria from the intestines (where it is ok to be) into his liver – where it would get infected, and the infection would spread to his blood stream. After two trips to the Intensive Care Unit because the infection had gotten so bad that it was causing his heart to race and his blood pressure to drop, the doctors decided that the segment that was not being perfused needed to be removed. So, exactly 6 weeks after the transplant, Scott had a second surgery to remove part of his new liver. Then there was an abscess (pocket of infection) in his liver – and an infection of the fluid in his abdomen. Scott had to stay on very powerful antibiotics for a long time until they finally removed the bile drain in early December. Unfortunately, the antibiotics caused damage to his hearing, and due to an inability eat and digest food properly, lost a lot of muscle weight and became extremely weak.
In December he finally went home and got to stay home for a while and celebrate the holidays with his family. However, there were signs that the liver was not functioning as it should. Scott had fluid build up in his abdomen called ascites. This is caused when the blood in the vein that supplies the liver can not go into the liver at a fast enough rate and backs up (imagine a partial dam on a river). The fluid has no where to go and the fluid separates from the red blood cells and leaches out through the veins into the abdomen. When the ascites gets really bad, as it frequently does, it affects his stomach capacity, breathing, balance, and abdominal muscles. Scott was also either losing red blood cells – or simply not making enough to keep up with the normal deterioration of the cells. So he had to be transfused with red blood upon occasion.
In early January 2007, the doctors decided that there was a constriction of the vein leading to the liver, and opening up the constriction might help reduce the ascites. They used a process similar to the angioplasty used to open up arteries in the heart. But the procedure was less than a resounding success. Not only was the blood flow improvement less than hoped for, but Scott started running a fever that night – they had disturbed a pocket of bacteria that was lying dormant in his liver – and spread it through is blood stream. To make matters worse, this particular strain of bacteria was very resistant to almost all antibiotics – except the one that damage Scott’s hearing even more.
Twice Scott was sent home on IV antibiotics, with the doctors believing they had the infection under control, and twice he spiked a fever and had to go back to the hospital. He now had not only another abscess of infection in his liver that they tried to drain unsuccessfully, but a new area that was “not healthy” because it was also not getting an adequate blood supply due to a clot. But this one could not be simply removed, because there would not be enough liver left for Scott to survive.
We had a few dark days where it was believed we had run out of options and antibiotics that would work. But the doctors had “one more ace up their sleeve”. They would hopefully get the infection under control (but not get rid of it) then put him on the transplant list for new liver. The antibiotics that they had to use are known to damage Scott’s hearing, but this was the very last antibiotic available to try, so if it didn’t work we were really finished. Fortunately, the antibiotic worked well, and Scott was put on the waiting list for a new liver – this time from a cadaver (instead of living donor) so that it could be full size, and contain the duct that leads from the liver to the large intestines.
As of end of March 2006, Scott was home on the IV antibiotics and waiting for a new liver, and hoping to get it before there is other permanent damage, or the bacteria becomes resistant to the antibiotics. If he became infected again – he could not get a new liver. They will not put a good liver into an infected body. Scott has become completely deaf and even hearing aids will not help anymore. Although he was a candidate for a Cochlear Implant, it was not an option at this time due to his fragile condition. So we learned new ways to communicate and adapt.
In early June, Scott was successfully weaned off the antibiotics, his PICC line was removed, and his liver had improved enough that it was decided that he did not need a liver transplant at that time. He might still need another transplant someday – but nobody knew if the liver would continue to improve or get worse again someday. So we had a few weeks when we thought we starting to see the light at the end of the tunnel.
But by mid-June, Scott’s hip started hurting. And we realized the light at the end of the tunnel was another oncoming train. We went to several doctors trying to figure out what was wrong. First we determined it was not a hernia. Then we went to a orthopedic surgeon who said it was probably just tendonitis or something – “but we need to do an MRI to rule out the slim chance of condition called Avascular Necrosis, which can be caused by the steroids used for anti-rejection”. Sure enough, it was the Necrosis – and it was in both hips. Avascular Necrosis is when the blood supply to the joints in the hips are interrupted, and the bones in the hips literally die. Then they deteriorate, “collapse”, become very arthritic, and extremely painful. Most of the time full hip replacement is the only option to be able to continue to be mobile.
In early September, one year after the initial transplant, Scott underwent a procedure to try to save his hips called a Bone Decompression, where they drill holes into the ball of the hip to try to relieve some of the pain and possibly save some of the hip bone so he will not need a full hip replacement someday. But it would take a least six months to find out if helped and the chances were less than 50%.
Just as his hips were healing up, and looked like maybe a positive outcome, the gods peed on Scott once more. At the end of September, 2007, on his way home from work, Scott was involved in a traffic accident, which landed him in the Emergency Room. Traffic stopped quickly, but the driver behind him didn’t, and the car became the meat in a three-car sandwich (Scott was the cheese). Fortunately, his neck, skull, and back all checked out fine, but he had a large lump on his forehead, and three cuts where he hit the steering wheel. This should all heal up with time, and it seems like the car can be repaired.
So now the question becomes even more appropriate: is Scott unlucky that all this has happened to him, or lucky because he has survived it all?
In November of 2007, Scott’s bilirubin count started to go up. ( Bilirubin is normally removed by the liver and this is what gives liver patients the typical “jaundice” or yellow appearance in their skin and eyes.) Various tests were run to see if there was anything that needed to be – or could be fixed. No real cause was found. As of December it was believed that all the infections have cause enough damage to the liver that was starting to not work as well – and it would probably not get better.
At the beginning of 2008, Scott’s liver function continued to deteriorate, but he must be “number 1″ on the list in the Maryland area in order to get a liver. But in Maryland, the ratio of folks waiting for liver to number of livers that become available is fairly high, especially compared to less densely populated areas such as the south. It was noted that someone as sick as Scott could get a liver if he moved to somewhere like Memphis, Tennessee, but in Maryland, he must wait until he sicker. But we had faith in the surgeons at Hopkins and the idea of moving to another state and leaving our children behind was not something we wanted to do. So we decided to wait until Scott got sicker and became number one on the list.
That happened in March. Scott became very sick. He was extremely jaundiced (yellow skin) , his kidneys were doing worse, and his clotting factor (an indicator that his liver is in it’s finally stages) was up (example – he got frequent nose bleeds and it took a lot to get them to stop.) Martha took off work so that she could be near him if we got “The Call”, since he could not hear the phone ring. But it still did not come. When he seemed at the sickest he had been, the surgeons called, the main surgeon at Johns Hopkins who had been with Scott since the beginning had been put on “Administrative Leave” – and none of the other surgeons could or would operate on Scott if a liver became available, since he was considered “high risk” (second transplant as well as high risk of infection). We would have to go somewhere else – out of the area. (If this part of the story does not make a lot of sense to you – welcome to the club.) Scott’s hepatologist had worked with several of the hepatologists in Memphis, Tennessee – and could vouch for the surgeons as well.
So we packed a bunch of clothes, kissed our daughters goodbye (they stayed with Grandma), drove/raced for two days down to Memphis to the Methodist University Hospital, which specializes in liver, kidney, and pancreas transplants. We got there on a Wednesday afternoon, had BBQ that night, and he was admitted for more testing on Thursday. His numbers had gotten worse yet, and he was deteriorating rapidly. On Saturday, they decided to put him on the Tennessee liver transplant list – and he was instantly on the top of the list. On Monday, March 31, 2008 a liver became available and the surgery was performed that night.
Scott spent the next week and a half in the hospital, with only a small infection at the site of the incision. But when he was released, we were not allowed to leave the Memphis area, so the Methodist Foundation paid for a hotel for us to stay in until it was safe for him to leave. They even helped pay for some of our food. Even more important, through the donation of some frequent flier miles, they flew our daughters in for short visit! At the beginning of May we were allowed to leave the Memphis area and drove slowly back to Maryland, returned to our own home on May 5.
Over the next few months the liver continued to improve. Although, his kidneys seem to have recovered and the numbers look normal, they have been damaged and there is roughly a 50% chance he will need a kidney transplant someday. The liver enzymes and bilirubin got much better, but as of the end of 2008, they had not returned to “normal”. And his spleen has never shrunk down to normal sizes either and he continues to be anemic. Scott has gone back to working full time, but mostly from home due to his low energy level.
In November 2008, he finally got a Cochlear Implant to improve his communication. Three days before Christmas it was activated and by Christmas he was able to hold limited conversations. As of the writing of this summary, his communications skills are continuing to improve and that has put a very happy ending to 2008.
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At this point we would like to add a simple plea that you consider organ donation, and be sure that your driver’s license and family agree with your decision. We certainly hope that none of you suffer a premature demise, but if such an unfortunate event were to occur then at least someone in need (usually many people) can benefit, and some good can come from the tragedy. Only those who are clinically brain dead but their heart is still beating can be used to harvest the major organs such as hearts, lungs, livers, etc. Less than half of those who have suffered a brain death but they are still on life support do their families agree to donate their organs. It is a tough decision, one I would not wish on my worst enemy, and the hardest part is accepting that your loved one is truly gone although their heart is still beating, but remember some good will come from your tragedy and your loved one will live on through the folks their organs save.
We would also add, if you can, to please donate blood through the Red Cross. For many reasons, there is frequently a shortage of blood and it is much need by folks such as Scott. Thank you.
