Donate Life

My sister is only one of the 114,070 Americans currently waiting for an organ. Like so many of the people on waiting lists, Shaina had no control over the diseases that have caused her to need a transplant. She was only 19 when she received her first liver. As of last month, there were 1,801 children waiting for an organ. Like my sister when she was first listed, many of those waiting have their entire lives ahead of them.

I know first hand that life after the transplant is what these patients' families dream about as they sit at home or in the hospital waiting and watching their loved one get sicker and sicker. The only hope you have as you watch your sister, mother, uncle, or child suffer is that you will soon get that call from the transplant team saying that they have found the perfect organ.

But that call doesn't come in time for every person in need. There are simply not enough donors to go around; every day 18 people die waiting for an organ. Shaina has watched friends die from diseases like hers. And while we feel incredibly lucky that she has been approved for a 4th transplant, we know that there's still a chance it won't happen if they can't find the right liver for her.

My beautiful sister, Shaina.

We often talk about the other organs Shaina has received. Though we don't know who they are, the gratitude and love that we feel for the donors and their families is overwhelming. What a selfless act these strangers made that allowed my sister to keep fighting. There is no way we could ever repay them or thank them enough. Without their sacrifice after death, my sister would not be living. Shaina lives each day with the knowledge that a piece of that donor lives on inside of her.

I know that becoming an organ donor is not something that most people think of as they go about their daily lives. But it is something that my family and I think about every day. And I hope that whenever I die, I can help as many people as possible through organ donation. To give back for the gift that those donors before gave to me: to talk to my sister on the phone after a hard day, to feel jealous when she gets a new pair of hot heels, to see her smile at my dad's stupid jokes. All of this, every second of the past 10 years with my sister would not be possible without the selfless generosity of organ donors.

April is Donate Life Month. If you have not taken the time to sign up as an organ donor on an official registry, I hope you will take a few minutes to do so today. This page offers information about becoming a donor in each of the 50 states. Each of us has the capacity to give people like Shaina the chance to live a full life. It's one of the greatest things you can do for someone else.

The battle is upon us

One day last week, while Shaina was in the hospital, we had a talk about her status on the transplant list. The mood was somber.

Unfortunately, because of all the recent complications and the fact that her liver is no longer processing her medications like it should, her liver is far more damaged than her labs reflect. Because her labs are not high, her MELD score is not high, meaning she is very low on the transplant waiting list. If we wait for her labs to raise her to a higher position on the list, she will be too sick to undergo the actual transplant surgery. Believing that Shaina is the strongest now that she will be, her doctors want to get her transplanted as soon as possible, while she has the best fighting chance to survive the surgery and the inevitable complications that will follow.

The plan is to clear Shaina’s body of the infections she is currently fighting, and then immediately begin the search for a new liver. This means the transplant will take place much sooner than anyone anticipated. As her family, it will be difficult to put her in an extremely dangerous situation when she doesn’t seem as sick as she has been before previous transplants, but we are confident in her doctors’ decision that now is the time to move forward.

Shaina feels very fortunate that she has been given the chance to receive a fourth liver transplant. Most doctors would not attempt such a technically and clinically complicated surgery. But with the best doctors in the country behind her, and with her strong will to survive, Shaina is ready to fight this last fight.

Shaina is out of the hospital now, taking her antibiotics and fighting the infection from home. We should know in the next week or so if the infection is gone. I will continue updating this blog to keep everyone informed of her status. Shaina is helping me write many of the posts, and she encourages you to comment here and ask any questions that you may have. It’s going to be a tough fight, the toughest one yet, and Shaina needs everyone’s love and support to make it through the days ahead.

Diabetes?

For two days leading up to her admittance to the hospital, while Shaina was still quite foggy from a mental standpoint, she started craving sugar. Anyone who knows Shaina knows that she has quite a Dr. Pepper addiction. But it was way beyond that. She no longer wanted any food or drinks other than really sweet stuff – fruit, soda, sweet sports drinks. And she couldn’t get enough; she was gulping the liquids down faster than we could bring them to her. We gave her what she wanted, because we didn’t want her to get dehydrated. I asked her liver doctor if the cravings could be associated with encephalopathy, but he was not aware of any such correlation.

The night after she was admitted, her labs came back showing a blood glucose level of 1,031. Normal levels range from 70 – 145. When her nurse came into the room, she was visibly concerned - they had never seen glucose levels that high before on the transplant floor. They tested her blood sugar with a handheld monitor, hoping that the lab result was incorrect. But the meter, which only registers levels up to 500, read 500+.  The nurse immediately called the doctor on-call, who ordered an insulin drip and electrolytes, and her sugar was checked every 30 minutes throughout the night. The kidney doctor arrived the next morning, surprised that we weren’t in the ICU. He said he had never seen a patient with glucose levels that high who wasn’t in a coma.

The kidney doctors ran a test to see if Shaina’s sugars had been high over the last 3 months. It turned out they had. First they thought that she might have another autoimmune disease affecting her pancreas, but the next day they found that her body was producing some insulin on it’s own. So they have diagnosed her with Type 2 Diabetes and put her on long-acting and short-acting insulin shots. She has to check her blood sugar four times a day and change her diet. Her sugars seem to be stabilizing. We are hoping that the sugars were affected by the high Prograf levels, the infections, and the other imbalances in her body and that they will go back to normal once everything settles down.  

At least she has her mind

Shaina has always been the smartest of the three of us. Don’t get me wrong, David and I are bright, but Shaina has always had a very sharp mind. Anything she reads or sees on TV, she remembers. She is an encyclopedia of cooking and cultural factoids. She has always been an amazing writer. With anything she writes – cards, stories, but especially her poetry - it is obvious that she has the gift of the pen. Despite everything that her body has put her through physically, she always said, “at least I have my mind.”

There was only one time through all of this that Shaina really lost her mind. It was after her last transplant, and her Prograf level got way too high in the hospital. The Prograf toxicity brought on extreme psychosis. She spent 3 days in the ICU; she couldn’t talk, she couldn’t eat, she was tied to a bed and she was blank in the eyes. Shaina wasn’t there. She was like a helpless infant. The doctors warned us that there may be permanent damage, but thankfully, she snapped out of it on the night of her birthday. And just like that, Shaina was back, dirty mouth and all.

I thought about those couple of days when my mom called me earlier this month. I had just returned to New York after a visit to Dallas for Dad’s birthday. We had taken a car trip to see some fossilized dinosaur tracks in a riverbed, except we didn’t see any tracks, we just saw a river… long story… just imagine Chevy Chase in the National Lampoon Vacation movies, and you’ll get the gist. Anyway, everyone piled into Dad’s rented SUV excited for a family outing that didn’t involve doctors. Shaina seemed to have a really great time; we even brought Sato along. The next day before I was scheduled to fly home, Shaina was not able to swallow any food. She was having severe pain in her esophagus. Dad took me to the airport, Mom took Shaina to the hospital. They sedated and scoped her.

After the procedure, I called mom to see how everything went. She said Shaina was having a tough time waking up from all the drugs. The next day, Shaina still hadn’t “woken up” completely. She was in a fog. She couldn’t really talk, she only responded when you got right in her face and raised your voice, but even then she was confused and having a hard time opening her eyes. As the week went on, she got worse. She was incredibly thirsty and in and out of a coma-like state. Mom and Dad didn’t know what to do. She required 24-hour care. Mom was bringing her food three times a day, usually cream of rice, forcing her to take her pills, and helping her to the bathroom. When the doctors saw her, they said she was suffering from hepatic encephalopathy. One of your liver's jobs is to remove toxins, like ammonia, from your blood. Too much ammonia causes damage to the brain and nervous system. Shaina's doctors immediately put her on Lactulose and Rifaximin to help reduce the ammonia in her body.

Shaina seemed to get better for a couple of days, but the next week, it happened all over again. Dad called me around dinnertime on Friday and asked me if I would be willing to come back to Dallas to help with Shaina. My dad rarely calls me directly (I call their house at least once a day and talk to everyone), and he has never asked me to come home; he always offers to bring me down if I feel like a visit. This time, Dad asked me if I could fly down the next day to help, and I knew it was serious. I jumped a plane the next day and arrived to find Shaina totally out of it and Mom at her wits end. Shaina was back in a coma-like state. She had lost the one thing that was most important to her - her mind. To this day, she doesn't remember a whole week of her life.

The night I arrived, I told Mom to get some sleep and I would keep an eye on Shaina. Shaina was somewhat awake, but confused, and she was suffering from these violent involuntary jerks that made it difficult to sit up, let alone walk. (We later learned this was a severe case of asterixis, or “liver flap,” commonly associated with encephalopathy.)  I came upstairs to check on Shaina during dinner and saw her sitting on the floor near the door to her room. She had fallen down while trying to turn off her light. I was really worried, but she begged me not to tell Mom. So I didn’t. But I made her promise that she would call me when she wanted to get out of bed and I stayed in her room until she seemed to fall asleep.

Shaina's broken and bruised foot

Later that night, I was asleep across the hall from Shaina with both doors open so that she could call for me if she needed me throughout the night. I heard a large crash and Shaina cry. I ran into her room to find that she had suffered a jerk and fallen trying to walk to the bathroom. I checked her head and her body but didn’t see any blood. She was crying as I helped her back into bed, saying that she thought she had broken her foot. She said she had heard a snap when her toes curled under her foot and she stepped down on them. I looked at her foot, but didn’t see any bones protruding, so I thought she must have hit it hard on the door frame or somewhere during her fall and perhaps sprained her ankle. But when I went back to check on her an hour later, her foot was swelling up and there was an obvious hematoma spreading across the top from her ankles to her toes; her whole foot was turning red and blue and was terribly swollen. It was 2:45am. I asked her if she needed to go to the hospital right then, or if she could wait until the morning when we had an appointment with her doctor. She wasn’t able to respond clearly, but she seemed somewhat stable and I knew my mom could use a full night’s sleep. I decided to put ice on it and make her elevate her leg until my mom was up at 4am. I camped out on the floor in Shaina’s room until I heard Mom downstairs in the kitchen. By time she saw Shaina’s foot, it was even more bruised and swollen.  We got her dressed, wrapped her ankle in an Ace bandage, and made our way downtown.

As soon as Shaina’s liver doctor saw her, he said she needed to be admitted to the hospital. Though she was much more lucid that morning, he was concerned about her color, her recent confusion, and the fact that she fell in the middle of the night. Shaina was admitted for observation of hepatic encephalopathy. And a possible broken foot. When we were admitted, Shaina tried to sign and date her paperwork, like she always does. I sat beside her and watched my sister, one of the most eloquent writers I know, struggle to remember how to sign her own name. When I told her the date, she couldn’t remember how to write the number 2. It was hard to watch, heartbreaking actually. And it was even harder for her. She was totally frustrated, but lord knows she wouldn’t let me help. I took it as a good sign that she at least still had her strong will. It’s that determination that has gotten her this far, after all.

Shaina gets a cast

The first x-ray of her foot did not show any fractures. They wrapped her foot in a soft splint and told her not to put any weight on it, but it was killing her. A day or two later, however, they did a CT scan, and found a Lisfranc fracture in the bones on top of her foot. This was a break that was usually fixed with surgery, according to the orthopedic surgeon, but he did not want to perform surgery on Shaina because she was a high risk for infection and because he didn’t want to expose her to unnecessary sedation that could worsen her encephalopathy. Instead, they fitted Shaina for a cast that would have to stay on for 6 – 8 weeks. She is not allowed to walk or put any pressure on her foot.

While in the hospital, they also discovered that her Prograf level was way too high, which could be contributing to her encephalopathy. And she has several infections, which they are now treating with antibiotics. All of this in addition to getting her blood sugar under control (more on that in the next post) and lowering her pain medicines seem to have improved her mental state. She has had brief bouts of fogginess, but for the time being, she appears to have her mind back.

Getting up to speed

My sister Shaina is 28 years old. She was diagnosed with several chronic diseases at age 12, the primary diseases being Autoimmune Hepatitis and Primary Sclerosing Cholangitis, both of which affect her liver.

Our entire childhood was spent in Charlotte, North Carolina, most of which was happy and healthy. We spent family vacations in beautiful places like Maui, Laguna Niguel, Japan, and our grandparent's condo in Myrtle Beach. Shaina always said that she was going to be a Hollywood actress when she grew up. She attended an arts magnet school in middle school where she studied drama and dance. She said she would have a lot of babies, because she loved kids, and they loved her.

But as high school dragged on, Shaina got sicker. Eventually a tutor had to come to the house to help her graduate high school. She missed the graduation ceremony, but Mom had a party anyway and made her wear her cap and gown for pictures with the family in the living room. By this time, I was a college sophomore living in Raleigh, NC. I was able to come home many weekends and whenever Shaina was admitted to the hospital. The stress on our family was overwhelming, but we had no idea what the next several years would entail. We thought she would get a transplant and be the happy, healthy Shaina of her childhood.

By her senior year, enough damage had been caused to Shaina's liver that she needed a transplant. My parents searched for the best liver transplant program in the country. Soon after Shaina's graduation, they sold the dream home that they had built two years before and moved with my sister, brother, and two dogs to Dallas, Texas to be closer to Baylor University Medical Center. Shaina received her first two liver transplants at age 19. The first liver never took, and Shaina spent a horrific six weeks in the hospital, undergoing dozens of surgeries that came along with the complications of an unsuccessful transplant. After a bumpy start, the second liver worked like a dream. It lasted for three wonderful years until there was a thrombosis that cut off the main blood supply to her liver. This resulted in infection that landed Shaina in the hospital for the next 5 months, while she waited for a new liver. It was a trying time, to say the least.

Shaina in the hospital

Shaina's third transplant took place on November 6, 2005. She has had several major complications along the way, and half of her liver was lost due to thrombosis of the right portal vein when her blood thinners were mistakenly held for a procedure. But in traditional Shaina spirit, she kept trucking along, fighting through infections and complications, attempting to live a somewhat normal life.

Several months ago, Shaina was diagnosed with a severe enterovirus that, because of the immunosuppression she was on to keep her autoimmune disease under control, spread to every part of her body: skin, blood, brain, organs, etc. With no precedence for treating an enterovirus this severe, her infectious diseases doctor decided to try massive and extended doses of IVIG therapy, along with a healthy dose of crossed fingers. During part of this time, when the virus was at its worst, Shaina was hospitalized. The doctor who heads the liver transplant program came to see Shaina when I was there with my brother, David (a rare moment when we had convinced my mom to leave the hospital). He told Shaina that this virus would kill her. The only hope for getting rid of the virus was to lower Shaina's immunosuppression while trying the IVIG therapy. This was a problem because if they lowered Shaina's immunosuppression, her autoimmune disease would kill her liver. But this was her only shot. They would lower Shaina's immunosuppression, give her IVIG, and when the virus was gone, re-list her for a fourth transplant to get her a new liver.  Fourth transplants are rare, anywhere in the world. And we did not know if the IVIG would actually work on the virus. But this was the only way to save Shaina. So it was done. And the IVIG seemed to work. After almost a year, her cultures came back negative, meaning the virus was gone. The head of the program took Shaina's case to the transplant ethics board and she was approved for a fourth transplant. But then, the enterovirus came back. Thankfully, the infectious diseases doctor caught it early and, after another round of IVIG, the cultures came back negative again and it hasn't returned. Which brings us to the last couple of weeks...