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Propionic Acidemia (Liver Transplant)

Our daughter Kaitlin was born in 1999 with Propionic Acidemia. For 13 years she had numerous hospitalizations, fed 100% through a g-tube and used a wheel chair if we needed to be out and about. We had a few really good “healthy” years where Kaitlin did really well and was getting stronger and things were looking up. In 2010 she started heading downhill yet again. We couldn’t keep her ammonia levels down and she became increasingly lethargic. Her lethargy became so bad that she had to use her wheel chair almost all of the time. Then in 2011 we approached our metabolic team about what we could do for Kaitlin as her quality of life was declining. This wasn’t the lively little girl that we knew and it was heart breaking. We discussed medication and treatment options and one of those options was a liver transplant. My husband and I had a big decision on our hands. We set up an appointment to meet with the transplant team at Seattle Children’s Hospital. The initial consultation was incredible. The transplant doctor was so at ease discussing Propionic Acidemia and his knowledge of the disorder was incredibly reassuring.

As most of us know finding anyone in the medical field who had knowledge of metabolic disorders and can discuss it without glancing at a sheet of paper with facts about the disorder is nearly impossible! This man spoke to us like he knew what it was like to have a child with PA. We were “sold.” We went back a few weeks later for the full workup for the transplant. It consisted of lots of labs, ultrasound, and interviews with the docs, social work and the list goes on. At the end of the second day of tests the coordinator said she would call us and let us know if we were approved by the transplant team for Kaitlin to have her transplant. It was a nerve wracking wait and I can’t remember now how many days it took but the day we got the phone call that she was a candidate was a happy and scary day all wrapped into one. We signed all the necessary paperwork to have her put on the list and we got the call she was listed for a liver in October of 2011. That year we celebrated Halloween, Thanksgiving, Christmas and her 13th Birthday waiting for the BIG call. On January 11th 2012 we got the call to bring her in that a liver was on its way for her. Of course my husband was out of town, I was in an employee review meeting and both my kids were in school….So I did what any good mom does…Have a full blown panic attack! Between hyperventilating and crying I had to have my employee call my business partner to come pick me up so that I could get Kaitlin from school and to the hospital. My husband was now on his way home from his trip and the whirl wind of pre-transplant began. Labs, ultra sounds, and lots of waiting to hear if the donor liver is viable. Early the next morning they came into the room and told us it was time. The liver was a perfect size and match and they were ready to get started. I will tell you that Kaitlin has had lots of surgeries and hospitalization but this was by far the hardest time letting her go back with that team leaving her in their hands.

Fast forward 12 hours…Surgery went incredibly well with no complications. She was in ICU starting to wake up and we were about to experience having a new child all over again. She was taken off formula that day and was given just Pedia Sure. All her metabolic meds were stopped over the next week and the LOADS of transplant meds were started. After transplant the amount of medications is overwhelming! A few months prior to transplant Kaitlin had to start a thyroid medication and we taught her to take pill by mouth. I am glad that I did that! After her surgery she had so many pills to take it she did it like a champ. There was one medication that is an oral liquid that has to be swished in the mouth and swallowed four times a day to help prevent yeast infections. This was the hardest med to get her to take! She did however learn to do it and after a month would just suck it down.

Kaitlin had a couple rounds of rejection which isn’t uncommon after transplant. Since March of 2012 all of Kaitlin’s liver labs and metabolic labs have been normal. Going into transplant her ammonia was three times normal and post-transplant it was in the single digits! We had never seen that in her 13 years. For the last year Kaitlin has been formula free as well as g-tube free. She eats 100% of her food by mouth now. Some of her favorites are noodles, rice, shrimp, steak, and chocolate cupcakes.

For the last year we have gotten to know a whole new kid. Before transplant she was always kind of dazed and in a fog. Now she is witty and funny and talks all the time. She can tell us about her day in great detail which is so exciting. She has learned to ride a bike and loves to “race” us running. Her endurance is what has been the biggest change. Before transplant she could barely walk from the couch to her room without being tired. We had to push her in her chair if we left the house. In December of 2012 after transplant Kaitlin was granted her Wish from Make a Wish and we went to Disney World. She walked everywhere, stood in lines and then ran when she would see a princess. Just seeing her be able to have the endurance and stamina of a typical kid is one of the largest blessings we could have asked for.

I get the question all the time if it “cured” her propionic academia…She will always have PA but no longer shows any symptoms of the disorder. We traded one set of issues for another. We still have to be very diligent about medications, fluid intake, calorie intake and labs. But the stress of PA is no longer there for us. We feel as if a large weight was lifted from our family and the quality of life for all of us has changed tremendously. We live life to the fullest and there isn’t a day that goes by that we do not send a prayer to the family who had to have a significant loss for us to have a gain. We are eternally grate full to the donor family, the surgeons, the medical staff, family and friends who have supported and guided us through the last year.

byMichelle, Trent, Kaitlin and Maddy – Seattle, Washington | jacqulinemichelle@hotmail.com

From the Spring 2013 OAA Newsletter

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