Introduction

This is a blog about our journey through the transplant evaluation process. I am Lisa and am married to Gessner. Gessner has cystic fibrosis and just started the transplant evaluation process. This blog is to keep our family and friends updated about the process and also to educate others about cystic fibrosis and transplant. Here's a little about us:

Gessner was diagnosed with cystic fibrosis when he was three months old. He has one older sister who does not have CF, but she is a carrier of the gene that causes CF. Cystic Fibrosis is a genetic disease caused by mutations of the CTFR gene. The result of these mutations is that the body produces a thick, sticky mucus that clogs the lungs and other organs (particularly the pancreas). Beginning at an early age, Gess started to take pancreatic enzymes to help his body absorb food. He also did daily treatments that include chest physical therapy (CPT) to break up the mucus. As a teenager, Gess developed portal hypertension as the result of mucus build-up in his liver ducts, result in cirrhosis of the liver and an enlarged spleen. He also developed CF-related diabetes (CFRD) and is insulin-dependent.

Gess has frequent lung infections that often require IV antibiotics to fight the bacteria pseudomonas aeruginosa, which he chronically cultures in his lungs. The liver disease also has the effect of suppressing his immune system. His enlarged spleen causes his blood clotting factors to be low as well. He has also experienced frequent episodes of bleeding from the lungs (hempotysis) and has had multiple bronchial artery embolizations (BAEs) to stop the bleeding.

The hempotysis can be very dangerous and the multiple BAEs have damaged his lungs. This, along with lost lung function due to the progression of the disease have lead to the decision to consider transplant. Gess's situation is particularly difficult because of his liver disease. By traditional transplant evaluation measures his lungs are not "sick" enough for him to receive a transplant. Additionally, his liver is not sick enough for transplant. But, because of the difficulties that his case presents, his CF doctor at the University of Washington Medical Center decided to present his case to the lung transplant committee. Their initial decision was to refer Gess for a lung transplant evaluation and to consider a staged approach to the transplant process. Under that approach, the lung transplant would happen first and then after he recovered from that, he could get listed and have the liver transplant. We honestly have concerns about this approach and will be talking to the team about it. The lung team will also consult with the liver transplant team and get their input.

According to the United Network for Organ Sharing (UNOS), only 37 dual liver/lung transplants have been done in the United States. So we are in pretty uncharted territory. We are doing all the research that we can on our end and will be actively involved with the team during the process.

In the meantime, Gess continues to work as the Offshore Initiatives Project Manager for an online travel company.

Gess met Lisa while in college and they have been married for 11 years. They have a cute, 9-year old beagle named Beauty.