Cystic Fibrosis Patient Receives Rare Dual Lung, Liver Transplant at UT Southwestern Hospital

Cystic Fibrosis Patient Receives Rare Dual Lung, Liver Transplant at UT Southwestern Hospital

Josiah Ferrell, a 25-year-old patient with cystic fibrosis (CF), is successfully recovering from a dual lung and liver transplant. The rare procedure, performed by surgeons at the University of Texas (UT) Southwestern Medical Center’s William P. Clements Jr. University Hospital, has been performed only 74 times in the United States, and Josiah is the first patient to undergo the dual-organ transplant in North Texas.

Lung disease occurs in more than 95 percent of patients suffering from cystic fibrosis. On the other hand, liver disease affects only 25 percent of CF patients, while liver cirrhosis occurs only in 8 percent of the patients. Despite following medical recommendations and an appropriate lifestyle throughout his life, Josiah experienced progressive lung and liver failure, and his organs needed to be replaced.

A series of factors come to play in a surgery of this magnitude. Besides finding a matching donor for both organs, it is rare for a single patient to need these organs simultaneously and at the same time be healthy enough to endure the surgery and recovery process.

The procedure was performed by transplant surgeons Dr. Michael Wait, chief of the Cardiovascular and Thoracic Surgery Service at William P. Clements Jr. University Hospital, who performed the lung transplant, and Dr. Malcolm MacConmara, also trained in immunology, who performed the liver transplant. Overall, the transplant surgery lasted 12 hours and involved more than a dozen physicians and other team members, organized in two teams — one to remove and replace the lungs, and a second to remove and replace the liver.

The team of experts, in order to minimize the time between organ removal and transplant, decided to remove one lung, complete the transplant, and then perform the removal of the second lung and subsequent transplantation, a strategy that allowed the surgeons to avoid having the patient on a heart-lung bypass machine. The abdominal team was then able to perform the liver transplant, using a piggyback technique that leaves in place an important vein that returns blood from the abdominal organs to the heart, helping stabilize the patient.

“The lungs were implanted without much of the difficulty we had anticipated and planned for, and the transition from lung to liver transplant was seamless,” Wait in a news release.

wait-ferrell-macconmara
Dr. Michael Wait, Josiah Ferrell, and Dr. Malcolm MacConmara

“This surgery showcases the best of what we have at UT Southwestern. We communicated exceptionally well in the planning. It’s an example of a well-thought-out, well-executed plan between the cardiothoracic surgery team and the abdominal surgery team, as well as all the other professionals involved,” MacConmara said, also highlighting the extremely careful planning and coordination necessary for the procedure.

Josiah, now at home, has a long recovery process, as the first nine months are a period of intense follow-ups, with a series of examinations and medication adjustments. For the first time in two years he is now able to walk outside and breathe without the oxygen tank he has carried around 24 hours a day. The surgery has allowed him to plan exciting things for an until-now uncertain future.

“There are some things I’d like to study. I’m interested in studying computer programming or engineering,” Josiah said. “I’d like to visit Japan someday. I’ve been teaching myself Japanese online for several years. I originally got interested through manga [Japanese comics], but now I’m interested in Japanese culture generally. I’d like to see the cherry blossoms. The cherry trees bloom for one week.”

 

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