Transplantation

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Key points

  • Despite improvement in median life expectancy and overall health, some children with cystic fibrosis (CF) progress to end-stage lung or liver disease and become candidates for transplant.

  • Lung and liver transplants have the potential to extend life and improve the quality of life, but require strict adherence to a complex medical regimen.

  • Chronic lung allograft dysfunction continues to be a major barrier to the success of lung transplants.

  • Determining the optimal time to move forward with a liver

Indications

General indications for LTx

Summary

Bilateral LTx and OLT offer survival benefits to patients with end-stage CF lung or liver disease if selected appropriately and timed well.88 It is not an easy path. The medical regimen is complex. The lifelong need to pay close attention to detail is intense and unrelenting. Although quality of life measures are heartening, outcomes as related to morbidity and mortality from complications remain poor and have not changed substantially in the last 20 years for LTx recipients.

It is hoped that,

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