Lung transplant for cystic fibrosis

Surgery Overview

Lung transplant is an option for a few people who have severe lung problems that are caused by cystic fibrosis. The procedure removes the diseased lungs and replaces them with healthy lungs from a recently deceased donor.

Sometimes a procedure called a living-donor lobar lung transplant is performed while a person is waiting for a full lung transplant. In this procedure, the lower section (lobe) of one lung in each of two living donors (who may be related or unrelated to the person who has cystic fibrosis) is transplanted into the person who has cystic fibrosis. Because this complicated transplant involves two living donors, it is performed less often than other kinds of lung transplants.

What To Expect After Surgery

After lung transplant surgery, you will be put on a ventilator, which is a machine that temporarily takes over the breathing process. If there are no complications, you will use the ventilator for 1 to 2 days.

Generally, you will need to stay in the hospital for 2 to 3 weeks after the transplant.

Why It Is Done

Some people who have severe lung damage that was caused by cystic fibrosis may get a lung transplant. Experts aren't sure if a lung transplant helps a child with cystic fibrosis live longer.1 People who have mild or moderate disease do not usually get a transplant because the risks are greater than the benefits.

Several tests can help determine how well a lung transplant or a living-donor lobar lung transplant might work. These tests include lung function tests, arterial blood gas tests, and exercise capacity.

In order to be accepted for lung transplant, you must have an evaluation to find out how severe your lung disease is, whether you have any other diseases, and whether you are able to follow a complex pretransplant and follow-up regimen.

A person who is age 12 and older is assigned priority for transplant based on a Lung Allocation Score. The Lung Allocation Score uses the following measures:

  • Waitlist urgency: the expected number of days lived without a transplant during an additional year on the waitlist
  • Post-transplant survival: the expected number of days lived during the first year after getting a transplant
  • Transplant benefit: the post-transplant survival measure minus the waitlist urgency measure.

A child who is age 0 to 11 is assigned priority for a transplant based upon how long he or she has been waiting.

In addition to the criteria above, all lung transplant recipients are chosen based on blood type, tissue type, size of the organ, medical urgency, time already spent on the waiting list, and the distance between the donor and the recipient. More information can be found on the United Network for Organ Sharing (UNOS) Web site: www.unos.org.

How Well It Works

  • About half of the people who receive a lung transplant survive at least 5 years after the procedure.2
  • People with cystic fibrosis have the same survival rate after a lung transplant as people who have lung transplants for other health problems.
  • Lung transplant is used only for people who have very severe cystic fibrosis. For people who have mild or moderate cystic fibrosis, the risks of the procedure are greater than the benefits.

Risks

The main risks of lung transplant are problems during the surgery and rejection of the donated organs. Infection and rejection of transplanted organs have similar symptoms, including fever, tiredness, and trouble breathing.

After a lung transplant, preventive antibiotic therapy starts right away. Immunosuppressant medicines are also given, usually for the rest of your life. Although immunosuppressant medicines raise the chances that your body will accept the new lungs, they also make it harder for your body to fight off infection.

What To Think About

  • Lung transplant surgery does not prevent or improve any problems that cystic fibrosis may be causing in other parts of your body.
  • Lung transplant surgery is generally recommended only for people who have severe lung damage because the risks can be greater than the benefits
  • If you are a candidate for a lung transplant, you may have to wait 6 months to 4 years. The United Network for Organ Sharing (UNOS) regularly updates its guidelines for transplant selection. For more information, go to www.unos.org/policiesandbylaws/policies.asp?resources=true.
  • Having a lung transplant is still a limited option in the United States and is usually available only in certain major medical centers. You may have to travel or move to another city to have the operation.

Complete the surgery information form (PDF)(What is a PDF document?) to help you prepare for this surgery.

References

Citations

  1. Liou TG, et al. (2007). Lung transplantation and survival in children with cystic fibrosis. New England Journal of Medicine, 357(21): 2143–2152. [Erratum in New England Journal of Medicine, 359(e6).]
  2. Health Resources and Services Administration (2006). 2006 Annual Report of the U.S. Organ Procurement and Transplantation Network and the Scientific Registry of Transplant Recipients: Transplant Data 1996–2005. Available online: http://www.optn.org/data/annualReport.asp.

Last Updated: June 23, 2009

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