Cystic fibrosis (CF) is a heritable disease that leads to frequent lung infections and progressively interferes with a person’s ability to breathe. The chronic disease is caused by a defect in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which leads to the overproduction of thick, sticky mucus that accumulates in organs such as the lungs or pancreas. In the lungs, the mucus clogs the airways and promotes the growth of bacteria.

CF patients experience many respiratory system symptoms, including persistent coughing, at times with phlegm; frequent lung infections such as pneumonia or bronchitis, wheezing, and shortness of breath.

Although there is currently no cure for CF, there are treatments designed to ease the symptoms and increase life expectancy. However, many CF patients need a lung transplant.

What is a lung transplant?

A lung transplant is a surgical procedure where unhealthy lungs are replaced with healthy ones from a donor. The procedure is complex, and its risks and benefits must be assessed carefully for each patient.

For most patients, a lung transplant is able to extend and improve patients’ quality of life, but it is not the first option since it is a difficult and demanding procedure that involves an extensive evaluation process and a commitment to a new lifestyle that is essential to keep the new lungs healthy.

Before the lung transplant

The process of lung transplantation starts with a medical evaluation. Each patient is assigned a lung allocation score (LAS), which reflects the patient’s age, body mass index (BMI), and medical test results. A higher LAS score represents a more urgent need for a lung transplant.

If recommended for a lung transplant, patients must decide whether they wish to be placed on the transplant waiting list. If so, the next step is to wait for compatible donor lungs to become available.

There are always more patients who need lungs than there are donor lungs available. When a donor dies, the lungs are offered to the patient with the most urgent need.

When a compatible organ donor dies, the patient is notified and evaluated by a team including a transplant surgeon, a transplant pulmonologist, transplant nurses, a social worker, a psychologist or psychiatrist, a dietitian, and an anesthesiologist. During the process, the team requests interviews, a medical history, physical exam and diagnostic tests, while it performs a psychological and social evaluation, blood tests, diagnostic tests, and other preparations such as asking the patient to stop smoking.

The procedure

The lung transplant is performed under general anesthesia. The surgeon first makes an incision in the chest. The patient may be put on a cardiopulmonary bypass machine that sends blood and oxygen to the body during the procedure while the heart and lungs are not available. Then, the lungs are removed and replaced, while the blood vessels and airways are reattached. The surgeon closes the incision with stitches or surgical staples. A bandage or dressing is put on the incision site, and small tubes are put into the chest to remove air, fluid, and blood and allow the new lungs to fully expand. The surgical procedure may take between six and 12 hours.

Recovery and life after a lung transplant

After a lung transplant, patients stay in the hospital’s intensive care unit (ICU) for seven to 14 days on average. Physicians will monitor the heart rhythms, blood pressure, breathing rate, and oxygen level, and patients will have a breathing tube in the throat connected to a ventilator (a machine that inflates the lungs, assisting with breathing) until they are breathing comfortably on their own. Some patients need to be on a ventilator for several days; others are off it a few hours after surgery.

Patients will have to stay on immune suppressant medications for the rest of their lives to prevent the immune system from attacking the transplanted tissue.

Patients will no longer have CF in their lungs, but they will still have CF in their other organs, so it is important to continue taking CF medication.

The survival rate for CF patients following a lung transplant is good, with more than 80 percent of patients being alive one year after transplant surgery. According to a study in Canadian CF patients, 67 percent of patients were still alive nine years after a lung transplant.

Despite the encouraging results of lung transplants as a treatment for patients with CF, there are risks associated with the procedure. The main risks include bleeding, infection, blockage of the blood vessels to the new lungs, blockage of the airways, and fluid in the lungs. However, the most common and severe complication is rejection of the new lungs, which is why patients should watch for symptoms such as fever, body aches, tiredness, shortness of breath, cough, or new chest pain.

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Cystic Fibrosis News Today is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.