Cystic fibrosis (CF) is a genetic and inherited disease that causes patients to suffer from constant lung infections and progressively interferes with their ability to breathe. The chronic disease is related to a defect in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which causes patients to overproduce thick and sticky mucus that accumulates in organs like the lungs or pancreas.  While in the lungs, the mucus clogs the airways and promotes the growth of bacteria. In the pancreas, it disables the release of digestive enzymes, which are needed for digestion.

Patients who suffer from cystic fibrosis experience symptoms like very salty-tasting skin; persistent coughing, at times with phlegm; frequent lung infections like pneumonia or bronchitis, wheezing or shortness of breath, poor growth or weight gain in spite of a good appetite, frequent greasy, bulky stools or difficulty with bowel movements, and male infertility. Despite the fact that there is currently no cure for cystic fibrosis, but there are treatments designed to ease the symptoms and increase life expectancy.

When Is a Lung Transplant for Cystic Fibrosis Patients

Cystic fibrosis affects different parts of the body, but the lungs are usually the focus of patients and physicians, since it makes it difficult for patients to properly breathe. Most commonly, patients are treated with CFTR modulator therapies, which are designed to correct the function of the defective protein, while inhaled medication like mucolytics and antibiotics are used to better reach the airways and fight infections. In addition, airway clearance techniques (ACTs) are needed by CF patients to loosen thick, sticky mucus and enable the clearance of the airways and lungs.

However, cystic fibrosis is a progressive disease, which means that the symptoms progress over time and the medication may become less effective. In some cases, lung transplant becomes the only option to improve patients’ quality of life. 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 process that involves an extensive evaluation process and a commitment to a new lifestyle that keeps the new lungs healthy.

Before the Lung Transplant

The process of lung transplantation starts with the evaluation for the procedure. As explained by the Cystic Fibrosis Foundation (CFF), “there are more people who need new lungs than there are available donor lungs. Therefore, when donor lungs become available, they are offered first to the people with the most urgent need for them. Need is represented by a number called the Lung Allocation Score (LAS). The LAS is a number, ranging from 0 to 100. It is the result of a calculation that takes into account a person’s age, body mass index (BMI) and certain medical test results. A higher LAS represents a more urgent need for a transplant.”

When an 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 psychiatrist or psychologist, a dietitian, a chaplain, and an anesthesiologist. During the process, the team requests interviews, medical history, physical exam and diagnostic tests, while it performs psychological and social evaluation, blood tests, diagnostic tests and other preparations like asking the patients to stop smoking. Before the procedure, the transplant team explains the procedure to the patient, and asks him or her to sign a consent form and not to eat or drink for 8 hours before the surgery. Right before the surgery, physicians may recommend a sedative to help patients relax.

Lung Transplant Procedure in Cystic Fibrosis Patients

In order to undergo a lung transplant, patients need to stay in a hospital for a period of recovery time. There are different types of lung transplants, according the patients’ needs. During a single lung transplant, only one lung is transplanted, whereas in a double lung transplant both lungs are transplanted either separately in a bilateral sequential procedure, or bilateral single procedure where both lungs are transplanted at the same time, while in a heart-lung transplant both lungs and the heart are transplanted. After being admitted at the hospital, patients will be put on an intravenous (IV) line in the arm or hand, and catheters in the neck, wrist, under the collarbone (subclavian), or the groin. Before the surgery, patients are put under general anesthesia not to feel any pain.

During the surgery, the surgeon makes an incision in the chest. Depending on the type of transplant, the patient may be put on a cardiopulmonary bypass machine that sends blood and oxygen to the body during the procedure. Then, the lungs are removed and replaced by the new ones, while the blood vessels and airways are reattached. After finishing the process, 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 in the chest to remove air, fluid, and blood from the chest and allow the new organ to fully expand. In addition, patients will awake after a few hours in the recovery room with an epidural catheter to receive pain medication.

Recovery and Life After a Lung Transplant for CF Patients

After a lung transplant, patients stay at the intensive care unit (ICU) for seven to 14 days on average. Physicians will monitor the heart rhythms, blood pressure, breathing rate, and oxygen level, while the patients will continue with a catheter in the bladder until they can urinate alone, and a breathing tube in the throat hooked up to a ventilator. Special IV medicine are used for the blood pressure, heart, and any bleeding problems, as well as pain medicine and anti-rejection (immunosuppression) medicines. In addition, patients will start physical therapy and breathing exercises with the help of nurses, respiratory therapists, and physical therapists.

“Lung transplantation is the ultimate therapy for end-stage cystic fibrosis (CF) lung disease; however, the debate continues as to whether lung transplantation improves survival. (…) Eighty transplants were performed in CF patients, 11 (13.8%) of whom were children. Mean age at transplant was 26.2 years (95% confidence interval: 24.4 to 28.0). The Liou raw score at transplant was -20 (95% confidence interval: -16 to -24), resulting in an estimated 5-year survival without transplantation of 33 +/- 14%, compared with a 5-year post-transplant survival of 68.2 +/- 5.6%,” state the authors of the study “True survival benefit of lung transplantation for cystic fibrosis patients: the Zurich experience.”

Despite the encouraging results of lung transplants as a treatment for patients with cystic fibrosis, 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, severe pulmonary edema or fluid in the lung, and blood clots. However, the most common and severe complication is rejection of the new lungs, which is why patients should be aware of symptoms like fever of 100.4°F (38°C) or higher, flu-like symptoms such as body aches, fluid in the lung, tiredness, shortness of breath, cough, and new chest pain.

Note: 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.