Cystic Fibrosis Program

The Cystic Fibrosis Program at Cleveland Clinic Abu Dhabi specializes in the treatment of cystic fibrosis, a genetically-inherited disorder that mainly affects the lungs, but can also affect other organs. The Cystic Fibrosis Program, which offers treatment for adults, is managed and overseen by a team of expert pulmonologists as well as a multidisciplinary group of specialized nurses, respiratory therapists, and dietitians.

As cystic fibrosis involves multiple organ systems, the program closely collaborates with physicians and other clinical staff from both the Surgical Subspecialties Institute and the Medical Subspecialties Institute - specifically, from the otolaryngology (ENT), endocrinology, hepatology and rheumatology specialties.

What We Treat

Cystic fibrosis is a genetic disorder that mainly affects the lungs, but can also affect the pancreas, liver, kidneys, and intestines. Common symptoms include:

• Difficulty breathing
• Frequent coughing
• Chest and sinus infections
• Production of sticky mucus
• Salty-tasting skin.

The Cystic Fibrosis Program at Cleveland Clinic Abu Dhabi treats adults with mild to severe cases of cystic fibrosis, as well as both pulmonary (lung) and non-pulmonary manifestations of the disorder.

The average life expectancy of patients with cystic fibrosis is generally less than 50 years old in the developed world. While there is no cure for cystic fibrosis, once diagnosed, the disorder can be managed through treatment to improve life expectancy and quality of life.

Diagnosis of Cystic Fibrosis

In this initial consultation, the pulmonologist will perform a detailed review of the patient’s history, a physical examination, and a review of previous test results. Diagnostic testing would be ordered, such as genetic testing and/or a sweat chloride analysis.

Once the diagnosis is established, further testing can be done to determine the extent of the disorder and treatment required. These tests include:

• Pulmonary function tests: A range of tests that measure how well the patient is able to breathe in and out, and how efficiently the lungs provide oxygen to the blood.
• CT scan or x-ray: These tests provide detailed images of the lungs and inside of the chest, and are particularly helpful in ruling out other conditions with similar symptoms.
• Sputum microbiology: An analysis of mucus the patient has coughed up.

As cystic fibrosis is a multisystemic disorder, further evaluation may be necessary based on the patient’s symptoms and test results.

Treatment of Cystic Fibrosis

Treatment of cystic fibrosis requires complex multidisciplinary care. Patients should expect to come in for regular follow-up visits at the pulmonary clinic, along with regular respiratory therapy and lung function measurements. Depending on the severity of the disorder, many patients may also require oral or inhaled treatments.

Cleveland Clinic Abu Dhabi provides all state-of-the-art pharmacological treatments for cystic fibrosis. These include the latest gene modifying tablets, Ivacaftor and Ivacaftor/Lumacaftor, which may be useful for selected patients, as well as inhaled treatments, including mucolytics and antibiotics.

For patients with very severe lung disorders, the program also offers evaluation for a possible lung transplant, in collaboration with Cleveland Clinic in the United States and Cleveland Clinic Abu Dhabi’s Lung Transplant Link service.