Centers of Excellence

The Lung Center

Adult Cystic Fibrosis (CF)

Cystic fibrosis (CF) is a life-threatening condition which causes mucus buildup in the body, severely damaging the respiratory, digestive and reproductive systems. Cystic fibrosis, most often diagnosed during childhood, is caused by an inherited gene mutation. Although cystic fibrosis cannot be cured, many CF patients are living longer, healthier lives thanks to progress in research and care.

The Adult Cystic Fibrosis Program at Brigham and Women's Hospital (BWH) Lung Center is a collaborative program between BWH and Boston Children's Hospital. Working together, specialists from these two medical centers provide comprehensive, continuous care for cystic fibrosis patients from childhood to adulthood.

Learn more about cystic fibrosis.

Adult Cystic Fibrosis Topics

Causes of Cystic Fibrosis

Cystic fibrosis is a genetic (or inherited) disease. A baby will be born with CF if two CF genes are inherited—one from the mother and one from the father.

Risk Factors for Cystic Fibrosis

The birth of a child with CF is often a total surprise to a family, since most of the time there is no family history of CF. Some people carry the CF gene without being affected by the disease. They usually do not know that they are carriers.

If there is a family history of CF, testing is recommended for anyone who has a family member with the disease, or whose partner is a known carrier of CF or affected with CF.

Learn more about the causes and risk factors for cystic fibrosis.

Symptoms of Cystic Fibrosis

All newborns are tested for CF, which means that parents will know if their baby has the disease and can take precautions and watch for early signs of problems. Infants born with CF usually show symptoms by age two. Some children, though, may not show symptoms until later in life.

The following are the most common symptoms of cystic fibrosis.

  • Abnormalities in the glands that produce sweat and mucus. This may cause a loss of salt, which can cause an upset in the balance of minerals in the blood. This can lead to abnormal heart rhythms, and, possibly, low blood pressure and shock.
  • Thick mucus that clogs certain organs, such as the lungs, pancreas and intestines. This may cause malnutrition, poor growth, frequent respiratory infections, breathing problems and chronic lung disease.

The following signs are suspicious of CF, and infants having these signs may be further tested for CF:

  • Diarrhea that does not go away
  • Foul-smelling stools
  • Greasy stools
  • Frequent wheezing
  • Frequent pneumonia or other lung infections
  • Persistent cough
  • Skin that tastes like salt
  • Poor growth despite having a good appetite

Read more about cystic fibrosis symptoms.

Diagnosis of Cystic Fibrosis

Cystic fibrosis is most often diagnosed during newborn screening. If the condition is not discovered at birth and is suspected in an older child, the physician will obtain a medical history and conduct a variety of tests—such as genetic, blood, sweat pulmonary function and stool tests—to diagnose cystic fibrosis.

Read more about the tests used to diagnose cystic fibrosis.

Treatment for Cystic Fibrosis

Although there is no cure for cystic fibrosis, expert medical care and psychosocial support can help the patient effectively cope and live a productive, active life.

Treatment aims to slow progress of the disease and lessen the severity of any symptoms. Treatment generally includes:

  • Management of respiratory problems with exercise, chest physical therapy, medications and antibiotics
  • Management of digestive problems with diet and supplements
  • Psychosocial support
  • For end-stage cystic fibrosis patients, lung transplantation may be an option

Learn more about treating and managing cystic fibrosis.

Research

The Adult Cystic Fibrosis Program includes researchers who are conducting studies on cystic fibrosis. The results of their work are translated directly into exceptional patient care.

What You Should Expect

The Adult Cystic Fibrosis Program brings together the full range of services and expertise of BWH and Boston Children's Hospital. The team develops a personalized care plan for each patient based on current research. Your health will be closely monitored and managed to promote optimal lung function and improved quality of life. You will also have access to cystic fibrosis community organizations.

Multidisciplinary Care

Patients benefit from the teamwork of physicians, social workers, physical therapists, nutritionists and dedicated nurses at BWH and Boston Children's Hospital. They collaborate closely to provide comprehensive care as the child with cystic fibrosis transitions to adulthood.

Resources

Visit our health library to learn more about cystic fibrosis.

Visit the Kessler Health Education Library in the Bretholtz Center for Patients and Families to access computers and knowledgeable staff.

Access a complete directory of patient and family services.

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