Cystic Fibrosis

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Diagnosis

To diagnose cystic fibrosis, doctors usually perform blood tests. They may test sweat for high salt content, which can indicate cystic fibrosis. If the diagnosis is confirmed, doctors may evaluate the condition with a chest x-ray, chest or abdominal CT or MRI, abdominal ultrasound, or sinus CT.

Treatment

There is no cure for cystic fibrosis, but treatment can ease symptoms, reduce complications and improve quality of life. Close monitoring and early, aggressive intervention is recommended to slow the progression of CF, which can lead to a longer life.

Managing cystic fibrosis is complex, so consider getting treatment at a center with a multispecialty team of doctors and medical professionals trained in CF to evaluate and treat your condition.

The goals of treatment include:

Preventing and controlling infections that occur in the lungs

Removing and loosening mucus from the lungs

Treating and preventing intestinal blockage

Providing adequate nutrition

Medications

Options include:

Medications that target gene mutations, including a new medication that combines three drugs to treat the most common genetic mutation causing CF and is considered a major achievement in treatment

Antibiotics to treat and prevent lung infections

Anti-inflammatory medications to lessen swelling in the airways in your lungs

Mucus-thinning drugs, such as hypertonic saline, to help you cough up the mucus, which can improve lung function

Inhaled medications called bronchodilators that can help keep your airways open by relaxing the muscles around your bronchial tubes

Oral pancreatic enzymes to help your digestive tract absorb nutrients

Stool softeners to prevent constipation or bowel obstruction

Acid-reducing medications to help pancreatic enzymes work better

Specific drugs for diabetes or liver disease, when appropriate