health

There is no cure for CF. Treatment is aimed at thinning the mucus secretions,
keeping the patient’s airways clear, preventing infections, and
maintaining adequate nutrition.

Medications used to treat CF include:

• Mucolytics. These are drugs that thin the mucus in the lungs so
  that it is easier for the patient to cough it up. Pulmozyme is the
  most commonly used drug of this type.
  
• Antibiotics. These are given to treat bacterial infections in the
  lungs and throat. Some antibiotics can be given in aerosolized
  form and delivered directly into the airway.
  
• Bronchodilators. Bronchodilators are drugs that help to keep the
  bronchi (tubes leading into the lungs) open to ease the patient’s
  breathing. They can be taken through inhalers or nebulizers.
  
• Oral enzymes and vitamin supplements. Patients with CF need to
  take pancreatic enzymes in the form of pills or capsules with their
  meals in order to absorb nutrients from their food. Extra vitamins
  are also needed to prevent malnutrition.
  
• Pain relievers. Some children with CF are helped by taking
  ibuprofen (Advil or Motrin) for headaches. This particular
  non-aspirin pain reliever also may help slow down loss of lung
  function.

Adults as well as children with CF usually need to follow a special
high-calorie high-fat diet to maintain good health. A professional dietitian
can work with the patient to include foods that the patient enjoys
eating as well as meeting the calorie and fat requirements.
Patients with CF also need to clear the mucus from their lungs at
least twice a day. This clearing can be done by having the patient lie with
his or her head over the edge of a bed and thumping or clapping on the
back and chest for twenty to thirty minutes to shake the mucus loose so
that the patient can cough it up. As an alternative to having a physical
therapist or trained family member thumping the patient’s chest, the
patient can wear a device called a ThAIRapy Vest, which contains a compressor
that vibrates the patient’s chest wall at set times to loosen the
mucus. The vest costs about $16,000.
CF patients with advanced lung disease may benefit from lung
transplantation.