Treatment Options for Cystic Fibrosis

Cystic fibrosis was once considered a death sentence. Babies who were diagnosed with cystic fibrosis rarely lived beyond a few years. Modern medical treatment options and continuing research have changed that, though. While there is still no cure for cystic fibrosis, many people with the disease can enjoy life well into their 30s and 40s.

Treatment of cystic fibrosis focuses on clearing the respiratory airways, preventing lung damage by treating infections, and providing proper nutrition. Below are some of the currently recommended treatment options for cystic fibrosis.

Treatment Options for Cystic Fibrosis: Chest Physical Therapy, Breathing Treatments and Exercise
The major issue with cystic fibrosis is the congestion of the lungs and airways with thick and sticky mucus. Survival of the patient requires removing it from the airways to allow free breathing and prevent lung damage from infections.

One way to do this is through Chest Physical Therapy (CPT), also known as postural drainage. CPT involves a family member or friend pounding their cupped hand over the patient's chest and back for several minutes at a time. This is referred to as percussion. The family member or friend will also use a flat hand to 'vibrate' the same areas. The percussion and vibrating dislodges mucus built up inside the lungs. During the percussion and vibrating, the patient will sit in various positions designed to drain the mucus from one airway to another, working it into a position where it can eventually be coughed up and out.

For people who do not have regular access to someone who can help them with CPT, or who find it uncomfortable, mechanical devices are available to simulate the hand movements. These include a mechanical percussor (also known as a chest clapper) and an inflatable vest that uses high-frequency airwaves to vibrate the mucus trapped inside the lungs.

Breathing devices are also used to clear the airways. The Oscillating Positive Expiratory Pressure device (Flutter device) vibrates the airways, breaking up the mucus. The patient breathes through the device a few times, and then coughs to expel the mucus. In Positive Expiratory Pressure (PEP) Therapy, the patient wears a mask that is attached to a resistor set. They breathe in normally, getting air down into their lungs (and behind the mucus), and then breathe out with a bit of extra force. This helps to dislodge and move the mucus.

Aerobic exercise is encouraged as a way of removing mucus from the respiratory airways. Exercise makes a person breathe harder, which helps loosen the mucus, as well as strengthening their lungs. Children with cystic fibrosis should be encouraged to run and play like healthy children. They can even participate in sports, either through their school or with friends and family. Adults should likewise keep an active lifestyle, making exercise a normal part of their daily activities. Patients who exercise on a regular basis often need less chest physical therapy than non-active cystic fibrosis patients.

Treatment Options for Cystic Fibrosis: Inhaled Medications
As with most medical conditions, medications are frequently prescribed when treating cystic fibrosis. Many of these medications are administered in mist form, using an inhaler or nebulizer, to get them directly into the airways where they are needed most.

Common inhaled medications used in cystic fibrosis treatment include:

Albuterol, or other bronchodilators - Albuterol (most commonly known for treating asthma attacks) relaxes the muscles in the airways and opens them wider, making it easier for thickened mucus to move through them.

Pulmozyme - The thick mucus common in cystic fibrosis contains high concentrations of DNA. The medication Pulmozyme breaks down the DNA, which thins the mucus enough for it to be coughed out of the lungs.

Hypertonic Saline - Hypertonic saline solutions contain high amounts of salt (7% salt, as compared to 0.9% in normal saline solutions). The mucus in cystic fibrosis becomes thick because it contains less salt than normal. Using a high salt saline replaces that salt, which in turn draws water to the secretions. This causes the mucus to thin, making it easier to cough it out of the airways.

Treatment Options for Cystic Fibrosis: Antibiotics
Bacterial lung infections are very common in people with cystic fibrosis, which means antibiotics are just as common to them. Depending on the type of bacteria involved and the severity of the infection, antibiotics may be inhaled, injected, given through an IV, or administered orally.

Recent research shows the antibiotic azithromycin (Zithromax) offers additional benefits to cystic fibrosis patients beyond fighting infections. During a clinical trial conducted over a six month period, patients who took azithromycin (as compared to patients given a placebo) experienced a 6% improvement in lung function, weight gain, and spent 47% fewer days in the hospital for lung infections.

Treatment Options for Cystic Fibrosis: Nutritional Planning and Tube Feeding
Cystic fibrosis patients generally face malnutrition. This is due to the thickened mucus blocking the flow of pancreatic digestive enzymes, which are needed to break down proteins and fats and absorb nutrients from the food. Children experience low weight and poor growth, while adults struggle with maintaining a healthy weight and illnesses such as diabetes and osteoporosis.

Good nutritional planning is an important part of the cystic fibrosis treatment. People with cystic fibrosis require extra calories to maintain or increase their weight. They are generally deficient in fat-soluble vitamins (vitamins A, D, E and K) and need to find ways to increase their intake of important minerals (calcium, iron, zinc). As cystic fibrosis causes large amounts of salt to be lost while sweating, healthy forms of salty foods need to be eaten on a regular basis, especially during hot and humid weather. In addition, the lack of pancreatic digestive enzymes means some foods will not break down in the intestines as easily as others, leading to potential bowel problems. Considering all these special dietary concerns, it is important for cystic fibrosis patients to work with a dietician who specializes in the disease. Their doctor, area children's hospital, or local Cystic Fibrosis Foundation chapter should be able to recommend someone for nutritional counseling.

Despite the use of a well-planned diet, there may be times when tube-feeding is required to ensure proper nutrition and caloric intake. There are two options for tube-feeding: Nasogastric tubes and Gastrostomy tubes. A nasogatric tube is a long, thin tube that is inserted into the nose until the tip enters the stomach. This is generally inserted at night and removed in the morning. A gastrostomy tube is inserted directly into the stomach through the abdomen, where it remains. Both types of tubes are used to deliver specially designed liquids that contain high levels of calories and nutrients.

Treatment Options for Cystic Fibrosis: Antioxidant Therapy
Recent research shows antioxidants can slow cystic fibrosis. Medical researchers suggest cystic fibrosis patents increase their antioxidant intake through diet, especially by adding fruits and vegetables, and supplementation.

Some doctors will administer N-Acetylcysteine (brand name Acetadote) to help the body produce glutathione. Glutathione is a naturally occurring antioxidant that supports the immune system and protects the lungs from damage. Studies show cystic fibrosis patients have lower levels for glutathione than people without cystic fibrosis.

Sources:
Cystic Fibrosis Foundation:
What You Should Know
Therapies for Cystic Fibrosis

Mayo Clinic:
Cystic Fibrosis: Treatments and Drugs
Cystic Fibrosis: Lifestyle and Home Remedies

MedicineNet:
How is Cystic Fibrosis Treated?

National Heart Lung and Blood Institute:
How is Cystic Fibrosis Treated?