Cystic Fibrosis - Treatment

There is no cure for cystic fibrosis, but there are many types of treatment that can help patients live longer and more comfortable lives. Early diagnosis is an important element of treatment. With proper management, many people with CF engage in the full range of normal activities.


People with CF often do not receive full benefit from the foods they eat. They may need a high calorie diet with vitamin supplements. In addition, pancreatic enzyme pills are often recommended. The pills provide enzymes to the patient's digestive system that would normally come from a healthy pancreas.

Some people cannot absorb enough nutrients from foods even with special diets and enzyme pills. For these people, tube feeding is an option. A tube is inserted through the nose or through a hole made in the abdominal wall. Food can be supplied through the tube at any time of the day or night, allowing constant intake of high-quality nutrients. The feeding tube may be removed during the day, allowing the patient to eat meals normally.

Respiratory Health

The key to survival for many patients with CF is careful monitoring of the respiratory system. Lung function tests are done frequently. These tests measure the amount of air the person's lungs are able to take in. Sputum (spit) tests are done to find out what kinds of bacteria are present in the lung and how many there are. Chest X rays monitor any changes in the size and shape of the lungs.

An important element of good respiratory health is exercise. Exercise keeps the lungs healthy, active, and free of bacteria.

Clearing mucus from the lungs also helps protect against infection. Physical therapists have developed a number of techniques to help CF patients prevent mucus from collecting in the lungs. For example, a patient may lie on his or her stomach on a tilted surface with the head downward. An assistant then thumps on the patient's rib cage to help loosen mucus.

Drugs also can help maintain healthy lungs. For example, bronchodilators (pronounced brong-ko-die-LATE-urs) are substances that cause small air passages in the lungs to expand, allowing air to flow more freely. A group of drugs known as mucolytics (pronounced myu-kuh-LIH-tiks) also may be used. Mucolytics help loosen mucus and allow it to be expelled from the lungs more easily.

The use of antibiotics also may be necessary. Some doctors have their patients take antibiotics continuously to protect against lung infections. Other doctors use antibiotics only when an infection has actually developed.

In extreme cases, patients with CF may need oxygen therapy. Oxygen therapy is a procedure in which patients breathe air that is rich in oxygen. The higher oxygen content makes it easier for the body to get the oxygen it needs without working too hard.

Lung transplantation is a treatment of last resort. However, this form of treatment has its own set of problems. The drugs used during and after a

transplant operation cause severe problems of their own. One such problem is the weakening of the immune system.

On a long-term basis, the best hope for treating CF may be genetic therapy. Genetic therapy is a procedure by which correct copies of the CFTR gene are injected into a patient. The hope is that the correct genes will take over the task that the defective CFTR genes are unable to perform. Although much research is being done in this area, gene therapy is still in the experimental phase.

Alternative Treatment

Alternative practitioners recommend many of the same treatments used in traditional medicine. For example, they suggest the use of mucolytics to thin mucus and pancreatic enzymes to aid in digestion.

Efforts are also made to improve the patient's overall health and immune system. A variety of herbs is available to achieve these goals. In addition, hydrotherapy (water therapy) techniques may be helpful in relaxing the body and helping the patient breathe better.

It may be possible to cure cystic fibrosis with gene therapy. The problem is to find ways to get good copies of the CFTR gene into a patient's body. Scientists are now working on techniques for accomplishing this goal.

One approach is to make good copies of the CFTR gene in the laboratory. These copies are then combined with viruses. Viruses are very good at infecting cells. This property is usually not beneficial for humans because it is the reason people become ill with viral diseases.

The combination of CFTR gene and virus is made into a nasal spray. Some fat droplets are also added to the spray. The fat serves as a carrier that takes the virus and gene to cells in the patient's body. If all works well, the virus enters cells in the patient's body, bringing the good copy of the CFTR gene with it. The gene begins to function properly, and the patient is cured.

Trials of the new nasal spray have been going on since the mid-1990s. The first step in the trials is to make sure that the spray does not harm people. The second step is to find out if the spray really works. That is, does it actually deliver good genes to the patient's body?

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