Cystic Fibrosis
How is it treated?
Currently, there is no cure for cystic fibrosis (CF). However, technological advancements in medicine allow many individuals with CF to live into adulthood. However, CF patients who do live longer are at a higher risk of developing complications of the disease such as lung infections and bowel blockage. It is important to note, however, that the severity, number, and type of symptoms can vary widely from patient to patient. Additionally, because CF affects many different systems of the body, a wide variety of techniques and medications are used to treat the condition and its complications. While the treatment of CF depends upon the severity, stage, and progression of the disease; general goals are:
- to ensure adequate calorie and nutrient consumption
- to increase airflow in the lungs
- to decrease the volume and thickness of lung mucus
- to prevent/treat infection in the lungs
There are currently 115 Cystic Fibrosis Foundation (CFF) treatment centers scattered throughout the United States. These unique treatment centers specialize in diagnosing and treating cystic fibrosis. For more information regarding CFF treatment centers, you can visit the following link: http://www.cff.org.
Nonpharmacologic (non-drug) therapies
Nutrition/Exercise
CF patients often do not absorb enough calories, protein, and vitamins from their usual daily diet to maintain adequate nutrition. As a result, individuals with CF are advised to increase their daily intake of calories to 120%-150% of the recommended daily allowance (RDA). CF patients should also increase the amount of protein in their usual daily diet by 200% of the RDA of protein. Nutritional supplements, such as Ensure, Sustical, and Scandishakes, may help CF patients maintain the amount of nutrients they require each day.
CF patients should exercise as tolerated to help loosen mucus build-up so that it may be removed more easily. Exercise also helps to expand the lungs and increase lung capacity (amount of air the lungs are able to hold). However, exercise may not be appropriate for all CF patients. It is important for patients to talk with their doctor before beginning to exercise.
Chest physiotherapy/postural drainage/percussion/chest vest
Because CF can cause patients to produce abnormally thick and sticky mucus, a variety of methods are available to help remove the secretions. Postural drainage is an alternative process used to help clear mucus from the lungs by altering the patient's position. Classic techniques, such as chest percussion (tapping on the chest and back) and the chest vest (a vibrating vest) have also been used to help dislodge and remove excessive mucus in the lungs of CF patients. In addition, innovative breathing devices, such as oscillatory positive expiratory pressure (OPEP) devices (such as the Acapella and the Flutter) can be used to loosen mucus. By exhaling into the OPEP device, vibrations are created which help move the abnormal secretions from the lungs.
Oxygen & Transplant
In severe cases of CF, the patient's damaged lung function could become life-threatening necessitating the need for an oxygen machine. If the physician determines that the patient is a good candidate for transplantation, he/she will be put on a lung transplant waiting list. It should be noted, however, that a lung transplant will not cure CF. Patients with the following conditions should not be considered for lung transplantation:
- Cancer
- HIV infection
- Major psychiatric illness
- Severe damage to other body organs
- Severe overweight
Pharmacological (drug) treatment
Digestive Enzymes
In patients with CF, defects of a gene known as CFTR alter the function of a protein that regulates ion transport, which is the normal movement of sodium, chloride, and water in the body. This alteration causes secretions to be abnormally heavy, thick, and sticky. Normally in healthy people, the pancreas is a gland that produces digestive enzymes (proteins that help break down food such as amylase, protease, and lipase) which are excreted through small channels into the small intestine when food is present. However, the abnormal mucus in CF patients clogs these channels in the pancreas, resulting in an insufficient amount of digestive enzymes in the stomach. As a result, food is not digested correctly and intestinal problems such as diarrhea and bowel blockage occur. Because CF patients usually do not have enough digestive enzymes in their intestines to break down the food they eat, they must take supplemental oral digestive enzymes. Ordinarily, digestive enzymes are taken with every meal or snack in often large doses.
Bronchodilators
Maintaining adequate airflow is important to CF patients because a reduction in the ability to breathe can often cause a great deal of anxiety as well as hypoxemic (lack of oxygen) complications. If CF patients do not obtain enough oxygen, the organs (such as the brain) of the patient are at great risk for obtaining damage. To combat this potential problem inhaled bronchodilators have been widely used to help maintain adequate airflow and lessen the breathing difficulties that CF patients experience.
Inhaled/oral corticosteroids and ibuprofen
As a result of the numerous lung infections that CF patients experience, chronic inflammation of the lung tissue can occur. If the lung tissue remains inflamed for a great deal of time (days-months), damage can occur, thus resulting in the loss of lung function. In order to decrease the amount of lung inflammation, drugs such as corticosteroids (natural hormones normally made by the adrenal gland) can be prescribed. By decreasing the frequency and severity of lung inflammation, corticosteroids (such as prednisone) may prevent future lung problems. Non-steroidal anti-inflammatory drugs such as ibuprofen are another group of drugs that may help decrease the severity of symptoms in CF patients by decreasing inflammation. Because of potentially severe side effects, it is only appropriate to take ibuprofen exactly as your physician prescribes.
Mucolytic or Mucus-thinning drugs
Since CF causes patients to produce abnormally thick and sticky mucus, drugs that thin the mucus can be given to patients. Thin mucus is easier to be cleared from the lungs of individuals with CF, thus allowing them to breathe easier. For example, a drug called DNase (also known as recombinant human deoxyribonuclease or dornase alfa) works to thin mucus by breaking apart the genetic material (DNA) contained in the abnormally thick secretions. This action decreases the viscosity (thickness) of the secretions, therefore allowing the mucus to be easily cleared.
Antibiotics
Lung infections, in general, are difficult to control and cure. In CF patients, however, the challenge to treat lung infections increases. The bacteria that cause these lung infections are able to multiply and flourish in the presence of the sticky and thick secretions found in individuals with CF. Mucus can also prevent antibiotics from reaching the infection site, consequently making it more difficult to treat. In light of this complicated situation, new inhaled and oral (tablets and capsules) antibiotics have been developed to help control recurrent lung infections in CF patients.
Please click on the following links to learn more about the various drug treatments for CF.
Drug classes used to treat Cystic Fibrosis Aminoglycosides Carbapenems Cephalosporins Digestive Enzymes Inhaled Beta-2 Agonists Monobactams Mukolytics Penicillins Potassium-sparing Diuretics Quinolones Vancomycin
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How is it treated?
