Cystic Fibrosis Medical Definitions

The following page defines terms commonly associated with cystic fibrosis.

Aerosol Medications

Aerosol medications are drugs delivered to the lungs in a fine mist, using a machine called a nebulizer. By means of an air compressor, the nebulizer sends air through a tube so the air can mix with the medication. The person holds the mouthpiece of the nebulizer in his/her mouth and breathes in the medication. Parents of infants can use a mask instead of a mouthpiece to deliver the aerosol medication to their baby's mouth and nose. Aerosol medications are used to open air passages, decrease inflammation, thin mucus and treat infections.

Apgar Score

Apgar score is a number between zero and 10 that is used to assess a newborn at one minute and five minutes after birth. The Apgar score represents the sum of scores between zero and two for each of five areas including the baby's color, heart rate, breathing, muscle tone and cry.

Chest Physical Therapy

Chest physical therapy is a way of moving mucus from the lung's small airways into the larger airways so that the mucus can be coughed out. With the child sitting or lying in special positions, parents use their hands to clap over the child's lungs. With the help of gravity, the clapping shakes mucus loose and coughing moves it out of the airways.

Delta F508

Delta F508 is the name of the most common mutation of the cystic fibrosis gene. A cystic fibrosis gene mutation is a change within the cystic fibrosis gene (cystic fibrosisTR) that prevents the gene from working correctly. Although more than 850 mutations of the cystic fibrosis gene have been identified, the delta F508 accounts for 70 percent of all cystic fibrosis gene mutations.

Inhaled Medications

Inhaled medications are drugs delivered to the lungs by a small handheld device called an inhaler or metered-dose inhaler. When pushed or turned, the inhaler delivers puffs of medication into the mouth to be breathed into the lungs. Parents of infants can attach a spacer device and mask to an inhaler to deliver the medication to their baby's mouth and nose. Inhaled medications are used to open air passages, decrease inflammation and treat infections.

Intravenous

Intravenous refers to a way of giving medication directly into one's vein. Usually a small plastic tube is inserted into the vein and taped in place so the medication can be administered on a regular basis.

Meconium Ileus

Meconium ileus is an intestinal blockage found during the first few days of life in newborns with cystic fibrosis. Meconium refers to the baby's first bowel movement. It is usually dark brown or black and sticky. In infants with cystic fibrosis, the meconium is thicker than normal and it can block a part of the small intestine called the ileum. Meconium ileus occurs in about 10 percent of newborns with cystic fibrosis. For these infants it is usually the first sign of cystic fibrosis. Physicians treat meconium ileus by giving the baby a special enema. Sometimes surgery is required to relieve the blockage in the intestines.

Pancreatic Insufficiency

Pancreatic insufficiency occurs when the pancreas produces little or no digestive enzymes. The three main digestive enzymes produced by the pancreas are trypsin, amylase and lipase. Trypsin digests proteins, such as those found in meats and dairy products. Amylase digests starches, also called complex carbohydrates, such as those found in potatoes, pasta and breads. Lipase digests fats, such as butter, cooking oils and lard.

Trypsinogen

Trypsinogen is an early form of the enzyme called trypsin produced by the pancreas. This enzyme normally passes into the intestines to aid digestion. However, elevated blood levels of trypsinogen have been found in infants who have cystic fibrosis. Therefore, measuring trypsinogen blood level is a good way to screen for cystic fibrosis.