Cystic fibrosis is a disorder that causes severe lung damage and digestive problems that could result in malnutrition. The disease is inherited when both parents carry a recessive mutant gene. A child cannot inherit cystic fibrosis if only one parent carries the mutant gene. There is no known cure for this disease. Life span for cystic fibrosis patients have increased by nearly 25 years since the disease was first diagnosed.
Cystic fibrosis affects the cells that produce sweat and mucus. Both of these are important to the body. Sweat helps cool our body, and mucus keeps our essential organs lubricated so they can work properly. Mucus is thin and watery in healthy patients but in those with cystic fibrosis the mucus is thick, discolored, and sticky. Mucus plugs essential passageways to the respiratory, digestive, and reproductive system allowing infection to pile up. The treatments for cystic fibrosis patients are aimed at preventing complications, relieving symptoms, and improving the quality of life.
Specific signs and symptoms will vary from person to person. The basic problem for every patient is the thick mucus; symptoms can vary from patient to patient. Symptoms will vary in the degree of the severity for each person. One child may have respiratory problems that suggest they have cystic fibrosis while another may have severe digestive disorders. Some cystic fibrosis patients may not be diagnosed until they have a delay in the onset of puberty. Occasionally a patient may be a young adult before they are diagnosed but this is rare.
A child with cystic fibrosis may be malnourished and their growth could be slowed. If the disease affects their digestive tract, foods are not digest properly and essential nutrients do not get sent to other areas of the body. An infant may be diagnosed with cystic fibrosis early in life if they suffer from an intestinal blockage. This happens when meconium, which is normally passed out of the body a day or two after birth, instead backs up and blocks the intestines. Meconium is a substance that is naturally expelled through the bowels and shows as greenish black stools.
Other signs in an infant or young child is failure to grow, thick and greasy stools, frequent respiratory infections, bloating and excessive gas. A child may taste “salty” when they are kissed. Patients with cystic fibrosis have a higher than normal amount of salt in their sweat. Older children who have blocked bowels, greasy and smelly stools should also be tested for cystic fibrosis. Wheezing and coughing that brings up heavy colored mucus is another sign of cystic fibrosis. A child may also have part of the rectum protrude from the rectum caused by stools that are too thick to pass or frequent coughing.
An unusual but common sign of cystic fibrosis is enlargement of the fingertips and toes. Known as clubbing, it is a symptom for CF but can also occur in people with heart problems and other lung diseases. If your child shows any of these signs, call your doctor and have them examined and tested.