Cystic fibrosis (CF) is a disease of the lungs and digestive tract which causes thick mucus to build up and cause blockage. This disease is caused by a defective inherited gene for which an estimated 1 in 29 Caucasian Americans are carriers. Some of the most common symptoms are delayed growth, salty tasting skin, no bowel movement within the first 48 hours of life, clay colored stools, recurrent episodes of pneumonia, coughing, increased mucus in the lungs and upper airway, fatigue, nausea and weight loss.
Sweat chloride testing is used to test salt levels on the skin. If levels are elevated it could indicate cystic fibrosis if other symptoms are present. This is the most common test for CF. Other tests than can be used to identify cystic fibrosis include fecal fat test, chest x-ray, lung function tests, secretin stimulation test and upper GI or small bowel series.
Treatment for cystic fibrosis centers on treating existing symptoms and preventing worsening of disease. Some respiratory treatments include nebulizer treatments, enzyme replacement therapy to thin mucus, antibiotics, postural drainage and chest percussion therapy multiple times per day and oxygen therapy. Treatment for bowel issues include administration of pancreatic enzymes, high protein/high calorie diet, vitamin supplements and laxatives or stool softeners for hard stools.
Light exercise two to three times per week can help with lung function and clearing secretions. Parents and caregivers need to know their child’s limitations without discouraging them from achieving milestones for their age group. Although these children will require less exertion and more frequent rest times and respiratory treatments, they can generally match the developmental abilities of peers their age.
