Supporting Loved Ones

What Is Cystic Fibrosis?

Cystic fibrosis (CF) is one of the most common and serious genetic diseases in America. CF affects the respiratory (lungs), pancreatic, and gastrointestinal (GI) systems. It can also affect the sinuses, liver, spleen, and reproduction. It is a chronic disease that currently has no cure.

In the 1940's, CF was a fatal disease of early childhood. Today, thanks to advances in medical care, children can expect a much longer lifespan. Most children grow into adulthood. Many attend college, marry, and lead very productive lives. The median age of survival (half live longer, half die younger) is the late 30s.

Cystic fibrosis is present at birth, although it is not always found then. CF occurs because both parents carry a CF gene and each parent has given one to their child. It's important to remember that every child with CF has CF genes from both the mother and the father. The CF gene is common (one in 28 Caucasian Americans carries the gene) and marriages between carriers occur frequently (about one in every 400 marriages). Not every child from a family will have CF. Other children could receive a single CF gene from just one parent and become a CF carrier. Or, they could receive no CF gene and be free from CF.

How Does Cystic Fibrosis Affect The Child?

Glands in the body that usually make thin, slippery secretions (like sweat, mucous, tears, saliva, or digestive juices) make, instead, thick, sticky secretions. These thick, sticky secretions plug the ducts (small tubes) that carry the secretions either outside the body or into hollow organs like the lungs or intestines. This can affect vital body functions such as breathing or digestion.

In The Lungs?

People with CF have thick mucus, which can trap germs in the lungs. Thick mucus is hard to clear, so the germs stay and grow in the lungs. Once these germs make a home in the lungs, there are more frequent lung infections. Frequent infections start a cycle of inflammation and infection, which becomes a chronic problem. Most people with CF die from respiratory failure.

In The Gastrointestinal (GI) System?

The GI system is also often impaired in children with CF. Thick secretions block the pancreatic duct, so the enzymes used to break down food can't get to the small intestines. This is called malabsorption. It can lead to cramps; gas; bulky, foul-smelling stools; and poor growth.

Cystic Fibrosis Care

Most people with CF have to work hard to stay healthy. Their daily care and treatments can take a lot of time. The treatment of CF varies with most children; but many will need to clear the thick secretions from their lungs. This is called airway clearance. There are several types of lung clearance therapies. Chest physiotherapy (CPT) involves clapping on the child's back to loosen lung mucus so that the child can cough it out. The Vest™ is a machine that is worn which vibrates the mucus loose. Devices like the Flutter® and Acapella™ also provide airway clearance.

Children with CF take many medicines. They may be on bronchodilators, steroids, anti-inflammatory medicines, or antibiotics. They may need to take antibiotics by mouth, intravenously (IV), or as a breathing treatment to fight lung infections. It's hard for some children to gain weight, so they must take pancreatic enzyme supplements with meals and snacks to help their digestion. Vitamins may be prescribed.

Care for the healthy CF child can take up to 1-2 hours a day. This involves taking the medicines and doing airway clearance. The amount of care goes up if the child is sick. At times, a child with CF needs IV medicines. These can be given at home. At other times, a doctor will admit the child to the hospital for 1-2 weeks for IV antibiotics and intense airway clearance.