• More than 30,000 people in the U.S. currently have CF
• Approximately 1,000 new cases are diagnosed each year in the U.S.
• About 70,000 CF cases worldwide

• Most CF patients are 18 years or older
• About 75% of patients were diagnosed by the time they were 2 years old

The biggest risk factor of CF is the genetic mutation that patients may inherit.
CF is most common in people with Northern European ancestry

Incidence:

• 25% of children in the U.S. are diagnosed with CF (1 in 4 children)
• 50% of children in the U.S. are carriers of the CF gene
• 25% of children in the U.S. will not carry the CF gene

Having Cystic Fibrosis increases the risk for lung infections, constant coughing, damaged airways and even respiratory failure.
When CF also affects the mucus in the digestive tract, there is an increased risk for malnutrition; making it very difficult for the patient to reach a healthy weight. The reason for this malnutrition risk, is that when the mucus coats certain enzymes in the digestive tract, they are unable to absorb nutrients. When the patient consumes food, their body is not absorbing any of its nutrients.

In most cases, CF affects the lungs and bronchial tubes the most. Typically, mucus dehydration in the airways initiates the disease.
While CF has a major effect on the lungs, the thick mucus that is produced by this disease can also be found in the digestive system, sweat glands, and reproductive tract. This can lead to malnutrition and poor growth in most pediatric patients because the mucus in their digestive tract is so thick, that the enzymes located there become trapped and cannot absorb nutrients. Many pediatric patients will present with very slow growth even they may be eating an adequate amount of food.

Cystic Fibrosis is caused by a mutation in the Cystic Fibrosis Transmembrane Conductance Regulator gene (Cftr). The role of Cftr proteins is to control the ions that pass through the cell membrane. Typically, when a Cftr protein becomes irregularly folded, it does not allow the plasma membrane to properly bring Chloride to the cell; which therefore does not bring enough water into the cell. This results in cells that produce mucus that is too thick due to the abnormal amounts of sodium and chloride at the cell membrane.

Medications:

• Medications for treating gene mutations
• Antibiotics for lung infections such as Penicillins
• Anti-Inflammatory drugs for the lung airways: Prednisone or Hydrocortisone
• Hypertonic Saline: thins mucous and helps the patient cough up mucous more easily
• Bronchodilators such as Albuterol
• Stool softeners for patients with constipation
• Pancreatic enzymes to help absorb nutrients
• Antacids which improve pancreatic enzymes
• Diabetic and liver disease drugs if needed

Techniques for Clearing the Airways:

• Clapping hands on the back of the patient to loosen mucous
• Breathing exercises
• Vibrating vest to loosen mucous
• Exercise can help the lungs work up extra mucous
• Oxygen Therapy if needed
• Feeding tube for those with nutrient deficiencies as a result of a build up of mucous in the digestive tract
• Lung transplant in severe cases
• Liver transplant in severe cases