Cystic Fibrosis

Cystic fibrosis is an inherited disease of the mucus glands. It causes chronic, progressive damage to the respiratory system, chronic digestive system problems, and can affect other organs. The signs and symptoms of this disorder are caused by the production of abnormally thick, sticky mucus in the body's organs. Problems with breathing are among the most serious symptoms.

For more information about cystic fibrosis please visit the Cystic Fibrosis Foundation.

Cystic Fibrosis FAQs

What is cystic fibrosis?

Cystic fibrosis is an inherited disease of the mucus glands. It causes chronic, progressive damage to the respiratory system, chronic digestive system problems, and can affect other organs.

The signs and symptoms of this disorder are caused by the production of abnormally thick, sticky mucus in the body's organs. Problems with breathing are among the most serious symptoms. Mucus can obstruct the airways and cause bacterial infections in the lungs, leading to chronic coughing, wheezing, and inflammation. Over time, mucus buildup and infections lead to permanent lung damage, including the formation of scar tissue (fibrosis) and cysts in the lungs.

Most people with cystic fibrosis also have digestive problems. Mucus can block the ducts of the pancreas, preventing enzymes produced by that organ from reaching the intestines to help digest food. Problems with digestion can lead to diarrhea, malnutrition, and weight loss. Some babies with cystic fibrosis have meconiumileus, a blockage of the intestine that occurs shortly after birth.

Infertility, or the inability to conceive a child, is common in men with cystic fibrosis, but infrequent in women with the condition.

How do you get cystic fibrosis?

Cystic fibrosis is inherited in an autosomal recessive pattern, which means two copies of the gene must be altered for a person to be affected by the disorder. Most often, the parents of a child with an autosomal recessive disorder are not affected, but are carriers of one copy of the altered gene.

Mutations in the cystic fibrosis conductance regulator (CFTR) gene cause this disease. The protein made by CFTR controls the movement of salt and water into and out of cells. Mutations in CFTR block this movement, causing cells to produce the abnormally thick mucus characteristic of cystic fibrosis.

How common is cystic fibrosis?

Cystic fibrosis is a common genetic disease affecting Caucasians (whites) in the United States. The disease occurs in one out of every 3,000 Caucasian births. It also affects other ethnic groups, but is much less common: one in 15,000 African Americans and one in 31,000 Asian Americans have the disorder.

How is cystic fibrosis treated?

Cystic fibrosis and a comprehensive, multidisciplinary treatment program can lengthen survival time and improve quality of life. Specialty clinics for cystic fibrosis can be found in many communities and may be helpful.

Medications include antibiotics for respiratory infections and pancreatic enzymes to replace those which are missing. Vitamin supplements are sometimes prescribed. Inhaled bronchodilators are used to relieve chronic obstruction of the airways.

Other treatments include postural drainage, chest percussion and other breathing treatments.

Lung transplant may be considered in some cases.

New treatments include replacement of the DNAse enzyme. This is available as a medication called dornase (Pulmozyme). Genetic research is ongoing in hopes of correcting the disease by artificially inserting a "normal" gene into the person. This treatment is called gene therapy, and an intranasal form of gene therapy is currently undergoing clinical trials. Research on possible methods which could be used to correct the disorder before birth seems promising.

Where can I obtain services for cystic fibrosis in Missouri?

See the list of Treatment Centers.

What services does DHSS offer?

The Adult Cystic Fibrosis Treatment Program, established in 1975, provides assistance to Missouri residents who have been diagnosed with cystic fibrosis. Services are provided to individuals 21 years of age and over who meet the eligibility criteria of the program. If you are not financially eligible for the program, service coordination is available to help you find providers of service for your condition.

Eligibility: Applicants must meet both medical and financial eligibility guidelines. Medical eligibility is documented with a written diagnosis of cystic fibrosis. Financial eligibility guidelines, implemented in 1992, are based on the U.S. Department of Health and Human Services Poverty Income Guidelines at, or below, 185% of poverty. The Missouri Department of Health and Senior Services (DHSS) is the payor of last resort. All third party payors must be utilized before DHSS will consider reimbursement. Applicants and program participants must apply for MO HealthNet (Medicaid) medical assistance and complete the application process to obtain or maintain program eligibility. Please see the listing of the Missouri Department of Social Services, Family Support Division county office locations.

Program Services: DHSS will provide financial assistance for the following services related to the treatment of cystic fibrosis:

• Inpatient Services

• Outpatient Services, including, but not limited to, physical therapy and nutritional counseling

• Prescription Medications

• Home Medical Equipment - Approval for this service must be obtained through DHSS prior to the utilization of services.

• Emergency Care

Cystic Fibrosis Treatment Centers

Children's Mercy Hospital
2401 Gillham Road
Kansas City, MO 64108
(816) 234-3066

Saint Louis University Adult Cystic Fibrosis Program
3660 Vista, Suite 204
St. Louis, MO 63110
(314) 577-6190

University of MO Health Care
One Hospital Drive
Columbia, MO 65212
(573) 882-6978

Washington Univ. Medical Center
At Barnes - Jewish Hospital
1 Barnes - Jewish Plaza
St. Louis, MO 63110
(314) 454-8640

See Cystic Fibrosis Foundation for a listing of chapters and treatment centers.

Cystic Fibrosis Foundation

Cystic Fibrosis

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