What is Cystic Fibrosis?
Cystic Fibrosis is an inherited disease that affects the lungs, mucus, sweat glands, and digestive systems of approximately 70,000 children and adults worldwide.
- About 1,000 new cases of cystic fibrosis are diagnosed each year.
- More than 70% of patients are diagnosed by age two.
- More than 45% of the CF patient population is age 18 or older.
- Males and females from all racial and ethnic groups can get Cystic fibrosis.
- It is most common among Caucasians of Northern European descent.
- More than 10 million Americans are carriers of a CF gene.
- Most do not know that they are carriers of the gene.
Symptoms of Cystic Fibrosis:
- salty-tasting skin
- rapid heart rate
- persistent, phlegmy coughing
- frequent lung infections
- wheezing or shortness of breath
- poor growth/weight gain in spite of a good appetite
- frequent greasy, bulky stools or difficulty in bowel movements
Cystic Fibrosis Progression:
The severity of symptoms of Cystic Fibrosis can vary wildly and may not even become evident until the adolescent years.
As the disease progresses, the symptoms become progressively more severe. Pulmonary infections are often resistant to traditional antibiotic regimens. Patients are at a higher risk for osteoporosis, diabetes, and problems with fertility. Because of the potential for compromised pancreatic and digestive function, many CF patients experience pancreatitis, intestinal blockages, liver disease, and gallstones.
Cystic Fibrosis in Daily Life:
Cystic Fibrosis is a disease that affects each person with varying degrees of severity and therefore each person with CF will follow a different, individualized treatment plan.
For many of those with cystic fibrosis, life consists of:
- Daily physical exercise
- Inhalation therapy (nebulizers) to keep the lungs free of thick mucous
- Eating a high calorie diet
- Following a pancreatic enzyme regimen
- Taking vitamin supplements
- Visits to a CF clinic
- Reducing exposure to sick people
- Effective and regular hand-washing for at least 30 seconds each time.
- Regular flu shot immunizations (flu shot locator here)
- Avoiding second-hand smoke
Cystic Fibrosis Screening:
All states in the United States screen newborns for CF using a genetic or blood test. If a genetic test or blood test suggests Cystic Fibrosis, a doctor will confirm the diagnosis using a sweat test.
Other screenings include Genetic Carrier Testing. Because more than 10 million Americans are symptomless carriers of the defective Cystic Fibrosis gene, the blood test can identify the carriers who could otherwise pass the genes onto their children. To inherit Cystic Fibrosis, a child must inherit one copy of the defective gene from each parent.
Statistically, if both parents are carriers of the defective gene, each time they produce a child, the chances are:
- 25% the child will have Cystic Fibrosis
- 50% the child will carry the Cystic Fibrosis gene but not have the disease
- 25% the child will not carry the gene and not have the disease
If you or your partner is known to be a carrier of the Cystic Fibrosis defective gene, you will be advised to undergo genetic counseling prior to or during a pregnancy.
Cystic Fibrosis Diagnosis:
The sweat test is the most useful test for diagnosing Cystic Fibrosis.
It is performed when the doctor triggers sweating on a patch of skin and measures amount of salt in the sweat sample collected. The test is usually repeated. Higher than normal levels of salt indicate Cystic Fibrosis.
If you or your child has CF, there are other tests that your health care professional might recommend, including:
- Genetic tests to find out what type of Cystic Fibrosis genetic defect is causing your CF
- Chest x-rays
- Sinus x-rays
- Lung function tests
- Mucus/sputum cultures
- Prenatal screenings if you are or become pregnant. These tests include amniocentesis and chorionic villus sampling (CVS) to determine whether the fetus has Cystic Fibrosis
Treatment of Cystic Fibrosis
Today, Cystic Fibrosis has no cure.
However, recent years have seen tremendous advances in treatment that have vastly improved patients quality of life and prognoses.
The primary goals of treatment include preventing and managing lung infections, loosening and eliminating mucus from the lungs, preventing intestinal blockages, providing adequate nutrition, and maintaining appropriate hydration.
Related Resource Pages on Band Back Together
Cystic Fibrosis Resources:
Cystic Fibrosis Foundation -a non-profit donor-supported organization, working toward the development of the means to cure and control cystic fibrosis and to improve the quality of life for those with the disease. It has a ton of links, information, for those suffering CF and the families affected by it. Find a Clinical Trial on their site.
Cystic Fibrosis Pharmacy - pharmacy devoted to cystic fibrosis. Prices are kept low as drugs are bought in larger quantities. The pharmacy also works with insurance plans to cover CF medications. The staff is all trained in CF and is devoted to promoting the highest level of care for those who have cystic fibrosis.
National Institutes of Health Clinical Research is the primary federal agency for conducting and supporting medical research.
CF Legal Hotline - devoted to helping those with CF help navigate the medical and insurance system as well as making certain that those with CF are in full understanding of the laws surrounding the disease.
CysticLife - is a social network just for the cystic fibrosis community. This positive, uplifting web site is the central location for the CF community to share tips, questions, ideas, experiences and encouragement.
Cystic Fibrosis Canada - Non-profit organization in Canada with many resources for CF; it advocates for high quality CF care, promotes awareness of CF and funds research into care and control of cystic fibrosis.
American Lung Association - Non-profit volunteer organization focused on saving lives by improving lung health and preventing lung disease through education, advocacy and research. The website has extensive information on several different lung diseases, including cystic fibrosis.