CF is a serious life-long condition, but the severity of the illness can vary greatly. The average life expectancy for someone with CF is about 35 years. Although, some with mild forms of CF can live to age 60 or beyond.
CF is a genetic disorder. A child with CF inherits defective genes from each parent. Parents who have the gene, but do not have CF, are called carriers.
Factors that increase your chance of CF include:
Parents who are known carriers of the CF gene
Siblings with CF
Parents with CF—more often the mother since men with CF may be sterile
The abnormally thick mucus of CF blocks certain organs. This causes many of the symptoms of CF.
Symptoms in infants may include:
Difficulty passing the first stool
Intestinal obstruction, sometimes requiring surgery
Mucus that causes blockages in the lungs may lead to:
Coughing and wheezing
Shortness of breath
Difficulty with exercise
Abnormally shaped clubbed finger tips
Mucus can also block the pancreas. This can block enzymes used to help you digest food. This can lead to:
Bulky, bad-smelling, floating stools, due to poor digestion of fats
Overall, girls are affected more severely than boys.
You will be asked about your symptoms and medical history. A physical exam will be done. CF is suspected in a child with classic symptoms, especially if a sibling has CF.
CF is often diagnosed by symptoms, family history of CF, or a positive screening test in newborns. The diagnosis may be confirmed with genetic testing. Other lab tests that may be used to confirm CF include:
Tests may also be needed to check the pancreas. These tests may be done to assess symptoms or determine treatment.
There is no cure for CF. Treatment is aimed at:
Improving the amount of nutrition your body receives
Preventing and treating lung and sinus infections
Keeping the airways and lungs as clear as possible
Treatment for CF includes:
Better nutrition will help improve overall health. It will also improve growth and development in children. Children who have returned to normal weight within 2 years of the diagnosis have fewer coughing episodes and better lung function. Some nutritional steps that may help include:
A high-calorie diet planned by a registered dietitian
Nutritional supplements, including fat-soluble vitamins
Pancreatic enzyme tablets with meals to improve digestion and absorption of nutrients
Drinking lots of fluids and salt replacement, especially in hot weather or during illnesses
Managing Lung Infections
Thick mucus in the airways increases the risk of respiratory infections. The infection can also be more severe because of the mucus. Treatment of a current infection often requires antibiotics. Prevention of new infections may be done with:
Antibiotics (usually inhaled)
Lung and Airway Support
Medications help keep the airways clear. Most will be delivered through an inhaler or nebulizer. Medications may include:
Bronchodilators—to relax muscles and open the airway
Mucolytic agents—to reduce mucus and help it move out of the lung
Steroid inhalers—to decrease swelling and irritation (only when necessary)
Other steps that may help clear mucus from the lungs include:
Hypertonic saline is a special type of salt water. A nebulizer machine creates a mist of this saline, which is inhaled. The mist may help thin out the mucus in the lungs.
is rhythmic clapping over the chest. They may help clear mucus from airways.
It is most helpful if done at least twice per day.
may be required as the disease progresses. Ventilation may also be needed.
Talk to your doctor about whether these are options for you.
Surgery may be required to treat blockages in the intestine.
may also be considered.
Support is important for those with CF and their families. Ask you doctor about local support groups or counseling options.
If you have the defective genes, there is no way to prevent CF.
Adults can be tested to see if they carry the genes before having children.
can determine if a baby will have CF.
About cystic fibrosis: what you need to know. Cystic Fibrosis Foundation website. Available at: http://www.cff.org/AboutCF. Accessed February 12, 2018.
Cystic fibrosis. Healthy Children—American Academy of Pediatrics website. Available at: https://www.healthychildren.org/English/health-issues/conditions/chronic/Pages/Cystic-Fibrosis.aspx. Updated November 21, 2015. Accessed February 12, 2018.
Genetic testing. American Medical Association website. Available at: http://www.ama-assn.org/ama/pub/physician-resources/medical-science/genetics-molecular-medicine/related-policy-topics/genetic-testing.page. Accessed February 12, 2018.
4/16/2009 DynaMed Plus Systematic Literature Surveillancehttp://www.dynamed.com/topics/dmp~AN~T116913/Cystic-fibrosis-CF: Lai HJ, Shoff SM, Farrell PM, Wisconsin Cystic Fibrosis Neonatal Screening Group. Recovery of birth weight z score within 2 years of diagnosis is positively associated with pulmonary status at 6 years of age in children with cystic fibrosis. Pediatrics. 2009;123(2):714-722.
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