Cystic fibrosis (CF) is an inherited disease of the mucus and sweat glands.It affects mostly your lungs,pancreas,liver,intestines,sinuses and sex organs.
Symptoms in newborns may include:
Delayed growthDelayed growth
Failure to gain weight normally during childhood
No bowel movements in first 24 to 48 hours of life
Salty-tasting skin
Symptoms related to bowel function may include:
Belly pain from severe constipation
Increased gas,bloating,or a belly that appears swollen (distended)
Nausea and loss of appetite
Stools that are pale or clay coloredStools that are pale or clay colored,foul smelling,have mucus,or that float
Weight loss
Symptoms related to the lungs and sinuses may include:
Coughing or increased mucus in the sinuses or lungs
FatigueFatigue
Nasal congestion caused by nasal polyps
Recurrent episodes of pneumoniapneumonia.Symptoms in someone with cystic fibrosis include:
Fever
Increased coughing
Increased shortness of breath
Loss of appetite
More sputum
Sinus pain or pressure caused by infection or polyps
Cystic fibrosis (CF) has no cure.The goals of CF treatment are to:
Prevent and control lung infections
Loosen and remove thick,sticky mucus from the lungs
Prevent or treat blockages in the intestines
Provide enough nutrition
Prevent dehydration (a condition in which the body doesn't have enough fluids)
Treatment for lung problems includes:
Antibiotics to prevent and treat lung and sinus infections.They may be taken by mouth,or given in the veins or by breathing treatments.People with cystic fibrosis may take antibiotics only when needed,or all the time.Doses are usually higher than normal.
Inhaled medicines to help open the airways
DNAse enzyme replacement therapy to thin mucus and make it easier to cough up
Flu vaccine and pneumococcal polysaccharide vaccine (PPVPPV) yearly (ask your health care provider)
Lung transplantLung transplant is an option in some cases
Oxygen therapy may be needed as lung disease gets worse
Treatment for bowel and nutritional problems (see:Cystic fibrosis - nutritional considerationsCystic fibrosis - nutritional considerations) may include:
A special diet high in protein and calories for older children and adults (see:Cystic fibrosis nutrional considerationsCystic fibrosis nutrional considerations)
Pancreatic enzymes to help absorb fats and protein
Vitamin supplements,especially vitamins A,D,E,and K
Your doctor can suggest other treatments if you have very hard stools
Care and monitoring at home should include:
Avoiding smoke,dust,dirt,fumes,household chemicals,fireplace smoke,and mold or mildew
Clearing or bringing up mucus or secretions from the airways.This must be done one to fours times each day.Patients,families,and caregivers must learn about doing chest percussion and postural drainage to help keep the airways clear
Drinking plenty of fluids.This is particularly true for infants,children,in hot weather,when there is diarrhea or loose stools,or during extra physical activity
Exercising two or three times each week.Swimming,jogging,and cycling are good options.Avoid contact sports,scuba diving,and endurance activities such as marathons