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Cystic Fibrosis

Cystic Fibrosis. Symptoms. In newborns: Delayed growth Failure to gain weight normally during childhood No bowel movements in first 24 to 48 hours of life Salty -tasting skin Related to bowel function: Belly pain from severe constipation

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Cystic Fibrosis

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  1. Cystic Fibrosis

  2. Symptoms • In newborns: • Delayed growth • Failure to gain weight normally during childhood • No bowel movements in first 24 to 48 hours of life • Salty-tasting skin • Related to bowel function: • 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 colored, foul smelling, have mucus, or that float • Weight loss

  3. Symptoms Continued • Related to the lungs and sinuses: • Coughing or increased mucus in the sinuses or lungs • Fatigue • Nasal congestion caused by nasal polyps • Sinus pain or pressure caused by infection or polyps • Recurrent episodes of pneumonia. Symptoms in someone with cystic fibrosis include: • Fever • Increased coughing • Increased shortness of breath • Loss of appetite • More sputum, saliva mixed with discharges from the respiratory passages

  4. Prognosis • Children with cystic fibrosis are fairly healthy until they reach adolescence or adulthood. • They can participate in most activities and attend school. • They can finish college or find employment. • Lung disease eventually worsens to the point where the person is disabled. • The average life span for people with CF who live to adulthood is approximately 35 years • Death is usually a result of lung complications.

  5. Treatment • For lung problems: • Antibiotics to prevent and treat lung and sinus infections • Inhaled medicines to help open the airways • DNaseenzyme replacement therapy to thin mucus and make it easier to cough up • Flu vaccine and pneumococcal polysaccharide vaccine (PPV) yearly • Lung transplant (is optional) • Oxygen therapy may be needed as lung disease progresses

  6. Treatment Continued • For bowel and nutritional problems: • A special diet high in protein and calories for older children and adults • Pancreatic enzymes to aid in absorbing fats and proteins • Vitamin supplements, especially with vitamins A, D, E, and K

  7. Treatment Continued • At home: • Avoiding smoke, dust, dirt, fumes, household chemicals, fireplace smoke, and mold or mildew • Clearing mucus or secretions from the airways (done 1-4 times per day) • Drinking plenty of fluids • Exercising two or three times each week

  8. Prevention • There is no way to prevent cystic fibrosis: • Screening people with a family history of the disease may detect the cystic fibrosis gene in 60 - 90% of carriers (depending on the test used • Tests & diagnosis: • blood tests available to help detect CF • test looks for variations in a gene known to cause the disease • Immunoreactivetrypsinogen (IRT) test is a standard newborn screening test for CF • high level of IRT suggests possible CF and requires further testing • Sweat chloride test is the standard diagnostic test for CF • high salt level in the patient's sweat is a sign of the disease

  9. Prevention Continued • Other tests: • Chest x-ray or CT scan • Fecal fat test • Lung function tests • Measurement of pancreatic function • Secretin stimulation test • Trypsin and chymotrypsin in stool • Upper GI and small bowel series

  10. THEEnd!

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