1 / 8

Cystic Fibrosis

Cystic Fibrosis. Taken from Deturk W, Cahalin L. Cardiovascular and Pulmonary Physical Therapy. An Evidence-based Approach. United States of America:McGraw-Hill Companies Inc;2004:465-467. Cystic Fibrosis. An inherited, autosomal recessive disease

oakley
Download Presentation

Cystic Fibrosis

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. Cystic Fibrosis Taken from Deturk W, Cahalin L.Cardiovascular and Pulmonary Physical Therapy.An Evidence-based Approach. United States of America:McGraw-Hill Companies Inc;2004:465-467

  2. Cystic Fibrosis • An inherited, autosomal recessive disease • The altered gene is located on the long arm of chromosome 7 • A deletion of a protein leads to altered Cystic fibrosis transmembrane conductance regulator (CFTR) • The defect affects chloride ion transmission across epithelial cells • Results in excessive sodium reabsorption that leads to • Dehydration of surface fluids • Abnormally salty sweat • Thick mucus that clogs tubes, tubules, ducts

  3. Cystic Fibrosis • Approximately 30,000 children and adults in the United States have cystic fibrosis • Most common lethal genetic disease of the Caucasian population • Both parents must carry the genetic defect • In 1998 mean survival rate was 32.3 years • According to the Cystic Fibrosis Foundation in 2008, the median predicted age of survival rose to 37.4 years • 90% of those with CF die of respiratory failure

  4. Diagnosis of CF • Commonly dx at birth, but may not be suspected until later in life • Positive Sweat Test = >60 mEq/L of Sodium • Meconium ileus at birth has become hallmark sign for dx

  5. Other Signs used for Diagnosis • Familial hx • Exocrine pancreatic insufficiency • Chronic pulmonary changes • Problems with reproductive functions • Males are azoospermic • Females have ↓ fertilization

  6. Characteristics of CF • Short stature with ↓body weight • Voracious appetites due to ↑ resting metabolic rate with intestinal malabsorption • Osteopenia / premature osteoporosis • Barrel chest with flattened diaphragm leads to a sense of fullness and ↓food intake

  7. Pathophysiology • Changes begin in the bronchioles • As CF progresses larger, more central airways become involved • Usually infected with Staphylococcus aureus or Pseudomonas aeruginosa • Mucus secreting glands become hypertrophied and ↑ secretions reduce lumen of airways • Normal mucociliary mechanism is impaired causing duct obstruction • Structure of airways are altered and brochiectatic reconstruction occurs

  8. Secondary impairments • Pulmonary hypertension • Right sided heart failure (cor pulmonale) • Pneumothorax • Hemoptysis

More Related