1. What are the signs and symptoms of cystic fibrosis?
- very salty tasting skin
- persistant cough
- frequent lung infections
- wheezing or shortness of breath
- poor growth/weight gain in spite of a good appetite
- frequent greasy, bulky stool or difficulty in bowlel movement
2. How common is this disorder?
- There are 70,000 people diagnosed world wide. About 1,000 new cases of CF are diagnosed each year
3. How is cystic fibrosis diagnosed?
- a gene from each parent
- sweat test: measures the salt concentration in sweat
4. How is cystic fibrosis inherited? Does everyone who has a mutant gene for the protein have cystic fibrosis?
- Cystic Fibrosis is inherited from the parent(s). It doesn’t have to be an active gene.
1. Explain the normal function of the protein that is defective in cystic fibrosis.
- the protein should allow chlorine to exit.
2. What happens to this protein in CF patients and what are the consequences for the health of these individuals?
- the protein is blocked and therefore no water is let in to moisten the building mucas. When the mucas dries it sticks and becomes harder to remove. This injures the individuals lungs.
1. Explain at least 3 treatments for the symptoms of cystic fibrosis.
- Medications: Antibiotics, used to treat lung infections, Mucas-thinning drugs, reduce stickiness in mucas so you can cough it up easier, Bronchodilators, keep airways open and relaxed
- Therapy: Chest Clapper, hand held divorce that mimics cupped hands clapping over ribs, Inflatable vest, vibrates at high frequency to loosen chest mucas, breathing device, performing specific breathing exercises while exhaling through device’s tube or mask
- Surgery: Feeding tube, delivers nutrients, Lung transplant, in cases of severe breathing problems, Bowel surgery, emergancy bowel removal.
2. Discuss at least 3 ways for parents to help their children who have cystic fibrosis.
-oral enzymes and better nutrition
- drink lots of fluids
-keep immunizations up to date