Cystic fibrosis Flashcards
(38 cards)
Is CF an acquired or genetic disease?
purely genetic
Which organs are affected by CF?
lungs and the pancreas
Can cause other complications and affect other organs
What is the prevalence of CF carriers
1 in 25
What is the chance of having a child with cystic fibrosis by carrier parents?
1/4 chance of having a child with cystic fibrosis by carrier parents
Is CF gene dominant or passive
passive
Outcomes of CC and cc parent (CF and non-carrier)
100% of a healthy child of 1 CC parent and 1 absolutely normal parent
What are the classes of CF?
First 3 are the most severe
I- No protein is made
II- Protein is abnormal and destroyed before it reaches membrane
III- Protein reaches membrane but channel is blocked
IV- Protein reaches membrane but channel does not work effectively
V- Protein reaches membrane and works properly but not many are made
lung cells with and without CF
Chloride channel is affected, causing Cl to be trapped within the cell. This causes salt to stay inside the cells causing less water to be transported out of the cell (water follows Na)
This results in a sticky, concentrated mucus
Why is thick mucus a problem?
Thick mucus can block several organs:
•Pancreas
•Sinuses
•Reproductive -> infertility
•Intestines-> obstruction
•Airways in the lungs-> difficulty breathing
Thick mucus can accumulate, get infected and cause inflammation. The perfect storm for bacterial growth!
Symptoms of CF
Persistent cough Difficulty with growth Abdominal pain Bloated stomach Salty tasting skin Clubbing of fingers
What is the main reason for malnutrition in CF
Pancreatic Insufficiency (84% prevalence of insufficiency)
What is the reason of pancreatic insufficiency in CF?
It occurs because the thick mucus in your pancreas blocks pancreatic enzymes from entering the small intestine
Why are there different brands of pancreatic enzymes available?
various tolerance by different subjects
What are the enzymes in pancreatic enzyme drugs?
They contain lipase, protease and amylase
When should pancreatic enzymes be taken and what does the dose depend on?
- Need to be taken just before eating
- Number of pills may need to be adjusted depending on the meal/snack
What are the other management tools for CF and why?
Pulmozyme- sed to improve lung function in people with cystic fibrosis by thinning pulmonary secretions and reducing the risk of respiratory tract infections. Inhaled
Tobramycin (TOBI) and Cayston- antibiotic
Azithromycin
Bronchodilators or corticosteroids
Multivitamins- due to digestion problems and low absorption of vitamins
Vitamin D and Calcium
Iron supplements, Zinc, Omega-3 as required
Prednisone as needed- glucocorticoid medication mostly used to suppress the immune system and decrease inflammation
Lax-a-day, nasal wash as needed
Physiotherapy- to clear out lung mucus
What are the 3 factors that dictate the compliance to treatment? How do they affect CF treatment compliance
1) Long-term-> CF treatment is very long-term
2) Asymptomatic - CF is often asymptomatic
3) Complex
Low compliance with CF treatment
Complications of CF
Frequent infections requiring hospitalization- as mucus harbours bacterias
usually 3-4 per year
Nutrient deficiencies due to malabsorption
Gastro-oesophageal reflux disease
Inflammatory Bowel diseases
Osteoporosis- due to decreased absorption and digestion of food and supplement Vit D
plus the use of steroids to help with inflammation which are very blood thinning
Intestinal obstruction (DIOS)
Liver disease (ex: fatty liver)
Kidney disease
Heart problems
CF related diabetes- due to thick mucus scarring on pancreas; This scarring prevents the pancreas from producing normal amounts of insulin; so, like people with type 1 diabetes, they become insulin deficient.
What is Distal Intestinal Obstructive Syndrome (DIOS)
involves blockage of the intestines by thickened stool
What are the risk factors for DIOS?
Risk factors include severe genotype, pancreatic insufficiency, fat malabsorption, dehydration, history of meconium ileus (obstruction at birth_ or DIOS, organ transplantation, etc.
Speed of symptoms appearance constipation vs intestinal obstruction
obstruction symptoms are very soon to show
constipation symptoms are slower to show and the condition is not as severe
Symptoms of DIOS
Symptoms include acute onset of severe abdominal pain, nausea, vomiting (including bilious material) and inability to pass gas or stool.
Treatment for DIOS
Incomplete DIOS:
Oral rehydration with stool softeners and laxatives (ex. PegLyte)
Complete DIOS: IV rehydration Nasogastric aspiration Enemas, laxatives Surgery may be required
patient may be NPO during obstrucutin
have to refeed slowly-> clear liquid to start
Precautions after DIOS
Patient may have anxiety towards certain foods or eating in general
Changes may need to be made on enzyme dosage
Patient may need to keep track of their water or fiber intake
Patients may have to add laxatives to their usual routine