Cystic fibrosis Flashcards
Define cystic fibrosis
- genetic disease
- affects lungs and pancreas
- can cause other complications and affect other organs
How many Canadians are:
- carriers of CF
- have CF
- carriers= 1/25
- have CF= 1/3600
What is the common mutation of the CFTR gene called
deltaF508
T/F
In 2018, 11.7% of female adults and 7.5% of male adults with CF are classified as overweight
F
Classified as underweight
If 2 parents are carrier of CF gene, what is the probability that child:
- develops CF
- carries CF gene
- doesn’t have CF
- 25% prob develops CF
- 50% prob carries CF gene
- 25% prob doesn’t have CF
Can a person with CF and a no,-carrier of CF have a normal child?
Yes
0% prob that the child will have CG (will always be carrier)
What are the 5 classes of CFTR mutations and the effect on protein
I- no protein 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 doesn’t work properly
V- protein reaches membrane and works properly but not many are made
Which mutation classes are the most severe?
Classes I, II, III
Why is thick mucus a problem?
- thick mucus can block several organs (pancreas, sinuses, intestines, reproductive, airways)
- thick mucus can accumulate, get infected and cause inflammation
Symptoms of a patient with 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 patients?
Pancreas insufficiency
What do pancreatic enzymes contain
lipase, protease, amylase
Different brands of pancreatic enzymes available
Creon 5,10,25, Pancrease, Viokase, Ultrase
When should pancreatic enzymes be taken?
Just before eating
Other management tools available for CF patients
- Pulmozyme
- Tobramycin and Cayston
- Azithromycin
- Bronchodilators or corticosteroids
- Multivitamins
- Vitamin D and Calcium
- Iron supplements, Zinc, Omega 3
- Prednisone as needed
- Lax-a-day, nasal wash as needed
- Physiotherapy
Predictors for non-compliance
- Long-term
- Asymptomatic
- Complex
Complications that can occur in CF patients are:
- frequent infections requiring hospitalization
- nutrient deficiencies due to malabsorption
- gastro-oesophageal reflux disease
- Inflammatory Bowel diseases
- osteoporosis
- intestinal obstruction (DIOS)
- liver disease
- kidney disease
- heart problems
- CF related diabetes
What are risk factors for developing DIOS (Distal Intestinal Obstructive Syndrome)
severe genotype pancreatic insufficiency fat malabsorption dehydration history of meconium ileum organ transplantation
Symptoms related to DIOS (Distal Intestinal Obstructive Syndrome)
ACUTE (fast) onset of severe abdominal pain, nausea, vomiting (including bilious material) and inability to pass gas or stool
Treatment for incomplete DIOS
-oral rehydration with stool softeners and laxatives
Treatment for complete DIOS
- IV rehydration
- nasogastric aspiration
- enemas, laxatives
- surgery may be required
What changes after DIOS in the patient
-more anxiety towards food and eating
What medical/dietary changes should be made after a DIOS
- changes on enzyme dosage
- keep track of water or fiber intake
- add laxatives
2 symptoms that help determine a plan for CF related-diabetes
- inexplicable decline in breathing functions
- inability to gain weight (or weight loss)
Assessments tests performed on CF patients
- breathing test at each visit
- weight monitoring at each visit
- X-ray of lungs or abdomen
- blood tests yearly and/or during exacerbations
- Dexa scan
- ultrasound
- ECG
- OGTT yearly
- colonoscopy if IBD present
Which possible vitamin deficiencies could occur when you have CF?
-vitamin A, D, E, K deficiencies because are fat soluble
What is the energy requirement of an individual with CF?
110-200% of that of a healthy individual of same age and gender
Why are energy needs higher in CF patients ?
- due to higher work of -breathing/coughing
- presence of inflammation
- malabsorption
- complications
What BMI should you aim for a women and a man with CF
Women: 22
Man: 23
Which factors might influence eating in patients with CF
- presence of dyspnea
- sinus problems
- medications (steroids
- GI problems
- slow intestinal motility
- history of DIOS
Why is there weight loss in patients with CF
Infection –> increased kcal needs and decreased appetite –> potential weight loss
Typical meal plan for CF patient
- high protein, high energy diet
- supplements added to diet as snacks
What should be taught to CF patients?
Teach ways to increase energy/protein in each recipes (ex: adding milk powder to shakes)
What is the median age of survival of patient with CF
52.1 years
What should always be monitored in a patient with CF
- weight
- nutrient levels in blood
What drug has been approved for 11 different mutations and by Health Canada ?
Kalydeco (ivacaftor)
What is a new drug combination to help bpateints with 2 comes of the F508 delta mutation
Orkambi
also Symdeko more recently
What is the problem with Symdeko and Trikafta
Very $
On a hot summer day what would be the cancer for a patient with CF?
- sodium levels decreasing in the body
