Cystic Fibrosis Flashcards
1
Q
Manifestations (9)
A
- Resp - chronic suppurative airways disease
*Head-to-toe:
- Sinus
- Pancreatic
- Malnutrition
- HPB
- GI
- Reproductive
- MSK
- Psychiatric, social, adherence
2
Q
Rx lung non-pharm (3)
A
- Neb hypertonic saline
- Chest CT
- Exercise
3
Q
Rx lung pharm (6)
A
- Pulmozyme - breaks down mucous, assists expectorate
- Azithromycin - MAC
- Prophylactic inhaled antibiotics
- Bronchodilators
*Potentiators/correctors
- Ivacaftor (G551d)
- Homozygous del508 - lumacaftor/ivacaftor
4
Q
Pancreatic (3)
A
- Endocrine: CF-related diabetes
- Exocrine: pancreatic insufficiency - Creon titrate to steatorrhea, fat soluble vitamin replacement (ABDEK)
- Pancreatitis
5
Q
MALNUTRITION (3)
A
- marker of disease activity
- height, weight, BMI
- diet, PEG feeds, NGT, CF-dietician
6
Q
HBP (4)
A
- Cirrhosis (focal biliary)
- Portal HTN
- Liver Tx
- GB disease
7
Q
GIT (3)
A
- GORD
- DIOS - distal intestinal obstruction syndrome
- Paed Hx - intussusception, rectal prolapse
8
Q
REPRODUCTIVE
A
- Males - obstructive azoospermia, no vas deferens - IVF, sperm harvest
- Females - tenacious cervical mucous
*family planning, contraception, high-risk pregnancy centre
*screen partner - 1/25 chance of being CF carrier
9
Q
MSK (3)
A
- OP
- HPOA
- CF arthropathy
10
Q
Psychiatric (2)
A
- Depression/anxiety
- Eating disorder
11
Q
Social (3)
A
- Supports/financial
- Stability
- Lifestyle
12
Q
Adherence
A
- Periods of non-adherence and reasons for?
13
Q
Discussion - monitor progress (9)
A
- Manifestations of CF and current Rx
- FEV1 trend and radiology progress
- Sputum culture
- Freq of exacerbation
- BMI/nutrition status
- Function
- Social
- Adherence
- Monitor Cx: BSLs, OGTT, CT, BMD, ABG
14
Q
CF Foundation (2)
A
- Fund Rx
- Provides spirometers, nebulisers
- CF support groups
15
Q
Tx candidate
A
- FEV1<0.4L or Cx: PTx, haemoptysis, NIV
