CYSTIC FIBROSIS
DIAGNOSTIC CRITERIA FOR CYSTIC FIBROSIS
- Presence of typical clinical features( Gastrointestinal/ Respiratory)
OR
- History of CF in sibling
OR
- Positive new-born screening test
PLUS
Lab evidence of CF transmembrane regulator dysfunction-
- ( 2 elevated sweat chloride concentration on separate days
OR
- Identifying ” CF mutations
OR
- Abnormal nasal potential difference measurement)
INVESTIGATIONS
- Sputum/cough swab
- Spirometry
- Bloods
- FBC, clotting Glucose
- LFT’s, U&E, bone profile Vit A/carotenoids/Vit E
- Ig’s/ IgE/Asp RAST/Asp precipitins Vit D
- Chest x-ray
DRUG TREATMENT: (start on sputum sensitivity)
Inhaled salbutamol
Inhaled salbutamol
NEBULIZED TREATMENT:
| Colomycin/ Promixin( Colistin) solution for injection | 1 (or 2) million unit/vial + 4 ml 0.9% saline Usually BD |
| TOBI/ Bramitob | 300mg BD 28 days on, 28 days off. |
| Hypertonic saline (7%) | 4 ML OD – BD |
| DNase | 2.5mg OD |
OUTPATIENT ANTIBIOTICS FOR CYSTIC FIBROSIS PATIENTS
ORAL ANTIBIOTICS
a)On-going PROPHYLAXIS:
FLUCLOXACILLIN |
100mg/kg/day | BD/TDS | All children with Staph infections |
CO- TRIMOXAZOLE |
6wk-6month- 120mg BD
6m-5year -240mg BD 6yr-12yr -480mg BD >12 years -960mg BD |
BD | In Exacerbation/ Haemophilus |
AZITHROMYCIN |
15-40 KG -250mg
>40KG -500mg |
OD | If pseudomaonas isolated |
DOXYCYCLINE |
0- 8 years- 4mg/kg | OD | In recurrent Infection(without Pseudomonas) |
b)FIRST LINE TREATMENT
CO-AMOXYCLAV |
50mg/kg/day | BD/TDS |
OR ADD
AMOXICILLIN |
100 mg/kg/day | BD/TDS | If on Flucloxacillin Prophylaxis |
Amoxicillin+ Flucloxacillin |
100mg/kg/day(max 4 gram/day) | BD/TDS | If Staphylococcus isolated |
c) SECOND LINE TREATMENT:
- New cough started, repeat first line
- If cough has not resolved, change to second line
- Get Sputum sample
AZITHROMYCIN |
10mg/kg/day(max 500mg/day) | OD-14 days | |
Or CEFEXIME |
8mg/kg/day(max 400mg/day) | BD | If on Azithromycin prophylaxis and no Pseudomonas in 6 months |
OR CIPROFLOXACILLIN |
15-30mg/kg/day (max 1.5gm/day) |
BD | If on azithromycin prophylaxis +Pseudomonas |
d) THIRD LINE TREATMENT:
- Productive cough more than 5-6 weeks of antibiotic therapy
- IV antibiotics
VITAMIN SUPPLIMENTS
INITIALY DAILY DOSE/ ADJUST TO LEVELS |
|||
| Vitamin A | Vitamin D | ||
| ABIDEC/ VIT A& D | 1.2ml | 8000 | 800 |
| <1 year | 1-5 years | >5 years | |
| Vitamin E (liquid 1ml= 100mg) | 50mg 75I.U |
100mg 200I.U |
200mg 400 I.U |
| Vitamin K | 10 mgs (in liver disease) | ||