Cystic fibrosis: management (2)
All children with CF should have physiotherapy at least twice a day. Parents and older children are taught how to do some of the following:
· chest percussion;
· postural drainage;
· deep breathing exercises;
· use of flutter or acapello device.
Most experts recommend antibiotic therapy.
· Oral during periods when well: against Staphylococcus aureus and Haemophilus influenzae.
· IV for acute exacerbations: initially courses of antibiotics can be administered via an indwelling long-line that should last a number of weeks if needed. However, as infections become more frequent, a permanent form of IV access (such as an indwelling Portacath) will help.
· Nebulized for those chronically infected with Pseudomonas aeruginosa.
· Annual influenza immunization.
· Bronchodilators for those with reversible airway obstruction.
· Mucolytics: recombinant DNAase 2hr before physiotherapy; or inhaled hypertonic (7%) saline used before physiotherapy.
· Oral azithromycin (long-term anti-inflammatory).
· Lactulose: 1mL/kg/day.
· Oral acetylcysteine solution: prophylaxis 15mL of 10%/day in <7-yr-olds and 30mL in >7-yr-olds. Treatment doses are double to three times this amount.
· Gastrografin®: oral dose can be used as a single treatment dose (50mL for children 15–25kg, and 100mL for those >25kg). Fluid intake should be encouraged for 3hr after administering the Gastrografin®.
· Pancreatic insufficiency: treated with oral enteric-coated pancreatic supplements (Creon®) taken with all meals and snacks. Ranitidine or omeprazole may be useful if the response to enzymes is unsatisfactory.
· High-calorie diet: children with CF require 120–150% of normal energy intake.
· Salt supplements: salt depletion is a risk in CF patients during the first year of life, and in the summer months in older patients. In exceptionally hot weather supplements include 500mg/day during the first year, 1g/day in <7-yr-olds, and 2–4g/day in >7-yr-olds.
·Multivitamins: Dalivit® drops 1mL/day or multivitamin tablets.
·Vitamin E: 50mg/day if <1yr; 100mg/day 1–16yrs.
·Vitamin K: if there is evidence of liver disease (hepatosplenomegaly or abnormal clotting).