Paediatrics: Cystic fibrosis: management (2)

Cystic fibrosis: management (2)

Pulmonary care

Physiotherapy

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;

·  self-percussion;

·  deep breathing exercises;

·  use of flutter or acapello device.

Antimicrobial therapy

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.

Other therapies

·  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).

Gastrointestinal management

Distal intestinal obstruction (meconium ileus equivalent)

·  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^®.

Nutrition

·  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.

Fat-soluble vitamin supplements

·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).