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Cystic Fibrosis

Table of Contents

Introduction

Cystic fibrosis occurs in about 1 in 2,500 births in Victoria. All patients are seen at the Royal Children's Hospital at the time of diagnosis, which is usually in the first year of life. Some patients living in the south-east of the city or eastern Victoria are cared for jointly by the department of Thoracic Medicine at the RCH and the Department of Paediatric Respiratory Medicine, MMC.

The prognosis has improved very considerably over the last 20 years and most patients now reach adulthood. In the ward the resident medical staff will see patients who have acute flare-ups of chest infection and those with severe lung disease. The 50% of patients who have little chest trouble are rarely admitted to the ward, so staff are likely to have a biased view of the condition.

The development of a very positive attitude by the parents from the time of diagnosis seems to be very important in the prognosis. The handling of the family at the time of diagnosis is carefully planned. If they are given a gloomy outlook, this can be hard to change.

Inpatient Management

Admission of Infants and Children with Possible CF

If the possibility of CF has been raised with the parents, the Consultant will tell you. If it has not, the words 'Cystic Fibrosis' should not be mentioned to the parents. If they ask why a sweat test is being done, say it is to sort out the baby's problems. If they want further information they should speak to the consultant. Once the diagnosis has been established, both parents will be seen by the Consultant and the diagnosis given. If the parents ask for the result of the sweat test prior to the interview, it is best to say that the result is not yet available.

The Paediatric Respiratory Physician will outline the patient management following diagnosis.

Admission of a CF patient for Treatment of Exacerbation of Chest Infection

Children admitted with an acute exacerbation are usually kept in hospital until they have returned to their previous state or their condition has plateaued. A 'tune-up' is usually is for a period of two weeks. If further benefit is anticipated from a longer admission then a patient may stay for a further week. Admissions for more than 3 weeks are very unusual.

  1. Physiotherapy

    Notify the Physiotherapist (Christine Blackburn) and Dietitian (Alison Caiafa) of the patient's admission. The physiotherapist will organise chest physiotherapy 2-3 times a day and the dietitian will order appropriate meals from the diet kitchen.

  2. Antibiotics

    Insert a IV with a heparin lock. If after 2 attempts the RMO cannot insert the IV, the Registrar should be called. If the cannula comes out between 11 p.m. and 7 a.m. it is normally replaced the following morning, unless the child is very ill.

    To decide on antibiotics, consult the protocol currently used or the Paediatric Respiratory Physician.

  3. Pancreatic Enzymes

    The dose of enzymes that the patient was receiving must be continued in hospital. If malabsorption is suspected, send a stool specimen for fat globules to the Microbiology Laboratory.

  4. Inhalations

    Most patients receive nebulised salbutamol before chest physiotherapy. Some patients find inhaled hypertonic saline (6%) prior to chest physiotherapy, and inhaled gentamicin (40mg) following chest physiotherapy beneficial however, these agents are not given routinely for the management of an acute exacerbation.

  5. Electrolyte powder

    All patients receive added salt in their diet either as electrolyte powder or salt tablets. The dose may need to be increased during the summer months as the salt loss from sweating increases.

    Infants under 2 years require approximately 1/4 teaspoon b.d. in winter and t.d.s. in summer. Children over 2 years, 1/2 teaspoon.

  6. Pulmonary Function Tests.

    These are performed in children over the age of 7 years in the Respiratory Function Laboratory on the second floor in the Department of Respiratory Medicine (ext. 2278). They are done on admission, at weekly intervals during the admission and immediately prior to discharge.

  7. Chest X-Ray

    A chest x-ray should be performed if the patient is very unwell or if a CXR has not been performed during the past 6 months.

On discharge From Hospital

  1. Antibiotics
    • If no lung disease - none
    • If minimal lung disease - probably oral Trimethoprin, Ceclor or Augmentin.
    • If mild-moderate - oral Augmentin.
    • If in doubt discuss with the Paediatric Respiratory Physician.
  2. Pancreatic enzymes, plus salt supplements, vitamins, inhaled medications if required.
  3. Follow-up appointment

    Outpatient appointments may be arranged by telephoning Dr. Freezer's rooms (x2552). Combined RCH/MMC CF clinics are held twice yearly at MMC.

Meconium Ileus Equivalent

  1. Arrange an abdominal XRay
  2. Notify the Paediatric Respiratory Physician, Dietitian and Physiotherapist of the child's admission.
  3. If the child has been vomiting, give IV fluids (nil orally). If there have been only 1 or 2 vomits, it may be reasonable to persist with oral fluids.
  4. If pain rather than vomiting has been the problem, give clear fluids.
  5. Oral laxatives eg Golytely may be useful.
  6. Consider the use of a disposable enema (phosphate or microlax).
  7. The Paediatric Surgeon on call is consulted if the symptoms are troublesome, particularly if there is doubt about the diagnosis.
  8. Prior to discharge review the dose of enzymes and the patient's diet.