Management & Approach to Treatment
General Management
The outlook for the individual with CF has improved dramatically. Many of the clinical features previously thought to be inevitable can be prevented, delayed or improved by intensive treatment. The introduction of a more positive attitude to management and the more widespread use of aggressive treatment regimes have been major factors in improving longevity and quality of life. Better survival is associated with more frequent use of antibiotics and more frequent review at CF clinics. As insulin is to people with diabetes, so are regular, high doses of antibiotics to people who have cystic fibrosis.
Communication at the Time of Diagnosis
It is difficult for parents and/or patients to obtain more than a general impression of the condition when it is explained for the first time. Not only is CF a very complex disorder but parents are usually shocked and unable to follow detailed explanations at that time. There is a need to consolidate the information they receive at their first visit.
A team approach must be followed. Clinic visits should include consultation with medical personnel, physiotherapists, dieticians, clinic sisters, pharmacists, social workers, psychologists and parent support groups. Information must be made available to general practitioners, caregivers, teachers, relatives and friends. This information should be available in hard copy from the clinic. Additional sites of information, such as the Internet, may be of use.
It may be helpful for relatives to talk to the families of other affected individuals. Mutual support is generally most beneficial. "Remember, you are not alone".
General Management by a NON-CF Specialist
Some CF patients will be living far from a CF clinic and will find it difficult to attend CF clinics. They will be cared for by general practitioners (GPs) who do not have much experience with CF and who do not have ready access to the support of allied medical staff. It is essential that the GPs align themselves with the nearest CF Clinic and send their CF patient(s) at least yearly for assessment at the CF Clinic. Summaries of CF clinic visits should be sent to primary care doctors. As a relationship is built up with clinic doctors, so the GP will feel more comfortable about telephoning for advice and referring the patient when necessary.
General Facts Discussed at the Time of Diagnosis
- CF remains a serious disorder despite the major advances of recent years.
- The condition of the patient and the long-term outlook depends on the effectiveness
- The outlook continues to improve year by year.
- Individuals who have CF will always need regular follow-up at a hospital. The condition is so complex and advances in treatment so rapid that all patients must be cared for under the guidance of the CF specialist at the regional CF clinic.
- The hereditary aspects of CF.
- Families are told about the Cystic Fibrosis Association. If they agree, their names are forwarded to the relevant CF association.
General Precautions for the Individual who has CF
There are a number of reasonable precautions that should be observed by the CF individual and the family:
- Immunisation is very important (see Section 4.3, p*).
- NO SMOKING (active or passive). Smoking is particularly bad for people with CF.
- Starting nursery school or crèche should be delayed (ideally until 3 years of age).
- Reduction of exposure to friends and relatives who have just started with a "cold" as this is when they are at their most infectious.
- If an infant with CF is admitted to hospital, every effort should be made to provide a cubicle to reduce the risk of acquiring an acute viral infection from other acutely ill children.
- Avoidance of close contact with stables, compost or other forms of rotting vegetation is advised because of the risk of the inhaling Aspergillus spores or infection by Burkholderia cepacia.
CF patients should attend a specialist CF Clinic
- Patients should attend a CF Clinic every one to three months.
- Here the patient's progress must be reviewed by the entire team, if possible.
- At every visit the patient must be weighed and measured.
- At every visit a sputum sample or cough swab should be sent for microscopy, culture and sensitivity.
- Parents should have a sputum container at home to send to the laboratory in the event of new respiratory infection or production of unusually purulent sputum.
- From the age of five years, spirometry should be performed (by experienced personnel).
- Oxygen saturation should be measured using a pulse oximeter.
- To avoid cross infection when using all respiratory function equipment, the use of bacterial filters is advised.
- All staff must wash their hands between patients.
- A comprehensive CF assessment is recommended at diagnosis and annually
History/examination
Anthropometrics (height, weight, etc)
Immunisation status
Family/personal smoking
Confirmation of diagnosis
Sweat test
DNA testing
Lung status and Tests
Respiratory function
Bronchodilator test
Physiotherapist's assessment
X-ray chest
Sputum culture
Aspergillus precipitins & RAST (depending on age and symptoms)
Total IgE
Gastrointestinal status
Dietician's assessment
Electrolytes
Faecal human pancreatic elastase 1 (at diagnosis)
Modified GTT (if >10 years or younger if losing weight or there are symptoms
suggestive of diabetes mellitus)
Vitamin A, D, E serum levels (if available)
Adult diagnosis: ultrasound of liver and portal system
Additional tests
Full blood count
Liver function tests
Adult diagnosis: bone mineral density estimation
Other
Social worker consult
Immunisation
Normal childhood immunisations including pneumococcal vaccination should be administered since viral and bacterial respiratory tract infection can have a detrimental effect on the patient's lung function and disease progression.
An annual influenza vaccine covering the expected strains for that season should be given as a routine in March/April except if there has been anaphylaxis to egg.
Passive immunisation against the respiratory syncytial virus (Synergis®) for children under the age of 2 years is thought to be useful during epidemics.
Immunisation against chicken pox and hepatitis A is recommended.
A vaccine to Pseudomonas aeruginosa is under trial at present. When/If it becomes available it should be given prior to colonisation with the organism.
Annual Review
History/examination
Anthropometrics (height, weight, etc) and review of progress over the year
Immunisation status
Lung status and Tests
Respiratory function and review of the year
Physiotherapist's assessment
X-ray chest preferably a high resolution CT scan of the lungs every 2 years.
Sputum culture and review of the year
Full blood count
Total IgE, Aspergillus precipitins & RAST (depending on age and symptoms)
Gastrointestinal/Nutritional status
Dietician's assessment
Sodium, potassium, urea, creatinine, cholesterol, calcium, magnesium, alkaline phosphatase blood levels
Faecal human pancreatic elastase 1 if pancreatic sufficient at time of review
Modified GTT (if >10 years)
Vitamin A, D, E levels (if available)
Ultrasound of liver and portal system (>10 years)
Additional tests
Adults: bone mineral density estimation
Confirmation of both CF causing mutations re : future treatment modalities.
Other
Social worker review
Review and discussion of genetic/family issues.
The results of assessments and tests should be discussed with the patient and/or parents.
Included in the discussions should be:
Included in the discussions should be:
- Current health status
- Meaning of the changes (if any) over the year reviewed (good and not so good news)
- Adjustments to treatment regimes for the coming year
- Aims of the adjustments
- Discussion of the patient's CF care in general
- Planning for life events in the coming year e.g. school, employment