When a baby is first diagnosed with CF, they and their parents are usually seen weekly until the family is managing the required therapies, such as giving enzyme replacements, monitoring weight and physiotherapy.
Following a diagnosis, it is important that the baby’s family gets relevant information about Cystic Fibrosis and education on how to care for a child with this condition. This will include information about the need for and the giving of enzyme replacements, the need to maintain a good bodyweight, twice daily physiotherapy and the importance of being seen regularly by health care professionals.
Not all babies with CF need enzymes to help them digest food. In some variations of the CF gene, the pancreas provides sufficient enzymes to correctly digest the food, in other gene variations the pancreas does not produce enough enzymes therefore they need to be supplemented with an enzyme replacement capsule. To find out if enzyme supplementation is required, a faecal fat test is done to determine if the baby is producing sufficient enzymes themselves.
Hospitals in New Zealand give parents with a newly diagnosed infant, and Information Pack from Cystic Fibrosis New Zealand (CFNZ). This pack includes items such as: an introduction to CFNZ and the services we can provide, contact information for CFNZ representatives, and information for new parents written by New Zealand CF health professionals and parents, and a notebook to keep track of those all-important details.
If you do not receive this pack please contact the CFNZ Office.
Genetic Counselling is available for all families of newly diagnosed infants, and is provide by Genetic Services. This counselling provides an understanding into the genetics of Cystic Fibrosis, the likelihood of further offspring being affected by CF, and the options available to families wanting more children.
Genetic Counsellors can also recommend which members of your extended family should be tested to see if they are carriers of the CF gene variant, and can arrange for this testing to be done. The test requires only a simple blood sample to be taken.
Children and adults with CF often have planned admissions. If they become unwell with increased cough and sputum, they are usually treated at home with oral antibiotics and with an increase in their normal twice daily physio sessions. The GP or the doctors at the hospital monitor them over the course of the oral antibiotic treatment. If the treatment is clearly not improving, then the patient is admitted to hospital for more intensive therapy. Often they will be reviewed at clinic and then be admitted on the same day or when it better suits the child or family.
Hospital treatments generally involve a two week course of IV therapy with intensive physiotherapy, a dietary review and a social worker review. Hospitalisation can be a time to look at all the treatments the patient may be having at home and reviewing each aspect.
Once a year the children have an Annual Review. This normally coincides with their birthday month and is generally longer than a routine clinic visit as extra tests are scheduled. Patients are seen by the Doctor, the Dietician, the Physiotherapist and the Nurse Practitioner from the Child Liaison Team to discuss any emotional concerns. Checks are made on compressors and nebuliser bowls, spacers and asthma devices etc.
During the Annual Review, blood tests, a chest x-ray and sputum or throat swabs are usually done. If small children are having problems with the taking of blood tests or chest x-rays, your Nurse or Physician can arrange for the Play Therapists to assist the child. Play Therapists will generally see the child at an extra visit and it may take a few sessions until the child is able to go through with the procedures.
The Annual Review is a time for a general overview of the patient, for all members of the team and the parents. It is to make sure that the child is being adequately treated and growing well.
There are paediatricians in every regional hospital in New Zealand who look after children with CF. Sometimes there is just one in a centre but sometimes all of the doctors have some CF patients each. Many of these doctors have been designated as Regional CF Paediatricians with responsibility for ensuring that babies detected by the new-born screening program have follow up clinical assessment and sweat testing to confirm or exclude diagnosis.
In early 2011, Cystic Fibrosis New Zealand launched a new data registry for people with Cystic Fibrosis in New Zealand.
This data registry, Port CF, has been developed by the Cystic Fibrosis Foundation in the USA and is currently also in use in the United Kingdom.
The registry is a benchmarking and data collection tool that will assist clinicians to compare their CF Clinic patient outcomes against those of other clinics around NZ and across the world. It is our intention that the registry will be a source not only of important data information, but will also be use as a quality improvement tool.
All people with CF in New Zealand have the option of being part of this data set, and only data from those who give permission will be entered into the registry. If you have not been approached by your CF team, you can ask to join, or make enquiries through the CFNZ Administration Manager.
Once up and running, each year an annual data ‘snapshot’ will be taken, from which a Data Registry Report will be compiled. This snapshot will allow us to compare clinics within NZ and across the world, identify points that need specific attention, and help define quality improvement strategies.