Referral for Lung Transplant Assessment
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Lung Transplant Assessment for a child with Cystic Fibrosis
Lung transplantation should be considered when there is a high risk of death from lung disease within 2 years if lung transplantation is not performed. To be suitable for transplant, candidates need to have a high likelihood (>80%) of surviving at least 90 days after lung transplantation and a high likelihood (>80%) of 5- year survival post-transplant. Transplant should be viewed as an option of last resort when there is progressive disease despite maximal medical therapy.
Lung transplant has been performed in New Zealand since 1993, with over 230 transplants carried out to date (see http://www.donor.co.nz/facts-and-myths/statistics/ for current information on transplant numbers and survival rates). Lung transplant is only done in NZ for teenagers (age >15 yrs) who are at least small adult size. For younger/smaller children, current best practise throughout Australasia is to be referred to The Alfred Hospital, Melbourne. This centre has a lower age limit of 5 years.
Referral for transplant and placement on the waiting list are 2 distinct processes. There is general agreement that referral to a lung transplant programme should occur early in patients who have a lung disease that is amenable to transplantation.
Note: Referral for lung transplant assessment will inevitably mean frank discussions with the patient and family/whanau about the risk of death from cystic fibrosis and from transplant.
Paediatric referral process
Referral criteria for lung transplant assessment in cystic fibrosis include:
- FEV1 <30% predicted or rapid decline in FEV1 despite optimal treatment (particularly in female patients)
- frequent exacerbations and/or frequent need for intravenous antibiotic therapy
- daytime supplemental oxygen requirement
- massive or recurrent significant haemoptysis
- refractory or recurrent pneumothorax
- poor quality of life
- pulmonary hypertension (systolic PAP of >35mmHg on echo)
- an episode of acute respiratory failure requiring non-invasive ventilation (NIV)
- life threatening haemoptysis despite embolisation
If a child with cystic fibrosis is thought to be approaching these clinical parameters, we recommend there be a preliminary discussion between the child's primary paediatrician and the Starship Cystic Fibrosis team.
It can be difficult to predict the pace at which a child will reach the above threshold for assessment, but health professionals should try to have these initial conversations one to two years before a transplant referral is considered. A discussion might be indicated when the child has a maximum FEV1 of <60%, or after they have had a significant pneumothorax of haemoptysis.
The Starship Cystic Fibrosis team recommend that a child for whom lung transplant has been considered should be referred for multidisciplinary inpatient assessment at Starship Hospital. The purpose of this is to determine if any aspects of routine cystic fibrosis care can be optimised to improve the current clinical status. During this assessment, intensification of routine therapy may be suggested eg commencing non-invasive ventilation, having overnight feeds. Optimising nutrition prior to lung transplantation can help to improve perioperative and post-lung transplant clinical outcomes1. The team will also liaise with the Adult lung transplant team and (if appropriate) arrange for a clinic review by a member of the lung transplant team
At this admission, information will be shared about the reason for consideration of lung transplant and the risks of dying before receiving donor organs, and of death post-transplant. The family also need to be made aware that:
- any decision to proceed or not ultimately rests with the patient, and not the family/whanau. However to be a transplant candidate the patient must have a reliable social support network.
- acceptable choices are to proceed, not to proceed, or to seek further information before deciding
- the lead time for paediatric transplant is typically longer than for adults (because the smaller size means that donor organs often take longer to become available).
- if a patient decides to proceed, this means they will go forward to formal lung transplant assessment
- depending on the outcome of this formal assessment, it is at this stage that they will either be accepted onto the programme or declined.
Information last reviewed: April 2017
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Information about the Paediatric Society of New Zealand