De Soyza AG, Corris PA. Last updated November 2002. When should a patient be referred for transplantation? [online]. Freeman Hospital, Newcastle Upon Tyne, UK. Available from http://www.cysticfibrosismedicine
As a 'rule of thumb', patients are referred to the transplant centre when their FEV1 is less than 30% predicted normal. However, other factors should be taken into account such as the rate of decline in respiratory function, quality of life, a more frequent need for iv therapy, poor weight profile.
It is very difficult to predict precisely when an individual should have a transplant. In general decisions about the need for transplantation need to be addressed at least 2 years before the operation is likely to be essential. The physician needs to allow sufficient waiting time for both transplant assessment and the local availability of donor lungs that are in short supply. It is noteworthy that CF patients tolerate the waiting list poorly and are one of the patient groups most likely to die whilst waiting for transplantation whilst they achieve some of the best post-transplant survival rates.
Lung function
Early studies suggested that an FEV1 of less than 30% predicted was associated with a 50% 2 year mortality and this has been widely used as a benchmark when referring patients to the transplant centre. More recent evidence suggests that some patients with FEV1s as low as 15% predicted can survive for many years. In a study by Robinson et al only 1 of 30 patients dying from 1994-99 had an FEV1 <30% predicted normal two years before death. Doershuk et al reported that among patients with FEV1 less than 30% predicted , median survival was 4.6 years with 25% of patients living over 6 years. Similar results have also been reported by Augarten who found that patients with FEV1 <30% survive longer than patients post-lung transplant.
Weight
Low body weight is a risk factors for deterioration but does not influence the pos-transplant outcomes in most series.
Blood gases
Arterial blood gas analysis can also be useful and the international guidelines incorporate a paCO2>6.7kPA or paO2<7.3kPa as a referral criteria.
Other
Other recognised risk factors for deterioration include female sex.
Burkholderia cepacia is a well-established risk factor for deterioration. Recent reports suggest that the presence of Genomovar (G) III carries a poorer prognosis for transplantation with published mortality rates of 70-100% as compared to those for genomovar II and V (100% survival rates in our experience). A similar pattern excess mortality in GIII patients has been noted at the N Carolina transplant program. Combining the two reported series GIII patients do far worse than those with other non-genomovar III Burkholderia cepacia complex infections. This appears independent of the clonal origin of the Genomovar III strain as the N Carolina strains associated with mortality were GIII cable pilus negative (therefore not ET-12 clones). It is notable that infection with multi or panresistant Pseudomonas is not associated with poorer post-transplant outcome.
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