Q. What types of lung
transplant procedures can be performed on patients with cystic fibrosis?
In the United States, patients with cystic
fibrosis undergoing lung transplantation almost exclusively receive
bilateral (double) lung transplants. Single lung transplantation is
not applicable to patients with cystic fibrosis because of the chronically
infected nature of cystic fibrosis lungs. If a single lung transplant
was performed in a patient with cystic fibrosis and a native lung was
retained, the new grafted lung would be contaminated from the native lung.
In other countries, in particular, Great Britain, there is extensive
experience of heart / lung transplantation for cystic fibrosis. There
are no advantages of heart / lung transplant over bilateral lung
transplantation; hence, bilateral lung transplantation is preferred, which
has the additional advantage of providing a heart for another recipient for
cardiac transplantation.
Q. In which patients with cystic
fibrosis is lung transplantation recommended?
Lung transplantation is recommended in
patients with cystic fibrosis for improvement in quality of life and
prolongation of life. The specific complications of lung
transplantation in patients with cystic fibrosis are severe disorders of
other systems, and resistant organisms in the sputum (multi-resistant
organisms are not necessarily a complication), and excessive loss of
physical and nutritional reserves. Previous thoracic surgical
procedures including talc pleurodesis are not a contraindication to lung
transplantation.
Q. When should patients with cystic fibrosis
be referred for consideration for a lung transplant?
Patients with cystic fibrosis should be
considered for lung transplantation when there is progressive decline in
lung function - normally below 30% of predicted, increasing frequency of
hospitalizations and decreasing weight despite nutritional support.
Q. What can the patient do while
waiting for lung transplantation?
It is important for the patient to keep in
close contact with the primary pulmonary care team as well as the lung
transplant team. Particular issues may require attention, including
prompt treatment of infection, early treatment of respiratory failure in
particular to avoid intubation and ventilation (the results of lung
transplantation are poor in this setting), nutritional support (including,
if necessary, a gastrostomy tube), and encouraging mobility. Waiting
for a lung transplant can be a very stressful time and it is very important
that the patient receives support from within the cystic fibrosis community
and the lung transplant community. This can be extremely helpful,
particularly when the waiting time becomes prolonged.