Cystic fibrosis is the most common lethal
genetic disease occurring in the white population. It is estimated that 3.5% of the 20,000 individuals with
cystic fibrosis in North America will die each year of end-stage
lung disease.
Lung transplantation (heart-lung or double lung) is becoming more frequent as more patients are referred for this procedure. Since January 1988, we have evaluated 60 patients with
cystic fibrosis for
lung transplantation and have accepted 30 (50%). Nine patients (30%) died while awaiting a donor. Fifteen patients underwent
transplantation (13 heart-lung and two double lung procedures). Actuarial survival at 1, 2, and 3 years is 76%. All survivors are without physical limitations. Pulmonary function, as determined by forced vital capacity, forced expiratory volume in 1 second, and arterial blood gas determinations, is within the normal range. Comparing these data with those of a group of patients without
cystic fibrosis who underwent
transplantation during the same period did not reveal any significant differences with respect to
infection, rejection, and outcome. Preliminary data suggest that obliterative
bronchiolitis is less prevalent at 1 year in patients with
cystic fibrosis (19%) than in those without
cystic fibrosis (41%). Patients with
cystic fibrosis present a number of challenges. The problems of pleural adhesions from repeated
infections,
pleurodesis, and previous thoracic procedures are now readily approached through the bilateral thoracosternotomy (clam shell) incision.
Insulin-dependent diabetes mellitus and low-dose
corticosteroid therapy are no longer considered absolute
contraindications. Both septic lungs must be removed at operation, either with
heart-lung transplantation or with double
lung transplantation. These data support the therapeutic efficacy of
lung transplantation for patients with
cystic fibrosis.