For the first several months following transplantation, children and their families are enrolled in a multispecialty rehabilitation program. Cardiopulmonary reconditioning is achieved in a monitored setting under the supervision of physical and pulmonary rehabilitation specialists. Patients and their families are instructed in the use of spirometers and pulse oximetry; patient logs are kept at home recording these data. Families are educated in the significance of changes in these data. Because the risk of infection is highest during the first 3 months posttransplant, patients are secluded from crowds until their immunosuppres-sion regimen is down to lowest levels; in the interim, patients wear surgical masks when in public.

Hemoglobinopathies Survival Curve
Fig. 13D.3. Actuarial survival for pediatric lung transplants between 1990 and 2001, by age group. (From Boucek MM et al. The Registry of the International Society of Heart and Lung Transplantation: Fifth Official Pediatric Report-2001 to 2002. J Heart Lung Transplant 2002; 21:827-840.)

Hospital readmission is relatively common (54%) during the first year posttransplant, principally for infection. But by 2 years follow-up only 13% of patients require readmission, again for treatment of infection (Fig. 12D.3). Functional status among lung transplant recipients is excellent. According to the ISHLT Registry, 96% of patients have no activity limitations at 2 year follow-up (Fig. 12D.4).

Regardless of preoperative diagnosis, the overall survival following lung transplant is 63% at 3 years and 54% at 5 years (Fig. 13D.3). Data from the ISHLT Registry suggest that recipient age has little impact on survival following pediatric lung transplant. The half-life following lung transplantation is approximately 3.5 years. Patients with pulmonary hypertension have a higher perioperative mortality rate, as do children who are mechanically ventilated pretransplant or undergoing lung retransplantation. It is noteworthy that among pediatric patients undergoing lung transplantation for cystic fibrosis, the one-and three-year actuarial survival rates were 90% and 73%, respectively. These data in patients with cystic fibrosis compare favorably with lung transplantation in adults.

The mortality rate posttransplant is highest during the first year following the transplant procedure. The principal cause of perioperative death is primary graft failure. During the first year p osttransplant death may occur from infection, hemorrhage, and cardiovascular death. Beyond the first year posttransplant, bronchiolitis obliterans (OB) (63%) and infection (22%) are the 2 leading causes of late death. The third leading cause of late death is posttransplant lymphoproliferative disorder (15%).

As in adults, OB is the principal limitation of all long-term survivors of pediatric lung transplantation, affecting up to 40% of three-year survivors. In the absence of infection or rejection, OB is heralded by progressive dyspnea and

Blood Pressure Health

Blood Pressure Health

Your heart pumps blood throughout your body using a network of tubing called arteries and capillaries which return the blood back to your heart via your veins. Blood pressure is the force of the blood pushing against the walls of your arteries as your heart beats.Learn more...

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