Long-term Issues

Outcomes

Following lung transplantation your child can expect to lead a happy and productive life. As the medications for treating rejection continue to improve the survival of children who have undergone lung transplantation continues to improve. Generally 70-80% of the children who undergo lung transplantation are alive after1 year and approximately 50% are alive 5 years post-operatively. Current survival data can be obtained via the Internet at the UNOS or ISHLT sites listed in the back of this handbook.

Complications

Obiliterative Bronchiolitis (OB) or Bronchiolitis Obliterans (BO)

Obiliterative bronchiolitis is caused by chronic or recurrent episodes of rejection. OB results from destruction of the small airways. OB is difficult to treat, however it sometimes responds to an increase or change in immunosuppressant medications. The exact cause of OB is unknown and it is the leading cause of death in lung transplant patients after the first year post-operatively. Symptoms of OB include shortness of breath, cough, increased oxygen requirements and diminished pulmonary function values.

Post-transplant Lymphoproliferative Disorders (PTLD)

Lymphocytes are white blood cells that are part of your immune system. They play a key role in attacking foreign invaders such as bacteria and viruses. There are two types of  lymphocytes called B-cells and T-cells. (For more detail see the section labeled the Immune System.) The immunosuppressant medications (Cyclosporine or Prograf) your child takes to prevent rejection work by suppressing the activity of the T-cells. The medications are not specific to the lungs, therefore, they suppress the entire immune system. Normal regulation of B-cell production is also altered when a child takes immunosuppressant medications. Since normal control over the B-cells is lost they can multiply rapidly.

When a person has a virus, the normal immune response is to produce more B-cells to fight the infection. The Epstein-Barr Virus (EBV) is a virus that directly attacks B-cells and causes an overproduction of B-cells. In a healthy person the EBV infection is called mononucleosis, and the patient recovers from the virus by controlling B-cell production. In a transplant patient the normal control over infected B-cells is compromised by the immunosuppression medications, allowing B-cells to grow unchecked. This is lymphoproliferative disorder. 

When the donor and recipient are an EBV mismatch (in other words, your child is negative for the virus and the donor is positive) your child is at greater risk of developing PTLD. In this situation your child will be given an antiviral drug called acyclovir to prevent PTLD. If your child develops PLTD the amount of immunosuppression medication is reduced to allow the immune system to recover and fight the overproduction of B-cells. However, decreasing your child's immunosuppression increases the risk of rejection. The usual signs and symptoms of EBV infection include fever, malaise, lymph node enlargement and liver and spleen enlargement.