Stephanie’s Story

The first pediatric lung transplant in the state of Maryland was performed at Johns Hopkins in 1996. In March 1998, Hopkins performed the first living related lobar transplant in the Mid-Atlantic. In early 1997, Hopkins physicians performed a bilateral lobar lung transplant on a 12-year-old with cystic fibrosis, the most common disease for which pediatric lung transplantation is required. Her story follows.

She was born on November 13,1984, but her second birthday is January 3,1997. That's the day a Johns Hopkins team of surgeons performed a double lung transplant on Stephanie Allen, a victim of cystic fibrosis.

Dr. Stephen Yang commented, "The decision to do it was the anguishing part. Stephanie was on a ventilator and would probably have died the next day, so it was a risk."

Diagnosed at age 3, Stephanie was in and out of hospitals until she was 6,

when she had a healthy period of four years, playing soccer and cheerleading like most normal children. By the spring of 1996, however, her Hopkins doctor noted that "...her lungs were shot. She was dying." By December 26, she was on a ventilator.

It was just luck that the lungs were available. Dr. Paul Colombani, pediatric transplantation surgeon, was involved in a donor operation when he learned the adult lung transplant surgeons could not use the lungs. The upper lobes had cysts. Stephanie happened to be the only child on the list, and again, luck prevailed--she was an appropriate match.

Drs. Yang and Colombani prepared to transplant the lower lobes of the adult lungs to Stephanie. The procedure required a heart-lung bypass to oxygenate the blood while one lung was removed at a time and the "new" lobes implanted. The procedure took 12 hours.

After transplantation, children require medications to prevent rejection. These immunosuppressant agents must be taken for the remainder of your child’s life. Most children receive “triple therapy,” a three-drug combination with Cyclosporine or Prograf^(R), Imuran^(R) or CellCept^(R), and prednisone. Each of the medications has some side effects, which will be discussed with you at the time of discharge.

Always administer the medication as directed. If you have questions about the times that the medicines are prescribed or wish to change them, be certain to check with the transplant physician or clinical nurse specialist first. Do not discontinue any medication without being instructed to do so. Contact your physician if you notice any physical or mental changes in your child. Do not try to make up for missed doses unless instructed by your physician. Due to the frequent medicine changes, remember to follow the medication sheet or verbal instructions from the physician and not what is written on the bottle. Ask for prescription refills days before you run out of medicine. Keep all medicine out of the reach of children. Always carry an extra bottle of medicine with you when you travel. Pack the medicine in carry-on luggage, so you will have it in case your luggage is lost.

Sample medication schedules are located at the end of this chapter to help keep track of the times the medications should be administered.