Laura and Bill O’Donnell were very confused. The new bicycle they gave their daughter Shannon remained mostly unused. Shannon would tell them she was “too tired” to ride it. The O’Donnell’s confusion grew to fear when Shannon fainted while playing at a friend’s house. Revived and taken to the emergency room, Shannon underwent a brain scan and an EKG, both of which turned up no explanation for her fainting episode. Shannon fainted several more times after the first episode, and each time doctors could not identify the cause.

Eventually it was a sprained ankle that helped lead to Shannon’s diagnosis. After spraining an ankle at school, Shannon’s pediatrician placed her in a halter monitor and noticed that Shannon had an irregular heartbeat. She and her family were immediately referred to cardiologists at Children’s Hospital Boston.

“One episode of fainting may not be unusual in a child,” said Mary Mullen, MD, assistant in cardiology at Children’s Hospital Boston, and Shannon’s primary caregiver. “But Shannon had two or three fainting episodes and that caused us to think that there might be something seriously wrong with her heart.”

Shannon underwent a three hour test at Children’s after which doctors diagnosed her with pulmonary hypertension, a rare condition in which the blood vessels in the lungs have a much higher pressure than normal, causing shortness of breath and the inability to do normal activities. In Shannon’s case, her pulmonary hypertension led to fainting spells.

“Shannon’s initial condition when she came in to see us was severe and life-threatening,” said Mullen. A cardiac catheterization was performed to get a more precise pressure reading in Shannon’s lungs. This procedure also led to the discovery that the right side of Shannon’s heart was not working to its full capabilities, which further hindered her breathing.

Although a lung transplant was considered, doctors decided to first try drug therapy to reduce Shannon’s pulmonary hypertension. Shannon is currently being treated with Prostacyclin, which is administered into her body through an intravenous tube that is attached to her at all times. The Prostacyclin has been instrumental in lowering the pressure in Shannon’s lungs, but the medical regime associated with the intravenous line is complex.

For people who had no previous medical experience, the daily medical ritual of administering Shannon’s medicine was at first very daunting. “We were trained by physicians every day for an entire week. They wouldn’t let us go home until we had it down pat,” said Bill O’Donnell. The O’Donnell’s now must make sure they have enough medicine on hand for Shannon no matter where they go.

Shannon now wears a backpack at all times that carries her medicine and extra oxygen everywhere she goes. “Halo is the name Shannon gave to her backpack,” said Kim Deckett, RN, a nurse who worked with Shannon while she was in the Children’s Cardiac Intensive Care Unit. “She named it Halo because it’s her angel looking over her as she goes through every minute of every day.”

While the intravenous medicine is currently working for Shannon, a lung transplant is still a possibility for her further down the line. “A lung transplant is something that is often brought up in children with pulmonary hypertension, and it’s a very hard thing to think about,” said Debra Boyer, MD, transplant physician at Children’s. “In one sense, it is a cure. You do replace the lungs that are sick. But a lung transplant, in and of itself, has a lot of risk associated with it, so we always think long and hard about when the right time will be to approach that option. Right now Shannon is doing extremely well with her medication, so we have presently ruled out the transplant.”

“Today, Shannon is doing wonderfully,” added Mullen. “She is full of life and has tremendous energy, and we feel that she has a much improved prognosis. New treatments are being developed both at Children’s and across the world for pulmonary hypertension. We hope that with the use of these therapies, Shannon will never require a lung transplant.”