Our Journeys

Caregiver Story

Jayna Wall

"He tried to explain everything to us but we were both in shock...this just had to be a nightmare and we would wake up right?"

Jackson, our little 14 month old baby boy, has been diagnosed with idiopathic pulmonary arterial hypertension (IPAH).

Up until this point, Jackson has seemed perfectly healthy. We had no idea or indications that anything like this was occurring in his little body. A few weeks ago he had a few bumps on his face and neck that we were just watching. I didn't want to take him to the doctor for just a few little bumps, but when some showed up around his eye, I finally decided to call them. They suggested that I bring him in. I took him in and the doctor examined him and he said that the bumps were probably just bug bites.

Jackson WallDuring his examination, however, he heard a heart murmur. He looked back through Jackson's chart and he did not see where anyone else had heard a murmur before. When he was first born they did hear a murmur, but the doctor said that it was not unusual for newborns to have a murmur since their hearts are still growing. No one had mentioned a murmur to me since he was born up until two weeks ago when they heard this one. The doctor suggested that we take him in for an echocardiogram. We finally got the appointment set up for this past Tuesday (August 21, 2012).

The pediatrician had told me that they wouldn't give us the results for four to five days, so I just thought I was taking him in for the echocardiogram and then we would find the results out later. I also thought, based on the apparent health of Jackson, that they were just going to tell me that it was a normal or innocent heart murmur and was nothing to worry about. My husband Matt was at work in Atlanta while Jackson and I went to the appointment since we didn't think we would be getting the results. Unfortunately, we were wrong. The cardiologist looked at the echo and came in to talk to me. The first words out of his mouth were "the prognosis is not good" and I stopped him and told him that I needed to call Matt. Once I got Matt on speakerphone he continued to tell us that he thought Jackson had pulmonary hypertension (PH) and that he could not see anything on the echo which could be causing the PH, so it was probably IPAH, it has no cure and it can be fatal. He tried to explain everything to us but we were both in shock...this just had to be a nightmare and we would wake up right?

Jackson WallMatt immediately jumped on a plane back home. The doctor called and told us to be at Duke at 10 am the next morning to be admitted and the next step would be a heart catheterization. Once Matt arrived home we both began to pray. Off and on all night long we prayed for our sweet little boy.

We arrived at Duke the next morning and finally got a room around 1pm that afternoon. They then did a lung scan which came back as clear, a chest x-ray which was also clear and they drew some blood and did a few other tests. We continued to pray and to prepare ourselves for the heart catheterization the next morning. During this time we discovered that Matt had an ironic connection through his job in Atlanta. Someone he knows in Atlanta is friends with the manager of clinical operations at the Cardiac Step-down unit of the Children's Healthcare of Atlanta. The Children's Healthcare of Atlanta is, from our understanding, one of two centers in the southeast with a pediatric pulmonary hypertension program.

On Thursday they finally came to take Jackson for the heart catheterization. We went with him to the room where they gave him the anesthesia through his IV and went to sleep. Walking out of that room was so hard with our little baby lying on that table. We had been told that they would go in and check everything out and then they would try to administer nitric oxide to see if it would help to dilate the arteries. We were hoping and praying (along with a lot of other people) that it would work. They updated us several times during the procedure and then told us that it was over and the doctor would be out to talk to us shortly. When the doctor finally came out and said we needed to find a private room, we knew it was not good news. We sat there in horror as he told us that Jackson's arteries in his lungs were shriveled up like prunes, that they did find a minor defect in his heart but that it wasn't something that could cause the PH, and that medications would probably not work since the nitric oxide didn't work. One of the doctors then came in to get the status of the procedure and after hearing it said that he thought a heart and lung transplant would pretty much be our only option. He did admit that they aren't experts in IPAH and said that he was going to call the leading doctor in pediatric PH, Dr. Dunbar Ivy, out of Denver, CO and see if they could come up with a plan.

"Dr. Terry Fortin, at Duke stopped by to see if she could answer any questions. She was so positive and told us stories of young patients that had PH/IPAH and were still alive and doing good today. We were finally starting to feel as if the doctors were on board and fighting with us."

Several hours later the doctor finally stopped by and said that he had had a good long conversation with Dr. Ivy and that they weren't just going to throw their hands up and give up. There were several different medications that we could try and we could see what happened. As soon as he left the adult PH specialist, Dr. Terry Fortin, at Duke stopped by to see if she could answer any questions. We asked her about the nitric oxide and learned that it really only works in a small number of patients (7-8%) and that a lot of patients still respond well to the drugs even if they didn't respond to the nitric oxide. She was so positive and told us stories of young patients that had PH/IPAH and were still alive and doing good today. We were finally starting to feel as if the doctors were on board and fighting with us.

"We thank God every day for bug bites and for catching this early. Hopefully the medicines will work fine and he'll live a long and happy life. Please consider PH."

Over the next few days Jackson was given Sildenafil progressively up to the full dosage and then introduced Bosentan. We were moved to pediatric cardiac intensive care unit (PCICU) in order to put in an arterial line to monitor his blood pressure. Everyone wanted to be extra cautious to make sure his little body didn't react negatively to the medications. We were both really upset and scared. At that time I think we were both feeling the weight of the world on our shoulders thinking about Jackson, treatments, our stay in the hospital, etc. When we got over there, I think our pediatric cardiologist, Dr. Brenda Armstrong, saw we were visibly upset and were getting scared about putting him in PCICU. She sat down with us and shared a story about her own experience. Her son had ruptured his appendix while running track and she almost lost him. While at the hospital she kept thinking how she wasn’t going to get him back, and how she could have prevented it. She said, “So I know what you’re feeling. I know how scared you are right now, and I’m telling you that we have the best caring for your child.” She was very comforting and gave us faith that the doctors here at Duke, and everyone else consulting on his treatment, will get us through this.

He has since been on full dosages of Sildenafil and Bosentan and has had no reaction to the medications. We are going to have an echocardiogram in a few weeks to see of the blood pressures in this heart have gone down any. We thank God every day for bug bites and for catching this early. Hopefully the medicines will work fine and he'll live a long and happy life. Please consider PH.

The information provided on the PHA website is provided for general information only. It is not intended as legal, medical or other professional advice, and should not be relied upon as a substitute for consultations with qualified professionals who are familiar with your individual needs.

© 2014 Pulmonary Hypertension Association. All Rights Reserved.

801 Roeder Road, Ste. 1000, Silver Spring, MD 20910   Patient-to-Patient Support Line: 1-800-748-7274
Webmaster@PHAssociation.org
    Privacy Policy   Provide Feedback & Report Bugs

NORD

The National Organization for Rare Disorders (NORD) awarded PHA the Abbey S. Meyers Leadership Award in 2012 for outstanding service to PHA members in advocacy, education and other key areas.