By Kelly Wiegele
“For most of her early life, we thought Riley was just a normal and healthy child. She would have the run of the mill viruses that seemed to hit her a little harder than most kids and a couple of hospitalizations, but we didn’t think anything of it. She ran and played just like any other kid would.
At 15 months old, the doctor heard a heart murmur when she had a fever at one of her sick visits. We were referred to a pediatric cardiologist who told us her pulmonary valve was thicker than it should be, and she would grow out of it. We went on with life and had follow ups every six months.
During one of her follow up appointments when she was 3 years old, she just wouldn’t let them do anything, so we were told that if we see any shortness of breath to call. Over the summer of 2014, she spent a lot of time in the pool as usual. This time I noticed her getting short of breath or gasping for air when she would swim. We didn’t really think a lot of it because she was a very dramatic little girl! She never had any other issues.
In the fall of 2014, Riley started getting short of breath when she would run which was very unlike her, so I made an appointment with the cardiologist, since it was almost the end of the year. Little did we know, that was the appointment that would change our lives. The doctor didn’t come back quickly like usual and I heard her on the phone with Cincinnati Children’s Hospital, mentioning Riley. My heart immediately sank. It was a week before Christmas and we were due to go to Cincinnati Children’s on Dec. 21, 2014 with our new diagnosis of idiopathic pulmonary arterial hypertension (PAH). We had no idea what it was, and we were terrified. Riley had her first heart catheterization and started on two medications. A few months later they discovered that she was getting worse and started subcutaneous Remodulin. It was so hard because shecould no longer take a normal bath or swim without a special suit. She had to be careful and quit doing a lot of the activities she loved. She had a lot of pain with it and was pretty much terrified to change it. A year later she started on IV Remodulin through a permanent IV line in her chest. This medication greatly improved her heart and lungs! It came with its worries such as IV line infections or getting pulled out, but it allowed her pressures to significantly decrease and switch to oral medications. Riley had a rough go at it during the transition to oral medication and it lasted several months, but now she is thriving more than ever and remaining stable!
During the whole process, we learned that Riley’s PH is caused by Hereditary Hemorrhagic Telangiectasia (HHT). With this diagnosis, the genetic mutation starts with her and didn’t come from either parent and that’s pretty rare! Things started to make much more sense once this second diagnosis came into play.
Riley has been an amazing PHighter during it all. She doesn’t let it get her down and always has a smile on her face! She has dreams of starting a non-profit to help families of children in the hospital and also send care packages to kids who are in the hospital. PH has been tough on the whole family, but we always try to look to the positives when we can. We have made lifelong friends and she has beat so many odds that we can’t help but be so thankful!”