BENEFITING: Pulmonary Hypertension Association Inc
EVENT DATE: Oct 18, 2015
Here is Jenn's story:
Tyler’s Story On May 4, 2014, Tyler was a healthy, normal, active little boy who was sports-obsessed and fighting naps and bed time. It was two months after his fourth birthday, and the very next day, everything changed. He came home from Pre-K and told me that he couldn’t “find” his breathing. On May 8, 2014, after a trip to the pediatric emergency room and three-day hospital stay, Tyler was diagnosed with Pulmonary Arterial Hypertension (PAH). Looking back now, I realize how lucky we are. Many people who suffer from PAH take years to get to a correct diagnosis. Tyler had a respiratory infection that seemed to get bad very quickly and we thought he had pneumonia and would quickly recover. We were waiting to hear we were being discharged when a group of doctors walked into his hospital room and dropped this bomb. I remember thinking, “Ok, so what is the cure and how do we correct this?” But they never gave me that. What they gave us were instructions on how to get oxygen services at home and set up a visiting nurse. They gave us a follow-up appointment with a Pediatric Cardiologist who works on a team that focuses on rare diseases. There was, and still is, no cure for PAH. Tyler was on oxygen 24/7 for about three weeks at home, and then once his virus was gone, he was able to cut down to oxygen just at nap time and overnight. If you’ve ever met a 4-year old, you would be shocked at how well Tyler adapted. He played golf in the front yard strapped to an oxygen tank. He wanted to go on wagon rides so his oxygen tank could ride along with him. It didn’t slow him down. We had to find a nanny to watch him at home with one day’s notice and somehow everything fell into place for him. He adjusted well to being home with a new face instead of playing with all his buddies at day care. We researched and went through specialist appointments and really it probably took us about eight months to fully understand what was happening with our amazing little boy. PAH is a disease that affects blood vessels in the lungs. They are small and constricted, and because of this they carry very little oxygen to the heart. The heart’s job is to pump oxygen-rich blood to all parts of the body, so to make up for this, the heart becomes enlarged and strained from pumping the excess required to help keep him active. Essentially, Tyler’s heart is always beating as if he were running a marathon. The disease is difficult to treat because researchers still don’t understand why the blood vessels are diseased. Along with the oxygen, there came the medications. First a three times/day option that gave him headaches and worsened the nosebleeds he was getting from the oxygen. Next (and through being connected with a true PH Specialist) we were able to switch to a once-daily medication that the insurance company had originally declined to cover. Slowly through the months we started to realize what PAH was and how it would affect him long term. PAH often comes as a side effect of another disease such as Lupus or Schlederma. For Tyler, it is Idiopathic – meaning they have no idea why he has it. As we questioned the long-term effects of the three medicines he was taking we got answers like, “If you want Tyler to live to be 28, these medications are his only chance…” and “If Tyler were born in 2001, he wouldn’t be here today.” Hearing these things and looking at our little boy who by all outward appearances looks totally healthy was extremely difficult. No parent ever wants to think of their child passing away before them. There are so many ways in which we are lucky but as we look into the future, we have to do everything in our power to change his quality of life and life expectancy. We are trying new treatments that have difficult side effects all in the hopes that we can get his heart pressures into the normal range. The longer they are in the normal range, the longer he can lead a normal life. PAH gets progressively worse over time and eventually leads to heart failure in most patients. We will not take this lying down. We will change his outcomes. We hope to avoid the next stage of treatments as long as possible since they will change his day-to-day life and ability to live life to the fullest. There need to be other treatment options. There need to be other resources for dealing with this disease. There has to be a cure. My plea to you is that you can help us get there.