A PHighting Spirit

We welcome Jackson’s mom as a guest blogger today to share her story.

Six year old Jackson was diagnosed in August of 2012 with Pulmonary Hypertension (PH). PH is a chronic, incurable, progressive, and life threatening lung disease that creates narrowing of the blood vessels in the lungs, which causes additional strain on the right side of the heart. If left untreated, PH can lead to failure of the right side of the heart. Within 48 hours of Jackson being diagnosed, we were told he wouldn’t survive the disease and that he would eventually need a lung or a heart and lung transplant.

PH is often misdiagnosed as less severe conditions, such as asthma, due to the hidden/similar symptoms of PH. Because it is often misdiagnosed, the disease continues to progress, and often patients are not diagnosed until it is too late. Primary Pulmonary Hypertension (PPH) or Idiopathic Pulmonary Arterial Hypertension (IPAH; what Jackson has) is so rare in children that only 1 in 1,000,000 are diagnosed with the disease. Because of it being so rare, even doctors do not understand the disease and treatments in its
entirety.

In the following days Jackson was started on two different oral medications to try to dilate the arteries in his lungs. Over the course of several months we saw improvements in his heart function, but the pressure in his heart remained very high. In January of 2013, after getting a second opinion from another pediatric PH specialist, we decided to put Jackson on the strongest medication available for PH. This medication is delivered subcutaneously, so he has to be connected to a pump 24/7.

Since he was diagnosed, Jackson’s heart function has improved from “we’re not sure if his right ventricle is functioning” to “his right heart function looks almost normal”. And his BNP (a hormone made by the heart, high levels of which can indicate that the heart is under stress or in failure) has dropped from the 1600s to the 100s. We still have a long way to go, but Jackson has made a lot of progress over five years since his diagnosis. Despite all he has been through, Jackson is a sweet, fun-loving, smart little boy with a PHighting spirit. We thank God for his early diagnosis. We have great hope that there will one day be a cure for PH, and that our little boy will live a long and happy life.

Jackson’s cake was provided by Sugar Angel April Murray with Main Street Cake in Burlington, North Carolina. Thank you April for all of your hard work and dedication to Icing Smiles. We look forward to seeing more “smiles” from you in the future!