If you could meet her, she’s the smiliest, happiest child you’d ever meet. She’d wave hello and blow kisses goodbye. She is our smiley Kylie-bear. She doesn’t look sick, but inside her, she has a rare, horrible, and terminal disease called Pulmonary Hypertension (PH). As November is national PH awareness month, we’re honored to share Kylie’s story with you.
Her journey to diagnosis started very early with a miraculous and kind of rare find. When Suzanne was pregnant and in her third trimester, the doctor told us Kylie’s heart was pumping akin to a jellyfish instead of a strong in and out pulse. After that visit, we sent out an email to 300 people with a cry called #PlanA that she would be ok. Our prayers were answered: she came into this world full of joy.
But the pressures in her lungs were too high, and they weren’t lowering as they should. The doctors spent the next 8 months ruling out heart and lung disease, structural abnormalities, and genetic problems. After undergoing a heart catheterization and a CAT scan, she was diagnosed with Idiopathic Pulmonary Arterial Hypertension: for an unknown reason, the arteries that run blood from her heart into her lungs for oxygen are narrowed. This causes extremely high blood pressure in those arteries and causes her heart to work harder to push the blood through. It’s like she’s running a marathon all the time. The end result of this disease is right heart failure and death because the heart has pumped so hard for so long that it eventually gives out. Thankfully, there are 14 approved drugs that force the arteries open to ease the strain on the heart, but like most drugs, nothing is FDA-approved for peds. Doctors use them anyway, hoping for few side effects in their tiny little bodies. And the drugs do not cure the disease, they only slow its progression. If pulmonary hypertension is left untreated, life expectancy can be as short as 2.8 years. The journey to find the right treatment is full of trial and error and uncertainty. We knew we needed help.
And seven months after the diagnosis, we found it in The Pulmonary Hypertension Association (PHA), which became our PHamily. We met our first other PH patient. We went to our first PH conference and met other PH kids and their families. We found hope and support. We became fighters for our PHighter. In January, we will be making our first trip to see a pediatric PH specialist and we can’t wait! Currently, there are only 7 hospitals in the US that have pediatric pulmonary specialists; seven, for all children under the age of 18. In concert with these facilities, the PHA promotes awareness among physicians, which in the case of a rare disease is key to promoting to early detection. They also help fund research in hope of someday finding a cure.
I love imagery. The ribbon color for PH is periwinkle because it is the color the lips turn when deprived of oxygen. They call PH patients medical zebras. It comes from an analogy that medical students are taught: “When you hear the sound of hooves, think horses, not zebras.” And most of the time diagnoses are common, like horses, but there are those one in a million -- the zebras. And the community that fights this disease has taken that those facts that can overwhelm us with despair and turn them into beautiful things like periwinkle nail polish and blue lipstick selfies. They wear zebra prints with pride.
The imagery I love to use with Kylie is my little panda bear. Still black and white like a zebra, but truly rare. Chubby cheeks and adorably cute and playful. Having a child with a disability, chronic illness, special needs, terminal disease, whatever you want to call it, has created a wellspring of hope inside of us. This disease has changed our family. We have allowed this disease to transform us into vessels of hope and love. We have become more compassionate and empathetic. Life is a gift. She is a gift to us. We been entrusted with the privilege of showing her what it looks like to live an alive life full of adventure, courage, risk, joy and dancing. She is a wellspring of life.
Moreover, taking this journey together has made us realize that we are all diseased. There are things inside of us that are so broken and sick that we are often more dead than alive. Anger will rot our souls. Sadness will swallow us whole. We have to hope. Hope empowers us; it creates beautiful things out of brokenness. Yes, we’ve been in a hospital more than we’ve wanted to. And we know harder days are ahead for her. That her future may become more complicated than taking her meds like you would take cough syrup. She may have an IV feeding meds into her bloodstream, or a subcutaneous pump. She will probably have days where she doesn’t have energy to get out of bed or walk from the car to school. It would be so easy to be angry or judgmental about it, but that doesn’t help her or us.
What helps is hope and love. We have discovered life is not meant to be lived alone. We have found shoulders to cry on, hands to help carry us, hearts that beat with joy and life enough for dozens.
So you may never meet Kylie, but allow knowing her story start to change you. That you can become more alive and full of hope and love. That you may be aware of your own brokenness and the brokenness of those around you; those in pain and struggling. Because disease wants us in bed. Hope pulls us up off our knees. Disease wants us to succumb to despair, but hope presses us to explore the possibilities. Disease wants us to be lonely. Hope drives us to community. Disease wants us to be selfish and pity ourselves. Hope calls us to be generous.
Our good friend Jennifer Hoffman is helping us raise awareness and funds for Pulmonary Hypertension! She sells LuLaRoe clothing (link below) and is donating a portion of every online sale (and the sales from any of her "Open Shopping House" in the LuLaRoom) to the Pulmonary Hypertension Association in the hopes that one day soon Kylie-Bear and her fellow PHighters will have a brighter outlook and shine here on earth longer!! We are so grateful for Jenn and her friendship. For her generosity in organizing and partnering with us for hope in Kylie’s future. Hope for a cure. Hope for life. Hope for a more beautiful and empowered humanity.
With love,
Ethan, Suzanne, Morgan, Sean and Kylie
Check out Jenn Hoffman's LuLaRoe page and click Shop Now!
A lot of our hope has been found at Mosaic; a community that follows Jesus and His teachings to live by faith, to be known by love, and be to a voice of hope.
A lot of our hope has been found at Mosaic; a community that follows Jesus and His teachings to live by faith, to be known by love, and be to a voice of hope.
