by Nikki Linscott
“In the summer of 2017, we noticed that our usually lively daughter Callie was seemingly less energetic than she used to be. At first, we didn’t think much of it. After all, it could have been anything from the summer activities, to the heat, to the traveling, to the typical behavior of a 4-year-old wanting Mommy to carry her everywhere. But as time went on, her abnormal sluggishness became more and more concerning.
“After our trip to Disney World for her fifth birthday, we paid a visit to the pediatrician for a thorough exam and blood work. When the tests came back, we were told she was coming off of mono and that was why she was so lethargic all summer. For anyone else it may have seemed like a good explanation, but it wasn’t enough for us. Given Callie’s rather colorful medical history involving heart failure and an AVM (arteriovenous malformation) in her brain called vein of Galen, we decided to take her in for a cardiology checkup just to be sure.
“Much to our shock, Callie was immediately hospitalized and diagnosed with severe pulmonary hypertension (PH) on Sept. 9, 2017. She was in right-heart failure. Her heart was so enlarged that it took up the entire left cavity of her chest. Fortunately, we caught it just in time, before she had a crisis, but it has been quite a journey since this discovery.
“Most of us are raised learning that when you get sick, your parents take you to the doctor, the doctor gives you some medicine, and you get better. At the tender age of five, Callie is learning the hard way that is not how her world works. There is no cure for her condition. The medications Callie now takes only slow the disease. She will be medicated every day for the rest of her life until a permanent treatment is found. In the meantime, the arteries that connect her heart and lungs will continue to narrow and constrict, making it harder for her heart to pump blood through her lungs, putting her at continual risk of right sided heart failure.
“Callie’s day-to-day now includes a very aggressive triple-drug therapy. She has to take oral medications three times a day. Another one of her prescriptions, Remodulin, must be delivered via a subcutaneous (sub-q) pump through a catheter installed under her skin 24/7. Because the sub-q site must be kept dry, Callie is restricted from enjoying simple things most kids do without second thought. A lot of forethought and prep work to waterproof her site has to go into a bath or swimming. She can no longer enjoy frolicking in a bubble bath with her big sister nor can she spontaneously jump into the ocean or pool as she once loved.
“Callie also requires oxygen when she sleeps, meaning that when she gets tired at night, she doesn’t have the luxury of just falling asleep anywhere as she has to make sure she has access to her oxygen to make sure she can breathe through the night.
“The list of things Callie can no longer do are difficult to explain to a child. We do our best, but we also try to focus on what she can do now that she could not do just five months ago, before she began her treatment.
“Callie can now climb a flight of stairs in our house without running out of breath or turning blue.
“Callie can now walk down the street to meet her big sister at the bus stop without having to be carried or to ride in a wagon.
“Callie can now attend school for 2.5 hours a day without having to take a three hour nap afterward to regain her energy.
“Callie can now breathe.
“And most importantly, she can now live.
“These are daily things that we can all do without a thought and take for granted every day. They are things that most five-year olds can and should do effortlessly.
“Callie’s enduring spirit in the face of her reality is inspiring to all around her, but she could not have come this far alone. One silver lining of our journey into the world of PH is our newly found connection with the amazing people in the PH community. We have met remarkable families of other children with PH, extraordinary doctors, dedicated nurses, loving caretakers, and wonderful organizations like the Pulmonary Hypertension Association (PHA).
“We are proud to have raised more than $8,000 in our first fundraiser for PHA – the annual O2 Breathe Walk in Baltimore – this year. We will not stop fighting for a cure! In the meantime, we will continue cherish every moment we have because, as Callie has taught us, you never know what tomorrow may bring.”