Pathlight Summer 2016 - (Page 18)

CONNECTING FAMILY PHOCUS Mother and Daughter Reflect on Daughter's Journey with PPHN By Kendall Hunt, a PH Patient and Her Mother, Ann Hunt Editor's Note: Kendall, age 15, and her mother reflect on Kendall's journey as a young woman born with persistent pulmonary hypertension of the newborn (PPHN). The following is a dialogue between the two. Kendall's words are italicized. "She's blue." The nurse's words echoed through the delivery room, sending a shudder of panic and fear through my body. My baby girl was not breathing, and I could only watch helplessly as the medical team rushed her away. I had a normal, healthy pregnancy without any complications. When I went into my regular prenatal appointment at 38 weeks, I was not expecting any surprises, stress or anxiety. Because we were nearing delivery time, and I was measuring large, my obstetrician decided to send me for an ultrasound to get an estimated fetal weight. After the ultrasound, the sonographer told me, "In my 20 years of experience, I have never seen a femur that long nor an abdomen that large. I expect this baby to be about 11 pounds, and I think we should talk to your doctor about getting this baby out as soon as possible!" I have always been punctual, almost to a fault. I hate to be late to anything. So, I guess it's no surprise I was born early. As for being a big baby, well, blame my dad. He's 6' 6" tall with size 18 shoes! This being my first child, when I learned that Kendall was too big and would need to be born immediately, I began to panic. I thought I had two more weeks, and I had plans for my mom to fly up from Texas to be at the birth. My blood pressure rose as quickly as my anxiety, and I developed preeclampsia (high blood pressure during pregnancy, sometimes with fluid retention), further complicating matters. The next morning, Kendall came into the world via emergency C-section, weighing a whopping 10 pounds, 6 ounces. For some reason, I love to tell the "Super Baby" tale. I was born with a disease called persistent pulmonary hypertension of the newborn (PPHN), a condition where my lungs did not "open" properly, and I could not breathe. In order to get oxygen into my lungs, the nurses put me on a respirator, a machine that breathes for you. But they weren't used to having a 10.5-pound baby in the NICU. I tore the IV out of my arm. Evidently, I had crazy baby strength, and I was fiercely independent, refusing to open up my airways without a fight, earning me the nickname of "super baby." Aside from ripping IVs out of my arms, there were many more complications with my treatment. After all, a hospital can be a dangerous place to be for a newborn with super baby strength. First, because of the unnatural pressures of the respirator, I had a collapsed lung and air filled my stomach, so 18 PATHLIGHT // SUMMER 2016 Kendall as a baby they had to cut open my chest and insert a tube in my side to relieve the pressure. Then, I got a blood clot in my umbilical line. Time and again, I was in critical condition. My parents still tell me about the nerve-racking memories of me fighting to survive for two weeks. But being a super baby, they should have known I'd pull though. Our pediatrician told us that if Kendall had been born about 10 or 15 years earlier, she most likely would not have survived. Neonatal medicine has made so much progress in the last 20 years. PPHN is still a life-threatening condition, but with the proper care, it is increasingly more survivable. Kendall was treated by caring and knowledgeable nurses and doctors, and I credit them with saving her life. The doctors explained to me that in utero, fetuses receive oxygen through the umbilical cord, so blood is sent away from the lungs to other organs through a blood vessel called the ductus arteriosus. Normally, when a baby is born and takes its first breath, blood flows to the lungs, and the ductus arteriosus will then permanently close. In babies with PPHN, the ductus arteriosus remains open causing blood to be directed away from the lungs. We still don't know the cause of Kendall's PPHN. It took her two weeks of intense treatment to finally relax and allow the ductus to close. Doctors told us that Kendall might have asthma and difficulty breathing as she grew up. They also feared the noise from the high frequency ventilator could cause hearing loss. And in the early years, we saw a pattern of breathing problems and susceptibility to severe colds and pneumonia. She also had extremely sensitive hearing and could not tolerate loud noises. We feared the doctors were right, and she would forever be limited in physical activity. As I grew older, asthma symptoms developed. I also discovered I was allergic to cats. To my disappointment, whenever I

Table of Contents for the Digital Edition of Pathlight Summer 2016

Message from the Chair
Message from the SLC Chair
Quick Takes
PHenomenal Lives
Family PHocus
A Day in the Life of a PH Physician
Research Corner
GO PHAR Research
Ask the Expert: Post-PE Monitoring
End-of-Life Conversations: Tough Questions & Important Answers
Lantos Highlight
Conference & Leadership Changes
Chapter News
Advocacy PHact or PHiction? Test Your Knowledge of PH Advocacy
Calendar of Events
Question of the Quarter:

Pathlight Summer 2016