Persistent Pulmonary Hypertension: Hannah’s Story
It was 35 weeks, but it seemed faster. Katie recalls the excitement she and her husband, Mike, felt as they prepared to welcome Hannah, their first daughter. They painted the nursery pink and gave her the middle name Ruby to honor Hannah’s great-great grandmother. They even put a black-and-white framed portrait of “Mema” on a shelf.
Having given birth to a son nearly two years earlier, Katie knew what to expect in those first couple of minutes: the cry that kick-starts a baby’s lungs, the cord cutting and the joyous presentation of the baby, capped and wrapped like a burrito.
With Hannah, they got silence.
“We were not prepared to see, hear or experience anything we did that day,” recalls Katie, who gave birth to Hannah via Caesarian section a few weeks early due to preeclampsia, a form of maternal high blood pressure. “From the silence we heard as we waited for any sound to come out of our daughter, to the NICU team rushing in the room and the look on everyone’s face trying to stay strong for us, it was at that moment that I lay on the table sobbing as they rushed my daughter upstairs that our lives changed.”
Suddenly, Katie went from experiencing life day by day to minute by minute as doctors gave the diagnosis. Hannah had been born with severe persistent pulmonary hypertension (PPHN).
“I remember telling this wonderful family that Hannah may not make it through the night,” says Prem Fort, M.D., a board-certified neonatology specialist in the Johns Hopkins All Children’s Maternal, Fetal & Neonatal Institute. “This is one of the most difficult parts of the job that we do as neonatologists. But you always give the family hope and you always keep fighting.”
Members of Hannah’s medical team did everything they could to ensure her survival. Katie kept vigil at neonatal intensive care unit (NICU), taking comfort in the white noise of the high oscillating vent and the caring staff.
“From the minute I was wheeled upstairs to see my daughter, I knew we were in a special place,” she says. “The first face I saw was a sweet lady behind the desk who could see the pain in my heart, and she knew exactly what to say.”
Persistent pulmonary hypertension happens when a newborn’s circulation continues to flow as it did while in the womb. This causes too much blood flow to bypass the baby’s lungs. During pregnancy, babies don’t use their lungs to breathe, so only a small amount of blood is needed to keep tissues healthy. A fetus’ circulation sends most of the blood away from the lungs through connections in the heart and large blood vessels. At birth, that changes when they start to breathe air. The change in pressure closes the pre-birth connections and redirects blood to the lungs to help with the exchange of oxygen and carbon dioxide.
If a newborn has low oxygen levels or trouble breathing, the normal changes might not happen. This causes the baby's circulation to continue to direct blood away from the lungs. Babies whose bodies don’t get enough oxygen face serious long-term health problems. Treatments include medicines, supplemental oxygen, an endotracheal tube, a ventilator and nitric oxide gas to help expand the blood vessels in the baby’s lungs. Doctors also use extracorporeal membrane oxygenation (ECMO) when the baby does not respond to other treatments. In this procedure, blood from the baby’s veins is pumped through an artificial lung. Oxygen is added and carbon dioxide is removed. Then the blood is returned to the baby. This procedure is available only in certain neonatal units, including Johns Hopkins All Children’s Hospital.
In Hannah’s case, she responded to the medicine and the clinical expertise of Fort and the team in the hospital’s NICU, which has a Level IV rating, the highest from the American Academy of Pediatrics. His research focus includes control of breathing and apnea of premature infants.
“We instantly connected with him. He speaks to parents very clearly and on their level,” Katie says. “He even does parent rounds. He asked how we were and encouraged date nights here and there.”
Twenty-six days later, Hannah, who once wasn’t expected to live more than 24 hours, went home to her pink nursery.
But the experience with the neonatal intensive care unit at John’s Hopkins All Children’s Hospital would not be the family’s last.
Three and half years later, on Oct. 2, 2019, baby Joshua arrived. Like Hannah, he didn’t cry.
“I had been having anxiety the weeks leading up to the delivery because I couldn’t get out of my mind what we had gone through with Hannah,” Katie says.
Lying in bed, she looked over and saw two familiar faces: a nurse and physician who were there during Hannah’s time in the NICU. Tears welled up in Katie’s eyes immediately.
“I cried because I was scared to go through this journey again, but then I also cried because I knew we were in the best possible place,” Katie says.
When Katie’s husband, Mike, got to Joshua’s NICU pod, he looked across the room and saw Fort. He ran to the specialist who helped save his daughter.
“There is something very special about him and the connection we have,” Katie says.
“I was standing in the NICU admission pod when I saw Mike walking in,” Fort says. “He’s a big guy and he suddenly ran up to me and hugged me. Nearly toppled me over.
“But the first thing I told him was, ‘This is not Hannah. Don’t you be thinking we are going through that again.’”
Fortunately, Joshua needed only some oxygen support and transition time. He went home a week later.
Joshua smiles and enjoys his infant swing. Big sister Hannah is now an energetic 3-year-old who sings and dances. She loves Butterbean’s Café, Peppa Pig and anything related to the movie Frozen. She adores Joshua and her 5-year-old brother, Frankie.
“She has a huge heart and loves to give hugs,” her mom says.
Her favorite color is pink.