Posted on Dec 18,2017
With tiny fingers resembling grains of rice and lungs not yet strong enough to draw in the oxygen around him, Jauhe’y (pronounced jay-HEE) entered the world quite by surprise at 25 weeks during a family vacation hours from home.
His mom, Saderrika, who goes by Erica, had scheduled a standard ultrasound while visiting St. Petersburg from Ocala because she wanted to know the baby’s gender. She also wanted to monitor her preeclampsia, a pregnancy complication characterized by high blood pressure. It was good intuition.
Still Erica did not expect the concern on the nurses’ faces as they observed the baby on the monitor. For a 25-week-old fetus, he looked 18 weeks. Erica’s preeclampsia was preventing the baby from growing, and doctors referred Erica to specialists at Johns Hopkins All Children’s Hospital. The maternal fetal medicine team immediately began talking about delivering the baby.
“They told my wife and I that there was a better chance of survival if we had the baby right away,” Erica recalls, shaking her head. Though she and Nequella, Erica’s wife, already have twins from Nequella’s first marriage, and they adopted a little boy, this was Erica’s first birth.
And she was frightened.
“We wanted what was best for the baby at any cost,” she explains. A cesarean section was performed at Bayfront’s Baby Place and the infant was immediately transferred to Johns Hopkins All Children’s neonatal intensive care unit (NICU) where he faced quite a fight to survive and learn to breathe on his own.
Fortunately, Jauhe’y, who was born at less than 1 pound, had one determined neonatologist on his side, and she just happened to have created a pulmonary task force that would make the lung issues he was fighting easier to handle.
Improving Long-term Outcomes for Infants
“Everything neonatologists do in the beginning of the baby’s life has a strong impact on their future. It isn’t just about surviving. We want these babies to thrive with great outcomes,” explains Joana Machry, M.D., Jauhe’y’s neonatologist. “Jauhe’y is a miracle baby that is truly loved. His parents were here for him every moment. He faced multiple issues. We knew we needed to build up his weight and strength, but his immediate issue was breathing.”
Machry had spent more than six years working on ways to improve respiratory outcomes for extremely prematurely born neonates. The goal is to avoid long-term lung damage and the diagnosis of bronchopulmonary dysplasia (BPD)—chronic lung disease—which is a long-term consequence of prolonged use of oxygen and mechanical ventilation. Babies who need prolonged respiratory support during early life are also at high risk for other long-term complications, such as neurodevelopmental impairment and frequent hospitalizations.
“Most of the time with the tiniest babies, like Jauhe’y, we must start supporting their lives with a breathing machine (ventilator),” Machry explains. “In the past, it was thought that extremely premature babies could not survive or thrive without the help of a ventilator, however at the same time these mechanical breaths are lifesaving, they are very invasive devices that come with negative consequences and side effects. An infant who is intubated on the ventilator can’t bottle-feed, needs more frequent blood draws and sedation, intravenous access and can suffer lung damage from the artificial ventilation and oxygen toxicity. Fortunately, science has evolved and doctors have realized that these babies can survive and thrive on less invasive devices for breathing support and that can lead to less long-term side effects with better long-term respiratory outcomes. The problem is that knowing what is best from the published medical literature does not always translate into change in clinical practice. A great deal of education for a change in culture is necessary to convince a large and experienced medical team that a less invasive approach could be as effective and have better long-term results what they are used to.”
Machry’s “NICU Pulmonary Task Force” set about re-educating the staff and persuading them that evidence has proven that non-invasive nasal ventilation with a continuous positive airway pressure (CPAP) device, which rests on top of the baby’s nose as opposed to tubing down the throat, could be as effective and less invasive.
In 2016, after years of team effort for the right age and patient size, the best nasal equipment/machines and staff education strategy, an evidence-based clinical practice guideline for the use of non-invasive nasal ventilation procedure was implemented for the most vulnerable patients in the NICU. Machry’s team closely monitored staff guideline compliance and patient safety for a smooth transition.
Outcomes were positive but early findings indicated that CPAP use was rough on the delicate infant skin and skin damage was a side effect of the new strategy. This important setback did not discourage Machry, who already was beginning to see the benefits of removing babies from ventilators earlier. She set about solving the problem with her method winning a poster award at the Johns Hopkins All Children’s Research Symposium in October. Upon further study, the results will eventually be published so other physicians can learn from Machry’s “Pulmonary Task Force” team success.
“We created a protective skin barrier and educated the nurses on our new placement procedure designed to protect the delicate skin as well as frequent monitoring for skin redness,” Machry says. “We have had a 100 percent success rate with that so far and the staff really took it seriously taking pride for skin integrity. We are very pleased because it allowed us to keep utilizing the CPAP.”
Awards and Rewards
“I am so pleased that Dr. Parimi saw the potential of this new breathing support strategy and allowed us to go forward,” she concludes, referring to Prahbu Parimi, M.D., director of the Johns Hopkins All Children’s Maternal, Fetal & Neonatal Institute and chief of the division of neonatology. Parimi was so impressed that he nominated Machry’s Pulmonary Task Force for one of the medical staff’s highest honors: a Clinical Excellence Award. It won.
“Of course, I am so proud of our task force, which has fought for years to make this method a reality, but the true reward for me as a neonatologist, is improving the outcomes and the lives of our patients. When we can prevent an infant from developing BPD, we are saving them from a lifetime of health issues.”
As for Jauhe’y, today a healthy 9-month-old, he successfully avoided BPD with the help of CPAP, which replaced his ventilator after several months. But it meant 45 days of sleeping in the NICU next to their baby’s incubator for Erica and Nequella until they felt confident enough to move to a Ronald McDonald House on campus for the remainder of Jauhe’y’s five months in the hospital. “We used up all of our savings in the first few months and Nequella had to give up her job in Ocala in order to be here for us,” Erica recalls.
“Dr. Machry developed a special chin strap to hold the CPAP in place on his tiny head,” Erica recalls. “She’s awesome. She was very upfront with us the entire time. She told us what to expect. She actually set goals for Jauhe’y, which we loved. She came in every day and told us what would be happening that day. And she spoke our language, not in doctor’s terms. She explained to us the more hands-on we were, the better he would do. She was right. We used all those months in the hospital to learn as much as we could. We took classes, we got to know the nurses. It was like a family and we knew they were doing everything they could to make sure Jauhe’y never developed lung disease.”
Jauhe’y did face a successful minimally invasive heart surgery at just a few weeks old to replace a valve, and had several eye surgeries to correct nerves that were beginning to detach because of the oxygen. These days, at a whopping 14.3 pounds, Jauhe’y is progressing beautifully. Machry plans to follow him throughout his childhood. And the family, now happily back home in Ocala, is more than happy to drive down to Johns Hopkins All Children’s for checkups every few months.
“Dr. Machry is his doctor. We don’t want anyone else taking care of him,” Nequella adds. “We owe her a lot. We were away from home and her team did everything they could to make us feel at home in the hospital. They made sure we had access to all of the resources we needed so we could keep going through all of this. We never felt alone. Jauhe’y definitely made his mark on everyone.”
He also made his mark on the pulmonary task force, validating that CPAP can successfully provide oxygen to infants much less invasively than ventilators, leading to better outcomes and happier babies.
Visit HopkinsAllChildrens.org/Neonatology to learn more about the Johns Hopkins All Children’s Maternal, Fetal & Neonatal Institute.