Scarlett’s Story - Congenital Diaphragmatic Hernia (CDH)

In October 2019, my husband, Patrick, and I found out we were expecting our first child, a baby girl, to be named Scarlett. We were beyond excited. However, at our 16-week ultrasound, we received devastating news. We were told our baby had a congenital diaphragmatic hernia (CDH). The left side of Scarlett’s diaphragm had failed to form, and her stomach, spleen, intestines, bowel, and the entire left lobe of her liver were all in her chest. Her heart was pushed all the way over against her right chest wall. Her left lung was so small it was nearly nonexistent, and her right lung was underdeveloped as well. At that first appointment, we were told there was less than a 20% chance that she would survive after delivery. We were absolutely heartbroken, but we had faith that our little girl was a fighter.

Throughout my pregnancy, Scarlett was monitored closely. I had 27 ultrasounds, twice-weekly non-stress tests, 2 fetal MRIs, and a fetal echocardiogram. We met with numerous specialists. They explained that CDH has a spectrum of severity, and, unfortunately, the testing showed that Scarlett was on the severe end of that spectrum. After consulting with several hospitals, we ultimately decided to relocate from our home in Knoxville, TN, to Cincinnati, OH.

Scarlett was delivered at Cincinnati Children’s Hospital via a planned C-section with more than 30 doctors and nurses in the room to provide her with immediate care. Scarlett was intubated and placed on a ventilator before she was even fully removed from my uterus (it’s called an EXIT to airway procedure for those who are curious). Scarlett went into respiratory failure when she was only 18 hours old and was placed on a life support machine called ECMO. Initially, the doctors were hopeful that she would only be on ECMO for a short time. We were told it would likely be less than a week. Unfortunately, that wasn’t the case. Scarlett’s health continued to decline, and she had her CDH repair surgery on day 11 while still on ECMO. Even after her repair, she showed no signs of improvement, she had developed severe pulmonary hypertension, and sadly, she was not responding to treatment. After 6 weeks on ECMO and 3 failed clamp trials, the doctors in Cincinnati told us there was nothing else they could do for Scarlett, and it was unlikely that she would ever be strong enough to survive off of ECMO. I can’t explain the pain we felt hearing those words. The thought of losing our little girl was absolutely heartbreaking. Scarlett had been fighting so hard since the moment she was born. She wasn’t ready to give up, and neither were we.

I desperately began researching and learned about Dr. Kays at Johns Hopkins All Children’s Hospital in St. Petersburg, FL. We contacted Dr. Kays for a second opinion, and after reviewing Scarlett’s case, he agreed to accept her as a patient. Dr. Kays was honest with us, Scarlett was extremely sick, and he wasn’t sure if he would be able to help her, but he was willing to try. I knew transporting while on ECMO was extremely risky, but it was a risk we felt we had to take in order to give Scarlett a fighting chance. Within 24 hours, we were able to obtain insurance approval for an air transfer, and the following day, Dr. Kays and his team flew up to Cincinnati on a jet to transport Scarlett down to Florida. Due to the size of the plane and all of the equipment that had to be transported with her, Patrick and I were unable to join her on the flight. As much as it hurt to be away from her, we knew it was necessary to get her where she needed to be. We immediately started the 14-hour drive to St. Petersburg, and we were back by her side the following day.

Scarlett spent another 25 days on ECMO after arriving at JHACH, but on August 9, 2020, after a total of 67 days, she was successfully weaned off of ECMO. She had surgery to remove the cannulas in her neck the following day. On August 17, after waiting 76 days, I finally held Scarlett in my arms for the first time. It was the most amazing feeling I’ve ever felt. On August 24, Scarlett was extubated to CPAP for the first time. She was making so much progress, and we were beyond excited. Unfortunately, after 7 days on CPAP, Scarlett began retaining CO2 and had to be reintubated.

At this point, Scarlett was over three months old and had still never had food in her stomach. She had been receiving all of her nutrition through IV TPN. Knowing it was likely going to be a while before she would be on low enough respiratory support to be able to eat orally, the decision was made to place a feeding tube in her nose so she could start receiving breast milk. Unfortunately, she did not tolerate feeds through the NG tube. Even 0.5mL of food would cause her to vomit. So, after talking with Dr. Kays, we decided it would be best for Scarlett to have a g-tube placed. On September 28, she had her g-tube surgery as well as a Nissen fundoplication and a scar revision, as her original CDH repair scar was not healing well.

A few days after her g-tube surgery, we noticed Scarlett’s face and neck were beginning to swell. Initially, we thought it was due to her operation, but within a matter of days, her head doubled in size. It was determined that Scarlett’s superior vena cava, the main vein that returns blood from the head to the heart, was extremely stenosed due to her time on ECMO. She needed a heart cath done and a stent placed, but unfortunately, at that time, there wasn’t a surgeon at JHACH who could do the procedure. So, Scarlett was transported by helicopter on October 16 to Orlando, FL, to have a stent placed in her SVC. Even though the operation was at a different hospital, Dr. Kays accompanied Scarlett on the helicopter, went with her into the OR during the procedure, and sat with us during her recovery before accompanying her back to JHACH. The surgery was a success, and only two days later, she was able to be extubated to CPAP again.

Her second extubation was going well, the doctors were able to wean her CPAP several times, and she was finally making progress. Unfortunately, on November 9, that all changed. That evening, out of nowhere, Scarlett’s heart rate started to climb, it got up to 260 bpm, and after several minutes of uncontrollable screaming, she became pale and unresponsive. The doctors and nurses ran test after test and tried countless medications, but nothing seemed to help. Her heart rate stayed over 260 for hours. It was by far the scariest night of my life. Even after everything she had been through, that was the first time I truly felt like we were going to lose her. I called my husband, who had traveled back to our home in TN for work, around 2 am and told him I didn’t think Scarlett was going to make it through the night and that he needed to get back to Florida as quickly as possible. Around 7 am the following day, Scarlett developed a fever of 109, and her blood pressure dropped dangerously low. The doctors ordered blood work which indicated she had developed sepsis and was now in septic shock. Scarlett remained in a comatose state for days, and doctors were unsure whether or not she was going to wake up. Scarlett had to be re-intubated again due to her illness. Scarlett’s unresponsiveness was concerning to her doctors, so they ordered an EEG to check for brain activity. The EEG showed nearly constant seizures. Scarlett was started on seizure medication immediately. The doctors then ordered a CT scan of her brain to check for a possible brain bleed. The CT showed significant fluid on her brain and enlargement of her ventricles. On November 19, she had a VP shunt placed to drain the excess fluid from her brain. Unfortunately, that evening it was determined that the shunt tubing was kinked, so she had to go back to the OR the following day for a shunt revision.

The day after Scarlett’s shunt revision, I noticed her arms and legs seemed limp and unresponsive to touch. I brought my concerns to Dr. Kays’ attention. He immediately assessed Scarlett and was shocked to find that she had completely lost the use of her arms and legs. She was taken down immediately for an MRI of her brain. The MRI showed diffuse inflammation throughout her brain and along her entire spinal cord. The neurologists believe this was either caused by the virus attacking her brain and spinal cord directly, or her body’s immune response overreacting and attacking her brain and spinal cord, a condition known as acute disseminated encephalomyelitis (ADEM). There was, unfortunately, no way to get a definitive answer. The neurologists told us at that time that there was really no way to know the extent of the damage to Scarlett’s brain or whether she would ever regain the use of her arms and legs. The CDH team was concerned that she might have difficulty breathing on her own due to her extensive hypotonia and muscle weakness. They began preparing us for the possibility that she may need a tracheostomy. However, Scarlett surprised us all, and on December 10, she was successfully extubated to CPAP for the third time.

Over the next several months, Scarlett’s CPAP settings were gradually weaned, and on February 15, 2021, she transitioned to a low-flow nasal cannula for the very first time. Unfortunately, Scarlett’s lungs weren’t quite ready for the transition, and she was put back on CPAP the following day. After much discussion with the CDH team, the decision was made to send Scarlett home on CPAP. This would allow her lungs more time to grow and her body time to regain strength without keeping her in the hospital for months longer.

Finally, on March 30, 2021, after 301 days in the hospital, we were able to take our baby girl home! Scarlett has significant brain atrophy from her time on ECMO as well as her meningitis infection and subsequent hydrocephalus, but she has regained nearly all movement in her arms and has started to regain movement in her legs as well. She is currently enrolled in occupational therapy, physical therapy, and speech therapy, and she continued to show improvement in her cognitive function daily. We are so incredibly proud of Scarlett and everything she has overcome. She continues to amaze us daily with her strength, we are so blessed to be her parents, and we can’t wait to see what the future holds for her!

Words can’t express how grateful we are for Dr. Kays, Dr. Stone, and the rest of the CDH team at JHACH for saving Scarlett’s life, they saw her strength, and they maintained faith in her when others had given up hope!