Tilly’s Story
Tilly had a thing for the day after holidays. On the day after Valentine's Day, we got Tilly’s CDH diagnosis. She died 4 years later, two days after her 4th birthday and the day after Father’s Day. But she did everything in her short life on Tilly time.
When we got her diagnosis, we had been living in Southern Africa for the past 4 years and first heard the letters CDH in Pretoria, South Africa. Like many other families I know, minutes after learning Tilly’s CDH diagnosis, I was offered the option to terminate. We had never heard of CDH before this appointment and said no, we needed much more information about this diagnosis to make any decision. We got Tilly’s diagnosis on a Friday, the next Sunday my husband, our 20-month-old little boy, and I all boarded an airplane back to the United States. We didn’t know it at the time, but that day our family was moving to Philly permanently.
We first held Tilly when she was 56 days old. I counted everything in the hospital, it was one of my coping mechanisms and it helped me. What can I say, I like numbers. Waiting to hold her was truly one of the hardest (maybe the hardest) things for me. A few days after Tilly came off ECMO, she went into a pulmonary hypertension crisis that almost killed her. The first time I held Tilly, I held her because the team didn’t think she would live past the night. But she did.
Pulmonary hypertension was Tilly’s biggest hurdle, and what eventually led to her death. Pulmonary hypertension and pneumonia. Over the first few months in the hospital, Tilly was put on “triple therapy” for pulmonary hypertension, including subcutaneous remodulin. Her pulmonary hypertension was so severe, we could not safely close her PDA without putting her heart at risk of heart failure. So at 6 months old, Tilly had a stent placed in her PDA to keep it open. Triple therapy and stenting her PDA helped stabilize her severe pulmonary hypertension and save her right heart from failing. Tilly battled severe pulmonary hypertension her whole life. Her heart and lungs had to work so hard, and after 1495 days, they stopped working and nothing could make them start working again.
We found our way to the Children’s Hospital of Philadelphia (CHOP). The first time I met and spoke with Tilly’s CDH surgeon, Dr. Holly, I knew she was Tilly’s best chance. We walked out of that appointment and decided then and there that Tilly’s middle name would be Holly. We had a hospital. We had a surgeon.
Tilly was in the NICU for 456 days. She was home for 1,039 days. She lived for 1495 days. I couldn’t begin to recap those 1495 days and do them justice. They were full of terror and fear, love and cuddles, tears and strength.
Tilly was put on ECMO about 6 hours after she was born and stayed on ECMO for 45 days. She was on 6 different ECMO circuits. She was repaired on ECMO with a large gortex patch. Tilly’s team described coming off ECMO as “jumping off a cliff”. The day we took her off ECMO, Dr. Holly told me it was time for Tilly to jump, and that she truly believed Tilly could do it. And she did. It wasn’t a graceful landing, but she made it.
After 456 days at CHOP, Tilly came home. From the moment she came home from the hospital until the day she died, Tilly’s dad and I managed all of Tilly’s care. In fact, Tilly’s dad went to nursing school. He started school with the plan to be Tilly’s nurse and take her to school. When Tilly died, he knew that the best way to honor Tilly was to finish school and become a nurse. And he is a nurse now. She would be so proud of him.
To be honest, despite months of managing TIlly’s care in the hospital, it took time for us to find sure footing and feel truly confident being in charge of Tilly’s care at home. Tilly time. We navigated some issues that helped build our confidence. Once we felt comfortable doing everything at home, it took time to feel comfortable taking her out of our bubble. But, we started small. Every week we drove to our favorite little park and went for a walk - Tilly and all of her equipment in our Wonderfold Wagon, her brother, dad, and mom - the 4 of us together. We kept Tilly’s big brother out of preschool the first year she was home, we lived in a small bubble and just enjoyed spending time together, under one roof. 10 months after she came home, we went to the Shore - Tilly left Pennsylvania for the first time. Over the next few years, we conquered countless day trips, Christmas in the mountains, four long road trips, sleeping in hotels, backpack hiking in a National Park, going to amusement parks, sitting front row at a Raffi concert, riding a train through the mountains, and countless other little adventures. None have gone perfectly smoothly, but we have troubleshooted and learned from each - we had to switch oxygen tanks on the highway, replace a Gtube in a hotel parking lot, and troubleshoot vent errors from a National Park. We mastered how to fit Tilly’s equipment into a jogging stroller or a backpack, and how to make her vent mobile for our biking, cruising, learning to walk, little girl.
Tilly never extubated. Her small, ventilator dependent lungs and severe pulmonary hypertension meant extubation was never a safe option. When Tilly was 4 months old, she got a tracheostomy. A trach is a scary decision for any parent to make, but we knew it was the right choice for Tilly. The trach let her be a baby, she got to sit up, put her hands in her mouth, be picked up by mom and dad anytime, and eventually come off sedation, all while getting the respiratory support and oxygen she needed.
Like many CDHers, feeding was a long road for Tilly. In the hospital, Tilly went from getting IV nutrition, called TPN, through a PICC line to getting fed through an NJ feeding tube, to a GJ feeding tube. She was discharged with a GJ and after a few months home she was able to move to a G tube. She mastered drinking from a straw cup, but she never mastered eating by mouth.
At month 8 in the NICU, Tilly got pneumonia, the week the COVID pandemic started. It was a week filled with fear and panic for our family and the world. The pneumonia hit Tilly hard, caused her pulmonary hypertension to flare up, and almost killed her. Eventually, Tilly’s team found the balance of pneumonia treatment, lung treatments and vent support, pulmonary hypertension medicine, and prevention to keep the pneumonia from returning. But, this took months, 3 full codes, and countless other scary, uncertain days. Almost 3 years later, she would get pneumonia again. And we would have three 911 calls - two that ended in the CHOP PICU and the final heartbreaking call where Tilly never came home. I truly do hate pneumonia.
Tilly turned 1 in the NICU, surrounded by the doctors and nurses and hospital staff who had known her for her entire life. Despite COVID restrictions, we were able to have me, Tilly’s dad, and Tilly’s big brother all there to celebrate. We took over a hundred photos. No one wants to spend their child’s first birthday still in a hospital, no one. However I can honestly say it was a day where Tilly was surrounded by love and joy, she smiled and opened presents and wore a pretty dress. It was a good day. We spent a year plus of first memories in the hospital - first bath, first tooth, first cuddles, learning to sit up, first Christmas/ Thanksgiving/ Easter/ Halloween/ birthdays - at times it was hard to stomach. But, some families spend a lifetime of last moments with their CDHer in the hospital. It wasn’t lost on me that CDH doesn’t play fair.
We experimented, we used trial and error, and we adapted to what worked for Tilly and our family. It wasn’t easy, but we did our best to enjoy mastering this puzzle.
In the spring of 2023, Tilly got pneumonia. Again. This was her third round of pneumonia. She fought for months, she would get better and then get worse, and then seem healthy, and then get sick again. We had two 911 calls where we knew Tilly was sick, we were working closely with her team and monitoring her, and she just went from sick to critically sick in a snap. Both times, we spent a few days at CHOP and our girl came home happy and feeling much better. In June, Tilly turned 4. She was so happy, her 4th birthday is a magical memory for everyone who was there. She didn’t seem sick, she was walking and happy and playful. Two nights later, Tilly went into cardiac and respiratory failure. She was peacefully sleeping at home, and her body just couldn’t keep working. We called 911, the hospital tried everything, but she was gone. After 1493 days of life, our Tilly died. Our hearts are broken. But we are determined to continue to share her story.
Every CDH journey is different, none are easy. Some have shorter hospital stays, some have longer. Some hospital stays end with a homecoming, but not all do. Tilly’s journey was long and filled with such hope and heartbreak. CDH isn’t fair. And this is why we tell TIlly’s story and why we started the Mighty MoJo Warriors. We will continue to advocate, to fight, and to raise funds for CDH research.
