CDH Awareness: Understanding Congenital Diaphragmatic Hernias
Alanah Lewis was told her baby, Miller, had CDH when she was twenty-six weeks pregnant. This condition, which affects 1 in 2500 babies, is as common as both spina bifida and cystic fibrosis, yet receives little funding for research. This is their story.
At twenty weeks, Alanah and her partner, Jeff, learned that their baby, Miller, would be born with a cleft palate. At twenty-six weeks, they learned he also had CDH and a 20% chance of surviving after birth.
CDH, which stands for ‘Congenital Diaphragmatic Hernia,’ is a birth defect caused by the diaphragm not forming correctly at around eight weeks gestation. As a result, organs that should remain in the abdominal cavity (like liver, intestines and spleen) travel into the chest cavity, which can prevent the lungs from developing. On average, the survival rate of a baby with CDH is 50%. Complications can include chronic lung disease, pulmonary hypertension, gastroesophageal reflux, failure to thrive, developmental delays and hearing loss.
The cause is unknown.
While some studies have shown that certain environmental factors may cause CDH such as exposure to pesticides and/or an imbalance of Vitamin A and Folic Acid, very little research has been done due to lack of awareness and funding.
While most parents would roll into a ball of depression by the second diagnosis, Alanah and Jeff were no strangers to facing tough news. In 2016, Alanah was diagnosed with Thyroid Cancer and had to undergo surgery and radiation. In 2018, when pregnant with her first child, Myles, she learned that she had Type 1 Diabetes. ‘Resilient,’ ‘optimistic’ and ‘warriors’ are just a few words to describe this couple.
‘After receiving the CDH diagnosis, we left the hospital in tears for a number of reasons. We were shocked, confused, sad, but mostly dismayed because our doctors seemed adamant that we should have an abortion. They also wanted us to do amniocentesis to check that our baby didn’t have any further chromosomal abnormalities, but the risk of going into preterm labor was too high for us. While I know a survival rate of 20% is low, it’s still something. From our research and conversations with parents in CDH support groups, we kept learning that CDH babies who had had a 5% chance of survival were alive and well and others who were given 90% survival didn’t make it. We didn’t want to get caught up on a number. We just felt like whatever the case, we’d face it at the end.’
Types of CDH
There are two types of CDH: Right/Left Sided (Bochdalek) and bi-lateral (Morgagni.) Miller had Right Sided Bochdalek, which is more rare and much more dangerous because larger organs are on the right side.
‘At our 30 Week Scan, we learned that Miller’s bowels, kidneys and stomach were in his chest cavity. They couldn’t detect any right side lung tissue and the left tissue was very small and compacted. But even with this bleak prognosis, we just kept our chins up and stuck to the plan outlined by our doctors and specialists. We spent our days trying to wrap our heads around every possible outcome. Our doctors explained that Miller would most likely need multiple surgeries and would probably need to stay in Sydney anywhere from three months to a year in special care.’
Due to the fact that Alanah and Jeff live in a remote area of Australia, the plan was to go and stay in Sydney from thirty-six weeks. Two days before they were planning to drive down, Alanah went to her local GP for a general check-up. Other than suffering from a few tightening feelings and polyhydramnios, which is an excess of amniotic fluid, she had few concerns and felt surprisingly relaxed. However, when they checked Miller’s heart rate, it was a bit fast, so she was sent to the local hospital for further observation.
‘Miller’s heart rate continued to stay high. After communicating with my Sydney specialists, they put me in an ambulance en route to the airport to be flown to Sydney just to be safe.’
Notice how Alanah said, ‘flown’ and not, ‘air-lifted.’ This was December 2019 when the fires were engulfing Australia. Every helicopter was in use, so she needed to be flown by airplane to Sydney. Because resources were so limited, the plane was routed to the nearby town, Orange, to pick up another patient who also needed to be transferred to Sydney.
‘When I envisioned going into labour with Miller, the last place I thought I would be was strapped down in an airplane. But by the time we touched down in Orange, my tightenings had become full-on contractions that were seven minutes apart and not slowing down at all. Since the other patient hadn’t arrived at the airport yet, the nurse made the executive decision to take back off to get me to Sydney. Unlike a helicopter transport that can land directly on the hospital, flying by plane meant that we had to land at Sydney Airport, which meant I would still need to travel a good one hour drive by ambulance. By the time we landed, my contractions were two minutes apart and incredibly intense. I’m talking take-your-breath-away-intense.’
It’s safe to say that laboring in an airplane and ambulance is not something any mother anticipates doing. There’s nowhere to pace, no pain medication, no crushed ice!
‘As my contractions became one minute apart, I could sense the fear in the student EMT’s voice when she pleaded with the driver to get to the hospital faster. We were already doing the whole flashing lights and sirens charade through every tunnel, but I appreciated her effort. I also appreciated that I was able to manage a few text messages throughout this whole ordeal because Jeff had just thought I was being sent for observation. Once he knew what was happening, he jumped in his car to drive the three and a half hours to Sydney to meet me, which luckily he did – JUST as I was being wheeled into the pre-op room.’
From the start, Miller’s birth was always going to be a cesarean because Alanah has big babies. Her first baby, Myles, was born weighing just over 9.5 lbs at 36 Weeks and 5 Days!
‘Miller’s delivery was perfect. Since I only had an epidural, I was awake and got to see him the moment he was pulled out of me. All 8.2 lbs of him. Jeff and I took in every inch before he was quickly taken to be intubated and transferred to the children’s hospital. Even though I was desperate to hold him, the doctors had explained that once he was pulled out, they wanted to avoid him taking a breath on his own because it forces air into the body, which makes it worse for CDH babies because everything inflates.’
Utterly exhausted, Alanah was wheeled to recovery while Jeff went to go see Miller. He proudly reported that he was doing well. His oxygen saturation was good, heart rate was strong, blood sugar levels stable and looking like he was going to be okay. That night, they slept. Sort of.
‘The next morning at 6AM, I was anxious to get all of my IVs out so that I could go and see Miller. When I arrived at the The Grace Unit (which is even higher care than the NICU,) everything had done a complete 180. Our sweet boy was not well. Desperate to hold him, the nurse made us wait because when you hold a newborn, you actually stimulate them, which can do more harm than good when it comes to keeping their blood sugar levels stable. They want the baby to be as still as possible. All we could do was watch over him until the doctor arrived to explain what was happening
About an hour later, the doctor arrived and pulled us into one of those side rooms you see in movies. Together with a counselor and our nurse, the doctor told us that despite all of their efforts, Miller would most likely not make it. Even though he was able to breathe in oxygen, his body could not expel it, which meant he was essentially being poisoned by nitrogen. On average, nitrogen levels in the human body are 7- 20 mg/dL. Miller’s was 120. They further detailed that even if he did pull through, he would have brain damage. They left it up to us to decide if we wanted to focus on making Miller comfortable or keep pushing to keep him alive. Jeff and I had always said we didn’t want him to suffer. In the end, we decided to keep him on life support until we could get our other kids there to say goodbye. I called my parents who brought our son, Myles down, while Jeff called his former partner to bring their twins, Betty & Ryder and his step daughters, Rosie & Charlie to the hospital.
While we waited for them to arrive, I held Miller for the first and last time. In between staring at his adorably chunky body, I remember watching his oxygen saturation level slowly drop. At first it was 100%. By the time Myles and my parents got there it was 80%, when the big kids arrived – it was 50%. The doctors had said, even if we chose to fight, he might just go anyway. I truly believe that Miller was holding on so that he could meet everyone. It’s like those stories you hear about a husband waiting for his wife to say goodbye before letting go. Once Miller’s oxygen saturation level was at 20%, we asked the kids to leave the room so that we could have our last few moments with him. The doctor came in and turned off the life support and removed all of the tubes. As Miller’s numbers continued to drop, Jeff took a turn holding him.
It was there in his dad’s arms that Miller went to sleep for the final time – forever one day old.’
When Alanah and Jeff were finally ready to head back to the adult hospital, they were faced with something no parent who’s just lost a baby should have to face: the maternity ward.
‘One of the biggest gaps in our healthcare system is that even if you had a stillborn or neonatal death, you have to go back to the maternity ward, especially if you’ve had a c-section. I will never forget being wheeled to my room behind another mother who was holding her baby. I simply could not stay there. Jeff and I were adamant about being moved, but evidently there was no other ward for me to go to. Desperate to not hear the sounds of another woman’s baby crying, Jeff and I signed the discharge form that stated we were leaving against doctor advice. We went and said goodbye one last time to Miller and told him we’d meet him at home. Once back in our town, we held a beautiful funeral for him and buried him with Jeff’s mother who had passed away years prior.’
When asked how she was navigating her grief, Alanah explained:
‘To be honest, Myles is the number one thing helping me cope because he is a daily reminder of all the good in the world. Shortly after we laid Miller to rest, Jeff and I went to the beach for a week with Myles and just celebrated life. I also found going back to work helped because as a hairdresser in a small town, I was able to talk with so many incredible women who shared their own stories with me. I know it’s difficult to know what to say to someone who has experienced infant loss, but I just wanted to feel like people were there. Even if it was a simple text with a heart or sunflower emoji, which represents CDH awareness. I don’t want people to tiptoe around us because I love seeing Miller’s name, photos and sharing his birth story. Our day together is something I want to celebrate forever.’