This “story” was intended to be a brief summary of our journey with Samuel, mostly so that new CDH families could find information (and maybe inspiration) without having to read the whole blog. Turns out, brevity is not my strong suit. Read the whole thing or peruse headings to find what you most want to know. Thanks for sharing in our family's journey with Sam.
In July of 2010, I found out that I was pregnant with our 4th child. That same night, I started having dreams that there was something wrong with our baby. The dreams all began with an innocuous, sometimes beautifully nurturing, theme. The dreams all ended with me laying in a pool of blood. I would wake up crying.
Dreams had always been a rich source of information for me and I knew that something really was wrong. At 9 weeks gestation, I begged the doctor to send me for an ultrasound to reassure me that everything was okay. She trusted my intuition and off we went. I got to see our wriggling baby and the sweet, sweet blink of heartbeat. I was instantly, intensely, in love. The dreams did not stop.
At 13 weeks gestation, we went for the routine screening blood work, ultrasound, and neuchal fold measurements. The results indicated that something might be wrong with our baby and so we went back a few days later for more testing. It was then that the ultrasound technician noticed something amiss and brought in the perinatologist.
Chris and I were told that our baby had a Congenital Diaphragmatic Hernia. The doctor showed us the heart beating on the right side of our baby’s chest, the stomach and bowel on the left side where the heart should be. Some, or all, of the left diaphragm was missing, a genetic mis-step that would have occurred at around 9 weeks gestation. For the first of many, many times, we were offered the option of terminating our pregnancy. I can still feel and see that room where we talked about CDH for the first time, where our lives tilted in a new direction.
Our days became consumed with appointments, research, tests and doctors. Our hearts and minds became consumed with 'what ifs' and anguish. By day, I tried to make things as normal as I could for our three boys. I made lunches and did school drop-offs and read stories. I went on field trips and made small talk with other mothers while our children played at the park. I told very few people and, as my belly grew, I developed a repertoire of over-sized sweaters and colourful scarves. I wasn’t strong enough to acknowledge my pregnancy, to be greeted with "congratulations!" and “when are you due?” while my heart was breaking. I just needed to be okay for a few hours a day. At night, when my boys were in bed, I hung out with Dr. Google, read all that I could on diaphragmatic hernia babies, and wept.
My Grandma and Samuel
The amnio results told us that our baby was a boy. I had thought, if I was to have another child, the name would be for my beloved grandmothers. Now here was another child and my grandma was dying. We named him right away so that she would know that her 20th great-grandchild – the first one she wouldn’t meet – was named Samuel Meir. Meira is Hebrew for Helen for her and Margaret for my other sweet grandma who passed almost exactly 20 years earlier.
My grandma died peacefully on October 8. A few days later, we had the echocardiogram of Samuel’s heart. I felt my grandma in the room with me, felt her telling me, “his heart is perfect, his heart is perfect.” When the technician left the room, Chris said to me, “you know your grandma was here. I felt her for about 20 minutes. She said his heart is perfect.” I said, "yes." The cardiologist came in a few minutes later and told us that our baby’s heart looked perfect. Samuel’s heart was squished over to the right side because of interloping abdominal organs, but structurally and functionally perfect.
We met with a surgeon, a geneticist, and various perinatologists and learned that CDH by itself had a 50/50 chance of survival. We already knew that our case was more severe based on its early detection – if stomach and bowel can already be seen in the chest at 13 weeks gestation, the hole in the diaphragm is very large and the opportunity for lung development is very small. We needed more information about co-morbid concerns in order to know what our baby might need.
The amniocentesis had suggested that the chromosomes were normal. Ultrasound indicated that the kidneys were normal. Echocardiogram said that the heart was squished but normal. We allowed ourselves to hope. I let in cautious, frightened shards of hope. Chris let in enormous moonbeams of hope. He is so brave that way.
At 19 weeks gestation, we had an MRI to get a better look at the defect. The results of the MRI were damning; no diaphragm on the left side, stomach, bowel, liver and possibly spleen in the chest, and an estimated fetal lung volume of 22.5%. The liver up so early was bad news. The 22.5% fetal lung volume was even worse news. Radiologists, perinatiologists and surgeons – CDH experts in various hospitals and research centres – reviewed the prenatal testing results and agreed that no baby with a picture like Samuel’s had ever survived.
Samuel was safe in my belly as babies don’t need their lungs in utero. We were told that he would be born unable to breathe, that he would likely die at birth or soon after. IF he lived, he would not be strong enough to go to repair surgery. IF he went to surgery, he would likely not survive the trauma of the procedure. IF he survived the procedure, he would likely have multiple disabilities resulting from his defect and from the invasive treatments that would be needed to save his life.
We talked to a neonatologist, a respirologist, more perinatologists. We got the same grim predictions from everyone. I felt submerged in grief but I needed to keep going, keep working at the job I love, keep taking care of my amazing kids. There was no reprieve, no time where I “forgot” that my body was nourishing a baby who couldn’t live. As a wise friend has said to me, when you are walking through the house of horrors, there is nothing you can do except keep your feet moving.
My relationship with Hope
I wanted to be hopeful that my baby would be okay, to deny all the information we were being given. But the experience of hoping and then crashing, of lifting myself up and then falling so deeply into the despair, was unbearable. There were two things I knew: my family would be okay no matter what happened AND I loved this little baby no matter how much time he was meant to spend in our family. My beautiful friends offered to hold hope for my baby on my behalf while I focused on loving and surviving.
At every appointment – and there were a lot of appointments – we were offered the option of terminating the pregnancy. It was one of the most angst-filled times of my whole life. I wanted to create the least amount of suffering for my baby and the rest of my family. One fall day, Chris and I walked together and cried. I tried out the words, “I think I am ready to let him go” and a gentle voice immediately responded, “Oh Corinne, you know that’s not true.” A wash of peace came.
And so we knew that, for us, we would go forward. Our son – and the path he was leading us on – would be in the hands of Spirit. Just as if any of our children were sick, we would hold and love them until whenever their ending was. It was a thoughtful, faithful process that led us to choose Sam.
Waiting for Sam
The pregnancy was difficult emotionally and physically. I got severe polyhydramnios, a condition where there is too much amniotic fluid. Babies swallow and process amniotic fluid but CDH babies have difficulty doing this because their digestive systems are squished up in their chests. The amniotic fluid continues to build. I became enormous to the point where I could barely walk and my symphisis pubis separated. I stopped working, was in bed as much as possible while still looking after my children. My husband and my parents picked up my slack and neighbours started bringing meals.
There was no hiding my pregnancy at this point and everywhere I went, people would smile and comment... did I know if it was a boy or a girl? Were we getting excited? Sometimes I would find a simple response and move along. Sometimes I would say that our baby was very sick and we didn’t know if he would be staying with us.
As we got closer to the end, the doctors said, “you never know, let’s just see.” It felt a little thin. What else were they going to say to this enormous pregnant woman who was verging on breaking in two?! But I was grateful.
We did Christmas and prenatal photos and my children and parents met Sam through a 3-D ultrasound. I picked out an outfit that Samuel could wear while we held him and another for him to be buried in. We had meals and child care support. I told my clients that I expected to be back to work in three months.
Chris and I discussed how to best help our other kids, what to tell them and not, whether they would get to meet and hold their brother. We helped them to understand what they could at 8, 6 and 4 years old. Chris and I, along with my parents, did as much as we were able to create a loving and stable container for them.
Samuel’s Birth Day
My water broke at 36 weeks and 5 days. Our two oldest kids went to school and we called my parents to be with our youngest. Chris and I went to hospital, knowing risks to baby and to me. I was so afraid. It was too soon. I wanted more time to be with him, to keep him safe. But our Sam would be born that day. Chris was with me, taking care of paperwork and calling my parents and the kids’ school and my heart-sister Lori, telling them to come.
A constant stream of doctors and nurses came to talk to me, shine their compassionate faces on me, hold my hand. My obstetricians, not on service that day, arrived and scrubbed in to do the c-section. The anesthesiologist was a resident at Daniel’s birth and I remembered his gentle voice and caring. The neonatologist spoke to us about what to expect, about our wishes.
It was shift change when I was heading into the OR and some of our day shift nurses stayed. A student nurse who’d been with us all day came in and stood on a chair over my shoulder to get photos of Sam’s birth; she took the only pictures I had of his face before he was intubated. I had Chris next to me squeezing my hand. I could see my mom and Lori on the other side of the glass and my mom’s eyes were on me while Lori’s were closed in fervent prayer. Just before Samuel came out, a bunch of people from the NICU came in through a side door. Considering how many people were in the room, it was oddly quiet.
Samuel and the NICU
Samuel’s first many hours were his honeymoon period. He needed very little oxygen support and seemed to be doing well. Later that evening, Samuel crashed and started “acting more like a CDH baby.” Every IV and central line, every monitor number, every beep of the machines felt terrifying to Chris and I.
On the fourth day, I was discharged from the hospital and I went home without Sam. My whole body knew that there was something missing, something wrong. I felt grateful that we lived so close to the hospital so that I could be with my boys and still be at the hospital for many hours each day and into the night. It was the beginning of my family having two different homes.
I was there almost all the time, sitting next to Sam, reading quietly to him, holding his head and feet. When he was a week old, he opened his eyes for the first time and we gazed at each other for 10 beautiful minutes before he slipped away again. Dr. Carlos came by and I told him that it seemed like Samuel was looking right at me. He said, "Of course, mama. He is in there and he knows you."
On the tenth day, I was scrubbing to enter the NICU when Dr. Carlos found me and said to hurry in because Samuel was being switched from the oscillator to a regular ventilator. Several people all moved together in the small space around our baby. Dr. Carlos stood in the centre, manually bagging Samuel while the new ventilator was put in place. I stood off to the side and prayed.
Samuel and Alberta Children’s Hospital PICU (10 days old)
Moving over to the Children’s Hospital was scary; I missed our NICU people like I’d known them forever and not just for 10 intense days. I soon realized the PICU staff were just as capable and loving. I started to attend rounds every day, to listen and make sense of what I could. Samuel was very sick and unstable and he was on a lot of life support. His monitors rang off all the time and the nurses and RTs would adjust this medication and tweak that setting to try to help him.
One day during a particularly bad pulmonary hypertension episode, the intensivist in charge came in and looked at our baby and said, “yes, well, this is how the diaphragm babies die.” I swallowed hard and asked her if we needed to have a conversation about letting him go. She said, “not yet. I will let you know.”
Each day in the PICU, the surgery question was asked. Each day, the surgeons, anesthesiologists, intensivists and respirologists said no, that he wasn't strong enough. When Samuel was 17 days old, they said yes, tomorrow they would do it. Within 20 minutes of that decision, Samuel spiked a fever. Sepsis cultures were sent to the lab and antibiotics were started.
This was Samuel’s first round of pneumonia. Talks of surgery were halted until he was well. That was when I knew I had to stop waiting for surgery, stop living for tomorrow. There was only this moment, this breath, and I would miss it if I was busy anticipating the next. When I was at the hospital with Sam, I was right there. When I was home with my boys, I was right there. I slept. I ate. I cried and prayed and laughed. I wrote for my own therapy. The dictate to “live in the moment” was not new but it was the first time I’d actually felt able to get inside it and do it.
Surgery (25 days old)
On Friday, February 25th, we were told that Samuel was as ready for surgery as he would be. This was our window. They were hoping that OR time and space in the surgeon’s schedule would appear and we were told to wait until 2:00. If we hadn't heard anything by then, surgery would be delayed again.
Chris and I spent the day together with Samuel. We didn't talk about our fear but it hung thick in the air. We laid hands on our baby and prayed and read to him. At 5 minutes before 2, a nurse stuck her head into our room and said they would be taking him to surgery. The hurry-up-and-wait preparations continued and it was 4:30 when they came to take him. The anesthesiologist told me to kiss my son good-bye and they would take good care of him.
Samuel was gone to surgery for nearly 5 hours. The surgeon came to tell us that things had gone smoothly with the repair. There was virtually no diaphragm on his left side and he did indeed have bowel, stomach, spleen and liver in his chest. His spleen had bled a little and they would be watching it closely for a few days. If there were any signs of ongoing bleeding or infection, they would have to go back to surgery to remove the spleen. But it was done. His insides were fixed.
Our surgeon told us that, when she pulled the intestines out of Samuel’s chest, a lung inflated on the left side. She said that she usually doesn't see lung at all on the defect side but that Samuel had a pink, healthy-looking lung there. Later that evening, the intensivist came into our room to show us Sam’s post-operative chest x-ray. It showed that Samuel had a full lung on his right side and about two-thirds of a full lung on his left side. Somehow, he had grown lungs with all those extra organs in his chest.
The predictions were that he could not grow lungs because we could see bowel and stomach in the chest at 13 weeks, because the liver was already up at 22 weeks, because the MRI showed 22.5% total fetal lung volume. The predictions were wrong. Here was our Sam with lungs, lungs that would grow as he grew.
Healing and holding
Samuel was very fragile for the next few days after surgery. He had desaturations and bradycardias to almost any stimulation. He was handled as little as possible and given extra sedation when he had to have even the smallest procedure. I couldn’t touch him. We whispered or spoke in the hallway. He wore ear plugs and bili-goggles (the sunglasses used by babies under jaundice lights). He was extremely swollen. We watched carefully to see if Sam’s body could heal.
Three days after surgery, his nurse told us that he was 72 hours post-op and he was getting better. By her tone, we knew that this was significant marker, that even though he still seemed so very sick to us, it was possible that Sam was going to hang on.
On March 11, I arrived at the PICU to find our nurses and the intensivist waiting outside Samuel's door. Sam was nearly two weeks post-op and nearly six weeks old and becoming less fragile every day. The same doctor who told me "this is how the diaphragm babies die" now said to me, "we no longer think he is going to die...” and “we think it’s time for you to hold him.” She said a bunch of other things that I didn't hear. I just stood there sobbing the tears of six and a half months of waiting for my baby to die.
I held him that day for 4 hours. It took three people to move Sam and his lines and to protect his airway. It wasn’t exactly a snuggle; it was more like his weight resting on me while I sat still as a statue. But it was glorious. Nurses and RTs came by to check on us and celebrate with me. I held him again the next day too and Chris came to be part of that. One nurse helped me make prints of Sam’s little feet and I realized that she did this all the time with the babies who had passed. Another nurse helped me give him a sponge-bath and Samuel got to wear a sleeper for the first time.
Sam started to get a bit of food through an NG (naso-gastric) tube. Now that his stomach and intestines were back in his abdomen, it was time to try to get his digestive system working. It was the smallest possible drip – 1 ml per hour so not even a full ounce over the course of a whole day. Even the slowest progress was sweet.
I eagerly took over as much of Samuel’s care as I could. I bathed him daily, making a meditative ritual of preparing a tub of warm water and the cloths and the lotion for his post-bath massage. I told him the same stories and sang him the same songs. I folded again and again the blankets that my mom had lovingly hand-stitched. Chris came every morning and read Sam the same book but he was primarily in charge of what happened at home – cooking and playing with our big boys. My parents filled in the spaces to hold our family together. Sam’s brothers came to visit him in the evenings, usually one at a time so as not to overwhelm Sam or create any additional chaos in the ICU. We had a routine for living under two roofs.
Blood clot and Sepsis
Just after those awesome holding sessions, Samuel started acting like a sick baby again. He couldn’t tolerate touch or noise. He needed more ventilation and pain medication. Cultures were sent again to check for sepsis and came back positive within 24 hours. Samuel had two different strains of staphylococcus, one in his lungs – pneumonia round two – and one in his blood. The blood sepsis was originating from a large clot in his femoral line, the central line they’d been using to administer medications and nutrition since Samuel was 11 days old.
Our baby was in danger again from two different infections that we hoped were not antibiotic-resistant and a blood clot that could dislodge and travel to his lungs or brain. Two different types of antibiotics were started right away, one for each strain of staph. Enoxiparin (a blood thinner) injections were started as well and the Hematologist explained that Samuel would need 3 to 6 months of these twice-daily shots to manage the blood clot. The femoral line would have to be removed but they needed to do a couple of days of the blood thinner first to minimize the chances of dislodging the clot.
At the same time, Samuel was having a lot of difficulty weaning from the narcotics and benzodiazepines; he’d been on these medications since birth and they had been increased along the way to respond to his instability and drug tolerance. We had an infant addict on our hands. He needed enough of these medications to keep him comfortable so that his cardiac and respiratory systems could heal but he needed less of the medications so that he could wake up and learn to breathe on his own.
Some of the doctors believed that Samuel may not be strong enough to come off the ventilator and so began the talks of a tracheotomy. A trach would allow Samuel to work less at breathing so that he could come off the ventilator and start developing like a normal baby. It also meant that Samuel would have an open airway for a year or possibly more. It meant that he would not be able to vocalize at all and would need adult eyes on him 24-7, including at night and in the car. His activities with our family would be dramatically restricted. I tried to wrap my head around how we would do our life if Samuel had a trach. I knew we would manage.
In my mama heart, I knew that Samuel did not need a trach, that his current problems were because of the sepsis and the sedation, NOT because he couldn’t learn to breathe. I advocated for more involvement from the Acute Pain Services people to manage Sam’s medication withdrawal. Dr. Ferrin came to see Sam, took him on as his own patient, offered us a plan and a bucket-full of hope.
This was a very difficult part of our journey. I felt that I needed to be happy and grateful because our baby was going to live – and I was those things. But I was also exhausted and still vibrating with grief. Every day, I walked down the ICU hallway and I ranted in my head about how I hated CDH and I was so done with this place. I silently screamed “give me my baby I want to go home my family needs to be together enough already I can’t stand it.” Then I walked through the doors of the ICU and my self-pity time was over. I told myself “this is hard but you can do hard things.”
One day, Samuel woke up for about 2 hours and looked all around with lucid eyes. It was beautiful but then he decompensated again and had to be medicated away. It was enough for us to know he was in there and so I put a photo of him on the door of his ICU room to remind us what we were working for. Parents often put pictures of their healthy children on the door to let ICU staff know who was inside that sick body. I didn't have any pictures of Sam as a healthy child. But I had this.
I invited captions for the photo. I'd found my way in to ICU humour as a way of surviving life in this place. As I sat in Sam’s silent, dark room and watched ICU staff walk by, read the captions and smile, walk by again, stick a post-it note of their own next to his picture. I already loved our ICU peeps; they cared for our little blob of baby with their brilliance and care and they taught, comforted, and respected his family. I loved them extra for the care - and laughter - that they surrounded us with during that really tough time.
Extubation (Two months old)
On April 4, at 63 days old, Samuel was extubated. It is his Breath-day – the day that he took his first breath without a ventilator, the day that we first saw his face without tapes and tubes, the day that we first heard his cry, the day that we were finally able to pick him up and hold him.
We were aware that Samuel might fail extubation. He might need to be reintubated to rest before they could try again. He might still end up with a trach. We waited and happily learned to manoeuvre around the bulky, leaky BiPAP mask so that we could cuddle him. On the third day of extubation, the intensivist told us that reintubation and tracheotomy were no longer on the table.
It sounds like such a small thing but we now had access to Samuel’s mouth. Several times a day, an RT took off his mask and monitored him while I did mouth care. I gently cleaned his mouth and lips, touched different textures to his face, offered him a soother. I knew that oral aversion was likely after being intubated for so long and I did my best to give Sam loving, non-invasive oral experiences. These were beautiful moments of watching him breathe and hearing his voice and gazing at one another.
Within the next three weeks, Samuel went from Bi-PAP to C-PAP and then low-flow nasal prongs to deliver his oxygen. The ventilator was wheeled out of his room. The sedative medications gradually came off until he was on only methadone and chlonidine. Other medications were few, including the twice daily enoxiparin shots that were riddling his tiny thighs with poke marks and bruises. The TPN that had fed him to this point was finally weaned off in favour of a highly digestible form of infant formula, delivered via NG tube into his stomach.
Samuel became a lot more interactive and a lot easier to handle. He got to have cuddles with his Nana and each of his brothers. I snuggled him every single day, smelled him and memorized him and stroked his little head while he slept.
After 3 months in ICU, it was time for Samuel to move to the step-down unit so he could learn to eat and breathe and grow. Our graduation from ICU was a trip outside the hospital, along with a nurse, an IV pole, an oxygen tank and monitor. It was the first time that Samuel had felt air and sun on his skin. The next morning, we said our good-byes to our ICU folks, some of the most inspired, loving, courageous, brilliant people I have ever known. For so many months we had been prepared for Sam to die. Now we prepared live and thrive and come home to his family.