Herle’s CDH Story

Because of the summer holidays, we didn't get an ordinary ultrasound until I was 20 weeks pregnant, (in Norway we get called in for an anatomy scan between weeks 17 and 19), and since we were so excited to find out the sex, we chose to pay for an early ultrasound privately at 14 weeks. The day that was supposed to be a happy joy-filled day, had been turned into anything but. We went in with butterflies in our stomachs and left there in shock and in anticipation of being assigned an urgent appointment in Oslo at OUS Rikshospitalet (4 hours drive from home). OUS Rikshospitalet in Oslo and St. Olav’s Hospital in Trondheim are the only two hospitals in Norway that specialize in CDH and we got assigned to the one closest to our hometown. Norway only sees about 10 cases a year, and Rikshospitalet gets about 8 of them. Their survival rates are at 90%, so we were in the best hands possible.

 We were going straight to a funeral after the ultrasound appointment. I didn't even know the deceased personally, but I almost dared to say that I cried more than his immediate family. Not just because of the long, happy life that was now over, but in fear of the little life inside of me that hadn't even started. Sitting at a funeral right after receiving such a message was absolutely horrible. In Oslo, they confirmed the diagnosis of CDH and strongly encouraged us to consider terminating the pregnancy. We quickly agreed that it was not up to us whether she should live or die, it was her fight. As long as she had no other diagnoses incompatible with life, we would continue the pregnancy. We had to wait 10 days for the results. The worst 10 days of my life to this day. I was planning her funeral in my head, everything from flowers, music, and what her gravestone would say. In the meantime, I searched for some kind of fellowship, and I found Tiny Hero’s community on Facebook. I wrote my story and asked for stories of hope in return, and the members did not disappoint. I got overwhelmed by the response and copied all the stories and images into a document that I printed and carried with me everywhere. I read it so many times the pages got worn thin. It was a physical hope to hold on to. 

 Our NIPT came back clear and showed that she had no chromosomal aberration. We were ready to fight! We had follow-up appointments at Rikshospitalet approximately every 3-4 weeks through the rest of the pregnancy and we were met with a more and more positive attitude that this should end well. They thought the hernia was small, perhaps even a candidate for keyhole (laparoscopic) surgery because at every control only small intestines and stomach were up. Boy, were they wrong! Herle was born by planned cesarean section at 38+1 weeks in a big operating theater packed with 24 surgeons, doctors, nurses, students, midwives, anesthesiologists, you name it. All dressed in green. I didn't get to see or hold my daughter (as planned) but I heard three faint cries before they ran off with her. I saw her upside down as the midwife passed my head on the operating table. She was immediately sedated, intubated, and taken to the pediatric intensive care unit. While we were waiting for me to recover from the spinal anesthesia so we could go see her, she rapidly deteriorated and the doctors decided to put her on ECMO (heart-lung machine) when she was only 3 hours old. 

Fortunately, it stabilized her. She had severely high arterial pulmonary pressure (hypertension) so she did not receive oxygen in her blood after birth. Neither the normal ventilator, oscillator, or medications were sufficient to save her alone. However, although she was stable on ECMO, her pulmonary hypertension did not respond to any medication still. The doctors were at the end of the road. With no signs of improvement, they did not want to keep her on the ECMO machine for more than a week, as the complications would queue up. She was given large amounts of blood thinners to prevent blood clots, and the risk of brain bleeding or bleeding elsewhere in her body was imminent. The last attempt to save her would be to repair the hernia to give the lungs more room, but the doctors hesitated as it was not desirable to operate on ECMO. In practice, the child has an acquired bleeding disorder and the chance of bleeding to death is high. But it was her last chance, and we had to seize it. We had an emergency baptism when she was four days old, and I stood with my heart in my throat as they wheeled our girl off to the operating room two days later.

Fortunately, the operation went beyond all expectations. We were subsequently informed that she was patient number three who had ever been operated on for CDH on ECMO in Norway, and the only one who had so far survived. It was also the first time for this surgeon - he was ecstatic! Two days after her repair, they successfully weaned her off ECMO and she maintained good oxygen saturation herself (in an induced coma on an ordinary ventilator)! The hernia was large, she was missing 75% of her left diaphragm (type C defect, borderline D), and all organs except the kidneys and parts of the liver were in the chest. Now her body needed rest to heal. For the first month and a half, we weren't sure if she would survive, it was tough just sitting by the incubator and waiting without being able to actively contribute. We fished for good news, but the anesthetist was sober throughout and the answer was always "As long as you have a child in intensive care, you're not out of the woods". Amazingly she never had any bleeding, strokes, seizures, or any of the daunting scenarios that really aren’t that uncommon.

 When she was 44 days old, I finally got to hold her for the very first time. During her first seven weeks of life, she had six operations: Placing ECMO cannulas, hernia repair, two additional operations to close her abdomen, removing ECMO cannulas, and closing of an open ductus in her heart (PDA). That's more than most people have in a lifetime. Everything has gone very slowly with Herle throughout. She was sensitive to even the smallest changes in settings on machines or medications. They had to come up with a whole new protocol to suit her slow-weaning needs. She struggled violently with withdrawal after a long time on heavy medication. For two months she was in an induced coma on a ventilator before she was successfully awakened and able to breathe on her own with the help of CPAP. She was dependent on this support 24/7 for another 2 months before she could manage with shorter sessions during the day and a bit of high flow. 

We spent a total of three months at Rikshospitalet and another two months at the neonatal intensive care unit in Kristiansand (our hometown) before we were allowed to finally go home on June 11th. I hadn't been home in my own house since January 10th. It was surreal to come home! 138 days in NICU and another 2 weeks prior to her birth for me, as I had to relocate in case she decided to come earlier than the planned birth. I was told she would most likely not have survived if she was born in Kristiansand, and after needing ECMO at 3 hours old, I know for sure that she wouldn’t even have managed the airlift.

We came home with a 5-month-old baby, with the skills of a newborn, as well as a whole equipment park of machines and medicines. It hasn't just been easy. It created a vacuum to no longer always have doctors and nurses around, and a red string to pull whenever you needed help or a break. We still haven't had any real breaks, but fortunately, there are two of us who at least can relieve each other. 

Developmentally Herle is delayed both in gross motor, speech, and a bit cognitively. She also struggles with eating. She had a G-tube placed before being weaned off the vent at 2 months old, as they predicted she would need CPAP for a long time and would not be able to bottle- or breastfeed. She has been weaned off the G-tube twice with the help of GIE but keeps getting sick and dependent on it again since she stopped eating. She is also sick for quite some time when she gets ill. The first winter was though and we spent a great deal of time in hospital, despite getting RSV shots every month and mostly isolating ourselves at home. 

Herle will be getting her RSV shots this winter too, but she is now a year and a half old and just started kindergarten in August (big milestone!). Things are really looking up. Our days consist of more and more regular family stuff and fewer medical appointments and procedures. We have delivered most of her medical equipment back to the treatment aid center, she does not take any regular medications except Nexium for her reflux and her CPAP is only in use for 30 minutes morning and evening during flu season. X-rays show no signs of curving of her spine, and her PT is slowly backing out from 1-2 sessions a week, to once a month and then once every six months when she has started walking, which is right around the corner now! We are planning another wean attempt to get her off the G-tube next spring, and we are positive that this third time’s the charm. We are just crossing our fingers now that those kindergarten bugs will be gentle with us. Despite everything she has gone through, Herle is the biggest ray of sunshine! There is so much hope!

It was on the 22nd of August 2022 that we received the diagnosis of our daughter. Walking out of those hospital doors into a void not knowing where or who to turn too was terrible. I decided there and then that I needed to read and learn as much as possible about CDH. I wanted us to be the last couple to walk out of there feeling hopeless and alone. Just 4 weeks later, on the 22nd of September, CDH Norge was founded. I am a very committed chairman of a small, but very important, organization creating hope for those who come after us. I have received so much help from Tiny Hero in establishing our organization and I hope for a great friendship and sisterhood in years to come! Thank you for being there for me when I needed a community and later helping us create a community of hope overseas too. As our community is still very small, I always recommend our members join Tiny Hero’s community as well. Together we are stronger. Saving lives through hope.