CDH International’s featured patient of the week is Kieran Johnstone. Kieran is the son of Melissa Van Tassell and Justin Johnstone. Melissa shares Kieran’s precious journey with CDH below:
“Our journey began like most other pregnancies, we had a sense of confidence and naivety that everything would go perfectly as planned with no complications although I stayed conscious of my diet and exercise having previous high risk pregnancies. The first few months of our pregnancy were like textbook. We had our first appointment and ultrasound and saw our baby’s heartbeat and movement. Everything seemed fine as we anxiously awaited our 20 week ultrasound to see the anatomy of our baby up close and the growth since our prior scans. We opted early on not to find out the gender of our baby and wanted to be surprised so we didn’t even go into our 20 week anatomy scan anticipating or expecting to find out our baby’s gender.
Our technician went through different areas of the body talking about and measuring each area, seemingly non chalant. She went over the stomach area and commented that it was farther away from the bladder and that the heart was more central in the chest. Then she went over the same areas again; heart, chambers of the heart, stomach, lung tissue. Once again, over the same areas. She measured the remaining areas and then had stepped out of the room and said that the doctor would be in to see us shortly. We had our ultrasound done by Maternal Fetal Medicine as a lot of expecting parents do and didn’t have any cause for alarm that something was different. That’s when after what felt like an eternity of waiting, one of the doctors came in and explained that it appears that our baby’s heart is more in the center of the chest and that the stomach is up higher than they usually see. They explained that it could be a possible diaphragmatic hernia but the calm way in which it was explained didn’t cause us instant alarm or concern. The doctor proceeded to measure and scan again and look over the same areas of concern and suggested that we come back in a few weeks after more growth to confirm if that is what we were seeing on the ultrasound. We were advised to not look on the internet at the possibilities as the information online can be overwhelming and to wait until our next scan to confirm the diagnosis.
We left feeling confused, scared, unsure and lost. I immediately started finding everything on Google that I could on CDH and it was devastating. I couldn’t believe that this is what they were talking about with our baby. Our baby that was moving and growing, seemingly fine. How could this happen? Was it something I did? Why were the doctors being so calm when this is something that we wanted answers to NOW? Justin was very calm, or certainly appeared to be that way initially. I was a panicked, nervous, scared wreck. We both had a lot of questions and not enough answers to satisfy a situation we knew nothing about and we had to just go home and try and process everything. We talked to our parents and explained, as best we could, what we had been told and that we would be following up in the next few weeks to confirm the diagnosis.
At 22 weeks pregnant it was confirmed that our baby had a Congenital Diaphragmatic Hernia. We had a genetic counselor talk to us along with the Maternal Fetal Medicine doctors and they went over what our “options” were. We knew that we wanted to have as much time with our baby as we could. Even though we were told there was a 50% chance that our baby would not survive if he made it to term. We were told that we would be working with Maternal Fetal Medicine for the remainder of the pregnancy as well as being contacted by the fetal center at the hospital we would have to deliver at. We had to change hospitals to where there was a Level 4 NICU that could manage our baby’s care. Our whole world was rocked and any sense of “normalcy” about our pregnancy quickly disappeared.
I also had a complete placenta previa and was hospitalized at 23 weeks pregnant from some bleeding. I remember being told that we wanted to try and keep our baby in utero until 24 weeks as the viability and odds would be better for our baby to survive. I couldn’t believe what we were hearing. I was put on bed rest and given medication to help develop the lungs and protect them and was told that I would have another shot in a few weeks for the same thing. I was discharged when we hit 24 weeks and was terrified to go back to work or do anything for fear it would harm our baby or shorten our pregnancy. As much as I wanted to try and predict our outcome or influence our outcome, I really had no control over anything and had felt completely helpless.
We had a fetal MRI and many appointments and ultrasounds to follow along with twice weekly visits with maternal fetal medicine for non stress tests and to check the volume of amniotic fluid. It felt like a full time job and the stress was tremendous.
Through the course of my google searches, I found an organization called CHERUBS and for the first time read information that was actually helpful. I mostly read information and made a list of questions for our doctors and nurses. Almost daily, I would add another question to the list. Sometimes we got answers and sometimes the response was, “We really don’t know until the baby is born.” That didn’t feel good enough, we wanted reassurance and there were many days where there was none.
On October 25, 2017, we met our team at the fetal center at the children’s hospital in our area. Although we met with a number of specialists and surgeons, we actually had a plan of action for our baby once born and a process for what would happen in the days, weeks and months to follow. We were told not to expect to hear our baby cry or to hold them and that they would have to take our baby right away and intubate within two minutes. They all spoke confidently of their abilities to prepare for our situation as they had seen other families with CDH also. It was reassuring to say the least and we are extremely grateful. We were tentatively scheduled for induction the week before Christmas in 2017.
We celebrated Thanksgiving in 2017 with an air of optimism as we had made it to our goal date and our baby was continuing to grow well and my amniotic fluid levels were remaining consistent. Every day was filled with anticipation, but we couldn’t help but be anxious and sad that we knew we wouldn’t just be coming home with our baby. We were told to plan on 1-3 months minimum in the NICU if our baby survived and to expect a roller coaster. We had no idea that would just be the beginning.
We had a baby shower with our close friends and family and continued to try and be as positive and hopeful as we could be and to enjoy every moment we had with our baby.
We celebrated Justin’s birthday the weekend of December 3, 2017 and on the morning of December 4, 2017 at 2:30 am in the middle of a snowstorm, I woke up to my water having already broken and knew it was time to begin our next chapter of our journey. I woke up Justin and we were scared, excited and nervous. After calling our designated hospital, we were advised to go to our local hospital to verify that my water had broken and that they would transport us if necessary. I knew based on the amount of fluid and it not stopping that it was not just a nighttime accident. At about 6am we headed to our hospital about 20 miles away as we knew it would be a long drive with the snow. I put headphones in and listened to music, stayed calm and prayed as hard as I could for our unborn baby. I wanted to have as calm and peaceful of a labor and delivery as possible since there was so much out of our control.
I was given Pitocin about 12:30 pm and called my older two kids schools to excuse them for the day while I waited for the contractions to start. Our maternal fetal doctor and the surgeon we had met at the fetal center were all on call that week so they were at the hospital when we arrived. By 4pm, the contractions were strong and labor was progressing well. Justin applied counter pressure while I breathed through each contraction, staying as calm as possible and trying to remain focused. Our labor and delivery suite had a window directly attached to the NICU where our baby would be passed through once born. I was checked just before 7pm and was told I was complete. When Justin asked what that means, the nurse told Justin, “That means it’s time to have a baby.” He furiously messaged family that he had been keeping updated throughout our labor and was then by my side again.
On December 4, 2017 at 7:27pm, our son, Kieran William Johnstone was born. Justin announced that we had a boy. Kieran let out two little cries and I was able to touch his head and his hand. Justin cut the cord joining us and helped to quickly pass him through the window to the NICU. I hadn’t even seen our son, but I heard his battle cry and I knew he was ready to fight. One of the NICU nurses took Justin’s phone and took a few pictures so I could finally see him. I remember it being so quiet after that. There were so many people in the room, and then it was eerily quiet and we both had a sense of emptiness.
We were told the goal was to get Kieran stabilized and he was on 100% oxygen and heavily sedated when I saw him hours later. He looked so perfect, flawless. How was this tube down his throat breathing for him and the machines? There were so many machines. We were told not to touch him too much so that he wasn’t overstimulated. I just wanted to pick him up and hold him, and I couldn’t. Justin and I had to take turns visiting to limit the number of people in the NICU. We were told that he was very sick and that he wouldn’t be transported to the children’s hospital until he stabilized.
On December 7th when I was being discharged, we got word that Kieran was going to be transported. I called Justin at work and told him they were moving him. He was trying to save up his time off until we brought Kieran home and was juggling his time between work and the hospital. There was an incredible amount of people involved in transporting him and he amount of equipment involved was terrifying. I couldn’t believe our little boy was in this bed covered by machines. I was 3 days postpartum and was walking quickly through the long hallways to follow our son. The cramping and bleeding that I experienced was a reminder that I needed to take it easy, but I wasn’t worried about my recovery, only about our son. I was pumping around the clock every 2 hours to try and provide the only help that I knew how for our son. I was exhausted, emotional and in recovery. Justin was exhausted, emotionally drained and we both were scared.
Kieran stabilized quickly and we were told that he would be getting his repair surgery on December 8th. We were shocked but told that was just the beginning. His surgery went quickly and the surgeon told us that Kieran’s defect could be repaired using existing diaphragm tissue. He had intestinal tissue in his chest but all other organs were below his diaphragm. They told us the next challenges would be feeding and breathing and that it would be a log road.
I first held Kieran at 6 days old, Justin at 8 days. We watched machines breathe for our son, we watched medicine pump through his body while he was sedated. Justin changed his first diaper. We both sat with him, talked to him, sang to him. But it wasn’t home. Kieran had many ups and downs but continued to surprise the doctors and nurses. With every milestone goal, he surpassed it. He continued to work on his feeding, both breastfeeding and bottle feeds. The emotions, cannot be put into words to those that haven’t lived it. There were so many highs and lows; it was exhausting trying to function ourselves, but we were driven by our little boy and his fighting spirit, his will to live. Kieran slowly weaned off of medication and pain meds, began growing and breathing better. We were told a few days before Christmas that if we were willing to continue his NG tube for feeding and oxygen, that we would be able to bring Kieran home, Justin scrambled to get his crib put together as we weren’t expecting to bring him home so soon.
We brought Kieran home on December 23rd and had our other home medical equipment dropped off that day. He had no medication but he did have oxygen and a feeding tube. Kieran needed to eat and I struggled to remember how to hook up his feeding monitor and the bag with the milk and formula. I couldn’t just nurse or bottle feed our baby. I cried. Justin and I felt helpless once again.
Kieran developed an infection at his incision site and was back in the emergency room on December 24th. Our surgeon was once again on call and we were able to come back home with antibiotics and a scheduled follow up instead of re admission into the hospital. We had visits from nurses, occupational therapists, and physical therapists in that first weekend many that followed. The pulse oximeter was constantly beeping or sounding alarms, the oxygen on low flow continuously night and day, I was still pumping every 2 hours. It was challenging, but he was home. He thrived.
Over the course of his first two months, he came off of his feeding tube, and was cleared of any pulmonary hypertension and no longer needed oxygen. He was nursing and bottle feeding well. Cardiology cleared him. His X-ray from the pulmonologist post surgery showed he had much more left lung tissue than anyone anticipated and we were told we would revisit at 5-6 years of age with pulmonology and annually with surgery.
Kieran met all of his developmental milestones and became quite the foodie. At about 5 months old, we took Kieran to a CDH activity and for the first time we met other families in person that knew what we were experiencing. There was so much love and support, and we finally felt a connection to other families.
Kieran showed off his zest for life and enthusiasm for play, it became apparent how much he loves with his whole heart. He loves people, pets, and life in general. He’s taught us patience and to live in the moment. He loves his older brother and sister and very much relies on them for play, love and support.
Kieran experienced mild colds and respiratory infections off and on. Overall, he was healthy and growing well. We celebrated his first birthday with joy and a newfound confidence that he was doing well. We found out in June of 2019 that we were expecting again and were cautious and nervous, anxiously awaiting our 20 week ultrasound to see if we would be experiencing this all over again.
In late August of 2019, Kieran became sick. He had a respiratory infection, then a suspected ear infection and this went on for a couple of weeks, off and on. The second week of September 2019, I had Kieran at the doctor 3 times within a week as he had started vomiting and was increasingly becoming weaker and more lethargic. Everything he ate or drank, he vomited. He had an X-ray on September 13 on his chest that came back normal but the doctor said if his vomiting continued into the next day, to take him back to the ER.
On September 15, 2019, Kieran’s vomiting became so frequent that even without food or water he would still vomit. No fever, no diarrhea, just vomiting. He would lay down and if he did walk, he was swaying and unstable and would then lay again. I was terrified. We knew something was not right. I brought him into the emergency room that night and the battery of tests began. Stomach X-ray, abdominal ultrasound, CT scan on his head, 2 sets of labs, urinalysis, multiple IVs due to dehydration, his vomiting was up to 6 times a day whether with or without food or drink. I pushed for a CT scan on his abdomen to rule out re herniation thanks to another CDH mom and our state rep that I had met through CHERUBS. There were no answers. Kieran was weak, exhausted; his body wanted to fight and move but he couldn’t. He just laid there and moaned. We were transferred to the infant unit after being in the ER and transfer unit for over 2 days. We already had surgery come and consult, then were advised it was not a surgical case.
Kieran was losing weight fast, over 2 pounds in less than a week and getting weaker. We were told there was a suspected paralytic ileus as a result of his respiratory infection a few weeks prior, that his intestines fell asleep and that they basically needed to wake up to start working again. He had an Andersen tube placed to start sucking air and fluid from his stomach. He had a PICC line placed to give him nutrition. I stayed with Kieran during the day, Justin came up after work and then he stayed with Kieran on the weekend while I worked. Kieran continued to worsen and surgery was called again.
On September 21, 2017 after I got home from work and prepared my bag for the week at the hospital, Justin called and told me surgery wanted to meet with us. When I got there, I was greeted by Justin and one of the surgeons from the team we had during Kieran’s repair surgery at 4 days old. He told his after doing another X-ray that he felt like Kieran was not improving as he should be that he felt it would be best to do surgery. We could either opt for that night or the next day. We just wanted Kieran to not be in pain anymore. We opted for surgery right then. Justin and I were terrified. We called both sets of grandparents and gave them the news. No one slept after that. The surgery revealed that Kieran had a bowel obstruction caused by scar tissue from his original repair surgery at 4 days old. He had multiple spots of scar tissue and a large band casing the blockage. The surgical team was able to preserve all of his intestines and remove the scar tissue. Now we just had to wait for his bowels to wake up and start working. Recovery was awful. He had breakthrough pain, was weak and on morphine and other medication. He couldn’t eat or drink anything and was starving. He didn’t want his sponge baths or to be moved. We met with physical therapy, pet therapy, and tried toys and play to help lift his spirits.
We were able to take him for a walk on September 25, and in true Kieran style, he was talking to nurses and patients, happy to be out of the room to walk and explore. Although he was unsteady, he was motivated. He had his Andersen tube clamped on the evening of September 25th and went 24 hours with no vomiting. By September 27, he was able to start drinking apple juice and popsicles. He couldn’t have been happier! That afternoon and the next day, he was allowed to eat soft foods. He loved his macaroni and cheese, applesauce and of course, more popsicles. He enjoyed his nurses and their efforts to entertain him. He would smile and tell them all kids of things, especially loving the Voicera that the nurses carried that played Elmo, Mickey and Cookie Monster among others.
On September 29, 2019 we brought Kieran home after 15 days in the hospital and he was as happy to be home as ever. He missed his kitties and his brother and sister, he also missed eating real food.
On October 8, 2019 our 20 week ultrasound revealed a healthy and CDH free baby measuring right on schedule. Kieran became a big brother on February 8, 2020.
We don’t take a single day for granted, we appreciate every moment. Kieran has taught us that. He loves unconditionally and fearlessly, he’s an amazing big brother as well as little brother, and we are incredibly grateful for our CDH extension family and all of their love and support. Although we don’t know what the future holds for Kieran, our hope is that through his story and in reaching out to other families and participating in research studies that we can see a cause and a cure for CDH in his lifetime.”
