All about Noah

As my first post, I wanted to explain a bit about Noah's background, since I will be frequently discussing his health issues. It is my hope that our story will provide insight, hope and knowledge for those going through a similar journey, raising a child with chronic health issues. My next blog, I will go into detail about Layla, who thus far, is healthy (thank goodness!). A fair warning that this will be long - and poor Layla's will be short (but in her defense, she is only 6 months old!)

Keith and I had been married for 16 months before we became pregnant with Noah. It was a bit of a struggle, since I suffer from polycystic ovarian syndrome (PCOS) and thus don't ovulate on my own. We sought out assistance from a fertility clinic and, with the help of Clomid, we were able to conceive Noah on our first round of medication.


Noah's pregnancy was not without its trials and tribulations: at 17 weeks when I was placed on bed rest for a heavy bleed which was the result of a subchorionic hematoma. At the time, we were not sure if we would carry Noah to term. So I was considered to be suffering from a "threatened miscarriage". However, at 22w I was able to go back to work, with limited activity because the bleed had diminished in size. We had many, many ultrasounds as the result of the bleed, fertility treatment and the normal ultrasounds that are scheduled during a pregnancy - but none were able to catch his multiple heart defects. During every ultrasound, being a cardiac nurse, I would ask them to show me his four ventricles - every time I saw them I breathed a sigh of relief. However, I do feel on some level that I knew something was amiss: I would frequently comment to my coworkers, "My biggest fear is that this baby will have a heart defect"; or to Keith, "something doesn't feel right - I don't know what or why...but it doesn't feel right" (after which he would get upset because there was nothing he could do to make me feel better and I had instilled a dose of worry into his day).


When I was overdue by a few days, I was induced for vision disturbances. All they had to do was break my water, and after that I did the rest on my own. After about 6 hours of back labour with a non-functioning epidural, Noah arrived - a healthy size (8lbs 12 oz; 21 inches). His breathing was a bit laboured, but after a nursing session and some kangaroo time, he calmed down. Everything appeared normal.


Before we were discharged, a pediatrician came to see him to check him out before we were able to leave. The doctor had difficulty finding his femoral pulses and commented on it. But then he seemed to find them and all was ok. I did however comment to Keith that it bothered me a bit. Keith also noticed how cold his feet were all the time. The medical team passed it off as immature circulation, which, I suppose could be a fair explanation...just not the only explanation.


At home we did ok - breastfeeding was brutal because of the pain, but he seemed to have normal energy levels and nursed constantly...literally, all day and night long! Being paranoid, I would listen to his heart daily because I knew that if he did have a defect, symptoms would appear around the time the patent ductus arteriosis (PDA) closed. This is the vessel that allows fetal blood to flow between the aorta and pulmonary artery, which closes about a week after birth. I didn't notice it close, but I did comment to the family doctor that I still heard a murmur when it should have been closed. She thought it was merely the PDA remaining open. 


Around 6 days after his birth, Keith sat and watched Noah sleep. He commented that he really seemed to struggle to breathe at night and was visibly concerned. I told him we would watch it and then bring it up to the doctor at our next visit. Over the next few days, Noah's breathing became faster, but it wasn't until my grandmother pointed it out when she was holding him. Once she pointed it out, it was all I could notice. He was breathing over 100 breaths a minute at rest. Normal is about 60! He literally appeared to be panting like a dog. 


I mentioned it to the doctor and to telehealth on the phone. They both brushed it off (likely thinking I was just an anxious first time mom). I turned to Dr. Google, and everything kept coming up as a heart defect of some sort. 


By 10 days after his birth, my mom called me and I was in tears with worry. She said, "If something doesn't feel right, just go to the emergency...you will feel better for trying". At this point Noah had significant indrawing with each breath and his breathing just seemed to get faster by the day. 


When I worked at the bedside, I worked in pediatric cardiology at the children's hospital in town - and I knew their emergency room would be swamped. So Keith and I decided to return to the hospital I gave birth at, because they had an urgent care clinic with no line up. When we got there we were approached in the waiting room by a nurse who informed us that they don't see infants once they are discharged because the children's hospital was down the road. She looked at me and asked why we came there. I explained our reasoning and she said, "Your son is in respiratory distress...you will be seen immediately". It was then that we both realized that he really wasn't well - but with what or why was yet to be determined.


The nurse was right, we were seen very quickly. After an x-ray, Noah was diagnosed with wet lungs, likely from pneumonia after birth. He was started on triple antibiotics and was going to be admitted for 10 days. I burst into tears...this isn't how you are supposed to spend the early days with your baby and 10 days seemed SO long. I asked him to do an echocardiogram to check the heart, just to make me feel better. He said, "We are aware you are a cardiac nurse...when he is on the floor we will get an echo done". That made me feel better. 


As we were waiting to go up to the infectious disease floor, the doctor and resident from that ward came to check out Noah before admitting him to the floor. They asked a bunch of questions and probed deeper into the situation. They then were very smart and did a 4 limb blood pressure. As soon as I heard that the leg and arm differed greatly I started freaking out saying to Keith, "Oh my God...he is a coarct....he is a cardiac kid". Keith, of course, had no clue what I was babbling about. 


Shortly after they did an echo. The doctor doing the echo was teaching a few other residents and was speaking out loud. He pointed out the coarctation of the aorta (basically, a kink in the aorta) and then he started saying more to them. He pointed out a ventricular septal defect as well! I burst into tears and my husband kept asking me, "what does that mean? What does that mean?". The residents looked alarmed and the doctor doing the echo merely passed it off, "Oh, she is a cardiac nurse". I heard it all and knew our situation was getting worse and worse....with a longer and longer hospital stay. Certainly longer than the original 10 days! I tried to explain as best I could to Keith (even though this wasn't my job!) and I think he just felt so confused and upset. I can't imagine how it would have felt because noone was explaining anything to him!


After the echo, they let me nurse Noah, even though he was breathing so fast, and they made plans to admit us. I realized then that we weren't going home until he was repaired. They wanted to admit us to the cardiac critical care unit and I begged them to admit us to the ward instead. They agreed to place us in the step-down unit. By now, it was 4am (we arrived in the early evening the night before). By 6am we were on the ward and Keith and I were settling in. I just nursed Noah and the nurse came in and said, "What are you doing? You can't feed him...he may go to the OR today!" That was it....we both lost it. It was too soon after hearing the news. After a lot of talking and negotiating, I chose my surgeon (the best in the hospital!) and he agreed to take the case. Thank goodness he is cautious because he wanted more echo's and an MRI done on Noah. 


Once things settled down, Keith and I were given a patient room on the ward to sleep in, since the ward wasn't busy. We laid down and held each other while we cried. It was the most pain I have ever felt in my life. Little did we know, there would be so much more to come.


After the next few days, Noah was sedated and intubated for the MRI, he had multiple echo's and we spoke to countless doctors. Apparently his case was more complicated than we originally thought. The gist of it is that he had a hole in the ventricles, one in the atria and he had the kink in his aorta. The VSD was not clear cut - as it rode over to the side a bit and started to take over the aortic valve - which had only 2.5 leaflets, rather than 3. The surgeon told us that Noah's valve was borderline in size, so he may not be able to do the simple repair he wanted to do. Instead he told us he didn't know what he was going to do. After conferencing with the doctors and surgeons in the hospital we had three options:


1. Fix the VSD and ASD with a patch, repair the coarct, and hope that the valve grows with him, allowing the increased bloodflow (from having the holes patched) to stretch the valve a bit more so it functions better - this was the ideal situation
2. Do a Ross procedure - Basically taking his pulmonary valve and putting it where his aortic valve is.
3. Make him a single ventricle - which we didn't want, since he had two normal ventricles. We were given a mortality rate of 50% with this surgery.


So on May 27th, 2008 - 17 days after his birth, Noah had his first open heart surgery. He went in around 8am and was done by 1pm. People came to visit us, some even brought food. I fell asleep on Keith's lap and stayed there for nearly the whole procedure. The situation was out of my hands and I felt I could rest with some peace at this point. At 1pm I went to pump (my milk supply was taking a huge hit between Noah not eating, stress and not pumping enough) and as I walked out, the surgeon walked in (I had just missed him) and he called Keith to the conference room - with a very straight and a blank facial expression. Poor Keith had to talk to him alone but the news was great...he was doing well but they were taking their time closing him up because they couldn't get the bleeding under control.


We had agreed to allow Noah to be part of a few studies, one of which had him externally paced, even though he didn't need it. So he had four pacemaker leads, two drains and many IV's. He stayed in the ICU for a week. 


A few days before our anniversary, Keith and I left the hospital for the first time to eat at a restaurant, as a friend had graciously given us a gift card to this restaurant. When we got back, we learned that Noah had been extubated. When we walked into the room, Noah was struggling to breathe, experiencing stridor and head bobbing. I freaked out - mostly because his nurse was not with him at this time, and no one was doing anything. Stridor is basically an inflammation of the airway - and the doctors came in and wanted to reintubate him. I flat out refused. They did not like that, because if they reintubated him, they could control his blood gases and breathing easier. However, my argument was that his airway were swollen enough from the extuabtion - how did they think they would get the tube back in there without doing more damage? Keith and I had heated arguments with the doctors over this but we wouldn't relent. The doctors kept telling us to go to bed (it was the middle of the night at this point) but we refused because we knew the minute we left the room, he was getting reintubated. Instead, we agreed to CPAP to assist his breathing. We sat by him the whole night until the staff physician came on during the day and looked at him and gave a "so-so" gesture with his hand. He asked why noone had given him dexamethasone - a steroid that would diminish the swelling of his airway. We were furious - he had a medication that could have fixed this problem hours and hours ago and noone thought to give it?!?!


By the time he got the medication, his breathing was improved but by that point, the damage had been done to his blood gases and we had to spend another few days in the ICU waiting for it to correct itself. Keith and I sat by his bed the next day, with me sobbing and asking the nurse, "Why does noone care that our baby is dying?". This event was so painful that I still tear up thinking about it. We really thought we were going to loose him that day.


Shortly after we got Noah's breathing under control, Keith started to worry that he had gone without food for so long. It had been a few days, so after speaking to the nurse, we got him some TPN - that is, nutritional supplementation via an IV. He was only on this for 24 hours before I tried to nurse him. Once he got the nutrition, he seemed to improve significantly. It was hard to get nursing started again, after the break and with nasal prongs - it all seemed so hard! It wasn't until Keith suggested we try nursing without the nasal prongs, that we seemed to get the hang of it. We were also battling a case of thrush, so that complicated matters further. With the help of nutrition, he got better so much faster, so we were able to get up to the ward after a few days.


After arriving on the ward however, we were home in a couple of days. Noah had developed a blood clot in his femoral artery so I was giving him twice a day injections of blood thinners and he had some reflux as the result of the surgery and being intubated - but otherwise he did quite well. We didn't have to endure any parental education, so we were home a day or two earlier than normal. He was back to breastfeeding but since he hadn't breastfed in weeks, and my milk supply was very low - we had a rough go of it. It wasn't until he was 4 months old that we got into the swing of things. He ended up nursing until he was 16 months old - when he self weaned one day, likely because I was pregnant with Layla.


Time passed and Noah grew and got quite chunky (an abnormal situation for the cardiac child!). He was doing well and we were told by the cardiologist that his long term prognosis was that he was "just like a healthy boy". 


Noah's health overall was great - however, after a set of immunizations, he seemed to be having absence seizures - they were so freaky. His eyes would roll back in his head and he would zone out for a few seconds. They happened over and over and over - probably 100x a day. We had a neurology check and all turned out ok and the episodes stopped eventually. We still have no idea if they were truly seizures or what really caused them.


At his one-year check up, he was doing very well but we had to book a follow-up for 9 months later because there was some muscular crowding occuring below his aortic valve. He was in no failure at all, but the muscle bundles would have to be removed eventually. Otherwise, his aortic valve was holding its own and his patch repairs were holding up nicely.


We just got back from the 9 month check up - unfortunately, his surgery will be sooner rather than later. The crowding has grown some more but he is not yet showing signs of heart failure. We were also told the valve isn't holding up as well as we had hoped, so he may still have to have the Ross procedure done, or have a mechanical valve inserted. Keith is researching this a lot (because that is what he does), but I am trying not to think about it because it upsets me that Noah now needs at least two more surgeries. Also, since they believe the tissue under the valve to be muscular, rather than fibrous tissue, it will keep growing back - which means that he will need many more surgeries over his lifetime. News we could have done without.


Regardless, Noah is a strong and very healthy looking 2.5 year old boy. He is tall and heavy for his age - in a good way, of course! He keeps up with the other kids at his daycare and has the nicest personality you could ask for. He is well mannered and at times acts older than his age - which I suppose is what happens to chronically ill children. He amazes us more and more every day. 


For now I will leave you with pictures of our gorgeous boy. He is truly a miracle - speaking of which, that is where his name came from: through all the trials we endured when pregnant we chose the name, "Noah Kayden" which translates into "Peaceful fighter". He is the most peaceful child you will meet and he truly had proven himself to be a fighter. 

Noah at birth

The night before his surgery

About two weeks after surgery

1.5 months old

2 months old

First Halloween

6 months old

First Christmas

9 months old

First Birthday!

1 year

Apple picking with mommy: 1.5 years old

Second halloween: 1.5 years old

Enjoying the snowfall: 1.5 years old 

Playing basketball: Nearly 2 years old

On the playground: 2 years old

Second birthday!

Apple picking with daddy: 2.5 years old