Noah, 10 months, Anchorage AK | Congenital Heart Defects (TGA)

NOAH, 10 months, Anchorage, AK | Transposition of the Great Arteries
written by his mother, August
photographed by The Birth Photographer 

Being pregnant was the best feeling. Sure, I was sick for weeks on end but I didn’t care because I knew that meant my baby was healthy. This was our “rainbow baby” (meaning a baby that is born following a miscarriage, stillbirth, neonatal death or infant loss) so I embraced every “annoying” thing that came with pregnancy. When we went to the ultrasound at 20 weeks we found out we were having a boy. He was very active. We tried everything possible to try and get him to turn for pictures of his heart but he wasn’t really having it. After an hour-long ultrasound, the technician decided they had all the pictures they needed.

At our 32 week ultrasound, our baby measured average on everything. Three days before my due date, we were just unloading our last load from moving before we decided to take a dinner break. I was walking outside and my water broke! Ten hours later, my beautiful boy was born. He was a healthy 7 pounds 10 ounces and we named him Noah Charles.

A few hours after he was born, I had mentioned to one of the nurses that his hands and feet looked pretty purple still but she assured me that was pretty normal in newborns since the blood disburses to other more important parts of the body first. About five hours after he was born, a different nurse noticed his dusky color and she didn’t like it at all. She asked me to follow her to the nursery with Noah just to check on his oxygen levels. His oxygen levels were in the 70’s and even with oxygen would only go into the 80’s.  We got my husband and brought Noah down to the Neonatal Intensive Care Unit (NICU). I was hoping that he just had a small amount of fluid in his lungs and that’s what was causing this.

When we got to the NICU, they said they do two procedures when babies are having trouble bringing their oxygen levels up: draw blood and do an echocardiogram (ultrasound of the heart). As we were sitting there waiting for an answer, a nurse came up to us and wanted to know if either of us has family in Seattle and Portland where the hospitals were. We didn’t know what she meant because we hadn’t been informed Noah needed to be moved. She got chewed out for it by her superiors but we weren’t mad or upset about it.

Dr. Wellmann came over to where we were sitting and explained to us that he found something on the echo. Noah was born with a heart defect called Transposition of the Great Arteries (TGA) and he also has a hole in the lower septum of his heart (VSD). He said they would have never known he had a heart defect because babies born with heart defects are usually severely underweight. TGA is a defect in which the arteries of the heart are switched so the oxygenated blood just goes in a circle while the deoxygenated blood goes back out to the body. This required an operation called an Arterial Switch. 

Dr. Wellmann was awesome and let us know that after his surgery, Noah could live a relatively normal life. We were still scared. How was it possible that this perfect child had a defect of any kind?

That night Noah was flown out to Seattle on a medivac. I had family that was able to meet him there so he wasn’t alone; I couldn’t ride with him and I couldn’t go on a commercial flight alone. My husband and I flew out a few hours later. I was so excited to finally see him but he definitely looked different, which they warned us about. He was extremely puffy from the sedative he had on the plane and he had a breathing tube in. I probably cried the hardest after they took out his breathing tube because he tried to cry but it was muffled and his voice was hoarse. It was hard seeing him that way. The next few days were just the waiting game until his open heart surgery. The cardiologists at Seattle Children’s Hospital actually found that Noah had what was called Dextro-Transposition of the Great Arteries (D-TGA) so his surgery might be about eight hours long instead of the projected six!

Noah was born on Friday and his surgery was scheduled for Monday. That day we were worried and everyone seemed to be fairly silent. We had planned on waiting to get him baptized until he was at an age to ask us, but we had a priest do it right before his surgery. I never knew it was something I actually wanted to do but I bawled the entire time. The priest was so nice and checked on Noah throughout his journey.

Soon, the date of the surgery came. They told us to say our goodbyes and give him love. They gave us the option of walking back with him but I couldn’t do it, so I gave him a teary kiss instead. It was too hard to walk back with him, let alone wait for the surgery; that was the longest six hours of my life! We were supposed to get updates throughout his whole surgery but something happened and we never received anything. I finally had my husband ask someone because, at about five hours or so, we had heard absolutely nothing. They told us that he actually did great and they were just working on stopping bleeding before they stitched him back up. In that same moment, we found out that my great uncle passed on. I believe that possibly his life ended so Noah’s could continue. A little while later, the heart surgeon came to talk to us and told us that Noah did amazing. The relief that we felt in that moment was probably something we will never feel again.

The first week or so was trying to get him to breathe on his own without the breathing tube. They would have specialists come in and try but it wouldn’t work and they would have to put it back in. Eventually, he got the breathing tube out but still had to have an oxygen cannula (tubes delivering oxygen through the nose). Once his breathing improved, the next few days were spent trying to get him to eat. As soon as he would latch on to me or the bottle, he would fall asleep. He really excelled in everything that was expected of him eventually. After almost two weeks of being in the hospital, we were released. He was on two diuretics and we had to fortify my pumped milk with formula to help him catch up on gaining weight. He only needed these things for about a month. After that, he was just a normal baby. He caught up on gaining weight going from the 8^th percentile when we got out to being in the 80^th percentile now at 10 months old.Before his surgery, we were told it was very possible that he could experience developmental delays. I know he is my first and only child but I truly believe that he is very smart for his age and he is hitting all his milestones when he is supposed to achieve them. 

He is 10 months old now and he is a huge blessing to anyone with whom he comes in contact. If you didn’t know him or his story, you wouldn’t know that he had a rough start. Noah can put a smile on anyone’s face. I am so glad he’s a happy baby after everything he went through. I am a firm believer that a stress free pregnancy equals a happy baby so maybe that’s the reason his condition went undiagnosed. Since we have had a baby with a heart defect, we have a higher chance of our other kids experiencing some sort of heart problem, so I will have to see a specialist for my future pregnancies.If there is anything I want people to take from this, it’s to make sure the ultrasound technician gets a good picture of your baby’s heart. If your baby isn’t cooperating, maybe try rescheduling. You could even go as far as asking for a pulse oxygen level on your baby once there to make sure their oxygen levels are good. I didn’t realize until this all happened that 1 in 125 babies born are born with a congenital heart defect. That statistic means this is very common but I never thought it would have happened to me, nor anyone I knew. Thank you for taking the time to read Noah’s story!

HANNA, 11 (Kodiak, AK) | Kabuki Syndrome, Epilepsy, Autism

HANNA, 11 (Kodiak, AK) | Kabuki Syndrome, Epilepsy, Autism

written by her mother, Kathy
photographed by Breanna Peterson Photography 

Even after 11 years, I remember the day Hanna was born so freshly in my head!

We had a little bouncing baby girl. She was 5 pounds at birth. We came home from the hospital a day later.

Boy, were we in for it. Hanna decided to only sleep in in 20-minute intervals. She also didn't eat correctly…barely eating half an ounce at a time.

When Hanna was 6 months old, we started receiving referrals to different doctors frequently…it seemed almost weekly. We live on an island, so having to travel to Anchorage each time came with its own set of challenges.

By the time Hanna turned one, she wasn't even crawling, let alone walking. We were referred to Infant Learning Program, and then occupational therapy and physical therapy.

As first time parents, we were overwhelmed. Then, out of the blue, Hanna started having seizures. An emergency trip to the pediatric neurologist was then needed, where Hanna was diagnosed with epilepsy.

Well, things didn't end there. We were referred to Seattle Children's geneticist. That trip came to last about 3 years back and forth. They did test after test, then say she had this and that. But I didn't agree!

Finally, on the 3rd year, Hanna’s test came back and she was diagnosed with Kabuki Syndrome. All these weird names were just thrown at us. To us, it was just a name. We loved our daughter dearly just like any other child.

Kabuki Syndrome is a rare, multisystem disorder characterized by multiple abnormalities including distinctive facial features, growth delays, short stature, and other varying degrees of difficulties.

Boy, we have been through the ringer. Just last August, we saw an allergist. Hanna has suffered allergies all her life. But we were there for 6 hours and found out that Hanna has no antibodies, which produce B cells. Hanna would get sick with everything so easily. We also found out that we had to learn to give Hanna weekly IGG infusions (immunoglobulin therapy). We wondered what else could be thrown at us!

Immunoglobulin therapy is the use of a mixture of antibodies (immunoglobulins) to treat a number of health conditions

Hanna is now 11 years old and has defied many odds! We love her to the moon and back we always tell her so! She receives services through Hope Community Resources, has weekly occupational and physical therapy, and we are part of an online Kabuki Syndrome support group.

Hanna has many diagnosed syndromes, but that doesn't stop her, or makes us love her any differently! She is our Alaskan Warrior child!