First Weeks
At about 9 am, we all left to go up to Seattle Children’s to see our baby (it’s about a 2 hour drive). When we got there, the baby was in this tiny NICU room in a small bed, connected to all sorts of cords and tubes. This is my baby! So weird to think that this baby was inside me yesterday. Moving around and kicking. This baby was so small- 31 weeks old, but the head was measuring about 35 weeks. I was still in shock. That whole day was filled with doctors, specialists, and nurses coming in and introducing themselves, getting x-rays, and other tests. The “team” included the main doctor, the RN, RT (respiratory therapist), nurse, otolaryngologist, geneticist, endocrinologist, urologist, orthopedist, neurologist, pulmonologist, nutritionist, lactation specialist, and social worker. They all seemed very nice and concerned about our baby, but wow my head was so full and overwhelmed. We were also finally told her birth weight of 3 pounds 12 ounces, and a little over 15” long.
That evening we left to go home, but left for Seattle early the next day. It was another day of doctors and test. Very overwhelming, but amazing seeing our little baby! It felt like I was in this weird dream. The doctors kept referring to me as “mom” and that was strange to get used to! Not to mention I just gave birth- I was still in pain from the stitches, and being exhausted. Dustin had to push me in a wheelchair for the first couple of days throughout the hospital. I also started doing pumping for breast milk every 2 ½- 3 hours, which is a journey in itself.
That first Friday was a BIG day. We drove up nice and early and got to the NICU. A couple of hours later with our baby, we were told that our “team” wanted to meet with us. We went into a small meeting room with our team of doctors. The told us that with all of their tests and research, they had found a diagnosis- Campomelic Dysplasia. First off, this means that our baby is actually a girl! Her chromosomes still is xy, but her body is a girl. When they were explaining the logistics behind what happened with the chromosome difference, I immediately felt a spirit of confirmation come over me, telling me that this is correct. I was praying to know what her gender really was, as I had noticed during diaper changes that she looked like a girl, but her DNA said differently. I know that gender is crucial and pre-chosen and there is no way I was about to choose wrongly. No wonder it didn’t feel right about naming this baby a boy name, she is a girl! We decided her name right then was to be Emma Bianca Krall. Also during the meeting, we talked about the other characteristics of this diagnosis- she has a weakened trachea and has trouble breathing on her own. Her airway collapses without the help of the breathing tube. This diagnosis also includes the shortened/ bowed legs and club feet. She also has “dimples” on her legs where the bowed bones are- a big giveaway of her diagnosis. Along with the legs, this syndrome also includes a small chin, with something called a Pierre Robbins sequence. It was a lot to take in! They told us that we need to get her breathing under control first. If not, she might not survive. Online research told us that 5-10% of infants survive. I was trying not to think of that, as the doctors were very hopeful and confident that she would make it.
That next day we spent at home. We needed a day of emotional and physical rest. I needed to heal from giving birth, we needed sleep, and we also needed time to just digest what is going on. It was a difficult day, as it was the first time we were away from our little Emma (already the name felt natural). It was a day of rest, but a day filled with anxiety. Also guilt, as we wanted to be with her. We also needed to be home for a day to just tidy up things at home as we just dropped everything and left, to take care of our dog (that was being dog-sit by Dustin’s family), and going grocery shopping. The day after we spent with Emma though.
That next Monday, we both went to work. We both wanted to keep our jobs, and we needed the money- especially now that we have medical bills coming. That whole day of work consisted of wrapping things up. It was extremely draining- emotionally and physically. I ended up leaving my job shortly after, as it wasn’t working out living the double life. Dustin still had to work though. He worked Monday- Thursday, then spent Friday making meals, shopping, cleaning, and then coming up to Seattle for Saturday and Sunday. It was really tough on us, as Dustin felt guilty at work and I only saw him every couple of days. But that was our life at this time.
In her first days (and weeks), Emma slept a lot. She also was put under a blue light for jaundice prevention. She needed to wear a face mask so it wouldn’t get in her eyes. That was tough not seeing her eyes. Another surprise was that she moved rooms. It was a little scary at first, as we went to our old room and it was empty! Gave us a heart attack until we were told that she was a different room. A much bigger room with a large window and a nice recliner. It was nice to sit in the sun.
About a week in, we got to stay the night in the hospital. In the floor above, the were very small parent sleep rooms just for NICU parents. Down the hall are bathrooms, a small kitchenette, lounge area, and a patio. It was so nice not to drive back and forth tonight! And this is all free. What a blessing! When we saw her the next morning, she was still under the blue light. She was also put under midline restrictions, meaning that since she is so little and that her diagnosis comes with fragile bones, they ordered her neck to not be moved, which meant no holding her. At this point, I have only to be able to hold her once after she got to the hospital, and only for 2 minutes as she needed to get under the blue light again. That was a little disheartening, as I wanted to hold her and see her face. Hopefully soon. We just let her sleep.
A couple of days later, I was able to change her diaper for the first time. It was a little scary as she is so fragile, and the nurses have a certain way they change them to keep everything inline and sterile. It took a while, but I finally got the hang of it. We are also doing a little bit of her cares- taking her temperature, cleaning out her mouth (with sterile water and sponge on a stick). A couple of times we were able to give her a cotton swab of breast milk to taste. At first, she hated it then she realized that it actually tastes good, and stops getting grumpy. When she is upset, her face gets red and her eyes squeeze tighter and she moves her arms around. Since she has a breathing tube in, she can’t cry or make noises.
At two weeks old, the doctors wanted to do a trial extubation- a removal of her breathing tube and put an oxygen nose supplement tube instead. The doctor, 2 nurses, 2 ENT’s specialists, and RT’s were in the room. I was there to watch it come out. They then hooked her up to an oxygen tube through her nose. For about 5 minutes she did great and it was amazing to see her whole face and not being squeezed by tape! She was then struggling and working hard to breathe. Then all of a sudden, her stats dropped and the alarms started beeping. The doctor put an oxygen mask on started bagging (pumping air by hand) into her mouth. It covered her entire face. She started getting really red, flailing, and arching her back. The team was rushing around me and responding to the alarms. I was trying to keep my composure as I had my hand on her little body, and I couldn’t take it anymore. I left quickly out of the room and into the NICU waiting area were Dustin was. A nurse followed me out until I found him. It took a while for me to stop crying. I never want to see her like that again. After a while, the doctor came out and told us that she obviously failed the extubation, but that was okay. We learned that her trachea isn’t strong enough yet and that she isn’t big enough yet. But it is a good sign that she did okay for a little while. When we got back in the room, she was pale. It took a little longer to get her color back- I also hated seeing her like that.
The day after her extubation trial was very relaxed. Emma needed to re-gain her strength, sleep, and grow. We noticed that she likes her head and feet held (cupped around her) and her hands held. She also loves being swaddled. She usually just sleeps, but when she gets her regular cares, diaper changes, or repositioning, she gets grumpy. She also loves getting her hair stroked (she has a lot of hair so I love it too!). she is slowly starting to open her eyes a little more. She has large, very dark eyes. She is still very disoriented and can’t focus when she does open them, but she does try to look towards us when we talk or when we are near. I have sung children’s songs to her when she sleeps, and I’m not sure she can hear me or not, but it does help me feel close to her. It’s a little bit of a struggle to connect, as I can’t hold her a lot, play with her, or feed her. She is getting my milk through a feeding tube in her nose though. The longer I sit with her, the more I feel more connected.
That weekend was July 4th, so we went home to live a little normal life. We were able to see our family and go to church. It was so good to see everyone, to give updates and to thank everyone in person for their help. We have been getting cards, money, and lots of amazing meals. Our church even had arranged the meals to all be delivered on Monday night for the upcoming week as we can go up to Seattle packed with food. We are so thankful for all the support we have gotten!
The doctors wanted to trial an extubation again, after another week. We decided to both leave the room this time. It was hard to leave her when she had such a struggle the last time, and we couldn’t bear that again. When it was done, the doctor came and got us. It was out! She had an oxygen tube though her nostrils instead. She was given a sedative to keep her from squirming, so she laid there motionless and pale. Her eyes were open. She was also breathing rough- her stomach creating deep retractions. It was a little scary actually. She was in shock- I was too! She was like this for about 15 minutes (double of last time), until her stats started dropping slowly. They said they wanted to intubate her again, so we left the room. After about another 15 minutes the doctor came to get us. He said she was just fine now, and that her trachea looked good (they scoped it with a small camera), but it looks like the only reason she failed was that her chest cavity was too small. She was just working so hard to keep up the breathing that she got too tired. He said we needed to wait for her to get bigger and stronger. Overall, I guess it was good news, but I wasn’t quite sure what to feel. I felt terrible, but not sure how to shake it. We just know more now. The rest of the day went better though, as she was able to get the heat lamp turned off, as she could finally regulate her temperature! They also took her IV out finally. And Dustin was able to finally hold Emma for the first time since his hand surgery. Those were nice little victories after that morning.
A couple of days later, we had a huge scare when I was holding her. After getting set up in the recliner with Emma on my chest and all the tubes and wires gets moved over, I had been holding her for only 30 minutes (they want us to hold her at least an hour so we don’t move her as much). Then we had a big fright. Her alarms started going off stating that her heartrate was dropping, then she started breathing shallow and gasping, and then jerking movements. She started turning pale and then not moving at all. Her oxygen alarm was going off too. All this was happening all on top of me, while the nurse was trying to suction her and reposition her on my chest. She called the charge nurse over to help. Emma still wasn’t moving and her stats were dropping so they quickly put her back in bed and added more oxygen to her breathing tube. Slowly her heartrate started going back up and she looked less grey over her tiny body. I was so shaken and filled with panic. I was barely holding it together while this was happening, as I didn’t want Emma to feel my fear. After they got her comfy and breathing right, we had to get to bed as it was late, but I didn’t want to leave. The nurse told us it was probably just a big “secretion” that needed to come out that she couldn’t cough up. The next morning, I wanted to hold her again, despite what had happened the night before. Again, shortly after holding her, she had another scary episode. I couldn’t control my feelings toward the end. She had again stopped breathing and turning blue within seconds in my arms. After the nurse put her back in bed and got her feeling better again, we had to leave soon for home (for some baby showers for Emma). The nurse stopped to talk to me. She said that it is okay to cry and that I didn’t need to be strong for anyone. It will take time. She urged me to hold Emma one more time, just for a little bit as she didn’t want to leave with a bad feeling, and to have a good experience. She gave me a hug and I was able to hold Emma one more time that day. It went just fine- thank goodness! I am so grateful for good nurses at RT’s at Seattle Children’s.
She continued to have multiple of these scary episodes- some bad where she needed more oxygen or bagging, and some she got her stats up on her own. The emotional toll of each of those is exhausting. We still don't know what was happening. We feel helpless, as we don’t know how to make them stop. It happened multiple times a day, and at any time we could lose her so easily to one of these episodes.
At about a month old, Emma started receiving occupational and physical therapy in her hospital room. At this point it was a lot of just moving her arms and legs and small foot stretches. She also got her first real tub bath at this time too! The nurse held the tubes and directed us, and Dustin and I did all the work. Emma was unsure at first, but loved it! She was calm and looking around the entire time. She started falling asleep when we were putting soap in her hair. She loves getting her hair played with and her head rubbed. It was fun giving her a bath and getting some “normalcy” in the NICU. We also discovered that she loved to suck on a pacifier! It was super cute to see her sucking on it. Since she had her breathing tube in her mouth, we were somehow able to get that in there too, but had to hold it for her. She loved it so much, for a while that was how she fell asleep, so we had to hold it for her. Eventually, we found out how to prop it up with NICU positioners so we didn’t have to stand there for an hour. The nurses are very awesome too- every week or two they would make a postcard size sign for Emma with her hand or footprints on it- so small! They will be forever cherished. She also loved looking at her first stuffed animal- a tiger. I think it had something to do with the big eyes on it.
At her fifth week, her red blood count was low, so they had to give her a blood transfusion. We found out that she is difficult at getting an IV in, so they had to put it in her head. As they were putting it in, she had about 10 of her episodes where her oxygen dropped. She also had stomach issues and a low-grade fever. She had an infection and had to get anti-biotics for a couple of days.
On August 7th, ENT decided to do a camera scope down her airway as she was still having these terrible episodes. She had some pretty scary ones in the last week that needed bagging and extra oxygen. The ENT doctors were able to see that the breathing tube needed to be pushed in a little and that she has tracheomalacia- a collapsing airway. A couple of days after the scope, we decided to have a “team conference” meeting to talk about giving Emma a tracheostomy. It was a very long, but good meeting. We discussed that Emma would have to get a trach and it would happen this Thursday. She will be sedated for a week, then have a trach check procedure. The trach will be connected to a ventilator until she can wean herself off of it. But most likely this will be semi-permanent. We still won’t be able to hear her cry and her food will still be through a feeding tube through her mouth or nose to her stomach. She will need to stay in the hospital for a couple more months after this. Then we will have an in-home nurse(s) for 16 hours a day at best. It was a shock, all this information to take in, but it was really good to talk about everything openly. It felt very scary, but we did feel that it is the right decision to have Emma progress and come home. We just want her to be comfortable and happy. She is counting on us!
