Do you ever wake up suddenly by a noise and try to spring into action? It’s dark, everything is a little blurring, and your body just works on auto-pilot. Sitting in the MRI room watching Elsa was a blurry dark mess of emotions. Yet in the mess there were sparks of light, sparks of hope.
Tests came quickly and I pushed to have orders in fast for Elsa. I wanted to get as much done as soon as possible despite the fatigue. Our first night was sleepless. I was still in a bit of shock. I didn’t know when I would be coming home and I missed my boys. Around 11pm the phlebotomists came in for the first round of blood work. I helped one hold Elsa on the crib while the other carefully tried to find a vein. I had done this before and I wasn’t squeamish with needles. On a 7 weeks old, veins are small. On a 7 week old with PWS, they are even smaller. First attempt was a failure. You have to understand, due to the hypotonia, Elsa did not cry, she did not make cooing noises and she barely moved much at this point. During the first and second attempt of blood drawing she cried and riled her body like I have never seen before. During the second attempt, she cried so hard she passed out. I was about to walk out of the room as my eyes filled with tears again. I could feel the heat in my chest rise as she lay limp and tired on the crib. I might have been rude in the moment but I demanded they get the IV team to draw blood. I would not watch another phlebotomists wiggle a needle in her arm or foot again.
The IV team came in after midnight. Two nurses gently searched for a vein and found one on her ankle. She was dead tired from the previous activity. Thankfully this nurse was smart and placed an IV line for drawing blood, first attempt and kept it in for future draws to avoid more poking. Elsa barely cried, probably due to to fatigue, but this time it was much smoother.
I continued to feed Elsa every three hours with expressed breast milk. It took 30-40 minutes each feeding and positional changes to keep her awake. I slept a total of 3 hours that night, chopped into bits and pieces. I wore the same clothes from the day before, my hair in a permanent pony tail out of ease. It wasn’t more than 7am when the doctors started flowing in. Three neurologists, two pediatricians, a lactation consultant, a nutritionist, a case manager, and two nurses were on her team. Soon we would add a physical therapist, occupational therapist, and speech language pathologist.
Each doctor did an exam, the same reflex testing, the same once over for muscle tone, the same questions. Each time a doctor came in I pulled myself together wiping the tears that seemed to have a continuous flow and communicate her care and needs. The day went by quickly. Late in the evening a nurse came to escort us to the MRI room. Again, we walked the maze of hallways to a dark room. I stepped through a metal detector with Elsa. We were ready for her MRI. I helped the nurse place her on the table. She was swaddled, earplugs and larger ear muffs were placed, foam around her head, and a blanket around her again made her still in the small cocoon. She was fast asleep. I was given earplugs and told I could sit in a chair in the corner of the room. From the corner I watched her small frame glide into the MRI. The loud clanking began and continued for half and hour. It was dark. Only the red button from the exit sign, the screen showing her placement in the MRI machine, and a sliver of white under the door gave off light through my blurred teary eyed vision.
Sitting in that chair made my head swirl with emotions. I was finally alone. I wept out loud with grief, anger, sadness, and a deep pain to care for my daughter. I felt helpless. It seemed like I should know what to do to help my own child. That dark room was my safe place to let everything out. I held my emotions down to deal with all the medical stuff and to keep our normal routine at home. Not here, everything seemed to be brimming over.
It didn’t seem long until daily rounds were starting on our floor. This time, twelve doctors crowded in our small room. One read her report, the next told us the plan of action, and another discussed a naso-gastric tube (NG tube) to be placed to due poor weight gain. I immediately had questions. She was taking in three to four ounces of milk from her bottle and I was not convinced we had tried all that we could before placing a tube. I requested an evaluation from a speech pathologist and an occupational therapist. I also requested we try fortifying the breast milk. The neurologists wanted a swallow test performed first to test for silent aspiration. If she could pass this test, we would continue bottle feeds with fortified milk.
My gut was telling me Elsa could do this. She could pass this test. I had my reservations as a natural pessimist and was anxious at the thought of placing my daughter on tube feedings. I spoke with Angela, my friend from Anchorage, who reassured me it was a temporary solution until she was strong enough to feed on her own. My pediatrician called me and again, reassured me an NG tube would only be for a few months. This did calm my nerves and I was more at peace with the possibility of an NG tube, yet something in me was still hoping Elsa could bottle feed on her own.
Another sleepless night passed and a nurse escorted me down the halls with Elsa in my arms. A speech pathologist met me in the x-ray room and set up a chair for Elsa to sit in while she explained the procedure. Another technician came in to monitor the videos while the x-ray took continuous snapshots of Elsa feeding. We all donned our lead aprons and Elsa began taking the first 40ml of Barium. As she slowly drank from the bottle, the speech pathologist held her cheeks and chin to support her lips, the same as I did every three hours for feedings. I was nervous and began to pace. I think the technician could tell I was anxious so she talked me through what she was seeing. Elsa took the next 40ml. There was a short delay after some of her swallows. She held the liquid at the back of her throat for a second or two, then swallowed a few more times. She was getting tired. At the end I asked more questions than the probably liked. Finally I asked if she had passed. From their observations there were no silent aspirations. We could continue bottle feedings!
Before I left the x-ray room, the speech pathologist gave me several Dr. Brown bottles. These bottles have a vent system eliminating bubbles that create gas in babies. They also preserve some of the vitamins in the formula or breast milk. There are preemie nipples available as well as the Dr. Brown Specialty kit which contains a valve for infant-paced feeding. She drank more fluid from these bottles than I had ever seen. She had less air trapped in her belly, less spit up, and the nipple was thinner and longer which made it easier for her to suck with her high palate. I was so thankful we finally found a bottle that would help Elsa feed faster and with less effort.
Although we did not need an NG tube at that moment, we did need to continue to work on effective feedings with fortified breast milk. This was a big obstacle that Elsa overcame and one that we would continue to master with each feeding. I never knew she would need to overcome as much as she did in her short 7 weeks of life. Each day she gained weight, was a little stronger, more alert, and engaging with her surroundings. It was finally time to take Elsa home. That day in the dark, the moon reflected the light a little brighter.