The morning after the MRI results Mike and I walked into the NICU for her second morning feeding to find Dr. B, the Pediatric Geneticist. She was glad to see us as she had some questions about our family/medical histories. She measured our heads, the distance between our pupils, and generally looked for possible genetic clues in our physical traits. Sensing the mix of exhaustion and despair in the air she asked plainly, “Has anyone explained the huge range of outcomes with this diagnosis to you two?”
“Range? What?”
She proceeded to tell us that while some with this condition have severe and profound mental and physical handicaps, others go on to live normal lives. She explained how occasionally an older adult may have an MRI (perhaps to check their brain after a fall/ accident) only to find they’ve had this condition their entire life and no one knew. She also explained that per her evaluation and our family histories, she didn’t see any indication not to be hopeful. Although Maryn had already had her chromosomes checked and the results were normal, Dr. B explained the original panel was more generic and she wanted to run some additional checks.
The pediatric neurologist stopped by later that morning. He echoed Dr. B’s sentiment. While Maryn would most likely be delayed in meeting her milestones it didn’t mean she couldn’t eventually catch up. However, a diagnosis of ACC did put her at a much greater risk of developing seizures. He explaned that an infant MRI is not as clear as one of an older child so there was detail we weren't seeing that could give us clues about what her future holds. Additional brain anomalies would be an indication that she would likely have more severe challenges. But, we couldn’t see this now and it was too early for a prognosis. Only time would tell.
After a nice long talk with the new neonatologist that day we were starting to feel some hope. Maybe her prognosis wouldn’t be all doom and gloom like we were initially led to believe (in all fairness by people who just didn’t know much about the condition or at least the range of outcomes). This crazy rollercoaster ride had certainly changed pace again, but what we didn’t realize then was that it was just a short lull before the next big drop off.
Mama discharge day came and it was bittersweet. I was excited by the thought of leaving the hospital environment, getting into my own clothes and taking a shower at home, but it was so sad that she wasn’t coming with us. Thankfully it wasn’t far from our house, so we continued to be there for most morning, afternoon, and evening feedings. The inevitable routine of daily life slowly crept back in. We started referring to the NICU as her apartment. The joking helped relieve some of the emotional tension and anxiety that was always lurking around us. “Hey, what do you say we head over to the baby’s place for dinner tonight?” “Yeah, what should we bring?”
Most evenings we braved the freezing cold to visit her for her late evening feeding. As usual, this particular night, Mike dropped me off at the door and went to park the car in the garage. He actually beat me to the NICU, though, as I had stopped to get some water. When I arrived he was standing in the waiting area. Normally he would have washed his hands and after a quick call been buzzed right in. “What’s up?” I asked. “They asked that we wait out here until her nurse gets a chance to come out to chat.” That’s strange, I thought, but it didn’t occur to me at that point that something could be wrong. Nurse A came out a few minutes later.
“Your daughter woke up earlier than normal and appeared hungry so I started feeding her. While drinking her eyes begun to rhythmically blink and then her arms and legs matched the rhythm in sharp jerks. She had had a seizure.” We both immediately sat down, fear taking over once more. “She is resting comfortably and is receiving medication that should prevent her from having another episode. -
How is it we were back in this state, again, so quickly?- The medication will also likely cause her to be sleepy, so she probably won’t wake up while you are here tonight.” But we were just starting to feel a little hope.
“Do we think this is related to her ACC?”
“Yes.”
Devastation. The neurologist had given indications that seizure activity could be a sign of more problems. Were we now headed in a different direction from what we were hoping for? We walked into the NICU. Maryn was asleep on the other side of the room in a different bed hooked up to tubes and monitors with a huge dose of Phenobarbital coursing through her veins.
Her neurologist was on vacation and would be unavailable for a couple of weeks.
The bloodwork normally run after a seizure came back quickly and we heard the news early the next morning. They weren’t expecting any of her levels to be out of the ordinary because they had checked them not too long before, but they were wrong. Her calcium levels were dangerously low.
The next day when I arrived alone (Mike had to work that morning), Maryn's head nurse asked me if I had been taking my pre-natal vitamins and getting enough sun near the end of my pregnancy. This floored me. How this seasoned nurse could look in the face of a tired, scared first time mom and conclude it was okay to ask that question was unfathomable. This wasn't the first time she had directed a loaded comment my way, but it wasn't until then that I realized she was either totally clueless or without compassion. Ultimately, she was blaming me for my baby's seziure and she lost my respect in that moment.
Later, the neonatologist told us they sometimes see seizures in otherwise healthy infants who have difficulty transitioning from the womb. Levels may be off as the infant’s body struggles to take over where the mother’s left off. However, this type of problem usually presents closer to birth and since we were currently over a week out, he thought this was unlikely to be the culprit. He also assured us that conditions like Epilepsy typically don’t present until a child is 3 or 4 years of age, so this was also highly unlikely to be the cause. Months later an endocrinologist out of a children’s hospital would tell us the seizure happened as a result of the low calcium which was inversely affected by the potassium in the formula she was given right after birth. I’m not sure how this could have been avoided, as far as I understand formula is warranted in blood sugar issues to avoid seizures, although the endocrinologist mentioned formulas w/o potassium (or lower potassium) can be used to reduce the likelihood of this.
She had already been their pincushion, but this upped the ante. My sweet baby had prick marks all over her tiny body from the tips of her toes to her forehead and scalp. She had multiple IVs (they kept blowing out veins and would need to move to another area) and multiple blood draws each day. The calcium riders (large doses given by IV) were hard on her veins so they decided to bring in the PICC team to insert a PICC line and asked that we wait outside while this was inserted. A flexible tube that’s fed into a larger central vein through smaller peripheral veins, this can be left in place for a prolonged period of time, isn’t as damaging to the veins and got medication to her more quickly in an emergency.
Bottle feeding was put on hold as she could choke if she were to have another seizure while eating. An NG tube was inserted through her nostril. We were still there for feedings but now instead of feeding her the bottle we held her and sang to her as the milk was pushed through the tube into her stomach.
The PICC line didn’t last long as she started to develop signs of an infection in her thigh.
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