Tuesday afternoon turned into total mania and I felt like the eye of the tornado running around my house on the phone with this doctor, that doctor, Craig, my mom and handling Aleck, it was pure insanity. At about 5 minutes to 4pm we got a call from Aleck’s neurologist who left a message asking if we could do the scope on Wedensday (yes, the very next day) this way they could do an EMG or Electromographyhttp://en.wikipedia.org/wiki/Electromyography, and a NCV or Nerve Conduction Studyhttp://en.wikipedia.org/wiki/Nerve_conduction_study
We had been at the neurologist earlier that day and she was really disappointed that she wasn’t going to be in town on Friday for when we had our scopes scheduled. Aleck would need to be knocked out for the EMG & NCV as well, adults take these tests fully awake but since he’s just a baby he’d be really upset and they wouldn’t be able to get good reads on him if he was completely awake. You can’t say to a 5 month old, “Now you are going to feel a slight sting, it’s O.K.” Well, I guess you could say that but it’s not going to make him stop screaming. When we first met this doctor back in November she had mentioned doing these tests down the road, trying to give us some answers as to why he has trouble moving now but also why he wasn’t moving these limbs in utero. Is it related to the nervous system? Are there synapses that aren’t firing correctly? And maybe more importantly, can this lack of movement be treated pharmaceutically to allow Aleck a greater range of motion and movement, potentially life closer to “normal”, closer to the movement of other babies/children/teenagers/ and eventually adults of his age?
My first reaction when I met Dr. Kuntz, the neurologist was that I liked her right away. She has this really calm, really warm manner where she’s studying Aleck but she’s gentle and soothing in her voice and demeanor. Then, when I was hanging out at Children’s another time I ran into clients of mine who also see Dr. Kuntz and just love her completely. Her reputation is outstanding and we’ve been told how lucky we are that the hospital hooked us up with her even before we were discharged (if only the pediatric orthopedist they forced on us was that awesome). So when she says that she wants to run these tests on him to try and get us some more answers I saw no reason to refuse the request. I mean, why not get as much information about Aleck’s condition as possible. That’s what our intent is with the scopes, why not go for the grey matter as well. And to do it all at once, just one day in the hospital, knock him out for two hours, it seemed to make sense.
With anytime a baby is being given anesthesia, or anyone for that matter, there are always very specific feeding times to adhere to so the body takes to the anesthesia and reduces the side affects. The last time I could give him formula would be 2:30AM, clear liqu
ids, 6:30AM. I was up at 1:20AM with worry, stress, and then to make sure he got as much formula as possible before his long day. He was going to need all the strength I could give him. We arrived at 8AM at the hospital and were in our room at 8:45AM. It was then they notified us, my mom came with me, that the ENT who was performing the scopes had an emergency and we wouldn’t be going in until 10/10:30AM instead of 9:45AM. Not too bad all things considered, and Aleck was carried off by the anesthesiologist at 10:20AM. At 12:20PM on the nose they came and got us, Aleck was in recovery and it was time to talk to the doctors.
First we spoke with his ENT, Dr. Holinger, who performed the scopes. Aleck has a cleft larynx which means the wall between the esophagus and the trachea is sloped down allowing for food, liquids, saliva, to get into the airway which is why he aspirates so much when he feeds. According to the doctor this is something that can potentially correct itself over time, maybe about a month or two to see if it fully develops on it’s own. If not they can surgically repair the cleft. At this moment I have to give major kudos to Dr. Cahan, our pediatrician, who told me he thought it might be a cleft when he recommended the ENT for the scope. Since according to most sources this occurs in 1 out of 10,000 infants, that’s pretty remarkable. Oh yeah, we are definitely staying with this pediatrician. In order to see just how much of an effect the cleft is having on Aleck’s ability to eat properly they want to do a swallow study. For this Aleck would be fed a bottle of Barium, which I know he’s OK with because he drank that up like Kool-Aid when he had his upper GI. While he’s drinking they X-Ray his swallow patterns to see how much is getting into the airways. Another note that Dr. Holinger gave us was that his lungs, his esophagus and his bronchial tubes looked really good. Better, he noted, than most babies and kids alike who come in for the study. That’s got me thinking that even though he’s choking and coughing, maybe Aleck is getting that food and other liquids out before they get into his lungs to do their damage. If that’s the case, then maybe the cleft is really mild and we won’t need surgery. So should we do the swallow study?
Obviously that’s a question to discuss with Dr. Holinger, but yesterday I just felt so completely wrecked from the scopes and nerve tests, that the idea of putting Aleck through anything else right now seemed just cruel. He looked so terrible when he woke up, his face was puffy, he has marks on his body from the nerve study, I can count about 8 or 9 places where they pricked him, and his voice was hoarse from the scopes. Everything about him looked and sounded miserable. Trying to cry because he’s hungry but only being able to suck down the glucose water because of his sore throat, being so tired and out of it he had difficulty even eating that due to sheer exhaustion. My heart just broke. Here was my precious little boy, beyond uncomfortable, beyond unhappy, beyond unsettled, I couldn’t help but feel it was all my fault, that I chose to do al
l of this to him. In the end for his own good, but that’s a very small consolation.
To accompany the cleft larynx he also has laryngomalaciahttp://en.wikipedia.org/wiki/Laryngomalacia, a softness in his larynx where the cartilage collapses inward during inhalation which is why his breathing is so noisy. Now this is very common and should correct itself as well. It’s definitely comforting to know that there isn’t anything wrong with his breathing, nothing caught in his chest or airways that’s been making this noise. Nope, he doesn’t have asthma, yet. Granted it’s taken me 3 days to pronounce this condition and hopefully Aleck will have outgrown it by the time I learn how to spell it. Whew.
What’s cracking me up about all of this, especially the cleft larynx, is that there are support groups for people and parents with this affliction. In fact, I think there are more support groups for this than for Arthrogryposis. Because that’s what I need, another support group to join, another label to slap on my baby boy. No thank you. I think the one is more than enough. Of course my OT pointed out that if he didn’t have the AMC we just might be signing up for the cleft larynx support groups since that would be the primary ailment we were fighting. I’m not sure what this says about perspective and I think I’m just going to leave it be before my head spins off my body.
In the end he may not be allergic to milk, to soy, to wheat, to my breast milk (though that ship has thankfully sailed), so I guess now it’s time to get a handle on all of that as well. It would be nice to feed him some formula that has some kind of recognizable food group in it instead of just oils, proteins, minerals and vitamins.
Regarding the results of the neurology tests, it sounds like they were inconclusive. Granted, I was desperately and unsuccessfully trying to settle Aleck when she came in to go over everything, but it sounded like everything was firing correctly and we don’t know much more than we did before for why Aleck has AMC, but at least we know that everything upstairs is functioning on all cylinders and the muscles are receiving the messages even though they have a hard time responding. I’ll be getting a written report in the mail which will better help me digest the outcome and allow me to read it all through during nap time.
The next step is to talk with the ENT about the swallow study and consult with my pediatrician about changing his diet. In the meantime I’ve been trying to add an additional feed to our routine to try and get 25 oz of formula in Aleck a day since even with consistent formula bottle feeds our little man is still not packing on the pounds. We are finally graduating to size 2 diapers because even though he’s still in the weight range for the size 1’s, they are now sitting a little too low up the back and we’ve been having poop explosions regularly. In fact, while I was trying to rearrange everything for Wednesday morning I was in the middle of cleaning up a poop explosion when Aleck started peeing, I caught that pee with a paper towel, breathing a sigh of relief, only to lift the paper towel to see how he was doing there after a few minutes for him to soak me and one of his grey shoes with a second round of pee. I stood in the middle of his nursery laughing my face off, because it was just that funny.
Go here to watch a video of Aleck moving around in his crib for a good kick session before bed:
http://www.youtube.com/watch?v=bMDz4UZRTlM&context=C3fd8915ADOEgsToPDskLBDJCEYNm5dpsQ-Mslv2-W