Well a week later, here we are back home. It was definitely an eventful week — one that made my head spin. In fact, I think my head is still spinning.
First up on our Children’s Hospital trip, Andrew had a 4.5 hour evaluation with the Developmental Unit. They confirmed what I already thought — he is developmentally delayed and has some sensory integration difficulties. Unfortunately our insurance wouldn’t cover a formal speech evaluation by an SLP. Guess they think the school district should cover that — which would be fine and dandy if I lived in a metro area that had therapists with different approaches, or even a therapist capable of making a DX — but that isn’t the case. Anyway, they said he definitely has a speech disorder but couldn’t go in anymore depth due to the absence of the speech evaluation. His highest score was 18% with cognitive skill using the Bayley assessment, and his lowest was 5% with fine motor. I should receive the full report, with a sensory diet and therapy suggestions, in 2 weeks.
Next up for Andrew was his overnight sleep study. It went amazingly well — not the results, but the actual process of the study itself. As sensory seeking as Andrew is, I find it fascinating that he has always cooperated and almost enjoyed his previous EEG’s. This time was no different. He cooperated while all the electrodes were placed. He even tolerated the few on his cheeks and chin and he didn’t mind the wires on his chest, legs and toes either. Unfortunately his saturations weren’t were they wanted them. He spent more than 10% of the time below 91 and because we live at a higher altitude he didn’t meet their protocol and O2 supplementation had to be used. The technician said that he only had 2 episodes of central apnea observed, which is probably within normal limits for his age. No obstructive apnea was observed, which means he can keep his adenoids. But what is causing his desaturations while he is asleep … I have no idea. His ENT did an in office scope a few weeks ago. We were able to view his epiglottis, which was firm and standing upright, no longer floppy like when his laryngomalacia was active. So maybe its his tracheamalacia that is causing him to desat, but if so, why doesn’t he have stridor? I have a call in to his pulmonologist to see what she thinks. Its been a year since his last bronchoscopy, so who knows, maybe she will want to do another. All I know right now, is that the poor kid is absolutely miserable with a cannula on at night. Its a nightmare to keep on him and an even bigger nightmare to get him to sleep. Of course there is no way I would have him sleep with O2 tubing everywhere when he could possibly get tangled up in the middle of the night (he is a wild man when he sleeps!), without having a pulse oximeter with an alarm. Thankfully the O2 company had one in stock. Still, its nerve wracking. And its bringing back all sorts of memories. Memories that I had thought were going to be just that — memories. I guess not.
Poor Alexandra. Seriously this girl can never seem to catch a break. When they discharged her from the hospital (the second time around) we finally understood why she was so irritable the last 3 days. It wasn’t the potassium in her IV, no, it was the fact that when the RN put a board on her foot to hold the IV in place, he put it on too tight. And then for 3 days no other nurse changed it, or unwrapped it, or peaked at it, and it left a large compression ulcer which was later diagnosed as a deep tissue injury by a wound care dermatologist at Children’s Hospital 2 days later. Its been 9 days since she was discharged and I’ve seen it go through different stages. It started off opaque, with tissue loss, resembling an ulcer. Now it is turning black — or maybe its just bruises. I don’t know. She has another appointment with a plastic surgeon next week. Hopefully it will heal without causing permanent damage or a skin graft. Its just one more reason why I can’t trust my local hospital. I guess at least this time they aren’t trying to deny that this is due to negligence on their part.
Alexandra’s swallow study didn’t go well. But honestly with her recent pneumonia, I wasn’t expecting it to. Its such a small window of time when the fluoroscopy can be visualized. She didn’t aspirate thin liquid at first but after turning the machine on and off, allowing her more time to drink her bottle, a barium residue showed up in her air way — which meant that she aspirated. How it happened is anyones guess, since we weren’t able to visualize the aspiration itself. But she did aspirate. So next up was nectar consistency. They didn’t take as long with this, partially because Alex was not interested in the taste of the mix they made — they didn’t use her regular pediasure that she is used to, so not surprisingly she turned her nose up. We tried adding kool-aide, in hopes that the sweetness would temp her to drink a bit more, but she wasn’t having it. So in the end their recommendation was nectar consistency. After long talks with both the OT and ST, I realized that there is no clear cut way to prevent her from having pneumonia again. We want her to eat orally but its going to take trial and error to figure out what techniques work — and what works is what doesn’t give her pneumonia.
On the GI front, the doctor wants to start her on Reglan to speed her gastric emptying and he wants to double her Prevacid. The thought is the less acidic her stomach acid is, the less likely she is to get pneumonia. The low pH of stomach acid prevents a greater risk for pneumonia than food and liquid aspirated through the oral route. I can’t say I’m excited about giving her Reglan. I’ve always veered away from this medication — but the alternative would be to change her G-tube to a J-tube and feed her through her intestines. I’m just not ready to do that yet. Nor am I ready for a nissen fundoplication.
Her rehab doctor was able to squeeze her in for her botox injections. She received 6 injections in her lower back. This time there were 2 doctors administering the injections, so it was quicker and less painful for Alex (at least that is what I tell myself). The rehab doctor said that he tried something a little different this time and that hopefully the botox will kick in sooner and last longer. Of course I forgot to ask about the phenol block, so I’ll have to wait until next time. There was just too much on my mind.
The last thing that Alex had to endure was eye surgery for her bilateral exotropia and a repeat MRI. She was under sedation for 2 hours, which was a bit nerve wracking for me. But she did well. Recovery was a bit difficult given her pneumonia. She took 4 hours to recover and at one point her O2 saturations were in the low 70’s even with 6 liters of O2. She bounced back though and didn’t need to be admitted. And the ride back home (8 hours!) went great. She didn’t rub her eyes and she tolerated everything without a fuss. Her follow up eye appointment is in two days. I’m not sure how she is healing but I can notice a huge difference in her eyes. They aren’t wandering anymore, its great!
Alex’s MRI results were consistent with her previous MRI. She still has hypoplasia of her perisylvian region, although this time they are calling it diffuse white matter loss and they are saying she has it almost globally, although its more apparent in the perisylvian region. Her corpus callosum is still thin and incomplete. Her ventricles are still enlarged (mildly) and this time they noted she had sulcal prominence. There is question that her right optic nerve is hypoplastic. Its there, its just small. What all this means clinically, her neurologist isn’t certain. I asked him if one can have the DX of bilateral perisylvian syndrome and not show abnormal gyri on a MRI. He said that polymicrogyria doesn’t always show on an MRI, that retractable seizures is common with PMG and sometimes a type of brain surgery can help with these seizures. At the time of the surgery a biopsy is taken, which pathology later reveals the gyri to be abnormal, consistent with PMG. But in short, its too rare of a DX and there isn’t a lot of literature on it. Because of this, he is sending it to the neurology review board. And I am sending it to Dr. Dobyns. Hopefully then, I’ll get some answers.
So yeah, I guess right now I’m just waiting … waiting for a doctor to tell me what’s next, because there is always something.
Filed under: Late Night Rambling, Medical Rambling , aspiration, barium, botox, central apnea, corpus callosum, deep tissue injury, enlarged ventricles, epiglottis, exotropia, eye surgery, Laryngomalacia, Perisylvian Syndrome, PMG, pneumonia, polymicrogyria, pressure ulcer, skin graft, swallow study, Tracheamalacia
