Dear Son Medical Update~Follow Up Appointment

Dear Son had his follow up appointment on Wednesday with his pediatric neurologist. He functions as Dear Son's primary physician as well, since he's considered medically complex. We discussed many issues, but the main ones were Dear Son's choking on his saliva, his inability to sit for very long and his inability to move his legs and feet.

As you may recall, Dear Son was on the Scopolamine patch to dry up his secretions and last month it was determined that the patch was causing urinary retention (so much so that his bladder was huge) and most likely was a source of his pain. As a result, the patch was removed and they recommended that we try a Scopolamine gel from a compound pharmacy however it was unsuccessful. As a result, Dear Son was put back on the patch and did not seem to have any issues with it. The crying that they had assumed was caused by urinary retention did not return. Ironically, the crying seemed to dissipate as well once the pneumatosis was gone; the CT scan on February 3rd confirmed this. Dear Son remains on the patch and the bulk of his crying is gone so I have to wonder if the pneumatosis was really the source of his pain.

Since the choking on his secretions was so bad, the neurologist spoke with the ENT regarding possible surgical intervention to remove the salivary glands. Of particular concern was whether or not Dear Son could handle the sedation (it causes respiratory depression and Dear Son can't clear his airways) and the surgery in general. Surgery is a huge risk for Dear Son because of the sedation and the risk of aspiration. In addition, I had concerns about the surgery because Dear Son's only means of communication right now is to cry out or moan when he needs to be changed. The surgery would make that harder.

The ENT seemed to feel that the surgery would be fine. The neurologist asked him about the extent of the pain from the surgery and the surgery in general. The ENT seemed to feel that even with Dear Son's issues, it was doable. The surgery is fairly involved so that doesn't mean it would be easy but he felt he could do it with minimal complications. I asked if they would recommend doing one side or both and they said they'd do both sides.

In addition to the secretions, Dear Son still had some pain when sitting in the recliner. I noticed that he had pain when I raised the footrest and screamed out when I lifted his leg to put foot cream on him. He has continued to have some pain when sitting in the recliner and can't seem to sit for more than one hour or one hour and fifteen minutes without fussing.

One other major concern that I had, was that Dear Son has not really been able to move his legs, his feet or his toes over the last three months. Typically, I do expect some deterioration after a hospital visit but this was quite pronounced since Dear Son's legs were always the most active part on him. He could swivel the recliner by moving his feet, he could rustle his legs in his bed and he could kick the wall if needed to let me know he needed to be changed (Dear Son has no language so he would yell out or kick the wall when he was in bed and needed changing.). He can't do any of that anymore. I did notice that he had maybe two or so times over the last three months where he moved them slightly, so I know they aren't paralyzed but overall, it's a significant difference. It's a concern as well when he's sleeping too since he does not move his legs at all once I turn him over. I must fully position his legs, his arms and his entire body otherwise Dear Son can't move at all. His inability to move further increases the possibility of bed sores or a skin breakdown however I am having to turn him over more and to date, he doesn't have any breakdown.

The neurologist checked his legs and noticed severe tightness. He also had concerns about Dear Son's spinal cord and recommended an MRI. An MRI of the spine can be dangerous for Dear Son due to the sedation necessary and typically spine MRI's are longer than a brain MRI. The neurologist felt it would be doable though since the brain MRI in January was successful and because he could request limited views. MRIs are typically done on an outpatient basis and my concern was that for the brain MRI, we had to convert Dear Son's seizure meds to an IV for the MRI, to prevent aspiration. (Also, they removed his Scopolamine patch and changed him over to Robinal, along with stopping all feeds prior. The side effects from the sedation caused low blood pressure and respiration which were resolved with a bolus of fluids but it takes a few hours until he's back to normal.) My concern was that this would be difficult to manage on an outpatient basis. The neurologist didn't feel however that the insurance would cover this procedure as an in-patient so we'd have to hold off until he was admitted again.

Other things I asked about was the Miralax. Dear Son became constipated once that was removed last month. As you may recall, they removed the Miralax and changed some of his medications to pill form since they had Sorbitol in them; medication, Sorbitol which was one of them, can be a cause of pneumatosis. When Dear Son became constipated, I attempted to give him prune juice, but did a google search and found out that prune juice had Sorbitol in it so I couldn't do it. When I called the neurologist, he put him back on the Miralax, but every other day. Since then, we haven't had any more issues and I wanted to know if it was o.k or if we needed to get another CT scan to check for a pneumatosis. He said we should be fine.

I had a few other issues to discuss with him but overall the neurologist felt that Dear Son had deteriorated significantly and that his disease is progressing. In light of that, he asked me what I wanted to do regarding the surgery and I said that I didn't want to drive that issue. We talked some more and I asked him if we were to do the surgery, is this something we need to do now or later and he felt if we did it, we should do it now since he won't be able to manage it later. He told me that there is a chance Dear Son would need to be intubated after the surgery and that the intubation could be quite lengthly. He explained that Dear Son will die of respiratory issues. I did not get the sense that he felt Dear Son would really make it through the surgery even though the ENT thought the surgery was doable. I also had some difficulty in doing a surgery so soon after we had been released. My gut tells me that Dear Son would not survive this surgery. I saw how hard it was in November for him to breathe and I can't imagine doing that again. In light of his respiratory issues, I suggested that we wait three months or so and then revisit this decision. That would give me time to get the therapy vest and a suction machine. (I have a low durable medical equipment limit and I needed to wait for the January hospitalization to be paid in order for this equipment to be covered at a higher percentage. Otherwise, it would go as the deductible and the secondary insurance doesn't cover respiratory conditions.) I need to order the vest and the suction machine regardless but if he did have surgey, then at least I would be prepared when we got home.

Overall, I don't think Dear Son is doing that great. While he's not crying much anymore, other than when he sits, he still is quite weak and sleeps most of the day. He moans when he sleeps at night and requires a small dose of Risperdal to help him sleep and an antihistamine to help further dry up secretions. I asked about the possibility of returning to school, since I wanted to help get his feet moving again and I think it would help his morale. School had stated that he couldn't return unless he could sit for 2.5 to 3 hours. I said that I don't know what Dear Son is capable of doing until we try it. He can sit briefly and could perhaps make it to school on the bus and if they lie him down once he gets there and alternates the sitting and lying down, it would be doable. Also, they could work on his legs in PT. He agreed to a few hours or half day once a week.

So in summary, he felt that Dear Son had deteriorated significantly. We agreed to revist surgery to remove the saliva glands in three months and we'll do an MRI as soon as we can. In the meantime, he can return to school. I contacted school and explained the situation to them and they agreed to try it. They are very excited to have him back and assured me that the classroom was all ready to go and they could manage him.

Dear Son will return to school next Friday, for a half a day or a few hours or so. I'll let them work out the times and once they get the bus scheduled, we should be good to go. They asked me what day of the week would work best and I said Friday because every Friday at school is, "Fun Friday" where they play color bingo, have music therapy and do fun stuff. At this point, that is what Dear Son needs the most. He needs some fun and to be around his friends again. I think going back to school will be a very tall order for Dear Son. I can't say that I think he's really ready although if we don't try to get him back, he'll never go back again. I really think we need to try to get some functional movement again, if possible.

And finally, we discussed the Make a Wish trip. Dear Son can't go until he can sit up and sustain the plane ride and getting to the airport. We need to see how school goes and if he can get through that, then we can re-evaluate whether or not the trip is doable. If he can't get through that, I don't suppose he'll ever be able to make it. I think I already know that but I suspect we'll know more in a month.