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.

Dear Son~School Visit

As you may or may not know, Dear Son has not been able to go to school since he got the swine flu/viral pneumonia in November. Although he has recovered from those, he is still not able to sit up very long and he is unable to clear his airways; since he can't clear his airways, he is not allowed to return to school until cold/flu season is over since if he got sick, he might never recover. His last day of school was at the end of October. In the meantime, we got a visit from his teacher and his aide (CNA). She brought him a little scrapbook they made at school to show him what they have been doing. He really enjoyed it!


He also enjoyed visiting with his aide, although I didn't get a picture of her. I didn't realize I didn't take it until later! Dear Son was having such a good time with his teacher that I couldn't help but take out my camera.

During her visit, we talked about going back to school, maybe starting with some home visits and then a trial visit to school. Today, I received notice that they will require him to be able to sit up 2.5 to 3 hours at a time. Currently, Dear Son can't sit for more than an hour without being in pain so we are a long way away. We really need to get him back in school, not only for his mental well being (He is happiest when he is around other people.) but to help him move his feet and legs again. Prior to the November hospitalization, he could move his feet and legs in his bed, he could move his feet to swivel the recliner and move his toes. Now, he can't move his feet, legs or even his toes on his own. (This is problematic, since I need to keep moving him so he doesn't get pressure sores.) His only means of communication now, is a moan, or if I ask him if he wants to turn over in his bed, he'll move his shoulder slightly to let me know he wants to be turned. In addition, prior to the hospitalization, when I turned him over in his bed, he could move his arm for me; now when I go to turn him, I have to move his hips and then move his arm. I am hoping the physical therapist can help him with that to make it easier for him and for me.

After she left, she followed up with a video of Dear Son at school last year, however blogger couldn't process it. I tried last night for a while and today as well however blogger has known issues with video processing; for some reason it hangs in the processing mode. If anyone has a work around, I'd love to hear it.

We follow up with the neurologist next week.

War and Peace

Some days you can’t help but think, when the ax might fall. It’s times like these, when I am alone at home, and Dad has Dear Son, that I think about that. Times like this when I have time to paint my nails and not worry that I’ll ruin them if I’ll have to change a diaper. Times when I can do whatever I want, because there is no one to care for. You revel in the solitude, the peace and the quiet, but are careful not to enjoy it too much for fear it could be permanent. A little dose here or there, is a luxury, but permanent silence would be deafening.

I don’t suppose we give much thought to when someone might die but this isn’t a normal situation. We have a child who we know has been very, very sick and it’s only a matter of time before we lose the war, even if we’ve won the last battle. War changes you over time. You can no longer imagine time alone when you’ve been in every battle together. I’ve sat at his every bedside, wiped his little brow and doted over his every breath. When you are together at the hospital, you wait for the time when you can get out and go home. As you imagine the end of the battle, you can’t help but be swept away by thoughts of the war and when your real life will begin. Instead, your life is always a catch 22, where you eagerly await the time when you can begin your life, once the war is over, only to lose your best friend in the battle and have nothing to return home too.

It’s tough sometimes too, when people die, especially after a long battle. It doesn’t matter if you know them or not, just hearing their story on the news, whether it’s a celebrity, a politician or just a random local news story can evoke a reaction. The bigger the figure, the more you are affected. You can’t help but be affected because you know deep down, that your own war is nearing the end, and when someone else dies, you imagine your own battle and how it will end. And when they die, you feel like you’ve somehow dodged a bullet, because you know your end is coming. The tears you cry for them, often catch you by surprise, but your tears are never really for them, they are for you. They are yours because you realize just how close you are to the end of your own war. When that happens, you can’t help but hug and kiss your child, as if they had run out into the street and dodged a speeding car. It’s as if doing this somehow, prevents the end to your own war.

It’s strange too, when you’ve been in the trenches together. There is peace in the battle, knowing that you are together, even if only in the same room. That is where the battles are won, even if only briefly. It’s there that they tend to the wounds of the battle only to release him to find out that the battle isn’t really over, it’s just that the location has changed. You leave the battle as scarred as they do, only your wounds are inside. Whatever physical scars they have from the battle, have been internalized by you. It’s like watching a war in 3-D, somehow you feel you’ve been in it too.

During the war though, you learn to value the soul. The battle scars or bucket of parts is sometimes just that, a bucket of parts, some of which still work and others, not working so great. At some point, you stop worrying about the physical skills and what they could do and focus instead on the person within, for therein lies their soul. That is the person you know and love. It matters not what they can and can’t do because you love them regardless. Over the years, you cherish the child they were and the man that have become. War does that to people. You learn that the time you spent together was not just for him, but for you too. Their battle was your battle and you never look at any war the same way after that without being affected. As you get near the end of the war, you realize that the life you imagined after the battle, may never live up to the war.

And those times of solitude, can be a grim reminder of what is to come. I am always careful not to enjoy them too much. And when the time comes, when Dad brings Dear Son back home, and the front door opens and I see his shining face, I make sure to kiss and hug him really good. I am thankful for the opportunity to care for him once again. Once again, the battle rages on. It’s a good kind of battle though, because it means the war hasn’t ended. And as his mother, I'll do what I can to fight until the very end. After all, I have my little buddy to take care of…and that’s a very good thing.

Note: Dear Son is eighteen years old and suffers from intractable seizures, dystonia and severe mental retardation as a result of a random mutation of the ARX gene. In addition, he suffers from a progressive neurological disease.

A Valentine's Day Tribute to Dear Son

This, I will remember,
when the rest of life is through,
the finest thing I've ever done,
is simply loving you.

Happy Valentine's Day Dear Son...thanks for the memories.

With love,

Mom

Note: Poem is by Robert Sexton

The Chair

Photo of Dear Son, Age 11

I remember the conversation distinctly, as if it were yesterday. The geneticist told me that a disabled child’s average lifespan was five years after they first started declining. In my mind I knew exactly when that happened. For Dear Son, that year was 2004. That was the year he got his feeding tube put in, just for meds, and the surgical nurse who did all follow up visits kept messing with the size every time he had a visit, trying to find the right size but to no avail. The tube was either too tight or too loose. It wasn’t long before Dear Son began vomiting. He vomited on and off for seven months and had numerous hospitalizations before the radiologist, Dr. X, figured out the too large feeding tube was blocking a small pyloris (the stomach opening) and not allowing the food to empty. In addition, it scratched his entire stomach, leaving multiple abrasions that were easily viewed from the upper GI. And the food, well, it just sat in the stomach until it putrified and then he would throw it up. He was hospitalized month after month that year with no results until that last hospitalization in December of 2004/January of 2005 was when they figured it out. Unfortunately, by that time, Dear Son was weak. He slept the next ten months or so and never recovered. He wasn’t able to go to school. Prior to that error, he was a happy young man, who walked on his knees. Yes, he could walk on his feet with his leg braces in a special gait trainer, but for home use, he walked on those knees. Needless to say, I’ve never forgotten that nurse or that hospitalization. It was the beginning of the end.

Photo of Dear Son, age 13, in January 2005, after he was released from the hospital, where he lost of a lot of weight from the feeding tube error.

A few years prior to that, I had purchased a rocker for him. My husband and I had been looking for dining room chairs for our kitchen table and were in a furniture store. They had a wonderful maple rocker there and as Dear Son started to fuss in his wheelchair, I placed him in the rocker. He loved it. So much in fact, that we bought it for him. He used that rocker for years up until that hospitalization.
Photo of Dear Son's first rocker; he had almost outgrown it at this time. He used this rocker for about nine years.

After that hospitalization and recovery, he needed more support. The maple rocker was fine but with his long legs and lack of tone, he required more back support. It was hard finding a rocker for him with a tall back. With a rocker, Dear Son was able to rock and to scoot the rocker around a bit to get around the house. Granted, he couldn’t go far but it did give him a little independence to look out the window or to spin around and watch t.v. Since he needed more support, a regular chair wouldn’t work very well. Soon, I found a taller rocker with a wide back, much like a Windsor chair. With that rocker, he was in business again and could look out the window. It was important to keep him active. At least if he couldn’t walk on his knees, he would still be able to use the rocker. Photo of his next rocker.

Soon, he outgrew that one as well. More important though, was that the rocker wasn’t sturdy enough to move him. He soon lost his ability to move around very well by himself in the rocker. He could no longer use his feet to turn the rocker and I’d have to turn the rocker with him in it. This required a sturdier rocker with a tall back and more support. After looking around, I found this rocker on-line. I went to check it out and it was perfect.
Photo of his last rocker.
That rocker lasted a year or two until Dear Son grew some more. By this time, he was pretty tall. Not only were his legs longer but his torso had grown. No longer could he sit upright without falling to the side or falling forward. His progressive disease was kicking in and he could no longer sit upright on his own. It was then that I had to move him from the window, which he loved to look out, to the dining room table. From there he would sit at the table and look out the window. Sometimes, he’d lie his head down on the table. But it wasn’t long before he couldn’t do that and soon, he couldn’t lift his head up but instead would just lie his head on the table and listen to music. Photo of Dear Son listening to his Mr. Christmas music box.

But last year, he lost that ability. Soon, he had no tone and when he’d lie his head on the table, it left big red marks and he would cry. It didn’t matter if you put a pillow or anything on the table first, it was just that he couldn’t support himself and the weight of his head was too much. By that time, we knew we had to get him more support so we got him a recliner. The recliner would support him more and the swivel action would allow him to turn the chair so he could watch t.v. or look out the window. Photo of his recliner that swivels.

Photo of Dear Son in August of 2009.
But that’s all about to change. He’s been falling over to one side in the recliner for a while now so I have to prop him up. He no longer uses his feet at all to swivel the recliner. Instead, he sits exactly where you put him. At times when he sits there, he looks like an old man in a retirement home, one with dementia who has lost his ability to move. The only difference is that Dear Son is much younger. He’s getting tired though. Not only does he cry from the pain from sitting but he just looks exhausted in the chair, as if his body can’t do any more.

After I got the recliner, I moved the other rocker out of my living room and into the garage. I placed it near the gait trainer that he no longer uses and hasn’t used in five years or so. Somehow getting rid of them is hard. It means he’ll no longer use them. I know that, but the reality is that those items represent the life he once had. If I get rid of them, I must admit to myself that he’s getting progressively worse. As parents, it’s easy to see our children in terms of their milestones, their first shoes, their first words, etc. As parents of special needs children, we wait a long time for those first anything’s to occur. Now, we no longer count the milestones that he has achieved, but the ones that were taken away. Who needs a health history when you can just look at the chairs? And one of these days when he's lying in bed, I'll look back at the chairs, and remember the good old days, when he could sit.

Note: Dear Son is eighteen years old and suffers from intractable seizures, dystonia and severe mental retardation as a result of a random mutation of the ARX gene. In addition, he suffers from a progressive neurological disease.

Check out these Kitchen Design finalists...

If you get a chance, you might want to pop over to Kristin Drohan's Boulevard Interior Design's blog today. She has the National Kitchen and Bath 2010 kitchen design finalists. My favorite kitchen is the one above. If I were designing it from scratch, I'd probably opt for crystal chandeliers and a little softer palette, maybe green, for the walls and rug. They have some beautiful finalists though. And if you haven't checked out Kristin's blog before, she has an Eco-Friendly Furniture Line.

Dear Son Medical Update~Finally, A Smile

This past week has been mixed. The good news is that Dear Son has been sleeping better since the new regime I started last Friday. The new regime involved an extra can of formula and .5 ml of Risperdal at midnight, on the nights that he moans in his sleep, which has been every night except one. With the extra formula, he's not waking up hungry at 3:30 -5 for his formula to be started. In addition, after a few days of this regime, he started to seem like his old self. He would smile and be happy at times. I started talking to him about his "Make a Wish" trip and the possibility of going back to school, once he's feeling better. He smiled when I talked about that and he hasn't done that since October prior to the whole swine flu hospitalization.

The bad news is that he is having major issues with saliva. The scopolamine patch isn't controlling his secretions very well and I am afraid he will choke on them. In the past, it was not uncommon from time to time that Dear Son would have times where the patch didn't work as well as others. I don't think it's the patch as much as it is the fact that some days, things are worse than others for him and some days he may have more secretions than others. On Wednesday night, he was up virtually the entire night trying to breathe. I will be ordering the therapy vest and a suction machine shortly, as soon as the January hospital bill is processed. Once that is done, we should meet our deductible and be close to the stop loss, so it shouldn't cost as much. I did try to do some chest pt this week but he seemed to scream out in pain when I did his upper right lung. I am not sure why since he is not presenting with a fever which means there isn't a respiratory infection.

The other major issue is that he still can't sit up in the recliner or sit up without pain of some sort. On Tuesday, we took him for the CT scan and he fussed in the car all the way down and all the way back. I sat in the back seat with him so I could hold his hand and comfort him. His hand was shaking as well at times so I am not sure if he was having some seizure activity to boot. While we were in the waiting room, he continued to yell out and make noise most of the time.

After the CT scan, we attempted to get lab work however they were not able to get any blood. The patch makes it very difficult to get blood from him so after 35 minutes or so, I said that was enough and took him home.

I called the neurologist today since Dear Son's saliva issues were fairly bad. He suggested adding an antihistamine at night to help dry up the secretions. He said it would be short acting and would help get him through the night. I thought this was brilliant. I hope it works.

We also discussed the CT scan. The pneumatosis is completely gone, so that is the good news. The bladder is still very large. There weren't any other findings to explain his pain from sitting. Interestingly enough, I got to see the CT scan while the tech was doing it. Dad was holding Dear Son on the table and the tech suggested I take a seat in the room with him. I saw the curvature of his spine and asked the tech if it was the test or if his spine was curved like that and he said it was his spine. I don't know if that is playing a role in his sitting issues.

In addition, Ped Neuro Doc e-mailed the ENT last week with regards to the saliva issues. He recommended two options: botox and removing the submandibular and parotid glands bilaterally. He said he'd prefer the first option since it's a reasonably involved surgery. I reminded Ped Neuro Doc that we already had the botox surgery/injections in January 2008 and it was unsuccessful. He'll speak with him again with regards to next steps. If he does choose the surgical option, I'd like to have some discussion as to where one or both sides should be done. In the past, Ped Neuro Doc suggested one side be done. I'll let them figure that out.

As far as urinations with the patch, they remain unchanged. I am tracking his output and the number of urinations seem to be the same as without the patch. As of yet, I don't think there is pain with that. Dear Son does moan at night and I am unclear if that is from the pain from urinary retention or something else but overall he seems better this week other than the secretions. On the back burner is the bowel movements. The frequency is slightly less and the volume is a lot smaller so I hope he's not backing up. I have been feeling his abdomen to make certain it's not hard or distended as of yet and when I push on it, he doesn't seem to be in pain. Without the Miralax, I think we need to keep an eye on that.

Overall, I would really be glad if this were over soon. It was very stressful on Tuesday taking him for the lab work and CT scan while worrying he might catch something down there and be back in the hospital. I didn't realize how traumatic that was for me. In addition, we have to get things back to normal soon. He is still sleeping much of the day and there aren't too many alternatives when he can't sit up. It has been good seeing him more like himself this past week. I can't even explain how thrilled I was that he was smiling and happy at times, just like the old Dear Son. In December, the docs had told me that his crying was "disease progression" and he'd probably cry the rest of his life. I was beside myself to think that Dear Son would cry forever and that I'd never see my happy-go-lucky son again. I am so relieved that the bulk of his pain is gone. All of the sweating is gone and other than the pain from sitting up and the moaning at night, he's not crying any more like he used to. In retrospect, it is so hard to think back to these last ninety days and everything he has been through. It has really been a tough time for both of us.

On a different note, I normally read a lot of different blogs however this week, I stumbled upon an old favorite of mine, "The Examining Room of Dr. Charles. When I started blogging in 2006, Dr. Charles was one of my favorite blogs. I'd read the "Grand Rounds" every Tuesday and soon was reading his blog all the time. Back then, most of the blogs were physician blogs and there were very few patient blogs, unlike today. He used to write these stories that were fascinating. In 2005, he self published a book, "Legends of the Examining Room". Over time, he switched to this science blog and wrote more about science related issues than these patient stories however recently I saw that he's moved again and has a new blog. Here is a clip from his latest post, "Attending to a Patient's Funeral":

"You walk into the funeral space. Many people are gathered. You sense pockets of light humor and recalled happiness amid dark clouds of sadness and gloom. Although you know this is not about you, your ego can’t help assessing how others perceive you. Most of those present barely notice, but others recognize you. Is it surprise registering in a few faces – that you’ve come to observe your patient’s defeat, that you’re emotionally invested in the person who once called you their doctor, or that you’re willing to dirty your powerful white coat with the stains of ultimate impotence? Or is it gratitude, that even in this darkest of reflective hours you’ve come to pay your respect to another who trusted you, confided in you, and who reached out to you for whatever healing you might bring?"

You can read the rest of the post here. Dr. Charles blog, "The Examining Room of Dr. Charles" can be found here.