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.

A Patient’s Perspective: The Real Cost of Lack of Organization in Hospitals

I remember the hospitalization well. Dear Son had just been put on the ventilator and we were only on day 3 of the hospitalization. After a while, you get to know the routine. Not only is it hard when your child is in the ICU, but after you have done it a few times, you know what to look for in terms of whether or not your child is really improving or not. I have little sayings that I’ve coined over the years to describe the way things are going, such as:

• “More people, bigger problem.” Back in 2006 when Local Hospital missed the MRSA pneumonia and I arranged for Dear Son’s transfer to Big Academic Medical Center (BAMC), he came to them in septic shock and acute respiratory distress syndrome. I had called BAMC on a Friday night to try to get someone to help me since Dear Son was ailing so bad. When he was admitted, they worked on him for fourteen hours straight in the ICU-the entire room was filled with doctors and people the entire time trying to get him stabilized.
• “When the vent settings are increasing, things are bad, settings going down, things are heading in the right direction.”


• “When you start to die, things go haywire.” This means that when you see they can’t control your blood pressure or your blood levels (potassium, etc.) and your skin starts turning gray, things aren’t going well. Of course your extremities are cold too since the blood flow is centered on your main organs.
• “More frequent anything means you are in deep doo.” For example, if they are doing blood gasses more frequently, you aren’t stable. Chest pt and nebulizer treatments, same thing. Basically, anything that’s done every two hours or so, means you are in trouble.
• “If the IV pole with the IV meds the patient is on, resembles a condo tower, meaning there aren’t any more places on it to place the pump, you are really messed up.”
  

It’s easy to get depressed when all of this is going on. Often times, I worry that Dear Son won’t make it or I wonder how many more times I can put him through this. Of course, when they make it, you are glad they made it but when you are going through it and watching them suffer, it’s not very easy and you question what you should do.

This past November, the hospitalization was especially grueling. Dear Son was a week away from his Make a Wish trip and we couldn’t go. He got the swine flu only after we couldn’t get the vaccine from BAMC, even though we set up the appointment weeks earlier. He was fighting for his life again, just six months after we were in the ICU in May from a hospital error (he nearly died when they used heavy sedation vs. general anesthesia to replace his VNS-the sedation suppressed his respiratory system, he got a mucous plug and his lung collapsed). Due to infection control procedures with the swine flu, I wasn’t able to leave the room. I soon developed the swine flu as well. That, coupled with trying to go home, bring all of my food down (they didn’t offer a parent meal), go grocery shopping while your kid's in the ICU and you are sick, plus lack of a hot meal in the hospital (with infection control you weren’t allowed to use the microwave or go anywhere in the hospital unless you agreed to wear gloves, mask and hospital gown 24/7 in the room with my son) made for a very long stay. But one of the most depressing parts of the hospital stay was the state of the hospital room. It was a mess.

I don’t think people realize how depressing it can be when everyone who enters the room brings in their supplies and just leaves them all over the room. Because I couldn’t leave the room, I watched each employee as they came in the room. Everyone would come in the room, dump the supplies on the counter in the room, do their task, throw 75% of it away, leaving the rest on the bed or the floor, and then leave the room. Due to infection control, they had to use a disposable supply to listen to Dear Son’s lungs. Each nurse put it in a different spot so each nurse spent time every day looking for it prior to doing his vitals. Even the same nurse couldn’t locate it when after she just did vitals the time prior. When they had to do an IV, they might look and see if they had the supplies, otherwise, they’d bring in a ton of supplies, use what they need, toss them on the counter in no particular order and leave. The amount of time wasted looking for supplies and money wasted on supplies was staggering. But it’s not just the nurses who don’t put things away, it’s the respiratory people too. I watched each one and over the course of a few days, even when Dear Son had nebulizer treatments every two hours, not “one” employee bothered to toss away the little plastic container that the albuterol came in, in the trash. Not one! All of them left it on the bed, on the tray table or in the room. If you don’t think this is a big deal, then think about this for a minute. If you aren’t in healthcare and are let’s say, in the business world, can you imagine for a minute going into a client’s place of business and leaving your trash on their desk, say a water bottle? What would the client think if you left an empty water bottle on the CEO’s desk before you left, or a Kleenex? Do you think he would want to do business with you or your firm? Do you think he would think you were “unprofessional” if you did that? Would you leave your trash on a conference table? Probably not. Only in healthcare will you find employees who think nothing of leaving trash with the client (patient). Probably because most healthcare workers don’t think of patients as clients.

The biggest drain on me was the state of the room. I knew with his vent settings increasing, we weren’t going home any time soon. I remember telling my mother that I would “never get out of here”. It was so depressing. I wondered if my Dear Son would live. And I couldn’t help but be depressed by the state of the room. I wonder if the staff ever stops to think about how their actions affect the patient’s mood or even how their messiness affects everything. Lack of putting things away in a patient’s room or a lack of order sends these messages:

• A messy room generally indicates that you don’t care about something. If you like your job because of the patients, do your actions of not putting things away, reflect that?
  
• How do you feel when you are in a messy room-whether it’s at home, a friend’s house or anywhere? Does it make you feel good and bring you up or does it bring you down?
  
• Due to Infection Control procedures, whatever comes into a patient’s room can not be brought back to the supply room, so the patient is charged for these items. Do you think it’s fair to overinflate a patient’s bill due to your lack of organization? Would you like your hospital bill like this?
  
• Many people disdain waste today and are into recycling and being green. Do you think your actions are “green” when you bring in excess supplies only for them to be tossed? Do you thing that’s being “green”?
  
• What impression do you give your patient’s when you leave trash in the room or when you can’t locate simple items in the room because you misplace them. Do you think that gives the patient or caregiver the impression that this is a “magnet hospital” or that you are a “top 100” hospital?


This is a hospital tablescape I created with the plastic plates I brought from home, the flowers Dear Son received for his birthday. I created this because I was happy that Dear Son was extubated the night before I hoped he was getting better. To see more of my tablescapes, click here.

• If you were critically ill and recovering, let’s say in your own bedroom to help give you a picture, would you feel better if the room was a disaster (mess) or would you feel better if it were clean? Would you feel better if someone brought you a fresh glass of water or would you think it’s o.k. if every time you needed something that people brought it to you but left the old glasses, old water, trash, etc. in your room?

On or around ten days of the hospitalization, I had enough. While I certainly pick up after myself, I decided to get the place organized. Remember, I was deathly sick myself with the swine flu and an ear infection, but I needed to do something since I was so depressed. I called the nurse and asked for six empty bathing bins (these are the pink bins that hospital have with bath supplies in them) and attached labels to them. I put changing supplies in one, feeding supplies in another one, monitoring supplies (anything from electrodes for pulse oxygen to heart rate leads, thermometers and so forth) in another, etc. until it was all done. I organized their needles, syringes and flushes the best I could since I didn’t have anyone to ask about all of the tasks they used these for. I made everything as nice as I could. I decided that I may not be able to change their habits, but I would ask them to throw their trash out when they were done with a task, assuming they didn’t.

A funny thing happened when I organized the room. The nurses stopped when they came in the room. They attempted to put things in the right order. They saved time when they did their tasks because they could find things. They commented on how nice it was. When the respiratory people came in, if they tried to leave without throwing out their trash, I simply asked them nicely to throw it out and mentioned matter of factly that I have to live here all day and I’d like to keep the room clean. If they didn’t then I would pick it up and throw it out myself.

Granted, I am a professional organizer so I notice these things but I don’t think that I am being overly picky when I want a patient’s room to be clean and neat. (I should also tell you that I’ve worked in the corporate side of healthcare for many years and oversaw operations as well so I tend to look at how people do things in order to save time or money.) I don’t think any of us want to spend time in a hospital that isn’t clean or is very messy. Given a choice, you always think the hospitals that aren’t clean or are very messy don’t give good care.

After we got out of the ICU, we went down to the main pediatric floor. I did the same thing and used the pink bins. I’ve done this for some time now but I guess it’s never been as bad as it was in the ICU this time. I remember people now by how they leave the room. There is one little old lady that draws Dear Son’s blood. She leaves a mess every time tossing only 75% of her trash and leaves bloodied cotton balls and band aid trash on the bed, every time. I know which cleaning people will always clean the floors and which ones skip over that task. I know which nurses put their tools away when they are done and which ones aren’t organized.

And finally, I should mention this before someone brings it up. I do realize that nurses are very task oriented and that they are very busy and don’t have time to “organize” a room. I am not expecting that. What I do expect is that a healthcare worker do what the rest of us do: put things away when we are done and take care when bringing supplies into a room.

In the end, I came to a few conclusions:

• The best nurses were the best managers of their time and had good organizational skills.
  
• The worst nurses were poor time managers and had the poorest organizational skills. While I am careful when I say this, it was easy to see the examples. For example, the ICU night nurse, who had only Dear Son to manage, waited until 6 a.m. when we were changing him and wanted to give him a bath. Both radiology (chest x-ray) and respiratory were outside the room waiting to do their tasks when she wanted to start a bath. And yes, she sat at the pc outside the room all night looking bored.


• 90% of the nurses never made a list of supplies that were needed for the room prior to fetching them. A list would have saved them time from running back to the supply room again and give them "more time" in their day. This was especially problematic when they would attempt a procedure with another nurse, find out they don't have the supplies, start the procedure and send the nurse running back to get more only to find when she returned that the other nurse forgot to mention one of the supplies they need. From an operations standpoint, I might suggest hospitals create a standard supply list for each procedure, then when nurses are rushed, they get what they need without having to think about it.
  
• When given a list of supplies that I needed, only about 70% brought back the items on the list. Some of them forgot they had a list or forgot to read it. I know this because they told me even though I didn’t ask. Keep in mind that in the ICU at this hospital, the nurses only have one or two patients max so often Dear Son is their “only” patient, unlike the pediatric floors.

So the next time you spend time in a hospital or if you are a healthcare worker, ask these questions:

• Am I picking up my trash and putting things away when I am done?
  
• Am I padding the patient’s bill by bringing in excess supplies?
  
• Am I being “green” when I overstock a patient’s room with excess supplies because I didn’t think about what is needed?
  
• Am I upholding the hospital’s image of a “top 100” hospital or “magnet status” when I do these things?
  
• Would I feel good in this hospital if my room were left in this condition?

Keep in mind, we are all busy at our jobs today and that is no excuse to leave our trash and not be organized. There are a lot of people that would be happy to have a job.

Photo of Dear Son's hospital bed (with his Tendercare headboard) at home.

And the next time you are in the hospital, take a look around. What can you do to help keep things neat and organized and make it easier for everyone? After all, doesn't everyone feel good sleeping on clean sheets in a nice clean room?

Note: Dear Son is eighteen years old and suffers from a progressive neurological disease. In addition, he has intractable seizures, dystonia and is severely retarded due to a random mutation of the ARX gene. He was hospitalized in November 2009 for 26 days, 24 of which were in the ICU at a Big Academic Medical Center, for the swine flu and a viral pneumonia.

Dear Son Medical Update~Better

Things have improved with the Scopolamine patch back on. While I can still hear the secretions, it is slightly better. Also, I made two changes that seem to help. He had been moaning in his sleep each night and while I tried to turn him over to make him more comfortable, it didn't seem to help. I am assuming the moaning was from pain since he never did it prior to this pain issue. Usually he would start moaning around 9 p.m. or so and when he did that, I gave him .5ml of Risperdal and it seemed to help and then another .5 ml around midnight, when I gave his other seizure medications. (Ped Neuro Doc had given me the o.k. to use this.) That small change means he's more comfortable and not moaning as much; it also means he's sleeping a lot better. Now, he only wakes me up to be rolled over or changed. I also increased his feeding slightly. Prior to the swine flu, he was on 4.5 cans of formula a day. After that, we decreased the formula to 4 cans so he wouldn't aspirate. It stayed like that but then Dear Son kept waking up earlier and earlier for me to get his food started. When he was consistently waking up from 3:30 - 5 a.m. I decided to increase it to 5 cans a day (which is 1250 calories vs. 1000 calories which is 4 cans). Now he sleeps later and is waking up around 6 a.m. I always know when he wakes up and is hungry because I'll try turning him over and seeing if he's wet when he's fussing then start his food if those don't work. Once I do that, he always quiets down and goes back to sleep so I know he was hungry.

Anyway, I am on day 3 or so of the new plan and he's sleeping better and so am I. It is always amazing how much better I feel and how much more I can get done when I have had almost a full night's sleep. Dear Son is waking up and seems happier. The smiles are few and far between yet, but he definitely seems better with the new regime.

I am anxious to see what the CT scan will show. Dear Son still is not able to sit up in the recliner but I do try to sit him up every day. Typically, he'll sit in there for a bit until he feels pain and starts to cry. Then I move him back to his bed. I have to try to sit him up every day though to help keep his lungs clear. Once they figure out what's causing his pain from sitting, we'll probably be able to dump the Risperdal.

Dear Son Medical Update~Back to the Patch...for now.

Over the past few days, Dear Son continued to have difficulty managing his secretions. He has a lot of trouble sleeping at night with the secretions and was moaning out a lot of the time. Although I have the head of his bed raised and I frequently roll him over to change positions, he still has great difficulty breathing and I can hear the rattling of the secretions in his throat. Some of his moaning can be quite loud while he is trying to clear his airways. While you and I might cough to do this, Dear Son really doesn't cough at night and has trouble coughing during the day. He also moans out in his sleep regardless. This is new and I am not sure if it's pain related or what but it's hard to listen to when nothing I do seems to help.

I spoke with the doctor today and talked to him about the gel. I told him that I didn't feel it was working at all and I was quite concerned he might aspirate on his own secretions. As a result, he put him back on the Scopolamine patch for now but we'll follow up with another CT scan of the abdomen next Tuesday to see what is going on with his bowels. There really aren't any other pharmaceutical options to dry up these secretions since it is my understanding that Scopolamine is really the drug of last resort. I must admit I was somewhat relieved by that since I was so worried he'd choke to death.

A few years back, we had tried botox to assist with his secretions but the benefit was short lived, say two weeks or so before he was back to choking again. At that time, we had discussed the removal of the two on the salivary glands, the parotid and the submandibular gland but on one side of the throat only. I asked Ped Neuro Doc (he functions as Dear Son's primary now since he's medically complex) if that might be a viable option now. While I understand the importance of protecting the airway, I also worry about using the patch and having Dear Son screaming in pain again from urinary retention. The doctor will be talking to the ENT with regards to possible next steps. Although surgery (sedation) is certainly a huge risk for Dear Son, I know that we have to get Dear Son to a point where he can sit comfortably, urinate without hurting and breathe without choking on his secretions. It will be good to hear what they recommend.

In the meantime, I am still trying to sit him up in the recliner briefly every day, to help keep his lungs clear. While we are not where we need to be, he is still better than he was a month ago and that is important.

We also had a visit with the school nurse last week. She had called and asked to visit earlier this month when Dear Son was in the hospital. She came to our home to see how Dear Son was doing. I must admit, Dear Son and I were definitely ready for a few visitors! She also wanted to know if we might have an interest in some more visitors from school. We are definitely interested however we have to determine if that might be a viable option with his docs. Certainly, we have to make sure everyone is well first! We also discussed him going back to school at some point. He hasn't been to school since October and the Pulmonary Doc does not want him back in school until late February or early March, after the cold/flu season has passed. Since Dear Son can not clear his airways, he can not risk getting sick again. Also, Dear Son isn't ready to go to school until we can have him sit up without pain and essentially be able to get through his day.

The good news is that they offered to try some therapies at home. We'll see Ped Neuro Doc at the end of February and see if we can get some approval for therapy and for his teacher to do home visits, say once a week or so. If he tolerates that, then we can figure out the next steps in terms of riding the bus and going to school. They have offered to do whatever schedule he needs, anything from school for two or three hours one day or week or whatever he needs to get back in the swing of things. Even if he can't sit up, they can do some therapy while he is in his bed. They have to check the IEP to see how they can meet his educational goals to do this.

I think having some home visits by the therapists might be helpful. I can have the therapists do some leg exercises and leg massage to help increase the blood flow in his legs/calf. I can have some speech therapy to help me with keeping his teeth cleaned. After Dear Son had all of that suctioning, he's reluctant to let me brush his teeth now, something he used to love. They have exercises they can do to help Dear Son relax his muscles and open his mouth for me. In terms of therapy in bed, I am hoping they could help me help him turn over. Dear Son can't roll over so I have to turn him over at night. I grab his hips to turn him however he used to be able to take his arm and move it over as I moved his hips but now he can't do that and I have to lift his hips and then once they are positioned, then lift his arms and turn the top part of his body over. It's not very easy. Plus, it's also a bit self serving. I would be thrilled to get a little help here with some of his tasks. While many people have offered, I have always turned down any help but now I am getting a bit weary of 24/7 care and I am ready to put anyone that offers to work, lol!

We are quite fortunate to have such a wonderful school system that is willing to do whatever to help Dear Son. On a sweet note, his teacher wrote me and was asking when she could schedule a visit. She mentioned that she was assigning new jobs for the students for the new semester when one of the girls asked what Dear Son's job was going to be for the new semester. They have jobs like shredding, plant watering, folding towels, etc. She said Dear Son's job was to "get healthy"! I think that sounds like a great plan.

As for me, I am ready for all of this to be over. I still have a stack of medical bills to go through. Hopefully, I'll get that done next week. This week, I set up a new home healthcare provider and worked on another medical issue. I am anxious for Dear Son to be well again and to see that happy face again. I am also ready to get back to my tablescaping and some other things. I haven't had any time to do anything for me or to get out of the house much so I am definitely ready for that. Thank you for all of your wonderful comments and suggestions for Dear Son.

Dear Son Medical Update~Scopolamine Gel

Dear Son has been using the Scopolamine gel since we received it on Friday afternoon. I had paged the neurologist on Friday afternoon due to Dear Son's crying. He suggested a small dose of Risperdal Friday afternoon and at midnight. Between the gel and the Risperdal, Dear Son slept great. Friday night could not have been better. I went to bed at 12:30 p.m. and he didn't wake up until 4:30 a.m. Sometimes he gets hungry and likes his food started early so I started it around 5 a.m. I was up on and off after that with him for food, meds and to turn him over but generally he woke up happy, with a smile on his face and things seemed great. As a matter of fact, it was one of the his best days yet. He seemed like his old self.

On Saturday morning, I sat him up in the recliner again and he cried. For some reason, he can't seem to sit up more than ten to fifteen minutes before he starts crying out in severe pain. This is concerning to me since it's been going on for quite some time and we don't know why. I try placing pillows behind him to position him differently but nothing seems to work. He cries so much that he has a seizure. I end up using his VNS a few times and putting him back to bed. He is happy to lie down again.

After he left the hospital, I felt confident that the urinary retention was the problem for his pain and still do. At the hospital however, he never got out of the bed. They don't have a hoyer lift there and it's impossible to sit him up in the chair so we had no way to know that sitting was still a problem. I am concerned at to what would still be causing the pain-is it the bladder or is it pain from the pneumatosis? They say there should not be pain from that but I wonder. When I take him out of the recliner and put him back in his bed, he's happy.

While the gel seems to allow Dear Son to be pain free, since it's not causing urinary retention, I don't believe the gel is working at all. Dear Son's secretions have increased and he is choking on them which means that the Scopolamine gel is not drying up secretions like the patch did. The pharmacist said I could use up to .4 ml/day which I did on Saturday but it doesn't seem to have much effect. I am worried he will choke to death. In the meantime, I am trying to give it a few days to see if it will work. I have also started to track how much I am giving him. I was using it whenever he choked on Saturday but tried to decrease the amount since then using it only at night. My fear is that he will choke on his secretions when I am not in the room or he will choke at night when I fall asleep. He choked on his secretions last night when I went to bed and had a few episodes of apnea after some of the choking. It wasn't a great feeling. I worry he'll choke or aspirate on his saliva. I was also up a fair amount, every 90 minutes or so, could be more, I can't be sure, so it wasn't as good as it was Friday night, although I had not given him anymore Risperdal.

The actual medication is more like a foam than a gel. It's almost as if it the medication were "whipped". It seems to be mostly air to me. When I attempt to administer .1ml and put it on my gloved finger, .2 ml comes out. It's extremely difficult to get the exact amount out of the syringe. Also, when I try to pull back on the syringe, to pull .1 ml back into the syringe, it seems like it might have worked and yet when I go to adminster the next dose, sometimes nothing comes out at all so I wonder if anything was retracted at all. I am rubbing it on both wrists and sometimes on one wrist so I probably need to make another call to the pharmacist to make certain I am administering it correctly. I wonder how much remains on the rubber glove.

So at this point, I need to give it a few days to see how it goes. As with most medications, you really need to give it a week or two. Also, because he can't swallow or clear his airways, I'll have to be pretty careful. I get an awful feeling however, that something isn't quite right with this sitting. I would have expected that to improve somewhat but things haven't changed much where that is concerned.

In terms of the big picture, I am beginning to think that we are entering a "no-win" situation. We have to keep his airways clear. If the gel doesn't work, then perhaps the only option is surgical removal of two of the saliva glands. If Dear Son's two greatest risks are sedation and aspiration, then surgery would present a risk with the sedation. I don't know what other options there are, but I'll have to leave that to the physicians.

Dear Son Medical Update~Things are a Mess

This is a picture the day he got released from the hospital, earlier this month. It's not the greatest picture, with his long hair and all, but we were happy to be going home after two weeks.

Things have been difficult this week, to say the least. Dear Son is yelling/crying out a lot. I have removed his patch, put half the patch back on and then put the full patch back on and yet nothing seems to help. All of this has been to try to stop the crying.

Last night, Dear Son was up a minimum of once an hour from 2:30 a.m. on. I am exhausted. This went on for hours. Finally, I got up and tried to sit him in the recliner for a while but he began crying out so much that I had to put him back in his bed. The fact that he can't sit up at all is concerning to me. I ended up paging the doctor to see if there was anything I could give him to stop the crying. He told me to give him some Risperdal.

The good news is that the courier delivered the Scopolamine gel today. The gel replaces the patch and allows us to adjust the dose so essentially, he gets a dose that will last 4-6 hours to help dry up secretions at night and then conceptually, he won't need it during the day. As you may recall, he began to have the side effect of urinary retention with the entire patch; his bladder was huge and he was in pain trying to urinate. Hopefully, this resolves his issues.

I thought it might be interesting to talk about the types of questions that I ask when he gets a new drug. Many parents ask me what types of questions should they ask so I thought I'd share some of the questions that I ask and talk a little bit about why I ask them.

In this case, we have a drug from a compound pharmacy in a form we've never used. Since the drug was delivered by courier, I didn't have a chance to speak with the pharmacist directly but he did tell me to call him when I recieved the medicine. In this case, I was not able to check out the pharmacy, but in cases where they are making the drug, I would have liked to have seen the pharmacy, specifically, to see how clean they were. In this case, while I didn't get to see the place, Dear Son's physician specifically recommended the pharmacy so I had some assurance that they would be o.k. In addition, their automated voice recording states that they are a preferred provider of the largest insurance firm in our state.

This is how the gel arrived. This is a three month supply.

Prior to calling the pharmacist, I am going to get my questions ready. After seeing the packaging, I could tell that the prescription should be stored in a dark area and it looked like it might need to be refrigerated. I would have assumed it would have come in a refrigerated type package, but it doesn't hurt to ask. I'll confirm both of these issues. Next, the Scopolamine patch that Dear Son was using was a 1.5 mg patch meaning it delivered 1.5 mg of Scopolamine over three days or .5 mg per day. So if the goal is to use the drug at night, I assume that the dose would be .25 mg or half of the daily dose of the patch. Although the directions that came with the gel state to use .1 ml (.25mg) to .4 ml as directed every 4-6 hours, I will have him explain this to me. Next, I need information on how it should be applied. Over the phone, he mentioned to Dad that it should be applied on the wrists and rubbed in. Next, I wanted to know if the gel had a half life and finally, I want to know if there are any contraindications of the gel meaning what are the signs that I should stop using the medication asap. You should always know that for each medication as well as know the major side effect of the drug.

So to summarize, prior to my call, these are my questions:

• Does the gel need to be refrigerated?
• Does it need to be away from light?
• The Scopolamine patch was 1.5 mg patch for 3 days or .5 mg/day or .25 mg for 1/2 day or 12 hours; with the Scopolamine gel, is my goal to use the same amount as before for x number of hours?
• How do I apply it?
• Does the gel have a half life?
• Are there any side effects or contraindications of using the gel?

In this case, I am looking to speak with the pharmacist himself and not a pharmacy tech, to get the answers. I call the pharmacist and we discuss the following:

• The gel can be kept at room temperature and does not need to be refrigerated. Refrigeration could break it down. (This is good information and that's why you want to ask these questions. Also, if it were summer time and it was warmer inside, you need to know if it should be refrigerated.)
• It needs to be stored in a dark place at room temperature.
• Regarding dosing or how much to use, he explains that the Scopolamine patch works differently. It delivers .33 mg/day but the penetration rate is higher than the gel and it also has a slower release into the bloodstream. The advantage of the Scopolamine gel is that it is an immediate release into the bloodstream.
• Dosing-He goes on to say that it will be trial and error with the gel to get the dosing right. Each person absorbs the gel differently and there isn't any way to tell how fast or slow that a person will metabolize the gel. (We know from past experience that Dear Son is a hyperutilizer of medications.) The pharmacist has us start with .1ml (.25mg) at bedtime and see how he does. It should last 4-6 hours. The recommended dose is 2-4 times per day (24 hour period) however that is if we wanted the same coverage as the patch; in our case, we are looking for partial coverage or coverage at night and then no coverage during the day. He explains that the advantage of the gel is the ability to adjust the dose and it's a different release mechanism; the gel can be more cumbersome to administer in higher doses however if we get to the point where he is using a higher dose or the same amount that the patch delivered, then we need to go back and talk to the physician.
• In terms of application, he states we can apply it to the wrists, the tops of the feet or at the base of the neck, anywhere where there are blood vessels where it can be absorbed. (In Dear Son's case, we know that he has edema in his feet so his feet would not be the ideal place to apply this gel.)
• Does the gel have a half life? Yes, the gel's half life is the same as the patch, 3-6 hours.
• Are there any side effects or any situation where I should stop using the gel? The gel is hyperallergenic; the only contraindications would be urination (or lack thereof) and if his breathing gets worse.

After our call, I decided to try out the gel on Dear Son. First, there is the practical application of this gel. The goal is to use it at night so I am planning on giving it to him at midnight with his other medications so it will last hopefully until 6 a.m. Now then, here is the syringe and I am supposed to give him .1ml, or a tenth of this syringe. I can barely read the syringe in broad daylight and with reading glasses on, lol, let alone do this at night in the dark. Next, I'll put it on his wrists and rub them together. Sounds easy until you see Dear Son. His arms are tense and it's not the easiest thing to do.

This is the picture of the syringe. It is a white gel with white letters. I will be giving him one tenth of this syringe for a single application. I think black letters might have been easier to read considering the gel is white.

Around ten p.m. tonight, I changed his diaper and he was choking on his saliva. I decided to give him a second dose of the gel since the goal is to keep his airways clear. This time, I use rubber gloves and put the exact dose on the glove under the kitchen lamp, so I can see. This way, I won't need reading glasses. When I do that, .2 ml squirt out. Yikes! I use the syringe to put .1 ml back in so hopefully, that occurred. With the rubber glove on, I rub it into his wrist and then try to rub his wrists together. I may end up rubbing this in on both wrists myself instead of trying to rub his wrists together when he's sleeping. (The rubber glove is extremely important! You do not want to get Scopolamine in your eyes or on you since it can have the same effect. If you get it in your eye, it will cause your pupils to dilate and blurred vision. I know this because it happened to me once. I must have got it on my fingernail when I removed his patch. Although I am a diligent hand washer, stuff happens. My pupil was dilated and it took three days for my vision to return in that eye. )

So hopefully, tonight will go well and he will sleep. The last three months have been really challenging for him and for me. I think it's probably been the most difficult for me namely due to all of the crying and lack of sleep. I sincerely hope that this gets resolved. My nerves are about shot trying to make him more comfortable.

The great news is that it will resolve the urinary retention issue with the patch. Regarding new prescriptions, you certainly don't have to ask all of these questions but I like to understand how drugs work. As an advocate for Dear Son, it's helpful to learn these things. Now certainly, I've grown into this role over time. I can't imagine that when Dear Son was a baby and he got medications that I would be asking how they worked since I am sure I'd be overwhelmed with everything and the process. But now that we are in the end stages of his disease progression, and now that he is really medically complex, it helps to understand how things work since there are so many issues going on.

Dear Son Medical Update~Solution?

After my last post, Dear Son continued choking on his secretions. I tried 2/3s of the Scopolamine patch and then had to put the whole patch back on so he wouldn't choke or aspirate. Once I put the whole patch back on, Dear Son hasn't cried out from choking. The only time he cries a lot is when he sits up in the recliner or wheelchair. We are not sure why. Lying down, he simply sleeps and cries out when he needs to be turned over. He is sleeping however most of the day and night but does need to be turned over quite a bit so I am still not getting a heck of a lot of sleep.

I spoke with Ped Neuro Doc on Monday and we are going to switch from the patch to a Scopolamine gel that will be made by a compound pharmacy. It takes a few days to make so it should be ready by tomorrow or Thursday. The gel can be rubbed on Dear Son before bed and will last from 8-10 hours; if he needs another dose, I can do that again. This will give him the benefit of the Scopolamine drug in drying up his secretions but also prevent urinary retention since it's only on part of the day.

If that works, then the only issue remaining is to figure out why he cries when he sits up in a chair. At the hospital, he was lying in bed the entire time because they don't have a hoyer lift or any way to lift him out of bed into his wheelchair. I am not supposed to do that anymore since I had back surgery so I don't risk it.

Once we get the gel, we'll see how things go. I imagine at this point, Dear Son just needs to sleep and get well after these last few weeks. Dad has him today and tomorrow, so I can work. It will feel great to get a good night's sleep.

Dear Son Medical Update~ Mixed Bag

Things have been a bit of a mixed bag since we've been home. As you may recall, Dear Son was hospitalized for pain/crying two weeks ago and was diagnosed with Pneumatosis Intestinalis and urinary retention due to the Scopolamine patch (He wears that to dry up secretions.). Thursday night, he cried out on and off all night, much more than he did the last few days at the hospital. He slept all day on Friday and then Friday night, things seemed better. He slept relatively well and it was as good as it was at the hospital-very little crying out except when he needed to be rolled over.

Friday morning, he looked great. I got him up and sat him in the recliner and he started crying. He cried out as if he was in pain for quite some time. It seemed like leaning back even slightly, causes him some pain. Finally, I moved him to his wheelchair since the back is straighter and he is upright. That still didn't work. He cried out in pain for some 2.5 hours. I wasn't sure if I should give him some Tylenol for pain for try some Risperdal. I paged the doc but didn't hear back. Finally, I gave him some Risperdal and put him in his bed where he settled down and slept for a few hours.

Last night, he cried out most of the night. The issue seemed to be secretions. The half patch isn't working very well in terms of drying up secretions so he's choking on his saliva. In desperation, at 3 a.m. I gave him some Risperdal. Some time later, I removed the Duoderm and put the whole Scopolamine patch back on. I am sure much of the med that was on that side of the patch had been removed, but it was still more than the half. In general, I know I need to protect his airway so that he doesn't aspirate however I worry that if I go back to the whole patch, he'll be screaming again in pain from the urinary retention.

In terms of urinations, it's a mixed bag. They have decreased over time:

1/09/10: 9 urinations (Decreased patch to 1/2.)
1/10/10: 7 urinations
1/11/10: 8 urinations
1/12/10: 5 urinations
1/13/10: 3 urinations (Stopped IV fluids and TPN and started our formula/water regime.)
1/14/10: 3 urinations
1/15/10: 1 urination
1/16/10: 3 urinations

After being up on and off most of the night, he's sleeping now. Typically, he has the most issues between midnight and six a.m. They tell me that most kids have the most trouble at night.

I suspect that he may never be totally well again. While things seem bad at times, for the most part, it's a heck of a lot better than the blood curdling screams he had prior to being hospitalized. The only time that occurred was yesterday for 2. 5 hours when he was in the recliner and wheelchair. I thought perhaps it might be the pneumatosis however the GI surgeon assured me that it doesn't cause pain. I'll have to touch base with his doctor tomorrow.

Dear Son Medical Update~Home

Photo of Dear Son's Tendercare Hospital Bed

We just got home from the hospital yesterday. Since I last wrote, we made a lot of progress.

For the pneumatosis, Dear Son completed the antibiotics they prescribed. They had him on a seven day course which was done as a precaution. One of the causes of the pneumatosis intestinalis can be infection and although he didn't show any signs of that, they prescribed a seven day course, just in case.

In addition, they took him off of the Miralax and changed his Phenobarbital from a liquid form to a pill form. One other cause of pneumatosis can be medications. In this case, they know that too much Sorbitol can sometimes cause this condition and while they don't know what caused it, they are doing everything they can. They reviewed all of his medications and determined that Miralax, Valproic Acid, Phenobarbital and Lamictal had Sorbitol in them. They removed the Miralax, left him on the Valproic Acid since there isn't a pill form, left him on the Lamictal since it already was in a pill form and changed the Phenobarbital to a pill form. My main concern was the Miralax. They decided not to replace the Miralax with anything and I worry he might get backed up since I know that he needs some laxative along with his formula. They told me that when they analyzed his medications, there were 14 grams total of Sorbitol in them and 10 of those grams were in the Phenobarbital. So the good news is that most of the Sorbitol was removed by simply replacing the liquid Phenobarbital with the pills.

In addition to these two things, they had taken Dear Son off his formula tube feedings and replaced that with IV fluids and nutrition via his central line. This gave his bowels rest for a week. They do not believe that the pneumatosis causes him pain however nor causes him to cry. Moving forward, they did not do another CT scan of the abdomen and the only other recommendation from GI Surgery was for Dear Son to have a colonoscopy in a few weeks after this pneumatosis subsides.

In analyzing his CT scan, they noticed that his bladder was huge. They determined that one possibility was the Scopolamine patch causing urinary retention. They decided to use half the dose; since it is not recommended that the patch be cut in half, pharmacy recommended that half the patch be placed over some Duoderm (it's a clear plastic piece of medical type tape), which is what we did. Since he got only half the dose, his frequency of urination increased. Initially, he seemed to have severe pain ten to twenty minutes prior to urination, where you could actually see him crying out and trying to bear down to pee. When this happened, they consulted with Urology. They did a renal ultrasound to look at his bladder and kidneys. In a small percentage of cases, kids who are on Topamax can develop kidney stones. The renal ultrasound was normal and no kidney stones were found. The painful urination subsided after two days or so once he started urinating a lot. He went from two urinations a day to eight or nine with the half patch (although is total liquids increased from 1500 ccs to 2350 ccs). On Tuesday/Wednesday, he returned back to his normal volume of 1500 ccs and his urinations decreased to five and then three. His crying decreased drastically as soon as the half patch took effect.

On the down side, as soon as the patch size was decreased, his secretions increased so I have to watch him carefully to make sure he can clear his airways. As a result, we are going very slowly on his feeds and feeding him 24/7 at a rate of 60 ccs an hour.

Regarding the central line, it started to get red and hard and show signs of infection after just three days. They removed it and tested it for bacteria and it came back negative.

As for seizures, Dear Son had become very agitated and was shaking a lot. It had gotten worse since December which was when another Attending Neuro Doc had made some medicine changes. Dear Son was shaking so much at times that he looked like he had Parkinson's. I spoke with Ped Neuro Doc about increasing the Klonopin dose and he increased it nearly back to previous levels and Dear Son improved.

During our stay, they also had turned off his VNS for a day, to see if that might be a source of his pain however it wasn't. That was prior to the CT scan of his abdomen. When they turned it back on, they lowered the settings a bit. The only down side is that Dear Son is having a few more seizures. He had one this morning before meds and seems to have one every few days prior to his morning meds. At this point and time, Ped Neuro Doc wants to leave our meds and VNS as is. I am able to control these seizures with the VNS however.

Dear Son improved quite a bit with the switch to the half patch. It appears the crying was due to the urinary retention. While he still cries when he needs to be changed, rolled over or for a seizure, there was little other crying other than a few episodes here or there. I felt very good that he was no longer crying and screaming. Interestingly enough, one of the night nurses came by on Tuesday morning to see how Dear Son was doing. She was glad the crying was resolved and said that she heard a kid crying and screaming at the top of his lungs last week and asked the other nurses who was screaming. They said it was Dear Son. She heard him all the way down the hall at the nurse's station. She has known Dear Son some ten years there and told them that it wasn't like Dear Son to cry like that since he NEVER cries.

So I felt pretty good when Dear Son had stopped crying at night. You could see the pain had lifted from his face. Prior to that, he always seemed in pain, even when sleeping. I felt really good and thankful that this issue had been resolved. I was on cloud nine. It was really something to think that only a week or so ago, they had told me he may never stop crying since they thought it was disease progression. I felt even better when one of the nurses stopped by Wednesday night to see how Dear Son was doing and he started laughing, smiling and flirting with her. Then I knew that Dear Son was back! It was such a pleasure to see that smiling face again!

At that point, I knew we needed to get home and see how Dear Son would do in his normal environment. Dad came down to pick us up for the discharge. When he put Dear Son in his wheelchair and leaned him forward, Dear Son screamed out in pain. I was a bit concerned but still optimistic. We left the hospital and he cried in Dad's car all the way home. Sometimes, he cries in his car and I am not sure why. I don't know if it's the angle of the seat or whatever but it happened prior to his issues. When we got home, he sat him in the recliner and it seemed like he was in pain. Dad kept saying how Dear Son looked to be in pain and I said that we needed to give Dear Son some time since he hasn't sat up in two weeks. Well, Dear Son started crying and Dad left. Like really crying again. I was demoralized. I could not believe it was happening. I was almost suicidal to think that after such great results with hardly any crying the last few nights at the hospital that this was happening again. I tried to remain calm and got his bed ready then moved him to his bed with the hoyer lift. As I removed his pants, I noticed that he had a dirty diaper, hence the source of the crying! Yay! The night went o.k. and then from 1 a.m. on he cried out on and off and I got up to see what was going on. I stopped his continuous feeds at 3 a.m. since I heard him gurgling on his secretions. He continued to cry out once an hour or so all night until this morning. He had a seizure this morning prior to meds.

Overall, I do think we resolved his issues with the crying. I think it will take a little time for Dear Son to recover from everything and I expect that he still might cry however it is nothing like it was. I am so happy that they were able to resolve his pain issues. I knew that he wasn't crying for nothing and I knew he was in pain. Moving forward, I suspect our next issues will be motility, or lack thereof since he's not on the Miralax and controlling his secretions.

In the end, I am really thankful that they got this resolved. Dear Son's physician, Ped Neuro Doc really did an outstanding job. He worked tirelessly and spent a ton of time on this issue. I can't thank him enough. He reviews all of the test results himself, checked out the CT scan and noticed the bladder issue, came by to see if the MRI was done prior to going home. He just gives his best all the time for his patients. He has been Dear Son's physician for 18 years now and I just can't imagine what would happen if we had someone else. We have received such an outstanding level of service over the last eighteen years that I honestly have no idea how he does this. He not only takes good care of Dear Son but all his patients like this. I am going to write him a thank you note later today or tomorrow, when I get a spare minute.

And finally, I apologize for the delay in updating you. While Dear Son was in the hospital last week, I received a notice from my apartment complex that there had been a recall on the sprinklers so they needed us to remove all of the furniture in our apartments located under the sprinklers so they could replace them. Well, I had just got home from the hospital and tried to move everything really quick. In doing so, I tried to push the solid wood base of my dining room table with my right foot and injured it. I either broke or strained the first metatarsal in my foot and can't walk. I was in severe pain trying to work on Wednesday and driving was horrible. I had an x-ray done on Wednesday and found out that I sprained my foot. In the meantime, I am in a lot of pain so I wasn't able to sit down to update you prior to now. I have been icing it and taking some pain relievers but it still hurts to try to stand, walk, sit or do much of anything.

Thank you for all of your comments, concern and prayers for Dear Son over these past few weeks. It is really appreciated.

Dear Son Medical Update~Progress

Dear Son remains at Big Academic Medical Center and will be there through Wednesday. GI and GI surgeons poured over the CT scan of the abdomen and have seen Dear Son. GI surgery states that he has Pneumatosis Intestinalis. Essentially, Dear Son has a significant amount of free air in the lining of the bowel wall. They are not sure how it got there however typically there are a few causes of this: 1) medication, 2) infection and 3) not enough blood flow to the area. This condition is typically seen in preemies but rarely in adults. The free air is located on the right side of the bowel lining and some on the left.

In Dear Son's case, he doesn't have any infection. He doesn't have a temperature and the blood works looks good. His abdomen is not distended nor does he have diarrhea and he is not constipated (Actually, he's totally cleared out!). He does not have any bloody stools. They did a stool sample to make sure that he didn't show any signs of this (the blood can be microscopic) but I knew he didn't have any bloody stools because I change him all the time. I also knew he wasn't backed up because I track bowel movements and between his all natural formula with fiber and the Miralax, it wasn't an issue. That's the good news.

They said that there are cases in the literature of this with prolonged use of Miralax. The culprit seems to be Sorbitol, which is in Miralax, so they reviewed all of his seizure medications to determine if Sorbitol is in them. Dear Son is on six seizure medicines, in addition to his VNS. They found that three of the seizure medications, Lamictal, Phenobarbital and Valproic Acid, contain Sorbitol. They have left him on the seizure medications but discontinued the Miralax for now.

In isolated cases of Pneumatosis Intestinalis, the cause is most likely an infection. While Dear Son does not show any signs of infection, they still must treat it as such so he is on two antibiotics both via the IV. He is being treated with Flagyl and Ceftriaxone. One has a seven day course which ends on Wednesday so we know he will be hosptialized until Wednesday at the earliest.

Since he doesn't show any signs of infection, they assume this is incidental. They are treating him by stopping his feedings to give his bowels a rest.

On Friday morning, they put a new IV in his hand and on Friday evening, put an central line in him so they could give him nutrition via the IV, in addition to his antibiotics. They weren't sure if they could get a central line in since he's a hard stick with the Scopolamine patch drying up everything, however fortunately, they were successful. It was also challenging because Dear Son's biggest risks are sedation and aspiration. Sedation is a big risk due to respiratory depression; last May they used a sedative that depressed his respiration and he developed a mucous plug and his lung collapsed. Aspiration is a big risk because he can't clear his airway. The central line was a big risk for him since it required sedation and required him to lie on his back. Since he can't clear his airways, we had two risks here. Fortunately, they were able to use a light sedation like they did for the MRI and it worked.

Note: As parents, you always want to understand the medical risks for your child and to ask questions. In this case, I wanted to know what sedation would be used. I also ask them to spell it and wrote it down. I wanted to know how they give it, meaning do they give a dose and does it put them to sleep or is it continuous. In this case, it was continuous and the sedation is given via the IV during the entire length of the procedure. Finally, you need to understand how the sedation works. In this case, the sedation will lower his heart rate and blood pressure as opposed to suppressing his respiratory system. They used Precedex, a short acting sedation, which does not cause respiratory depression, a risk for him. I should also note that on Tuesday, when they did the MRI, they used this as well. That was good information to have because they were debating which sedation to use and I said that the Precedex was used for the MRI. Finally, know what the side effects are and how long the sedation should last. In this case, the side effects are low pulse and low blood pressure and the sedation should last from 1 to 6 hours after it's been stopped.

In the case of the MRI on Tuesday, there was a glitch-they had to use a special coil because Dear Son has a VNS (the VNS is turned off for MRIs and a head coil needed to be used) and when the head coil could not be plugged in, they had to take him to the adult hospital's imaging center to perform the MRI. This meant that Dear Son was sedated for 2.5 hours versus the hour or so for the MRI. You want to be aware and note what is going on, in case things don't work out as planned. You also want to write everything down and keep good notes. In the case of the MRI, when his blood pressure and pulse were low, I was able to give the docs in his room information on when the sedation was stopped, so that we had a time frame to know when the sedation would be wearing off.

In addition, since sedation and aspiration are big risks for Dear Son, it's important to be aware of what people are doing and using since they don't always communicate well between departments. In Dear Son's case of the MRI, he came up from the MRI with a very low pulse (63) and low blood pressure (71/30), both of which are not normal for him. You should track your child's pulse and blood pressure at the office visits so you know what is normal for them. In this case, they were able to consult with the Pediatric Intensive Care Doc and gave Dear Son more fluid which increased his blood pressure and then he awakened shortly after that. I was also able to give them this information when we came back from getting the central line.

As a parent, I view myself as part of the medical team. I am an advocate for Dear Son and as his advocate and part of the team, I am there to give and get information on what is being done. That does not mean that I am making decisions for them but rather, giving them and getting from them imporatant information for the best possible outcome for Dear Son.

So in summary, they believe it's incidental and are treating it with: IV antibitiotics, stopping feeds to give his bowels a rest and switching him to IV nutrition. When we do resume the feeds, we need to go very slowly. Actually, we were going very slowly up ever since he left the hospital in November. Going forward, the only thing we could do is a colonoscopy, but that can't be done with the Pneumatosis.

While there can be some pain with the Pneumatosis Intestinalis, they do not believe that this is the cause of his crying. In review of the CT scan, they noticed that his bladder was huge. In fact, Ped Neuro Doc said that he had never, ever seen a bladder that large. They think that perhaps Dear Son is retaining his urine due to his Scopolamine patch (he has a Scopolamine patch to dry up secretions in his throat since he can't swallow due to the disease progression from his progressive neurological disease.) The Scopolamine patch can cause urine retention, which can be quite painful. The Scopolamine patch essentially dries up secretions, which means that it dries up Dear Son's saliva, but also does not allow a person to sweat and make it hard for them to urinate. It is typically used after surgery and for motion sickness however it is also used for cases like this. We had tried Botox injections and Robinul prior to using the Scopolamine patch to dry up his secretions and none of them worked. The Scopolamine patch has been great at drying up his secretions and without it, Dear Son couldn't breathe since he can't clear his airways.

Once they thought he might be retaining his urine, they did a catheter after he voided to measure the amount of urine that was left in his bladder. Two of the times there wasn't anything significant and one time there was more. So in a twenty four hour time period, they had put one IV in Dear Son, put a central line in and put catheters in his penis three different times. My poor baby! That hurts!

Note: As an FYI to other parents, I am always very protective of Dear Son whenever someone wants to put a catheter in since most residents in the ER will almost always want to do this to look for a urinary tract infection, which he's never had! Unless they have a very good reason, I will always challenge this since it hurts. Most residents and medical people have almost never had this done and don't realize how painful it is. In this case, it was necessary and I was o.k. with it but in cases in the ER, I always insist that they pursue non-invasive tests first and I challenge the Attending Doc unless there is good reason. You have to be a good advocate for your child.

Since that time, I started watching him and noticed on a few occasions that he was in pain some ten minutes prior to urinating. He seemed like he had trouble trying to urinate. I shared that with the doctor and they may be consulting with Urology.

They discussed using a contractility agent (meaning something that works against the Scopolamine to help him urinate) but prior to that decided to use just 1/2 of a Scopolamine patch. Since it's not recommended that you cut the patch, we are using tape and then placing half of the patch on his skin and the other half on the tape so only part of it is absorbed. Since that time, he urinating quadrupled and he is crying out less.

At this point, they are watching him and determining next steps. I will return to the hospital today and may not be able to give you an update until Tuesday or Wednesday. We don't have a release date until we get the crying resolved or the urinary retention issue resolved but at a minimum, he'll be there until Wednesday.

Thank you for all of your comments and support for Dear Son.

Dear Son Medical Update~Answer?

It's been a busy week. Dear Son was admitted on Sunday and a variet of blood work was done, including some non-specific markers for infection. The blood work, along with seizure labs, urinalysis and urine culture were all negative. A pelvic x-ray was performed on Sunday to see if there might be any reason for the crying since issues with the pelvis can be difficult to detect in a physical exam. Yesterday they did an MRI and today they did a CT scan of the abdomen with contrast.

The radiologist just read the exam about twenty minutes ago and found "free air in the bowel wall". That's the "official" term. The resident just called me to tell me and I don't have a whole lot of information. From what she told me, there can be a few causes of this: medication, infection and not enough blood flow to the area. They are calling the pediatric surgical resident now to do a physical exam on Dear Son. Since they don't know what caused it, they'll stop his feeding, since feeds require blood flow to the area. In addition, they'll start him on IV fluids. They'll do another CBC test and a stool test (sorry I didn't get the name) to see if there is fecal blood. I asked if I could see it if there were blood and she said sometimes yes and other times no, because it can be microscopic. I haven't seen any blood in his stool and I change his diapers all the time.

The other good news is that he doesn't not have a fever so there probably isn't any infection. In addition, his belly is soft and he's not vomiting. All good signs.

This also fits with why he didn't like to lean back very much. He would cry when I would try to put the footrest up on the recliner and also cry when Dad tried to lean his bath chair back to wash his hair. A few weeks ago, Dear Son would scream bloody murder when he would recline the bath chair leaning him back in the bath chair; when Dad gave him a bath the other day, Dear Son was o.k. and not crying like he was. Over the past few weeks, Dear Son was crying a lot so it was very hard to pinpoint any one spot where he cried more than others.

At this point, we'll probably have to wait and see what the pediatric surgeons say tomorrow. I did tell the resident that the ARX gene mutation can express itself in this area so I really don't have any way to know if there is any connection to the mutation or if this is just a random event.

I'll update you when I have more information. I had to work today so I am home tonight and I'll return to the hospital tomorrow morning. I may not be able to update you until I return home but I'll do my best.

Update~10:59 a.m. 1/7/10: The free air is located outside the bowel wall and is significant. The GI surgeons have been called in and are deciding how to resolve this issue. I suspect that since it's significant, it will most likely require surgical intervention. Just a guess on my part.

Back to the Hospital

Dear Son's crying episodes have continued despite medicine changes. I have been paging the doctor almost daily now and we've been trying some different things without success. Last night, he cried on and off all night and I am exhausted. Nothing I do seems to help him. Out of desperation, I paged the doctor again today and he'll admit him tomorrow. Dad's working today and I can't get him there without some help. I'll post again once I have some information. Hopefully, we can get the MRI done while we are there. At this point, I am desperate to rule out anything that might remotely be a cause for this crying.