Texas-sized Christmas

We have never been down at Cook's near the Christmas holiday.  They go all out with decorations - inside and out.  The pictures here are just a small sample.  The circle drive out front has a 40 ft tree with some decorations that are themselves the size of a person.  Not a live tree, more of an sculpture/art work version of a tree.  Then in front of the main entrance there is a 25 ft Santa in his own little candy land/toy land.  It's a big state so they need a big Santa.

The Poulter's happened to be passing through town from the other direction on our first day here (yesterday).  Here's Pat and Peggy not being menaced at all by the Lone Star sized Santa.  Pat and Steve couldn't stay long and Adam was still dozing from surgery, but it was nice of them to drop in.

Here's a shot of the big atrium inside with tree, candy canes, snowman with list, and a more human size Santa thrown.  Santa wasn't in while we were there so we didn't get to sit on his lap.

What a difference a day makes.  Adam has been his old self today.  He hasn't napped, has eaten well, has been sitting up a lot and was even up and around some.  The main difference from last time is the absence of the severe 'spinal headache' type pain.  He has some discomfort around the incisions and such, but at very manageable and expected levels.  We expect to be able to go home tomorrow.

The pump is turned on and he was getting a small starter dose for most of the day.  The pump is currently set to 100 units of medication per day.  Even at that level he has noticed some more control of his hand and he was able to stand flat on his left foot briefly.  A 'high' setting might be 600 units per day.  This evening they bumped him up to 150 and assuming that works out okay, then to perhaps 200 in the morning.  That is the likely level they will send us home with at this point.  Then we'll come back in a couple weeks for a follow-up.  The trick will be to find the sweet spot where the spasticity is as relaxed as you can get it without turning the rest of the body into too much of a noodle (the Baclofen is a muscle relaxant).  The test we had last August where he was quite a bit looser for a short while was a 100 unit injection, but that was all in one quick shot, so it would be equivalent to something higher per day than these initial levels were at today.

All I Want for Christmas is a Baclofen Pump!

I think I need to catch up the story line a bit here...after the disappointing results from two different Deep Brain Stimulator (DBS) placements (and many, many adjustments, rounds of both physical and occupational therapy) and our frustrating week of a baclofen trial in July, we were all a little weary of thinking about treatment options. We shared our frustrations with Dr. Marks at a follow-up appointment in August, and he suggested an alternative test which would involve an out-patient procedure where a single dose of baclofen would be injected into the cerebrospinal fluid (CSF). This test could let us know if baclofen might have a positive impact on Adam's spasticity. Long story short, Adam had the out-patient procedure the next day, results were very encouraging, and a plan for implanting a baclofen pump was set.

That brings us up to this trip. Yesterday we arrived in Fort Worth for Adam's pre-operative appointment, in preparation for Intrathecal Baclofen Therapy (ITB) surgery. If you want to learn more about ITB, here is the link to the manufacturer's website: http://www.medtronic.com/patients/severe-spasticity/therapy/ 

Our day today began with check-in at 5:45 am, surgery lasted from around 8-9:30 am. Following the surgery (which went according to plan), for most of the day today Adam has been sleeping. This actually worked out fairly well, as he was supposed to stay flat on his back for 24 hours, to try to avoid spinal headaches, shifting of the catheter, etc. As of this writing (around 8 pm), he isn't really eating or drinking yet, still enjoying a lovely saline solution via his IV. And though he has had some short stints of alertness, he then returns to sleepy-time. The team assures us that this is very normal, and that in addition to the body's reaction to anesthesia and surgery, Adam is also getting used to a steady stream of baclofen in his body. All of these events compound to make for one drowsy young man.

The hope for tomorrow is that Adam will be more alert, and ready to let us know if he notices any differences in his spasticity following the surgery and initiation of the baclofen therapy. He is currently on a fairly low dosage, and it may be that the dosage is adjusted before we leave the hospital. He will likely have additional adjustments to the dosage after his body adjusts to the therapy.

The hope for tonight is that we can all get some very much needed rest!

Good News/Bad News

First the good news:

We're back home again, we've had a couple of days to rest up a bit, and we're getting back into our normal routines again.

Adam has not experienced any pain since we left the hospital.

Now the bad news:

Well, it is really less bad news than somewhat frustrating news.

We really don't know any more than when we started our week. Since the trial ended before the medication reached the potential beneficial level, we don't know if it would be a possible treatment for Adam. We don't know if it was the catheter placement that was the problem, or the medication, or perhaps that Adam is very sensitive to a catheter inserted in his spinal column and would've needed a longer time to adjust. We just don't know.

We're not sure yet about the next step--we'll head back to Fort Worth at some point to confer with the doctor. Until then, we're just glad to be home!

If Anything Can Go Wrong...

If you're following our adventure, I apologize for the gap in reporting. It has been a long, strange week, and it appears that even the ending is a little more complex than we had hoped.  Let me catch you up a bit.

After the initial good report following the catheter re-positioning on Tuesday, things just started to go downhill again. He slept well on Tuesday night, but on Wednesday started experiencing more pain. This coincided with an increase in the baclofen dose which may or may not be related. On Wednesday he also stopped feeling up to eating, which clued us in just how bad he was feeling. More pain meds followed with occasional periods of feeling a little better and spending time checking his social media connections via phone and laptop. We even watched a movie, Wreck-It Ralph, which he enjoyed. But the pain kept coming back. He told us on Wednesday that he just wanted to stop the trial and take the catheter out.

Thursday morning was no better, and we shared his wishes with Dr. Marks. He agreed that the pain was not worth continuing the trial, and made arrangements to remove the catheter Thursday at the end of the day. Essentially, we would need to get the dose of baclofen to a level that was double the one he was currently on to determine if it was going to be beneficial, and even if we did that, the pain that Adam was experiencing was going to be likely to trigger muscle tone so we wouldn't learn what we needed to learn. So the baclofen dose was adjusted down instead to allow his body to slowly wean back off of the drug.

Probably the high point on Thursday came when Adam received morphine in advance of the catheter removal, and we saw him the happiest he had been all week! He even shared a joke with the staff, that they should refer to "baclofen" as "back-no-fun" to describe the pain that he'd been experiencing all week in his back as a result of the testing. The plan was that we would be released from the hospital on Friday morning, and begin our return to normalcy, which added to the festive mood.

The actual removal of the catheter went smoothly until the very end, when the tip of the catheter snapped off and remained in his body. An x-ray confirmed the location of the remainder of the catheter and the team determined that it would require a surgical removal which would be scheduled for Friday. As of this writing, we are looking at the surgical procedure to remove the catheter taking place within the next few hours.

Unfortunately, this is most likely going to delay our departure until Saturday morning. They would like to see a more significant decrease in Adam's headaches, and will likely send us home with additional pain meds to continue after departing the hospital.

So...all in all it has been a disappointing, frustrating, and for Adam, very painful week. The doctors have told us repeatedly that they have not seen this series of circumstances take place before, and really don't know with certainty exactly what went wrong. The trial should have been only mildly uncomfortable, the catheter shouldn't have needed re-positioning, the catheter shouldn't have broken upon removal...and ultimately, Adam should not have experienced the miserable week that he has. So maybe now you can see why we didn't rush to keep you updated, we just kept hoping that the situation was going to improve and that we would have some good news to report. At this point, the good news is that we really should be about done here, and should be released tomorrow. But then...this hasn't been a week where the things that were supposed to happen have actually happened. We'll keep you posted.

Storms brewing over Cook's

Last night Adam began to complain more and more about pain in various places (legs sometimes, back a lot and neck and head). He had a pretty bad night and was in a very bad place much of today. We moved to stronger pain killers but the relief was short-lived and he was having trouble with basic activities. It became clear that something was amiss and by midday the doctors were scheduling some imagery (CT scan with dye) to take a look at the catheter position. This is not the usual response to the catheter placement. He was gone from about 3PM-5:30PM getting scanned. By this point he was given some Morphine. The scans didn't show anything clearly problematic but they did back out the catheter a few inches. They had some theories about what might be causing the trouble, but basically they don't really know. But he has been much, much better since coming back from the catheter adjustment. He has been alert and his old self this evening and even now at 9 PM when his earlier narcotics have pretty well worn off, he has only minor discomfort.

Big storms missed us just to the north this evening. And big storms of pain left Adam around the same time. We have yet to get to do any assessment of how the Baclofen might or might not help him. They suspended it for a good chunk of the day and he was in no shape for Physical Therapy testing.

Deja vu 2

We are down at Cook's Children's hospital in Fort Worth again. Last year we tried two different electrode locations with Deep Brain Stimulation. Both showed some promising improvement for a time, but in each case and with each adjustment the trend was for his condition to gravitate back to the baseline. There were a lot of emotional ups and downs.

Last spring it was suggested we try a Baclofen pump. Baclofen is a muscle relaxant used to help control muscle spasticity. It can be taken orally, but typically for strong spasticity as one might find in Cerebral Palsy or Multiple Sclerosis (or Adam), one can't orally take a dose large enough to get the levels needed in the effected muscles. So instead a smaller dose is pumped directly into the fluid surrounding the spinal cord. Before actually inserting the small pump under the skin, the patient's response to the drug can be tested. That brings us to where we are today. Adam had a very short surgery this morning where a catheter was inserted in the fluid filled area around the spinal cord. He can then be given baclofen in a manner similar to having an IV and see how it goes.

This is actually an older more classic procedure than Deep Brain Stimulation. Some doctor's might have done this first, but there are pros and cons. The Baclofen pump is less targeted (for instance, one can't just send the drug to one side of the body). The Baclofen pump has been around a long time and is considered very safely reversible if it doesn't work out.

We have been quieter about this trip than probably any of our others. Experience has taught us to be careful of managing expectations. The surgery itself went fine and Adam has been re-cooperating today. He has had some pain off and on but that is not unexpected. They did start some Baclofen late this afternoon but it is too early to judge things at this point. We are here and tired and we appreciate everyone's thoughts and good wishes. And we are enjoying unseasonably cool, rainy conditions that only happen a few times a century in Fort Worth in July (two consecutive days of highs in 70's with light rain and no sun all day).

Commencing initial programming...

After the October surgery and the very hopeful signs during the operation, it was difficult to wait until the day when we would return to Fort Worth for the DBS to be turned on. That day finally arrived this week, and we made our way south on Tuesday, Nov. 13. During our multi-hour visit, various settings were tried on each of the four contact points in the new location (STN). The most promising setting was identified, and we saw Adam being able to stand, take steps, and move his arm and hand with more control. Before we left we discussed a return to PT and OT, possible use of braces for his elbow and ankle/leg, and a follow-up appointment in January. We also were instructed in one setting change that we would be able to make at home.

When we left Fort Worth we were cautiously optimistic about the success of this new placement.

The first day after returning home, Adam felt that the benefits from the DBS were holding for the most part, with perhaps less effectiveness noticed by the evening. The second day, he felt that all of the affected muscles had tightened back up, and felt similar to how they had prior to having the device turned on.

While this was disappointing (especially in its similarity to the pattern we saw of initial benefit followed by a return to the baseline during the previous round of DBS), we are all trying to keep a very optimistic outlook and to remember that it is very early in the programming for the new location. We have been told before that the full benefit of the DBS won't likely become evident for a few weeks, and may take several months to a year to fully develop.

For now, we are working on re-starting PT and OT, doing stretches at home, and Adam is very much looking forward to the successful end of his first semester at college!