Fusion! 3-28-2012

We have had a very busy 2 weeks of follow ups to mark Sam's progress six months out of surgery.  Last week we took him for his 4th sleep study.  This one was to see how his apnea levels were without a CPAP after healing.  Half way through the night they put a CPAP on him to guage his setting for continuing to use it.  My assumption based on that was that he would still definately require one.  We followed up that study with his nuerologist in Peoria this last Tuesday.  The first good news is that Sam no longer has central sleep apnea!  This was the unusual condition that alerted the doctors that something was very wrong in the first place.  He showed levels that would be expected of any average individual.  Yeah!  What this means to me is that Sam's brainstem is now functioning correctly.

Good news number two is that Sam's obstructive apnea is now boarderline.  He still qualifies to be a CPAP user, but we have some options.  We will spend the next few months figuring out if he feels better rested and more alert when on the CPAP or if it makes no difference.  His nuerologist said he would recieve some benefit with the CPAP, but not so much that she is concerned if we no longer use it if he doesn't notice a difference.  We don't have another Nuerologist visit until next year unless we have problems.

Today we traveled into Chicago for the next follow-ups.  Sam enjoyed taking lots of pictures out the window.

Sam had a full series of spinal and brain MRI's (3 total) and one CT scan.  These started very late as the technicians were concerned that even with titanium rods that their magnets would be too strong for Sam to safely go through the particular MRI machine he was scheduled for.  They finally resolved this issue and carried out the scans as planned.  We then shuttled over to Dr. Alden's office to get the results.  His clinic was very busy and we had quite a wait there.  Finally, we got in.

Good news number three - Sam has a completed fusion from the base of the skull to his 2nd cervical plate.  Dr. Alden was very pleased with the solid bone growth that was there.  He indicated that he considers it a complete successful fusion and it would be fine if no further bone growth occured.  This was the result we were looking for.  Also, the MRI's showed no syrinxes (cysts) on the spine or brain.  This will be one of the things they will be looking for the annual MRI's in the future.  It also showed a healthy amount of spinal fluid in the spaces that no room had previously been available.  All very good news!  We will not need to visit Dr. Alden again for 6 months for a regular x-ray, and then in a year for his next MRI.  We like Dr. Alden a lot, but are happy we will not be seeing him quite so often!

After all this we went for a very late lunch to the Austrian Bakery on Clark St. (Sam's request) where he enjoyed a big plate of spatzel, his favorite!  Back at grandma and grandpa's house now and will be on the road home soon.  This feels like a bit of an ending as there is thankfully not too much more to go on to report.  We are almost down to regular annual visits and no longer expecting more changes.  I will take this time then to thank you all once again for the tremendous support you have given us.  You have supported us, prayed for us and loved us through what is easily classified as the hardest thing our family has ever been through.  Most of all, we are thanking God for His hand being on Sam and our family, for His grace and care, and for carrying us through this.  Praise God!!

January Followup 1-30-2012

Thankfully, it has been a while since I had anything to blog about.  In the 1 and 1/2 months that Sam has been out of his neck brace he has been really enjoying it and getting back to regular life.  He has been running a little and swimming at the gym, chasing his brothers around the house, joining Nerf battles, and basically feeling a bit normal again.  He is so thankful to be able to join in to activities again.  I have also found him to be pretty concerned and compassionate toward even strangers who are going through medical problems.  God has definitively given Sam a heart for people who are in difficult situations with their health.

We had a followup appointment in Chicago again today.  This was another normal followup with an x-ray and consultation with Dr. Alden.  On a bit of a side note, Sam has grown a 1/2 inch since his last appointment.  I have some reasons to believe it was beneficial to have all this done prior to his adolescent growth spurt and it looks like we were in the nick of time!  Today's appointment went well.  Dr. Alden was very pleased with the x-rays.  At December's x-ray he indicated there was a "bridge" of bone growth in between the fusion.  This means that there was a thin bridge of bone extending from one part to the next.  This x-ray seemed to show a good size column of bone.  A CT scan is still the only definitive film for making this determination, but everyone is quite pleased with what can be seen in the plain x-ray.  All of his hardware is also staying solidly in place.  I have to admit I have been a bit nervous the last month as I have watched Sam be so active and then other times slouching in terrible posture on a chair.  All I could think was that it seemed like all those things would put such terrible stress on his neck.  As usual this mother's worry has amounted to nothing and Sam is doing fantastic.  The doctor even gave him permission to jump (feet first) off the diving board, and with a great deal of caution and safety measures in place go sledding (gulp, let's just say I am praying for our non-snowy winter to continue!).

Sam's spirits are really high.  I can see the stiffness and lack of movement in his neck, but when you ask him he indicates that he doesn't feel any lack of mobility at all.  I believe he doesn't feel like he has lost much, and to me that is way more important than what is fact.  If he is frustrated with anything it is the restrictions he is still under, and much of that will eventually be gone.  Hopefully, at some point we will be down to no contact sports and no roller coasters and that will be it.

Our next steps will come in March.  He has a busy schedule.  We will have a sleep study (to see what, if anything, the surgery did to his central apnea) a Neurologist appointment, a CT scan (to check bone growth), a spine and brain MRI (to see how everything looks 6 months post-op and make sure no spinal cysts have developed), and a Neurosurgeon followup.  We already have the sleep study and Neurologist set up.  I am hoping to schedule the MRI, CT, and Neurosurgeon all in one marathon day in Chicago.  But my first step is contacting insurance.  Our referral (that permits us to see an out of network doctor) has run it's course once again and I need to reapply for more visits.  Let me say here that with all the bad flack insurance companies take, I have been more than satisfied with our experience.  It takes a lot of work to stay on top of things, but we have been treated fairly, compassionately, and competently.

Prayer requests would be as follows:

1)  A new referral would be issued with out any problems.
2)  A beautiful MRI and CT scan showing everything healing as it should.
3)  Sam's tolerance of all the appointments in March.  He really hates the sleep studies.  And he has chosen to not be sedated for his MRI's.  It will take 1-2 hours of him laying perfectly still in that loud, small banging tube. I think I would need to be sedated!  But he has some decent experience with such things at this point and feels he can do it, and that it would be better than going through sedation.  (Can I just say how proud I am of this young man!)
4)  This is a stretch, but we have a big God.  I would love for Sam to be able to get off his CPAP after the sleep study.  I am not clinging to this being the outcome, but it would be wonderful for him to have a tangible benefit to all he has gone through.

Mostly I am just praising God for where we are.  There were times that it seemed the outcome of this was going to be so much worse.  I am overjoyed to be where we are today.  Thank you, Lord!

No More Neck Brace! - 12-19-2011

For the last month we have been weaning Sam off of his neck brace by adding time out of it at 4 day intervals.  We finally made it to 8 hours a day out of the brace and today's follow up appointment.  At this appointment we had a new set of flexion/extension x-rays taken.  Let me take a moment to mention here that Sam still has significant loss in looking up, but he moved his chin within an inch or so of his chest.  I was floored.  This is way beyond anything I would have dared to hope.  We were told to expect 95% loss of movement.  We still have not tried any side to side.  Still praying that Sam will have as much mobility as possible.  But being able to look so far down will make a huge difference in his ability to walk without accident, take stairs with confidence and read comfortably. 

Dr. Alden studied these new films to be certain that with the increased freedom from the brace that none of the hardware was loosening up.  He showed us the x-rays and indicated that all of the screws were holding in place and that the plate was not shifting.  This is the result we and the doctor had hoped for.  Sam was then given permission to no longer wear his neck brace.  (He had taken it off at 8 o'clock this morning, which allowed for him to still have it off at his afternoon appointment.  He had said he had hoped he wouldn't have to put it back on at all.  I am so pleased he was right!)  We have one happy boy this evening.

The doctor was also able to show us a few other things on the x-rays that we have been waiting to see.  You may recall me saying earlier that the titanium hardware was really only the temporary fix to fuse his neck.  There was a bone graft placed during surgery and the hope is that it grows new, dense bone that solidifies the connection from his skull to his spine.  This was the first set of films to show that there appears to be some increased bone growth along the bone graft they put in between his skull and his second cervical plate (C2).  Dr. Alden said that a solid fusion in that area is the minimum we need for a successful result.  So far it appears to be beginning that process.  Furthermore, the doctor pointed out the size of the gap that is between the C2 and C3 plate.  That gap is significantly smaller than it was after surgery.  Having a complete solid bone there would make the fusion results that much better, and it appears also to be heading in that direction.  Praising God!

Along with no longer having to wear the neck brace, Sam has been released to increase some of his activities.  One of the things he was enjoying prior to his diagnosis was jogging.  He has been restricted from it for several months now.  Dr. Alden has given him permission to jog as he feels able.  He can also begin to try swimming again, although I think this will take some "re-learning" with his lack of neck movement.  He is thrilled to be able to run and try to keep up with his friends again.  Pray for this mother that I might not be a nervous wreck and ruin his good time with constant cautioning.

Next step for us is another follow-up on January 30th.  They will do another flexion/extension x-ray at that time to continue to monitor that no hardware is coming loose with increased activity.  After that we will follow up again in March with a CT scan.  This will give the doctor a more detailed and exact view of the bones and show how much healing and growth have taken place.  We also have a sleep study and neurologist appointment in March.  This sleep study will let us know if Sam is still having central apnea at night.  We are cautiously hopeful that the surgery may have helped this condition, while at the same time remembering that the purpose of the surgery was not fixing existing damage, only preventing more catastrophic damage to Sam's brain.  Please pray for God's will for all of these outcomes.

Thanks for all of your prayers on this journey.  We have come so far, and I am so thankful for everything God has done for us.  Awed and amazed, that's all I can say.

11-21-2011 Weaning off the Brace

We had a follow-up appointment today in Chicago.  Dr. Alden met with us briefly to examine how Sam's rods would react with movement.  This is the first time in 3 months that Sam has had the brace off while sitting up.  The doctor then slowly bent Sam's head as far forward as possible, while feeling through his neck to see how the hardware was holding.  The idea was to make sure that given some movement that the rods, plates and screws would all stay where they belong.  I am excited to say that his forward motion is better than I was expecting!  He then went on to tilt his head back, while still making sure the hardware felt OK.  Sam was more than happy to do this, but there was quite a bit of grimacing along with these first movements.  Sam said it felt partly painful and partly just super stiff. 

Dr. Alden was happy with what he saw and felt and sent us on for a set of flexion/extension x-rays.  In these x-rays they did the same forward/backward motion and took x-rays in those positions to be able to see that everything has stayed in place.  Sam said the second set of movement, while still really stiff, was less painful.  A short time later we were back with Dr. Alden.  He said that all of the hardware was staying in place very well.  Our very funny doctor then turned around to stare at Sam with a passive and straight face and just sat there looking at him.  Finally, he says, "you look like you want something from me...?  Oh, yeah, we can start weaning you off the brace."

Now that's a happy boy!  Love seeing that smile again!

We get to start with one hour a day for 4 days, then two hours for 4 days, and so on.  Most of you will not see him right away without the brace, other than above, as the doctor wants him to pick slower times at home as we start this process, and be sure to take it easy.  He is also not allowed to give demonstrations of how far he can move his neck quite yet.  We then need to return to Dr. Alden on December 19th.  By that point Sam will be at eight hours per day, and near the end of the weaning process.  The doctor will then take another set of flex/ext x-rays to be sure nothing has started to loosen with increased movement.  If we pass the test Sam will be set free from his brace just in time for Christmas.

I will take this time to once again thank God for the tremendous blessings He has given us.  I praise God for the birth of Jesus, for holding on to our family and drawing us close to Him, and for the work He has done, and continues to do in Sam.

Neurology follow-up 11-9-2011

Hi friends and family.  We had a last minute follow-up today with Sam's Neurologist in Peoria.  This is not the surgeon, but the doctor who found the original diagnosis, monitors his sleep apnea, and keeps an eye on how his brain is functioning.  This follow-up was scheduled for a few weeks from now, but had to be changed and we took a last minute opening rather than wait a lengthy time or see an assistant.

A few entries ago I had indicated that Sam's AHI index (how many times he stops breathing per hour overnight) was a bit out of whack.  It has lowered from the worrisome highs we had seen, but continues to be a little higher than we would like.  I have had many phone conversations with his neurologist about this and we can not come up with a good reason.  We had him wear a monitor overnight that showed he was not having any lows in his pulse or oxygen levels.  This indicates that while his CPAP is showing he is stopping breathing, he may not be.  The only way to know for sure is another sleep study.  The doctor feels that since he is not showing any other signs of distress, we will hold off on another sleep study for now.  Instead, we are going to wait a few months.  The neurosurgeon has indicated that by late March any improvements and healing from his surgery, at least neurologically, will be as good as it will get.  At that time we will then have a sleep study with out his CPAP.  This will show us if he still needs the CPAP or not.  This sleep study will take place March 20th.

It is important to note here that while we would be thrilled and thankful if Sam's sleep apnea has been solved by his surgery, that was not the goal.  The goal of everything he has been through was to keep him from having further damage to his brain.  The possibility exists that some of the symptoms, including his central apnea, could improve, but was not the reason for the surgery.  That being said, please pray that Sam's apnea has resolved and he could get rid of the CPAP.  It would be so wonderful for him to be able to see a tangible benefit to everything he has had to endure.

The neurologist asks Sam to do a variety of things that shows her how his body and brain interact.  In the beginning she saw some muscle weakness, uneven strength between the two halves of his body, and abnormal reflexes.  These were some of the signs that led her to believe he needed an MRI, leading to his diagnosis.  After retesting these things today, she said there may have been a very slight improvement, but nothing of significance.  As I said earlier, no one necessarily expected improvement to these things, but it is slightly disappointing anyway.  With this in mind I am being cautious about hoping that his apnea will have resolved, but I also know God can provide miracles.  We will be content in whatever the end result ends up being.  Other than that, the neurologist was thrilled with the success of Sam's surgery.  She has received all of the notes from our neurosurgeon and was more than satisfied with the stabilization that was achieved.

Our next appointment (and milestone) will take place November 21st in Chicago with our neurosurgeon, Dr. Alden.  At this appointment he will decide if we can start to wean Sam off of his neck brace.  I am praying that this will be possible.  Sam is VERY excited about the possibility of getting out of the brace, and I believe he would be extremely disappointed if not.  If we get the go ahead, it will take about a month to wean him off completely while he regains neck strength.  This means he may be able to be out of it completely by Christmas.  This would be an excellent gift for him.

Overall, Sam is doing fantastic.  He has no pain, is able to do all activities necessary to take care of himself, and feels good enough that I have to constantly remind him he still has to be careful and take it easy.  Thank you again for all of your prayers.  I will update you again after his Chicago appointment. 

10-10-2011 Another Follow-up, Hardware Pictures

We had another trip to Chicago for a follow up with the Neurosurgeon.  This time we spent an enjoyable weekend with my parents prior to today's appointment.  It was our first overnight trip since leaving the hospital.  I was a bit anxious about handling some things (like not having his high back chair to rest his head against, and some other items that have just made stability and daily tasks easier) but everything went very well.  Sam is at the point where we are no longer pushing him to move more, but instead trying to slow him down a little.  Aside from the neck brace, one would never guess what he went through less than 6 weeks ago.  It is a constant balance of helping him try to enjoy many of the things he always enjoyed before, and cautioning him that he still has a long time until he is considered recovered.  He shows often now the full of spunk, grinning boy we have always known him to be.

Upon arriving at Children's Memorial we had a new set of x-rays taken.  These are then transferred over to the Doctor to look at.  Dr. Alden had a brief examination of Sam and felt he was doing very well.  He always gets a pretty good laugh out of Sam (and us) by repeatedly whacking Sam's reflexes while insisting he hold still.  He said the incision site looks great.  I have to agree.  The scar will be as minimal as possible.

Dr. Alden was very pleased with the x-rays as well.  He said the rods, plates and screws looks very solid and well aligned still.  I have shared some x-ray images below.  They are both post-operative, just slightly different positions.

In the one on the left (taken today) Sam's body was just tilted enough that you can see all of the screws if you look closely.  On the right (taken Sept. 19th) the x-ray was taken in such a position as it was straight on and you can only see the closest set of screws.  I will describe what you are seeing below the picture.

This is obviously a side view of the body.  The really bright white parts are the Titanium pieces that were placed during surgery.  Starting at the top:  At the back base of the skull there is a titanium plate attached with very short thick screws, two on each side.  From that plate extends two rods.  You can see they are bent in order to follow the curve under the skull and down to the spine.  Next is a set of long screws holding the rods to the second cervical plate.  Right above the image of these screws you may (but probably not) be able to see very faint bone mass.  It will look very light in comparison to the other bone visible in the x-ray.  This is the bone that was placed that should grow dense and strong and eventually grow fused between the skull and cervical plate.  This is what they consider the desired permanent fusion.  The titanium, while it will not be removed, is only considered the short term fix.  Below that set of screws you will see another set of long screws.  These secure the rods to the third cervical plate.  These screws gave the doctor the good, solid hold on the rods that he wanted.  The area where you see the titanium placed is the length of neck that Sam will now be unable to move.  The rods, and eventually bone, will prohibit any flexibility or rotation throughout that area.  We are still very hopeful that as young as he is, Sam will learn to compensate for that loss by developing greater flexibility in other areas.  Kids are usually highly adaptable to circumstances.  We are blessed and thankful to already see it happening a little.  He already turns his entire body pretty well and doesn't seem to have too much difficulty adjusting himself to see things not in his direct line of vision.

Our next follow up will be in 6 weeks.  At that time Dr. Alden will take a similar set of x-rays.  If he is still happy with how everything looks at that point he will send us back to radiology for another set of x-rays that will take pictures of Sam's neck in various stages of flexion and extension.  The idea is to see in an x-ray that everything is staying where it should as the lower part of the spine bends back and forth.  If he is happy with these x-rays, we will be given a plan to wean Sam off of the neck collar.  Over about an additional month we will begin to take off the collar starting with an hour at a time and increasing until he has regained sufficient strength to be without the collar full time.  Sam was very excited to hear the doctor talk about taking off the collar at some point even though it was still 6 weeks away.  He was also a little disappointed and suprised (as were we) to find out it would take a month of weaning him off after that before he was rid of it for good.  But in the end we all understand why.  Major surgery and three months in a neck brace would certainly diminish the muscles he uses to hold up his head and it only makes sense that they would have to have time to strengthen.

Thanks for continuing to hold up Sam throughout his recovery.  We pray that the alignment would stay straight, and that the bone would grow strong.  Please pray as well that Sam would remain patient and not push too hard to resume activities that will not be safe for him for quite a while yet.  Please pray as well that as he has resumed a lot of normal activities that he would not fall, be bumped hard or otherwise have any accidents that would comprimise his surgery before he is fully healed.  (The expectation is that it will be about 5 more months before he has fully recovered and a year to be back to 'normal' or at least the new 'normal')

We continue to praise God for holding our family firmly in His grip.  We praise Him for all the wonderful friends and family who have sent cards, gifts, meals, the list goes on and on.  (It was actually about 2-3 weeks before we had a day that the mail did not bring at least a card, usually two or three, for Sam.  Thank you, thank you, thank you for showing so much love to my sweet boy!)  We continue to praise God as well for His sovereign plan and the work He is doing in our lives.

9/19/2011 - Birthday, 2 week followup, and a great picture!

Sam celebrated his 11th birthday this past Sunday.  While it was definitely slower paced than we are used to, it was a good day.  He received lots of Legos, saw Kungfu Panda 2 a second time, went back to church for the first time since we left for Chicago, and had Speatzel (his favorite -a German noodle/dumpling) for dinner.  We of course were very thankful indeed to be celebrating Sam's birthday with him on the mend.

Today we traveled back up to Chicago for Sam's 2 week follow-up appointment.  First stop was x-rays, then on to see the doctor.  Dr. Alden thought Sam's surgical site was healing beautifully.  He removed the stitches today, which Sam did not particularly enjoy, but I am sure he is glad they are gone now that it is done.  So far there has been no evidence if any leakage of spinal fluid, or infection.  Praise God.  We then went to another room to look at the x-rays.  They are looking great.  The x-rays are taken from the side and everything appears to have the correct alignment still.  I wasn't sure after the surgery how Sam would feel about Dr. Alden at this point, but it looks like Sam and the Doctor will be able to stay friends.

I have been trying to figure out how to share this picture.  Dr. Alden had the idea that I could just take a picture of his side by side view, so I hope this will come across OK.  This is the shot that is priceless to me.

The first picture is the before, and the one next to it is post surgery.  The red circle shows the top of the spine that is poking in to the brain stem  Notice how much straighter it is in the second picture.  Also you can tell that the indent into the brain stem is no longer present.

The black circle shows where the Chiari malformation is.  In the first picture you can see, if you look closely, how the brain tonsils (bottom material of the brain) descends down and is pressed between the spinal cord and the cervical plates.  In the second picture the tonsils have risen back up and is no longer compressed or pressing against the spinal cord.

The blue circle is around a small triangular area that Dr. Alden used to show us some distance markers. I know it has more significance than what I can explain, but this is as far as my understanding could go.  In the first picture the top of the spine presses right up against the triangular space.  The second picture shows that the triangular area is now a full centimeter away from the spine.

 Here is the side by side without marks so you can see it a little clearer.

On a funny side note, the medical records show that in about a 3 week period from before to after surgery, Sam gained just over a 1/2 inch of height.  They definitely accomplished stretching things out!

Last, I am happy to share that we have no explanation, but Sam's apnea numbers that have not been good since arriving home, have continued to lower slowly.  The last two nights have been low enough that they are considered to be within acceptable limits.  We don't know why they went up, and we don't know why they are going down, but I am choosing to simply thank God that He has continued to keep His mighty hand on us.  Sam's spirits are continuing to improve as well.  He is choosing to pursue interests a little more readily, and is starting to look forward to getting re-involved in regular activities at home and with friends.  Thank you again for your prayers on these issues.  At this point we will continue to pray for good solid bone growth and that in the meantime his alignment stays looking good.  We return to Chicago for his next followup in three weeks.  I will look forward to updating you on his progress at that time, unless anything comes up to share beforehand.  Thanks again everyone.