March 25, 2014 . .. . Update Part Two -- Hope's Back Surgery for Severe Scoliosis

And now for an update on Hope's scoliosis and the back surgery that is recommended to correct it.  Hope's condition is very severe and complex.  I'll do my best to try to explain the details around it so you can know best how to be in prayer for her and for us as we make decisions. . .

Prior to Hope's brain injury, she had a perfectly straight back.  So, the scoliosis that developed about 2 months after her brain injury (right after the baclofen pump was placed) is most likely related to two things.  The first is dystonia which is a medical term used to describe a very wide variety of abnormal muscle tone and movements.  Muscles can be too contracted or too flaccid.  For example, Hope's arms were flexed so tight in towards her chest that she would leave marks on her sternum and so we used splints and teddy bears to protect her.  Another example would be when Hope tries to walk, she tends to walk on her tippy toes which is also a result of dystonia (or muscles not doing what they're supposed to).  One more example would be when Hope was sitting in her wheelchair, her legs used to kick straight forward when she would try to do anything (a form of involuntary movement that she was not able to control).  Her dystonia was severe, complex and involved pretty much every muscle in her body after her brain injury.  Hope has seen huge improvements in her dystonia over the past two years of her recovery, but it is still evident in many areas, one of the worst being her back scoliosis.

Hope's dystonia is a result of her severe brain injury and I talked about the severity of her dystonia in the last post/update that resulted in her receiving the baclofen pump to treat it.  The baclofen pump treated the severe dystonic spasms that were life-threatening for Hope, however, it also probably caused Hope's trunk/back muscles to be weaker and thus less able to hold the correct shape, as well.  So it also most likely played a small part in the severity of Hope's scoliosis. . . in fact, her scoliosis showed up the day after the baclofen surgery on x-ray.  However, Hope's scoliosis is called a neuromuscular scoliosis and is primarily related to her brain injury.

According the orthopedic back surgeon, this brain injury and possibly baclofen pump related back scoliosis will NEVER get better.  There is no treatment and there is no hope for it to heal on it's own, EVEN when the baclofen pump is removed.  When we asked him how exactly brain injury causes the scoliosis, he told us that they are not exactly sure how it occurs and why it can look so different in various people.  Trevor has done a lot of research on different kinds of scoliosis and severity and treatments. . . it was not encouraging.  One of the few treatments that have actually shown some improvement in idiopathic scoliosis is exercise based treatment programs that also usually involve bracing.  However, I called a scoliosis smart care clinic in the USA, and Hope is not a candidate for this treatment because she cannot stand by herself for a minimum of 15 mins.  Also, idiopathic scoliosis has a very different etiology than Hope's brain injury related scoliosis.  Little is known about how brain injury causes scoliosis. . . just that it does, and it is untreatable and irreversible.

That is the first piece of discouraging news.  The second is the severity of Hope's scoliosis.  In Trevor's research, people who were trying exercise based treatments had scoliosis curves in the 40's or 50's.  He found 3 severe cases, where surgery was done as the only option, and these people had scoliosis curves of 120, 125 and 130 degrees.  These were the most severe cases he found.  Hope's main curve in the middle of her back is 126 degrees. . . to put it bluntly, it is brutal.  You can see the scoliosis in her back easily. . . she has a huge hump on the right side where the back almost folds in half!  But this is not the only problem area.  Hope also has a compensatory curve above the bad one that is 48 degrees where her body has tried to keep her head upright.  And then to make matters worse, Hope also has a severe pelvic tilt.  This tilt is most significantly left/right (down on the right and up on the left making her left leg shorter than the right), but it is also forwards/backwards (pushing the left ribcage forwards and sinking it down into the left hip).  In considering surgery, whenever the pelvic tilt is 15 degrees or greater, they immobilize the pelvis also . . . Hope has a 30 degree tilt!  Another way to describe this is that when Hope sits, she only sits on her right bum cheek due to the severe pelvic tilt.

So we last saw the orthopedic surgeon last August, when he put Hope on the 10 month waiting list for corrective back surgery.  He said there was no urgency as her curve was quite flexible and able to be bent out fairly straight when he placed her on her side over his knees, with no discomfort to Hope.  He said that being on the list meant if she suddenly started having problems and needed the surgery, we would have already started the process, and if it could be postponed, then we would do that.  So there was no urgency.  However, he did emphasize again that it would never get better on it's own and there was nothing that could be done to treat it.  Back surgery was inevitable, we would just watch her and determine the best timing for it.

Hope started having rib pain about one month later.  We had a wheelchair reassessment done and they made some major changes which helped for about 3 weeks.  Then the pain came back and has slowly progressed until present.  Currently she has daily pain in her right rib cage.  It is worse when she is in her wheelchair or siting upright.  Although we have been told that the baclofen pump should not be causing rib pain, we really believe (along with Hope!) that the pain is from her right rib rubbing or dragging on the pump.  Most people complain of pain on the side where the ribs sink down into the hip, and although Hope has a severe case of this, she says that her left side does not hurt.  Her ability to describe the location and pain is a huge help!

Hope had an MRI of her spine in November which we have not discussed the results at present with the surgeon.  We received a phone call in January letting us know that they had scheduled Hope for her back surgery on April 28th!  We were quite surprised to hear this as it seemed quite a bit earlier than we expected and we hadn't even seen the surgeon again to have Hope reassessed.  So we asked for an appointment sooner than the preop appointment in April and saw the doctor on Mar 15th to discuss it further. 

We went into this appointment with the orthopedic surgeon with hope that he would still feel Hope was plenty flexible and with the baclofen pump coming out, which would likely address and resolve Hope's right rib pain, would be in agreement to postpone the back surgery scheduled at the end of April.  Hope had another series of x-rays that morning prior to the appointment.  However, the surgeon instead was quite insistent that Hope needed to have this surgery. . . the sooner the better.  We asked A LOT of questions to try to understand the surgery, the result of the surgery and his new urgency to do the surgery.  To be very honest, there was very little information that sounded encouraging. . . rather we were left discouraged, unsure, and heart-broken that this could be Hope's only option.  Hope herself was fighting back tears, lip and chin quivering, until the tears started to pour when she heard that she would not grow anymore, as a result of the surgery.  I will try to summarize the information for you.

First to describe the surgery a bit more for you. . . Hope will have her back fused all the way from her neck down to her pelvis.  This means that they manipulate the spine (the nurse described it as "mashing things up") to cause each vertebrae to touch (or bone on bone) which then causes the bones to heal and fuse together.  Parts of the vertebrae are cut off.  In order for the bones to have time to heal and fuse, they will also insert long rods, the length of the spine, that are screwed into the vertebrae.  In addition to fusing the spine, they will also fuse Hope's spine to her pelvis and immobilize the pelvis to try to correct the severe pelvic tilt that I described to you.  Additional rods and hardware will be screwed onto the pelvis and attached to the spine to do this.  All of the rods and hardware will stay in as another surgery to remove them is more risk than benefit.  They use traction to get the spine as straight as possible during the surgery. . . this will mean screws being inserted into both sides of each of Hope's knees to use to attach the traction to.  The surgery will take from 6-10 hours.  Significant complications are damage to nerves resulting in paralysis, infection, blood loss, and post op seizures.  This surgery is permanent and cannot ever be reversed.

Other post op information we were given was that Hope would be in the hospital for most likely one week following surgery, then at home for two weeks, and then a modified return to school for another 3 weeks.  However, we were also warned that Hope's ability to bear weight and walk would get worse before it would get better (likely for 6-12 months) as she would need to relearn how to do it with a straighter spine.  And as I shared earlier, Hope's spine will never grow again due to being fused.  Her legs and arms will still grow, which can result in some issues, but we would have to wait and see.

Finally the surgeon was eager to do the surgery sooner than later because of the nature of Hope's scoliosis.  Again, he emphasized to us that there is no treatment other than surgery and it would never get better on it's own, even with the removal of the baclofen pump.  Her scoliosis will become more severe as her spine tries to grow and simply feeds the severe curve.  Also her scoliosis curves in her back would become more stiff and unable to be straightened as time goes on.  So no gains would be made by waiting, only risks of a less straight back as a result.  Also a more stiff spine means that the surgery will be significantly longer and thus have potential for more complications.  The surgeon felt that her scoliosis was progressing and waiting could mean a less effective result after surgery.

There was some encouraging news. . . the surgeon said that he does not usually do this surgery on kids that can talk (helps in many ways such as assessing pain control), are as healthy as Hope, and that are continent or in control of bowel and bladder like Hope is (which significantly decreases risk of infection).  He also was encouraged and almost surprised to see how flexible Hope's spine is and felt that he could get her pretty close to straight with the surgery.

Okay this post is becoming quite long and so full of information. . . sorry!  I'll finish off here with our thoughts and concerns regarding all this information, and then the final outcome.  This decision is a really difficult one to make for Trevor and I, and Hope too!  We left the surgeon's office that day with our heads swimming, our hearts aching and seeking discernment and peace from the Lord in this decision.  Hope was still on the schedule to have surgery on April 28th and we would go in for pre op work up and teaching on April 15th.  We would so love for Hope to be straight enough that she could have the potential to walk again and this back surgery seems like the answer to that.  The surgery also means that Hope will not become stiff in such a deformed position which would be quite debilitating and painful over time.  We understand that the surgery is truly "necessary" by all medical standards.  We are also desiring to be submitted to this option as the Lord provides leading and makes His plan for Hope's life known.  However, we have some concerns about the timing of this surgery being so soon, and in conjunction with or so close to the baclofen pump removal surgery.

I'll quickly go through some of our concerns as to the timing of Hope's surgery.  If Hope is able to grow more and still remain flexible as she is now, then she has gained more height and opportunity to be more proportionate.  Another concern would be the amount of flexibility that Hope would lose. . . Hope would be completely unable to bend forwards/backwards or left/right throughout her entire torso and pelvis.  This means only her neck would bend and her legs/arms.  On top of her gross motor movement difficulties due to her brain injury, this will affect her ability to do many of the things that she now can do, such as leaning to the right to pull up the foot of her recliner chair. . . something she LOVES to do all by herself EVERY time!  That stiffness from being fused would also impact our ability to move and lift her as her flexibility has really helped us in many situations.  It would affect her ability to sit herself up as she bends herself forwards and uses a rocking motion to sit up. 

Another concern with the timing is whether Hope's scoliosis is indeed worsening or not.  The x-rays taken in August last year were taken with me holding Hope's hands above her head as she was not able to sit on the chair by herself.  However the ones just taken this month were with her now sitting by herself (because she can now!).  This difference makes the x-rays incomparable, and so not really a good indication that her scoliosis is indeed worsening.  This was the first time that we got a measurement of her curve.  Her flexibility appears to be the same as last August in her being able to have her curve straightened over his knees without pain.

And the final and probably most significant concern is that we all (Hope too!) felt a huge peace with having the baclofen pump removed FIRST!  Having Hope be completely healed with no risk of CSF leak or serious infection in the CSF feels to be the best decision.  It also means dealing with Hope's daily rib pain as soon as possible rather than waiting to try to work around the back surgery.  So, when the hospital called with the surgery date for the baclofen pump as April 15th which now conflicted with Hope's pre-op appointment for the back surgery, as well as being only 13 days away from a major surgery, we all felt a peace that we needed to postpone the back surgery.  So, I called the ortho nurse this past week and discussed the pump removal surgery date and our concerns with it being so close to the back surgery date with her.  I'm SO thankful that the Lord makes His will known clearly. . . the nurse agreed that this was the best decision and even went on to say that postponing it for a few months would not be a problem or concern at all!  It affirmed our hearts that the Lord was indeed giving us His leading to postpone the back surgery for now.  The nurse will call us in the next month or so when she receives the summer schedule around the surgeon's vacation and reassign us a new surgery date.

So. . . how can you be praying after this very lengthy update. . .

First and foremost, would you please continue to join us in praying for a miracle!  We know that our God is able to heal beyond anything medically possible or understood, and He has done that in Hope's recovery already.  We have been able to avoid every surgery that had permanent results up to this point so far, and that has truly been a miracle!  We are so thankful and we would love to see this continue.  We are going to be praying for this diligently as a family over the months ahead.  However, the flip side of this request is that you would also join us in praying that we would be joyfully submitted to whatever His good and perfect will and plan are for Hope's life, and if that includes having this very scary and permanent back surgery, that we would have a peace that passes understanding in giving the final consent to go ahead.  We desire our lives, and our precious Hope's life, to glorify God in the plan and purposes that He desires, even in the things we would not choose or do not understand.  We want to trust Him explicitly with one of the most precious gifts in our life. . . our Hope and her future!

Secondly please pray for us as we continue to research and seek some other treatment options for strengthening her torso muscle strength and perhaps her ability to hold her back more straight giving us a better assessment of her scoliosis vs dystonia.  Improvement could mean that Hope would require less fusing of her spine or pelvis.  Again, we realize this would be a miracle!

Finally please pray for wisdom for us, as well as the surgeon, as to the timing of the back surgery.  We refer to it kinda like Goldilocks and the Three bears'. . . not too soon, not too late, but JUST RIGHT!  Only God knows that just right timing, and so we desire to trust His leading in that, and the direction that He will give both us and the doctors!

Thank you for sticking with me for such an incredibly long and detailed update.  I hope and pray that this will further equip you as you pray for Hope, her healing, our family, and the doctors caring for her.  We are so thankful for the doctors who are very knowledgeable and skilled, and the kind nurses as well!  We are so very thankful for the amazing people who continue to care so deeply for us and pray for our girlie and our family!  And most of all, we are SO very thankful for our mighty God who is over it all and working out His good and perfect plan and will in our lives and in the recovery and future of our sweet Hope!

You are loved, dear ones!!!