The treatment plans and interventions for congenital scoliosis can vary greatly between patients.  Some will be lucky and only need to be monitored throughout their life, others will require complex surgical intervention at early ages, some will endure numerous surgeries throughout their childhood and some will only require one or two. Because  each spine and each patient is truly unique, we have decided to share the stories of others here.

Carson’s Scoliosis Journey –The Face of Resiliency!
-told by his mom

My husband and I noticed Carson’s spine appeared to be curved when he was just 5 months old.  I brought up our concerns to his pediatrician at his 6 month appointment.  She dismissed our concerns, saying babies are just flexible and they don’t have very strong muscles to hold themselves properly.  When Carson was 10 months, he was having testing done for another medical condition.  During that test my husband asked the radiologist to shoot a film of his spine because we were so certain he had scoliosis….and there it was…a very obvious “s” shaped curve, 60 degrees thoracic and 45 degree lumber.  With that we started our research.

We started with a local orthopedic doc where we lived.  We knew that this wouldn’t be the doc to manage him long term, but we wanted to get things started.  He did an x-ray and told us Carson had a congenital defect, a hemivertebrae between L4-L5.  A couple weeks later Carson had an MRI and CT done under general anesthesia.  We looked for docs that were treating a lot of infants with congenital scoliosis.  We found that many claimed to specialize in pediatric scoliosis but we wanted a doc that was treating infants and toddlers, not just teenagers.

We found a doctor at St Louis Children’s and contacted his office.  After sending all of Carson’s images to him, he reviewed his case and agreed to take Carson as a patient.  We saw that doctor for the first time in December 2012 when Carson was 16 months old.  The orthopedic doctor we had in our hometown had said that this hemivertebrae would need to be addressed surgically so we were prepared to hear that from this doctor as well.  St. Louis did agree that Carson would need surgery, but felt he was stable enough to let him get a little bigger and stronger before we operated.

post-op x-ray

He was put into a brace that he was to wear 6-8 hours a day while awake.  He adjusted well to his brace.  I expected that he wouldn’t walk very well for awhile since he had just started walking 2 months prior to getting the brace.  It was a rough week of adjusting for him but he did very well.  His biggest obstacle was learning how to get up to a standing position.  For the first couple days he would lay on the ground and cry because he couldn’t bend his torso to stand himself up.

He wore that brace until September 24, 2013, his surgery day, just 1 month after turning 2.  The plan was to remove the hemivertebrae and fuse L4 and L5.  The total OR time was to be about 3 hours.  Everything went smoothly with his surgery.  His curves decrease to 48 degrees thoracic and 38 degrees lumbar.  We saw him in PACU at about noon that day.  He was screaming and in a lot of pain.  The next 48 hours where the worst days I have ever experienced.

I knew this was a painful surgery, but I really didn’t expect it to be quite this hard on him.  He was molded for a brace while in the OR, but that wouldn’t be ready until the next day.  Pain management had a very hard time controlling his pain.  For 48 hours he was kept sedated on pain meds for most of the time.  During the times his meds would wear off or they would try to wean him off, he would cry and thrash so much that both of his wound drains were pulled out, he lost 1 of 2 iv’s, and his foley catheter was dislodged.

1 day post op brace fitting

Finally after a very long discussion with pain management and 2 extra nights in the ICU, Carson’s pain was under control and he was on oral pain medications only.  He was eating, drinking, and most importably, SMILING!  After 3 nights in the PICU and 1 night on the ortho surgical floor, Carson was discharged.  He was in a brace that went over his shoulders, down to his knees.  This brace was made to keep him from walking, running, rotating, everything.  He was not to be weight bearing or walking until our first post-op appointment 11 weeks away.

We got a special carseat from the hospital with a wedge that kept him straight since he couldn’t bend at the hips.  We got a wheelchair that reclined flat for moving him around that house and taking him for much needed walks outside.  We used a crib mattress and lots of pillows to position him high enough to eat.  After 2 weeks, he was completely off pain meds and doing so extremely well.  Five weeks post-op he was starting to roll himself off the couch and into a standing position.  I called the doctor and he said he was fine to do that.  Days later he had figured out how to propel himself around the house walking, even though he couldn’t bend at the hips.  This kid was unstoppable!  He never whined or complained despite only leaving the house 1 time in that 11 weeks.
I get it now…kids are so resilient and strong!

At our first post-op appointment, Carson got one of the leg cuffs cut off his brace.  He also was allowed to have the brace off to sit in the carseat and in a high back high chair for meals.  For 11 more weeks he would have the one leg cuff on his brace.  During this post-op time my husband and I started to do more research on what our next step should be.  We ran across Mehta casting before his surgery, but always read that congenital cases weren’t being casted.  Now that his hemivertebrae was removed, we wondered if he would be a candidate for casting since his large thoracic curve had to be addressed.  Our surgeon in St Louis recommended more bracing until old enough for growing rods.  We brought up casting to him at that first post-op appointment and he was onboard with our plan to get other opinions, specifically from those who are Mehta trained, since he doesn’t cast.  We had a trip planned to visit family in Chicago in January 2014.  We rearranged our flights and days and made appointments with Chicago Shriners and Cincinnati Childrens.  We also made an appointment for a month later with our surgeons partner in St Louis.

Our first appointment was with Chicago Shriners.  We were hit with some very hard news that Carson most likely had another congenital vertebrae defect, a bar from T10-T12.  We were told that his disc spaces in his thoracic spine weren’t normal, but never that he had another boney abnormality.  Chicago said that he was not a candidate for casting.

We drove to Cincinnati a week later.  The doctor agreed that Carson did most likely have a bar.  He said casting on congenital issues was not often done, but it could delay the progression of his curve, buying us very valuable growth time.  We had a feeling Cincinnati would be the place for us, even before meeting them and after that appointment we were sold.  My husband and I were so extremely happy with them.  In fact, the nurse had even given us a casting day even before our appointment and said we could cancel if we needed but this way he was on the schedule and could start soon.  So we ended up canceling our 3rd casting opinion in St Louis, went home and started booking flights for just a month later to get Carson first cast.

Carson just had cast 7 placed in early October.  Since he is now 4 years old, his rate of growth has slowed.  He now will be wearing his casts for 16 weeks, with a one week break in between.  This means he will get 2 casts a year and then go into a summer brace for 4 months.  We opt to do breaks in the summer since casting is not a “cure” for him.

Casting has successfully held Carson’s thoracic curve at about 53 degrees.  That’s a huge success in our minds.  Our doctor believes that if we would have just braced him all this time that his curve would have progressed, and we also agree.  Our future plan is to take it one step at a time.  We will cast for as long as the benefits out way the risks.

comparative x-rays, before surgery, in the OR and 11 weeks post-op

At about 60 degrees our Cincinnati doctor would like to put in magnetic growing rods (MAGEC rods).  Our surgeon in St Louis felt that we could let him get closer to 100 degrees as long as his spine remains flexible.  Both have very good points as to why they would operate when they would.  So when it does come time for magnetic rods we will get several opinions.  Our hope is that Carson would be at least 6 years old before magnetic rods.  They feel that at age 6 he will be big enough to get by with only having one set of rods as opposed to outgrowing one set and needing longer growing rods inserted later.  After magnetic rods, he will get a permanent fusion.  There is a small chance that if his curve holds at 50-ish degrees through all of his growth that he wont need surgery at all.

Carson is everything a little 4 year old boy should be and we are so grateful for his strong, resilient personality!

 

---

This is The 15 year “Wait and See” Scoliosis Journey of Micheal
-told by his mother, Veronica

Michael was born a few weeks early in January of 1998 with birth defects associated with VATER Association now known as VACTERL Association. (VACTERL association is a disorder that affects many body systems. VACTERL stands for vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities. People diagnosed with VACTERL association typically have at least three of these characteristic features.) His doctors completed an x-ray of his spine to rule out the “V” in this condition and found multiple vertebrae anomalies. It’s quite possible that his congenital scoliosis would have gone unnoticed for a while if they hadn’t completed the x-ray because it wasn’t obvious looking at him that there were issues with his spine. The x-ray revealed a hemi-vertebrae on the left at T3 and T7; butterfly vertebra at T10 and a final left unsegmented hemi-vertebra at S1. He did not have an obvious scoliosis curve and was considered overall compensated and well balanced. Michael was placed temporarily into foster care after birth into a wonderful medically fragile foster home. When he became available for adoption at about one year of age I started the process of adoption. I saw a picture of his gorgeous face and knew that I wanted to be his mom.

Michael was placed with me for adoption at the age of 16 months old. I was more concerned about some other medical needs at that time and luckily his back was considered stable. He would have x-rays completed every year and we had to change doctors a few time because of insurance changes. He had surgery at 2 ½ years for a tethered spinal cord, which is not uncommon for children with spine issues. The first scoliosis curve degree I was given was at age 6. The curves were reported to be 35* and 22* with the second curve being a compensating curve. It varied for the next few years going up and down. He never had any physical restriction placed on him. He remained in the “wait and see” stage since a brace was not an option for his congenital scoliosis.

First picture I saw of him

Adoption day photo age 2

At age 10 Michael had to go to another new orthopedic specialist. His new surgeon had a very calm demeanor and really helped to put Michael’s anxiety at ease. He started having x-rays twice a year at age 11. By age 12 Michael had some symptoms of tethered spinal cord again and had a repeat surgery at age 12 ½. He had his first CT scan of his spine completed and it gave a little more details to his vertebral anomalies. It was very cool seeing his spine in 3D. Michael always had a great sense of humor through his scoliosis journey. He could not wait until 7th grade to do the scoliosis test in PE class. He of course, did not tell the visiting nurses that he already had a diagnosis because he wanted to see their faces when he bent over. He finally told them after they got the 3rd nurse go come over and look at him.
When we asked his surgeon what the “magic curve number” was before considering surgery we were told 50*. Michael reached the number at age 14 years. He then decided to wait a little longer to give him more time to grow. The doctor stated fixing a 60* curve was not much different than fixing a 50* curve so that’s what we decided to do. He started having x-rays done every 3-4 months at this time.

In January of 2013 Michael’s curve increased to 63* and 53” and it was decided it was time to consider surgery. He repeated MRI’s to make sure there were no changes to his spinal cord. He was scheduled for surgery in April but due to some issues with lab work at the very last minute it was continued to May of 2013. His surgeon did a great job of explaining the surgery in detail. It was very hard to hear that the plan was not to “straighten” his spine, but to stop the progression from going any further. He explained that attempting to straighten his spine completely could compromise his spinal cord and was too risky. The thought of going through such a big surgery without the benefit of looking better afterwards was pretty devastating for Michael to hear. The surgeon decided to not fuse the bottom compensating curve to leave him with more flexibility and the hope that it would compensate back. Michael had a pic line put in the day before surgery as an outpatient procedure.

His surgery was supposed to start at 7am the next morning, but was delayed until about 9am. It was a stressful morning for Michael. It was stressful once they started the surgery because we did not receive any updates until the surgery was done at about 7pm. It then took another hour and a half to close him up. The doctor ended up completing a posterior spine arthrodesis and fusing from T1 to L2. He said he was a little nervous halfway through the surgery because he was so small and thin but it all worked out when it was time to close him up. I was not able to actually see him in recovery until about 10pm. The doctor had prepared me for his face to be swollen but I don’t think anything could have actually prepared me for what he was going to look like. It almost looked like someone else’s child. When they are on their stomachs for so many hours it’s natural for that to happen. It took a few days for the swelling to go down.

The hospital course was quite stressful. Michael was resistant to walking and the various tasks that were required to be discharged but the nurses and physical therapists were persistent and got him through. His surgery was on a Tuesday and he was released on Sunday afternoon. The hospital is about 20 minutes from our home but it seemed like hours. Every bump was painful for him. We had his bed setup in the family room so he didn’t have to go upstairs initially. He stayed downstairs for probably a week or two longer than needed, but that’s alright. His physical therapist came to the home for the first month, which was quite helpful. He hated car rides initially.

Michael had his first post x-ray at about 6 weeks after surgery. It was amazing to see the difference. He actually gained 2 inches in height when he came home from surgery. This x-ray was the first time we could see his 2 rods and 20 screws. Even though the plan wasn’t to straighten his spine, his back looked great. The compensating curve did not compensate back as hoped so he has a 38* curve on his hip that has stayed consistent.

Michael was cleared to ride roller coasters for his 16th birthday trip to Disneyland in January of 2014. He was nervous but he did it. He’s now 2 years post op with very minimal restrictions. He still has the compensating curve that has not changed since surgery. He’s now 5’5” tall! He was just above 5’ tall after surgery 2 years ago. He loves to play basketball and is working hard on being able to dunk. He’s also a fan of riding skateboards and pro-scooters. We decided to do online school the following school year after surgery and that has worked out very well for him. He’s made the honor roll and is on schedule to graduate on time in 2016. He’s still being monitored by his surgeon for the bottom curve because he’s still growing. He expects to follow him into adulthood.

Michael has had a total of 9 major surgeries and many day surgeries/procedures; however spinal fusion was definitely the most intense of all of them. I’m glad we are on the other side of the “wait and see” journey. I’m glad that we listened to the doctor’s recommendation and waited the extra year to have the surgery and gave his spine more time to grow. I no longer stare at his back worrying if it looks worse or not. My “x-ray eyes” were never very accurate. When I thought it looked bad it was fine and when I thought he was looking good was when he had progression. It’s a relief to worry about regular teen stuff now like studying for driver’s permit, prom and school work. Time really does heal and patience with the process really did payoff.

Prom 2015  and working on his dunk – May 2015

This is Ethan’s Congenital Scoliosis and VEPTR Story
– told by his mommy

At 20 weeks gestation, we learned that I had a single umbilical artery and also that Ethan’s left kidney measured a little larger than normal. We met with a perinatologist and a genetic counselor and both thought all was well with Ethan despite the new findings. Around 26 weeks, I started having contractions and was put on bed rest. The medications and shots didn’t stop the contractions, so I had two rounds of steroid shots to help develop his lungs in case he came early.  I think that because I was being monitored so closely since the contractions started, that’s the only reason they found “issues” with his spine, which was around 28 weeks. At this time, they told me that the kidney resolved and was now normal in size and the ultrasound tech talked with the perinatologist about the spine and he wasn’t concerned. Two weeks later when they saw it again, they ordered a fetal MRI, which showed nothing. Around 33 weeks I saw the perinatologist to discuss MRI results and he took a really good look at the spine and said that he suspected hemivertebrae and missing ribs, but we would just have to wait it out until Ethan was born, but assured me that this wasn’t something to be concerned about….I was very concerned.

 

 

I’ve learned many things throughout this journey. Always trust your instincts and be the best advocate for your child that you can be. If something doesn’t feel right, get another opinion. I used Facebook to search for groups, which is where I found the Congenital Scoliosis page and also the VEPTR Kids page. I talked to many people asking about the VEPTR trying to get an idea of what to expect because no one could relate more than other mothers with a child who has a VEPTR. We take it day by day, do what we need to do to keep Ethan happy and that’s a good day in my book.  He is a happy little boy for all he’s been through and I will do my best to continue to keep it that way.

Also, feel free to contact me regarding the VEPTR or questions at brinner456@gmail.com