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.
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.
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.
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.
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.
This is Ethan’s Congenital Scoliosis and VEPTR Story
– told by his mommy
At 39 weeks 4 days, Ethan was born. He didn’t require anything special, no oxygen, we went to a normal room, he was doing wonderfully, just breathing faster than normal. They didn’t run any tests until a few days later, but we learned that he was missing his left 4th rib, had 4 abnormal ribs above and below the missing rib, had congenital scoliosis and his left kidney was still dilated. At discharge, they set us up with orthopedics and a geneticist, which we saw soon after we were discharged. The geneticist told us that he didn’t feel it was a syndrome, but more so from the two vessel cord and not enough blood flow to the area during development. Orthopedics evaluated him, took an x-ray and said all was well and to come back in a year. This is where something in my gut told me something wasn’t right…a whole year? We saw pulmonology when Ethan was 3 months old and he just said that Ethan may need a surgery when older for his pectus excavatum (sunken chest) but it would be cosmetic.
Again, something wasn’t sitting right with me. At this point, Ethan was holding his head up well and when I held him under his arms, I could feel that his shoulder blade was high in his neck and I couldn’t feel the bottom of it. I got on the internet and looked up doctors who specialize in chest wall deformities, San Antonio and Children’s Hospital of Philadelphia (CHOP) seemed to be leading hospitals with this expertise. I sent Ethan’s records right away so the doctors at each hospital could look over them and get back to me. We live in New Mexico, so I wanted to make sure a trip for a second opinion was warranted. Both San Antonio and Philadelphia thought Ethan would need a VEPTR (vertical expandable prosthetic titanium rib) and that we needed to schedule and evaluation. The VEPTR helps children with scoliosis to the point where it is affecting their lung function. The device can be placed rib to rib, rib to spine or rib to pelvis. It helps expand the ribcage, giving the child room to breathe, while helping maintain the curve in the spine.
At this point, my husband and I talked about where to go, we wanted the best for him, how do we choose? My husband is in the military, so we put that into consideration too, moving every few years certainly wouldn’t make it any easier. After much discussion, we decided to go to the Children’s Hospital of Philadelphia, which has the surgeon who is one of the inventors of the VEPTR and also has the most experience implanting them in young children.
We went to Philadelphia for the first time in August of 2014, Ethan was 6 months old. We met with an orthopedic surgeon, a thoracic surgeon, a pulmonologist, nutritionist, physical therapy and had x-rays, MRI’s and a CT scan. This is when we found out that Ethan has a partial fusion at T2-3 and T4-5 and a butterfly hemivertebrae at T2. We also learned that his left lung was only able to inflate to 60ml’s, where the right lung was 211ml’s. And, that his left shoulder blade had dropped into his chest cavity where the missing rib is and was attached to his lung, preventing it from expanding. Because of this, our surgeon wanted to do the surgery ASAP, as he thought it would benefit Ethan since it was already greatly involving lung function. The surgeon said that if it weren’t for the shoulder blade being in the chest cavity, we would have waited it out. We had to get weight on Ethan for the surgery, because he breathed so fast, he burned calories much faster than he should have. Surgery was scheduled for 3 months later.
In November 2014, we went back to Philadelphia for the surgery. He had a pulmonary function test the day prior to surgery for a baseline that we can compare to in the future. They placed two VEPTR’s, both on the left side. They removed the shoulder blade from the chest and repaired the lung where it was attached and put a VEPTR under his shoulder blade and also one by his left armpit. The recovery was rough until his pain was controlled and since he was so young, his lungs were collapsing. In older children you can have them walk or use an incentive spirometer, cough and deep breathe, but not with a kiddo this young. They started IVP breathing treatments every 4 hours around the clock, which forced air in and out of his lungs, hoping this would cause him to cough. He did run a pretty high fever for 2-3 days, but around day 4 we started to see our little guy coming back. He started to smile and wasn’t as medicated, just overall, doing much better. He was in the hospital for 9 days, we stayed in Philadelphia for a few days after discharge to make sure all was well before flying back home to New Mexico.
With the VEPTR, surgeries are required often, usually every 4-6 months, to expand the rods as they grow, which will continue until he is done growing in his teens. Our surgeon felt that Ethan wouldn’t need his expanded until 9 months, so we are now scheduled for his first expansion on August 11th, 2015
Ethan has done amazingly well with this surgery. He wasn’t crawling before surgery, but was close and two weeks after we got home, he was rocking on all fours, then crawling two weeks after that, something I thought he wouldn’t do. We did get him enrolled in early intervention services, so he has been getting physical therapy and occupational therapy since we got home. He is now 16 months old, is climbing stairs, cruising furniture and starting to get a little daring and letting go when standing up. He has been behind with milestones, but he learns fast and is progressing very well. We also see a few other people to help him out with his sensitivities, having surgery so young has made him a very untrusting little fella and is sensitive to certain noises. He’s doing better with that, but his central nervous system gets overwhelmed so fast, so he’s been getting help with that and he’s been doing better and better.
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 firstname.lastname@example.org