Your child has just been diagnosed with the Early Onset Scoliosis. Any time you receive a rare disease diagnosis it can be confusing just trying to understand it and not knowing where to start. Chances are your pediatrician isn’t that familiar with the disease much less the latest treatments. It’s important to understand what Early Onset Scoliosis means and how the condition and treatment for it is vastly different than Adolescent Scoliosis. Early Onset Scoliosis can encompass Infantile Scoliosis (occurring from birth to age 2), Congenital Scoliosis (a structural abnormality in the spine that is present at birth but can go awhile before being detected), Syndromatic Scoliosis (scoliosis brought on by an underlying condition) and scoliosis that sets in between the ages of 2 yrs old and 8 yrs old.
This distinction is incredibly important. Here’s why: The majority of cases of ‘scoliosis’ are adolescent scoliosis. In adolescent scoliosis treatment happens on a very mature spine that is either completely finished growing or very close to being complete. Early Onset Scoliosis treatment happens on a very immature spine that has a lot of growth ahead. That major growth that still needs to occur provides a lot more challenges and changes every aspect of how the disease should be treated. A rapidly growing spine that already has a curve naturally has the ability for that curve to grow larger and faster.
Early Onset Scoliosis is rare. Congenital Scoliosis is even more rare. There are orthopedic pediatric surgeons all over the United States that specialize in ‘scoliosis.’ However, the number of pediatric orthopedic surgeons who have extensive experience in Early Onset Scoliosis and Congenital Scoliosis is much smaller. Let me repeat that because it’s an important statement. The number of pediatric orthopedic surgeons who have extensive experience in Early Onset Scoliosis and Congenital Scoliosis is much smaller.
Over the last couple of years, I’ve connected with more parents than I can count who have a child who has been diagnosed with some type of Early Onset Scoliosis. Unfortunately, some of them have not been seeing the right surgeon because it’s not something that is clear for parents to understand. You can love your doctor’s personality and bedside manner. He or she can have great experience in treating a much older and mature spine but that doesn’t necessarily make him or her the right fit for your child’s EOS diagnosis and treatment. There are many well meaning surgeons out there who treat adolescent scoliosis and then one day the rare Early Onset Scoliosis patient is carried in their door and they are probably intrigued and excited about the opportunity to have a rare case. But if they aren’t up-to-date on current treatments, training, and more or less have extensive experience treating this complex disease of young children, then frankly they are not the best choice.
Early on in our daughter’s diagnosis we sought out a second opinion. We saw the Chief of Pediatric Orthopedic Surgery at a top five ranked Children’s Hospital in the United States. He was the top guy in the department and his bio said specialty with scoliosis and spinal deformities. But it was clear after meeting with him and after doing a lot of research on congenital early onset scoliosis that he was absolutely dead wrong in what his treatment plan was. He didn’t have expertise in early onset and frankly he should have said so and referred us to the proper hospital just a few miles away (a hospital I didn’t even know anything about at the time). He was a ‘nice’ doctor and I’m sure great in other areas of orthopedics that he specialized in but what if we had only seen him and followed his treatment plan?
So how’s a parent to know? Here are some key questions to ask to make sure you are seeing the right type of surgeon: (Note these are not questions that are specific to understanding your child’s diagnosis)
1. Is your background in treating adolescent scoliosis or Early Onset Scoliosis?
2. How many patients have you treated with Early Onset and/or Congenital Scoliosis or how long have you been focusing on the EOS group?
3. What organizations are you involved with that are dedicated to advancement in research, training, and education of EOS? (Be sure to and look up the ones mentioned)
4. How many patients have you treated with EOS? How many do you treat in a year?
5. Do you have training in Mehta/EDF casting? If not, do you back the philosophy/ treatment of Mehta/EDF casting for EOS idiopathic and if appropriate for Congenital EOS, and do you refer patients to surgeons who do have extensive experience in Mehta/EDF casting?
6. Do you support the idea of your EOS patients getting a second opinion? Are there specific surgeons who you recommend for second opinions?
If your surgeon would like to cast or brace: What is your goal for casting/bracing? To cure, or provide some amount of correction through growth or to simply hold the curve to buy growth time?
Nora is in her eighth cast right now and over the past two years I have learned a lot about Mehta/EDF casts. While Nora’s casting goals are different than those with idiopathic EOS I’ve learned a lot about casting treatment for both. I’m not an expert by any means but I have learned enough to know I can go toe-to-toe with the best of the experts. That being said, myself and many other parents who have been deeply involved in the Mehta/EDF casting life, have seen some really bad casts out there. I’ve seen pictures of babies with large permanent scarring on their bodies from poorly applied casts that dug into the children’s skin. I’ve seen casts that had tiny tummy holes that caused the child to throw up several times a day for days on end. I’ve seen casts that were so loose and ill fitting there was no way they could have been effective. I’ve seen casts that incorporate a lot of these things and have caused so much pain for a child that the surgeon’s answer was to prescribe narcotic painkillers to an infant. A properly applied cast should not cause discomfort much less pain. I’ve seen casts that just don’t make any sense at all. Casting is just as much a science as it is an art and there is a learning curve to it.
So if your surgeon is suggesting casting as a first line of treatment how do you know your surgeon is going to apply a proper cast? Here are some important questions to ask:
1. Have you been trained in Mehta/EDF casting? Where did you receive this training?
2. How long have you been casting?
3. What materials do you use to apply a cast? (Typically they are made of plaster and often have a fiberglass layer on top. If your surgeon just uses fiberglass and not plaster ask why? Be sure to ask if they have patients that had success in casting with fiberglass only)
4. Does the hospital have the proper casting table that’s specific to casting an infant/toddler and/or young small child? What kind is it? (This is extremely important and be sure to look it up or check with others to make sure it’s a proper table)
5. Will the cast have shoulder straps? (More and more surgeons who are casting do not use the straps. The shoulder straps are needed when the apex of the curve in the spine is anywhere from T-8 and above. However most idiopathic curves are below T-8 but there are some surgeons who no matter where the curve is, will only apply casts with shoulder straps and that’s perfectly ok too. Some of the best looking casts I’ve ever seen come from surgeons who have been doing this a long while and only use shoulder straps.)
6. Can you show me pictures of casts you have applied?
7. Can you put me in touch with any former or current patients who have or are casting to hear their experiences?
8. Have you had to remove casts early because of structural or application issues that have caused problems? If so why? And how often has this happened?
And here’s what a properly applied cast should generally look like:
• The cast should start at the chest and wrap all the way around the back. It should lie just under a child’s arms and be about three inches above the shoulder blades. This upper half of the cast should be snug but not too tight. It should not be loose at all.
• Most casts should have a small, SMALL, “D” shaped hole in the back on one side. Now the “D” hole in some cases aren’t necessary, generally if there is little to no rotation in the spine a hole on the back isn’t needed but that isn’t common.
• The front of the cast should have a wide, I repeat WIDE, tummy opening. This allows for proper growth of the ribs/chest wall cavity. It also is necessary to allow the digestive system to expand and contract naturally. A small opening is worrisome and something we are seeing more and more of. If you see pictures that show casts with small tummy holes ask why?
• Now the literature supporting mehta casting says the front tummy hole should not only be wide but also hourglass shaped. Where the “sides” tuck in around the rib cage to support it. That being said, I have seen many casts that just have a LARGE round tummy hole. There is one facility that is very reputable and is considered one of the best places for EOS and Mehta/EDF casting treatment and their casts don’t have the hourglass shape but do have the large round tummy hole. Some surgeons seem to be customizing the tummy hole depending on the location and severity of the curve. If the pictures of casts the surgeons shows you are not consistent in shape and design, ask about the benefit of the changing shapes and what your child’s cast will look like.
• The bottom of the cast should have a snug bar that wraps around the belly and to the back. There should be a snug “nip” at the waist of the cast as well.