Pneumonia and A Change of Plans

Nora’s had a cold recently and yesterday we found out it has turned into pneumonia. This can be more complicated for a couple of reasons. I should back up a little, she’s also out of her cast.  She’s been out for over two weeks now.  Not ideal.  We had to postpone her casting twice because of her cold and now once again because of pneumonia.

But back to the complications.  Because Nora isn’t in a cast right now her spine has ‘relaxed’ into its normal state (it could possibly have progressed some too she hasn’t had an x-ray since August so we don’t know for sure). Her curve is already measuring 60 plus degrees. Since its relaxed out of cast, it’s pressing into her lungs now. This is one reason why we cast her, to get her spine straight in the cast so her lungs can grow properly. Now with pneumonia and the pressure on her lungs it could make it harder for her to kick the illness. Thankfully it was caught early so that’s on our side.

The other setback here is this will push her next casting out even further, most likely 4 weeks or more. The anesthesia team will not put her in the OR so soon after an infection in the lungs. Being put under puts a lot of strain on the lungs as it is, if they aren’t fully healed it can easily bring on a very serious infection or illness.

So this could change our casting and bracing plans for summer. This could change our summer vacation all together, which is upsetting and frustrating. But we’ve learned a long time ago with this diagnosis we have to just take it one step at a time. Right now we just need to focus on getting her healthy. Thankfully she’s a tough little tater tot. She’s smiling through it all, a good sign indeed! We can do this!

 

Rainbows & Lollipops or Casting, Bracing & Surgery

Next month Nora will get her ninth cast. It will also (hopefully) be her last cast. Is it strange to say the feelings this brings up are bittersweet? It is strange. I’ve had a love hate relationship with Mehta casting.

I love that our surgeon is open minded and aggressive yet conservative too. Those are the ideal qualities you want in a surgeon. I’m grateful he wanted to do casting with Nora at just 14 months old to try and slow her curve’s progression and buy very valuable growth time. Casting for congenital scoliosis isn’t considered by many surgeons yet; many still don’t think it can work as a delay tactic for surgery in these kids. I love that casting has worked for us for almost two years. I love that casting hasn’t held my daughter back from doing most of the things normal kids do. I love that she’s young enough that she probably won’t remember any of the difficulties involved with all of this. I love that she’s young enough to be proud of her cast and not embarrassed or worried about being different and being teased.

But I hate the cast too. I hate, and I mean hate, the actual casting process at the hospital. I hate how difficult it is to take your tiny little child to the OR and be given anesthesia to be put to sleep while she’s then placed in traction to have a heavy plaster cast applied to her torso. I despise even more how hard the process has been for us coming out of anesthesia. Nora has a horrific time coming out of anesthesia. I’ve done this nine times now and I’ve yet to make it through without tears and begging and pleading with God. I hate that I don’t get to have the collapse-in-your-arms-and-feel-your-child’s-chest-breathing-on-yours type of hugs. I hate not being able to stroke her back. I hate sponge baths as much as she does. I miss water and sand and not worrying about my child getting head-to- toe dirty like a kid should. I hate getting smacked in the head, face and shins from that hard-as-a-rock cast in the middle of the night. I hate that this has been two years of our life, of her life, when she’s so little. I feel robbed in ways.

But I would do it all over again. And I would, without hesitation, encourage anyone who has the opportunity to cast their congenital (or idiopathic) early onset scoliosis child to do it. The benefits are far too great.

So cast life will soon be coming to a close for us. I’m really really excited but I’m also anxious and scared because now I have to think about our next steps. I’ve been able to live in this, in some ways, safe casting world for so long now, I’ve lived cast-to-cast and now that’s ending. Once this last cast comes off in May she will be fitted for a brace for summer and then have surgery late summer.

Now I have to think about surgery. We haven’t made any specific decisions yet on what the surgery will be, we have some time for that, but I still have to think about it now. I’ve made the mistake of watching an actual surgery on YouTube that is a potential option. Why? Because I’m a little crazy. I didn’t watch long but now when I think about surgery, I think about that happening to her. To my sweet little Nora. I think about the how long the surgeries are, the risks, the recovery. My heart races and I can’t help but think I’d much rather be in the recovery room after a casting. That’s right, the thing I just said I’ve despised the most suddenly sounds like rainbows and lollipops in comparison.

For a little longer, I can just focus on this last cast. And on that note, I think it’s time to dig into our Duct Tape collection and pick out our next and final decorative cast tape. Bittersweet.

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Tummy Time, Back to Sleep and Infantile Scoliosis….What Do they All Have In Common?

I was asked to researched and write the following article for the Infantile Scoliosis Outreach Program.  Please note this piece is not considered a valid research study.  I do, however, reference peer-evaluated studies that have been accepted by the broader medical community.  I would also like to note, while my focus for this blog has generally regarded my family’s personal journey and/or has been specific to congenital scoliosis, the information in this post DOES NOT have any bearing on congenital scoliosis. Congenital Scoliosis is present at birth and means there are abnormally shaped vertebra(s).  There is nothing that can be done to prevent CS from occurring and there is nothing that can be done to affect the progress of CS besides proper medical intervention.    This post addresses concerns around the infantile/early onset idiopathic (no known cause for scoliosis) scoliosis group only.

 

In the early 1940s, Dr. Harold Abramson, a New York pediatrician, pored over heartrending reports of babies who accidentally suffocated while they slept. As he reviewed case after case, he noticed that a vast majority of the deaths occurred when babies slept on their stomachs. After decades of additional research the federal government, the American Academy of Pediatrics and child advocacy groups formally launched the Back to Sleep campaign, instructing parents to place infants on their backs for sleep for the first year. There’s no question the Back to Sleep campaign has helped save lives. Since 1994 the rate of Sudden Infant Death Syndrome (SIDS) has declined by more than 50 percent. What this campaign has also effectively done is scare new parents so much that they don’t want to put their babies on their tummies ever.

More research suggests taking away “tummy time,” cuts off a pivotal avenue of development. The less time infants spend on their stomachs, the slower they generally are to acquire motor skills during their first year, which means the potential delay of simple feats like lifting their heads as well as more-complicated movements like rolling over, crawling, and pulling to stand. Doctors have hesitated to sound the alarm about this, since children usually walk shortly after their first birthday regardless of how much tummy time they’ve had. But a growing body of evidence now suggests that the timing of the motor-skill milestones that precede walking is crucial and can even factor into long-term health and cognitive ability. Pediatricians however, have had mixed reactions to this and have passed this off as inconsequential. Others, including the American Academy of Pediatrics, champion of the Back to Sleep campaign, have seen the head shapes and motor hang-ups as a harbinger of future problems and recommended supervised tummy time when a baby is awake.

Here’s where infantile scoliosis fits in. Parents are seeing the potential of death as outweighing the potential of delayed motor skills. What parents aren’t hearing are the potential increased risks of Infantile Scoliosis, the most challenging orthopedic condition in babies, from not having sufficient tummy time.

Prior to the 1980s the incidence of infantile scoliosis was much higher in Europe where infants were commonly placed on their backs to sleep. During this time babies in the US were traditionally placed on their stomachs to sleep and the incidence of infantile scoliosis was a rare phenomenon in North America accounting for less than .5% of all diagnosed cases of scoliosis. During the 1980s Europeans adopted the tummy sleeping position for children and the incidence of infantile scoliosis dropped to record low numbers.

Now take a look at Scotland before the 80s, where parents were routinely advised to place their infants to sleep on their backs, cases of infantile scoliosis accounted for 41% of all diagnosed scoliosis cases. After 1980 Scotland reversed their stance on back sleeping and the incidence of infantile scoliosis in Scotland dropped to 4%. At the same time there is research going as far back as 1966 that states one of the benefits of stomach sleeping was the prevention of scoliosis.

So what do parents do with this conflicting information? Putting an infant to sleep on his or her back is without a doubt the recommended sleep position for a baby’s first year of life. However, tummy time is equally important and recommended for motor skill development and many would say for the prevention of scoliosis. The key is getting a sufficient amount of tummy time in. Parents should be encouraged to have their babies spend a healthy chunk of awake and supervised time on their tummies. This should begin soon after birth once the umbilical cord stump has fallen off. Several times a day so the child becomes used to it early on and likes it. There are lots of ways parents can practice tummy time, propping a baby on a nursing pillow while on the floor with them or even on a parent’s chest are great ways to get that added tummy time in and keeping everyone comfortable. Baby wearing is also greatly encouraged, as it too also helps promote physical development and decreases the risks of a baby developing infantile scoliosis. When parents choose a baby carrier it’s important to look for one that is comfortable to wear but is also ergonomic for baby.

How Do You Know You Have The Right Pediatric Orthopedic Surgeon?

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:

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• 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.

 

There’s A Place For Each Small One…

During the holidays, we dug through the drawer with all of our old DVDs looking for the Christmas movies to introduce to Nora.  Disney’s Mickey’s Christmas Carol was one of them. The Mickey’s Christmas Carol DVD has three other “bonus” cartoons, with a special one titled Small One. I had never seen this animated short film before that was produced in the late 1970’s.  I fell in love with it instantly.  And to me,  the message in this movie speaks to our hardest hit scoliosis warriors.

The story tells of a young boy, outside Nazareth, who has a small donkey that can no longer live with a farming family because he cannot earn his keep. The donkey is playful and good spirited, but he is older and smaller than the other donkeys. The father tells his son that the donkey must be sold, but when the son brings the donkey into town, the pair is ridiculed and mocked. The son loves the donkey and is hurt over all the abuse that they suffer. The redemption comes when they encounter a man seeking to purchase a donkey and he shows them respect and kindness. The man needs a gentle…special donkey to carry his pregnant wife to Bethlehem. The boy sells the donkey to the kind man and watches as the couple and donkey leave on their journey as a bright star appears in the sky.

While they don’t say it, it’s clear the kind man was Joseph. And he was looking for a donkey to do the very important job of carrying Mary, pregnant with baby Jesus to Bethlehem. The point is that despite his appearance, this donkey will play an important role in history. It’s a story that has Christian themes, but transcends religion and really is a story about friendship, sacrifice and purpose. And how each individual deserves dignity, everyone has their place, and kindness should be shown to everyone.

The beautiful, haunting and even heart-rending, theme song has such meaningful lyrics. The obviousness of the song immediately reminded me of all the stories I have read from parents, teens, and adults on numerous support groups about their struggles with what severe scoliosis and the surgeries they have had to endure have left them with… How they have been robbed of something that seems so basic…Stature. So many heartbreaking stories that showcase the cruel world we live in where being exceptionally short and different is a punishment in this world.

The song from this movie is kind, sweet, and purposeful.  Reminding that all are  loved and needed and they too can play an important role in history.  Maybe this is why I fell in love with this movie the way I did and got chocked up every time I watched it. Here are the lyrics:

Small one, Small one, Don’t look so blue
Somewhere a friend is waiting for you
Someone still needs you to brighten his day
There’s a place for each Small One
God planned it that way
Time is passing, days are few
Give while you can to one smaller than you
All things living great and small
Each brings a gift that is needed by all
Small one, Small one, Don’t look so blue
Somewhere a friend is waiting for you
Someone still needs you to brighten his day
There’s a place for each Small One
God planned it that way.

You can watch Small One here on YouTube or just listen to the theme song which is played in the opening credits.  https://www.youtube.com/watch?v=MGa6oCxhpZA&noredirect=1

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Social Media Hooked Me Up with My “Match.com” Style, Perfect Mom Match.

Since the day we got Nora’s diagnosis at just 5 months old, I’ve thought about something related to her condition or treatment Every. Single. Day. I’m not complaining, I know we all of have something we are dealing with in life, I’m no different than anyone else.  And I’ve taught myself how to deal with it so that it doesn’t constantly weigh me down. Like anything, some days are better than others. But I’m so incredibly grateful to have gotten this diagnosis in the age of the internet and social media. I can’t imagine trying to navigate through all the ins and outs of this complicated rare disease without all the resources and support online.

Through social media, I’ve met so many other parents and families who deal with some of the same things we do. I’ve learned so many helpful things, they have been there as an amazing support system that unless you are living this you can’t fully comprehend; they can hear my tears and help wipe them away through the keystrokes of their computers or tablets. And they are right there to celebrate victories with emotionicons and uplifting typed words. I have strong feelings and friendships with many of these people, yet I’ve never met them. It’s weird and really cool at the same time.

A while ago I met a mom in one of my smaller support groups that’s dedicated to just congenital scoliosis. It’s a much smaller group simply because it’s so rare. She was new to the group and was sharing her daughter’s story one day. I was instantly excited and intrigued, her daughter’s case sounded very similar to Nora’s. It was incredible, that just doesn’t happen with congenital scoliosis, they are all so very different. Our daughters were only a few months different in age, they both have hemi vertebras at T-10 (Nora has one at T-11 as well but at the time we were hoping it wouldn’t be as big of a problem as we later learned it is) and had the same degree of curve, they were both the same height, weight… the list went on. And we learned we were both in the process of setting up appointments to meet with the same surgeon who is considered one of the best in the country. It was just what I needed at the time, to actually find someone whose child’s case was so similar.

We shared with each other every single report and x-ray we received from our surgeons. I even shared it all directly with our surgeon asking question after question. In time, I found out that, yes, our daughter’s cases were very similar yet they were still different. We were going down different directions of treatment and soon we learned Nora had two discs in a row that would have to be addressed, changing our treatment options and her daughters case changed a bit too.  This was difficult and confusing for me at the time, it was hard to wrap my head around the different directions and it scared me. I kept thinking our cases were still similar; they had to be treated the same way. It took time on my part to fully grasp the complexity of this disease and deal with my own denial yet again.

We still continue to share every detail we get with each other. We’ve also both learned that our daughters will be facing lifelong battles with scoliosis; this isn’t a one surgery and done situation. Another punch in the stomach neither of us were really expecting.

This woman’s daughter is scheduled for surgery next week. For weeks now we’ve been in contact with each other pretty frequently, often discussing all the details, coming up with questions to ask the slew of doctors who are involved, talking about how we will actually handle surgery day, how surgery will impact the rest of the family, etcetera, etcetera.

I think about her, her daughter and her family a lot and lately…all the time. I talk about her and her family with Matt pretty often too. So much so that Matt is even asking often how things are going knowing full well how stressful it is leading up to surgery. Yet I’ve never met her or even spoken live words with her. It’s the oddest thing to feel very connected to someone you’ve never met. I’m convinced if we lived near each other we would be friends. I wish I could be there to give her a hug or hold her hand when her daughter is in surgery. I know I will be thinking of her that entire day, hoping and praying.

I hope to actually meet her face-to-face one day because we understand each other in ways no one else really can. It’s a bond I can’t explain. And yet it’s still strange to me on the surface. I hope our daughters become friends too one day, some type of modern day pen pal or something. Somehow in my crazy head it’s a comforting thought in the sea of scary facts and unknowns swimming in my head, the idea that our girls could have someone else out there who they can relate to, confide in and is someone who actually understands firsthand what each other is going through, is one of my hopes for both of these girls.

 

The Curious Incident of the Bucket of Sand and the EDF Cast

If you have a child in an EDF/Mehta cast you know there are a few innocent, even wonderful things that are now looked at as products of the devil himself: water, wood chips, rocks and sand. Thanks to a child who was old enough to know better, we came face-to-face with that devil called sand.  To say I was not happy was an understatement.

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A bucket of sand was dumped all over Nora’s head. Sand quickly made its way all over inside her cast. Sand inside a cast is the equivalent of shards of glass if it remains in there. Children have had to have casts removed early because of sand. It can quickly break down the skin which can be a big problem and delay casting. Skin has to be in good condition to be casted.

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So anyway, we rushed home to try and fix the problem.  I did everything I had heard to do when something is in a cast that shouldn’t be. We removed her knit rite under shirt, I wiped away as much sand as I could, I used a hair dryer on the cool setting to try and blow it out, I “flossed” the cast with pantyhose and wipes. I thought we were ok till that afternoon when I looked again, still so much sand everywhere and her belly was red from it.

It was then when I inquired with my online support group family of what to do. That’s when baby powder also known as talcum powder was suggested. Our surgeon was also quick to get back to me with the same baby powder suggestion.

Let me tell you folks, your very first step after removing as much sand that you can visibly see, is to pour baby powder into the cast from every angle be generous. Then I found a gentle little twist and shake of my sweet Nora helped really get it in there. The baby powder has this magical way of adhering to the sand and releasing it completely from skin. My next step was to use the hair dryer on a cool setting to blow it out. That was it, the sand was gone!

Here’s what I recommend NOT DOING:

• DON’T do any type of flossing when sand is involved, it just rubs the sand into the skin or pushes it into the padding.
• DON’T use wipes either, you don’t want to get the sand wet.
• DON’T panic and take the cast off, you might be able to save the day.