Spina Bifida FAQ’s: Everything You Ever Wanted To Know About SB (updated 10/23/14)

October is Spina Bifida Awareness Month. For those that don’t know,ย Spina Bifida is the #1 permanently disabling birth defect in the United States. More people are affected by it than muscular dystrophy, multiple sclerosis, and cystic fibrosis *combined*. I was born with it. And so in honor of awareness month, here is a list of SB related facts. Some were posted on my FB last year, some are brand new. Lengthy as this entry is, it is by no means a complete list and if anyone would like me to add anything, please feel free to leave it in the comments section and I will fact-check it then add it. Still, it’s a pretty comprehensive list. Maybe bookmark it and come back and finish the 3 pages later. ๐Ÿ˜‰ As for my credentials to be saying all these things, I’m not a doctor. But I am an almost 40 year old man who has SB, and hydrocephalus and Chiari II among other secondary conditions that accompany SB. And I paid attention to doctors when they said things at the National SB conferences I attended for 9 years in a row. These are nationally renowned experts, in some cases world renowned experts, in SB. For some, their entire careers have been learning more about SB. So as far as I know from personal experience and from all those doctors saying all those things, every SB fact below is valid.
Don’t forget to click on the pictures to enlarge them. And all the links to learn more. As well as reblog, share, reTweet, repost on various social media and get the word out about this post. Let’s spread the word about Spina Bifida!! Pay close attention to #7 on page two. (Keep scrolling until you see the numbered pages, some people have missed that, and thought I only had 6 facts. I’m wordier than that) ๐Ÿ˜‰ So #7 is full of great information for new parents and for anybody who wants to know what the day-to-day is really like for us. Please click on all the links in #7 as well. And #8 is also pretty important in terms of educating able-bodied folks on something that is key when thinking of the way you see/interact with people with disabilities. Please click those important links also. And #21. Stop sharing that meme. Pretty please ๐Ÿ™‚

Happy reading ๐Ÿ™‚

Hi Milo! :)

Hi Milo! ๐Ÿ™‚

1. Spina Bifida (which means โ€œsplit spineโ€ in Latin) is a developmental birth defect often referred to as a “neural tube” defect. The neural tube is what eventually becomes the spine and brain. This defect, which usually happens in the first 30 days of pregnancy, before a woman even knows she is pregnant, entails the incomplete closure of the embryonic neural tube. What results at birth is that some vertebrae overlying the spinal cord are not fully formed and remain open. In the most common form of Spina Bifida called myelomeningocele -pronounced (no really) Milo My Ninja Seal- the baby is born with a sac on his back, filled with cerebrospinal fluid (CSF) and spinal nerves. The doctor will then place the nerves back into the baby’s spinal column and close up his back. The damage however, is done. This is not a “cure”. It simply stops the nerve damage where it’s at and closes up the back.

sb2. Spina Bifida is accompanied by a condition called hydrocephalus in 80%-85% of cases.

Hydrocephalus: “A chronic neurological condition characterized by an increased volume of cerebrospinal fluid (CSF) within the spaces of the brain called ventricles.”

Cerebrospinal fluid is produced by the choroid plexus, in the ventricles of the brain. This fluid then travels all around your brain and spine and serves to, among other things, protect and cushion those structures. Hydrocephalus is when that fluid doesnโ€™t reabsorb into the body, at which point it would then get reproduced, then reabsorbs, and so on. This cycle should happen 4x a day. With hydrocephalus, it gets produced as it should, but then just stays there in the ventricles. In younger infants, all that CSF can cause increased head circumference due to the skull not yet being one solid, hard piece. (Along with other serious complications including death. The bigger head is just the most obvious outward sign when youโ€™re a baby.) In older kids and adults, the intracranial pressure placed on the brain from all that CSF can cause headaches, blurred vision, decreased mental capacity and other cognitive changes including a change in personality or demeanor, nausea, vomiting, fever, infection, and if not treated with a shunt, can cause death. I have what is called a VPL (ventriculo-pleural) shunt which is a catheter that is placed within one of the ventricles, connects to a one-way valve, and runs down my neck, resting above my lung, in the lining between my right lung and my ribcage, where CSF is drained and then absorbed by my body. Sometimes, these shunts break. Or get infected, or the holes meant to drain CSF get plugged up. That causes the shunt to fail. Then a shunt revision becomes necessary. The picture below shows a ventriculo-peritoneal (VP) shunt, which is a more common place for the shunt to drain. VP shunt

And this is my head after my last shunt revision in August 2013.

shunt surgery

Hardcore, right? \m/(- -)\m/

3. Another condition that often accompanies Spina Bifida is called Arnold Chiari Malformation, or Chiari II. There are four different types of Chiari but if you are born with SB and also have Chiari, then you always have Chiari II. Part of the clinical definition is โ€œconsists of a downward displacement of the cerebellar tonsils through the foramen magnum (the opening at the base of the skull)โ€. Basically part of your cerebellum goes down into the top of your spinal column where itโ€™s not supposed to be. Among other things, this stops CSF from flowing freely. Also, your cerebellum controls autonomic functions, which are things you canโ€™t control consciously, such as breathing and swallowing and regulating body temperature. These autonomic functions are affected when you have Chiari II. Some symptoms are more severe than others. While some people end up with nothing more than a pretty bad gag reflex, for others it can be more serious and cause choking or severe breathing problems, sleep apnea or other sleeping disorders.
Here we have a typical brain and a Chiari brain. Note the herniated cerebellum, lower than it should be.

chiari and syrinxConveniently, what this picture also shows is a syrinx in the spinal column:

4. Syringomyelia. Or a syrinx. A syrinx is a fluid (CSF) filled bubble that forms in your spinal column. As previously mentioned, the spinal column has CSF running all through it. But within that, a cyst can also form, also filled with CSF. And it can be small or it can run the whole length of your spine. The length matters less than the width. If it’s too wide, it can crush spinal nerves and cause even more damage. In those cases, the neurosurgeon can try to drain it, although it may return, or she may place a shunt in your spine which should do the same thing it does in your brain; ie, continually drain CSF.

My syrinx is small, only at c7, and not obstructing CSF flow around it or crushing nerves. It was that size at an MRI in 1992 and that size at the MRI in 2013 and hopefully it will never get bigger.

5. Children with SB are often referred to as “million dollar babies” because of the estimated healthcare costs over a lifetime of care. (This source is calculated in 1980’s money, so it’s actually quite a bit more!)

sb baby6. Spina Bifida is the #1 permanently disabling birth defect in the United States. More people are affected by it (or would be….more on that in #7) thanย muscular dystrophy, multiple sclerosis, and cystic fibrosis *combined*. It’s also the most complex birth defect you could have and still survive. This is because it affects almost every part of your body and organs, either from the SB itself or from secondary or tertiary conditions. For example, one way your brain is affected is from the hydrocephalus, yes, but it can also be affected from the shunt surgeries that are meant to correct the hydrocephalus. Too many shunt surgeries can cause scarring on your brain which can lead to seizures. 40% of people with SB have some form of epilepsy. SB affects kidneys and can also have an effect on other internal organs for various reasons, including the fact that some of us have curvature of the spine, which can cause our lungs to not have enough room to adequately expand in our rib cages. So fact #6…..SB is not only the most common permanently disabling birth defect but is also the most complex in terms of body systems affected.

But if we are in fact #1, then how many of us are there? Well, rough estimates from various sources say there are currently 166,000 people in the US living with SB. I know what you’re thinking….”But wait….shouldn’t that number be higher?! If it affects more people than CF, MS and MD combined, shouldn’t there be more?!” Yep. Which brings me to SB Fact #7 and an explanation for why there aren’t more of us if we are ahead of those other more well-known conditions:

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48 Responses to Spina Bifida FAQ’s: Everything You Ever Wanted To Know About SB (updated 10/23/14)

  1. I don’t know if you’re still posting on this blog, but I just came across this post today. Today I also sat through multiple tests and then a meeting with my husband and over 8 specialists to talk about our unborn son who was just diagnosed with SB. Thank you for writing this. We are just outside of Denver and are going to Children’s here and have been given nothing but hope for our little boy. Thank you for being honest and straight forward with how your life is while living with SB. You give me hope as a mother and reading your story helps me prepare for how to help our son the best we can.

    Like

    • I’m so glad you came across it and it was helpful to you. Yay. โ˜บ๏ธ I’m also in Colorado and go to both children’s and University for care. I know you will he be in good hands with the staff at children’s.

      Liked by 1 person

  2. Jeanette R. Banahan says:

    You said that 65% of unborn babies with possible spina bifida are aborted. What is your source for that figure? I’m not saying it is or isn’t accurate. I would just like to know how you obtained that figure. Thank you.

    Like

  3. Trish Ings says:

    A very interedting article. Even as a person with Spina Bifida I learnt a thing or two!

    Like

  4. CorieSue Freeman says:

    Thank you so much for such an informative, easy to understand article. My 4 1/2 year old grandson, Ryan Noyes, was born with SB and has a shunt too. He almost died from meningitis while still in CHOP. Today he is racing with braces and crutches, and is so bright I call him my Doogie Howser. The down side is he’s still in diapers. There was a great article in the Reading Eagle about him and his best friend Gema. You’ll probably get a smile from it. CorieSue Freeman. Coatesville, PA

    Like

  5. lori nichols says:

    My sister is 23 and she has spinabiphida, she unfortunately is paralyzed from the waist down. She has a very happy life despite all she has been through. Three shunt replacements, 7 back surgeries, multiple pressur sores and even a blood clot in her leg because some “genius” pediatrician thought putting her on BirthContol would help her with dealing with puberty….that was such a horrible hospital stay, now her leg swells all the time as if she didnt already have circulation problems. She was born in 1992 in Orlando, fl. Out of the 5 high risk births that night, my sister was the only one to go home. A social worker literally visited every parent and talked them all into giving up their child, all except my parents.

    Like

  6. Jennifer Nichols says:

    My daughter Angel Nevaeh was born on May 30th 2012 with Myelomeningocele with hydrocephalus. She is almost 3 years old and has had a rough but happy life. She’s had 5 surgeries and two of them were for her VP shunt. She’s been in and out of the hospital just this last year for ITP. ITP is when your blood platelets get low and there’s no reason as to why. She finally has overcome that. When I was told at 22 weeks pregnant that they need to consult the doctor first I already knew what was happening, I was going to have another special needs child. I was afraid for only one reason – is my child going to live only as long as my son did? My son was born with anencephaly and lived 80 hours. I was blessed with the time I had with him. They asked during both pregnancies if I wanted to “terminate” my answer was HELL NO! Then I asked will it hurt me or the baby to go full term and their answer was no. My daughter is struggling with moving her legs. But she can do it a little, mostly at the hip. She LOVES her wheelchair since she can get around like her cousins can and she’s always been the happiest child ever. God gave me this special gift to make me a special person. She isn’t disabled in my eyes and no one has ever been disabled to me. We all have things we have to deal with. I just want the best for my Angel. I never even knew either of these birth defects were out there. I don’t have any genetic disorders in my family but I do have a blood clotting disorder where I clot way to much -MTHFR. Which is also where I have to double up on vitamins since my body doesn’t keep much or any nutrition in. Thank you for your story. I’ve learned so much

    Like

  7. Lavonne says:

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    Like

  8. Michelle Noris says:

    One of my son’s friends, and his basketball coach, have SB. I learned a lot that I would not have asked them. Thank you.

    Like

  9. cuadramarcia28@yahoo.com says:

    My daughter have spina bifida club feet she is 5 years old very smart,happy little girl she was in the wheel chair for 3 years two surgeries was needed n all the sudden she just decide to walk bless to have her, like every mother out there that are told by doctor to have the option of not having I decides to have her never in my mind would give up on her ,I learn so much of this condition n love having my beautiful isabela in my life a gift from God..

    Like

  10. Lisa says:

    While this is a good article, there is a major error although not related to SB. I have MS, and it is NOT a birth defect. I hope you will correct that fact. Thanks!

    Like

  11. Pat says:

    I was born with SB myself, its anything but easy. Ive had 16 surgeries In my short life and none of them have been a walk in the park. Sure there are some perks to it, but for someone like me, spending my entire life in a wheel chair isnt so much fun as it may seem. To be honest, Its quite cool to see a fellow SB patient share their story and be totally open to help everyone and anyone. Props to you my friend.

    Liked by 1 person

    • Props to you for still being here. The world needs you. ๐Ÿ™‚

      Like

    • This is going to be a TOO long post but I’m buzzing because it is a splendid end to a great day! It might even inspire my own blog posts…. Found this article amazing because I’m planning a project called “It’s Not Just Me” on living with mild CP, PTSD and all the stuff in life that has made me less than the stereotypical supercrip from not being happeeee all the time and the UK Government to the stuff that gets you called ‘not a real disabled person’ (even by other disabled people) or rejected by either the fully paid up wheelies or the people who come to it later (see Spoon Theory) I’ve plenty to say on disability politics and politics, how they mess up the relationships of those everyone thinks have all the same opinions and life experiences – sad and and heroic, NB: actually we should explain to them it’s like office politics with more wheels and less paychecks!?
      Every post I’ve seen today on any topic has been serendipitously linked in some way to the previous one and towards my surviving rather than succumbing to doom. . Friend’s husband shared this via FB minutes before the Daily Mail (UK) put up an article about how CP (cerebral palsy) is probably genetic in 54% of cases so they’ll soon be able to test for it and…yes…you know THAT …then they’ll be able to get it…sorted. Sort of….stuff. Gee. Thanks! Life unworthy of life and all that, eh? So pleased to meet you my fellow medically identifiable anomalies! …. ๐Ÿ™‚ Thanks for the uplift!
      Re the sharing outside SB community? Will do. – Friend’s husband who shared this is worth a mention because, yes we do have husbands. Even those of us who were told to abort our longed-for babies because of our CP and PTSD have husbands though it’s a wonder our menfolk survive being told to abort our babies either when they have to assimilate the sheer prejudice all in one hit in a doctors surgery – he’s still here though whilst we refused to abort, we simply didn’t get lucky.
      We were STILL handed a packet of Folic Acid with the words “For the prevention of Neural tube defects and other abnormalities) in bigger letters than “Folic Acid” even though the doctor didn’t ask us to consider -she DEMANDED I terminate. (Errr… I was already taking it thanks….)
      HUGE props to any one of us with the guts to think outside the box brave enough (lol, I know!) to say it how it REALLY is! I have CP not SB but I’m fascinated by the similarities and differences. Not going to pretend I understood all the numbers stuff – but that’s OK because I skimmed then went….. “NLD??? “YIPPEE they have it too?” (I now even have _word_ for my ‘maths stuff’ it that is neither ‘brain damage’ nor a list that sounds like a medical textbook (spatial awareness issues, no depth perception, prosopagnosia, dyscalculia – phew! ๐Ÿ™‚
      Very very informative, funny and erudite and packing more identity politics, academic thought and sheer blooming how we wish people wouldn’t say “OOOh, you’re having a WALK today, well done!!” just because we were daft enough to try to walk round the shops instead of trying to take a quick break before the walk back….That’s not inspiration, that’s forgetfulness!
      Finally: You explained (possibly) why when my allergies were through the roof I couldn’t eat bananas. It’s just enough latex in my early life to provoke a ‘nooo’ from my immune system. Or not… ๐Ÿ™‚ A blog I’ll be coming back to often!

      Like

  12. Tony says:

    My name is Tony and I was born in 1969 in Brisbane, Australia. I have neither hydrocephalus nor Chiari malformation. No learning difficulties either. Very blessed. Looking forward to living into the next couple of decades and beyond, God willing. Am happily married since September 2004 with 2 beautiful teenage stepchildren.

    Liked by 1 person

  13. It’s 3:45am…and I’m reading a again for the umpteenth time. Same results as always. I laugh. I tear up. I’m happy to call you friend. I mean, you have not been retweeted by Mick Mars, but you’re still pretty damn awesome to me.

    Like

  14. Rebecca says:

    Thank you so much for this wonderful list full of accurate info, resources, experiences and history! My brother was born in 1975 – during the time period when myelomeningocele (open SB) was considered not to have a quality of life and parents (including mine) were pressured into not even having the lesion closure surgery. Due to this, infection can obviously enter the exposed spinal cord, which happened to my brother and he died at exactly 2 months old. My parents, especially my mother, have always been guilt-stricken by this.
    Fast forward, when my husband & I decided to start a family, I wanted to be sure I did everything I could to make our baby as healthy as possible, so I scheduled a pre-conception health exam. At the appointment, I explained my brother’s SB history and asked what I could do to help prevent SB in my own children; the NP acted like I was crazy and said it’s not genetic at all. I kept pushing for at least a recommendation for higher FA intake and she finally told me the amount recommended for women who have already had a child with SB (yes, let’s wait until after the fact shall we?!). I started taking prenatals as well as the extra FA months before we started trying to conceive. We went on to suffer 2 miscarriages (one a set of twins), but also had 2 completely healthy, full-term, typical babies. Then we were expecting baby #3 (pregnancy #5) and at 18-wks gestation, my husband and I found out our 3rd baby had spina bifida – myelomeningocele (L1-S). The doctor kept repeating in a grim tone that the opening was “very large” and kept pushing abortion. We refused abortion as an option. I was then referred to a high risk OB with an open mind. She told us multiple times that current standard of care is to only recommend the 4,000mcg of FA to women who already have had a child with SB, but due to my brother and my son both having SB, she would immediately personally start looking at the woman’s whole family history and recommend it for women with SB in their family at all. (Thank you!) Our little guy is now 15-months old, smart as a whip, a true charmer, has Chiarri II, hydrocephalus, VP Shunt, bowel and bladder issues, paralyzed from his hips down, incredibly strong upper body, and had his ZipZac wheelchair genuinely mastered by 12-months old which he chases his brother and sister in and gets into trouble just like any other toddler. His lesion is larger than my brother’s was (I’ve seen pictures of him), which tears my parents up inside seeing how un-disabled their beloved grandson is with life and thinking how HIGH quality my brother’s life would have been.
    Thank you in behalf of parents of babies with SB for giving your friend the speech. My husband finished RN school while I was pregnant with our kiddo with SB (he took and passed his RN boards while we were in the hospital for weeks after delivery!) and it’s absolutely true that Spina Bifida was barely touched on and skewed – my husband actually ended up educating his teacher and classmates about it! That 65% is largely the fault of medical education for sure and has to change.

    Liked by 1 person

  15. Jessica says:

    Thank you for writing this! It is so informative and easy to understand! I have a 9 year old boy who was born with spina bifida. I found out when I was 7 months pregnant. I got the same grim story from the doctors, He will never be able to walk, his brain isn’t going to develop, your to young and already have another child to think about (I was 25 and had a 4 year old son). I was heart broken by hearing all this, but didn’t care i wanted to give my son a chance. Like i said he is now 9. He walks with out help has partial control of his bladder and bowels. Loves baseball and basketball. Struggles with math and at least now thanks to your article i understand why and will pass this on to him. I could not imagine my life without him!

    Like

    • They said almost the exact same thing to my mom back in the day. She was 23 when I was born and had a 3 year old son, my older brother, already. I’m so happy you chose to have your boy โค

      Like

    • My God. The phrase ‘late term abortions’ just took on a whole new meaning. and I was born at 7 months myself.
      God bless you. It takes a lot of courage to be the sort of person who tells a doctor where to go. I know.
      I had to when expecting. Hugs. Thank you from a person with CP who also had to hear ” You MUST have an abortion!”
      Thank you.

      Like

  16. Reblogged this on shirleydonkey and commented:
    This blog on Spina Bifida was written by a good friend of mine. I hope you enjoy. Go to his page and read his other entries too.

    Like

  17. Susan Winters says:

    This is Awesome! My daughter was born in 2001. Myleo L5-S1 hydrocephalus chiari 2 neurogenic bowel n bladder (and more). This is the most comprehensive written sb explination i have seen to date.

    I have said for years (counselling other parents at the childrens hospital, various sites, in general conversations as a parent that has been there) that a non scare tactic explanitory realistic view information packet/book/speech should be provided to parents instead of the insane amount of digging research tears fears us that are thrown worst case scenarios and left to fend for ourselves in trying to make an informed choice. Bless you for being an advocate.

    Liked by 1 person

    • Thank you, I appreciate your kind words. As I told that doctor back up in #16, it would be medically irresponsible to sugar coat the diagnosis and pretend everything is gonna be just great. But scaring the living daylights out of parents with worst case scenarios is, I believe, equally as irresponsible. That contributes to the 64% abortion rate. ๐Ÿ˜ฆ I also wish they had a non scare tactic, updated, 2014 version of medical information to give.
      Did you happen to click the last link in the blog? I only ask because it might come in handy someday. ๐Ÿ˜‰ It’s suggestions (and only suggestions ๐Ÿ™‚ ) for raising a child with a disability, so that they become self sufficient independent adults (as much as possible with each child’s situation of course.)
      Thanks again for you kind words. ๐Ÿ™‚

      Liked by 1 person

  18. Meg says:

    There is another form of Spina Bifida, Lipomeningecele. That is the type I have. There is a fatty tumor usually near the base of the spine where the break is. It’s rare, I believe 1 in 50,000 births.

    Like

    • Shirley White says:

      I am 69 years old and have the lipomyelomeningocele too. I have led a pretty active life until I had an untethering when I was 52. Before surgery I walked unaided. Now I use a cane, walker, or scooter depending on how much walking I need to do.

      Like

  19. Nikki says:

    MY son is using like a shoe insert, its customized and his doctor made a comment that when he is done with his growth spurts , I suppose around 18, that he is a good candidate for a surgery that would make it that he did not need any walking aides or braces at all.

    Like

  20. Paula says:

    64%?? That astounds me. Leaves me speechless! My daughter is now 13 and has Spina Bifida. When we were told that she had Spina Bifida they asked us what we wanted to do. Our response was “Well, I guess we will have to learn a lot about it and quick” I can’t imagine having aborted her…it was never an option to me. My biggest struggle was finding real information…information on how real people and families lived with SB and dealt with things. I found some medical things, but I felt that was mostly worse case scenario. I really wish more people would share their experiences more. I’ve even thought about starting a site about how we’ve gotten through things and tips and tricks that we’ve found helpful.
    Thank you so much for this! I learned things I didn’t know and plan on sharing this.

    Liked by 1 person

  21. Julie says:

    Very well said – my daughter will be 23 in November and we were told she would not live to 18. Shared it on my facebook

    Like

  22. Nicholas John de Klerk says:

    Hi I’m Nicholas, but you can call me Nicky. I am 15 years old and was born with SB. Is it possible that people with SB will be able to walk at any time of their lives without the use of crutches, braces, etc.?

    Liked by 1 person

    • Hi Nicky ๐Ÿ™‚
      All I can tell you is about my experience and those of my friends with SB. And some of us do walk. With afo’s (braces), or forearm crutches or walkers. For example I can walk with braces, but I prefer not to, because they really help with balance. But there are also those that walk without any assistance at all! I can think of 4 friends off the top of my head that walk without aid. It’s not that their SB is “mild”. I think that term us inaccurate. I think their SB just affected them differently; affected different nerves than on other people. One who walks unaided has pretty severe kidney issues for example. It’s just different for everyone. As far as going from aids to walking without aids, I think anything is possible, and with technology being what it is, who knows what can happen even 5 years from now! ๐Ÿ™‚

      Like

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