Thursday, August 2, 2012

Everything you need to know about SB

As a mother of a child with spina bifida, it irks me to no end to read how prenatal vitamins containing folic acid will prevent neural tube defects, such as spina bifida.  This statement is incorrect and usually written by those who have no medical training or experience with neural tube defects.  The truth is - folic acid reduces the risk of your baby developing a neural tube defect.  It does not completely prevent it and if I can be honest here, that incorrect statement is an insult to all of us who have babies with these conditions.

What is spina bifida?

In simple terms, spina bifida occurs when the bones of the spinal column do not properly form around the nerves of the spinal cord as the baby develops in utero.  Because this happens within the first 30 days of pregnancy, it is recommended that all women of childbearing age take folic acid supplements.  If you have a child with spina bifida, you are at an increased risk of having another baby with a neural tube defect.

Spina bifida occurs in approximately 1 of every 1,000 births worldwide.

When Anna was born, a portion of her lower spine (think nerves) was contained in a jelly-like sac.  The majority of babies with spina bifida are born via c-section to reduce the risk of infection to the baby.  I say 'majority' because sometimes it isn't known that a baby has spina bifida until after birth.  Although she underwent surgery within hours of her birth to close her spine, affected nerves remained damaged.  Those cannot be repaired.

There are three different types of spina bifida:  spina bifida occulta, meningocele and myelomeningocele.  Anna has myelomeningocele, the most severve form of spina bifida.

Why does Anna have a shunt?

When the spine doesn't form properly, other conditions result.  Anna has been diagnosed with an Arnold Chiari II malformation, which is common with myelomeningocele.  Basically, a portion of her brain is pulled down from where it should be.  We all have fluid in our brain and that fluid drains down through our spine.  With an Arnold Chiari II malformation, the fluid is blocked from draining and causes hydrocephalus, which is accumulated fluid in the brain.  Hydrocephalus is painful and can cause damage to the brain if left untreated.

Anna was diagnosed  with hydrocephalus (and spina bifida) during a routine ultrasound at 17 weeks gestation.  The excess fluid was measured during every follow-up ultrasound to ensure that it wasn't expanding.  The fluid was measured and monitored after her birth and it was decided (as we suspected it would be) that she needed a shunt.  So a shunt was placed in her brain at only a few days old.  The fluid still was not draining properly so a week later, she underwent her third surgery - shunt revision.

Her shunt is all internal.  A stranger would never know that she has one.  She has a small bump on the top side of her head, which is completely covered with hair.  The tubing runs underneath her skin behind her ear.  If you were to closely examine her, you would probably see the tiny bump from the tubing.  She also has a small scar on her midsection, where the shunt drains.

The shunt redirects fluid from her brain to her abdominal cavity.  Unfortunately, there are quite a few scary downfalls to shunts.  Her neurosurgeon has even referred to them as 'rudimentary.'  They can become infected or an infection in another part of the body can travel up the shunt tubing into the brain.  The shunt can fail or become blocked, resulting in a back-up of spinal fluid and pressure in the brain. Extra tubing was rolled up and placed in Anna's abdominal cavity so as she grows, the tubing will uncoil with her.

Anna's neurosurgeon has told us that as long as her shunt is working as it should, it will not be replaced.  There is no magical age or size of the child to indicate that a new shunt is necessary.

If Anna is an identical triplet, why don't her sisters have spina bifida?

First let's talk about how identical triplets happen.  A fertilized egg splits in two - forming identical twins.  One of those twins splits again.

Spina bifida occurs as the baby's spine is developing.  For some unexplained reason, Anna's spine was the only one that didn't form as it was supposed to.  I've scoured the internet and found several sets of identical twins where both have spina bifida and an equal number where only one has spina bifida.  No doctor has ever been able to explain to us (and believe me when I say that I've asked all of them) why only Anna has this condition.  There are theories, of course.

My Maternal Fetal Medicine specialist told us that abnormalities are common in identicals due to the splitting.  Maybe Anna was from that second split.  Maybe I simply didn't have enough folic acid in my body for three babies.  I still, all these years later, rack my brain trying to remember if I had taken my supplements every day.    
And then there's the genetic aspect.  There are some cases of neural tube defects running in families (about 5% or less) and researchers are still trying to understand a certain gene's relationship to spina bifida.  Interestingly enough, Ireland has the highest rate of spina bifida.  I have also discovered that Americans of Irish descent have higher occurences of spina bifida.  My father side of the family is 100% Irish.

While I was in labor with Abbey, I was a bit surprised to be told by the anesthesiologist that I have a very slight case of scoliosis.  They had some trouble placing the epidural because of it.  My c-section with the girls was a repeat.  Who knows if this is related to anything but I feel the need to note it.  It could mean something.

Why does Anna wear a pull-up and need to be cathed?

Along with everything else, Anna also has a neurogenic bladder.  This is directly related to her having spina bifida.  Basically, the nerves that would allow her to feel when she has to go to the bathroom and then control the muscles to go to the bathroom were damaged.  I don't want to go into too much detail here but there are bladder and bowel management programs and surgeries to help with these issues.  Let's put it this way - just because you have spina bifida doesn't mean that you have to wear a diaper for the rest of your life.

Anna's future

Spina bifida is a condition that cannot be fixed or repaired with surgery or cured with medicine or grown out of with age.  We are asked quite often if Anna will always need to be cathed or always have to have a shunt.  I think that this been one of the more frustrating aspects for me and Rich as Anna's parents - explaining to others that this is permanent.

The Spina Bifida Association's website states that 80% of those with SB are of "normal intelligence."  A few months ago, Anna was screened in three different areas (language, concepts and motor skills) for kindergarten.  The point of the screening was to identify, before the school year begins, kids who may be at risk.  The letter home told us that most kids score between 25-75%.   There were only 3 line results:  25-75%, 17-25% or below 17%.  Anna scored in that top rating for both language and concepts.  Way to go, Anna!

Of course, Anna's health and happiness are of the upmost importance to us.

So far, Anna's future looks bright.      

Disclaimer:  I am not a medical professional.  This was written based upon my 5+ years of experience with specialists for my daughter.


Mama Meerkat said...

As another spina bifida mom, the folic acid stuff frustrates me too. Folic acid does not "cure" or definitively "prevent" spina bifida!

There is also a fourth type of spina bifida, which I've seen categorized different depending on what source you check, lipomyelomeningocele. That is the type my daughter has, and it is very rare.

That's great that Anna is doing so well!

Just the Tip said...

^^I always have trouble knowing what exactly to call Peyton's, "issue." She had a lipomyelomeningiocele. Some hospitals refer to it as a form of SB, I really wish people could get on the same page about it, because in my opinion, we go to the SB clinic, children with the lipomyelomeningiocele can have chiari malformations too, I just feel like we are always stuck in a 'weird' gray area. I sometimes tell people tethered cord with lipoma, sometimes mild SB. I told one dr tethered cord and he was like well whats that, and I said a mild for of SB and he was like, "oh, ok." We are still struggling with the neurogenic bladder and (lack of) cathing, as always!

I have a friend in Georgia who has a little girl, she's about 6 months old now and has SB, the same kind that Anna has and she has a shunt, she has had 3 or 4 shunt revisions as well. She had someone at an ER ask her one time when the baby "got" SB. I was like Oh gosh! Did you tell them within the first 4 weeks of life!


And as for the Folic Acid issue, I think i've said this before, BUT I took FIVE mg of folic acid for A YEAR before I got pregnant with Peyton and the entire pregnancy. I have migraines so the dr wanted me on a 'seizure prophylaxis dosage,' He said having migraines could put you at risk for neural tube defects, etc.

As always, love to read about things that hit close to him, especially the way you lay them out. Hugs to you & your perfect Anna.

Michelle said...

Thanks for sharing all of your experience, I feel I am in a better position of knowledge to explain SB should anyone ask me about it.

It's great to hear that Anna is doing so well, I wish her all the best! :)

Anonymous said...

Thank you for sharing all of this. I'll admit I don't know much about SB and this was very helpful. I'd googled a couple of the things I wondered about Anna but the results were too technical and went straight over my head. This was much easier to understand. I'm glad that Anna is thriving and I know that with such a supportive family she'll continue to do great things.

Christi said...

Thanks for the information. My ob/gyn and family dr lost a child to a neural tube defect. I didn't ask either a logo questions since I knew it was personal. Now if you want more children what is your risk of it again? Anna is an amazing girl, so please do not mean anything negative. A good friend's son had the shunt put in at a year...he's 8 now. He has serious issues the first year but since then you'd never guess what he's gone through. My son had the extra fluid in utero (he was an identical twin) but I wasn't aware of any concerns except per term until somebody pointed out his headsize. Oh the things you learn as a parent.

Leslie said...

My daughter has Apraxia, which is a speech disorder. When she was diagnosed at an early age, they told us there was a chance she might not be talking by kindergarten and my heart just sank. But, 6 years later, believe me she talks. She starts 2nd grade on Monday and her speech therapist thinks she will test out of speech this year. I guess I'm telling you this because sometimes you get a diagnosis that can seem so grim and hopeless, but you never know what your child can overcome. My daughter will always sound different from other children, but I wouldn't change it for anything since it's one of the things I love about her. I also think having this disability has taught her to work hard. Through her therapy, she has really learned to focus and is a very driven little girl. I love reading your blog and seeing the smiles on your daughter's faces - they are precious. I know your Anna will grow up and overcome her obstacles. I'm so glad you did share about spina bifida because that is a disorder you don't hear much about.

Anonymous said...

One of my identical daughters, who was stillborn, had an encephalocele, which is quite similar to spina bifida except it was her brain that didn't get properly enclosed and was protruding from her skill in a sac. The folic acid thing pisses me off whenever I read it, because I was taking prenatals for months before we got pregnant, simply because we kept postponing when were going to start trying to get pregnant. And I think like you, maybe I just didn't have enough for three babies, but by the time I knew I was having triplets at 7 weeks, the damage had already been done.

Merks said...

I've been following your blog for quite some time, I remember first finding it when I saw it in your signature block on the bump's photography board. I hope this doesn't sound creepy, but I love reading about your girls - they're gorgeous - and you've inspired me to get my sewing machine back out and I've been busy crafting dresses for my oldest girl. Although I'm still trying to convince myself to take the plunge and make some princess dresses since my girls love the Disney princesses, too.

Thank you for candidly sharing your experiences. I often see my own daughters' behaviors when you describe what your girls are up to.

I also don't know much about SB and appreciate you sharing what you know to help inform the rest of us. I have a friend who's newborn was just diagnosed with CF and he's been doing the same - informing others as much as he can.

And I'm not going to tell you not to beat yourself up over the prenatal vitamins because we mothers never listen to others and are always the hardest on ourselves. As an outsider looking in, you can see the love you have for all of your girls. They are truly blessed. :)

Vesti said...

I absolutely agree with you regarding the folic acid! I also took FIVE mg for over a year before I conceived Davis. Sometimes all the folic acid in the world won't matter. And, I also hate having to repeatedly explain how SB is not something that can be cured, and the issues he has will be something we/he will always deal with. Ugh. Davis is only two, and I'm always sooooo encouraged seeing and reading about Anna an how well she's doing!

Sarah said...

Thanks, everyone, for your comments! I really truly appreciate them.

I actually hadn't heard of lipomyelomeningocele. I'm glad 2 of you mentioned it.

Christi - we decided permanently when the girls were babies that we wouldn't be having any more children. There were many reasons for that decision but it was due in part to having had so many surprises.

Hey, Merks - I remember you from the bump photography board - your girls are cuties! I hope your friend's baby is doing ok. (CF runs in Rich's family - his niece had it.)

Anonymous said...

This was a great post! Thanks for taking the time to explain all of this. Anna is beautiful and thriving. You guys appear to be doing a great job! (with all your girls!)

Anonymous said...

Huge hugs for putting up with people's stupid questions regarding her 'growing out of SB'.

Your girls are beautiful and they are very lucky to have you for their mom.

Heather said...

Thank you for sharing! Both of my children were born with a patch of hair and pit/dimple at the end of their tailbone and i'm sure you know this is a sign of SB. They both had to get checked for it and neither one has it. We have asked many doctors why both children have this patch/dimple (born 4 years apart) and if it is possible that this means there is a higher chance that a future child would be born with SB. Nobody is able to answer that question though and they all seemed puzzled. Anyway, Anna is a adorable and I love reading about your girls!

EWO said...

I've always thought Anna so courageous for defying odds left and right. When I was in high school, one of my classmates had SB. She definitely did not have it easy from a high school standpoint (she wasn't "normal" and of course anything other than the norm is NOT easy at that age) but she did so great. They never thought she would walk, and she did albeit awkwardly (though she had to leave class a few minutes early to navigate the halls before the swarms of kids), she was very bright (in honors level classes with me), and had friends. She went to college, became a teacher, did everything a "normal" person would. Anna has great things ahead of her!

Meggan said...

Great post! I've been silently following your blog for quite some time and just think your girls are so precious! I have one little boy and have wanted twins or triplets my whole life, so I was drawn to your blog when I read that you have identical triplets! I, too, think Anna's future looks darn bright! It is easy to see how much you love your girls.

Lucy said...

Hi! I'm new to your blog but love it so much I'm reading back over your old posts, so you may not even see this comment on an old post.

My younger sister does not have SB, but she does have Chiari malformation. Despite living in a city with a *huge* medical community (Houston), it took nearly 20 years for her to be diagnosed - she had corrective surgery two years ago and now has a shunt. She had to have corrective surgery for her shunt this past summer, but otherwise she is doing fairly well. It's so interesting to read about someone facing the challenges that your darling daughter faces and compare them to Darcy's challenges. Obviously, not having SB or bladder issues, she is in a much better boat, so to speak. But Anna is the first little one I've heard of to have chiari, and it's so nice to read someone who knows all the terms and knows how it works - I get so tired of having to explain what chiari is, how it has affected Dar her whole life, even though she wasn't diagnosed.... I'm sure this frustrates you too, on top of the whole "are they triplets?!" thing!

Thank you for writing such a wonderful, honest blog.