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, 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. 😉 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) 😉 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 🙂

Happy reading 🙂

Hi Milo! :)

Hi Milo! 🙂

1. Spina Bifida (which means “split spine” 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 doesn’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 you’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 “consists of a downward displacement of the cerebellar tonsils through the foramen magnum (the opening at the base of the skull)”. Basically part of your cerebellum goes down into the top of your spinal column where it’s not supposed to be. Among other things, this stops CSF from flowing freely. Also, your cerebellum controls autonomic functions, which are things you can’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) than 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:

 CONTINUE TO PAGE 2

Advertisements
Posted in Disability Advocacy | Tagged , , , , , , , , , , , , , , , , , , , , , , , , , | 48 Comments

An Open Letter to My Mom (Mother’s Day – updated 2015) and “A Child’s Angel”

Recently I was able to get all my medical records. 90% of them anyway, from the two main hospitals at which I was seen. No, a truck didn’t show up with pallets and pallets full of records, dumping them on my front lawn! Almost. But not quite. It’s a good 300+ pages of written reports. Also, CD’s filled with x-rays and ct’s and mri’s. Interesting stuff. I see everything I went through, all the surgeries I ever had, all the complications from those surgeries, all the ER visits, all the SB clinic visits. Knee injuries, broken wrists, stomach aches. It’s all there.

And a lot crosses my mind reading those things. Mainly two things. A. How the hell am I still alive??? I’ve almost died like 4 times from complications of SB…..how the hell am I still here??? And B. My mom. My mom was a constant through it all. She’s everywhere in those records. “We talked to his mom who came with him today about” so and so procedure. “His mom feels strongly” this or that about whatever decision. “Mom thinks he is ready for” whatever surgery. She’s just everywhere. My mom. Mami.

As I read my records, almost 35+ years worth, it didn’t take very long to see what an important role my mom played. I didn’t have to get too far, didn’t have to read too much, to see just how much my mom loved her middle son and how much she fought for me in life. I saw pretty quickly that I would be nowhere and nobody if it weren’t for some of the things my mom did and said back in the day. Here’s just one example taken from those reports:

12-2-80

“His mother is aware that in September 1981 he will be ready for kindergarten. Mom visited Valverde Elementary, a pubic school, where it was recommended to her that he instead attend Boettcher School for the Disabled. After visiting Boettcher, his mother feels it was not an appropriate school for him and that he needs to go to school with his peers and be treated as a non-disabled child. She feels he would not be able to fulfill his potential if forced to attend Boettcher. We agree that it would be unfortunate if he were to develop a self-image that would not be consistent with what appears to be his intellectual ability. Mom asked if there were tests he can take to prove his capabilities to DPS. Therefore we will be administering intellectual and language testing this January which will hopefully help in mainstreaming him.”

That made this author tear up. Mom fighting the system, so that DPS didn’t just stick me where they thought I needed to be, where I wouldn’t be able to thrive and where nothing would be expected of me. And she did that in 1980. Back when there were no IEP’s, no diagnosis of learning disabilities I may or may not have had, no accommodations of any sort, no nothing. Mom kicking ass and taking names, advocating for her son in a way that was all too rare in 1980. Dad was around, doing the equally important job of making the money that paid for medical procedures and PT and hospital visits. But it was mom yelling at whoever she needed to yell at, talking to whatever doctor she needed to talk to, just doing whatever she had to do so that I could live as normal a life as possible, all while teaching me to ask for what I needed and to advocate for myself. “You talk to the doctor, you’re the patient, not me. Y dile que te duele mucho. Nomas asi hacen algo. Tell him it hurts a lot. That’s the only way they’ll do something about it.”

That was my mom. Still is. Kickin’ ass and taking names.

So……..how do I thank a person for doing all that? For being there for me literally from the second I was born with that thing she couldn’t even pronounce, let alone begin to understand. Myelo what??? Spina who?? Hydro, say it again??…..With a huge bubble on my back filled with fluid and spinal nerves, doctors telling my mom and dad that I won’t live more than a year (and here I go, pushin’ 40.) She never wavered. From the second I was born, advocating for my best interest, while teaching me to advocate for myself.

How do you thank someone for the times she stayed with you in the hospital, sometimes weeks or months at a time day and night, in high school when it seemed like every other month was another shunt surgery? AND for two shunt surgeries in summer 2013 for which she was also there?!  Along with my loving wife, who had never experienced anything like emergency shunt surgeries, my mom was there for these shunt revisions; surgeries I hadn’t needed in 17 years.  Always being strong for me, when I was done being strong after my right lung collapsed from the 3rd or 4th complication from a surgery I just had. (“Strong” in that she never cried in front of me, but years later she admitted that she’d leave the room, completely lose it, only to come back in with more strength to give. ❤ ) How do you thank someone who is always there to cheer you on, support you, love you no matter what…through SB and non SB related things like marriage….and a divorce, which by the way, is still the most difficult and painful thing I’ve ever gone through; harder than any surgery, hospital stay, or complication from SB. And she continued to be there, to see me relearn myself, rebuild myself, restart my life, even have a new marriage. How do you thank her for that, and the countless other things I have no space to write about. How do you do that? You don’t. There aren’t any words to convey how grateful I am for my mom. All I can say is……happy Mother’s Day mom. Thank you for being my mom.

Your son,
Bleeding Ink

A Child’s Angel

Once upon a time there was a child ready to be born. So one day he asked God:

“They tell me you are sending me to Earth tomorrow but how am I going to live there being so small and helpless?”

“Among the many angels, I chose one for you. She will be waiting for you and will take care of you.”

“But tell me, here in Heaven, I don’t do anything else but sing and smile, that’s enough for me to be happy.”

“Your angel will sing for you and will also smile for you every day. And you will feel your angel’s love and be happy.”

“And how am I going to be able to understand when people talk to me, if I don’t know the language that men talk?”

“Your angel will tell you the most beautiful and sweet words you will ever hear, and with much patience and care, your angel will teach you how to speak.”

“And what am I going to do when I want to talk to you?”

“Your angel will place your hands together and will teach you how to pray.”

“I’ve heard that on earth there are bad men. Who will protect me?”

“Your angel will defend you even if it means risking its life.”

“But I will always be sad because I will not see you anymore.”

“Your angel will always talk to you about me and will teach you the way for you to come back to me, even though I will always be next to you.”

At that moment there was much peace in Heaven, but voices from earth could already be heard, and the child in a hurry asked softly

“Oh God, if I am about to leave now, please tell me my angel’s name.”

“Your angel’s name is of no importance, you will call your angel, ‘Mommy’ “

~author unknown

In response to The Daily Post’s writing prompt: “Dear Mom.”

 

Posted in Disability Advocacy | Tagged , , , , , , , , , , , | 4 Comments

What I Did Wrong

What I Did Wrong
~Greg Behrendt co-author of “It’s Called a Break-up Because It’s Broken”

It’s easy to blow off a job you don’t have. It’s easy to not call friends you don’t have, blow off a workout you don’t have, or a hobby you don’t have. It’s easy to defer a dream you don’t have. But it’s hard not having a life. When the smoke began to clear and I sobered up, I realized that the focus of my life had been her and the lack of her. You have to know this about me. I love being in love. I love butterflies and the love of the first kiss. I love notes and e-mails and phone messages. I love the smells, the sounds, and the tastes. But at a different point in my life, I also liked being sad and fucked up, drinking excessively, listening to depressing indie rock, and sleeping through the day. I was either The Guy Who’s In Love or The Guy Who’s Been Crushed by Love. Both of these images gave me an identity. But when the dust of my uber-breakup began to settle, The Guy Without Any Plans and that had to change. This is when I had the most amazing thought: “I get to start over.” I know it doesn’t sound amazing, let alone revolutionary, but that is exactly what it was. It’s weird how you can hear certain words through out your life and they bounce right off you. But when the reality of that situation hits you, when all you want is another chance, when you hate how you’ve been living and suddenly an option appears, those very words can be a life preserver. And in the blink of an eye, the world seemed full of possibilities. How it happened I don’t know. But ever since, I’d begun taking care of myself by getting out of bed, showering regularly, and leaving the house, I became increasingly open to new ideas because I began to truly feel and recognize that the way I’d been living wasn’t doing it for me. Gosh, it only took me eight months to figure out that the first step was getting out of bed every day.

Posted in Parables, Stories and Excerpts I Wish I Had Written | Tagged , , , , , , , , , | 4 Comments