Spina Bifida: A Working Definition

Author Note: I originally wrote this piece back in 2013 for one of my nonfiction writing courses. I decided to post it after coming across it again recently. I think it stands up pretty well, but I might be a tad biased. Please see the End Notes for added commentary, and as always, thanks for reading. Piece now also appears on Medium.com.

Spina Bifida Myelomeningocele. Hydrocephalus. If I had to guess, I’d say many of you are unfamiliar with one or both of these words. In that case allow me to give you a crash course in the definitions of these words and in that way add, in some small way, to your knowledge base.[1]

Spina Bifida-from the Latin meaning “open spine”, Spina Bifida is a neural tube defect that causes the spinal column to not properly close in utero. The resulting opening, or lesion, can and often does result in some form of paralysis and loss of function in certain areas of the body. Closing of the opening is the first operation a child with Spina Bifida will undergo, often hours after birth. The severity of effects is determined by the location of the lesion (i.e. the higher up on the spine the defect occurs, the greater the chance of paralysis and other complications). [2

On average, fifteen hundred babies are born with Spina Bifida in the U.S. each year. [3] It is classified as the most prevalent birth defect that is compatible with living into adulthood.[4] Most of the medical research that has been done on Spina Bifida took place within the last thirty to forty years. Before that time, a baby born with Spina Bifida had little chance of survival.[5]

Today medical advancements, including a surgery to close the Spina Bifida lesion in utero, have greatly improved the life expectancy and quality of life of those living with this disability.[6] However the medical community is still unfamiliar in many ways with the needs of adults with disabilities, as the previous generation of babies born with the defect have had the greatest number yet to grow into adulthood. Finding appropriate medical care is one of the greatest barriers to beset this group of individuals. [7]

Another factor in the impact of the defect has to do with the type of defect that occurs. There are three types of Spina Bifida that are most commonly accessed. The first, Occulta, is the least common and also the least severe[8]. This form of Spina Bifida is characterized by a lesion that affects the outermost areas of the spinal column and is accompanied by limited paralysis. People with this form of Spina Bifida will likely have little to no impaired function and may have no outward physical signs of having Spina Bifida.[9]

The second type of Spina Bifida, also the second most common and severe is Spina Bifida Meningocele. In this form the lesion affects a deeper portion of the spinal column involving exposure of nerves. Subsequently there is a greater incidence of paralysis associated with Meningocele. Issues involving bowel and bladder are common as is the need for some type of mobility aid, either crutches or a wheelchair.[10]

Spina Bifida Myelomeningocele is the most common and also the most severe form of Spina Bifida. In this form nerves in the center of  the spinal column are completely exposed. Accompanying this form are higher levels of extreme paralysis, and a greatly increased incidence of problems related to the bowel and bladder.[11]

It should be noted that no two cases of Spina Bifida are identical, even within the same form. It is entirely possible for a person with Meningocele to be as physically healthy as someone born with Occulta.[12] There is no way of knowing before hand what medical challenges a person born with Spina Bifida will encounter. However all forms of Spina Bifida are likely to be accompanied by maladies such as neurogenic bowel and bladder and clubbed feet. Hydrocephalus is also a common secondary diagnosis connected to Spina Bifida.[13]

Hydrocephalus is defined as a build up of cerebral spinal fluid in the ventricles of the brain. In a brain unaffected by Hydrocephalus the fluid naturally cycles through the blood stream relieving pressure on the brain. For a person affected by Hydrocephalus this natural cycle does not occur creating a build up of fluid and pressure.[14] This is relieved by the placing of a mechanism called a shunt. The shunt is a small pump attached to tubing that runs into one of several locations including the peritoneal cavity or heart, depending on the shunt type.[15] The pump filters the spinal fluid through the blood stream there by relieving the pressure.

The shelf life of a shunt is around eight to nine years, after which time it will need to be replaced. The replacement procedure is usually simple, involving a minimally invasive brain operation and a short hospital stay after.[16]

Apart from the initial closing of the lesion and placement of the shunt, it cannot be determined with any certainty how many other operations a child with Spina Bifida may have to undergo.[17] Other types of operations may be necessary to correct issues related to clubbed feet and problems related to neurogenic bowel and bladder.  However with the correct medical care, it is reasonable to assume that a person living with any type of Spina Bifida can expect to live a perfectly healthy life.[18]

 

[1]  I had hoped that this piece would be more informative on the topic of Spina Bifida and less of a rant about my life. I do not intend to patronize by sharing this knowledge. I only hope to share and define something that has shaped and defined much of who I am as a person.  Also, when I said that many of you would most likely not be familiar with the terms above, I was making the assumption that neither you or anyone you know have been diagnosed with either of these conditions.

[2] If you’re still with me, kudos. I never realize until I see these words written out how intense this definition might be to someone who’s not familiar with it. It’s just something I’ve lived with all my life, probably the thing that sets me apart the most. Because of that I’ve come to  think of the diagnosis as a personality quirk or a individual quality that makes me unique.

[3] Fifteen hundred babies. That’s a mental image I still can’t conjure. But that statistic always reminds me of this poster for SB awareness I saw once. It had a baby sitting naked on the beach with his back to the camera so you could see his lesion scar. He had one arm raised up in the air and below the picture it said “Less than one percent of babies are born with Spina Bifida. He’s proud to admit he defied the odds.” That is without a doubt one of my favorite awareness posters.

[4] Every time I read that sentence my stomach flips over. Something about the phrase “compatible with living into adulthood” I guess.

[5]  This is not to say that adults with SB don’t exist. I know a few. But being born when I was afforded me a better chance of joining their ranks.

[6]  The operation I’m talking about here was introduced in the early nineties and is becoming a more increasingly offered method of treatment. Although no studies have been released yet, it is believed that early closure of the lesion alleviates much of the paralysis that would otherwise be present.

[7] Unfamiliar is being kind. The lack of knowledge I have encountered on some occasions is staggering. But in those instances I try my best to smile and inform.

[8] SB Fun Fact: Ever heard of John “Cougar” Melloncamp? He has this form of SB!

[9] I have often wondered if his SB was the reason his dance moves on stage were so stilted at times.

[10] This and the next form I describe are the two that are the most similar symptom-wise.

[11](Mylo-my-ninja-seal) This is the form of Spina Bifida that I have. Everything I describe as being connected to Mylomeningocele I have dealt with in one way or another.

[12] This is something I have learned over the last couple years. One thing that I have come to realize is that even with everything I have gone through because of my disability, I am fortunate.

[13] A common terminology for hydrocephalus is water on the brain.

[14] This is something else that I recently learned. I had always assumed that my blood was diluted (i.e. my blood type was B-/CSF). This is in fact not the case.

[15] I have had two types of shunts: Ventriculoperitoneal (VP) and Ventriculoatrial (VA). I currently have a VA shunt.

[16] It recently occurred to me that I am closing in on year nine and have not seen my neurologist since this shunt was placed. Gotta put that on my to-do list.

[17] I have had around 50 surgeries spanning week one of life to the age of 16. I count getting my wisdom teeth out Christmas of junior year solely because I was completely put under.

[18] Definitely one of my goals. I’ve been lucky so far and I can only hope my luck continues. I’ve put a lot of life in these 22 years but I’m nowhere near finished.

 End Notes: Bringing this up to present day, I am now 28 (soon to be 29). I am happy to report that I have done a lot more living in the last almost 7 years. I got married, moved back to my hometown and have held a few jobs. And yes for those concerned, I’ve seen a neurologist. I actually have a nifty new proximal (inflow) catheter after a malfunction 3 years ago this December. I’m doing pretty well if I do say so myself and I look forward to continuing to redefine Spina Bifida.

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