September 21, 2007

Spina Bifida

We have had a number of people ask us what spina bifida is, so this post is in response to those questions. The short answer is in the first paragraph. A much more technical answer follows.

Spina bifida is a birth defect that occurs early in a baby's development. As a baby forms in the womb, the vertebrae fold over the spinal cord to create the spine. Spina bifida occurs when the vertebrae do not fold together, leaving a gap in the spine where the spinal cord is exposed. Spina bifida literally means "split spine."

What causes spina bifida?
The medical community still does not fully know what causes spina bifida, but there is research showing that folic acid helps prevent neural tube defects like spina bifida. In our case, Susan was taking prenatal vitamns that include the necessary daily allowance of folic acid since we learned that she was pregnant with Fiona in Septmber 2005. So the lack of folic acid is not the only cause. There is also some genetic component to spina bifida, but to our knowledge, none of our relatives have spina bifida. The mystery of how Whitney developed spina bifida is one of our frustrations.

Types of spina bifida
There are three kinds of spina bifida. Spina bifida occulta occurs when a vertebrae does not fully close, but the spinal cord does not protrude. Spina bifida occulta apparently occurs in 3-5% of the population, many times without people knowing it. Meningocele spina bifida is much more rare, and occurs when the protective coatings of the spine (meninges) come through the opening in the spine. The most severe form of spina bifida is myelomeningocele (mī′ĕ-lō-mĕ-ning′gō-sēl) spina bifida which occurs in a little less than 1 in 1,000 births.

Myelomeningocele spina bifida occurs when a portion of the spinal cord is undeveloped, the overlying vertebrae are not fully formed, and there is no skin covering the open bones or spinal cord. This can occur anywhere along the spine, but is most common in the lower portions of the spine. The spinal cord protrudes from this opening into a neural pouch out of the baby's back. This protrusion is called a lesion. Pediatric neurosurgeons typically operate to close the lesion within 72 hours of birth. Whitney's lesion extends from the third lumbar vertebrae to the first sacral vertebrae--roughly the area of her lower back around the waistline.

Effects of spina bifida
Unfortunately, spina bifida is a complicated disorder that affects numerous body and brain functions. Here are the basics, but we are both relieved and anxious because we don't know the full effects of Whitney's spina bifida on her, and won't know for years.

Effects on lower body
The spinal cord below the lesion typically does not develop properly, so body functions controlled by the nerves at or below this level in the spine are affected. Typically, people with myelomeningocele have progressively more difficulty with bladder control, bowel function, and lower body movement based upon the location and severity of the spinal cord injury. In practical terms, we expect that Whitney will have some degree of paralysis from the waist down.

The information we have seen suggests that Whitney will likely be able to walk with full leg braces. We were initially very encouraged by this, but after attending a recent event with families with children with spina bifida, we learned that Whitney will probably need a wheelchair for her entire life. That said, we praised the Lord after seeing how mobile these kids with spina bifida are. Some of them even play tennis and hockey.

Effects on the brain
In people with spina bifida, the brain is positioned further down into the spinal column than it should be. This is called Arnold Chiari II malformation. This malformation prevents spinal fluid from circulating around the brain. Fluid becomes trapped in the ventricles in the brain, increasing the pressure on the brain. This abnormal collection of fluid is called hydrocephalus or "water on the brain." Around 85% of people with myelomeningocele spina bifida require a shunt to relieve the pressure. The shunt is surgically implanted in the head with a portion that extends into the ventricles inside the brain. The shunt drains spinal fluid into the abdominal cavity. Shunts are likely going to be one of the biggest problems for Whitney.

Effects on learning
Children with spina bifida tend to have slightly lower IQs than the general public and have some learning problems. From what we've gathered, people with spina bifida tend to have significantly better verbal than mathematical skills. Some people also have poor short term memory and poor organization skills.

We have been told that many children with spina bifida are exceptionally extroverted and have very pleasant personalities. We are praying that the Lord will bless Whitney and her family in this way.

Related medical issues
Latex allergies: children with spina bifida are at a high risk for developing allergies to natural rubber. When you consider all the surgeries that these children tend to have, you quickly see how big a problem latex allergies can be. We'll have a nice sign for Whitney's bassinet saying "NO LATEX."

Bone fractures: because people with spina bifida do not bear weight fully on their legs, their leg bones may become thin and easy to break. People with spina bifida also frequently have problems with dislocated and degenerating hips.

Seizures: about 1 in 20 people with spina bifida tend to experience seizures, compared to 1 in 100 for the general population.

Eye problems: people with spina bifida may develop lazy eye. If lazy eye is not treated early, it can become permanent.


Jamie said...

My little one has spina bifida and hydrocephalus. It's quite a journey, but it's wonderful!

Anonymous said...

I am an adult with spina bifida. I think the information you all have given to the public is informative and important in many ways.

Anonymous said...

Really good explanation of Spina bifida! I'm a 17 year old with Spina bifia and I'm always having to explain what 'it' is. It's nice to see that you have such a positive attitude about something which can be as confusing as a Donkey with a top hat on.
Ali x

Anonymous said...

Hello, I also have spina bifida and hydrocephalus. Im not sure what level of spina bifida i have but i walked with crutches and braces til the age 11. have lived on my own since 18. im now 34 and Very independent raising a beautiful little boy! :) You have beautiful children.

Nicky said...

Hello, I think that the information you have given are great. I have Spina Bifida too, but I don't have hydrocephalus. I'm 21 and studying. I think that in most cases this disability doesn't affect the brain. All people who I know with SB are perfectly normal, so I think that you don't have to be afraid :)

Dusty Staub said...

I have a 48-year old friend with spina bifida and he had his knees fused at a 90 degree angle when he was a child, so he has lived in his wheelchair all his life. If this an old procedure or is it still being performed on people with severe spina bifida?

Dusty Staub said...

How often do SB victims have their knees fused in the sitting position? 1 of a 100? 1 of 1,000? Is it an older procedure which isn't done any more?

Courtney Madenford said...

I have spina bifida I wish I would a found this a long time ago