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.
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.