Calvin’s body didn’t develop the same as everyone else’s.
Around the 4th day after conception, when his spine and brain were developing, they didn’t close up quite like they should have.
He had a hole in his back (myelomeningocele or MMC) down around his 2nd lumbar spine.
Because of the hole, cerebral spinal fluid poured out when it should have been circulated down and back up to his brain.
This caused the back of his brain to herniate down into his spinal column.
That malformation is called an Arnold Chiari type 2.
The back of the brain acts like a plug and doesn’t allow for the spinal fluid to flow like it’s supposed to.
So fluid gets stuck in the top of the brain, and causes enlarged ventricles or hydrocephalus.
With the hydrocephalus, pressure in the brain is increased.
Sometimes there are symptoms associated with it, sometimes there aren’t.
When a kiddo becomes symptomatic, a shunt is placed to drain the excess fluid.
There are 2 options for repairing the MMC (hole in the spine)- prenatal or postnatal.
We qualified for the prenatal repair and had open fetal surgery to patch up the hole.
Having the MMC repaired prenatally means much better outcomes for these kiddos.
When the hole is repaired before birth the brain has time to heal and re-route the fluid while the baby is still in the womb. Most often the Arnold Chiari herniation completely reverses and disappears, the plug is removed, fluid is able to circulate like it’s supposed to, and there is no hydrocephalus.
The prenatal repair also allows for protection of the spine against the amniotic fluid, which causes further nerve damage.
With the postnatal surgery 85% of kiddos end up needing a shunt.
With prenatal surgery that number drops to 40%.
The only truly dangerous thing with this diagnosis is if the Arnold Chiari Malformation becomes symptomatic.
With the fetal surgery this is very, very rare, as the Chiari typically reverses and goes away.
It is even very rare with the postnatal surgery.
Symptoms include headaches, dizziness, nausea, difficulty swallowing, impaired gag reflex, etc.
Sometimes, but not very often, the Arnold Chiari Malformation can cause breathing problems and death.
When considering the fetal surgery we looked at risk of death.
In the MOMs trial, the study we used to make our decision, 2 babies died in each category.
In the prenatal group the deaths were from prematurity and in the postnatal group they were from complications with the Arnold Chiari.
Now for Calvin –
His Chiari did not reverse like it should have.
We were disappointed because that meant an increased possibility for a shunt, but we never, ever considered anything more serious because his Chiari wasn’t that bad to begin with.
His ventricles also didn’t shrink like they sometimes do, but they also didn’t get bigger, which was good.
The first week after he was born, his ventricles and head circumference remained stable.
We were excited and hopeful that he wouldn’t need a shunt.
After that first week his head started to grow.
At first we hoped he was just going through a growth spurt, but as we watched it get bigger and bigger we began to figure a shunt was probably inevitable.
Before we left Denver our neurosurgeon told us there was no reason to shunt – no matter how big his head got – until he was symptomatic, and the only symptom to REALLY worry about was stridor, which is a high pitched breathing sound that indicates his throat closing.
So we went home hopeful but cautious.
When we had been home for about a week and a half Calvin started spitting up – a lot.
I asked around and most people said it was normal for a baby to spit up a lot.
Calvin hadn’t spit up once in his whole first month of life, so I was a little skeptical, but decided to wait until his next Dr.s appointment to ask about it.
He also was looking downward a lot, which we attributed to premature eye muscles.
2 weeks after we got home was Calvin’s first follow up with a neurosurgeon in Las Vegas.
She looked at Calvin for about 30 seconds and sent us to the hospital across the street for an emergency shunt the next morning.
Turns out his spitting up was actually throwing up from increased pressure in his brain, and his looking down was also from the pressure.
We went home a little disappointed, but also relieved.
Now we wouldn’t have to worry about watching for symptoms, about his head growing, or about the pressure in his brain.
We never expected his Chiari to be a problem.
When Cal stopped breathing on Wednesday the first test they did was an MRI to check his shunt.
His shunt appeared to be working, and symptoms pointed to a symptomatic Chiari, but the MRI wasn’t showing that there was increased pressure.
We decided to wait a week to see if things in his brain evened out and he started breathing again.
At this point we are fairly certain the problem is the Arnold Chiari.
We are going to take him off sedation on Wednesday to see how he does, but most likely we will be going back to the OR on Friday for a decompression surgery.
During this surgery they remove part of his skull and parts of the top 2 vertebrae.
It sounds more dramatic than it is.
He will grow the bones back, apparently.
Like Harry Potter.
I’ve talked to 2 other women whose babies have had this next surgery.
One of them said after the surgery, her baby got better so fast she hardly had time to think!
We were initially hoping that he would get better and not need this surgery, but at this point I want to get it over with and get my baby back!!!
Watching him lay there with the breathing tube, watching (but not hearing) him cry, worrying every second about his breathing, bradys (low heart rate), and de-sats (low oxygen in blood), not to mention being-in-the-hospital problems like pressure sores, IV infiltration, infections, mean nurses, etc…
And then the guilt – oh the guilt!
Not being able to feed him when he seems hungry, not being able to hold him and snuggle him when he’s sad, sitting by his side at all times in case he wakes up and is scared, being terrified to leave him…
…that all this is somehow my fault.
At this point I won’t say there isn’t anything worse –
I’ve learned my lesson, there is always something worse-
but it’s pretty freaking hard.