What do you do? Do you fall to the floor and throw a tantrum or do you raise your chin, look that SOB in the eyes and reply, “Bring it.”
Last week was supposed to be my vacation. Five days away from my full time job to spend with my kid, giving my husband a break so he could hunker down before the start of the school year. It was also to be my endurance block, where I’d spend 2+ hours on my bike every day. By Wednesday, I was well on my way to 500 miles, having logged 260, both indoors and out.
And then Thursday morning happened.
You see, when my son was 13 months old, he was diagnosed with Dandy-Walker Syndrome , agenesis of the corpus callosum and hydrocephalus. With these brain defects, my now 13 year old is developmentally and emotionally 5 years old. He has trouble with balance, hand-eye coordination and speech. Children with these issues are affected in different ways. Some don’t survive birth, die shortly thereafter or can live most of their life without ever knowing they have DWS until they have a CT scan.
There is no treatment for DWS or ACC and hydrocephalus is managed with a shunt. Basically a plastic catheter is implanted into the ventricle that doesn’t drain spinal fluid and a small rubber unit that has a vacuum bubble draws out the fluid and it is reabsorbed into the body through the abdominal cavity. Like all plumbing, the plastic tubing or rubber drain can become clogged with ‘stuff’ and no longer drains correctly. If the fluid is not drained, the brain swells and could result in death. The only way to diagnose a ‘shunt malfunction’ is a CT scan.
This treatment has not changed since it was first developed in the 1950’s.
Our son is also kind, affectionate and has a smile that lights up the room. Anyone who meets him is immediately taken in with his good nature and sense of humor. His laugh is infectious. He loves to help out and brings his pan and broom everywhere with hopes to be a master of the custodial arts.
When a child has had a shunt in their brain to treat hydrocephalus for as long as my son has (not the same one, mind you – he’s had 11 surgeries to replace them), it’s a foreign object in a place not meant to have a foreign object. For that reason, he is now affected by migraines, or so we thought. Think.
Through the summer, we and our neurologist thought he was having migraines. We followed the protocol to treat them and for the first time ever, we were drugging our kid with a medication for seizures, which he does not have (thank god). However, within 3 weeks, the treatment didn’t seem to be working. His behavior continued to deteriorate along with his balance and the headaches would wake him up at night.
Last week, he spent most of his time on the couch, exhausted. He refused to eat. There were a few hours each day where he felt good enough to venture out but he would request breaks to sit down and rest or put his head on my shoulder. He left his pan and broom at home. We were worried. Very worried. He wasn’t following the same list of symptoms for a shunt malfunction he had in the past so it didn’t come to mind until Thursday morning, when he woke up crying that his head hurt and promptly vomited like Linda Blair in The Exorcist.
By noon, we were on Rt. 76 heading for Philadelphia. Traffic. Navigating around U of Penn. Emergency Department. Trying to convince him and his irrational fear of the CT machine that they were only taking a picture of his head. X-ray of the shunt line. Hours of waiting to review the CT films.
By 7pm, the neurosurgeon resident was in the room showing us the comparison CT to that day’s film. He pointed out how the catheter from the vacuum unit to the ventricle had pulled out and the ventricle itself was 5mm wider than the baseline scan. We were stunned.
“Did he have a growth spurt?” the doctor asked.
“Yes, he grew 4 inches in the spring.”
“Well, there you go. Usually when that happens, the line breaks in the chest or in the neck because of calcification. With him, it pulled out. We’ve tagged him on the surgery list for tomorrow. He’ll be in intensive care until then because you know, he might have seizures. Have a good night!”
My husband and I just stared at each other, knowing that there would be no rest for the next 3 days. But there will be pizza, lots of pizza. And gastritis, for me. Joy.
It is now Monday and my son is in the other room, laughing hysterically at something on his iPad, a 5 inch incision with bright blue stitches on his head.
When asked how he feels, his reply is, “I feel great! When can I go to school because I want to show off my new backpack.”
Tomorrow, buddy. And Mom gets a day by herself.
ps. Oddly enough, my 8/25 Facebook posted that in 2009, we were at Children’s Hospital of Philadelphia doing the same exact thing.