Earlier this week I was putting sunscreen, swimsuits, and shorts into a suitcase. As I was putting in the final selections I realized I was attempting to be excited to escape this Midwest cold and snow to replace it with sun and beach, however there was a nagging ache in my gut. I sat in front of that suitcase and began to cry. Cry because I knew Jack was not feeling good, and I knew I could not leave the country with my fragile son not feeling well. So my gut led us to doctors ruling out this, ruling out that, but nothing was clear, its never clear with MPS. It’s always a guessing game and the best resources are parents with experience so I called my MPS moms brainstorming the issues and possibilities. Could it be… What tests… Should I try… this is the language we talk, these are the conversations we have. Most people seek medical professionals, we seek each other to help guide the medical professionals. This gut kept going back to his reflux getting worse or with his increased lethargy a possible shunt malfunction. By Thursday Jack no longer was Jack, sleeping on/off and extremely irritable. I knew at that point the shunt was most likely the culprit.
On Friday morning we ended up in the ER with my lists of symptoms and the plan of action that needed to occur. The team was great, we had X-rays and MRIs done first. The X-rays showed an intact shunt but the MRI showed very enlarged ventricles in the brain. The shunt was not working. Thankfully our neurosurgeon was on call and in the OR so he came over and read the scans and he felt quite confident the shunt needed to be looked at. And immediately. So after a 10 hour day in the ER, Juliet as entertainment, Jack was sent into the OR for a shunt revision. It ended up being the catheter, it was blocked by scar and brain tissue so it was not able to do its job of draining the cerebral spinal fluid(csf). The csf was building up causing a lot of pressure in his brain. The poor kid was having painful headaches for who knows how long? Anyhow the catheter was replaced.
Jack recovered from anesthesia quite well and was admitted to in patient. He had a good night sleep on pain meds however we tried to take off morphine and he was in considerable amount of pain so now he is back on it. He also had another MRI yesterday making sure placement was all good. As of last night Jack is still not able to eat and get up, we are praying this him adjusting to normal pressure. And praying its not the shunt again. Shunts in MPS patients are very tricky, with GAG build up to the abnormal brains – this makes the already commonly faulty shunt more so.
So maybe our suitcases are not heading to the airport this week, but we have learned that dealing with MPS makes vacations as rare as the disease itself. Moving forward, we now focus on packing our bags and going home with a happy, healthy boy! Soon!