In early 2007, Keegan started having head pain. I can't even call them 'headaches'- the pain would just be so intense. Our family physician referred us to a local neurologist, and she had an MRI done. This showed that he had a Chiari 1 Malformation. The lower portion of his cerebellum was herniating outside of the skull through the hole that the spinal cord enters. We were told these are nothing to be concerned about, but she went ahead and referred us to a neurosurgeon to evaluate him. He told us the same thing, that they are nothing to worry about, weren't causing his symptoms, and said he must just be having migraines.
We spent the next year treating them as migraines. Not only was it not working, but he was getting worse. He'd started having difficulty swallowing to the point we no longer allowed him to eat unless someone was right beside him, and he started to lose weight. He would have these head pains several times a week, nausea, dizziness, and a host of other symptoms. He'd come home from school, fall asleep, often sleep through dinner, planned activities, or playing with friends, and then wake up to go to school again the next day. He started having pain in his chest and weakness in his hands and arms. I was starting to feel like one of 'those Moms' at the doctor's office, but I knew something wasn't right. I found a group called the American Syringomyelia and Chiari Alliance Project, and another Mom there encouraged me to get a second opinion.
We decided to see Dr. Jodi Smith at Riley. I went in prepared this time with loads of questions and research backing up that this was in fact a 'something'. I never needed to get any of that out. Dr. Smith immediately saw that he had a syrinx in his scans that had been missed by our first opinion. A syrinx (syringomyelia) is a pocket of cerebrospinal fluid that forms in the center of the spinal cord, destroying the tissue in the center of the spinal cord, and is caused by the Chiari Malformation. She had a full spine MRI done that showed how far down in his spine it went, and recommended brain surgery to make more room in that part of his skull. Dan and I were overwhelmed with what she was saying, but she just had a way of talking to us and connecting with Keegan that put us all more at ease.
He had decompression surgery in June of 2008. We'd started seeing an increase in symptoms in his sister, Kaylee. We learned that these can be genetic, and Dr. Smith had an MRI done. Kaylee had it too, Dr. Smith did her surgery in December 2008, six months after Keegan's. Keegan was pretty worried about Kaylee, having already been through this. When he came to see her in the hospital, this huge magical castle pulled up in her room. The child life specialist had the kids throwing these gel figures at the window, racing each other's down to see whose would win! It completely distracted all of us. That day, and several times since, we've found child life specialists to be PRICELESS :)! They've helped in the pre-op areas to explain in kid friendly terms whatever the procedure or surgery involves, and helped distract them with activities in their room as they are recovering. I'm sure that this is something not covered by insurance, but is very important in the healing process!
Both of the kids seemed to do well for about six months after their surgeries. Unfortunately, their symptoms returned. It was this year after spinal taps for them that we learned the kids have Intracranial Hypertension, the pressure of the spinal fluid in their brain is too high. Dr. Smith thinks this may be what caused their brain to herniate and caused the Chiari originally. Dr. Smith put an LP shunt in for Keegan in April, and Kaylee in June. Keegan went from being home all of the time, laying down nearly his entire day to being a typical kid! He was able to return full time to school, play on his baseball team--they even won their league tourney! It was fantastic to see him feeling so great, and was a direct result of Dr. Smith's continued help! Unfortunately, the L-P shunts kept breaking and needing to be fixed, so they needed a different kind of shunt.
Keegan had his L-P shunt converted to a V-P shunt first, and then Kaylee. Keegan had his last shunt revision in 9/14. We lovingly refer to Keegan as the 'guinea pig'! Kaylee had some complications after her shunt surgery. We emailed Dr. Smith at about 10:30 on a Sunday night, and she responded immediately. When she put the dressing on their shunt incisions, she cut them into shapes, Kaylee's was a heart, Keegan's was a football. What physician does these things?!?!? A Riley one! We have been blessed by fantastic nurses too! During Keegan's most recent stay, his nurse Beth on 9 West went out of her way to joke and tease with him, we've found laughing and distraction to be the best medicines of all :)!
Since our journey with all of this began, we've seen Riley specialists in pain management, neurology, endocrinology, cardiology, adolescent medicine, and rheumatology. We've experienced wonderful MRI and CT, xray, and lab technicians. Riley seems to specialize in finding those with exceptional technical skills AND who are kid/family friendly! While we wish we'd never had a need for Riley, we feel exceptionally blessed to have such wonderful physicians and health care providers! Thank you for raising important dollars to help all of those families who are in a position of needing Riley :)!!!