**For those of you who may not know, CJ is my husband**
CJ was sick at the age of 14 and ended up spending over a year in the hospital and had multiple surgeries. In the end they diagnosed him with a disease called cystic angiomatosis. Which as they were describing it was a cousin to cystic fibrosis. His ribs were creating a fluid that was eating away at his lungs causing them to continuously collapse. His surgeries were to attach his lungs to his ribs to keep them from collapsing and to close the space where the fluid was forming. He was told he would not live to see 30 (he just turned 35 this March). During this time he was roughly the 100th case EVER diagnosed with this disease so you can imagine how little information there was.
When CJ and I started dating in March of 2004 he was upfront about his illness it was not something that had been an issue for a couple of years. It was the least of my concerns.
Some of the medications that they had him on when he was sick caused side effects many years later. One of those being migraines. In January of 2010 the migraines got worse. To the point of it was one constant migraine and nothing helped it. Off to the doctor he went who wanted to refer him to a neurologist but he needed a primary care physician for that referral with the insurance that we had. So we went to a PCP who decided to do a CT scan before sending us anywhere. That CT scan showed a tumor on his Pituitary gland. So the doctor no longer wanted to send us to a neurologist but to a neurosurgeon instead. Now tumors on the pituitary are not uncommon and they are generally easy to remove (they go thru the nasal cavity). We met with a wonderful neurosurgeon through Duke Medical Center. He looked at the CT and said yes there is something there BUT I don't think that is what is causing your issues. That really isn't what you want to here when someone is looking at a scan of your husband's head!!! He ordered a Flow Study of CJ's brain. He was concerned about an area towards the base of his skull where it looked like the brain was herniated through the opening of the skull. The flow study confirmed the doctor's thoughts and scheduled us for surgery. And we received the official diagnosis of Chiari Malformation I. Unfortunately we found out of this at the beginning of April and couldn't be scheduled until June 2. Some of it was due to the doctor's schedule and some was due to ours.
June 2nd approached so quickly and I had to watch my husband be taken away into a major brain surgery. I am so thankful for my wonderful parents. My mom came and sat with me while my dad stayed home with both boys (Deqlan was 5 and Hayden was 8 months-and decided to start walking while CJ was in the hospital!!) Thankfully we did the surgery when we did. When the surgeon came to talk to me he was surprised that CJ's migraines really hadn't gotten any worse because the herniation had. The first night was spent in the ICU where I wasn't allowed to spend the night with him. And then he had to spend one night up on the floor and then we were discharged. It felt good to have him home but I was terrified something would happen. And for the next 18 months nothing did happen.
I say 18 months but in August of 2011 CJ started having more frequent migraines again so we went to the PCP and they started CJ on a nightly medicine and gave him something to take at the onset of his migraines. Those seemed to work until December.
Last December things got crazy. Three days before Christmas CJ had been up all night with a migraine. He just couldn't get it to go away with any of the medicines he was taking. Finally about 6am the next day (Dec. 23rd) it went away-we have no clue what finally made it go away. He went out to do his Christmas shopping for me and then we spent the rest of the day doing things around the house to get ready for the holidays (we spend Christmas Eve and Christmas Day at my parents with the exception of first thing Christmas morning). We went out to get pizza for dinner and then came home so I could get some appetizers put together. CJ went upstairs to wrap my presents and I realized he had been in there for awhile. I went to check on him and all of the lights were off in our room (tell tale sign he has a migraine) and heard the shower running. When I walked in the bathroom he was on his hands and knees in the shower in tears because the pain had gotten so bad. I just looked at him and asked if we needed to go the ER and he said yes. I knew it was bad because he HATES being at the hospital.
I called my parents to come stay with the boys and then my mom decided it would be easier for the boys to stay with them (they only live 4 miles down the road). So I packed some stuff up for the boys and threw some clothes on me so we were ready to go when my dad got here. It was after 9:30 at this point. We went to a smaller emergency room so we would be seen faster-they took us right back thankfully. It took almost 2 1/2 hours before they found the right combination of pain killers and nausea meds for CJ to actually be comfortable. I think that was the worse thing of all watching him writhe in pain. After running a CT and not seeing anything major on it the doctor decided to do a spinal tap on CJ-they needed to rule out any brain bleeds. The doctor tried three times and could not get any fluid off of his spine-she was so surprised she knew she was in the right spot. Because it was about 2am on Christmas Eve she decided to transfer us to the big hospital where they could do the spinal tap with an X-Ray. We finally got to the big hospital about 6am. His spinal tap ended up taking 3 hours. While I sat in this little curtain in the emergency room with no tv, an uncomfortable chair, and no outlet to plug my dying cell phone in. They finally brought him back about 9:30 and we didn't see the doctor til around 10. She said there was some blood in the tap so she wanted to run another test to make sure there wasn't a bleed and that it was just a traumatic tap. They ended up having a hard time getting fluid off of him as well. They finally determined that there was no bleed and that he was just having a severe migraine-mind you that nothing they had given him had made it go away. We finally got discharged about 12:00 Christmas Eve. My poor mom was trying to cook dinner while watching both of the boys!! Because of the spinal taps he was ordered to lay flat for 24 hours which meant we were staying at my parents overnight. Once we got discharged we stopped to grab some lunch and then headed to my moms (who had already got the couch ready for CJ). Once I had him situated I headed to our house to pack up clothes for us and all of our presents and stockings-well my brother had to come by with his SUV to load up most of the presents :-)
Because all of this happened right before the holidays it was very difficult to get into any doctors quickly. We ended up going to a neurologist who did a 3 day IV infusion treatment (that was ridiculously expensive) & that didn't work. Two weeks later I was able to get him into his neurosurgeon-this whole time we were convinced that it was the Chiari & the surgery had failed and he would need another surgery. CT's & MRI's showed that wasn't necessarily the case. The neurosurgeon decided we needed another spinal tap to get a pressure reading of CJ's spinal fluid. The day of the tap the doctor could not get ANY fluid off which gave him a zero reading. The issue with this was that he had been put on a medication to LOWER his pressure-when he didn't have any!!! The neurosurgeon eventually referred us to a neuroradiologist (the best in the world). Who was going to do blood patches by myleogram (basically a fancy X-Ray). She discovered that CJ's bone disease had spread to his back and was the issue for all of his pain. The disease, which we now know as Gorham's Disease, creates cysts up and down his spine that suck up his spinal fluid. This disease is what caused his Chiari. There is no cure for the disease. He underwent 5 full weeks of radiation treatments and we should know in November *hopefully* if it worked. We don't know how long he'll be able to work/walk. It is very possible that he will need back surgery in the near future to replace vertebrae that are deteriorating due to the disease.
I don't write this to make you feel sorry for all that we've been through. I write this so you know when I write complaining about him being in pain for the umteenth time this has been an incredibly long and hard process. We still have a lot of unanswered questions and the fact that we don't know how much longer our lives will be normaly