Journal entry by Kristen Kirkey —
Hello my Caringbridge dwellers! Or at least the followers not on facebook. :) I stopped posting here due to the password protection and getting very few readers. I started posting on my facebook page due to this. However I may have to start it back up for reasons I'll say in a bit. I'll try and do the play by play on what has happened since last May. WOW. Almost a year! Or the ones on FB you don't have to read. lol
Ok. I guess I left you hanging when I had my wheelchair assessment with Stephanie (the PT). Well my Seating guy did come over and do the in home assessment for my chair. And I did test drive a Smart Drive power assist. As I said I wanted to do we tried going up our hill. Well it was impossible to get up! I did not have the strength to propel at all. And coming down was a whole new issue. After this we went inside and talked Power wheelchairs. Lol. He really felt this was the best route for me to go. To be honest I think he did even before this. We went with a rehab chair with tilt and recline so I can have the option to take pressure off my joints and if my POTS gets bad. He did an assessment and my doors are all wide enough to get through if and when we make it accessible (which would be super easy to do). We of course had to do preauthorization and honestly thought we'd have to do appeals aswell. Notice how I said "thought"? Lol Well after only 3 months insurance APPROVED it on the first try! I'm now the proud owner of a power chair!
Onto the next little mishap with that. Well the whole idea of getting the powerchair in the first place was so I could be more independent. And well I can barely walk now. Lol. Anyway. My plan was to get started with Paratransit. Even just to the train station and then I could go from there since it's all accessible. Well apparently since I don't live within 3/4's of a mile from a stop I don't qualify. Ya I thought it was unbelievably ridiculous too! Anyway, so there my chair sits in the garage. The furthest I can go is down to the mailbox. Which I couldn't do before so I guess it's something. LOL We do have a carrying rack for dads truck though for when I'm out with them and if I have to be driven somewhere from dad I can take it. I can drive it pretty well! We have to still do adjustments because the arm rests are too low and we have to raise the foot plates up a bit because I'm bottoming when I go over a curb but other than that it's PERFECT! PURPLE of course! :)
Let's back up to June. I woke up Father's Day with the most excruciating pain I've ever felt. Head to toe! The night before it was dysautonomia madness. Then the vomiting started. I usually don't go to the ER unless I'm desperate or worried. Well this time I was desperate for pain relief! Mom and dad took me to IMC ER. I was very lucky that I got a very good attending and excellent nurses. I've actually had pretty good luck with IMC nurses and doctors. It helps that all my doctors are there and my file is likely huge. LOL
Anyway. My IV had infiltrated the night before so they had to start another line. It wasn't quick or painless due to me being a hard stick. Lol They started IV fluids and did a zofran push. They also gave me Oxycodone through my J tube. They did labs of course. When they came back my lactic acid was elevated. Sooo they came in with a another bag of saline. They couldn't let me leave until it was down. They also did a t series and CT which all came back "normal" (more about that later lol). Before they were going to discharge me I spiked a fever of 101. They weren't going to do an LP but after I spiked the fever they did. I was finally able to go home. My poor mamma was waiting with me.
Well with having EDS we are prone to leaking due to our ultra thin duras. You guessed it. My headache lasted longer than the usual 2 days and I was in a ton of pain with low pressure headaches. So I went into the ER at Alta View (a smaller hospital). Well due to not being able to do a blood patch under fluoroscopy they couldn't help me. But the attending was really helpful and booked one for the following day. Oh in between this I got a letter from the ER stating I had a UTI and to get some antibiotics. :p lol Hello! lol
After this we decided to make an appointment with Dr. Bolognese. I sent him my CT and LP (which was useless since they didn't do an opening pressure). I made an appointment for September. Thinking it was just a follow up and nothing was wrong (denial much? lol) he ended up throwing me a curve ball. lol. However just literally 2 weeks before my appointment my nystagmus and headaches started to get worse. Well he sat down and said well - yes there IS something wrong. My shunt is failing! I virtually have no ventricles. HOW did the ER miss this?! He said my shunt definitely needed to be replaced. This actually explains a lot of my low pressure symptoms! My head has been a mess and progressively getting worse. I likely didn't notice it because I do 2 liters a day. He also diagnosed me Eagles Syndrome. Or an elongated styloid process. This is causing a lot of my neck pain which I thought was my rods. I guess not! lol According to him mine are "very impressive"! He did order an LP before we even talked surgery. I had it and my opening pressure was 9.5. I'll explain below.
Speaking of which. Let's rewind a bit. I've been doing home fluids since last April. I started with a midline then when that crapped out on me we went back to PIV's. Oh sorry. We attempted a PICC line first but that was a fail due to my veins being too stretchy. lol. Well you can only poke veins so much before they develop scar tissue, not to mention the amount of blown veins I have. lol. After careful consideration Merin agreed to place a central line. I had my Hickman placed in August and it's been WONDERFUL!
Back in July I saw a geneticist who clinically diagnosed my Mitochondrial Disease. I then saw a neuromuscular doctor who did an EMG on me. When it was positive for myopathy he gave the "ok" to proceeded with the muscle biopsy. I haven't booked it due to everything else. lol. But I will after everything else. lol. The crappy part is I have to be off my supplements (mito cocktail) which I'm not looking forward to! lol. Sadly my dietician/NP has been more helpful with treating it.
Anyway. Haha I know I'm all over the place! lol. I had a follow up skype appointment with Dr. B in January. We went over my scans again. That's when he said my ventricles are "sucked dry". We also went over the STING study check list. And my other symptoms of leg tingling and frequent UTI's.
After my follow up appointment with him (oy my memory is bad lol). Oh ya - I called neurosurgery here and asked for them to look at my scans. Well according to THEM "I don't need a shunt replacement at this time". lol So I also sent my scans to a doctor in NV. He emailed me back and said he agrees I do need a shunt replacement. So that's 2/3 doctors. lol. Well after careful consideration we decided to go back to Dr. B. So I contacted insurance to proceed with the PPO Waiver/Gap Exception. Well it took 2 months and working with many people but I finally got Dr. B covered in network! It's such a relief!
The next chore was getting my testing done. So I made an appointment with my PCP to get that done. I had my Echo and EKG done Thursday. And a impromptu tube change due to me oh I don't know PULLING IT OUT! LOL Oh btw my surgery is April 27th! lol Ya cutting it short. LOL My EKG isn't even back yet. So I'm hoping they will get before my pre op on Monday! It'll be a lot of prep for pre op as well due to the mito. I've been doing weekly IV Carnitine and it's been helping so much! We put it in a bag of D5 and I get the use of my legs back for at least 5 days! AND my gut is kick starting a bit! I'm eating little bits! I'm still using my manual chair more as well which could be helping also.
As far as non medical stuff. I've been busy with my card business and I've branched out into Cricut stuff! I've made moms Mother's Day gift and I'll be making dads as well. Ya know I have to make up for last year! lol
Well this is the reason I've fired up this bad boy again. Im surprised I remembered everything. lol. Although It took a few times of writing off and on! Lol. I'm sure more has happened but this is the continuous stuff. lol. I'll have dad update when I'm in the hospital if I can't. So stay tuned and make sure you have email alerts! :) Please keep me in your thoughts and prayers. It's an "easy" surgery but with me you never know! lol. We leave for NY tomorrow and my pre op is Monday. Surgery is Thursday! Thank you! 😘. I've posted some pictures as well. I'll put the hospital address in the main page where my story is. :)
💜 Kristen
Ok. I guess I left you hanging when I had my wheelchair assessment with Stephanie (the PT). Well my Seating guy did come over and do the in home assessment for my chair. And I did test drive a Smart Drive power assist. As I said I wanted to do we tried going up our hill. Well it was impossible to get up! I did not have the strength to propel at all. And coming down was a whole new issue. After this we went inside and talked Power wheelchairs. Lol. He really felt this was the best route for me to go. To be honest I think he did even before this. We went with a rehab chair with tilt and recline so I can have the option to take pressure off my joints and if my POTS gets bad. He did an assessment and my doors are all wide enough to get through if and when we make it accessible (which would be super easy to do). We of course had to do preauthorization and honestly thought we'd have to do appeals aswell. Notice how I said "thought"? Lol Well after only 3 months insurance APPROVED it on the first try! I'm now the proud owner of a power chair!
Onto the next little mishap with that. Well the whole idea of getting the powerchair in the first place was so I could be more independent. And well I can barely walk now. Lol. Anyway. My plan was to get started with Paratransit. Even just to the train station and then I could go from there since it's all accessible. Well apparently since I don't live within 3/4's of a mile from a stop I don't qualify. Ya I thought it was unbelievably ridiculous too! Anyway, so there my chair sits in the garage. The furthest I can go is down to the mailbox. Which I couldn't do before so I guess it's something. LOL We do have a carrying rack for dads truck though for when I'm out with them and if I have to be driven somewhere from dad I can take it. I can drive it pretty well! We have to still do adjustments because the arm rests are too low and we have to raise the foot plates up a bit because I'm bottoming when I go over a curb but other than that it's PERFECT! PURPLE of course! :)
Let's back up to June. I woke up Father's Day with the most excruciating pain I've ever felt. Head to toe! The night before it was dysautonomia madness. Then the vomiting started. I usually don't go to the ER unless I'm desperate or worried. Well this time I was desperate for pain relief! Mom and dad took me to IMC ER. I was very lucky that I got a very good attending and excellent nurses. I've actually had pretty good luck with IMC nurses and doctors. It helps that all my doctors are there and my file is likely huge. LOL
Anyway. My IV had infiltrated the night before so they had to start another line. It wasn't quick or painless due to me being a hard stick. Lol They started IV fluids and did a zofran push. They also gave me Oxycodone through my J tube. They did labs of course. When they came back my lactic acid was elevated. Sooo they came in with a another bag of saline. They couldn't let me leave until it was down. They also did a t series and CT which all came back "normal" (more about that later lol). Before they were going to discharge me I spiked a fever of 101. They weren't going to do an LP but after I spiked the fever they did. I was finally able to go home. My poor mamma was waiting with me.
Well with having EDS we are prone to leaking due to our ultra thin duras. You guessed it. My headache lasted longer than the usual 2 days and I was in a ton of pain with low pressure headaches. So I went into the ER at Alta View (a smaller hospital). Well due to not being able to do a blood patch under fluoroscopy they couldn't help me. But the attending was really helpful and booked one for the following day. Oh in between this I got a letter from the ER stating I had a UTI and to get some antibiotics. :p lol Hello! lol
After this we decided to make an appointment with Dr. Bolognese. I sent him my CT and LP (which was useless since they didn't do an opening pressure). I made an appointment for September. Thinking it was just a follow up and nothing was wrong (denial much? lol) he ended up throwing me a curve ball. lol. However just literally 2 weeks before my appointment my nystagmus and headaches started to get worse. Well he sat down and said well - yes there IS something wrong. My shunt is failing! I virtually have no ventricles. HOW did the ER miss this?! He said my shunt definitely needed to be replaced. This actually explains a lot of my low pressure symptoms! My head has been a mess and progressively getting worse. I likely didn't notice it because I do 2 liters a day. He also diagnosed me Eagles Syndrome. Or an elongated styloid process. This is causing a lot of my neck pain which I thought was my rods. I guess not! lol According to him mine are "very impressive"! He did order an LP before we even talked surgery. I had it and my opening pressure was 9.5. I'll explain below.
Speaking of which. Let's rewind a bit. I've been doing home fluids since last April. I started with a midline then when that crapped out on me we went back to PIV's. Oh sorry. We attempted a PICC line first but that was a fail due to my veins being too stretchy. lol. Well you can only poke veins so much before they develop scar tissue, not to mention the amount of blown veins I have. lol. After careful consideration Merin agreed to place a central line. I had my Hickman placed in August and it's been WONDERFUL!
Back in July I saw a geneticist who clinically diagnosed my Mitochondrial Disease. I then saw a neuromuscular doctor who did an EMG on me. When it was positive for myopathy he gave the "ok" to proceeded with the muscle biopsy. I haven't booked it due to everything else. lol. But I will after everything else. lol. The crappy part is I have to be off my supplements (mito cocktail) which I'm not looking forward to! lol. Sadly my dietician/NP has been more helpful with treating it.
Anyway. Haha I know I'm all over the place! lol. I had a follow up skype appointment with Dr. B in January. We went over my scans again. That's when he said my ventricles are "sucked dry". We also went over the STING study check list. And my other symptoms of leg tingling and frequent UTI's.
After my follow up appointment with him (oy my memory is bad lol). Oh ya - I called neurosurgery here and asked for them to look at my scans. Well according to THEM "I don't need a shunt replacement at this time". lol So I also sent my scans to a doctor in NV. He emailed me back and said he agrees I do need a shunt replacement. So that's 2/3 doctors. lol. Well after careful consideration we decided to go back to Dr. B. So I contacted insurance to proceed with the PPO Waiver/Gap Exception. Well it took 2 months and working with many people but I finally got Dr. B covered in network! It's such a relief!
The next chore was getting my testing done. So I made an appointment with my PCP to get that done. I had my Echo and EKG done Thursday. And a impromptu tube change due to me oh I don't know PULLING IT OUT! LOL Oh btw my surgery is April 27th! lol Ya cutting it short. LOL My EKG isn't even back yet. So I'm hoping they will get before my pre op on Monday! It'll be a lot of prep for pre op as well due to the mito. I've been doing weekly IV Carnitine and it's been helping so much! We put it in a bag of D5 and I get the use of my legs back for at least 5 days! AND my gut is kick starting a bit! I'm eating little bits! I'm still using my manual chair more as well which could be helping also.
As far as non medical stuff. I've been busy with my card business and I've branched out into Cricut stuff! I've made moms Mother's Day gift and I'll be making dads as well. Ya know I have to make up for last year! lol
Well this is the reason I've fired up this bad boy again. Im surprised I remembered everything. lol. Although It took a few times of writing off and on! Lol. I'm sure more has happened but this is the continuous stuff. lol. I'll have dad update when I'm in the hospital if I can't. So stay tuned and make sure you have email alerts! :) Please keep me in your thoughts and prayers. It's an "easy" surgery but with me you never know! lol. We leave for NY tomorrow and my pre op is Monday. Surgery is Thursday! Thank you! 😘. I've posted some pictures as well. I'll put the hospital address in the main page where my story is. :)
💜 Kristen
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