Quick update!

Hi all! I am doing very well and just spending lots of time quietly recovering at home. My pain is greatly reduced and I have not take ANY pain killers in the past 48 hours. This is amazing to me how the human body can respond so well to a huge trauma. I am only 11 days out, so this is very surprising to everyone that I am so pain free. I also am much less stiff than I was at first. I am moving my head much easier and not needing muscle relaxers as often.

The one issue I have been having is that I am still incredibly dizzy and my heart rate is through the roof. I emailed my neurologist and he finally got back to me after the holiday. I doubled my heart rate med for now to try to get the heart rate under control. Hopefully this will help and so far this morning (I take that med at bed time) I am doing much better.

I will update more later, but I just wanted to put out a quick update so that no one worried from not hearing from me!

Home sweet home!

Hi all, I finally made it back home! It was a long day of travel on Christmas Eve, but I am very happy to be home. All bumps make my head and neck really hurt, so car rides and plane rides are pretty painful. The worst part of the day was when the plane landed, that was not fun at all! We finally made it back to my in-laws at about 12:30 in the morning on Christmas morning. We had a nice relaxing day on Christmas and just hung out for most of the day. My (almost) father-in-law always makes chicken kiev on Christmas day which is super tasty!!!

Sadly I am not feeling well at all, however. My pain and stiffness in my head and neck are under control with medications. I am still taking the muscle relaxers every six hours and will be for quite a while. The stiffness will persist for a couple of months probably and I tend to not move my head at all very often. In the long run I will permanently loose about 30% of my range of motion in my neck, but we are not sure how noticeable this will be to me because my EDS (connective tissue disorder) makes the rest of the joints in my neck hyper-extendable so they may compensate. There is a very small chance that I may need to get joints lower in my neck, or even spine, fused later from instabilities due to the EDS, but Dr. Rekate said he has only seen this once in the 140+ fusions he has done.

I am still taking a prescription pain medication. It is taken every 4-6 hours as needed and you can take 1 or 2 pills at a time. I have been starting to only take 1 pill every 6 hours instead of 2 every 4 hours, so this is a good sign. I normally am able to wean myself off of pain killer relatively quickly. My guess is it will still be a good 2 weeks before I am weaned off of them and then I usually take them on an as needed basis for school or work for another couple of months.

Here is what my scar looks like now. All of the steristrips have fallen off so now we just have to keep it clean and dry. I caved today and took a shower and washed my hair, so I feel much better! I don't have to do anything special with it, just watch it and make sure it stays clean and dry.

The crunchy looking stuff at the top of the incision is just a scab that will fall off eventually. I only have glue and dissolvable stitches, so you can't see any stitches in this photo. It goes down further beneath the hair line than my previous incision did, but it does not go up as high as the one from last year did. You can see above the incision my scar from the previous surgery, which is the pale white line that curves towards the right.

My nurse came today and accessed my port. I want to learn to do it myself still, but I need more range of motion in my neck before I can do it myself. She is wonderful and it is hurting less and less to put the needle in as my skin is becoming more used to it. We decided to use a larger needle than we have been this time. In the hospital they used a 1 inch long 19 gage needle. The gage is indicative of how wide the opening of the end of the needle is and, counter intuitively, the larger the gage the smaller the needle. We had been using a 3/4 inch 22 gage needle normally, but decided to use a 1 inch long 20 gage needle. This is slightly smaller than the one at the hospital, but is what my home delivery company has. The reason we decided to use this larger needle is we found that the IV goes quicker that way. My neurologist prescribed the IV to go at a rate of 500-333 ml/hr, but we got lines that at the fastest go 250 ml/hr. With the larger needle we can turn the dial past the end setting of 250 ml/hr and get the infusion to go at up to 500 ml/hr. I also got a delivery from my infusion supply company, so now we have plenty of supplies! We ordered a bunch before my deductible starts over on January 1st. I was completely out of IV bags, so this was a much needed delivery!

My heart rate has been very high and I have been extremely dizzy yesterday and today. We are not exactly sure why, but we think it is because my body is trying to cope with the stress from the surgery and from traveling. My BP has also been eratic and my temperature is slightly elevated. It is nothing to be too concerned over, but for sure something we need to watch. My heart rate did come down about 10 beats/minute after running a liter of IV saline, so we will do another liter tomorrow in hopes that it helps keep me more stable. I will update later with how I am doing.

Oh and I called my neurologists office this morning because he still has not responded to my email from the other day and they said he is out of the office until Monday, so I will not hear back from him until next week. Please send prayers and well wishes that I start to feel better again!

Merry Christmas everyone!

First off merry Christmas Eve/Day to everyone!!!!

Today we finally get to go home after my surgery last Thursday! We have been here for 10 days and we are all more than ready to go home. It has been a great trip and we got to see and do so much before my surgery, but we are all ready to go home and get back into our own routines and beds! I am looking forward to getting home because we have a tempurpedic bed at home! Traveling will be a long and tiring endeavor today. Our flight is not until 8:35 Eastern time, so we will need to leave the condo at around 6 o'clock and won't return back home until about midnight or so. The plan is to plop me in a wheelchair because I will be in pain and drugged up. Hopefully this will also help to speed up security and baggage checking! I also did 1/2 an IV bag today and 1/2 a bag yesterday, so hopefully that will keep me from crashing as well.

I did not sleep well last night, sadly. The medications from the hospital are completely worn off now and I seem to keep getting more pain. This makes sense as pain normally gets worse before it gets better. During the day I am doing pretty well, but am still requiring my pain medication and my muscle relaxer on a regular schedule, but overnight is when the pain tends to really kick in.

Yesterday I had my mom help me wash my hair because I am not allowed to get my incision wet yet. I also go some spray in no-rinse shampoo and was able to wash off my body, so I feel a lot better after that! I hope it is not too much longer before I can take a real shower!!!

Have a great holiday everyone and I hope all my spoonie readers (please read this post if you do not know what that means) are feeling as well as possible as I know how difficult the holidays can be on people with chronic illnesses!

Zipperversary and Recovery Update

With all the commotion I forgot to mention that Saturday was my 1 year zipperversary! The Chiari decompression surgery leaves a scar on the back of your head/neck that resembles a zipper, so for this reason the anniversary of your surgery is called your "zipperversary". So much has happened in the past year that I am incredibly thankful for. I have had 3 surgeries (wrist, port placement, and craniocervical instability), 4 new diagnoses (POTS, EDS, hypothyroidism, and CCI-craniocervical instability), and the start of appropriate treatment for many of my conditions. I have come so far in terms of my health in the past year and it just keeps looking better and better! I still have a few issues that are going unaddressed and some doctor I need to see, but in general I am much more stable than I was at this time last year!

Today was a little bit more difficult for me in terms of my recovery from craniocervical fusion. I woke up twice over night to take medications and there is a lot more pressure in my head today. This makes sense since many of the nerves that were cut and/or numbed during the surgery are starting to come back as the medications wear off. I also got very low on pain medications which I did not anticipate. I have more pain pills at home, but I am still in NY so that doesn't really do me a whole lot of good. I called my PCP, but she wouldn't fill it. I then tried my surgeon, but I guess they are not allowed to call it in because it is a controlled substance. Well after some conversation with insurance and my neurosurgeon's office we got a 5 day supply of pain medications called in to the local walgreens to last me until I get back home in a few days.

The bruises on my hands are still pretty bad, particularly the one on my right palm. I am not 100% sure what all of these bruises are from. Some are from IVs, some are from arterial lines (a line into an artery which can by used for blood draws and blood pressure monitoring), and I am guessing the rest are from the monitoring during the surgery. I have 2 puncture wounds in each palm, so I am guessing this was from a monitor they had to use for either motor or sensory function of my limbs. I am using the all natural cream I mentioned in the last blog post and that has seemed to be helping. The bruises on my stomach from where they injected the blood thinner (heparin) are also pretty sore.

A few of my steri-strips on my incision fell off today and the incision looks really good. I will post another picture of my incision once the other steri-strips fall off. I also got a really neat pillow from the hospital that has been helping a lot. It is purple with a triangular hole in the middle and raised sides. It supports my head, but does not put pressure on my neck. It is not comfortable to use while sitting up, but it is very helpful lying down!

I sent an email to my neurologist asking him a couple of questions. I want him to order a pump for my IV medications so that I can set the rate that I want the fluids to go at. My insurance said they will cover this as long as he prescribed it. I also asked if he had a recommendation for an endocrinologist for my under active thyroid that we just found. The last thing I asked him was if he thought that my rapid heart rate would come down on its own from the surgery or if I could up the medication that lowers my heart rate. I am thinking I might not hear back from him until after the holiday, however.  

The home infusion company is going to send a month worth of supplies for the day that we get back, so it will be nice not to have to reorder those so often. I am going to be getting 20 bags of fluids so we will have to find a place to store them all! My home nurse will come the day we get home as well, which will be nice because she can check my incision as well.

Please pray for safe travels back home and a happy holidays! I will update after Christmas some time :)

Wound Drain

I keep forgetting to write about the wound drain that I had in. They put in a small drain into the back of my head to drain the blood and any other fluid that collected in my wound. It looked something like this. The end of the tubing was stitched into my incision.













This drain helped to keep the swelling down and relieve the pressure that built up immediately after surgery.  They emptied the drain the first night and 90 cc of fluid came out. This is equivalent to about 6 tablespoons. In order to take it out I had to be draining less than 60 cc of fluid over 8 hours. The next morning (Friday), once I got into a regular room the physician assistant came in and checked the drain. I had only drained 30 cc more (about 2 tablespoons) of fluid. This meant we could take the drain out! It was slightly painful for her to take out because they had stitched it into my incision to hold it in place. It took some time to get those couple stitches out, and I could feel them digging around with the scissors to get them out. Once those stubborn little stitches came out they just pulled the tubing out. This part was not painful, it was just a lot of pressure when she pulled on the tubing. We just had to put gauze on the incision for a little while to stop the bleeding and you can't even tell where it went in anymore.

Unless I think of something else I forgot I will try to update again over the next couple of days, but I make no guarantees as we will be traveling back home to Chicagoland. Happy holidays all!

Pictures of incision and various odds and ends

Here a few photos of things I have been talking about since my surgery.

This is a photo of my incision on the same day I had the surgery, so the bandages are still on.

This photo is on Friday, the day my bandages came off. The tape strips are what is called steristrips and are used to help glue the incision together while it heals. They used the same incision as they did for my decompression, but it goes down a little further onto my neck than the other scar did. At least that is what it look like right now. We will see more after the steristrips come off and things start to heal a little bit more.

This is a picture of me in my Miami J neck collar. It isn't the most common neck brace, but it is the one my surgeon likes the most. I think it is pretty comfortable and have worn it a lot while I have been in NY. It helps a lot on planes, cars, and boats.  You can kind of see it under my scarf. The dark blue parts are the padding and the white/gray parts are the hard plastic to hole my head in place. I will try to find/take a better photo of the brace. P.S. this picture was taken at The Statue of Liberty with my fiance Joseph!!!

This last picture is of my port. The blue extension on the end is normally clear. It is the same thing, my home health care company just uses a different brand. The green thing on the end is the little caps I was talking about that keep the port sterile. They are called curos and I am hoping to get them for at home. I don't really like the way that this is dressed, but when I get home my home nurse will change it. The bandage is just a little bunched up and doesn't look/feel as comfortable as normal. The writing on the right side of the port says what size needle is in it: they have both a gage which is how wide the hole in the needle is (the bigger the number smaller the hole in the needle) and how long the needle is. I have a 19 gage needle in, which is fairly large because they wanted it for the medications for the hospital. For comparison I usually use a 22 gage or rarely a 20 gage needle. The needle is 1 inch long, which is a little longer than I normally use but not uncomfortable. I normally use a 3/4 inch needle, but then they can not use what is called a microbe disk. it is the little white disk that you can kind of see underneath the bandage that goes right around the needle. I don't normally use these because a 3/4 inch needle is too short to use one with, but we might switch over to a 1 inch needle for this reason.

 

Well that was a lot of updates for the last couple of days! I will update again after I start recovering and talk to a couple of my doctors. I am hoping you are all well!

Resting and recovering at home

My brother is visiting so he was able to drive me home from the hospital. This was very nice because then we did not need to take a taxi home. The drive was a little rough and bumpy, but overall not too bad at all. Once we got home the first thing I did was to fill up my pill container. I have a weekly pill container with all of my medications in it so that I do not forget a dose or take an extra dose if I forgot that I had already taken my medications. This took quite a while as I take 4 medications normally as well 4 supplements. I also take 2 medications for constipation, a pain medication, and a muscle relaxer for the first few weeks after surgery. Once this was done I sent my finace and one of his sisters to Walgreens.

The doctor prescribed a muscle relaxer (valium), but I did not want to get it filled because valium puts me into "la la land". I still had a refill on my other muscle relaxer (robaxin) from my decompression surgery last year that expired on 12/23/12, so I had them get a refill on that instead. Also, I had him pick up a medicine for my stomach because your lower intestines are the last thing to get working after anesthesia and straining is bad for your neck. The last thing I had them get for me was a no rinse shampoo. I can't wash my hair for at least a week and I have pretty greasy hair, so this shampoo you just spray in, massage it for a couple of minutes, and then brush your hair. It is not the same as a real shower, but it definitely helps! I also have a no rinse body wash from the hospital so I can at least wipe myself down a little bit.

My mom and fiance just helped me to wash out some dried blood and other gunk from in my hair and then we used the no rinse shampoo, so my hair finally feels semi-clean. We then took a paper towel and scrubbed off all of the sticky stuff from adhesive bandages. I had 6 monitors on my chest to monitor my heart rate, two on my ankles and two on my wrists to monitor sensory and nerve function, 2 on my stomach from where they did heparin injections to make sure I didn't get a blood clot, and a few on my arms from IVs, arterial lines, shots, and who know what else. All of these had bandages on them so they take some scrubbing to get all of the black sticky stuff off!

I have some major bruising on my right hand and wrist. The arterial line was in my right wrist, but we do not know why I have puncture marks in my hands. They might have been attempts to get arterial lines in, or they might have been from how I was positioned on the operating table. I have been using an all natural remedy that my mother-in-law found that is good for muscle aches and pain, but also bruising. This stuff is called Arnicare and has helped with the bruising from all of my IVs in my arms and hands. I will make a post later with a bunch of pictures of stuff from my surgery that I have been talking about.

I am feeling pretty well now. I have been taking my pain and muscle medications on a regular schedule, but surprisingly (and thankfully) I only woke up once during the night to go to the bathroom and take a couple pain pills and a muscle relaxer. After my decompression surgery night times were really rough for me and I woke up at least a few times a night for the first 2-3 weeks or so, so this was a nice surprise! I had forgotten to mention in my last post about sleep. You hardly get any sleep in the hospital because alarms that monitor vital signs are going off, nurses come in to check on you and give you medications, and the fire alarm even went off once. On Friday (the day after the surgery) I would fall asleep all of the time. In mid-sentence, during eating, while changing position, anytime. Thankfully by Saturday this had gone away completely and I was able to stay awake when I needed to.

I also forgot to add an issue I had right after surgery. I was fine when I was awake, but every time I would go to fall asleep my oxygen levels would drop dangerously low. We do not know why I was doing this (called desatting), but for this reason I had to wear oxygen in a nasal cannula (one of the things with two little prongs that stick up your nose to give you oxygen. I only needed this the first night and then all was good after that!

During the hospital I also had to get 2 scans done. I had a CT scan done while I was still in the ICU and an MRI done once I got to the regular room. The CT scan was super short again and the MRI was during the period where I still could not stay awake at all, so I just slept through it. It was a bit of a fight with medical records to get all of my records released so we are still fighting that. I got a copy of both of these scans with me, but I couldn't get the reports or the operative report right away. For some reason the hospital has me in their system twice: once with my middle name and once without. We should get those papers by about the middle of January, but I will probably have to follow up with that later.

As far as recovery goes I have to make an appointment with my PCP (primary care provider) for a couple of weeks from now. I am not sure when I am going to make this because I do have a home nurse who is coming out to do my infusions so she can check my incision as well and make sure it is healing. I then should need to do a skype conference in a few weeks with Dr. Rekate, then do a scan and  another skype conference in 3 months or so, and then probably return back to NY for a check up in a year from now.

Well this post is very long again already so I will update more later as things come about. I hope you are all well!

Home from the hospital!!!

We are home!!!!

I have been pretty out of it for the past couple of days, but am feeling much more "with it" it today.

Thursday we got to the hospital at 5:30 in the morning. They called my name and I went and got my vitals checked then went and sat for a little bit longer. The nurse called me back and even though we had been told that I could only have 1 person come back with me to the pre-surgical room my mom, my fiance, and my future mother-in-law were all able to come back with me. The nurse wasn't very kind and started an IV even though I didn't want one with my port... well she wasn't going to do anything until I convinced her to do fluids for me before I passed out! She did them very quickly for me after I told her that. Then I met with the anesthesiologist and discussed my concerns about nausea and vomiting with him. He ordered a few different IV meds to be given during the surgery. He also ordered an oral IV med for before the surgery that was supposed to last for 48 hours. I had never had this before and it for sure seemed to help a lot!

 After this the two surgeons (neurosurgeon and spinal surgeon) came back to talk to me and my family. They were both very kind and explained again what they were going to do with the surgery. The spinal surgeon, Dr. Insinga, I had never met before that morning, but he was incredibly nice!!!! He felt like everything looked great judging by my scans and that they would not need to do a hip or rib graft to take any extra bone.

This is when I got wheeled into the operating room. It took quite a while in there, but it was kind of nice because I got to meet all of the surgical staff. We had a little bit of confusion on the orders the doctor had written so we wanted to clear it up so I could give consent before being put to sleep. Dr. Rekate had written revision of posterior fossa decompression (brain surgery from last year), but we hadn't talked about me need that only the spinal fusion. It turns out that because they went through the same incision and were in the same area of my brain/spine that they had gone into last year it is considered a revision of the last surgery in addition to the new fusion. Once we got this issue cleared up I got put to sleep at about 8:15.

The neurosurgeon (Dr. Rekate) came out at about 12:45 to talk to my family and said that from his end everything looked great. The spinal surgeon was still working hard to get my fusion stabilized and screwed in place. The spinal doctor did not come out until about 4:00. The previous neurosurgeon who did my decompression last year in Chicago did not touch my first cervical vertebrae. This was not good because I needed this space to get more room for the Chiari compression which is in the back of the brain. The good news was that they were able to remove this C1 (first cervical vertebrae) and use it for more boney material that they needed for getting the fusion to set in well. Both doctors said that I definitely made them work very hard to achieve the results they needed and wanted. The fact that I had C1 still in there made it more difficult for the spinal surgeon and Dr. Rekate had more difficulty getting spine aligned correctly than he thought he would. They used electrodes and various other monitors to monitor my sensory and motor pathways that run through my body. These pathways all go through the brain stem, where my compression was, but we did not know how badly effected they were by the brain stem compression until the surgeons went in there. Because of this Dr. Rekate said my balance, coordination, posture, and muscle strength in my extremities should all get much better. He said he was extremely surprised at how drastically my strength came back in my arms and legs once they moved my spine and relieved the compression on brain stem. I have never complained of weakness in my arms or legs and no one has noticed it, but apparently it was extremely significant, so hopefully with time I will notice more strength in my arms and legs.

I was supposed to go back to the recovery room over night, which was not ideal because family can't stay and there are no rooms only curtains. Thankfully my neurosurgeon said he absolutely did not want me to go there and wanted me to go straight to the neurological intensive care unit (neuro ICU). In the Neuro ICU the first hour or two were extremely rough! I did not have a pump that has a pain killer 7 times stronger than morphine that I was supposed to have. I was not crying because I was in too much pain to do so, but I remember just saying one word at a time and saying ouch all the time! My mom and my brother (who had driven in from the army base out of town where he is stationed at) came back at this point and I was very happy to have my mom's hand to squeeze! After an hour or so of this we finally got the pain pump started and they gave me a large dose to start with, so that really took the edge off of my pain. I was sleeping for about an hour at a time and would wake up every so often to push the button, but was feeling ok. About 12:30 in the morning they took my urinary catheter out as well as a line in my wrist that is called an arterial line. The urinary catheter did not hurt like it did with my last surgery so I was thankful of that! This was at least two less lines that I was now hooked up to. I got up at about 4:00 and 8:15 in the morning to use the restroom, so this was a very good sign!

The next morning I was still feeling pretty well and at this point was managing my pain quite well.  The night nurse was not doing very well managing my meds. I was still using the "magic button" as I like to call on an as needed basis, but not as often as they thought I would. For some reason they kept trying to tell me that they gave me my medication to lower my heart rate during surgery, which makes no sense at all! They gave me a medication to lower my heart rate, but not MY medication that my body is used to. It took me until the next day (Friday) to convince them of this and I finally found a physicians assistant (PA) that was fantastic!!! She told me to just take my medication from home and not listen to them. Of course now my heart rate is all messed up because my medication time table is off. This was annoying to deal with, but at least we figured it out now! The same thing happened with one of my medications for low blood pressure called Midodrine. It says I am supposed to take it 3x a day, but I need it as soon as I wake up and then every 4 hours, not every 8 hours like the hospital wanted. They could not seem to get this through their heads! The same PA who had helped me that morning with my other medications and she told me to take it since I am at risk for fainting without it. Again she said this was ridiculous and let her know any other issues with my medications.

The one thing that was really bothering me was nausea still. I got phenegran, which is a anti-nausea medication that I use at home as well, so we know it works. Then I got another IV medication call Reglan which helped drastically with the nausea. After a while I got nauseated again, so we decided to try something different. I can't remember what the medication was called, but they injected it into the muscle in my arm. This in combination with my Phenergan and the pill I had taken before my surgery kept me from vomiting at all! I had also gotten two shots (once a day) in my stomach of heparin, which is a blood thinner. This hardly hurt at all and was just used to insure that I did not develop a blood clot since I was more sedentary in the hospital than my body is used to.

I also have pressure sores on my knees, hips and rib cage as well as a couple of spots on my head. They are just really sore red spots from how I was positioned for so long during the 6.5 hour plus surgery. The ones on my knees are gone and do not hurt anymore, so now it is just the one on my right hip and left rib cage right under my breast that still hurt a little bit. The nurses say this shouldn't last more than a week, however.

After that I finally got moved out of the ICU and over into a regular room. Once we got there the central line nurse came by and finally agreed to take out my two peripheral IVs in my hand and elbow and access my port. This was so nice because I kept setting off the alarms when I would bend my arm or kink the tubing. I was ready to be back to how I am used to with just my port accessed! It took a little longer to the needle in because the nurse wasn't as experienced, but we got it all okey dokey. The issue again at this point was that I was not supposed to leave the hospital with my port accessed. Again the magical PA came in and saved the day! She clarified with me that I needed it between now and when I go home, and I said yes I absolutely do, so she over rode whoever told her no and let me leave with it accessed so all is well with that. We also got some neat little caps that cover the the ports on the line coming out of my port. They keep everything sterile when not in use and are very handy to have. I will have to talk to my home health care supply company about getting a hold of more of these because they are very useful!

After this I was doing very well. I had weaned off of the PCA (pain medicine) pump overnight and was no longer using it. This was the first step in my going home! I needed to switch to oral pain medications and muscle relaxers at this point, but overall I was still doing very well. The physical therapy people came for the second time this morning to take me for a walk around the hospital again and cleared me from a PT standpoint to go home. I had done just fine yesterday, but they need to check on you everyday. I walked a lap and a half around the neurological unit and went up and down five steps with no issues. The PA that we love then came in again and said I looked great. She paged Dr. Rekate to come in and speak with me. He came a few hours later and was astounded on how well I was doing! He said he has never had a patient who had this surgery who was ready to go home within less than 48 hours. He was sad to see me go because he said I really brightened up the ward! That was kind of him and he was a very sweet man! He cleared me to go home at about 11 a.m., but by the time we made it through all of the discharge paperwork, the nurse changeover, and the final vital checks and what not we ended up leaving at about 3:30 in the afternoon. 

I am now at home and will update again probably tomorrow or the next day with how I am doing and what all is going on with my home care, but for now this is very long! I love you all, thank you so much for all of the prayers and well wishes!

New York here we are!

Hi all! Sorry for the delayed update, but we have been very busy since making it to NYC!!!! The travel went very smoothly and we got here on time with no delays. Packing was interesting because of how much medical stuff I have to bring with me! I went over the weight limit of 50 pounds for my suitcase so we had to transfer some stuff into another suitcase...oops! I had to bring IV bags and supplies for both before and after the surgery, all 4 of my normal medications and 4 of my normal supplements in their original containers for while I am at the hospital, the medications that I take on an as needed basis (nausea, pain, muscle relaxers, allergy, ect), my neck brace and extra pads for the inside of my neck brace, and various other odds and ends. I can't carry the heavy suitcases, so through the airport my "luggage" was to keep track of my youngest soon-to-be sister-in-law who is 8 :)

We got to the condo we are staying at around 1:30 eastern time after leaving my fiance's parents house at 6 a.m. central time... long day of traveling, but we made it! We got here and the first thing I did was take a nap! Then on Monday we had 3 different appointments at the Chiari Institute. We had to be there at 8 o'clock for a CT scan of my neck. I expected this to take forever because I have only had 1 CT scan before and am used to MRI's. Well I guess I forgot that CT scans are very short so it took only about 3 minutes tops and I was done!

Then we went downstairs and met with a nurse, which was supposed to start at 9:30, but she got us earlier. She was very kind and sweet! She just asked me a bunch of questions about all of my symptoms, medical conditions, and medical history. The one thing that she did that I have never had done before was something called The Beighton Scale. This is a measure of joint hypermobility (how far in the wrong directions that specific joints can go) that is used as a diagnostic criteria for the connective tissue disorder that I have called Ehler's-Danlos Syndrome (EDS). This scale is scored out of 9 points: one point is given for each pinky that bends backwards beyond the "normal" 90 degrees, one point is given for each elbow that hyperextends more than 10 degrees past the neutral straight position, one point is given for each knee that hyperextends more than 10 degrees past the neutral straight position, and the final point is given for being able to place your hands flat on the floor from a standing position without bending your knees. A 4/9 on the scale is considered clinically significant, and along with clinical symptoms is used to make a diagnosis of EDS. I scored an 8/9 on this test. I missed one point because I was not able to put my hands flat on the floor without bending my knees. This is just another confirmation of a diagnosis we already knew.

After we finished with her we had to go back upstairs and get a copy of the scan they had done that morning. The doctor wanted the physical disk so he could load it on his laptop and I wanted the copy to be able to keep for myself.  After we got the disk we went back downstairs to meet with Dr. Rekate's medical secretary. She and I have been corresponding for months regarding paperwork and insurance and such. She still needed a few papers to send to my insurance, so while we were waiting for the appointment with the doctor at 10:30 I had to call my primary care provider (PCP). I needed a note from her stating that she wanted me to see a specialist out of town, eve though she did not necessarily say this I knew she would be willing to write a note for me for the insurance. Then the only other thing that my insurance was requesting was a note from Dr. Rekate after he had seen me stating that I needed this operation. So, finally Tuesday afternoon after all of this paperwork was sent to my insurance they finally pre-approved the surgery! It was getting a little nerve wracking there thinking they might not approve it, but we are now all good for Thursday.

After this we had to wait for quite a while to meet Dr. Rekate. He was in a meeting that they had not expected to occur that morning, so my appointment that was supposed to be at 10:30 was pushed back until 11:15. We finally got to meet him and he was incredibly sweet! He talked with us for quite a while and was very interested when I told him I am going to school for speech-language pathology. He was also interested to hear that I have autonomic dysfunction, EDS, and Chiari. He is trying to get a grant to follow these patients after surgery and learn more about how we heal and how these conditions interact. I am excited to be able to speak more with him during my hospital stay about these things!

He first showed me my new scan. It was focused on the place where your neck meets your head. He had drawn a measurement on the scan called the Grabb-Oakes measurement. I can't find a good picture to explain exactly what this is, but basically the very top portion of your spine is called the odontoid process and this is a triangular shaped piece of bone. It is supposed to be straight up and down, but mine is bent backwards and pressing into my brain stem in such a way that the brain stem is actually draped over the top of this odontoid process. The Grabb-Oakes measurement tells you how far backwards the odontoid process is tilted. Anything over 8 is absolutley considered abnormal, and anything over 9 is considered surgical. My measurement is 11mm, so obviously this is what made me a clear candidate for the fusion surgery. The rest of the visit he basically just explained the surgery and said that he feels as if this surgery is going to help me tremendously, but only time will tell just how much

The surgery will take about 6 hours long. They will hopefully not have to use either a rib or hip graft to get more bone, but we will not know that until during the surgery. Dr. Rekate will be working with a spinal specialist named Dr. Insinga, and he will be the one making this decision. They will be fusing from my skull down to my second cervical vertebrae with titanium rods and screws. I should be in the Intensive Care Unit for 1-2 days then in a regular room for between 2-3 more days. My surgery is scheduled for 7:30 in the morning eastern time, so we have to be there at 5:30 in the morning!!!

Then on Tuesday I went to the hospital for my pre-surgical testing. They asked me a bunch of questions about my medical conditions and symptoms, took my vitals, and then did some blood work. This sounds simple enough, but in typical Sara fashion it was a pain in the butt. It took forever to do the blood work because I refuse to get an IV in my arm when I have the port in my chest. They put the port in so that I did not have to get stuck with a needle each time I needed blood work. Well due to some stupid hospital policy only a central line nurse was allowed to use my port. Because of this I had to wait for quite a while, but we got the blood drawn without sticking me with a needle!

At this point the only thing I have to do to get ready for the surgery is to wash my body with a medicated soap tonight and tomorrow morning to make sure all the bacteria on my skin is killed before the operation. I also am not allowed to eat anything after 11 o'clock tonight.

I will have my fiance Joseph update the blog periodically tomorrow so that you guys can stay informed. Thank you all for your prayers and well wishes!

New York Preperation!

So, yesterday I had my echocardiogram and my appointment with my general surgeon which both went well.

An echocardiogram is a scan done to get a detailed picture of your heart and the valves within your heart. We needed to do this just to make sure my heart was in good condition before the major spinal surgery next week. They put some very cold gel on your chest and then use a probe that emits sound waves in order to form a picture of your heart. It is basically the same thing as an ultrasound you get when you are pregnant, but of your heart. It took about a half an hour and is slightly uncomfortable, but not at all painful. I then had to get the results faxed over the the Chiari Institute today so that of course took a couple of phone calls, but thankfully we got it sent over all ok! I'm assuming that since I wasn't told otherwise that the results came back fine. I just had one in February that was fine, so there is no reason that this one should not be.

I then had my appointment with my general surgeon who put in the port 2 weeks ago. He said my incisions are healing wonderfully and they look great. I shouldn't have to go see him at all any more unless I have any problems! I did have some blood on the bottom of my bandage that is on my port a couple of times, so I asked him about that. He said it is completely normally and is just my skin getting used to having a needle in there for so long. The one issue I had was that he moved the bandaged to get a closer look at my incision and apparently the bandage wasn't sticky enough when he put it back on, so when I went to take a shower the bandaged filled up with water! Well that was interesting! So I had to do an emergency re-bandaging and re-sanitize everything. I think it should be just fine and Joseph (my fiance) and I got it all sealed up again!

I also had to get a few of my MRI reports and my operative report from my decompression surgery last year sent off to The Chiari Institute, so that took a number of phone calls and a visit to Loyola where my surgery was done last year. Thankfully we were already in downtown Chicago for Joseph's graduation so we were able to stop in on the way home. Everything got faxed over so we should now have everything we need from Illinois sent over the The Chiari Institute.

I did get a call from my primary care physician today with results from my blood work that they did on Monday. She had decided to check my thyroid levels since I mentioned to her that I was shakey, which has been a new symptom for me. Well the results came back that something called my TSH (thyroid stimulating hormone) is elevated. This means that my thyroid is hypoactive (or underactive). So when we get back from NY and I start to recover a little bit I need to make an appointment with an endocrinologist, who is the specialist who handles thyroid problems. This could be causing some of my symptoms such as cold intolerance, fatigue, brain fog, and constipation. It does not surprise me that my thyroid is off and it was something I wanted to get checked relatively soon, so now we will just get it checked a little sooner than we expected. It is a simple fix for most thyroid problems and it will just be another medication added into my regimen!

I had some concerns about flying with my port accessed (needle in it) due to security issues and what not, but we have contacted TSA and they said that we should be just fine. I will have to go through a couple of extra security measures, but hopefully we should have no problems. Please pray that this is not an issue!

I will update again once we are in NY either Sunday night when we get there, or more likely Monday after my appointments. The first thing I go do Monday morning is a CT scan of my neck that will show us the instability that is present in my spine, then at 9:30 I meet with a nurse for a neurological examination, and finally at 10:30 I meet with my neurosurgeon Dr. Rekate.

Echocardiogram

My home health nurse came and reaccessed my port. We went in the exact same spot as last time and it went in very smoothly. It is slightly painful initially, just like when you get poked with a needle for an IV but twice that. It has to poke through the skin and then through the port so it is almost like getting poked twice.  After that there is no pain at all for the week that the needle is left in. I did deaccess myself last night. I had scratched open my incision from the surgery and it had started to bleed under the bandage so I just took the bandage off and deacessed it. It was super easy to do!

I have an echocardiogram (pictures of my heart) scheduled for 1:15 on Thursday. This is the time my general surgeon appointment was supposed to be, but there are no other times that I could do the echocardiagram and that has to be done before I leave for NY. I switched my appointment with my general surgeon to 2:30 on the same day, but that means I will probably be late for work again on Thursday :( Oh well that has to be done before we leave for NY as well, so what can you do? I will update after those appointments.

Blood Work Frustration

I had my appointment with my general physician (GP) today for pre-surgical clearance. It went fine and everything cleared out just fine. I got an order for the echocardiogram that I needed, so I will need to call tomorrow to get that scheduled. I am not overly fond of the GP and I never go to see her unless I need pre-surgical clearance. She does not understand any of my medical conditions and really does not add any insight to my medical team.

So today I made sure that I had my port accessed so that they could use it for my lab work that I knew would need to get done. The nurse did not have port training so she went to find a nurse who did. Well that nurse claimed that ports can only be accessed in a sterile hospital setting. Now obviously we know this is not true as I access my port at home and they are able to be used at a doctors office as well. In the long run the nurse said it was "easier" for her to just take the blood from my arm. So I have a perfectly good central line that is supposed to make it easier for them and me to get blood work done and they won't even use it since it is not "easier" for her!!! UGH!!! So in the long run I will now have a bruise for a few weeks simply because she did not want to figure out how to use my port for the blood draws! Next time I will refuse to get the blood drawn from my arm and just go elsewhere! I just had to do it today because of my upcoming surgery and did not want to delay anything with the blood work.

Anyways I will update again after I get the results from my blood work and echocardiogram and after I meet with my general surgeon on Thursday.

Saline infusions

My infusions are going really well!!!! I have done 2.5 bags so far (I am currently running a bag). It is so easy to do and they help so much! I have more energy, don't feel as foggy, and my heart rate and blood pressure stay a little more stable. I was going to do infusions 2x a week, but that was just not often enough. I did my first one on Tuesday and then the next one on Friday, but Thursday was very difficult for me. I just was not nearly as alert and my heart rate was not as stable. I decided to go up to 3x a week on Monday's, Wednesday's, and Friday's, but after talking to some other POTS patients decided this wasn't quite right either because then every Sunday my body would crash because of the extended time without fluids. So, for the next week I am going to try every other day. I think this might be the right frequency to keep me hydrated and stable. I am so thankful that my neurologist put in my orders as PRN (as needed) because this gives me the freedom to adjust the amount of fluids I get to fit with my body and my schedule.

My home nurse is coming back on Tuesday because the needle and dressing that are in/on my port need to be changed once a week and I have not learned how to do that yet. I am hoping to learn to access (put a needle in) my own port after I get back from NY and start to recover from my spinal fusion. I now have to figure out how many fluid bags and supplies to bring with to NY for before and after I get out of the hospital as well as bring extra port supplies just in case something goes wrong and I need to change the dressing or anything else. I also need to count out and make sure I have enough of my normal oral medications and supplements to make it through the trip plus extra for if we end up needing to stay longer than expected. I will also need to bring my medications that I take on an as needed basis (muscle relaxers, pain killers, nausea medications, stool softeners, allergy meds etc.) so that I have them if I need them.

I got another delivery of supplies Friday night since I changed the frequency of infusions. When I talked to the woman at the supply company she informed me that they do not have any other lines that have dials to set the rate of infusion. My only other options at this point are to let it infuse by gravity only or to get an electronic pump to set the rate of infusion. I am not sure how fast (how many ml/hr) it would go in by gravity alone or if my insurance company would cover a pump, so I need to talk to the home nurse on Tuesday when she comes and see what she thinks. She is also going to help me figure out if I can fly with my port accessed (a needle in my port like it is right now). I am not sure what the security measures are for that, so we will have to figure that out before we leave.

I heard back from my neurologist about my blood work from a couple of weeks ago and it all came back normal. This means that my potassium and sodium levels are within normal limits so I do not need a potassium supplement as of right now.

I still have my pre-op appointment with my general physician tomorrow. She will order a bunch of blood work and a new echo-cardiogram (pictures of my heart). I decided to just get a new echo done instead of getting the results from my old one since I will need a new one soon anyways as I need one done once a year with my EDS (connective tissue disorder). I also scheduled a follow up with my general surgeon who put the port in for Thursday just to check the incision and make sure that everything looks good before we leave for NY.

Finally, (I know this is long already) I got put on the waiting list for a geneticist name Dr. Brad Tinkle that specializes in Ehlers-Danlos Syndrome (EDS). He is one of the leading experts in the country and just relocated to Chicago! This list is currently 6-7 months long, but at least I am on it and will hopefully get to meet with him next summer. He will be able to help determine for sure which type of EDS I have, discuss implications this will have on family planning for the future, what to expect for me in the future, and recommend any specialists or therapies he thinks I need or will need. 

I will update again later this week before we leave for NY next Sunday!

First port access!

I just got my port accessed for the first time! The home health care nurse came out today and accessed it for me. She was really nice and explained to me what she was doing and how to do everything. She said I was the most interesting and complex patient she has had in a long time! I get that a lot from people it seems! Eventually I will be able to access the port on my own, but that will take a while for me to learn how to do it and feel comfortable doing it independently. It will also be delayed with my major surgery coming up in a couple of weeks.

The delivery company dropped off the IV bags, IV pole, needles, and all of the other supplies last night to my apartment. It was extremely convient and they were very nice! We can adjust the order as we figure out specifically what I need. The nurse got me all accessed and we got a great blood return on the first try which means my port is working very well. I am now running the saline solution. My doctor wanted it run over between 2-3 hours. The problem with this is that the line that the supply company sent me that hooks me up to the bag of fluids only adjusts to a rate of 250 ml/hr. Well since each bag is 1000 mL, or one liter, this isn't fast enough! We just adjusted the dial to go past the numbers, but it still will probably take longer than 3 hrs. We will have to ask for a different line next time they send me supplies. We are hoping this will help to stabilize my heart rate and blood pressure and keep me from crashing. Right now we are scheduled to do the infusions twice a week and we can adjust it from there.

This is a picture of what my port looks like when it is not accessed. There are two incisions where the tape is and then the port is self is the lump under the bottom incision. It is not overly noticeable and hardly bothers me at all. The yellow and greenish tint that is around the port is just bruising from the surgery. I bruise easily with my connective tissue disorder (EDS), but it should go away within another week or so.

This is what the port looks like when it is accessed. The bandage is to keep the needle in place. The black wing looking things are part of the needle which is a special kind of needle used with ports called a huber needle. The blue and white dial I am holding is the dial I was talking about to adjust the flow of the saline and the white plastic piece is a clamp to stop the flow of saline when I disconnect the line.

I was able to pick up my cervical collar that I will need for after my fusion surgery. I picked it up before I went out of town for the holiday so it was very nice to have in the car! My neurosurgeon wants me to wear the collar for long car trips, on the plane, and whenever I feel I need it for symptom reduction. It is not the most comfortable thing in the world, but it really does help.  One issue I was having is that my chest was very sore from the port placement, so I couldn't wear it too long. The other issue I had was that my neck muscles are so tight from the instability that I can't wear the collar for more than a couple of hours without my muscles spasming from the pressure. I took some muscle relaxers and this really helped. I will post a picture of the collar to give you a better idea of exactly what it is later on.

The issue with the Chiari Institute and my insurance got figured out. For some reason the one representative that the lady I have been corresponding with spoke to told her that he couldn't find my doctor, when everyone else can. We have had three other people from my insurance company check and they all say that he is covered so we should be good! I also got a call and I have a rotational CT scan scheduled for right before I go for a consultation on December 16.

I still have not heard back from neurologist about my blood work results from the blood panel I had run a week and a half ago. I will have to re-check in with him and see what is going on with that. My pre-op checkup with my general physician got changed to next Monday so I will update after that appointment.

Power Port Placement Surgery

Surgery went very well last Wednesday! Sorry for the delayed update, but I was out of town for the holiday.

We got to the hospital just before 9:30 in the morning and went straight back. They immediately started an IV to start pumping IV fluids into me. The nurse actually managed to get the IV in with 1 stick, but of course she had to wiggle the needle around so most of my right hand is bruised... The IV pump then didn't want to work and kept beeping at us, but eventually we got it to go smoothly with some repositioning of tape and my wrist. The anestheologist came in and we talked for a long time about all of my conditions. He was intrigued and wanted to hear all about them! He said I was the most interesting patient he has had in a month or more :) He was very sweet and shortly after then my surgeon came in and asked if we had any questions. Well off we went to the OR.

In the OR they got me situated on the operating table, turned up the temperature because I felt like an icicle where the IV fluids were going in, and administered the sedation. I did not go under general anesthesia so that they did not need to assist in my breathing and there was not as much strain put on my body. Everything went well until about ten minutes before the operation was over when I woke up... That was not supposed to happen! It was ok though, I was just laying there and then asked "Am I supposed to be awake yet?" I think that gave them a slight fright! The anesthesiologist popped his head under the sheet that was covering my head and talked to me through the rest of the operation. It was actually pretty cool because the surgeon then talked to me about what he was doing. They also were able to ask me if I could feel what they were doing and if I was in pain, so they were able to give me more numbing medication the one time I said that I felt what they were doing. They explained how the catheter had a kink in it so he was going to take it out and re-thread it through my vein and then talked me through how he was closing up. 

I was then taken back to recovery and given another 2 liters of fluids and was discharged around 3 in the afternoon. I was not in much pain and was doing very well in recovery. My fiance went to the pharmacy during this time to pick up a pain killer just in case as well as an anti-nausea medication because I tend to get extremely sick to my stomach with anesthesia. We then got on the road at about 4 and headed down to Cincinnati to spend the holiday with family.

Thursday was the worst day for me. It hurt to move my arm at all and I was in a fair amount of pain. Thankfully by Friday I was feeling much better and by Saturday was just a little sore. I have two incisions from this surgery: one on the upper right side of my chest and one on the right side of my neck. Because of the incision on my neck it hurt to turn my neck, but today (Sunday) that is feeling much better. I took the large bandage off on Friday and some of the steristrips (small pieces of tape over the incision) off this morning. I now have 2 steristrips on the incision on my chest and one on my neck that I will leave on for a few more days. I have some bruising on my chest where they put the actual port, but even that is not too bad. Hopefully we will be able to start accessing the port shortly! I will post another update with the rest of the new medical stuff that has happened this week later today or sometime early this week. I hope you all had a wonderful holiday!

Date for port placement (finally) and some other updates

The new general surgeon was night and day different from the one that I saw that was incredibly rude and refused to put in my port. He was so kind and helpful!!!! I am going to get my port put in at the local hospital next Wednesday 11/27/13, yes the day before Thanksgiving! I am so excited to get this all set up and ready to go! I already spoke with the pre-surgery nurse this morning so she got all of my information in the computer and what not. I should get a call on Tuesday evening to confirm the time, but as of now my surgery will be at 11 a.m. I will need to be there at about 8:30 or so to receive IV fluids so that I do not get dehydrated like I did after my wrist surgery.  I also got a call from the home infusion center yesterday while I was at work, but when I returned their call this morning they didn't answer so now we are playing phone tag.

The fitting for my cervical collar was quick and easy, I just haven't been able to pick it up yet because it is in the town my parents live in and I am an hour away from there. I am going to go get it on Tuesday and then have a nice evening with my parents and my puppies! I will not be with them for thanksgiving this year as we are going to see my Fiance's family so it will be nice to get to spend time with them.

I also got some blood work done yesterday to check the levels of electrolytes in my blood. I have to get this panel, called a basic metabolic panel, done every couple of months because one of the medications I am on, called fludrocortisone, can deplete my body of potassium so we need to keep an eye on that and make sure that I do not need to take a supplement for that.  I haven't got the results of that from my neurologists office, but I should shortly.

On the downside I got a call from The Chiari Institute where I am having my surgery done in December and the lady that I have been conversing with finally got a hold of my insurance. The gentleman that she spoke with told her that The Chiari Institute and my doctor are not covered by my insurance. Now when I had spoken with them they had told me they were. Please pray that we get this figured out quickly so that my surgery and visit do not get delayed and/or not covered fully by insurance! I will update again after I hear back from my neurologist and after we contact insurance about the Chiari Institute and the issue that I had last week with my medication.

General surgeon appointment!!!!

I got an appointment with a new general surgeon who is going to implant my port! I have an appointment next Thursday, 11/21, with a local general surgeon! This doctor should be able to get me into surgery very quickly if all goes well :) I am so relieved that this is done!!!!!

I had my orthopedic surgeon appointment yesterday and it went very well. I have had the same orthopedic surgeon since I was 7 and absolutely love him! He said my hand and wrist are very stable and that I can go back to everything I was doing before without any restrictions. The one thing he said is to ease back into signing (ASL) gradually. I asked him if the cartilage was likely to tear again since it has already torn once and he said that he took out the piece that tore so no. That was a relief to not have to worry about that. As we were leaving he said that the best advice that he has for me is to stay out of his office! Then he thought for a second and goes "I feel like I have told you that before...". I love Dr. Krpan :) This was a great relief as it is now one more health issue that I don't have to worry about anymore!

I also picked up my allergy serum for my shots, so I don't have to think about that anymore because it will last me until October or November of next year. I am not sure if I will need a yearly appointment in May with my ENT or not, but I am having no issues. I do have to do another round of allergy tests to see how my body is responding to the shots, but that is not until October of 2015. My allergies are so much better and my sinuses feel great! It has been over 1.5 years since my sinus surgery and I am still infection free :) I have NEVER gone this long without a sinus infection before!!!!

I went to pick up the new dosage of one of my medications, as I mentioned in the last post, and for some reason Humana wouldn't cover it. They said they had already paid for that prescription.... which is true, but it is only a 30 day prescription and I last had it refilled on October 11, which is obviously more than 30 days ago (I don't take it on the weekends). So now I have to call the insurance and figure out what is going on with that.

I will update sometime next week after my cervical collar fitting and general surgeon appointment.

Small updates

Well I still have no news as to where  we are at with the port implantation... I emailed my doctor, after being told they would call me, since when I talked to his secretary (medical assistant maybe?) yesterday she told me to directly email him. Well he has not gotten back to me yet, so I am hoping to hear back from him tomorrow. I am pretty sure that my surgery for next Thursday was officially cancelled though :( I just want to get this ball rolling already!!!! I will update after I hear back from my neurologist on this.

I have an appointment with my orthopedic surgeon tomorrow for a check up, so I will let you all know how that goes.

I found someone to fit and make the Miami J cervical collar that I need for after surgery! I have to drive about an hour away back to where my parents live to get it fit because I could not find anyone in the town I am currently living in who can do it from an external physician that is with my insurance. I have an appointment on Monday to do the fitting and then I will have to go back to pick it up later in the week. I am very excited to get this as I am supposed to wear it for car and plane rides and can also use it for symptom reduction anytime that I feel I need it.

I also have to stop in at my ENT (ear-nose-throat) office to pick up more allergy serum for my allergy shots. I am running pretty low so I am glad that that came in because it can take up to 6 weeks to come. I just ordered it 3 weeks or so ago, so it came quickly!

I got my dosage upped on my newest medication, Strattera. This is the medication that helps me focus and fight through the mental fog that us POTSies call "brain fog". I really like this medication and it has helped tremendously with school! We started on a super low dosage to avoid side effects and I have now gone from 10 mg to 18 mg. The only side effects I have been having from this are lack of appetite and constipation. Both of which I suffer from anyways and they are not too bothersome.

That is about all that has happened the past week or so. I should have more updates for everyone within the next couple of days or so :)

Appointments gallore

Wow what a week it has been! My family and I have started to get travel plans in order for our trip to NY for my surgery. That has been stressful in itself, but there are still a million other things I have to do besides that.

I had an appointment with the general surgeon who was supposed to put in my port on the 21st of this month and lets just say it didn't go as planned. He totally overstepped his boundaries in my opinion and decided that I did not need the port. He was very confusing and just kept talking in circles! Long story short he said he absolutely would not put the port in for me if I was going to do the infusions myself and that he didn't think i was "sick enough" to need the port. He doesn't know me or my medical history so it is not really up to him to decide that! I went straight to talk to my neurologists nurse who is PHENOMENAL!!!!!! She was not happy at all that he said that so we are working on fixing this little predicament. She did find a home health care company that will deliver my supplies and have a nurse teach me how to do the infusions and do them for me at the beginning at what not. We are waiting to hear if the surgeon will agree to doing to port now that we have this company to do it and has talked to my neurologist again. I am not sure if the port is still getting put in on the 21st of this month or not... I will update again when I hear back from my neurologists office.

I made an appointment with my orthopedic surgeon for a follow up on my wrist just to make sure everything healed well and that I am cleared to do everything again. I have had zero problems with this recovery so I am anticipating a full release from him until the next joint goes out :) I also made an appointment with my PCP (primary care provider) for pre-surgical clearance for my fusion surgery in NY. That appointment will be on December 12.

I was prescribed compression stockings which are basically tights that squish your legs really hard in order to send the blood back up to the brain from your legs. I was able to get those locally and hopefully my insurance should cover them which is wonderful. These will be really helpful for long days on my feet and flying to NY. I need to also get a cervical collar for the flight to NY, long car rides, and post surgery. This is a stiff collar that will immobilize my neck. I will have to wear it for a couple months or more after surgery. I just need to find someone who will fit it for me in my area.

I will update again shortly I think! Hope everyone is doing well!

The Chiari Institute!

I heard back from The Chiari Institute (TCI) earlier last week!!!!!! It was not the news we expected, but not at all in a bad way! They do want to see me for a consultation, but what we did not anticipate was that they offered me a surgery date before they have even seen me! This is not something that TCI normally does, so they must have seen something significant on my MRIs that I sent in!

I have a consultation with the nurse for a neurological exam on December 16th at 9:30 and then a consult with the neurosurgeon at 10:30 that same morning. At this consultation I will be able to discuss all of my concerns relating to my health. These are some of the leading experts on Chiari in the world so they will be able to understand all of my complicated medical issues and hopefully have suggestions to help some of them. I then go for pre-surgical testing and paperwork the next morning at 10 a.m. and surgery is scheduled for December 19th at 7:30 in the morning.

The surgery they are doing is called a craniocervical fusion. I do not know all of the details, but the neurosurgeons are going to put in titanium rods and screws to fuse my skull onto my first two or three vertebrae. This will be done because the joint between my skull and top vertebrae is unstable. My skull wobbles on top of my spine. This is a diagnosis that I have been expecting for a while, so it does not come as a surprise at all. I will then have to wear a hard neck brace for at least a couple of months until my bones fuse around the hardware they put in. I will loose some amount of range of motion in my neck, but at this point we are not sure how much since I do not have full range of motion as it is.

The hope is that this surgery will greatly help to reduce my symptoms. This surgery should reduce headaches, neck pain, ringing in the ears, swallowing difficulties, difficult holding up my head, and hopefully general fatigue.

The surgery will be another big one. It will be a six hour surgery, which is about the same as the brain surgery (decompression with duraplasty) I had last year. I will then be in the hospital for an estimated 5-7 days, although I am hoping it will be shorter! This surgery is slightly less risky because they are not opening the covering of my brain, but is still considered a major surgery!

I am overwhelmed by everything coming up with my health so prayers for peace and acceptance for my family and I would be greatly appreciated! I am not sure what this means for school for next semester because last year I had great difficulty going back to school full time after my surgery.

Dehydration and hypovalemia plus surgery number 5 (or 6 if you count wisdom teeth out)

Hi all, so I have been emailing with my neurologist all week working some things out. We switched around the times I take a few of my medications which led to a rough week because my body was so confused getting off of the normal medication system. I am doing better now, but I haven't noticed a great difference from before. My resting heart rate is a little higher which is nice because it causes less fatigue, but other than that we will just have to wait and see if it makes a difference.

I am not sure yet if the Strattera is helping my brain fog. He said it will probably be a week to notice and it has been about a week plus a couple of days now, but I haven't noticed a huge difference. Hopefully I will start to notice a difference shortly!

The biggest thing that has happened this week is that my neurologist and I decided to go ahead and administer IV saline fluids on a semi-regular basis. Patients with dysautonomia benefit greatly from IV fluids because even though we drink so much, I drink 1 liter of electrolyte fluids a day and at least 2 liters of water, we are still dehydrated. For some reason my body just simply is in a state of chronic dehydration. The other issue with this is that many dysautonomia patients have low blood volume, or hypovalemia. This is very hard to test for because the ratios of platelets in the blood to red blood cells is normal, but the over all blood volume is often very low. Multiple studies at Vanderbilt University, which is one of the leading research hospitals for POTS, have found that POTS patients have an average of  betwee a 12-20% deficiency in total blood volume.

We are looking into me administering the fluids myself at home. This would give me the convenience of doing them when I needed and wherever I needed. I would be able to take them on vacations or to my parents house whenever I needed. This may sound scary to some of you, but honestly I am hoping that it will help to increase the amount of time I am able to go out and do things: give me a better quality of life.

In order to administer the fluids easier and safer my family and I decided that I am going to get a port installed. This is a line that will go into a vein near my heart and have a small rubber and metal device sitting bellow my collar bone underneath the skin. This will allow me to access it at home with far less pain and difficulties. I have small veins that like to roll, blow, and hide. This means it is difficult to start an IV in me and I get bruises that last for weeks with a significant amount of pain. It will look something like this after it is installed:

And this is what it looks like from the inside:

I have an appointment with the general surgeon on November 5 to discuss the procedure and get all of the details that go along with the port and the procedure. I requested to have it done at Rush even though it can be done closer to my home because we felt more comfortable having a surgeon who has done this procedure literally hundreds or more times perform the operation. This doctor will also be able to communicate with my neurologist so they can decide on the best model to be used. It will take a very short surgery to install the port and we should be able to access it shortly after it is put in. Surgery right now is scheduled for November 21, but it can be moved up if a sooner date arises. I doubt this, however, because I wanted to do it on a Thursday because that is the best day with my school schedule and my fiance's work schedule.

I will update with more details after I meet with the surgeon on November 5.

Oh and I still haven't heard from The Chiari Institute yet :( I am hoping and praying it will be very soon that I hear from them!!!

Brain Fog Medication

Hey all, just a quick update. I finally got a hold of my neurologist yesterday. I need to email him directly instead of going through his nurse because I am confused on many of the things that he suggested. We have already tried many of his suggestions and either did not like them or the way I am currently doing is better.

Anyways, he has prescribed a non-stimulant ADHD medication to be used as an alertness aid. For people who do not have ADHD the medications used to treat it can help to wake you up, keep you more alert, and work through the brain fog. I am going to start on a low dose of a medication called Strattera. I am starting at 10 mg, and can go up from there if necessary. Hopefully this will help me have more energy and be able to concentrate better!!!

I will update again later this week after I talk to my neurologist and hopefully hear back from The Chiari Institute in New York!!!!

Sara is a... penguin?

Hi all! I had a very fun weekend two weekends ago! After a couple bad weeks, which were not good at all health wise, I decided I needed to go have a fun weekend! I went horse back riding with one of my best friends, Jessie. I have not ridden for at least a year and it felt FANTASTIC to be back on a horse. I have ridden horses since I was 8 and it is the one sport that I love! I went for a nice long ride on a wonderful little horse named Tucker. I have ridden him a number of times previously and he is incredibly sweet! He did great, I didn't fall off (which would have been VERY bad), and I had a blast. I even jumped him a tiny little bit which was great! I did really well that day and didn't hurt too badly. It hurt my lower back a little bit along with my neck and my ankle, but nothing too badly. I got dehydrated because I rode for a couple hours without fluids, which was a really poor decision! I did alright though and drank tons of my electrolyte enhanced water after the fact.

The next day my hips felt AWFUL! I could barely walk and my sprained ankle really hurt, but it was SO worth it. I waddled around like a penguin for two days, which was fun for the people around me to watch :) My neck muscles were also killing me! This is not a good sign, but also not unexpected. I am so glad that I got to go riding!

It is the small victories in life that count and this was a very exciting victory for me and I hope to be able to go riding again before winter sets in!!!

Fun Weekend... NOT!!!!

This weekend started out bad and just got worse, but thankfully it got better on Sunday!

I started on Thursday complaining that my heart rate was jumping up way too much upon standing up. The "normal range" is an increase of less than 30 beats per minute going from a laying position to an upright position. This is the goal we try to keep my heart rate in, with medications of course. I normally do pretty well with that goal and only increase between 10-20 beats which is really good for me and even considered normal! On no medications this increase can be up to a 60+ beat increase just from standing up. This weekend it was going up at least 35 and up to 51 beats simply from changing position and this was with my medication to lower/regulate my heart rate.

As the weekend progressed my blood pressure also plummeted! I have no idea why and I was extraordinarily close to going into the ER. I felt truly awful and no matter what I do I couldn't get the numbers to come up. Generally I push the fluids and salt and I can get the BP to come back up. Now keep in mind a lot of people have low pressure with no issues, but for me I get extremely symptomatic. Also, I take 2 medications and 2 supplements specifically to raise my BP, so when I have low pressure that is on top of all these efforts to raise it! My numbers were down to numbers like 91/53 or 85/60. Both of which are much too low for me! I felt extremely fatigued, nauseated, shaky, and generally icky! I don't know how to describe it, but it truly is an awful feeling!

Anyways, I have no idea why it happened or what I did to make it better, but it is gone now! I just took my blood pressure sitting down and it was 107/75 with a heart rate of 66 :) these are perfect numbers for me and I am feeling much better now!

I will keep track of my blood pressure numbers for a while and email my neurologist to see what his thoughts are. He has offered me another medication to add to my cocktail if I need it to raise my BP, but I am not sure I want to add in another medication! He has also offered me a stimulant to take to help to decrease the brain fog. I am thinking I need to take this one because school is becoming increasingly more difficult to concentrate because I can't seem to get through the mental fog to focus.

I will let you all know when I hear back from him. Still waiting and praying to hear back from The Chiari Institute! They review cases on Monday's so hopefully I will hear back tomorrow, but I am not hopeful. I would guess it would be another couple of weeks before I hear back from them, so please keep praying for me and my appointment!

The Chiari Institute

This week has been a really rough one! Yesterday in particular I really struggled to even function. I did not make it to class, and thankfully did not have work yesterday. I worked on a project with my partner for a couple hours and even with prescription pain medication and muscle relaxers I barely made it through! My headaches and neck pain are getting so much worse :( I am also having a return of many neurological symptoms such as aspirating (food or drink going down the air way instead of the esophagus), pin and needles in my feet, and ringing in my ears.

With all of these concerns and issues I am finally ready to admit that I need help again! I am struggling to take care of myself and do simple things like remember to eat, keep up with housework, get homework done, and meet the needs of my pets. I decided that the best route for me right now is to see a true expert. Unfortunately there are very few experts in Chiari, and even fewer that specialize in both Chiari and EDS along with the dysautonomia. There are really only 2 true experts for these conditions when they are as complicated as I have them and only 1 takes my insurance.

I debated whether to share this with all of you yet or not, but I can't wait! I sent in my paper work to a center called The Chairi Institute in Long Island, New York! They called me back and had me send in my MRI scans from after my surgery in December. This does NOT mean that I will get an appointment at this center. The physicians will review my scans and forms that I filled out and then decide collaboratively if they think that they can help me. They cannot take every patient so it is not guaranteed that they will take my case on, but I so hope they will!!!!! It could take about 2 or 3 weeks if not more fore them to get back to me. They only review new cases on Monday's so that is why it takes so long. I am hoping to hear back before the end of October...

I will update more later, but that is the big news of the week!