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!