Earaches, Appointments, and Good News!

My left ear started bothering me last Tuesday so I finally went to the doctor on Friday. I do not have an ear infection, but my ear canal is red and irritated. We are not sure what this is from but it is not the first time it has happened. I saw my new primary care provider who I absolutely love! I called his office at about 2:15 asking for an appointment for my ear and they got me in at 4:00!!!! He prescribed some ear drops that are like topical aspirin. They help to relieve pain and also soothe the irritation. They have been helping significantly and I only have to use them as needed, which is nice that they don't have to be done on a schedule. Hopefully the earache will go away soon, but last time this happened (about 2 years ago) it lasted for months and then spontaneously went away.

Last week I had an appointment with a company that supplies wheelchairs. I am going to get a manual wheelchair that I can use for long distances. It will not be something I will use everyday because you can become dependent on them and your muscles will atrophy, but on long outings it will be so nice to have! It should help me to be able to do more longer outings without needing days to recover from them. It could take anywhere from a couple of weeks to a couple of months depending on how much my insurance company drags their feet, so hopefully that will come sooner rather than later!

Yesterday I went to the audiologist to get ear plugs made for noise induced headaches. These ear plugs are custom fitted to my ears so they are far more comfortable than store bought ear plugs. They come with a little filter that decreases the sound level (decibels) around you without distorting the sound. The audiologist and I decided that we would use a 15 dB filter which will bring the sound level down enough that I can hopefully avoid those noise induced headaches, but will not interfere with my ability to actually hear and understand what is going on. The only issue I had with this appointment is that my ear canal is so irritated right now that it was very painful to get the molds done. They squirt some foam into your ear canal and then you have to let it harden for two minutes. I made it through though and should be able to pick up my ear plugs in about 10 days to 2 weeks.

I had asked my rheumatologist to write a letter for me asking the geneticist that I am on the wait list for to see me sooner than next summer, which is when we were looking at. Thankfully the geneticist's office called earlier this week and said they will get me on September 4th!!!! I am so excited for this visit and hope to learn a lot about my health and my genetics. Please keep my family and I in your prayers as this is also the appointment during which we will discuss the risks of future children inheriting my conditions. This geneticist is world renowned in EDS and I am very blessed that he is only about an hour away from where I live. This will be a long, informative appointment that will hopefully provide many answers for us!

My right wrist, the one I had surgery on last year, is bothering me again. It went from shooting pains if I moved it the wrong way to now hurting every time I move it. I have my splint on it and am taking an anti-inflammatory medication twice a day. I will do this for a couple of weeks and see how it feels. Hopefully at that point I can start doing some of the exercises for the occupational therapist and it will start feeling better. If not I will make an appointment with my orthopedic surgeon and probably need to get a cortisone (steroid) shot in the joint to reduce the inflammation and then go back to occupational therapy. Please pray that my wrist heals on its own and we are able to get it feeling better quickly!

We are still working on getting the new gastrointestinal medication that I was prescribed. Like I said in my last post it is not FDA approved so it can not be dispensed at a regular pharmacy (Walgreens, CVS, Jewel, etc.) like all of my other medication. We might have found a way to get it covered by my insurance, but we are still working on that. Please pray we are able to figure out how to get this medication in a safe, cost effective way and that it helps my worsening GI symptoms!

Updates!

Wow it has been way too long since I have posted an update!!! Lots of things have happened medically since I last updated.

After I was released from the hospital with those excruciating headaches my mom came out to my house for a couple of days to help me since I needed to lay flat on my back as much as possible to help the spinal leak heal. This was tremendously helpful and then I went back to my parents house for a few more days to get some more much needed rest. After this I just took it easy for another couple of weeks and am thankfully feeling much better! The added bonus is that I found a medication that I can take as needed for my headaches, so that is tremendously helpful as everything I had tried previously was not working. I need to hear back from my neurosurgeon about the MRI still, so I actually just sent another email as writing this blog post jogged my memory.

I had posted about my gastroenterology appointment at the beginning of July. I got the blood work done and all of those results were good which means that my liver is functioning well and that I do not have any signs of malnutrition as of now.  I then got the ultrasound of my gallbladder done. This is just like an ultrasound they would do if a woman is pregnant, but of your gallbladder which is located in the middle of your abdomen on the right side. This test is supposed to be painless, but sadly because of my connective tissue disorder (EDS) that makes me hypermobile my ribs kept dislocating from the pressure of the ultrasound probe. That was not pleasant, but we made it through and this test also came back normal showing no signs of gallbladder issues or gall stones. I then went for an upper endoscopy. This is a tube that is inserted into your mouth and then down your esophagus into your stomach and upper intestines. This test really was painless! They used my port, so I didn't need an IV even. They put you under twilight sedation, which is a lot easier on your body than general anesthesia and you can maintain your own breathing and heart rate. They just gave me some oxygen and the procedure took maybe 10 minutes total. They took biopsies to check for infections and celiacs disease. All of these came back normal as well.

I then had a follow up appointment with my GI doctor last week (August 6th). We started an acid reflux medication a while back and that has been mildly helping, but there are still issues going on. Sadly he doesn't know much about me and my condition and we are running out of options. He has diagnosed me with a condition called gastroparesis. This means that my stomach does not empty fast enough which causes my pain, low appetite, nausea, and bloating. I will start on a medication to try to speed up my gastric emptying time, but the issue is that this medication is not FDA approved so we need to find a safe, cost effective way of obtaining this medication. The other concern with this medication is that it can cause heart rate issues, blood pressure issues, and heart arrhythmias. I am prone to all of these because of my autonomic nervous system dysfunction, but I emailed my autonomic neurologist and he said that it is safe to try this medication as long as I get frequent EKGs to make sure there are no changes in my heart functioning. I am looking into finding a new GI doctor that understands gastroparesis better, but this is proving to be challenging!

I was able to pick up my second AFO (ankle-foot orthotic) which has been WONDERFUL!!!! I love them so much. They do not let me move my ankle at all which makes me steadier on my feet, prevents ankle injuries, and helps keep my body in alignment. My hips have felt so much better since wearing them because if your ankle is in the right position then all the joints above it are in better alignment as well. The laces that came with the AFOs were way too long so I ordered shorter ones that are purple, so I was excited about that :) Finding shoes that fit was tricky, but we finally found a pair of gym shoes and pair of sandals that fit well and are cute (and both purple of course!). I normally wear an 8.5-9 women's, but these shoes are between a 10 and 11 women's, so I had to go up quite a few sizes! I will get pictures of them later to show you guys, but they take forever to put on, so I don't want to do that right now :P

I had an appointment with a new endocrinologist in my area to discuss my thyroid issues. I have mild hypothyroidism that we had started medicating for a few months ago. Since starting this medication I had noticed changes in my menstrual cycle and was not really sure if the medication was actually helping. We decided to test my thyroid levels, stop the medication, and then retest in 8 weeks. This will tell us how much the medication was actually helping and help us to determine if I need to continue this medication or just keep an eye on things for now. I will go back the first week of September to retest my levels and then the following week (9/11) for a follow up with the endocrinologist. As of now I am not sure if this medication was helping or not. I struggled coming off of the medication with some low BP issues and fatigue, but I am not 100% sure if this was due to discontinuing the medication or not.

I was able to go on vacation to Florida with a few of my friends and had a wonderful time! We went to a Harry Potter convention and to the Wizarding World of Harry Potter. It was such a fantastic time and my body held up really well! I borrowed a wheelchair from a family friend so that I did not tire myself out and also did a liter of IV saline everyday to make sure that I stayed hydrated in the heat. I did really well and was able to make it through the convention and have a wonderful time! I hope I will be able to go back next year! I also was able to go horseback riding with one of my very best friends! I haven't been in so long and it felt WONDERFUL!!!! Horseback riding is my favorite thing to do and I love it so much. We had a wonderful time and I did really well. Of course my hips hurt the next day, but my back, neck, and head did really well which tells me that the past two surgeries I have had helped tremendously.

Since this is already such a long update I will leave it at that! I am sure there is more and as I get back into the swing of updating things I am sure I will remember other things I need to add. Love you all and thank you for the continued support and prayers!

Unexpected Hospital Admission

I have been having horrible, unbearable headaches since last Thursday. On this day I went out and did things outside of my home, and I think I pushed my self too hard. We went to the ER late Saturday night and the ER doctor decided to admit me with a suspected spinal leak. They did a lumbar MRI to check for a spinal leak, but we didn't get the results back until Monday. Thankfully most of the nurses (except for the last nurse) were absolutely amazing!!! The doctors not so much.... They didn't know what to do with me so I just kind of sat around until Monday. The MRI did show a collection of fluid, but not enough to make them think it was a large enough leak to do an intervention on. At that point we were just doing symptom management and decided to send me home. It was a wonderful hospital room and almost felt like a hotel, so thankfully it was not too bad of a stay.

My neurosurgeon is hopeful it will heal on its own with bed rest and lots of hydration (IV and oral). Although it was frustrating that the hospital wouldn't do anything, looking on the bright side they were able to control my pain and nausea well. I was sent home with two different pain medications and have a prescription for nausea medications already.

My surgeon also suggested I put gauze on my incision and than put an ace bandage around my abdomen/pelvis to provide extra pressure on the wound. One of the pain medications I picked up is called Fioricet, which is specifically for headaches and has been working quite well in combination with my muscle relaxers.

The hospital refused to remove my stitches (long story I don't agree with), so I have an appointment tomorrow morning to get that done. Besides that, I have an appointment with a new endocrinologist next Monday, so I need to get all of my records (lab work, ultrasounds, etc) sent over to her office. Please say a prayer that she is the right fit for me as going to new doctors makes me anxious. I also got one of the two prescriptions that the GI doctor prescribed filled. This medication is meant to be taken twice a day, so please pray that that works!

Gastroenterology Visit

The other day I had an appointment with a new GI doctor. Digestive issues are something that I have struggled with for many years, but we have never been able to find any answers for why. I had a bunch of GI tests ran about four years ago, but that GI doctor just said it was irritable bowel syndrome because he couldn't figure out what else it would be.

I first met with the GI doctor's resident who was very sweet and knowledgeable. He took down all of my symptoms, asked me what I had tried in terms of medications and diet modifications, and what my main concerns were. He then went and spoke with the doctor and they came back in about 10 minutes later.

I had stated that my main concerns at this point are nausea and nutrition/lack of appetite. He knew about my autonomic dysfunction and connective tissue disorder (EDS), so we discussed how those can effect my digestion. He wants to be careful that we don't just assume my digestive issues are from these chronic conditions and miss another potentially harmful issue that is separate from my other conditions. For that reason he is ordering an ultrasound of my pancreas to rule out any issues with it and an upper endoscopy. I still need to schedule the ultrasound, but the upper endoscopy (EGD)is scheduled for July 17th. It is a quick, painless procedure during which they put a camera down your throat to look at your esophagus, stomach and small intestines. While he is examining these structures he will also take biopsies to check for a variety of conditions including celiacs disease and infections.  It is done under twilight sedation which means that you are sedated enough to not remember anything, but are still breathing on your own. The test itself will only take about 10 minutes max.

I also got blood work done when I was there to check my nutritional status and a couple of other things. The lab at Rush wouldn't draw through my port, but I went up to the oncolocy and hematology floor and they were nice enough to do my blood draws through my port even though I am not one of their patients. I have not gotten the results back from those tests yet due to the delay caused by the 4th of July.

The doctor ordered me two medications: an IV nausea medication and an under the tongue, dissolving acid reducing medication. Sadly, I am having some problems with insurance for both of these, so I haven't gotten either one yet. Hopefully we will be able to figure out these things this week.

After all of these tests are completed and the results from the biopsies taken during the EGD come back I will have another appointment with the GI doctor and we can discuss what his findings were and what kind of interventions he will do.

This week I so far only have an appointment with a primary care physician on Monday to remove my stitches from my tethered spinal cord release. This should be quick and easy as well.

Tethered Cord Release Update

I am doing really well since my tethered cord release last Tuesday. I have no pain at all from it! I only took prescription pain meds for about four days and haven't needed any since then. I still occasionally take muscle relaxers for the muscle spasms, but not very frequently. The only post op issue I have is that I can't bend over yet. It is still too tight to bend and I am not allowed to lift anything over 5 pounds for a couple of weeks yet. I also need to lay flat as often as possible to prevent a spinal leak. Since the covering of the spinal cord was opened there is a high chance of developing a leak.

Post op I did very well in general. I started the prescription potassium pills because my potassium is low. I haven't noticed a difference symptomatically since starting that medication, but low potassium can be extremely dangerous (causes cardiac complications), which is why we monitor my electrolyte levels frequently. I just got my electrolytes checked via a blood draw today (not for my autonomic doctor, but for GI which I will write a separate post about), but I haven't gotten the results from that back yet. I will need to get my electrolytes checked again next Friday to see how I am responding to the prescription medication.

One issue that I have had before with anesthesia is that my close up vision gets blurry. I always have problems with distance, which is why I wear glasses, but reading is never an issue. For some reason anesthesia makes my vision blurry for reading. Thankfully it only lasts a couple of weeks and then goes back to normal. It is nearly gone now and is really just more of an inconvenience than anything.

An issue I had this surgery that I never had with my other surgeries is a dislocated jaw. From the moment I woke up I said that the inside of my left ear was really hurting. No one new why as there was no obvious marks or redness and the way I was positioned during surgery should not have caused that discomfort. Once I started trying to eat and realized it was painful to chew is when we realized that it was my jaw that was in pain, not my middle ear. I went back to my surgeons office after I had been discharged and he checked on it and said that it will just heal on its own. Thankfully it is only a little bit sore now and it seems like it should heal up just fine.

I go on Monday to a primary care physician to get my stitches removed. We opted for non-dissolvable stitches this time because my body does not dissolve them the way most people's do.

This is my incision right now. It is on the very bottom of spinal column and appears to be sunken in a little bit. This was taken right after surgery, so I will get another picture after the stitches come out. It does look less red and irritated now than it does in the picture.

I will write another update very soon on the GI appointment that I had today. Thank you all for your continued prayers and support!

Tethered Cord Release Surgery and Recovery

Surgery was scheduled for 8:30 on Tuesday morning, so we had to arrive at about 6:30 in the morning. I was only allowed to take my most important medications (fludrocortisone, Midodrine, and synthroid) that morning, which of course made me nauseated because I can't take medications on an empty stomach. We got there and checked in very quickly I got changed and then went through all of the questions that the nurse asks you. Sadly, I had a very rude nurse! They always get mad at me for not starting an IV because I would rather use my port. I told her that I was not going to let her start an IV until after I spoke with the anesthesiologist, who said he would use my port and would only need to start on peripheral IV since they need two access points. The nurse got very rude then and told me that my doctors were wrong and that I needed to let her start it. I still refused and I am glad I did because then I only had to get stuck once, I was already under anesthesia when they put in the peripheral IV, and they took my peripheral IV out as soon as I got out of the recovery room because they still had my port that they could use for fluids and medications. It was nice to get it out for two reasons: one it was causing discomfort and pulling every time I moved and two I sleep on my side curled up in the fetal position which tends to set off the alarm because my IV gets kinked.

After the debate about my port all that we had left to do was wait. I signed a couple of release forms and talked to my anesthesiologist and my neurosurgeon briefly. At almost exact 8:30 they took me back into the OR. For some people this is intimidating, but I like it. Everyone is always so friendly and kind! They explained to me how I would be positioned and and what would happen during the surgery.  After the resident gave the all clear they gave me a sedative (not even the anesthesia yet) and it took about 15 seconds to kick in and I don't remember anything after that!

From the point that I received the sedation until I woke up in the recovery room was a total of just under 4 hours. During this surgery they made a four inch long (approximately) incision in my lower back. The surgeon then removed part of my lower vertebrae (called a laminectomy) in order to gain access to my spinal cord. He then found the fatty pieces of connective tissue (called filum terminale) that were pulling my spinal cord down and attaching it to the spinal column. After he separated these fibers from the nerves he then carefully cut each one in order to relieve the tension on my spinal cord. After this he closed up my incision and I was sent to recovery!

Recovery was pretty smooth. The pain was well controlled through IV medications as well as IV nausea medications. I am prone to vomiting from anesthesia, so I was very happy that they were able to control my nausea. The recovery room nurse was extremely nice and helpful! We were there for a few hours until a room opened up for me on the neurological floor. Thankfully I did not need to go to the neurological intensive care unit for this surgery. My pain was well managed and it was a big difference to not have had surgery on my skull/brain! It is much more intense pain when my upper spine was involved than my lower spine. I as getting an IV pain medication, IV antibiotics, IV potassium, and IV phenegran (nausea med) for the first night until I switched over to oral medications.

The first nurse and the night nurse on the neuro floor were both very nice and sweet! The only issue we had is that my neurosurgeon specifically told me that I did NOT have to lay flat on my back after the surgery. Some surgeons require this, but my surgeon does not. I was fine until about 9 or 10 at night when the nurse told me I needed to be flat on my back for complete bed rest. At first I refused and they called a whole bunch of people and they all said I needed to be flat. Unfortunately I lost that battle and they put my bed flat. Up until that point I was doing ok, but I was in tears after they did that because the pain was awful! They felt so badly for me that they at least let me sleep on my side. When we spoke to my surgeon the next day he was really mad that they put me through that when he specifically said it was not needed. Other than that the night went well, except for the fact that you don't get a lot of sleep. My room was right outside the nurses station and they were LOUD and very disrespectful to those of us trying to sleep. I did not like the day nurse that I had Wednesday. She was rude and not helpful at all! Thankfully I was released that day, so I only had to deal with her until lunch timeish :)

Sorry for the delay in posting an update! I will post another update with my progress since being discharged. I have a very important appointment with a new GI doctor on Thursday, so please pray that that goes well and he is able to figure out why I have been having so many gastrointestinal issues!

Ronald McDonald House and Pre-surgical Appointments

 We got into the Ronald McDonald house we are staying at early Sunday evening. The flight went really well and we flew out of Milwaukee instead of Chicago, so that saved us a lot of time and stress! It is just over a two hour flight during which, my husband and my mother slept most of the time and I read a really good book! The only part of the flight that was difficult for me was the landing. It still hurts my neck to get jared around that much, so I will need to remember to wear my collar more on airplanes. Once we got our luggage we went and got a taxi to the Ronald McDonald house, which is about 30 minutes away from Laguardia (one of NYC's airports). It took a significant amount of time to check in, but once we did it was so wonderful! The people here are absolutely AMAZING and so kind. We have our own private room with: two double beds, a desk, a TV, a night stand, and a rocking chair, our own personal bathroom that even has a tube, and there is a community kitchen that comes with a fully stocked pantry and a fridge with left over dinners and other various odds and ends. You can also buy food and store it on individually labeled shelves in both a fridge and a dry goods shelf. A company, family, or organization brings in food every night for the residents to share with their families. One night there was sandwiches, another was pasta, and tonight is going to be chicken I believe! Girl scouts were here the other day baking cookies and cupcakes which was extremely sweet of them! We all love it here! It is so quite, clean, and everyone is so very kind! It is the perfect place to stay!

On Monday morning we went to the hospital for my pre-surgical testing. They just ask me a bunch of questions to make sure that I am healthy enough for surgery. I answered all her questions, filled out the paper work that I needed to, signed the consent forms, and had all my vitals checked. This all looked good, so they just need to draw blood. As some of you may know I had some difficulties getting that hospital to draw blood out of port when we were there for my surgery in December. It seems as if they had a meeting to discuss why this was such an issue, which they told me there were going to do, because it went very smoothly! They did the blood draw through my port with zero issues and it was quick, effective, and painless!

All the results of those results from my blood work were normal except for my potassium. We check my potassium every couple of months because one of the medications I am on to raise my blood pressure, Florinef (also called Fludrocortisone), can deplete potassium. Ideal potassium levels should be between about 3.7-5.2. On Monday during my pre-surgical appointment it was 3.2 and on Tuesday when they tested it my potassium level went down to 3.1. They then checked it again on Thursday and it had come up to 3.7 after doing three bags of IV potassium. I emailed my neurologist and he wants me to get my potassium blood work done again when I get home and then start on a prescription for potassium for two weeks then we will check my labs again.

I warned the nurse about my nausea and vomiting so she could take note of that and also asked her to make note to put some squishy pads under my knees, hips, and ribcages so that I did not get gigantic bruises after the surgery. They did end up doing this and it was wonderful!!!

After this we then walked a short ways to my appointment with my surgeon at the Chiari Institute. His nurse came in first and asked a whole bunch of question regarding my symptoms due to tethered cord. These questions regard the lower half of your body including: bowel and bladder function, the pelvic region, legs and feet. Some areas have pain, some do not work efficiently, some are numb or weak, and others still are just fatigued. We waited a little while and then my doctor came in. It was a much shorter appointment than last time because I had already seen him and had a neurological exam with him. He said he is hopeful that this surgery should help me greatly with the lower half of my body. It does not help everyone and there is a chance that I will not improve, but he said for my personal case he is hopeful that it will elevate some, if not all, of my lower body symptoms.

He said that the surgery would be about 3-4 hours long and I would not need to go to the ICU (intensive care unit) after the surgery. We estimated that I would be in the hospital about 3 days. After that I just couldn't have anything to eat or drink after 11 pm and had to wash my body with a surgical scrub.

New York Preparation

Last week was crazy with everything that needed to be done before we left for New York on Sunday. I had lots of appointments last week and even more phone calls to make!

My New York Neurosurgeon ordered a CT scan of my cervical spine (neck) to check on my fusion. I got the prescription via email from him and scheduled the CT scan right away for a few days later. Unfortunately for some silly reason they would not take that prescription simply because the doctor prescribing it was out of state. I then had to go get a prescription from my neurologist in Chicago in order to get it done in time for New York. Thankfully this ended up working out! I got the scan done and then waited to get a disk to bring with me for my neurosurgeon to look at.

I then went to the podiatrist to get my left AFO (ankle-foot orthotic) casted and to get a checkup done on my other AFO. Like I said in my last time I updated I LOVE my AFO. I can't wait to pick up the second one in 2-3 weeks! I do not even have to make an appointment with him to pick it up; I will just drop by anti me after it comes in to pick it up. I do not need to continue seeing him unless there is an issue that arises or I have any questions.

The final appointment I had was with a new primary care provider (PCP).

AFO's, Endocrinology Upadate, Sinus Infection, and Autonomic Neurology Follow Up

So I am just a little behind on updating! I have been very busy as my husband and I just moved out of our apartment into our very first house a couple of towns away. We are now much closer to his work and in a bigger city than my college town. Of course the closing to the house did not go as smoothly as we had hoped and there were numerous things we had to do before we could officially close on the house and finally move in. We were able to get into the house a week ago and are still working on unpacking. We also did not have internet until Wednesday, so that made doing an update difficult as well. I have no work today and am having a lazy day at home with no appointments, so I figured I would take the time to do a long update on all of my medical changes and appointments that I have had in the last month or so. I will also try to get to an update on New York and my upcoming tethered cord release surgery shortly.

One of the last updates I posted was about my appointment with a podiatrist. He had casted my right foot for an ankle-foot othotic (AFO). It took about 3.5 weeks for my AFO to come in since they get them custom made in Arizona. I have had it for 3 weeks now and I absolutely LOVE it. At first it was difficult to walk, drive, and use stairs in, but I have gotten used to it and actually prefer wearing it. Driving was particularly difficult because I cannot bend my ankle at all in the AFO. I have to use my thigh muscle to push down my entire leg instead of flexing my ankle and foot. Stairs were the hardest for me though! Going up stairs was ok as long as I used the railing, but I kept getting my toes caught on the edge of a step and trying to fall down the stairs. I have finally figured it out and can now go down the stairs without tripping! The AFO has helped not only my foot and ankle to be in the correct position and prevent injury, but it also has made my knee and hip feel better. When you stabilize a lower joint it helps to take stress off of the upper joints. This is a great benefit and I cannot wait to get my second AFO done on my left foot. Shoes have been a challenge with my AFO though... I normally wear a 8.5 normal width shoe, but with the AFO I need a 10.5-11 double wide shoe! I got some sandals from Kohls, but with the address change from the move they have yet to get here. I would also like to get some gym shoes/athletic shoes, but neither Payless or Shoe Carnival has any wide enough. I think I am going to try ordering some extra wide shoes from Zappos online. It is also difficult right now because I do not have an AFO on my left foot, so I need two different shoes. I have just been shoving my right AFO into my poor size 8.5 gym shoes. I took out the insole that comes in the shoe and unlaced it part of the way, so this has given me just barely enough room. I go next week on Wednesday back to the podiatrist for a follow up on how I am doing with the AFO I have and to get a mold made for the left AFO. It will be about 3-4 weeks after that that I will get the other AFO.

I had a follow up appointment with my endocrinologist after I had started my thyroid medication. It takes about 6 weeks for the levels in your blood to even out enough that the blood can be tested to see if the current dose is too high, too low, or just right. It turn out that my current dose is working well, so I will keep on this dose for another 3 months and then go back and get retested. I have had more energy with this medication and have not been quite so cold (which is a sign of underactive thyroid). The only issue I have been having with this medication is changes in my menstrual cycle. This is starting to become problematic as I have multiple symptoms of pre-menstraul syndrome (PMS), but my actual menses is extremely delayed. Hormonal changes make my autonomic dysfunction symptoms worse, so this is the reason this particular symptom is affecting me so much. When we get back from New York for my surgery I will make an appointment with an endocrinologist in my new home town to address this issue. I do not want to have to drive nearly an hour to see the endocrinologist I was seeing since I was not overly impressed with him anyways.

The other large medical issue I have had since I last updated is that I had a sinus infection. This is the first sinus infection that I have had since my sinus surgery two years ago. I got some sort a virus (flu, cold, or something) and I recovered from that some fine with the help of a couple extra liters of IV saline so that I did not get overly dehydrated, but then about 9 days after I had first gotten sick I was still blowing green/brown mucous out of my nose and still had a shallow wet cough. I also had sinus pressure and pain, particularly on the right side of my face. I went to the immediate care since my ENT is about an hour from our old apartment. I wish I would have gone to the ENT, because it was difficult to get the urgent care doctor to listen to me! I have had so many sinus infections in my life that I know exactly when I have one and how to treat it. She agreed that I had a sinus infection, but did not want to give me the antibiotic that works for me. Most doctors us an antibiotic called Amoxicillin to treat sinus infections. This antibiotic does not, and never has, worked for me. It does not get rid of the infection and is useless for me to take. Well, she had difficulty believing this, but after a significant debate I convinced her to give me an antibiotic called Augmentin. This antibiotic is stronger, and therefore has a higher risk to cause serious GI side effects, but it is the antibiotic that works best for me. In the end she got tired of listening to me, so she wrote a prescription for a 10 day course of Augmentin twice a day. I started to feel better about 2.5 days into the course of the medication and by about 5 days on Augmentin I felt back to normal, though you always have to finish a course of antibiotics when they are given to you so that the bacteria does not grow back and become resistant to antibiotics. I am hoping that switching my allergy shots to every 3 weeks instead of every 2 weeks did not allow my sinuses to get irritated again. I don't think this is the case because I have not had an increase in allergy symptoms, but we will see. If I get another infection I will make sure to get an appointment with my ENT so we can get a CT of my sinuses and see what is going on, but for now we are going to just hope it was a fluke and I go another two years without an infection.

I called the geneticist that I am on the wait list for that is an EDS expert out of Chicago (well Park Ridge technically for those of you in Chicagoland) as I have been on the wait list for 6 months and have not heard about scheduling an appointment yet. The woman I spoke with said that they are no where near being close to scheduling an appointment for me and that it will probably be next summer before I get an appointment. From everything I have heard this doctor is worth the year and a half wait, but I am not a very patient person so waiting is not my favorite thing to do! I will call back in December when I will have been on the wait list for a year and see where we are at then.

I ended up cancelling my follow-up appointment with my orthopedic surgeon for my wrist. He is over an hour away from my old apartment and it was during finals week. My wrist is back to being absolutely pain free. I now how exercises and tools from the occupational therapist that I can use if my wrist starts acting up again. I also have a prescription anti-inflammatory medication from my orthopedic surgeon that I can take when any of my joints get injured or are acting up.

Overall, I have not been feeling overly fantastically recently. I saw my autonomic neurologist yesterday just for a follow up. I see him about 3 times a year just to make sure that he can do a neurological examination and other physical examinations to make sure nothing changed. We are going to leave all of my medications the same for now since they are keeping my vitals stable. He said a probable reason for not feeling well, aside from the stress of the move, the upcoming surgery, and my wedding in just over a week of course, is the weather. If you live in Chicagoland, or any part of the Midwest for that matter, you know that the weather is extremely variable right now! It keeps going from hot to cold, from rainy to sunny, and then back again! This messes with my autonomic dysfunction as well as my pain levels. My head in particular and my blood pressure just have not been cooperating. I hope that the weather will start to settle down soon so that I start feeling better!

I have an appointment with a gastroenterologist on July 3rd right after we get back from New York. I am hoping that he will be able to help me sort out my nausea, reflux, constipation, and stomach pain. He was recommended to me by another POTS patient, so I hope he is as good as she thinks he is!

Sorry for such a long gap in updates! I will try to update more frequently and I will absolutely make sure to make frequent detailed posts about my appointments and surgery in New York. I will make a separate post shortly to update on the preparation for New York.

Graduation and Moving

Hi everyone! I am way behind on updating and have a few new medical things to update you on. I will get to that very shortly hopefully! I just wanted to share a couple of personal developments first!

Most excitingly, I graduated from Northern Illinois University with my Bachelors degree!!!! I graduated in 3 years with a final GPA of 3.891! I had 6 surgeries during my 3 years of college, so this was a HUGE achievement for me. I felt very poorly on the day of my graduation ceremony, but I have AMAZING friends and family who helped me to make the day as easy and stress free as possible. I sat with a few friends during my ceremony which was really nice to get to sit with people I have gone through this experience with. My ceremony was in the evening and I got home and went straight to sleep, but I had also been able to go out to lunch with a group of ladies who have Chiari Malformation (my brain malformation) that morning. It was so nice to be able to go hang out with so many wonderful people who all understand what I am going through! It was also wonderful to be able to speak with a few younger women who will be starting college soon. It is always amazing to be able to share advice and inspiration for others who will be entering the stage of life I just finished!

Here is a picture of my husband and I after my graduation. You can tell I did not feel well, but I am incredibly proud of my accomplishments!

The other thing that has been keeping me so busy right now is that my husband and I just bought our first home! We are moving out of our apartment by my old university and moving about 45 minutes away closer to where my husband was transferred for his work. It is a LOT of work packing up everything and it has been exhausting for me! We are moving this weekend, so we are in the final stages of trying to get all of our belongings into boxes. It will be nice when we get there, but right now it has been a lot of work to get everything squared away for closing and moving. I am sad to say goodbye to my wonderful job as an after school tutor at the local middle school, but hopefully I will find an equally wonderful job in the new town we are moving to!

I will write a blog post on my medical updates, but I just wanted to share my life updates with all of you!

Date Change for NY

We decided to change the date of my tethered cord release to June 24th. We will have my pre-op appointments on June 23rd. This will make it so that we are in NY for a much shorter amount of time. This helps drastically financially and also helps tremendously with scheduling because my husband doesn't have to take as much time off of work.

I just wanted to update everyone on the the date change. There is not much else new with me right now. I have a follow up appointment with my orthopedic surgeon  on Thursday for my wrist. After doing occupational therapy is it feeling back to normal, so I have no concerns for that appointment.

Podiatry Appointment

When I saw the rheumatologist who specializes in EDS a few weeks ago she suggested I see a podiatrist for my hypermobile feet and ankles. The hope was that this would help not only my feet and ankles to have less pain, but also that my calves, knees, and hips would have less pain as well.

I saw a local podiatrist and he was absolutely wonderful! He was amazed at how hypermobile my feet and ankles are, but yet how tight my muscles were. This is very common in EDS and many joints all over my body. The connective tissue is so loose and hypermobile that the muscles tense up to hold the joints in place. The first thought that we had was to put custom inserts into my shoes. The front, middle, and back of your feet all move separately, so with a custom insert he is able to adjust to the different needs of each part of my feet. I have high arches in the middle, but they collapse towards the back of my foot and roll outward. Well the doctor's assistant called my insurance and they would not cover these inserts. They cost upwards of $600, so obviously I was a little unhappy with that option. I then brought up that the rheumatologist had more of an ankle-foot orthotic (AFO) in mind, instead of just the foot orthotic. Thankfully he thought this was the right path to go for me and my insurance DOES cover these. This will be a brace that goes up to the ball of my foot and extend up above the ankle. It will include the foot support that the shoe orthotics would have, but it also holds the ankle in place so that I do not roll it and sprain it so often. The type of AFO that this is will not cause dependence or weakening of my muscles because I am still using my own muscles, this is just supporting me. He recommends that patients start with one foot at a time so that your body can get used to walking with the AFO on. I decided to do my right foot first since that is the one that gives me the most problems. The doctor's assistant took a mold of my right foot using the same material that is used for orthopedic casts (such as if you broke your wrist), but far fewer layers. We just waited for it to dry and he was able to cut it off. This will now get sent to Arizona where they will make an AFO for me. This will take about 3 weeks and then I will be able to pick it up. After I pick it up I will wear that AFO for about 4 weeks and then go back in for a follow up appointment. If I like it and am happy with the results we will then go ahead and go through the same process for the left AFO.

As I mentioned the doctor was alarmed at how tight the muscles in my calf are. There are two groups of muscles in the calf: one, called the gastrocnemius, is higher up towards your knee and one, called soleus is down lower towards your ankle and runs underneath the gastrocnemius. The gastrocnemius is the muscle that is causing me tension and pain. To remedy this the doctor is having me place a non-stretchable item, such as a towel, under the bottom of my foot while I am laying on my back and pull it upwards so that my foot is flexed towards my head. This is then held for 15 seconds and then repeated four times for each foot. I have to do this twice a day: in the morning and at night. This will help gently stretch the calf muscles out so that they are not so tight. In addition to these stretches the doctor sent me home with a night splint for my foot. This is what it looks like:

 

It looks really big, but it is not that bad. It is not tight enough that it holds my foot firmly in place (I can still move my foot around). It is simply meant to keep my ankle at a 90 degree angle. When you are relaxed your feet naturally drop down and for most people this is fine, but since my muscles are over compensating we need to keep my foot at a 90 degree angle to stretch out those muscles. I wear the splint on one foot one night and then switch it to the other the following night. This gives my muscles a chance to relax every other night so that they are not stretched to the point of injury. I wore the splint on my left foot last night and it is definitely a little bit sore, but nothing unbearable. 

Back to New York

So like I said in my last blog post I've got some big news regarding my neurosurgical care. We are headed back to The Chiari Institute in New York for a tethered spinal cord release surgery. This is why I had to see a urologist and get those tests done.

The urologist did ultrasounds of my bladder and kidneys and a urine test. All of those came back completely normal, which is great! He then did a urodynamics test which studies how well your bladder fills and empties. He claimed this test was normal, but my neurosurgeon and I do not agree. They fill up  your bladder and the amount he put in my bladder was normal, but the output was nearly twice what went in. Obviously this is going to screw with the results of tests, but he didn't want to discuss that... We decided that even though these results are normal on paper that we are going to proceed with the surgery.

A tethered spinal cord is a condition in which the spinal cord is attached to the spinal column with a piece of fatty connective tissue at the bottom of the spine. The spinal cord is supposed to be free floating and able to move up and down freely. This piece of fatty tissue is stretched out over time to the point that it cannot be seen on an MRI. For this reason, it is called occult (or hidden) tethered spinal cord. This causes a progression of symptoms such as low back pain, pain in the legs, feet, and pelvis, decreased sensation in those areas, and urinary and/or bowel issues. Since these symptoms are progressive and irreversible it is highly recommended that surgery be done sooner rather than later.

This surgery will be slightly less lengthy than my previous neurosurgeries. It will take about 3-4 hours hopefully. They will open up my spinal column and cut any filums that are attached to the spinal cord. It is really that straightforward and unless they run into any concerns then it is a relatively low risk surgery. The recovery is also fairly quick. I will be in the hospital for 3-4 days. I have to lay completely flat because the biggest concern is that I will develop a spinal leak.

The surgery is scheduled right now for June 3rd and my pre-op appointment is May 29th. This might change though due to scheduling reasons, so that we do not need to stay in NY for so long. I am hoping that we can do something so that they pre-op appointment and the surgery itself are closer together.

General upate

Hi everyone! There are lots of little updates on how I am doing, so I figured I would make a general update post and then another post for a specific topic I will need to go into more detail about.

I'll start with a quick one first. I had an appointment with my ENT (ear, nose, and throat specialist) today for my 1 year allergy shot check up. I have to see him once a year just to make sure that everything looks good with my sinuses, ears, and throat. Everything looks great and he has no concerns for me! He wants me to try to space my allergy shots out to every 3 weeks instead of the every 2 weeks that I am currently doing. This will help my body to build up it's own immunity to my allergens (which is the goal of allergy shots), so hopefully in another few years I can get off of the shots. I have only been on them for 1.5 years and he said we will evaluate if we can discontinue the shots after 4 years. The only question I had for him was about how one of medications interacted with the allergy shot because when I had done some research I read that this class of medications (beta-blocker) can be contraindicated in allergy shot users. He said that this used to be a concern, but the research has proven that they are not an issue and that we don't need to worry about it at all! I also got a new prescription for epi-pens, since mine are expired (whoops!). Anyone who uses allergy shots is required to have epi-pens in their home just in case they have a severe allergic reaction to the shots, which can happen since the shots are a concoction of all of the things that you are allergic to.

My wrist is doing really well! The pain at rest has completely cleared up and the shooting pain when I lift something or push on something is greatly decreased. My exercises are going really well at home and I have been able to discontinue some of the more basic exercises that we were using at the beginning. We have decreased therapy to once a week instead of twice a week. I have an appointment with my surgeon in 2 weeks so hopefully we will get the all clear from him! I got a new brace as well that my therapist recommended. It is to provide compression and support only, not immobilize like my other ones do. I need to wear it when I am doing a lot of activity or heavy lifting. It is very comfortable and not as restrictive as my other braces, so that is nice!

I started on the vitamin C supplement that my rheumatologist suggested I try. So far I have not had any negative reaction to it, which I was nervous about since my mom reacts negatively to large doses of vitamin C. I haven't noticed a huge difference yet, but most patients say it is a gradual difference that occurs over a longer period of time, so hopefully it will help in the long run. I forgot to mention in my post about the rheumatology appointment that she recommended that I order a book called "Joint Hypermobility Handbook- A Guide for the Issues & Management of Ehlers-Danlos Syndrome Hypermobility Type and the Hypermobility Syndrome". It is written by the geneticist that I am on the wait list for, Dr. Brad Tinkle, and is supposed to be excellent. I am going to order it and will hopefully get some good insight from it! I have an appointment with a podiatrist on Monday to discuss getting fit for ankle foot orthotics to stabilize my hypermobile ankles. I am looking forward to this appointment and hope it will really help me with ankle, knee, and especially hip pain. Maybe it will even help with my lower back pain!

I got a call with the results from my thyroid ultrasound back today as well and that came back clear. This is great because we were concerned that the endocrinologist thought that there may have been a nodule on the right side. Overall, I am doing really well on the new thyroid medication. I am sleeping better and waking up easier. I have more energy in general, which is really nice! I go back May 21st and we will retest my blood levels to see if we need to increase the dosage at all or if I am in normal levels on this dose.


Like I said I have a few appointments coming up, so I will keep you all up to date with how those go. I also have a blog I need to write on the next step in my treatment neurologically, so keep an eye out for that!

EDS Rheumatology Visit

I met with an Ehler's-Danlos Syndrome (EDS) specialist for the first time on Monday. She has only been working with patients with EDS for a couple of year, and admittedly still has a lot to learn, but she is doing research and learning as much as she can! It was a good visit and I got a lot of good information. We talked about how EDS affects me and what my main concerns were regarding EDS. The main thing we discussed at this appointment was pain management. Thankfully I do not have severe joint and muscle pain as a result of the EDS, at least not yet I don't as pain in EDSers tends to get worse as you age, but it is still enough that at times I am quite uncomfortable. She refilled my prescription of an NSAID prescription pain reliever called Celebrex. This medication is similar to Aleve, but is stronger, longer lasting, and easier on the stomach. She also mentioned some options we could try for localized pain like a pain relieving gel or a lidocaine patch, but we are not going to start those yet.

She wants me to see a podiatrist to get ankle foot orthotics made that will stabilize my hypermobile ankles.  My ankles tend to roll easily even when I am walking on flat surfaces and we are hoping that if we stabilize my ankles this will help my knees and hips as well. My hips have been causing me increasingly more problems, so hopefully this will help! I haven't made an appointment yet, but it is on my to do list!

Some individuals with EDS can have a decreased bone density, which can cause major problems later in life. My rheumatologist recommended that I get a bone density screening just so we know if this is something we need to be cautious of as I age. I show no signs of low bone density, but it is important to know if low bone density is present. I was able to do the bone density scan that day and it only took about 8 minutes. It is almost like an open MRI machine that scans your hips and spine and then analyzes how dense the bones are. I got the results a few days later and thankfully my bone density is slightly above average for my age. I will continue to take the calcium supplement I am on (since I do not drink milk or eat a significant amount of dairy) and the vitamin D supplement I am on (since I am vitamin D deficient) and hopefully this will be enough to maintain my bone density as I age.

The final thing that this doctor recommended is that I start taking 2000 mg (or 2 g) of vitamin C everyday. Vitamin C will do a few different things for me. First, as most people know vitamin C boosts the immune system which is crucial for me because if I get sick it is very difficult on my body and I take a lot longer to get over an illness than most people. Second, vitamin C helps to heal bruises quicker and reduce inflammation. I bruise extremely easily because of EDS and my bruises tend to last for a long time. Finally, vitamin C has recently been found to aid in the production of collagen, which is what is defective in EDS. It is not known how taking vitamin C affects EDSers, but there has been some preliminary research and various case studies that suggest that it may help to reduce some of the symptoms of EDS in some patients. I haven't gotten to the store yet, but I am going to at least give this a try! My mom did warn me though that when she was younger she was recommended to take a high dose of vitamin C for bruising and she broke out into a rash. I will need to watch for this when I do start the supplement.

This rheumatologist confirmed that I need to see the geneticist that I am on the wait list for.  I am not sure when I will be able to get in with him as it is a very long list and he is one of the top two EDS specialists in the country (with the other being in Maryland). I got put on the list in December, but it looks like it could be next March before I am able to get in with him. She had me make an appointment for 6 months which is in October just to check back in and see how I am doing. I really liked her and it is nice to now have a specialist addressing my EDS issues!

Endocrionology Results and Hashimoto's Thyroiditis

I finished all of my blood work (which they did through my port super easily! They even had a separate room and nurse just for people with ports!) and ultrasounds yesterday. My endocrinologist called me back yesterday with the results. My adrenal glands were perfectly fine and my B12 actually came back slightly elevated. This will not hurt me and is better than being low, so we are going to leave it alone. My thyroid levels came back in the very high end of normal. Since they were elevated in December, people with my conditions are prone to thyroid issues, I am of child bearing age, and I show symptoms of low thyroid he decided to start me on a very low dose of thyroid medication. I now take 25 mcg (that is 0.000025 grams) of a synthetic thyroid hormone called Synthroid every morning. This is the starting dose and we will adjust it from there.

He diagnosed me with a condition called Hashimoto's Thyroiditis, sometimes called Hashi's for short. This is the most common condition that causes hypothyroidism (under active thyroid). Hashi's is  an autoimmune condition in which your body produces an antibody that attacks your thyroid gland. The disease tends to be progressive; more damage is done the longer the condition goes untreated, which is why it is good that we caught this condition while I am still young. Some symptoms of this disease are: fatigue and sluggishness, increased sensitivity to cold, constipation, unexplained weight gain, muscle aches, joint pain or stiffness, and excessive or prolonged menstrual bleeding (menorrhagia). As you can imagine, many of these symptoms overlap with my other conditions so it is very difficult to tell what disorder causes which symptoms.

One of the main reasons that my doctor decided to start treatment for hypothyroidism is because I am of child bearing age. Although my husband and I do not have immediate plans to have children, it is something that we would like to do at some point in within the next few years. An underactive thyroid can make it difficult to get pregnant, increase the risk of miscarriage, and increase the risk of intellectual and developmental disorders as well as birth defects of the face, nervous system, and thyroid of the baby. We want to start getting my thyroid levels stabilized now so that down the road when we do decide to have children we decrease the risk of running into these issues. 

I do not know what the results of my thyroid ultrasound were, so I will have to ask at my followup appointment in 6 weeks. I go back to see my endocrinologist on May 21st and we will see how I am doing then. He will most likely also order blood work to see how my blood thyroid levels are and if we need to adjust the dosage of the Synthroid.

Urology, Endocrinology, and Rheumatology

I have been super busy with appointments! First off, I have officially been discharged from home nursing! I can access my port and take care of all of the issues that may come up independently as can my husband. This is great news because it is one less appointment a week for me, but it is sad because I love my home health nurse :( It will be sad not to see her anymore, but it was a necessary step forward and it is nice to know that between my husband and I we can handle my port and everything that goes with it.

I had an appointment with a urologist last week. My neurosurgeon out in New York wanted me to see him to get some testing done called urodynamics testing. This test will help to determine if I have a nuerogenic bladder by seeing how much my bladder holds, how well the muscles work when I void, and how much sensation I have in my bladder. A nuerogenic bladder is simply a condition in which a person has nerve damage that is causing damage to their bladder. We are nearly certain that I have this and that this test will come back positive. What this test will tell us, along with other symptoms like back pain, leg and foot pain, and leg weakness, is that I have a tethered spinal cord. This condition occurs when a fatty piece of connective tissue binds the end of the spinal cord in the lower back, which is supposed to be free floating, to the bony part of the spinal column. We are nearly certain that I have this condition and the urodynamics testing will be the final step to determine this for certain because the tethered spinal cord cannot be seen on an MRI, which is refereed to as occult tethered cord (OTC). If this is the case as we suspect then we will be flying back to New York early this summer for another major neurosurgery. I have an ultrasound of my kidneys and bladder tomorrow to rule out any structural damage, which is unlikely, and then the urodynamics test will be done next Thursday. I am trying to get a hold of my neurosurgeons office to schedule the surgery, but haven't been able to do so yet.

Today I saw an endocrinologist for the first time. He was incredibly nice and thorough! During my pre-surgical testing in December my primary physician ordered a thyroid test to determine if my thyroid levels were off. Well we found that they were slightly elevated (meaning that my thyroid is underactive) and she requested that I see an endocrinologist. Between the major spinal surgery and 2 changes of insurance I was not able to do this until today. He agreed that yes I need to be started on a thyroid medication, but wants to do some further testing as well. He is going to retest my thyroid since it has been a few months, test my adrenal glands, and test my vitamin B-12. All of these things may be affecting my overall health and are things that are commonly wrong with people with the underlying conditions I have (Chiari 1, EDS, and POTS). I will get these blood tests done tomorrow at 8 in the morning because the test for the adrenal glands need to be done in the early morning. I then need to go get an ultrasound of my thyroid gland (in the neck) because he can feel a nodule on the right side of my thyroid. This could mean a wide variety of things, so we need to get a closer look at it and determine its exact location, size, and what it is made of.  After all of these tests are done he will call me back as to what the next steps will be, but at the very least I will start on a synthetic thyroid hormone called synthroid in order to lower my thyroid hormone. I will update again after I talk to him.

My appointment with rheumatology for my EDS was supposed to be last Tuesday, but they had to cancel it and could not reschedule it until April 28th. They called today and said they had a cancellation so I am going in on Monday to meet with this doctor. It will be a busy couple of weeks, so I will try to update as soon as I get information. I will definitely update by the end of next week though!

Occupational Therapy

The biggest thing going on with me medically right now (minus this dratted cold!!!!) is my occupational therapy. I have been doing therapy for my hand and wrist for 2 weeks now. I go twice a week (Monday and Wednesday) for about 45 minutes to an hour. She started out by doing a full evaluation to evaluate my range of motion and and strength. This evaluation showed that I had decreased strength and range of motion in addition to muscle atrophy in my right wrist. This was not surprising as I had not been using it and had it in a brace. We started with basic non-weight bearing range of motion activities. This was to start to get my wrist moving again. We are slowly adding on strengthening exercises but with my EDS (connective tissue disorder) we have to do this very slowly so as not to re-injure my wrist.

I have lots of home exercises that I need to do to keep up the range of motion. I have to roll my wrist in several different directions, squeeze a special sponge repeatedly, make certain formations with my hand and wrist, and do certain exercises with silly putty (my personal favorite) all multiple times every day. These exercises are expanded and made harder by my therapist as I progress through therapy.

My therapist is absolutely fantastic!!!! I was nervous about doing occupational therapy because my last experience with therapy (physical) was not a good one! That was before we knew about my EDS diagnosis and they ended up doing more damage than good to me. This time around I know a  lot more about my conditions and my physical limits, so I was hopeful that this would help. I have a sheet that I print out and bring to each one of my doctors appointments that lists my conditions, surgical history, and medications and I gave one of those to my therapist as well for my chart. This was on a Monday and by the time I came back for my next appointment on a Wednesday she had researched not only EDS, but all of my conditions to see how they would affect my therapy! It was so nice for her to take the time to look up  my medical conditions so that she knew how best to help me and most importantly how not to do more damage! She also is very innovative in the ways that we do my exercises. She is constantly changing them and modifying them to fit me and my needs.

So far my wrist has been starting to feel better. I am not wearing my brace all of the time and she has been taping it with athletic tape to help stabilize it while we are weaning out of my brace. The cortisone shot that my orthopedic surgeon did also seemed to help tremendously. I have about one week left of the anti-inflammatory medication that my surgeon prescribed as well. In the next couple of weeks we will go down to 1x a week of therapy instead of 2x. I will write another update soon on everything else that has been going on with me. Thank you for continuing to pray and send positive thoughts my way!

Wrist and Port updates + upcoming appointments

Hi all!

I have had quite a bit going on since I last updated! The biggest thing happening is that I have been continuing to have problems with my port. It has been increasingly difficult to access and continues to be tilted. My husband tried to access it 2 weeks ago and could not get it. It was a lot more painful than what it normally is and I ended in tears. The poor guy is now terrified to try to access my port. Well, we called the on call nurse to come on Sunday and she tried multiple times to access it and she could not get it either. She then called my nurse that normally comes to see me and asked what she wanted done and they agreed that I needed to go to the ER to make sure that it was not damaging anything and that it was not clotted or infected. All of the nurses and doctors agreed that it looked and felt like it was pointing up and to the right towards my right shoulder (remember the port is in my upper right chest directly above my breast and should point straight forward). Well we decided to take some chest x-rays to get a better idea of exactly how the port was positioned. The x-rays he took were then compared to the x-rays that they took when my port was put in. Surprisingly, the x-rays looked perfectly fine. They sent me home with a still unaccessed port and said they had no idea what was wrong.

Well we waited a few more days and it shifted back towards a more normal position. I was laying in bed and was seeing if I could feel the three little dots that are on the front of my port. To my surprise I could! I went right then out into the living room where all of my supplies are and thankfully was able to access it on the first try! Then this week we decided it would be best to try to access the port immediately after we deaccessed it so that there was no time for the port to shift or move at all. It took a couple of tries, but I was able to get it on Tuesday of this week. I then had an appointment with my new general surgeon on Wednesday. We love the old general surgeon, but when my insurance changes he is no longer covered :( He recommended another surgeon in our area and he was able to get me in really quickly. The appointment went really well. He manually manipulated my port and shifted it around, of course to do this he had to deaccess it :( He said that the port is indeed pointing upwards and to the right. He does not, however, recommend going back into the OR right now. He thinks that even if he went in and resutured the port that it would just move again. My connective tissue is not as strong as it should be with the EDS and as he puts it my body just doesn't do what it is supposed to do. We are going to wait as long as we possibly can to go back to the OR. We are going to try to wait until we cannot access my port at all anymore. Thankfully after a few tries, some blood, and frustration I was able to reaccess my port after work that day.

The other appointment I have had since I last updated was yesterday with my orthopedic surgeon. My wrist has been causing me a lot of pain on the pinky (ulnar) side, where it was torn before my surgery, but it has also been having shooting pains on the underside of my wrist on the thumb (radial) side of my wrist. I have been wearing my wrist brace full time for about 3 weeks now, which has really helped. Well my wonderful orthopedic surgeon was very helpful as always (I LOVE him!!!!!). He said that he thinks it is a good old case of tendonitis that is causing the radial pain. This was most likely caused by a minor strain/sprain and because of my connective tissue disorder and scar build up from my surgery has gotten progressively worse. The treatment for this begins conservatively. He did a cortisone (steriod) shot  on the radial side of my wrist, which will help to reduce inflammation and swelling. This will take another 2-3 days to kick in and then we are hoping that this shot will provide significant relief. He also ordered me an anti-inflammatory medication called Celebrex. It is similar to ibuprofen or Aleve, but is stronger and is much gentler on your stomach because it is intended for longer term use. I am to take this 2x a day (morning and dinner time) for 3 weeks, but he gave me a refill just in case I need it. The final thing that my orthopedic surgeon ordered is occupational therapy. This is to try to get the strength built back up, the inflammation down, and the range of motion back. The order is for 2x a week for 6 weeks. I go for my occupational therapy evaluation next Monday and then will begin actual therapy the following Monday. I then will go back to see the orthopedic surgeon in about 7 weeks, on May 8th.

I have had to change a couple of my appointments that are coming up. My appointment with the rheumatologist at Rush who specializes in my connective tissue disorder (EDS) is still set for April 1st. I am not sure exactly what to expect of this, but I am excited to learn more about this syndrome and what it means for me in the future. I am hoping it is a good visit and I have heard good things about this doctor from other patients who have EDS. April 2nd I was supposed to have an appointment with an endocrinologist, but I had to switch that and I now have an appointment with a urologist. I need to meet with him and get some testing done on my bladder. This will help to determine if there is neurological damage to my bladder. This information will then be sent to my neurosurgeon in New York in order to help diagnose another neurosurgical condition that he is nearly positive that I have. I will update more on this after I hear back from a couple of people and get a better understanding of how my neurosurgeon wants to proceed from here. I have my endocrinology appointment on April 9th now with a local endocrinologist.

That is all the information I have for now. I will try to update within the next week or so, but may not update until after my appointments on the 1st and 2nd of April. Thanks for continuing to pray and care about me and my journey!

Appointments

Hey all! Well since my new insurance (which is AWESOME) kicks in April 1st I have started to make some appointments. I have an endocrinology appointment on April 2nd to finally get my thyroid issues addressed as well as some other things checked and an appointment with a rheumatologist who specializes in EDS on April 1st. I still want to see the geneticist who specializes in EDS that I am on the wait list for, but I was just informed that the wait to see him is now out until next march (approximately 15 months from when I got on the wait list) we wanted to see someone a little bit sooner since my EDS isn't really being addressed by anyone right now. Those will both be done at Rush University where my neurologist is.

I also have an appointment on March 20th with my orthopedic surgeon for my right wrist. It has been hurting me a lot lately and I can't do anything with it without it being in pain again. Things like brushing my hair, turning a door knob, pushing open a door, or using a can opener put me just about in tears. I picked up my 4 1/2 pound rabbit the other day with two hands (with my brace on) and it hurt my wrist so badly that I was in tears. I am back to being in my wrist brace 24/7 now. I am not sure why it is getting worse again, but hopefully my surgeon will have some ideas.

I still need to schedule an appointment with a GI doctor for either April 1st or 2nd. I also need to email my neurosurgeon in NY. I have a couple of questions for him and I am pretty sure I also need to schedule my follow up scans and Skype appointment with him. I will let you know what he says and when the rest of my appointments will be. So far there has been no change in my port so we should be able to wait until April to get it fixed. Oh yeah, I need to schedule that appointment as well!

Port issues

So I have had absolutely ZERO issues with my port since I got it until last week... I went to access it thinking no big deal I've done this a number of times super easily now! Well my nurse and I decided we should let my husband try to access it since he has never done it before and that way he knew how just in case I needed help ever. Well he struggled, which is normal trying to access it for the first time, but what is strange is that he could not get my port to pull back or to flush. This has never happened before so after a few tries I decided to try it. Well I had the same issue... After attempting multiple times the nurse finally tried it and she had the same issue as well! She finally figured out that my port is tilted! Well we obviously thought that that probably was not a good thing, so we contacted my surgeon who put it in. Meanwhile it continued to tilt further until the right side can actually touch back against my rib cage and the access point faces towards my shoulder. He got me in for an emergency appointment today at 2 o'clock. Well as I expected he wants to make a trip back to the OR to fix it.

Since it is still working right now, just difficult to access, we are going to hold out until I get new insurance on April 1st. The problem is that then this doctor is not covered by my new insurance :( He gave me the name of another local surgeon to work with, so hopefully he is able to get me in! I will update again very shortly with news on lots of appointments and goings ons. Please pray that my port continues to work so that we can wait until April to get it fixed!!!

Update time :)

Hi guys! Wow have I fallen way far behind on updating! There have been a few things going on with me medically, but not too much.

The biggest thing that has happened was a trip to the emergency room a couple weeks ago. Well I had had a pretty bad headache for over two weeks now. We are thinking that the nerves are starting to come back online from the surgery. It got to the point where I was not tolerating it very well any more and we could not manage it very well at home. I then spiked a low grade fever with absolutely no signs of the flu or any other viruses. Well with my port we are always on the look out for infection and I have to go to the ER at the first sign of a fever or infection. An infection in my port can spread to my blood stream and then my heart relatively quickly and is potentially fatal if left untreated.We went to the ER to rule out any infection and thankfully there wasn't one, so we still do not know exactly what caused the increase in symptoms. Thankfully I am feeling better now!

I had an appointment with my neurologist/POTS specialist on 2/13. We discussed everything that has happened since I last saw him in August and decided not to change anything for now. I am very stable and doing great on the meds I am on now, so why mess with them? We did talk about getting off of one of my medications come this summer because it may be contributing to my increased pressure headaches, but I am not willing to risk going downhill with school going on right now. I only have 2.5 months until I graduate, so we will adjust things after that! The only other thing we talked about at this appoint was how my body would handle pregnancy. That is something that we want to start thinking about now that I am married and close to graduating. He said it is an option for us, but it won't be easy. I need to get off all of my medications and will have a difficult pregnancy (bed rest probable), delivery, and recovery period. For now it is good to know that from an autonomic nervous system stand point I could handle a pregnancy, but there are many other doctors to talk to and get opinions from. The one I am most interested to hear opinions from is the geneticist that I am on the wait list for.

I have started to access my own port! It took me a few tries to get the hang of doing it, but now I can do it with no problems! My nurse is coming one last time on Wednesday to discharge me and then I will be on my own. I am confident I can manage it though!

My right wrist (the one I had surgery on in August) has been increasingly sore for the past few weeks. I was hoping it is just with the weather change that it was sore, but I can't bear any weight on it (pushing myself up, crawling, pushing on a door, carrying anything heavy, etc.). It had felt fantastic since the surgery, so I am not sure why it is so painful right now. Hopefully it goes away soon otherwise I will have to make an appointment with my orthopedic surgeon to get it checked.

We got the medication delivery system set up with my new insurance. They deliver a 3 month supply of all of my meds and they are now only $20 a month for ALL 4 of them. This is about 1/5 of the cost or less than it was for my old insurance and I don't have to deal with picking them up multiple times a month since they were all on different schedules. This has been really nice and convenient. 

My insurance situation is changing once again... I still have not been able to get into an endocrinologist with all the insurance issues and at this point am hoping to wait until after April 1st because blood tests and scans will be much cheaper with my new insurance.

Right now the only appointment I have scheduled is on June 12th with my POTS specialist. I will update as soon as we find out more with the new insurance and are able to schedule the other appointments that I need. Sorry for the long gap in updates, but I have been busy with school and work. I am feeling really well and am happy with where I am at medically right now!

Brain fog

So it occurred to me today that I talk a lot about brain fog and the medication I take for it, but I never really explained what it is. Brain fog is different for everyone, but in general it is just what it sounds like: foggy brain! For me this means that I have to work much harder to process information.

I do not take my medication that I take for brain fog on the weekends because it causes some gastrointestinal side effects, so my stomach needs a break and I don't need my brain on the weekends anyways! I went out with some friends the other night to a pizza place. I didn't even think of taking my medication because it is not like I had school or work that I needed my brain to be clear for. Well I realized I should have taken it! The main issue of brain fog that I noticed was that I had a very difficult time following the conversation because of how many people were talking. If only one person was talking I was just fine, but when multiple people were talking all at once I just could not process what was going on. Another issue I have with brain fog is word recall. I know what I want to say, but cannot come up with the word. For instance, I wanted to say the word "magnet" but could not come up with the right word. I instead made a rectangle with my hands and said "you know the sticky thingy for the fridge with pictures on it". Thankfully my husband is incredibly understanding, but it is really frustrating! Another example was I was trying to come up with the word "rabbit". I have 4 rabbits so this is a familiar word to me, but instead I said "you know... the little furry, jumpy creatures". I know what I want to say, but it just won't work for me!

Another symptom of brain fog is that I will walk to another room to do something, the kitchen for example, and stand in the middle of the kitchen completely unable to remember why I am there. Sometimes if I stand there long enough I will remember, and other times I don't remember at all. Another way that brain fog affects me is when I am reading. I will read the same line or paragraph over and over and over again without realizing it or retaining any of the information. Sometimes it does not matter how hard I try I simply cannot gain information out of what I am reading. For those of you who know me you know I LOVE to read, so this is extremely frustrating!

No one is 100% sure why this occurs for me and other people with POTS, but the most likely explanation is that blood pools in my legs when I am sitting or standing. Since this blood is stuck down where it is not supposed to be it is not getting to my brain. My brain is then allocating all of the blood and oxygen that it does have to essential functions like breathing and other involuntary functions. It is not enough of a deficit to be dangerous to me, but this lack of blood going to my brain is likely why the brain fog occurs. My brain simply cannot maintain all of the necessary bodily functions AND do things like come up with words or remember things at the same time.

I hope this helps at least a little bit to understand what brain fog is and how it effects me :)

Long overdue update!!!

Hi all! It has been a long time since I have posted an update. Thankfully this is a good thing! I have gone back to work and then last week started school. I work 5 days a week for the first time ever! Only about 15 hours or so a week, but I am very happy I can physically do this. I am also taking a full load of classes, so that is very exciting as well!

Physically I have been doing very well. My neck doesn't hurt hardly at all anymore, my range of motion has come back significantly, my neck stiffness is nearly gone, and my headaches have improved drastically! Car rides do not cause me the same amount of pain that they used to. Every bump is not excruciating any longer and I don't need to wear my hard collar at all anymore! My swallowing/aspirations issues have COMPLETELY dissipated as well :) My incision looks good and isn't that itchy any more. Here is a photo of my incision at just about 1 month post op. My surgeon said it looks good. There are a couple of white spots on it which are actually stitches that are supposed to dissolve. With EDS (connective tissue disorder) my body rejects the stitches before they have a chance to dissolve like they are supposed to. It doesn't hurt and I expected it to happen because it happened last time as well. We just have to watch and make sure that I do not develop an infection from any of the rejected stitches.

 

I am still very low on energy. They say it takes about an average of 2-2.5 weeks for every hour you were under anesthesia for the toxins to get out of your body. Since I was under for over six hours it could still be quite a while before I start feeling better again. My thyroid levels being low are also probably causing some fatigue.

I have started to learn to access my port! It is very difficult to get the needle in the right spot. I can prep everything and am confident I know the correct procedure, but I am struggling to get the needle in the correct spot. It will take practice, but I am getting there at least! Hopefully I'll get the hang of it shortly, but it is not easy to do because it is on my upper right chest and I am right handed. Also trying to do it in a mirror makes it difficult as well. I had gotten some little alcohol filled caps that I put on the end of my port while I was in the hospital before and you might remember that I was trying to get some for at home because they reduce the risk of infection. Well we called the company that makes them (Curos they are called) and they sent us a "sample". Well this sample is a hospital sized sample and contains 250 caps in it! This is well over a year supply for me so that was exciting! :)

My neurologist also called me in a prescription for a nausea medication. I have been having really bad nausea and haven't found anything to control it besides this medication so I was extremely grateful for that!

I now have my new insurance, but we are still trying to sort stuff out so I have not been able to schedule any appointments that I need to yet :( Hopefully it will all get worked out very soon!

Right now the only appointment I have scheduled is with my autonomic neurologist on February 13. This is just a follow up/check up appointment, so I don't anticipate anything exciting happening. I will update once we get my insurance settled and I am able to schedule the appointments that I need to! I'll try not to let so long go between updates as well :) Sorry for the delay!

No news is good news!

Hey all, I really do not have much to update on right now. I am still having very little pain and am recovering very well. I am getting around easier and am starting to get more energy. Car rides are still painful for me, but I made it over an hour to my parents house without my neck brace on! I was supposed to go back to work tomorrow, but I work at a school and they are closed due to the frigid temperatures (-50 Fahrenheit with the wind chills!!) so I have one more day to rest up. I am ready to go back to work! I love working with the kids and seeing my friends at work.

I have not yet made my appointment with an endocrinologist like I need to because I am currently undergoing a change of insurance. Hopefully within the next couple of weeks this will take effect. It looks like my neurologist is covered, who I will need to see again in February, but we are not sure yet about my other specialists. I need to find out if my neurosurgeon, ear-nose-throat, orthopedic surgeon and the geneticist I am on the wait list for are covered by this new insurance. It will be difficult to switch over insurances, but in the long run will save my family a significant amount of money without sacrificing my care.

I got new serum for my allergy shots a couple of months back, but just started using the new vial right before we left for New York. For the first few weeks I need to do a shot every week instead of every other week like I normally do. I have one more shot to do on Thursday and then I get to go back to every other week! I struggle to remember to do my shot, so it will be nice to get back to my usual schedule!

Right now we are battling my current insurance over a couple of things. For some reason they do not want to cover the compression stockings I got a couple of months ago. They are claiming that they do not cover them even though we called the insurance company multiple times to make sure that they were covered. We need to write a letter to the insurance company in order to appeal this decision. Secondly, one of my medications requires pre-authorization from my insurance now. We are not sure exactly why, but I now need to get my neurologist to fax over some paperwork to my insurance in order to get the prescription covered by insurance. Finally, during my craniocervical fusion an outside company was apparently used to monitor my sensory and motor functions. This is fine except for my insurance is now saying that this is an out of network expense. This service was nearly $10,000 though.... The insurance company is currently reviewing this, so we will not have any information for the next 30 days or so on this. Please pray that this goes over smoothly and everything works out well!