Marco?

Wow! Long time no talk everyone!

Let's start with the very best update from me medically and for life in general :) I have a new partner in crime!

This adorable face is my new service dog, Reilly! He has been with me since December and is for mobility and balance. He helps me to get on and off the ground, picks up dropped items, helps me up and down the stairs, supports me when I am standing still, catches me if I stumble, gets my water bottle and medications for me, and so much more! He has helped me to become so much more independent and confidant! I am so thankful for everything he does for me and the support (both physical and emotional) that he is constantly providing me!

Another huge life update for me is that I am working FULL time! Yep that is right 37.5 hours a week :) I am a teacher assistant in a special education setting for students 18-22 years old. I LOVE it! It is so nice to be working and I absolutely adore working with students! If you were to ask me year ago if I thought I would ever be working full time I would have laughed at you because of how poor my health was! Now I am doing it, yes it is tough sometimes, and enjoying every second of it! I am also applying to graduate school for speech-language pathology! If I get into the program I applied to (which I won’t know for a while still) I will start in January. This is another huge step that I did not think I would accomplish in my life because of my health, but there is no doubt in my mind now that I can handle it!

As you can probably tell by the tone of this post so far I am doing VERY well medically. I am feeling great and the vast majority of my symptoms are stabilized by my various treatment methods. My medications have not changed much since I last updated with the exception of getting the GI medication that is not FDA approved. We ended up ordering this medication (Domperidone) through Canada and it has been a complete blessing! I am able to eat with few restrictions and am not nauseated nearly as often. I still cannot eat large meals and struggle to eat out, but overall my GI issues have been greatly improved!

I have had no major injuries or illnesses that have had lasting effects! I had one sinus infection and upper respiratory infection over New Years, but that cleared up with some antibiotics. I do not have any surgeries scheduled or even being talked about! That means we are nearing one-year surgery free!!! This is very exciting and has not happened for a few years now :) I am doing very well after my last 2 neurological surgeries and my symptoms have been greatly reduced. I just had a round of blood work (thyroid, electrolytes, vitamin D, and CBC) along with an EKG to check my heart rhythm and all of these tests have come back normal. I haven’t had many doctors’ appointments, which has been very nice!

I did have an appointment with a TMJ specialist who confirmed that my jaw joint (TMJ) is severely out of alignment and that I would benefit greatly from treatment. The first step in this process is to get a sleep study done in order to rule out sleep apnea. We do not think I have sleep apnea, but it something that we need to double check for before we pursue treatment for the TMD (temporomandibular dysfunction). I have an appointment with a sleep specialist in a couple of weeks so that we can get the sleep study done to rule out sleep apnea and then move forward with the TMD treatment. We are not yet sure exactly what this treatment would entail until we get further imaging and tests done.

I also need to set up an appointment with a physical therapist recommended to me by my geneticist. I cannot see a regular PT because they are more likely to do harm than good in EDS patients. This will help me to strengthen my body and protect my joints from injury. Hopefully it will be able to help reduce some minor pain I have in some of my joints by making them stronger.

I think that this is about all that I have to update on right now. It has been quiet for me medically and I have been busy working and enjoying life with my new partner. I will try to update more frequently, but thankfully there is not much to update on right now! Thank you all for your support and prayers throughout this journey!

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!