Wise words.
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Hello everyone! Today I had my appointment with my autonomic specialist to go over the results of all those test I had done last month! I absolutely love this doctor! He’s thorough and explains things really well.
My Level 3 cardiopulmonary stress test showed that I have low filling pressures and output. This means that when the heart fills with blood there’s supposed to be a certain amount of pressure to propel it out and to the rest of the body. My pressures are too low, and drop even lower (more than 60%) during exercise. This leads to low blood volume and causes all the symptoms. It’s consistent with veinous insufficiency. To try to treat this we are going to try really low doses of a few meds I’ve tried in the past (there’s only a handful of drugs out there to help this issue). The goal is to get to an effective therapeutic dose without causing any major side effects. We’re starting with a really low dose of Florinef, and will check in with the doctor in a week to see how things are going. If there isn’t much improvement we may try a super low dose of Mestinon, and the third drug to try is Midodrine. We have to be really careful because my Diastolic (bottom number) blood pressure is high whereas my Systolic (top number) is on the low-normal end. We don’t want to cause any hypertension.
The next thing is that my skin biopsy to look for small fiber neuropathy showed severe neuropathy. I’m in the <1% which means that my nerve fibers were barely detectable, which is really interesting since my last skin biopsy 3 years ago was normal. This absolutely suggests an autoimmune type autonomic disease. We’re going to do a second skin biopsy to test specifically for autoimmune reaction against the nerve fibers. If this is positive, which we are hoping for, I can start IVIG treatments to hopefully reverse the damage. My dr said that he has about 20 patients that present like I do and one in particular had improved so much that she is now able to be off of most of her meds! Granted, I will still be one a certain amount of medication because of the EDS related symptoms, but we’re hoping things will improve enough so that my GI motility improves and my energy levels improve so that I can start walking and being more active. I feel really hopeful about all this!
As promised, here’s the “Part 2” from the last post. But first, and update on the autonomic anomaly that I am.
I went to see my Cardiologist/Autonomic Rock Star and he actually shed some light one quite a bit. He said there is some definite signs of small fiber neuropathy going on. The small nerve fibers are what is responsible for all those involuntary bodily functions like breathing, temperature, regulation, cardiovascular function, and digestion. Yep, digestion! To be fair, we figured that my digestion and motility issues were partly autonomic in nature. It usually is for us EDSers. The bigger question was what was going on with my autonomic nervous system in the first place. Dr. Autonomic Rock Star said that given my symptoms, the fact that I don’t seem to fit the typical/more common type of autonomic disorders, and that my symptoms came on relatively quickly compared to most autonomic disorders, he really thinks that what I may have going on is actually autoimmune in nature.
Autonomic Nervous System: Parasympathetic & Sympathetic
He said that if it turns out it is autoimmune, there are treatments available that could help relieve the symptoms, possibly even help improve my intestinal transit. So before I have surgery, he wants me to under go a series of tests. Some blood work to check for antibodies against the cardiovascular system and the digestive system, sweat test as autonomic disorders can cause issues with sweating properly, skin biopsy to look at the small nerve fibers, and something called a Level 3 Cardio-Pulmonary Test.
The blood work was done in the office, no biggie. The sweat test will be done at the same time as the Level 3, I think. The skin biopsy was last week, but is, and deserves, a whole other post of its own. The Level 3 will be this coming Tuesday, and I have to say, I’m a bit nervous about this one as it involves a catheter placed through the jugular vein and goes into the heart and lungs. Oh yea, and I have to be AWAKE for this! A catheter will also be placed into an artery in my wrist, which can be harder to do than placing a basic IV. This test is to measure lung and heart function – According to the Mass General website it gives a “precise measurement of heart pumping function, blood vessel function and skeletal muscle function.”
So, until we know the results of all these tests, the surgery is post-poned.
I posted a while ago about the GI problems I have as a result of EDS and Mitochondrial Disease, in particular autonomic dysfunction associated with these diseases. Last month we had made the decision to go ahead and speak with a surgeon to get their take on my case. For a handful of years, I had tried nearly every medication out there, every combination of these medications possible, and even some alternative treatments and therapies available to me. Eventually all these medications, treatments, and therapies, stopped working, so off to the surgeon I went.
Going into the surgeon’s office I had a pretty good idea about what the plan would be. Being things as they were, it was looking like there is some decent nerve damage in the lower part of my digestive tract and knew that an ostomy was probably in my future. This is actually something that my doctors and I have talked about for a few years now. The first time it was mentioned I actually didn’t know much about ostomies and couldn’t believe that I would have to have one for the rest of my life. Not to mention that the first doctor to tell me this had an awful bedside manner and I often left his office in tears. Needless to say, I opted for a second opinion. While working with the GI Motility Specialist, we tried MANY different treatments like I mentioned before.
Smart Pill
He sent me for a Smart Pill Study, which involves swallowing an electronic capsule that measures different things to discern the transit time of the different parts of the digestive system. The Smart Pill showed that my small intestine was borderline slow, but my colon couldn’t be measured since the battery on the capsule only lasts 5 days. What does this mean? Well. It means that my colon doesn’t work.
A few more tests were run to get a better idea of what is actually going on, but I will spare you all the lovely details of those tests. The important part is that they confirmed everything we suspected.
Anyway, back to the surgeon, who is absolutely wonderful! I love her! If you are in the Boston area and in need if a GI Surgeon, let me know! So, I meet with the surgeon and discuss what’s going on and how I was aware that my only choice at the time would be an ostomy. I had spoken at great length to some amazing young women who have ostomies and/or who have been through the surgeries I may be about to go through. So, I have come to terms with the fact that this will be life long. Well, to my surprise, the surgeon said that it wasn’t my only choice! She said I was a perfect candidate for a J-Pouch, which means that my colon and rectum would be removed and a pouch would be made from the end of the small intestine and then attached to . I would have a temporary loop ileostomy while the pouch healed. Then after everything is all healed, I would undergo a second surgery to reverse the ileostomy and then start using my new plumbing. The surgery is all scheduled but may end up being postponed. As part of the pre-surgery testing and to be cleared for surgery, I needed to be seen by my cardiologist, who is also an autonomic dysfunction specialist. More on that in my next post…
To Be Continued….
The Flu is a nasty, nasty bug. I thought that since I received a flu shot that I would be all set. Oh no! Not with this flu!
I’m just getting over the flu. I still have a nasty cough and my voice is really hoarse, but thankfully the fevers have calmed down and my chest doesn’t hurt so much. Man… this one was certainly a doozy. I started feeling sick about one and a half weeks ago. Started with an itchy throat and ears, then turned into a sore throat w/ fevers, which then turned into a sore throat, cough, chills, body aches, and really high fevers that sent me to the Emergency Room. Of course, I don’t own a thermometer so I don’t know how high the fever actually got, but the night I ended up in the ER, I couldn’t sleep because I was coughing so badly and was in a lot of pain from coughing so much. I actually think I subluxated a rib because I was coughing so hard, plus I felt a lot of pain. I was also having trouble breathing. About midnight, I decided to try my rescue inhaler to see if it would help me breathe easier, which it didn’t. I tried going to sleep, but couldn’t. I started to feel really feverish earlier that night and took some Tylenol before bed, but it didn’t seem to do much as I kept feeling more and more feverish. Around 3am I decided I should probably wake someone up to go to the ER. I ended up waking up my grandparents and we tried more Tylenol and ice on my forehead. An hour later, I still didn’t feel much better so we decided to head out. Thankfully, our town has a small satellite ER that’s good for these types of situations, and at 3:45am we were the only ones there.
I have to say, for a small town ER that many people complain about, I was taken really good care of. They sent me right in, and took my vitals immediately and took my information as we went along. They also hooked me up with IV fluids and IV Toradal to try to get my fever down, it was 103*F when we got there, so who knows how high it actually was before I took the Tylenol an hour earlier. My heart rate was up in the 130s while resting, which is really high, but my blood pressure was ok, higher than MY normal, but still pretty good. They also gave me a breathing treatment hooked up to oxygen instead of the basic nebulizer and my oxygen was still only at 93% AFTER the breathing treatment, so I got connected to 2.5L of oxygen as well. It was kind of scary to see how sick the flu can really make a person. They were able to get my vitals stabilized and my oxygen got up to 97% (which is considered normal) and my fever made it down to 101*F and was instructed to keep taking Tylenol around the clock until the fevers stop. Oh, they also took a chest X-ray as the flu can cause pneumonia, and that’s something I want nothing to do with. Thankfully my lungs were clear! So now it’s been 5 days since the ER, and I started feeling sick about 5-6 days before that, and the flu typically lasts 7 days, so technically I should be over it by now. However, since when does my body with it’s crappy immune system do anything that’s “typical?” Haha! I’m just so glad I’m starting to finally feel better!
This is an article written by Ron from Ron’s Rants about the need for the distinction between those that are “disabled” and those that are “chronically ill AND disabled.” It’s very well written and makes some really good points that I’ve been trying to convey myself to others. Please read the article and share it!!!
The Paralympics and the chronically sick and disabled, a press release..
Day Two’s prompt was to pay a compliment to a stranger. The idea behind this one is to think about when we’ve been complimented even though we were feeling really bad and thinking that we must look just as bad, but someone had mentioned how pretty your hair looked, or how a certain color looked really good on you, etc. This makes me think back to my friend’s wedding a few months ago. Because of complications from my conditions, I’ve put on some undesired weight and have been feeling really uncomfortable about it. Not only that, I just don’t have the money to be buying new clothes, never mind a dress to wear to a wedding. I have a dress that I wore to another friend’s wedding and at the time it had been a little big on me, so I decided to wear it even though I still felt really self-conscious. I continued to feel self-conscious throughout the night, but what had made it easier was people telling me how good I looked, or how much someone like the way I had done my hair. It’s amazing how one little sentence can really perk up someone’s day.
I wasn’t even really thinking about the Challenge the other day, but I did say some nice things to many people at my boyfriend’s folk’s cookout. Just seeing their eyes light up and seeing them smile is so worth it. There is something nice to say about mostly everyone you know, or everyone you choose to hang around. It doesn’t take much to pay them a little compliment now and again.
I am SOOOOO behind on the challenge!!! I feel like such a slacker. I had planned on catching up with them yesterday and today (after the holiday stuff), however, my appointment at the pain clinic ended up being a 5 HOUR WAIT!!! I kid you not! The doctor was running SO FAR behind and was insisting on seeing everyone one-on-one to check-in with them. Usually I just see a Physicians Assistant to get refills and talk about what’s working and what’s not working. I don’t know why, but he decided to do it all in one day.
I met a young woman at my last appointment and it turns out she lives in the same town and has offered to take me too appointment’s if we schedule them at the same time. That way my poor grandparents and sister don’t have to drive 45min to get to my house and take me for an appointment (and thank goodness they didn’t have to take me yesterday!). We get there just in time for our appointment and all nervous because we had been running late and the parking lot was packed and a complete mess. Cars were parked in “non-spots” and another woman was waiting to pick up her husband who was just STANDING outside of the car while the rest of us were trying to get around them. Just STANDING there NOT getting into the car. Then people started yelling and honking, ugh… I like to avoid confrontation and just be nice to people but even I was getting really angry. We make it up there and there were people in camping chairs in the hallway outside of the office. No Joke! The waiting room was packed and hot and we are waiting in line to check in, then we went back out to the hallway and someone offered me their chair, but then we decided to wait on the first floor where the couches were and they would call us. The building is a big open building and you can see onto the first floor. I tried getting up and got really dizzy, my legs were shaking so badly I couldn’t move, I was having trouble focusing my eyes, and was going into a full autonomic crash of sorts. The nurse let us go into the back where there was an alcove with a gurney that wasn’t being used so that I could rest and cool down and get something to drink and eat. Every time I tried getting up, I lost strength in my legs. It was horrible. Eventually I did start to feel better and we went to wait downstairs. Thank goodness for those couches! I don’t know how else I would have made it through! Thank goodness my friend was prepared with waters, powerade, granola bars, PB crackers, etc! I just hope that it’s not this crazy next time. Sigh!
So, needless to say, by the time I got home I just felt like going to bed. I was so tired and so sore and achy all I wanted to do was put on my pj’s and go to sleep. Today, I still felt really tired and so, so sore. Tomorrow is going to be another busy day at a friend’s cookout.
I do plan on getting these done! I did work on them! in the meantime go over to Charis’s blog for the Prompts!
My friend, Charis, over at Full Frontal Ostomy has created the “30 Day Self-Esteem Challenge”. It pretty much speaks for itself, however, the idea is to reflect on our less desirable personal traits that may have aided us in dealing with chronic illnesses and how to turn them into something positive.
When living with any type of chronic illness it’s very easy to become overwhelmed, depressed, unhappy with our bodies, self-conscious about things, etc. This Challenge created to help pull ourselves out of getting stuck in these feelings. To gain some insight on how we can find better ways to cope with bad news, deal with any setbacks that may occur with our health in a good way, etc.
Here is the Day One prompt:
The prompt has several facets. First, acknowledge your less desirable personal qualities. Second, reflect on how these qualities may have aided in your fight against IBD (no need to get too deep into this yet). And third, begin to consider how these qualities can be used to help raise your self-esteem and allow you to regain control of your life – or at the very least, learn how to control your reactions to the bad things that may come your way. This may not make sense to you right now, but bear with me. I believe this will be a journey worth taking.
Charis is focusing on IBD (Irritable Bowel Disease) but I really feel that this can be geared toward any Chronic Illness and decided that I will join her on this Challenge.
My less desirable traits are that I am stubborn, can be moody, often say things that come out wrong or sounding rude, impatient, have a tendency to go on relentlessly about a specific topic, overly analytical, and I’m sure there are many more.
My stubbornness plays an important role as it will not let me give up on myself or the hope that better treatment options for my illness are out there or will be out there in the future. My impatience can often be a good thing in aiding motivation when I really don’t want to do something I need to do in order to stay as well as I can be. For instance, I really hate taking all the medications and supplements that I need in order to feel as good as I can. Instead of hemming and hawing over it, I just get it over with and move on to the next thing. My tendency to go on and on about certain topics can be really beneficial when it comes to advocating for awareness of my conditions and the fact that there are no cures and no real treatment besides pain/symptom management. By spreading information that Ehlers Danlos Syndrome is so much more than being flexible or having stretchy skin, that Autonomic Dysfunction is so much more than getting dizzy from time to time, etc. Being overly analytical has allowed me to really learn and understand my conditions, to ask questions when I don’t fully understand something, to speak up when I am uncomfortable about a particular treatment method or medication, etc.
I sort of combined part two and three of this exercise together, but you get the point. As far as some of the other traits like moodiness and tact, I will have to come up with ways to remind myself to think about what I am saying before blurting it out, which I have started doing anyway as I used to be REALLY bad about this.
I have a good feeling about this Challenge in that it will be really insightful and hopefully help me gain some self-esteem and feel better about myself.
Erin from the blog A Dress A Day wrote a really amazing blog post. So amazing that I have to share it with you all. As someone with a chronic illness, I’ve often felt frumpy, ugly, unattractive, etc., at least once per day. Because of living in chronic pain, comfort is a major component that goes into the decision making of what to wear. Why should we have to give up comfort to feel pretty? Why should we feel that we aren’t “pretty enough” to wear a certain dress or outfit? These are the major points of her blog post and I urge you to read it!
Genetically Incorrect 