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.
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.
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….
Today I would like to share a story written by a young man who lost his fiancee to complications of Ehlers Danlos Syndrome.
”The Jade Dragon” by Matthew Curran, written in honor of his fiancee, Amy Mak, who passed away from EDS in 2008. It made me cry.
There is a jade dragon pendant I wear constantly because of a broken promise. Usually promises are made regarding difficult or important things that mean a lot to somebody. They are also made in regards to trivial matters, but when those promises are broken they can often be forgiven and forgotten. The important matters are different, more vital to honor, and even when they are easy to carry out, the consequences of breaking them are massive. In fact, the easier a promise is to keep, the greater the consequences to one’s conscious for breaking it.
The jade dragon was a sort of gift from my fiancee, Amy, for protection. I was flying to Pennsylvania for a week, and it was the first time we’d been apart in nearly two years, except for a few nights when she was in the hospital and I slept at home for whatever reason. The carved dragon hangs on a faded red string tied in a stout knot, keeping the circumference too short to fit back over my head. Well, it is now at least.
Before she got in my little Toyota to go stay at her parent’s house for the week I would be away, she stopped and pulled the jade necklace from her purse. “Come here,” she said, and when I complied she reached up, standing on her tip-toes, and began to yank the tight string over my head, pulling down hard. “Don’t take that off until you’re back here!” she said in her stern voice, which was incredibly convincing, despite coming from a sub-five-foot-tall-in-high-heels Chinese-American woman. She accomplished this with her unique way of making a sentence into a single word; what she actually said was “DONTAKETHATOFFUNTILYOUREBACKHERE!” Naturally, I promised to wear it at all times until I returned, on the usual conditions of torture and dismemberment.
I flew out of San Francisco to Philadelphia and stayed with my parents for a week. I worried incessantly, even though Amy was supposedly safe with her parents, they hadn’t seen the worst of her condition which had developed after she had gone away to college; they didn’t know what to expect when she got really sick. Amy lived with a genetic condition called Ehlers-Danlos Syndrome, and it periodically made her life a living hell. EDS, as it’s known for short, directly affects the body’s collagen production, which reduces the strength of connective tissues responsible for things like your joints staying tight, and your skin not stretching like a rubber band. It can also cause curvature of the spine, vision degeneration, slow wound healing, and deadly vascular problems. In Amy’s case, all of these effects were present, along with the gratuitous addition of excruciating pain and nausea on a regular basis, usually described as an abdominal migraine.
Ehlers-Danlos is such a little-known and less understood disease that only a handful of doctors in the world know anything practical about it. Hospital admits were difficult every time. I had to be Amy’s eyes, ears, and voice, as well as her guardian angel and personal EDS expert in the emergency rooms and in-patient floors. By the time I knew she was having an attack bad enough to be admitted, it was too late to ask her opinion. Pain has interesting and varied effects on people, but of all the doctors, nurses, 9-1-1 operators, and ambulance crews I’ve explained Amy’s behavior to, none could understand it. After too many continuous hours of pain her consciousness would retreat, hide away somewhere inside, leaving only a primal creature on the outside, incapable of language and persuasion, until some morphine or sedatives could be injected. The understanding of pain is a huge subject in itself, but the basic problem is that bad pain is always unbelievably bad, because if we always remembered how it felt we would go insane; the problem is that your pain is unbelievable to anyone else because they cannot feel it with you. I pushed the boundaries of empathy with Amy, I watched over her constantly, I cleaned up vomit, I went without sleep, but even I could never share her pain, as much as I wanted to.
Fortunately my week away was relatively uneventful. Except for a few tears of loneliness, both Amy and I were fine. That is until I took the jade necklace off to sleep and hung it on the bedside lamp, where I forgot all about it. It’s certainly not that I wanted to break my promise to wear it, but just like most people take for granted they will wake up in the morning, I took for granted that I would remember the necklace and Amy would never know I’d removed it. Her dragon necklace remained hanging on the lamp for the entire week.
I finally remembered the necklace when I got on the plane back to California. At that point there was nothing I could do, I was going to disappoint the person I cared most about. I called my mom when the plane landed and asked her to ship it back to me right away. With a few days to wait for it to arrive by mail, I had to hope that Amy would be so happy to see me that she wouldn’t think about the dragon necklace for at least that long.
“Where is the necklace I gave you?” she asked me immediately when we got home. I had to confess that I’d disobeyed and taken it off to sleep, being unaccustomed to having anything on my neck. “This is why I told you not to take it off! It was supposed to protect you during your flights.” In Chinese folklore the dragon is a symbol of good luck, and holds power over stormy weather, so having me wear it was Amy’s way of trying to take care of me.
Amy was a very intelligent and educated woman but I think she held onto some ancient beliefs, in a semi-serious way, to stay connected to her parents and their culture. She was born in Hong Kong, but brought to the States as a young child to gain access to more advanced medical treatments. Though her parents were well versed in Eastern medicine, they still recognized the need for high-tech surgical procedures to save her life. Despite her pain, Amy was constantly grateful for this, and took pleasure in mentioning that if she had lived in China she would have been dead before the age of seven. By the time she was 27 years old she had been through eighteen spinal surgeries involving fusions with titanium rods to correct her scoliosis. I often lovingly referred to her as “my bionic woman” because of this.
The necklace was an important symbol for Amy, though she would never explicitly say so. She wanted me to have something of hers with me when I was far away from her, so we would still be connected. Having spent the previous two years as her caregiver, her friend, her lover, her driver and nurse and partner in all ways, I looked at us being together as a simple, undeniable, eternal truth. She talked about marriage and children from time to time, and I would always protest and ask why it was necessary. “Why, if you know I’ll never leave you, do we need to have a wedding?”
Romance was a difficult concept in a relationship made so intense by pain and life-or-death struggles, so there never was a big proposal moment for Amy and I. Without ever really noticing the change, we gradually started talking about our future in terms of before and after we got married. It just seemed right. The moment this idea finally solidified in my head is one I will remember more vividly than any other in my past or future. I was leaving for work after a difficult night spent next to Amy’s hospital bed, and I looked into her anxious brown eyes and said “Bye honey. I love you.” That was all. I didn’t have to say it, because we both knew it was true, but it was important. In that moment, seeing her look back at me, whispering “I love you too,” was when I realized I was meant to spend my life with her; I couldn’t picture a life without her. About five hours later she was in the intensive care unit, in a coma with a ruptured aorta. I had missed all the calls from the hospital until nearly lunch-time. I ran to the ICU with a gut full of dread. She died the very second I got there.
* * *
The day that Amy died, I found the jade dragon again and put it on. Threaded onto a simple red string and tied in a knot, it was never meant to be a permanent fixture; it wasn’t meant to last in the way my life with Amy was supposed to. In the three years since she passed away I’ve always had the jade dragon next to my skin, so I can stay attached to her in some small way, even as life moves on for me, taking me further from the world we built with each other.
Connections can form between people that even death cannot sever. I’ve never been religious (despite my mother’s best efforts), but to paraphrase The Bard, there is more in heaven and earth than is dreamt of in your philosophy. The weekend of Amy’s funeral coincided with the peak of the Perseid meteor shower at the beginning of August. Consumed with grief as I was, I remained oblivious to this fact until some time later. My first night home after the funeral, Amy appeared in my dream. She floated in a peculiar way which felt natural at the time. She took my hand, and dragged my incorporeal being out of bed, directly through the roof, then settled back onto the shingles to look at the sky. We sat there together for hours simply holding each other, watching the shooting stars, pointing out big ones to each other from time to time. All my feelings of loss and heartbreak were for a little while overcome with an incredible sense of peace. When she said she had to go I wasn’t surprised, because I knew I had lost her a week ago, but she said she would return and I felt comforted.
Almost a year after Amy’s funeral, on the morning of her 28th birthday, I woke from a dream about her to find the jade necklace had neatly tied itself a new knot, just above the pendant. Until this point the string had been just loose enough to be removed from my head, but now it was impossible. As tempted as I was to undo the knot to see how it had been made, I felt like it would be a desecration. I think the new knot in the necklace is her way of saying “Don’t forget this time! Or I’ll kill you in your sleep!” That was always an empty threat, but the way she would say it carried all the love in the world.
There is a special kind of bond between people which is born from suffering. Amy suffered more than I thought was possible, and I was there to see it, or at least as much as I could stand. There is another type of bond, born from guilt, that goes along with the bonds of suffering. Amy and I did a lot for each other, and not just because we had no one else, it was because we loved each other so completely it was like we were two halves of the same person. Though this kind of familiarity can quickly decay into taking the other half for granted when times are rough. Of all the things I regret in myself, in my entire life, this is the worst.
I will always see Amy again, I know this as a fact. She continues to visit my dreams from time to time, and whether this is my own imagination or something more doesn’t matter at all to me. I continue to wear her jade dragon, because I promised I would never leave her, and when I see her in my dreams she is peaceful and happy because I’ve kept this promise. I’ve moved on in life, made new plans, tried to become a better person. In every aspect of life I’ve used the jade dragon as a reminder of Amy’s constant strength, and her unwillingness to give up on a life filled with pain as long as a drop of love still exists. Through the dragon I remember the best and worst things possible in life, and Amy’s spirit which never quit, even when her body did.
I just recently stumbled upon Single Dad Laughing, a fantastic blog written by Dan Pearse. The link will open up to a post I just read (from 2010, currently being featured) about people and their pain and their need to compare their pain with the pain of others. It’s really a great post and urge you all to read it! I’ve had people tell me both that their pain is worse, or that their pain couldn’t be as bad as mine.
As someone who lives with a very rare connective tissue disease that causes many painful dislocates and partial dislocations on a daily basis (among other things) I deal with a lot of pain. Thankfully to a good pain regimen, I’m not in as much pain as I was before it. The most pain I’ve dealt with was when I had wide spread nerve pain. It felt like my whole body had the worst burns of my life and then being rubbed over with sand paper. I just laid in bed crying because moving hurt, cold hurt, hot hurt, clothes hurt, anything touching my skin hurt. Now I have some joint pain, some nerve pain in my limbs and feet, sometimes awful itchy due to nerve issues, and of course the pain of the dislocations, but it decent even compare to that time before the meds.
Having said that, I make sure I tell people this one thing, that their pain is their pain. I don’t know what it feels like, only they do. I only know what I’ve felt. Sure compared to most, I’d say I have a pretty high tolerance for me, but that doesn’t mean the other person isn’t experiencing the worst pain they’ve ever felt. I get both sides of the spectrum. Some people will tell me that their pain can’t be anything like what I deal with. Well, in a way, that’s true if they’ve never experienced nerve pain or dislocated joints or arthritis. But I’ve also only ever broken 2 bones in my lifetime. I don’t remember the first, and the last two were a bone in my hand and a bone in my foot, and I walked on that foot for a week before they found the break. I was 11, so it couldn’t have been that bad. Sure it hurt, but I was still able to walk. Then I also get the people where who say I mustn’t be in that kind of pain all the time… no one can live in that kind of pain like that!!! Well, yea they can, and they do. I know a lot of people who, in fact, DO live like that. My mother and I are two of them and I’ve met a lot of people with the same illness or similar illnesses who do. It’s all a matter of perspective, really.
It certainly has been a long time since I’ve made any sort of update on here. There’s been so much going on and it can be overwhelming. One of my last posts discussed my GI system and what is going on with it. I sort of left things hanging, didn’t I?
Well, there is a bit of a reason for it. The surgeon I was seeing at the time had one of the worst bedside manners I have ever encountered and often left his office in tears. I did end up seeking out a second opinion and here’s were I’m at. My GI symptoms have been getting worse. The pain in my abdomen used to only come after I’ve eaten, now it’s there all the time and will get worse when I eat and randomly throughout the day. Sometimes nighttime can be the worst. The bloating is incredible and I can go from having my jeans be big in the morning to not being able to get them closed at night. Close have become so physically uncomfortable that I prefer to stay in pajamas. My diet sucks. I’m still on soft foods and mostly liquids. There are very few things that I can eat that give me minimal discomfort. I’m nauseous a good portion of the time and don’t even want to think about food. I need nutrition, though. So here’s my day in food for the most part. In the morning I have something like a Gerber’s Grabber. Yep, that’s right. Baby food. It’s the only way I can get fruit in. Hey, when you’re this sick you have to get creative and improvise. So, that’s breakfast. Mid-day, I make my own protein milkshake that actually comes out quite yummy! I mix a cup of almond milk, with a scoop of whey protein (chocolate), 1-2 tbsp of cocoa, 1tsp of something called “veggie magma” which is a powder and pretty much how I get any veggies, 1 packet of splenda, a banana, and a bunch of ice. Toss it in a blender and it gets really frothy and velvety. I let it go a bit longer than it needs to because it builds up in volume almost an extra 1/3 of what’s in there! For dinner, I will usually have a gluten-free english muffin with almond butter and some nutella (there are something I refuse to give up). I used to be able to tolerate a 1/2 cup of low fat ice cream, but now I’m having too much pain for even that. I may give sorbet or coconut milk ice cream another try. During the day I’ll have a snack of a rice cake with some almond butter, or a yogurt. I just bought a giant bag of Dum Dums to have something for flavor throughout the day. Depending on how I am feeling each day there is some variation and I may be feeling a little more adventurous.
In a few weeks, I am seeing one of the countries’ top GI motility specialists. I have been waiting for this appointment for a long time. I’ve had a few tests* that he ordered already and hopefully they will give us the answers we need to figure out what to do next. Whether it means surgery and what kind of surgery, or if there is an alternative that previous doctors haven’t thought about. All I know is that I can’t keep going on like this.
See, last summer I was actually doing really well! I had managed to get my butt out of the wheelchair and started walking. Part of that had to do with getting adequate pain control. I started of really small and by September I was walking close to a mile! I felt amazing! I was still struggling with my GI system, but this gave me something to focus on. It was short lived, sadly, because as the pain kept getting worse and I was getting about the same amount of nutrition as I am now so my energy started to drain. My limbs feel heavy, I’m so tired all the time, I’m not sleeping well because of the amount of pain I’m in whether it be from general joint/EDS pain from subluxations and dislocations or from my abdomen, and my hair is falling out again. Each day I pull out fistfuls of hair. Thank goodness I have a lot of hair, but it’s still very upsetting. The last time my hair was falling out was when I struggled with Anorexia.
*One of the tests is actually really cool. It’s called a Smart Pill Study and the REALLY cool thing is that the motility specialist I am seeing is one of the researchers associated with The Smart Pill!! Basically, the Smart Pill measures the transit time of the entire GI tract. You swallow an electronic capsule and it transmits information to a recorder that you carry with you and hit a button every time you eat, use the bathroom, etc., then bring the recorder back after 5 days and the dr will download and review the information.
On Thursday October 7th, me dear dear friend, Alexa Simmons, lost her battle with EDS and all that comes with it. She was only 22 years old. She had been in the hospital fighting off infections and contracted meningitis and shortly went septic. She was doing great earlier in the week and was upbeat and positive like she always is, even on Thursday afternoon she was doing well. Something happened that evening and she ended up going into cardiac arrest. Her body couldn’t take it anymore and she passed away late Thursday night.
Along with EDS, Alexa also suffered from Autonomic Dysfunction, Chiari Malformation, Seizures, and was having blood clotting problems. Her fight was a long and hard one.
Alexa was one of the strongest people I know. She never complained, no matter how bad things got. She was always upbeat and positive and had a smile on her face. She was there for me when I was starting my journey in finding a diagnosis for myself. She helped me through the roller coaster emotions that come when you finally get that diagnosis and understand what it entails. We laughed together, we cried together. I take solace in knowing that she is no longer suffering and no longer in pain. I will miss her greatly and she will be in heart always.
I found this letter through a personal website of a woman fighting Gastroparesis and Digestive Tract Paralysis. Two things I also struggle with among other things. When I found this letter, I was struck by the honesty and truthfulness of it. So many of us struggle daily with seemingly invisible chronic and painful conditions that many don’t understand. How can they? They don’t live with it, which is why awareness is so very important. It’s also important for those who love us and care about us to understand some things about living with chronic illnesses. Ricky Buchanan also thought about this and decided to construct this letter. This letter has been spread around the internet by those of us living with “invisible” chronic illness because we desperately want those around us to have some insight. It is not meant to be insulting, just informative. Thank you, Ricky!
Having an invisible disability (ID) and/or invisible chronic illness (ICI) means that many things change. Just because you can’t see the changes doesn’t mean they aren’t real.
Most people don’t understand much about these disabilities/diseases and their effects, and of those that think they know, many are actually mis-informed. In the spirit of informing those who wish to understand …
… These are the things that I would like you to understand about me before you judge me…
Please understand that being disabled/sick doesn’t mean I’m not still a human being. I have to spend most of my day being very careful what I do, and if you visit I might not seem like much fun to be with, but I’m still me stuck inside this body. I still worry about school and work and my family and friends, and most of the time I’d still like to hear you talk about yours too.
Please understand the difference between “happy” and “healthy”. When you’ve got the flu you probably feel miserable with it, but I’ve been sick for years. I can’t be miserable all the time, in fact I work hard at not being miserable. So if you’re talking to me and I sound happy, it means I’m happy. That’s all. I may be tired. I may be in pain. I may be sicker that ever. Please, don’t say, “Oh, you’re sounding better!”. I am not sounding better, I am sounding happy. If you want to comment on that, you’re welcome
Please understand that being able to stand up for five minutes, doesn’t necessarily mean that I can stand up for ten minutes, or an hour. It’s quite likely that doing that five minutes has exhausted my resources and I’ll need to recover – imagine an athlete after a race. They couldn’t repeat that feat right away either.
Please repeat the above paragraph substituting, “sitting up”, “walking”, “thinking”, “being sociable” and so on … it applies to everything that I do.
Please understand that the effects of chronic illnesses and many disabilities are variable. It’s quite possible (for me, it’s common) that one day I am able to walk to the bathroom and back, while the next day I’ll have trouble sitting up. Please don’t attack me when I’m worse by saying, “But you did it before!”. If you want me to do something, ask if I can and I’ll tell you.
Similarly, my illness/disability may vary suddenly, meaning I may need to cancel an invitation at the last minute, if this happens please do not take it personally.
Please understand that “getting out and doing things” does not make me feel better, and can often make me worse. Chronic illnesses/disabilities may cause a secondary/reactive depression (wouldn’t you get depressed if you were stuck in bed 23 hours a day for years on end?) but they are not caused by depression. Telling me that I need some fresh air and exercise is not not correct and probably not appreciated – if I could possibly do it that, I would.
Please understand that if I say I have to sit down/lie down/take these pills now, that I do have to do it right now – it can’t be put off or forgotten just because I’m doing something else more exciting. Illnesses and disabilities do not forgive their victims easily.
Please understand that I can’t spend all of my energy trying to get well from my incurable chronic illness/disability. With a short-term illness like the flu, you can afford to put life on hold for a week or two while you get well. But an important part of having a chronic illness or disability is coming to the realization that you have to spend energy on having a life while you’re sick/disabled. This doesn’t mean I’m not trying to get better. It doesn’t mean I’ve given up. It’s just how life is when you’re dealing with a chronic illness/disability.
If you want to suggest a cure to me, please don’t. It’s not because I don’t appreciate the thought; and it’s not because I don’t want to get well. It’s because I have had almost every single one of my friends suggest one at one point or another. At first I tried them all, but then I realized that I was using up so much energy trying things that I was making myself sicker, not better. If there was something that cured, or even helped, all people with a certain illness or disability then we’d know about it. This is not a drug-company conspiracy, there is worldwide networking (both on and off the Internet) between people with similar and different chronic illnesses and disabilities, if something worked we would know about it.
If after reading that, you still want to suggest a cure, then do it if you must. Preferably in writing and accompanied by the scientific papers that prove it works. But don’t expect me to rush out and try it. I might not even reply. If I haven’t had it or something like it suggested before, and it sounds reasonable, I’ll probably take what you said and discuss it with my doctor.
Please understand that getting better from an illness can be very slow. And getting better from an invisible disability might not happen at all. People with chronic illnesses have so many systems in their bodies out of equilibrium, and functioning wrongly, that it may take a long time to sort everything out, if it ever happens.
I depend on you – people who are able-bodied – for many things.
But most importantly, I need you to understand me.