Living with Ehlers Danlos is Hell

I have Ehlers Danlos. On top that disease, I was given fluoroquinolone antibiotics and suffered a severe reaction which then lead to a rare and deadly fungal infection called Glabrata. This is what I have lived through over the three years.

Ehlers Danlos

Ehlers Danlos Syndrome (EDS) are genetic mutations that cause a lack of several types of collagen found throughout the body (skin, muscle, tendons, tissues, nerves and blood vessels). I have types I, II, & IV and crossover variations into several other forms. The type IV form that I have, also known as the vascular form, is a rare mutation form rarely ever seen (as of to date only three families including mine) and this is the most severe form that the NIH and John Hopkins has ever seen. This along with having more than one form (which is also extremely rare) has affected every part of my body and every system I have. It has left me with several other sub-illnesses, (some of them are common to all EDS sufferers and others not so common) such as hyperadrenergic dysautonomia, systemic mastocytosis, myalgic encephlamylitis (ME), pelvic congestion syndrome (PCS) and if God did not think he gave me enough to fight he threw in seronegative spondyloarthpathy with Crohns, which is seen in 35% of all female EDS patients. I also developed secondary Sjorgen’s Disease.

Ehlers Danlos plus Fluoroquinolones – Exploding Collagen

On top of all this crap I was given a fluoroquinolone drug by mistake, which is a collagen depleting drug!

After taking only four pills, my life turned upside down on a dime. I spent 64 days in the hospital fighting for my life, as my defective collagen exploded in every part of my body. Since then I have been fighting a daily battle of severe pain and organs and blood vessels that no longer know how or have the capabilities of functioning normally. It has now been almost three years since the fluoroquinolones and my body is still deteriorating.  Fluoroquinolones, as all my floxy friends know, is a nuclear bomb that slowly kills through DNA adduction and mitochondrial destruction.

The fluoroquinolones have left me with no immune system to speak of, so I am constantly fighting one infection after another. Last year, I fought the hardest battle of my life with a rare drug resistant fungal infection known as Glabrata; leaving me to spend 120 plus days in the hospital plus five months of home nursing care and daily IV’s of poison that nearly wiped out my liver. While fighting the Glabrata infection, I was also fighting the Crohn’s, liver damage, fluoroquinolone symptoms, sublexes, dislocations of my ribs from my sternum and a host of organ, nerve and blood vessel issues.

Pelvic Congestion Syndrome with Ehlers Danlos

One of the most painful issues is the pelvic congestion syndrome (PCS). The easiest way to explain this awful part of vascular EDS is to think of varicose veins the legs; the kind that when the person stands the blood begins to pool in their legs, the veins bulge out like thick blue ropes and begin to swell. After standing for a short period of time these people begin to suffer horrific pain down their legs to the point that they have to sit and put their legs up because it feels like razor blades cutting through them. Well, pelvic congestion syndrome is the exact same thing only it is the blood vessels in your abdomen. It can be the major arteries or the smaller ones known as the feeder veins, the ones that feed your organs.

For some of us with Ehlers Danlos, PCS affects only one or two veins and for others, like me, it affects several or all. For me, it is the feeders and the major mesenteric artery that are damaged. Every time I am in an upright position for any length of time the blood vessels dilate and fill with blood. This puts pressure on my organs and all the nerves that surround them. So, I suffer from both organ inflammation and dysfunctional organs because of the Ehlers Danlos. I also have severe nerve pain that can only described as a screaming pain. It makes me want to jump out of my own skin and run. The pain is so bad, I want to run to the nearest ER and beg for someone to help or end my life.

Unfortunately, there is no way to fix this other than to take enough pain meds to knock me out for days. Life with Ehlers Danlos, and especially PCS, revolves around being drugged with the strongest pain meds on the market and only getting up to shower and then back to lying down flat again. It leaves me unable to walk. Many people with Ehlers Danlos are in wheelchairs, but even that has its time limits.

Ehlers Danlos Specialists – Good Luck

In an attempt to hold on, people with Ehlers Danlos reach out to other patients, looking for any help they can. We search out specialists around the country, always praying that we will find that one doctor that is going to have the miracle we need. Because this is such a rare illness and my form of it is particularly rare, there is no protocol or research to follow. We are all on our own. Every one of us with Ehlers Danlos, no matter where we live in the country, have at some point, seen the same handful of doctors and received the same response – ‘there is nothing we can do.’

My journey has lead me to specialists from Chicago to Washington DC to Michigan and to Mayo, both my doctors and I have consulted with doctors from the NIH, John Hopkins to UCLA, and of course, every one of them tells me the same thing: ‘You can’t fix this [the exploding veins]. If you try [surgery]you will surely cause more severe damage to the remaining blood vessels or the surrounding nerves, which could, and has in some who have attempted it, left them in what is known as intractable nerve pain that is not treatable.’

Surgery to Repair Veins and Arteries Damaged by Ehlers Danlos

For people with Ehlers Danlos, healing is difficult and complications to surgery are very real and dangerous; attempts to stint, remove, bypass or replace the veins only causes other veins to erupt. Surgery is a bit like a dog chasing his tail. I have seen these complications first hand in others who have tried these surgeries, but there are a few, a very few people, who have succeeded and healed.

I know the risks and have spent the last two years searching out a doctor willing to attempt to repair my veins. I have begged and pleaded him to try it on me. Surgery to fix the faulty veins is very risky. The 80% failure rate is 80% and the repeat rate, 100%, meaning additional surgeries. And yet, I am willing to pursue the surgery anyway. What else can I do? There are no other treatments. My one feeder vein, which should have the circumference of a piece of pencil lead, now has the circumference of a quarter and is only getting bigger by the month. It is a feeder vein to my lower colon and rectum and is no longer allowing the organ to function normally. The pain is beyond anything I can take, even on pain meds. It is going to blow at some point and I am not willing to go there or lose my organs, so after much consideration, and two years of working with a surgeon, we are going to go in after this vein and we’re going to clamp it off and bypass it.

This vein is embedded in not only feeder nerves to the colon and rectum, but also, a ganglion of autonomic nerves. This surgery is extremely risky. If any of these nerves are damaged in the process, I would suffer not only intractable nerve pain, but also, loss of control of the bowel or bowel paralysis. This could also happen to my bladder, which eventually may cause me to lose the colon and the rectum (or as we Cronnies like to call it a “Barbie butt”, which is where they remove it all, put a bag on your side and sew up your butt like a Barbie doll).  On top of this, because of the autonomic nerves connect there; I could lose control over my heart rate, blood pressure and respiration. With all this being said, I’m willing to take my chances.

This surgery will leave me in severe pain for about 10 to 30 days. I will be bed ridden for those 30 days. This surgery will not get me off the pain meds but should reduce them dramatically. We’re only working on one vein right now; however, we may need to work on another.

Last Saturday, I woke up to filling the toilet with blood, a sign that something else has gone wrong. I took my pain meds, took my BP and heart rate and decided I was going to live. I went to the bathroom again, again later that day and this time I bleed out so bad that even after standing up I was still trickling blood and clots down my leg. It was off to the ER again.  After several hours they could not figure out where it was coming from but my hemocrit was high enough that I did not need a transfusion, so I told them I was going home and did.

The bleeding had stopped as usual, but I called the doctor, and spent the day at Northwestern with my doctor trying to figure out which vein blew. We think it came from the kidney and that was a self-contained bleed; it busted open and then clotted itself off. This is not my first bleed. I am constantly in the hospital for bleeds. My doctor says I need to come in and STAY in the hospital. I looked at her and said, “I spent just over 200 days in the hospital in 2012 and over 120 in in 2013 and I’m somewhere around 50 plus this year, I’m not going in anymore unless I’m dying.” It is not that I am giving up the fight but I’m tired damn it! So, I’m living and letting God take care of the rest.

Living Chronically Ill with Ehlers Danlos: Children and Spouses

This is only one of the many things I live with daily from EDS. The saddest part of all of this is now watching my babies go through this journey. The heart break and guilt runs deep and not being able to save them from this hell, is hell itself.  All we can do is to teach them how live with this horrible disease and be guinea pigs ourselves to help find ways to eventually treat them.

The men in our lives suffer right along with us. They feel helpless during our suffering. They have to pick up the household duties on top of working, and then there are the countless ER visits, the surgeries, the endless nights sleeping in chairs next to our beds in the ER, ICU or hospital, holding our hands and praying that we will get through this one too. They too have to deal with our screaming pain that many times leaves us unable to even hold a conversation. When we reach that screaming pain level, these poor spouses know it’s going to get ugly because it is all we can do to breathe. God help them if they even breathe too loudly because we are likely to snap the F@#k off and that’s when the crazy person comes out, the kind of crazy that even their mothers never warned them about because no one ever knew that level of crazy existed. They have learned the code words for it’s a really bad day, which means make sure I have a tall glass of water, my pain pill bottle and then leave me alone until I am either drugged enough or my body has adjusted to the pain level. My poor Tom not only deals with me but also my daughter who is now experiencing the pain and fatigue on levels that are leaving her to fight the crazy nasty pain devil that comes with this illness and that’s not to mention all the problems my boys have gone through because of this disease. God love these men and any faults they may have because they are angels to go through this.

Ehlers Danlos is a genetic disease that affects every part of our bodies, it affects every system and it disables us at every turn. There is no cure or treatment, there are few doctors who even know about this illness and even fewer that are willing to treat us. We actually get turned away as soon as they find out what we have. Our pain is so severe and incapacitating that most of the time there is not much that can be done other than to put us in the hospital and sedate us into oblivion! Those with the vascular form walk around with a cloud hanging over our heads not knowing when the next bleed is going to happen and whether it will be the one that finally takes us out.

I live with aneurisms in my brain, spinal cord and mesenteric artery. They will blow eventually just like the feeder veins. Some of us take palliative care, not that we are giving up the fight but because we hope they offer us something that helps. After being a hospice nurse for nearly 20 years, I could not do this. I walked out of the hospital and have chosen not to go there anytime soon. My head just cannot comprehend that yet.

Ehlers Danlos – We Look Normal

The worst part of this disease is that from the outside we look like everyone else, so no one can see our pain or what is happening to our bodies on the inside, so very few understand what our lives are like or what it takes for us to just get dressed. They can’t understand how different we are, only those that live with us, and unfortunately, our children who are now walking our path and the other EDS sufferers. It is also hard for some to not understand how one child in a family plagued with this illness can be so sick while another who has it too only suffers some minor problems or nothing until one of their arteries burst in the 5th decade of life. This is still being researched, the only thing they do know is that it is dominant gene and the mutation is true to the family, but the symptoms vary in degrees and systems from one person to another. It is a horrific disease that robs us of our lives and hides inside so others cannot see what we live with daily. At the bottom of this page I posted a pic from the National EDS foundations site to try and make people aware of what we look like on the inside, this is what EDS does to our bodies, organs, bones, nerves. If people could see what we really look like maybe then they could understand our pain, then maybe they would not criticize but instead praise us for continuing to fight, to get out of that bed, to continue to keep living despite the destruction! For me I continue to fight for my kids, I offer myself up for research, hell I’ll be a guinea pig like so many of us Ehlers Danlos sufferers. I will continue to get my ass knocked out by this disease but I refuse to stay down. No, I will get up and I will put a smile on my face I will laugh. I will go out and join the rest of the world, because that’s what we do!!

Postscript: Debra enters surgery today, August 28, 2014. We wish her well.