A Zebra’s Tale.

In 2018 I was an addict. I had been by then for a couple of years. It’s a dirty word. A title that smears people with a label that depicts weakness. Perhaps to you it invokes images of an unkept disheveled person with track marks; or a skinny junky hanging by the Westfields entry waiting for you to drop some coin in their hat. Those images aren’t necessarily wrong; but are more likely the extreme vision of addiction. Today, more than any time before, people who reach that end start as I did – with prescription opioids. Medication provided to treat symptoms of other problems.

We all have our problems; some are more obvious than others.

I write this now with a clear head. Though it is still not always that way, it’s more often clear than not these days. For a long time, my thoughts were never clear. Just one big fog of prescription drug induced blurred existence. It didn’t start with opioids; or end there either. They though were by far the most addictive.

I’m a statistic of medical limbo. One of many adults who wait many years from the point of seeing a general practitioner to the finality of a clear understanding of their problems.

I was born in 1980. Some may say the end of generation x, others the beginning of the millennials. I say a generation of the medically lost. People born in that time and the proceeding decades are more likely to be living today with undiagnosed life-long chronic conditions or diseases than people born from the late nineties onwards.

Why?

In the medical profession there is an old adage, ‘when you hear hoofbeats think horses, not zebras.’ This is a standard medical industry way of reducing workload for diagnosis of conditions. Why consider a medical condition that is statistically unlikely? That’d be a waste of resources.

Unfortunately, with this scenario many people are misdiagnosed with more common problems than perhaps the ones that better fit their symptoms – purely because the decided diagnosis is more statistically common than other possibilities. Sometimes someone is not diagnosed at all, left only with a list of symptoms and no diagnosis because a GP or specialist is unprepared to investigate further because any other possible diagnosis would be too rare to consider. I’ve formed my own conclusion that the rarity of some diseases and conditions is purely because they remain unconsidered possibilities, and therefore under diagnosed. Meaning of course they are statistically rare.

Sometime late last century this attitude shifted in consideration to children, with more diagnosis of conditions thought to be rare, such as Autism Spectrum (including Asperger’s), or various collagen disorders. You might hear someone say these things weren’t around so much when they were a child. Really the conditions were but weren’t recognized as such due to being thought of as rare, so therefore unlikely to be considered for diagnoses. Many adults born prior to late last century are today still undiagnosed as the general medical system belief seems to be that any rare lifelong conditions would have been picked up in childhood, so therefore those possibilities are still not considered. This gap starts to close as children are recognized for a condition, then one of the parents is recognized for their own shared symptoms with their child.

Officially I am diagnosed with Fibromyalgia, a common comorbidity to Ehlers Danlos Syndrome (EDS). Unofficially I have the latter condition also, though officially diagnosed as Hypermobile Joint Disorder. Fibromyalgia is a list of symptoms that don’t quite fit together for any other diagnosis. EDS has strict and limited diagnosis criteria that doesn’t include many of the symptoms associated with it. The additional symptoms are recognized as part of the condition, but not accepted as forming part of the diagnosis.

In short, Fibromyalgia is statistically more common and therefore easier to diagnose then EDS.  In turn EDS is less likely to be considered as it is statistically less common.

A diagnoses paradox is formed from this approach.

If a condition is diagnosed more often, and therefore considered less rare, would it make the condition considered as a possible diagnosis more often?

The mascot of EDS is the Zebra, a symbol taken from that earlier mentioned old adage. EDS has been maligned as a likely diagnosis for decades. Despite the symptoms been well documented, the condition has not been well recognized.

There are several official forms of EDS. The various forms of EDS make up just over a dozen of the several hundred or so recognized types of collagen disorders of different types and names.

Collagen is a structural protein. A glue that forms our connective tissues and holds our bodies together. It is found in almost every aspect of every tissue group, therefore leading to various problems that differ depending on which proteins are faulty in an individual. For some people this will affect their vascular system, others their skin, for some their ligaments and muscles. Some people with EDS will have a swathe of issues, some a few, some will have just one or two problems. The condition is genetic, passed down from one generation to the next.

For hyper-mobile joints, common to EDS, there is a measure called the Beighton Scale. This officially recognizes the extent of a person’s hyper-mobility. My adult measurement is four from a total of nine. My preteen daughter’s official score is seven. During a recent conversation we discussed her hyper-mobility and how that relates to her needing to care for her body. I explained how our ligaments are much like rubber bands that hold our joints in position, and that our muscles are what then move those joints. Her observation, verbatim, was ‘all my rubber bands are loose.’ Yes darling – they are.

The benefit of the cool party tricks that hyper-mobility provide is far outweighed by the cost of a lifetime of management and maintaining a level of strength and fitness which is not required for most people. In a reasonably fit EDS body of my type, pain and fatigue are the biggest, but far from the only, inconvenience. If my body is not well maintained, the issues become substantially harder to manage. At my worst time I required a walking stick to maintain my balance, and could only exert minimum effort before fatigue overtook my abilities.

In my normal day the small muscles used for standard movements and posture have to work substantially harder than those of an ordinary person due to the lax ligaments that would normally hold the body to position. This can result in ongoing sustained muscle strain. In other words: constant chronic pain.

I now wear the anguish of knowing I’ve unwittingly passed these faulty genes on to my child. My pain, physical and otherwise, will be my daughter’s pain too.

My journey to diagnosis started in 2013 after the strain of life had started to wear down my resolve to work within my physical attributes. I’d always had pain, strains, and minor injuries, and had figured these to be part of a normal body. They were of course my normal, but not the normal of that the average person.

My GP took the standard approach, as he was trained. I was worn down, stressed, this was, as far as he could diagnose, causing my physical symptoms. He prescribed medication to help with the pain. I didn’t ask what the drug was, just followed advice. It was an anti-depressant. A standard medical system response to anything not easily recognized for diagnosis. These left me feeling less stressed, but no less symptomatic. They also dulled my mind and senses. I was left numb.

The medications were changed, others were trialed. I was placed on a psychological roller coaster from manic highs to crippling lows. When those medications seemed ineffective, or not effective enough, more were added. After some time, I was diagnosed with Fibromyalgia to explain the muscle pain. In more time I developed Parkinsonism’s. These been symptoms of Parkinson’s disease not directly related to that disease. In my case it related to the medications. I was given medication to treat those symptoms. Medication after medication after medication… All trial and error – as that is the medical system once it enters the grey areas of its unknowns. My clarity of thought and ability to function left me. The business I had started and worked to build for nine years became increasingly ill as I did. With no options left, I called in the liquidators in May 2015.

The business was gone, so soon would be the stress, so I was told. I would be better. A few months passed. I wasn’t better.

After some research I found that my symptoms most closely matched a connective tissue disorder, caused by deficient collagen. EDS to be precise. The GP advised it was too rare, very unlikely, not worth considering. We both heard hooves. I was prepared to investigate the sound further. He was interested in pursuing a different possibility.

He considered Mitochondria disease, and instructed that I should have a muscle biopsy for such. I arranged the surgery with a brilliant surgeon I had previously seen for several hemorrhoid repairs. Yes, several. Lax collagen in the bowel and associated areas causes several complications, such as prolapses and similar. This however, yet another secondary symptom of EDS, was decided to be caused from straining, nothing more. This despite it been unusual for someone to have multiple surgical repairs of the type; especially before age thirty-five.

The muscle biopsy surgery proved slow to heal, another symptom of EDS. You can trace the reducing ability of my body to heal through the various scars from more than a dozen surgeries over thirty something years. The biopsy sample proved negative. The post-surgery recovery lead me further down that path of addiction with the introduction of Targin, a slow release opioid.

I had till that time regularly taken Endone. Though not a treatment of symptoms, it was a great treatment for pain; for a while. The nature of opioids is that over time the body adjusts to the dose, and a higher dose is required. The more you have the more your body craves. This is addiction in action. Targin provided the same relief as Endone, but extended over a slow release period of twelve hours. Sweet golden pain relief. It’s short lived though. Not by dose, or days. Short lived by effectiveness in a few weeks. The longer you take an opioid the less effective it becomes, the higher the dose you need for it to be effective for pain. The more you take the more your body needs the opioid just to function. You literally become ill between doses. You’ll be nauseous, vomit, have a fever and shakes. The pain feels worse. You take the opioid not just for the pain anymore, but also to not feel ill. Some refer to this as ‘dope-sick.’

Any pain isn’t worse than what it was before you started opioids, but it does feel worse between doses. Your mind adjusts and makes the pain seem worse than it is, or ever was.

Imagine a clamp on your arm, pinching your skin. You take an opioid and can no longer feel the clamp. It’s still there, you can see it, you can’t feel it. The opioid wears off. You feel the clamp again. Now though it feels as if there is ten times as much pressure. The clamp hasn’t changed. It’s still there, exactly the same. The mind seeks the opioid hit. The mind makes the clamp feel tighter, so you’ll give it that hit. Once you’re past the withdrawals of the opioid, the clamp feels the same as before the addiction. The clamp is still the same clamp. The mind made it seem worse to increase the chance of another opioid hit.

I’ve experienced the same pain before and after opioid addiction for chronic pain management. The pain feels far worse whilst managing with opioids; the very thing that is supposed to reduce that pain.

I did what every opioid addict does. I increased the dose. I took it more often than I should. I became less mobile, I gained weight, I became even more less mobile. My struggling ligaments failed to hold my weight. My unused muscles failed to support the slack of loose ligaments. So on and so forth. It’s a death of a thousand cuts. It’s slow. It’s deliberate. No one cut is the final blow. They just all add up. A time of reckoning eventually comes.

Unhappy with the little diagnosis progress made, so far as it being only to confirm what was not wrong with me, and nothing to confirm the experienced symptoms, other than a loosely fitting Fibromyalgia diagnosis, I returned to considering the one condition that matched my symptoms – EDS.

I contacted the NSW Hospitals Genetics program and booked an appointment. With an appointment confirmed I requested the required referral from the GP.

In the interim time from 2013 to July 2017 I had seen three neurologists, two rheumatologists, a couple of psychologists, several physios and a pain management doctor, and his pain management counsellor. I was even asked, on more than one occasion, by more than one specialist, what was the point of seeking to rule in or out EDS. As they reasoned, it couldn’t be treated or cured if it was confirmed. As far as they were concerned there’d be no benefit to a diagnosis.

What’s in a name?

A name provides the ability to identify the list of symptoms with one name to any future treating doctors. The ability to gain support through and from people also afflicted by the same condition. The ability to find a way to manage what you have; to play the cards you’ve been dealt.

The problem with specialists is they specialize; not one considering the other specialists’ areas of expertise. They all consider their own areas of knowledge only. Rarely, if not ever, working together to discuss the end patient’s needs.

There’s always an exception though, for me this was the geneticist. This specialist relies on all the information provided by all the other specialists, plus their own investigations, to form an overall picture of a person’s health.

In July 2017 I received a diagnosis, of sorts, of EDS. I’m still in medical limbo due to the semantics of the condition’s definitions. I demonstrate several of the comorbidities of EDS, including dietary, blood pressure and other problems. These comorbidities however recognized as part of the condition are not recognized as part of the diagnosis. I have hyper-mobile joints, however my score on the Beighton scale is too low for clear diagnosis purposes. This despite my upper-back being significantly hyper-mobile, as that area of hyper-mobility is not counted in the official Beighton Scale used for diagnosis purposes. Currently I’m labelled as having EDS that cannot be confirmed under the current diagnostic criteria. This could change with further symptoms developing, another close relative receiving a confirmed diagnosis, or a change in the diagnostic criteria. Semantics. I have been diagnosed as having Hyper-mobile Joint Disorder. In my view the version of EDS known as hEDS, just by another name.

Sometime during that same period I was started on another medication to coincide with Targin, with a view I may decrease one medication dose as I increased the other. This new poison, as all medications are a poison of some sort, was called Lyrica. This is a drug that, at least at the time, came with a little leaflet that explained that the manufacturers didn’t know how the drug worked, but they did know the affects it had, and therefore how it could be used. For me the intention was for Lyrica to reduce the body’s nerve system from feeling pain. For this Lyrica works, for a while, then you need to increase the dose, so on and so forth again. Lyrica also has unwanted side effects for many. Mine started a few days after starting the therapeutic dose, when I was hospitalized for several days for severe loss of functionality, including blurred vision, low blood pressure, and a racing heart rate. After that initial concern I had several weeks of mania. I felt good, really good, and made several stupid decisions based on no sensible logic. Then I crashed. The lights of my mind seemed to just dim. I could no longer read more than a few pages at a time. I could not concentrate; I could not hold an abstract idea in my mind. I couldn’t maintain short term memories. I felt dull. This made me frustrated. I’d traded pain for a loss of mind.

Opioids made pain worse. Lyrica stole my mind – at least the bits that counted to me. Both drugs were addictive. I looked for support from others on the substances. I discovered that my dose of Lyrica was double that of anyone else I knew. I was on the maximum dose that could be prescribed. I had to reduce the dose. I had to embrace the pain. I had to live through debilitating withdrawals from two addictive substances. One at a time, spaced out over twelve months.

Sometimes I would be reduced to sweating profusely, fighting fever, rolling on the floor in racking full body pain as my muscles contracted and contorted. This was a process I went through twice. Once for each addictive drug. The opioid my mind craved, the Lyrica my body demanded. Neither were needed in the end. The months taken to achieve freedom from addictive drugs was worth the effort. Despite the numbness of mind I felt through that time, there was one clear thought, life will be better once the addiction is over.  

The story does not end there. I wish it did. I’ve been clean of regular opioid use for over two years. I’ve been clear of the Lyrica for over eighteen months. I can think far more clearly. I can concentrate. I now read several books a month, usually novels. I’ve gained in life, yet have lost in ability to hide from pain. The pain is always present. Across my back, over my left shoulder blade; in my neck. In my stupid right ankle that flops and flips about from the loose ligaments. My activities will dictate which pain is more noticeable. My ankle when walking, my back when sitting, my shoulder when doing chores around the house.

People that have known me through the last few years of my life tell me I look better. Perhaps I do; not being an addict anymore may assist in the way I present.

Am I better? No. I am managing better. I have a condition; it has a name. From that name I’ve found I can manage the symptoms according to what works best for me.