In Detail

I slept for fourteen hours last night, with a break in the middle to feed the cats. I think that should be enough to get my act together and explain to you the incredibly complex disease that I have. Don’t worry if you don’t understand it all on the first read-through – it’s so complex that I listened to a three-hour lecture on the subject twice. I’m going to try to give it in layman’s terms, but if I use a medical term (I’ve been using them so long now that I might forget what is accepted as common English), please ask me to define what you don’t understand.

First, I will explain Myalgic Encephalomyelitis (ME). The term ME is often used interchangeably with Chronic Fatigue Syndrome (CFS), but they aren’t exactly the same thing. I mentioned in a previous post that CFS isn’t really a diagnosis, but rather the medical community’s admission that there is something mysterious wrong with a patient. Well, ME is a defined disease process, with recognized symptoms and origins of those symptoms. I’ve recently learned that there is a movement within the ME community to get rid of the term CFS. The reason is that CFS can describe myriad diseases, from depression to heart disease and many other defined disease processes. There is a common belief that CFS is a somatization disorder, which means that it is first a mental problem that manifests with real physical symptoms. Well, if you are a heart disease patient with CFS, treating you for depression isn’t going to make you better and it ignores the physical problem. Conversely, if you have depression, treating you for heart disease isn’t going to help either. I think of it as diagnosing a patient with “runny nose.” Well, the runny nose is a symptom, not a disease, and it could be caused by an infection or allergies (or other things, I suppose), and these defined diseases have very different treatments. With these limitations in mind, I understand the ME community’s concern about the terms being used interchangeably. I see another side of the coin, though. Without seeing these terms used synonymously, I would never have learned what my real disease is. I think that it might be more accurate to describe CFS as a category of separate, chronic diseases that look similar to one another but have different causes. The most helpful thing for me would have been to see a website that listed diseases that can cause CFS. I could have investigated each one of them individually and arrived at my answer more quickly.

I found that other countries did a better job of defining ME than the United States does (CFS is a definition used by our Centers for Disease Control. I am not certain that the CDC recognizes ME as a separate disease). I found the Canadian definition of ME to be a very accurate description of the symptoms I had that don’t even seem to be related to one another. Here’s my summary of the case definition in plain language:

  1. Expending energy, whether mental or physical, leaves the patient more exhausted than is reasonable for the level of energy expended. Also, other symptoms worsen when the patient has exceeded his energetic limits.
  2. The patient has all sorts of sleep disorders, from insomnia to hypersomnia (sleeping a lot), inability to sleep for a full night, trouble falling asleep, etc.
  3. Pain is present at the neurological level, in the muscles and in the joints. There may also be headaches of new types or intensity.
  4. The patient has trouble when receiving too much neurological stimulation. It can make him sensitive to noise or light and it can make him oversensitive to emotional stimulation. Also, the patient may have trouble with memory, concentration, disorientation, etc.
  5. The patient will have at least one of the following subset of symptoms: a) disorders of the autonomic nervous system such as dysregulated blood pressure, irritable bowel syndrome, bladder dysfunction, and heart rhythm irregularities; b) neuroendocrine symptoms (meaning the interaction of the nervous system with hormones) such as inability to tolerate heat or cold, abnormal appetite, weight change, hypoglycemia, and emotional instability; and c) immune symptoms such as swollen lymph nodes, sore throat, flu-like symptoms, change in allergies, and hypersensitivity to medications.
  6. The symptoms must persist at least six months. (I will note that the disease can be suspected sooner, and it makes sense to start treating right away so more symptoms don’t develop and the damage doesn’t become permanent. If it goes away before six months is up, it wasn’t ME, but the patient was still treated. Better safe than sorry.)

There are a lot of symptoms here, and I have had them all. They seem unrelated unless you understand just how the whole disease process works. The website for the Myalgic Encephalomyelitits Society of America is the best place I’ve found for getting a complete understanding of the disease. I will give you my simplified version here, in layman’s terms (to the best of my ability). If you want to understand the disease more completely than I am explaining it here, I suggest you explore the entire website I listed above.

To understand the disease process in its entirety, you have to follow the work of Dr. Paul Cheney. There is a link on the ME Society website to a three-hour presentation done by Dr. Cheney thoroughly explaining what I am going to try to simplify here.

First, you have to understand how the heart works. Basically, the heart relaxes and fills with blood, then it contracts, pushing blood through the circulatory system. Contrary to what you might expect, it takes more energy for the heart to relax than to contract. Think of this as if you have metal spring. It takes energy to pull the spring straight, but when you release it, the spring returns to its coiled (contracted) state. This is what is relevant for understanding my condition, but it is not the whole story. For a complete description about how the heart and circulatory system work, you can read this website.

The root cause of the ME, at least in my particular condition, is cardiomyopathy (heart muscle weakness). In traditional cardiomyopathy, there are problems in the systolic phase, or the contraction phase (generally structural problems in the heart, and sometimes blockages), so the heart doesn’t pump enough blood to the body. I don’t have that kind of cardiomyopathy. My cardiomyopathy is in the diastolic, or relaxation phase of the heart beat (and what’s more, my body is compensating for this heart malfunction). Due to a mitochondrial malfunction (which I think is too complex to explain here, but if someone wants me to do so I will be happy to write another post about that), the heart doesn’t have enough energy to suck the blood that was just oxygenated by the lungs back into the left ventricle (which is why this is called left-sided heart failure). In my case, some of that blood backs up into my lungs, making me cough. When heart failure leads to fluid being left in inappropriate places in the body, this is called congestive heart failure. The doctors thought this was asthma when I first presented with this phenomenon, but I was clever enough to realize that was the wrong diagnosis almost a year ago. I think (but I haven’t heard anyone say this yet) that when you start having trouble with congestive heart failure, your body compensates by decreasing blood volume to stop that from happening.

So, I’ve established that I have the heart failure. The next question is, how does it cause the symptoms of ME? Above all else, the body preserves blood pressure. I’m going to steal Dr. Cheney’s metaphor about using a garden hose to water your plants to explain what is happening here. Say you have the faucet your hose is connected to turned all the way up. The water from the hose can reach all the way to the back of your garden. All is well. Let’s say someone comes behind you (without your knowledge) and turns the water pressure down halfway. Now you can’t water your entire garden. So what do you do? You put your thumb over the end of your hose to increase the water pressure, and you can water the plants in the back again, but it will take longer to get the same volume of water to them. Well, the body does something similar. When your heart (the faucet in the metaphor) can’t put out the proper blood pressure, the body has to compensate somehow. The first response is for the body to access your adrenal response. It raises your adrenaline levels, which speeds up the heart, increases blood pressure and opens airways. The problem with keeping that up forever is that eventually, your adrenals will fatigue. When adrenal fatigue happens, your body has to compensate by, effectively, putting its thumb over the ends of the blood vessels and reducing the supply of blood to organs in reverse order of their necessity for survival. When this happens, you get the defined process by which ME occurs:

  1. Skin and thyroid function – when you get too warm, your body’s thermostat (we’re going to call this thermostat the central nervous system – CNS for short – but the reality is the hypothalamus recognizes actual temperature change. It’s just that in the end, all of this is about the central nervous system, and I’m trying to make this simpler) responds by sending blood to the skin to offload heat. What happens when you no longer have enough blood volume (above and beyond what is directly needed to keep your vital organs working) to offload enough heat? Either you die, or you downregulate your metabolism (heat is a byproduct of metabolism). The body does this by downregulating thyroid receptors, making you hypothyroid. For a while before this happens, though, you might have trouble maintaining a constant body temperature and may even have low-grade fevers.
  2. Muscles – The next thing to get under-fueled is the muscles. I think that if the mitochondrial defect in the heart is not limited to the heart, you might also have muscular dysfunction because of the metabolism problem. However, if you aren’t getting circulation to your muscles, lots of bad things happen. The muscles aren’t getting the oxygen they need to function, so they switch to an anaerobic (without oxygen) method of making energy that produces lactic acid. That’s what makes your muscles burn after an intense workout. Eventually, your muscles will waste away and you will be in constant pain. I think this explains why I can’t hold a chiropractic adjustment – the muscles that hold my spine in place are just too weak to do their job, so I get out of alignment quickly.
  3. The liver and gut – when your reduced cardiac output gets really advanced, you start having digestive problems. Have you ever noticed that, after you eat a large meal, you get exhausted? There’s a reason for that – in order to digest your food, your body has to send blood to your digestive tract to provide the fuel it needs to do the work of digestion. The bigger the meal, the more blood needed. What if you don’t have the blood to spare? Well, your gut then releases a chemical called VEGF which dilates the capillaries and blood vessels nearby to encourage greater blood flow. An unfortunate side effect of the open capillaries is called “leaky gut syndrome.” Leaky gut syndrome is when metabolic waste products and partially digested food get out of your intestines and into your blood stream. Chaos ensues (I will elaborate in the next step). In addition, your liver doesn’t get the blood it needs to function either. You may have a perfectly functional liver, but if it can’t get the blood it needs to do it’s job, it will fail to detoxify your blood making you feel sick.
  4. The brain – I am with Dr. Cheney in believing that the effects of the brain are more related to the toxicity coming from the gut than lack of blood flow (although I do think that blood flow to the brain might be a problem as well). The toxins from your gut get into your nervous system and dysregulate it. You can no longer control your blood sugar, your blood pressure, your body temperature, or even your hormone production. You can’t think, you can’t form memories, you might have seizures… I honestly think this may be related to what causes migraines in some folks. I’d be willing to bet that migraine sufferers could do a liver cleanse, then subsequently avoid foods like gluten, eggs, dairy, soy and other highly allergenic ingredients and they would have a reduction in symptoms.
  5. The heart – By the time your circulation defect is hitting your heart, I think you are in serious trouble. Look at all the previous systems that had to be deprived of fuel first – when you’ve gotten down to sacrificing the vital organ you need to keep yourself alive, that’s an act of desperation. The clue you’ve gotten to this point is that your heart will have an arrhythmia. You’ll even have chest pain that feels like a heart attack. If you go to the cardiologist, he’ll probably find that your mitral valve has prolapsed (it won’t close properly). At this point, your diastolic cardiomyopathy can turn into classic systolic cardiomyopathy.
  6. The lungs and kidneys – This is the final event that precedes death. Your lungs and kidneys shut down and you get toxic and die. That simply.

Here’s the cool thing, though – ME prevents you from getting too far along in stage five. Somehow, the body figures out that it can just cut your energy production, forcing you to rest and cut your demand for the energy it doesn’t have. The reason it does this is even more complex, and something I will only explain in more detail in another post if someone requests it of me. The short version of the problem is that there are two byproducts of ATP production (energy production) that can combine and become quite lethal – Nitric Oxide and Super Oxide. When they combine, they become Peroxynitrite, a potent free radical. It forms in a nanosecond and kills something in a picosecond. If you have tons of these Peroxynitrite molecules in your heart and you can’t reduce them to water and carbon dioxide fast enough (or if your heart cells are deprived of oxygen for too long), you will have cardiac cell death, which is what defines a heart attack.

So, how did I get into this situation in the first place, having a heart defect on the mitochondrial level. Well, the short answer is, I’m not sure and it almost doesn’t matter. Almost. It seems to be a combination of exposure to viruses, bacteria, toxins, allergens, and heavy metals. I think my problem started with an infection, probably when I was a very small child. The viruses that have the greatest affinity for the heart are in the Herpes family. No, I don’t necessarily mean genital or oral herpes. Chicken Pox and Mono are also in the Herpes family. I think about the fact that I caught Chicken Pox twice as a kid. That suggests to me that I never really fought it off well. The thing about Herpes viruses is that they live in your body forever once you get them, going through active and quiescent (inactive) phases. One way you fight this virus is with an enzyme called RNase-L. It chops up messenger RNA (mRNA) from the virus, keeping it from replicating. Mistakes happen in the body, and at some point it appears that the RNase-L starts to think that human mRNA is viral mRNA, so it chops that up, too. Eventually you get transcription errors that lead to mitochondrial malfunction. The significance of this is that I have to be careful about what infections I get because each time I get sick, I seem to get worse permanently.

So, that’s what happened. Complex, isn’t it? The next step is to get an official diagnosis from a medical doctor, because that can open up a few doors for me under the Americans with Disabilities Act. As much as I adore Dr B and owe him my life at this point (I will discuss this further in another post), he is a chiropractic doctor and his support of my self-diagnosis means nothing to those who enforce laws that will allow me to do such things as attend medical school in a modified manner (not having to go to class to hear lectures, taking exams when I feel well enough, that sort of thing).

All right, I’m back up and fighting. Look for more posts in the future about subjects like the psycho-social impact of the disease, the medical establishment’s role in keeping me from getting a diagnosis, and other related subjects. Just be patient – I’m sick, and it might take some time. 🙂

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2 thoughts on “In Detail

  1. It all makes so much sense. But to figure out what was causing what when you were presenting so many different symptoms, all I can say is WOW. No wonder it took this long to figure it out. I’m so proud of you.

    • Thanks, Vickie. I honestly came to this diagnosis by pure luck. My medical doctors were certainly no help. So many of them got hung up on my thyroid disease that they stopped looking. Even Dr B kept insisting that I would not feel better until the thyroid was properly regulated. After I presented this to him on Monday, he’s changed his tune. Now I just need to convince C, who manages my thyroid hormones…

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