Tuesday, September 5, 2017

DX Severe Osteoporosis: Part IV -- Zebras



The Will

"Sometimes you don't know how deep inside you can go until you put yourself in a place where there is nothing between you and the mirror in front of you to blur the image; a mirror that shows not just your form, but your capacity--what you are made of. To give off light, along with passion you also need will. Will carries you through hard times and loneliness when no one else believes you can accomplish what you have set out to do. The will focuses the flare of passion, it is your navigator, the director of our life force, the hands that tend your flowering. Like the skeleton, will gives you form. It doesn't just support your movements, nor is it just a reflection of your musclular capabilities, but your will defines the absolute raw intensity of your nature, and by this you can bring into being all that lies within you as potential."
Crucibles of Will


This is Part IV of a multi-part essay telling you about my own personal experience with osteoporosis.


Zebras

After receiving "the diagnosis" I realized I had been getting signals for some time that something was wrong. My hands and lower back ached constantly; I had about as much energy as a dog in August; and felt cold all the time, as if it were perpetually January. My skin, especially on my chest and arms was incredibly sensitive and I hated being touched in these places. I was irritable and I guess I was depressed, although I would have denied it completely. Sharp noises or gentle pokes from my children during play caused a sickening wave of adrenaline response to course through me. The two fractured ribs I sustained while training for the American Berkebeiner, a 50-kilometer cross-country ski race in Wisconsin, now made more sense. I had just been pulling hard on the ski poles going up the hills...no falls, no direct trauma; just intense muscular shear forces on the now obviously structurally unsound bone.

The newly discovered frailty also began to take its toll on my professional life as a chiropractor. Every time I entered a treatment room I felt as if I were taking on a sumo wrestler...heaven forbid if the patient was 6 feet plus and weighed over 200 pounds. Chiropractic treatment in general is very precise and gentle, but it does require some motions that impart more physical stress to the doctor than they do to the patient. Even when treating normal-sized adults, adjusting them would send shock vibrations rattling through me. It was as if my resilience and strength had faded away, and it wasn't the running shoe that was broken down in the morning, it was me.

Lab tests are an important source of information, not only about what is going on inside a body, but also about what isn't going on. Over the next five years laboratory testing would play a huge part in my life as I tried desperately to find out not only why I had osteoporosis but how to fix it.  Severe osteoporosis (-4.3 T score on bone density examination) in a relatively young male is rare. For several weeks after the initial diagnosis the endocrinologist thought there may be a more serious disease process lurking and that it was the source of the unusually severe bone theft from my body. The list of diseases that can cause extensive bone loss is very long. From the common condition where one of the parathyroid glands becomes overactive (hyperparathyroidism), to the deadly bone-destroying multiple myeloma. We had to rule each of them out. Initial lab work helped eliminate most of the dreaded disorders. The only real clues we came up with were that my urine was rich in calcium and had large amounts of bone collagen in it, the result of active bone destruction--way too much of it.

There is an old saying, "When you hear hoof-beats, don't think zebras." In other words, if you hear hooves behind you, don't expect to see a zebra when you turn around...it will probably just be a horse. Dr. Theodore Woodward, a medical doctor in the 1940s, coined this phrase as a way of encouraging fellow doctors not to delay treatment by looking for rare, complex diseases at the beginning of the differential diagnostic process, because in all likelihood, the patient's symptoms are probably being caused by something simple and easy to treat. There is a lot of truth to this...although sometimes, there are zebras.

To rule out the systemic mastocytosis the doctors had to drill a hole through my bone and take out a core sample to look at under a microscope. As an added benefit to this procedure, it was also a good way to see how my bones were really holding together and to assess the quality of my bones. 

Normal bone on the left. My biopsy on the right.
During the two weeks before the biopsy, I took two short courses of oral tetracycline. This would be absorbed into my bones and used as markers for the lab to determine the pace at which my bone was being made. The bone tissue was taken from the right side of my pelvis. The surgeon from UConn Health Center first injected a local anesthesia into the area, made a small incision and then, with a hollow hand-drill called a trephine, began drilling through the bone. The trephine was shiny but still medieval-looking, and had a rudimentary handle on one end with large jagged teeth on the other. The doctor took her time twisting the trephine; I could feel the teeth as they passed through each layer of bone. She explained that it was easy to damage the sample; osteoporotic bone is fragile, especially the trabeculae--the lattice of three-dimensional structural beams sandwiched between the inner and outer cortex that give bone its ability to be light yet strong. Trabeculae are designed to withstand the forces of normal activity, like running, but not drilling. If she used too much force, the trabeculae would snap and the pathologist would not get a good picture of their integrity, their "connectivity."

I must admit, having a hole drilled through my pelvis was unnerving. I know it really wasn't a major procedure, patients have a lot worse things done to them. It didn't even hurt that much except for the injections of anesthesia through my abdomen to help numb the inner part of my pelvic bone. But the whole procedure just seemed crude. My response had nothing to do with the doctor; she was extremely sensitive, caring and competent. Maybe it was the sedative she gave me, but during the surgery I kept thinking about the ancient Indians of South and Central American, and how archeologists have discovered skulls with holes drilled into them. Holes that had healed around their edges, indicating that the people/patients had been alive when the procedures were performed. Although they were performed with the intention to heal, it had to have been excruciatingly painful--such an invasion, an assault upon those individuals. What was being done to me had been done to them long before--the drilling-- the same procedure of a person twisting a tool into the inner physical being of another.

The doctor finally broke through the inner bone cortex to the softer tissues underneath, and pulled out a 3/4 inch, pencil-thick, piece of bone. She placed it carefully into a small bottle of chemical solution and handed it to me. I had never seen a piece of my body like that before. It was all so odd to me.

I had been told that the aftereffects would not be any worse than if I had bumped my hip into a kitchen counter. That description might have been accurate, but only if I had been running twenty m.p.h. through my kitchen when I hit it. For the next three days I dragged my leg around like a lead weight; I couldn't even think about running for two weeks.

Trabeculae in bone should be plentiful, robust, and intact. What we found when we looked at the biopsy slides was that mine were few in number, thin, and disconnected. In a crime lab with just bone to look at, a technician would have pinned me as a 100-year-old malnourished female. Rapid loss of bone does that to trabeculae. When osteoclasts become aggressive, devouring excess bone and leaving large deep gouges in the bone surface, even normal functioning osteoblasts are incapable of fully filling in the holes with new bone. The result is pitting in the cortex of the bone and trabeculae that get thinner and thinner until they separate from adjacent bone.

Looking at my bone biopsy under the microscope, we could see a lot of the trabeculae just hanging there like stalactites and stalagmites giving no strength at all to the bone. It was similar to the beech trees in New England that become weakened by a fungal pathogen. Hidden from view just under the bark, the beech's inner structural core is silently being eaten away. To the untrained eye, the trees appear healthy enough, beautiful majestic giants in the forest, until a small wind topples them over...the result of "beech snap." Osteoporosis is similar. Often silent, often afflicting healthy appearing individuals, but underneath hidden from view are overzealous osteoclasts eating away, like a fungus, at the structural core.

As with the wood in "beech snap," trabeculae, in osteoporotic bone, become incapable of resisting the stresses of everyday life. Hips snap and the spine crumbles, and like the broken beech that can't regenerate, the trabeculae can never re-attach. The manner by which bone forms does not allow for it to develop where there is nothing but empty space. This is where all those articles I had read about severe bone loss being "irreversible," began to make sense. But I did not want to accept that. I wanted to find a way around this seemingly impossible physiological impasse. There had to be a way, not just to gain back density by using a drug, but to gain back both the bone density and the structural integrity of newly remodeled bone.

The biopsy ruled out mastocytosis but actually brought up more questions than it did answers.
Zebra striping. Intermittent bone formation.
Besides having disconnected trabeculae, my bone had an odd appearance. The biopsy report called it "zebra striping," or intermittent bone deposition. There would be up to four layers in some areas, with new bone and then no mineralized bone, then bone, then no mineralized bone. It looked like the layers of different soils at an archeological dig. The endocrinologist estimated that the time-lapse between each stripe was days or weeks, not months. Neither the pathologist who read the biopsy nor my endocrinologist knew what to make of the queer stratification. They had seen this striping before on rare occasions with metal toxicity, but the biopsy had been stained for aluminum and iron and it came out negative. Lab testing of blood, urine, and hair for other heavy metals came up negative as well.

It was becoming clear to me that the only way to track down and destroy the beast that stole my structural strength was to immerse myself deep in the study of osteoporosis. I wasn't about to leave this solely in the hands of doctors who would drop me into a file drawer and leave me there for weeks and months at a time. I began to read everything I could about bone biology, and specifically, osteoporosis. I went to osteoporosis seminars and week-long conventions. I took courses on how to read bone density exams. I joined the American Society for Bone and Mineral Research, the International Bone and Mineral Society, and the International Society for Clinical Densitometry. I had dissected a lot of human cadavers in anatomy class at chiropractic school so I knew what it was like not only to study something intensely, but to pick through each and every part of a very complex puzzle. I wasn't just going to study this disease, I was going to rip it apart, piece by piece, clue by clue. Like a dog kicked into a corner, (s)he will come out fighting. I may have skipped a few beats when I first heard the words "severe osteoporosis" as my diagnosis, but my heart quickly rebounded, pounding like a competition-starved athlete ready to do battle. I was sure this beast wasn't much tougher than some of the big ugly guys I had competed against when I was vying for a spot on the USA Olympic Team. I might not win but I sure as hell would attack with everything I had, even down to the center of my physical being, down to my bones. Or at least what was left of them.

"When you go to the core of anything, you go to the heart of its reality, its truth, its real substance."



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