by Dr. David Bell
This is the case history of a nurse who improved dramatically after a blood transfusion. It is excerpted from Dr. Bell’s book, Faces of CFS – Case Histories of Chronic Fatigue Syndrome, published in August, 2000. “Mary Ann’s Transfusion” illustrates one aspect of cases he has followed since the mid-1980’s.
Reprinted with the kind permission of Prohealth.
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Mary Ann was a bold and highly competent nurse in the intensive care unit of our local hospital, and I depended on her. Given that my specialty is pediatric medicine, I was always a little nervous treating adult patients who were in the midst of medical crises. I quickly learned I could count on the vivacious Mary Ann, who was then in her thirties, for the most up-to-date and reliable information about my patient’s status. Often, she suggested successful solutions to problems for which I had never been trained in medical school, like constipation. There should be a course on constipation in octogenarians who have suffered heart attacks, but there isn’t.
In 1985, when the epidemic of CFS in Lyndonville began, eventually affecting more than two-hundred people in a town of 1,500 (or whatever), Mary Ann got sick, too, although some years passed before her diagnosis was readily apparent. The one constant, however, which began in 1985, was exhaustion. Mary Ann was weighted down with a panoply of other problems, as well: arthritis-like pains in her joints, sinus infections, allergies, migraine headaches, chest pain, shortness of breath. As the years wore on, these symptoms and many more resulted in her slide from being perhaps the finest ICU nurse I have ever known to little more than – in the eyes of the medical specialists she consulted – a hypochondriac. As a result, she was inhabiting a remorselessly bleak netherworld.
I tried to help Mary Ann, too, over the years. It was rapidly apparent to me, and eventually to her, that she had same disease from which so many of the patients she had met in the hospital in 1985 had suffered. I tried everything I could think of by way of drugs and other therapies to help her, but none of them worked to any appreciable degree. There was an aspect of her case that I found very interesting, perhaps because it seemed to me to be one of those elusive clues that might lead me to a more comprehensive understanding of the mechanism of the disease.
Mary Ann had a remarkable requirement for water. She rarely came into my clinic without a large container of ice water, from which she sipped continuously. I was pretty sure it was water, anyway; if it was vodka, I knew we were in for some big problems. It turned out not to be vodka, of course. Once, I asked Mary Ann to keep track of the amount of water she drank in one twenty-four hour period. Her consumption turned out to be four gallons a day. Persistent thirst is a sign of diabetes, and Mary Ann did indeed have diabetes, in addition to all her other problems. She had a rare kind, however, one called “diabetes insipidus,” which is not the “sugar diabetes” with which most people are familiar. In the primitive days of medicine, doctors would actually differentiate between the two forms of diabetes by tasting their patients’ urine. If the urine had a sweet taste, their diagnosis was sugar diabetes, known today more formally as “diabetes mellitus.” In the case of diabetes insipidus, the urine was tasteless, or “insipid.” Recalling this piece of medical history makes me wonder if I really want to return to the good old days of clinical medicine.
What struck me as most interesting about Mary Ann’s diabetes is the fact that it is caused by a deficiency of the brain hormone, vasopressin, which, as I have noted, is a common finding in CFS sufferers. Among its many functions, vasopressin helps the kidneys absorb water and maintain the body’s fluid balance. When vasopressin is low, or missing entirely, water runs through the body without being absorbed, like a bucket with a hole in the bottom. Usually, diabetes insipidus signals damage to the pituitary gland in the brain, which manufactures vasopressin. Paradoxically, in spite of her gargantuan intake of water, lab tests suggested that Mary Ann was living in a state of perpetual dehydration.
I decided to measure the volume of blood circulating in Mary Ann’s body. In case anyone was wondering, it is not a good idea to measure someone’s circulating blood by taking it all out at once. Instead, a high-tech system deployed in a hospital radiology department requires only two small blood samples to determine the body’s entire circulating blood supply. A normal person has about five quarts of blood in their system. It turned out that Mary Anne had only 51 percent of the amount of circulating blood that would have been considered normal for a woman of her age and size.
This was a startling finding. If a person loses as much as 40 percent of their blood volume suddenly, they will usually die from shock. In science and in medicine, when one is trying to calculate what is “normal,” and what is “abnormal,” one will always encounter pitfalls. Mary Ann’s radically reduced blood volume caused me to think about what constitutes a true “normal” value for blood volume. Mary had gained weight in the course of her struggle with CFS, for instance, and I wondered if blood volume can be reduced in people who are overweight. I tried to come up with all sorts of explanations for Mary Ann’s radically low blood volume. In the end, I could draw only one conclusion: no matter what the mitigating circumstances of her case, Mary Ann’s blood volume was below normal.
The blood volume assay has been used for almost thirty years, and is considered to be highly reliable. The fact that Mary Ann – no matter how hard she tried – could not possibly fake the results of this particular test, seems especially significant to me given the vast array of medical experts who continue to insist that CFS is a psychosomatic condition. I will give my little finger to anyone who can successfully argue that this finding was psychosomatic.
I must confess that I am not the first doctor to discover that CFS patients have dramatically lowered blood volumes. Dr. David Streeten had been measuring the low blood volumes in CFS patients for some by the time I tested Mary Ann for the condition. Mary Ann just happened to be my first patient in whom I personally observed the phenomenon.
Dr. Streeten and I, being doctors, were excited by these findings and were enjoying our collaboration. Mary Ann, the patient, was enjoying very little about her life. In fact, she told me she was eager to experiment with therapies. I felt that her informed consent was reliable, in part because of her nursing experience, which had left her well-versed in medical matters, and because she was, in my opinion, entirely rational. Thus, in December of 1997, I decided to attempt to raise Mary Ann’s blood volume level by giving her injections of vasopressin. I reasoned that, if she improved, I could reasonably suspect that low blood volume was at the root of her symptoms and might also play an important role in CFS.
Unfortunately, complications with this therapy set in almost immediately. Mary Ann began suffering from “water intoxication,” the main risk of this treatment, since vasopressin causes the body to retain water. The newly expanded blood volume causes a dilution of the salt in the blood and Mary Ann’s blood sodium levels dropped markedly. Upon measuring her blood sodium, I halted the drug therapy immediately.
I was relieved when I determined that Mary Ann had suffered no physical difficulties because of the sharp drop in her blood sodium. In fact, curiously, two days after the therapy was stopped, she began feeling better than she had felt in years. However, the risks that accumulate with water “intoxication” and low blood sodium levels, in my mind, failed to justify the treatment, and we abandoned that particular effort.
I found myself unable to sleep at night thinking about why Mary Ann had felt so much better after her vasopressin infusion, even when it had caused complications. I was, and remain, perplexed by this seeming contradiction. Clearly, low blood volume levels are important and meaningful in some way to our understanding of the mechanisms of CFS, but, I am sorry to report, the puzzle remains unsolved at this date. And, soon enough, Mary Ann was just as ill as she had been prior to the vasopressin treatment.
Then, a wonderful thing happened: she developed a bleeding ulcer. I suspected the problem could be blamed on the large doses of ibuprofen Mary Ann routinely swallowed to control her muscle and joint pain. At any rate, the hemorrhage inside her abdomen caused her so much pain that Mary Ann rapidly found her way to the emergency room of our local hospital. The staff there determined that she was bleeding internally, and they followed standard protocol for such a condition: they gave her a blood transfusion. In fact, they gave her several pints until her stomach quit bleeding, at which point they sent her home. She began keeping a journal the day she came home and called it, “Diary of Lab Rat # 1.”
I always ask my CFS patients to fill out a questionnaire before each visit. I sometimes suspect that they believe these questionnaires, which force them to respond to a great many detailed inquiries about their symptoms and their level of activity, are a kind of punishment. Inevitably, these forms remind them of just how thoroughly rotten they feel. Nevertheless, I have found these questionnaires to be extremely helpful in monitoring their illness. It turns out, in case anyone was wondering, that the secret to interpreting fluctuating energy levels in CFS is in the fine details.
Mary Ann filled out one of these questionnaires the day after her transfusion and wrote that she was “whoogy and very tired.” I have never demanded of Mary Ann that she define “whoogy” for me, mostly because I can pretty well guess by the sound of it. The following day, however, Mary Ann was able to walk through the rooms of her house and even venture outside to her garden. She also discovered that, for the first time in her recent memory, she was not thirsty.
Before her blood transfusion, Mary Ann typically experienced about an hour a day during which she could be upright. A week after the transfusion, however, Mary Ann’s life began to change radically. One morning, she wrote in her lab rat diary, “Did I get healthy blood–or what???” It seemed to her that virtually every symptom of illness had been muted – dramatically. One of the most exciting new developments was her newfound sense of mental clarity, something that CFS sufferers inevitably lose when they become sick. Before falling ill with CFS, Mary Ann had been a crossword puzzle whiz; now she began to work the crosswords again. She felt as if she was opening up all the dusty, long-shuttered cabinets in her memory, and was discovering that although they had been closed for a long time, the contents had remained in tact. She found she could keep her focus on the written page, too. She took up reading after a long hiatus. One evening, she set the newspaper down and realized with wonderment that, for the first time in years, she had read the journal from front to back.
She noted in her diary that she had pulled her grandchildren, all of them squeezed together in a red wagon, down the sidewalk. On the eleventh day after the transfusion, she wrote that she had played baseball with her older grandchildren for the first time. Soon afterward, she and her husband drove 200 miles looking for the perfect pair of bicycles – Mary Ann was anticipating long rides along the country roads. After all, she felt perfectly well. Mary Ann was even ready to do a TV commercial about her hair. One of the oddest symptoms of CFS is the complaint by many patients that they are losing their hair. Women in the most severe episodes of the disease often report they find clumps of hair in their brushes. My patients, women particularly, get annoyed with me over this particular issue, and not only because it’s hard for me to objectively assess this complaint. I rarely consider hair loss to be a pressing concern when there are so many more serious medical matters to face. I suggested to Mary Ann that blood transfusions were not currently the modality of choice to correct thinning hair.
Diary of Lab Rat # 1: “No nightmares like I used to have. I don’t feel ‘sick’ all the time. I’m praying that it will last, but if it doesn’t, I pray that I won’t get depressed. I know that God could heal me in a minute if He wanted, but He’s given me this illness, and now, feeling better, for a purpose. So I guess my job is to do His will and whatever He has in His plan.”
Whenever Mary Ann came to my clinic during her incredible respite from suffering, I was always reminded of the old Mary Ann who had worked in the ICU at the hospital. She was perky; she looked great. For the first time in years I was able to enjoy that wonderful, relaxed sense of comic timing that was her hallmark. It is incredible what CFS can do to a person.
The scientist in me was at war with the clinician in me over Mary Ann’s case. The scientist was insisting that Mary Ann’s metamorphosis could easily be ascribed to the placebo effect. Finally, I had to tell the scientist to stuff a sock in it. Mary Ann’s come-back-kid status had been precipitated by an event – a transfusion for an abdominal hemorrhage – that was virtually an accident. Still, I asked myself again and again: what was the secret of the blood transfusion? What was it telling me about CFS?
I began thinking about other blood-related clues I had observed over the years. People with CFS typically report they feel much worse after lab technicians draw multiple vials of blood from them for diagnostic tests. In addition, I had observed that by administering IV fluids to people with CFS, I could often generate a temporary improvement in their symptoms. Most significantly, I realized, the constellation of symptoms that make up the entity we know as CFS, particularly weakness, dizziness, and difficulty concentrating resemble the symptoms of shock due to blood loss.
Nevertheless, from my experience with Mary Ann, I had learned that simply expanding a patient’s blood volume wasn’t the answer. I have since speculated – but it is only speculation – that the answer might lie in the red blood cells themselves or the blood vessels carrying them. Scientists in Australia and England have suggested that red blood cells of CFS patients are misshapen, and appear to be damaged. They have advanced the hypothesis that the capability of red blood cells to carry oxygen around the body is impaired in CFS sufferers. Maybe the infusion of new red blood cells into Mary Ann’s body was what made the difference. Maybe this really was, as my patient had called it, “healthy blood.” In other words, normal, undamaged red blood cells, coupled with an increase in general blood volume, might explain Mary Ann’s rebound. Mary Ann’s deterioration began six weeks later.
“Day 46,” she wrote in her diary. “Starting to spiral down.” She had to put her crossword puzzles away; her mind was growing foggy and confused. “Fingers keep latching up and dropping and breaking stuff,” she wrote a few days later. On another day, she wrote, “Dizzy if I stand up and I have to hold on to something.” The following day: “It’s been worth it…but if it’s over I have to keep from falling apart.” And finally, “Quietly starting to panic.” She was unable to pull her grandchildren in their wagon.
Months passed. My heart broke whenever Mary Ann came to the office. Searching for a therapy that might help, or at least do no harm, I decided to give her weekly intravenous infusions of a liter, or about a quart, of saline water, the salty, purified water that is titrated to mimic the salt content of our bodies. I offered the therapy to her on the grounds that we might be able to raise her blood volume simply by administering extra fluid to her blood stream. Mary Ann felt a bit better after this treatment, though its good effects lasted just two days. I continue to provide Mary Ann with this treatment on a weekly basis, because it offers her some relief, which happens to be more than any other therapy can offer.
One might ask: why not give Mary Ann with another blood transfusion? The question goes to the heart of clinical medicine. As doctors, we must err on the side of caution. In fact, my profession and the federal government each has a responsibility to protect us all from medical charlatans. Before the government can approve a new treatment for a disease, for instance, scientists must apply for grant money, submit protocols for their experimental therapy to review boards, and those protocols must be approved by these boards. After all this, the therapy must be thoroughly tested on significant numbers of patients, and the results submitted to the government for further review. It’s a good system, and without it, great harm might be done.
Doctors who work each day in the trenches of clinical medicine cannot undertake this kind of research. We would require sizable grants from the government to begin the work, an enormous amount of free time, and a veritable hoard of interns and residents to carry out the study using real patients. Clearly, a small town doc like me, living near the end of the world, cannot test in an appropriately scientific fashion the effect of blood transfusions in CFS. And it’s hard to imagine any medical journal would publish Mary Ann’s case history, because it is merely speculative, even if highly provocative. Sometimes, I feel the only solution for me is to develop multiple personalities or else go mad.
When I am wearing my “scientist” hat, I remain skeptical. When I am in my office, talking to Mary Ann, I sometimes think I’m going to go ballistic over the scientific establishment’s onerous demands for expensive, lengthy, double blind studies. Yet, giving Mary Ann another transfusion cannot be done now, as there is no established protocol for
this treatment in CFS. I’m not just worried about going to jail. It is possible that another transfusion might cause Mary Ann harm. I just don’t know.
In the old days, patients rarely learned very much about whatever disease had laid them low. There were no support groups, no newsletters, no conferences, and no unscientific books like this one. As a result, patients were rarely frustrated about scientific progress in their particular disease. For the most part, they remained blissfully ignorant about new ideas and new developments that could directly impact their destinies. Most especially, patients did not read about crackpot ideas like blood transfusions as a treatment for CFS.
I remain convinced that CFS is a treatable disease. Perhaps it will be impossible to fully reverse CFS once it has a foothold in a patient, but I believe many of the symptoms will be alleviated, once we learn how. It saddens me profoundly to see Mary Ann as she is today. Only our shared faith that CFS may someday be amenable to treatment allows me to shield her from that sadness.
