Member Profiles
Walt Hutchens
This is way too long but it has been a long, complicated journey. Perhaps our story will encourage others to keep on plugging even when you're not getting help.
From the day Sharyn and I met in the fall of ‘97 we knew that she got short of breath much too easily but we thought that was because she smoked. She quit smoking right before we married the following summer but several months later her shortness of breath seemed worse so she saw our Family Practitioner. Nothing was found but one of the ‘old standard' asthma medications, theophylline, definitely helped. She had chronic sinus drainage which could make asthma worse; when antibiotics didn't fix that, she had three hours of nasal surgery. The sinusitis got much better but she was still short of breath after a quarter-mile walk.
Our FP sent us to a lung specialist who found nothing, then to an allergist who did no better. There still seemed to be the possibility of a very unusual lung problem so Sharyn saw Dr. P., a specialist in the pulmonary department at a university medical school. A complete set of tests there proved there was nothing wrong with her lungs. "Deconditioning" said Dr. P.; "She's just out of shape." Sharyn was put on a program of brisk walking for an hour a day.
I knew from my own experience with asthma that it is important to know as much as possible about one's illness. I already had a couple of basic medical textbooks; now I subscribed to a service that allowed searching Medline, a collection of about 12,000,000 research papers in the National Library of Medicine, and began looking for papers on shortness of breath. It didn't take long to discover first that this is a very difficult subject and second that Medline study is expensive. In most cases all that's online is a brief summary so you have pay $18 for a copy if you need to read the whole paper.
Halfway through the end of the month of walking, Sharyn was considerably worse -- not only was she short of breath with even less exercise but she now had pain in her right shoulder and the middle of her back when she walked as well, so she eased up. Back at the medical school for another appointment we all stood around the examining room trying to make sense of the situation. Clearly the problem wasn't just deconditioning. "What about her heart?" I asked. A chest x-ray was normal but an ECG (electrocardiogram, the basic test of heart electrical function) showed 'Left Bundle Branch Block' (LBBB), a fancy way of saying that the electrical signals that should operate the left side of Sharyn's heart are dead. This was hardly good news but at least we thought we had a problem we could get our teeth into.
"I'd like you to see my good friend Dr. D. in the cardiology department" said Dr. P. We got the first available appointment but since that was five weeks away our FP ordered an echocardiogram (ultrasound picture) of Sharyn's heart at our local hospital. It showed that the left side of her heart was pumping less than half as much blood per stroke as it should and on the strength of that bad news, Sharyn's appointment with Dr. D. got moved up.
It was time for more study and I bought a cardiology textbook. One of the first things I learned was that shortness of breath can be the brain's way of responding to pain in the heart. Heart pain is called ‘angina' and is basically a cramp in the muscle of the heart itself caused by inadequate blood supply but sometimes the brain interprets it as shortness of breath; when this happens, shortness of breath is called an ‘anginal equivalent.' Perhaps the picture was starting to clear a little?
We saw Dr. D., he did an exam and ordered another echocardiogram and a cardiac catheterization, a procedure which allows looking at and measuring blood flows in the chambers and large arteries of the heart. He started Sharyn on three drugs, one to reduce her blood pressure, a diuretic to help her eliminate water, and digoxin, a drug to make her heart pump harder. He also stopped the asthma medication she had been taking. He was friendly, answered our questions, seemed to be someone we could work with.
Sharyn's shortness of breath and pain seemed to point toward reduced blood flow in the heart muscle itself; the tests of her heart showed abnormal motion and reduced pumping ability. These facts could fit together if the arteries supplying blood to her heart muscle (‘coronary arteries') were blocked.
Surprisingly however the cardiac catheterization showed that Sharyn's coronary arteries were clear; even more surprisingly, her heart was now pumping as normally as possible considering her LBBB. Normal lungs and an almost normal heart but she can't walk up one flight of stairs without breathing hard? Again the scene of the doctors, the patient and the patient's husband standing around the exam room looking at each other. Reduced blood supply in the heart rarely improves and Dr. D. couldn't believe that her heart was working that much better. When a different and more reliable test showed conclusively that her heart was now pumping a near normal amount of blood, Dr. D. decided the first two tests had been wrong. I didn't think so: I had watched those tests (which were done in different places by technicians who were considered very skilled) and what the reports said was what I had seen on the screen. It wasn't just the numbers that seemed right; in the first test her heart had seemed to be ‘squirming', barely contracting at all, while the last tests looked normal to a non-expert.
From reading, I knew that heart muscle can 'hibernate' -- stop working but remain alive -- if for a time it doesn't get quite enough blood. If the blood supply can be increased the hibernating muscle may go back to work. The usual reason for poor blood supply is fat clogged heart arteries but no drug will improve that very much and in any case Sharyn's arteries were normal. Could something else cause a reduced blood supply? I continued reading textbooks and searching Medline and the World Wide Web.
We saw Dr. D. again. He discussed the results of the last test, diagnosed Sharyn's condition as cardiomyopathy without known cause (‘idiopathic'), ordered a lab test of her blood level of digoxin, took two unrelated phone calls and chattered for a couple of minutes about one of them. It seemed he wanted to talk about Sharyn's heart as little as possible. After one or two other questions I asked if chest pain could happen when the heart arteries are normal and as he darted out the door Dr. D. answered "It could". Sharyn and I looked at each other and left without making another appointment. We did not know what had happened but Dr. D. was no longer someone we could work with.
Cardiomyopathy means ‘sick heart muscle'. It can be caused by many things but when the cause is in the past or no cause can be found, little can be done. As the heart weakens, it can no longer pump enough blood for the rest of the body, a condition called ‘heart failure'. Early symptoms are getting tired easily, waking up at night coughing, and swollen ankles. You can fight a delaying action against the results but the course is generally downhill and only half of such patients seen at large medical centers live more than five years. Sharyn had none of the symptoms but other than that, the news was still getting worse.
The picture, however, was very confusing. Sharyn had been diagnosed with cardiomyopathy but had no symptoms of the condition. Her symptoms were shortness of breath, and now a feeling of pressure in her chest after a little exercise, even sometimes when resting. This was typical angina rather than the ‘anginal equivalents' she had been having. These symptoms were not explained by cardiomyopathy. They would fit clogged arteries but her arteries were clear. What was going on?
My study began to pay off. The combination of (1) chest pain, (2) normal heart arteries and (3) reliable evidence that the heart muscle isn't getting enough blood is called ‘cardiac syndrome X' or CSX. Though chest pain is most often caused by clogged heart arteries, about 40% of women and perhaps 10% of men sent to a medical center for evaluation of chest pain have normal arteries. Often the problem is outside the heart (several non-heart conditions can cause chest pain) but when the pain actually comes from the heart the cause is a restriction in blood vessels which are too small to see with tests -- so called 'microvascular angina'. Sharyn had not at first had typical anginal pain but MVA victims often have anginal equivalents. Medline had hundreds of research papers, some more than twenty years old, on CSX and microvascular angina.
I learned that CSX is generally good news as heart problems go. Victims may have a good deal of pain and interference with their lives, often they have lots of expense for repeated tests, but they live about as long as anyone else. Digging more deeply however I found that when there is actual heart damage such as LBBB and when the patient has a history of smoking, the future isn't so bright. (Later I was to find a study showing that the combination of LBBB and her good response to nitrate-type medication said the same thing.) Sharyn's illness seemed to be one of the uncommon exceptions to "generally good news".
While there is no standard treatment, MVA can be attacked in several ways to reduce symptoms and (in Sharyn's case) with the hope of preventing or at least minimizing further damage. One drug frequently used in research treatment was our old friend theophylline; now we knew why the asthma medicine had helped her shortness of breath! The blood pressure medicine Sharyn had been given is also good for MVA and had most likely been what snatched her back from the edge of heart failure after the exercise program but she was still getting gradually worse: it was urgent to get a real diagnosis and if CSX and MVA was the problem, do the best we could to control it.
We asked our FP to recommend a local cardiologist and I sent Dr. E. a note outlining my wife's history and the results of my reading. At the appointment the nurse took Sharyn, saying she'd call me when she had changed to a gown. Half an hour later, she called me as the doctor finished the exam and history taking. We discussed the situation briefly, he indicated CSX was generally benign, and diagnosed cardiomyopathy without known cause. We got some useful answers to our questions on exercise. Though he obviously saw me as a problem, we could work with this man. But since his diagnosis led nowhere and he didn't seem to know enough for his rejection of CSX to mean anything, why would we want to?
I wrote Dr. D. an E-mail asking whether an ECG had been done for Sharyn's surgery a year earlier. It seemed that a former smoker nearing 50 ought to have had one before being knocked out for three hours and it might useful to know if her LBBB was new since then. I also asked the results of the digoxin blood level test. There was no answer to either question but his nurse left a message on our answering machine to double Sharyn's dose of digoxin.
Ten days later Sharyn got lost in our grocery store and poured the dog food on the counter instead of in the dog's bowl. When she woke up the next morning so faint she could hardly walk we went to our FP, stopping on the way for her to vomit. The FP diagnosed too high a dose of digoxin and reduced it. Diabetes would also fit Sharyn's symptoms and her blood sugar at the doctor's office was at the high end of normal; the diuretic she was taking is known to sometimes push someone who is close, into actual diabetes. There was no sign the diuretic was needed (no substantial loss of weight caused by getting rid of excess water) and the FP stopped it.
At home I looked up the prescribing information for digoxin and discovered that she had been told to take four times the usual dose for a woman her size. The manufacturer emphasized the danger of overdose and the need to consider the patient's total picture and not make dosage adjustments just on a lab test of the blood level.
Digoxin makes the heart muscle work harder. That can be important when heart failure has gone far enough that the rest of the body doesn't get enough blood, but the drug usually isn't needed in mild heart failure. As we thought back over the last few weeks we realized that Sharyn had had more shortness of breath and chest pain since starting the drug: could it be causing some of her trouble? If Sharyn had MVA, her heart wasn't getting enough blood; perhaps making it work harder was a bad idea. She began to gradually reduce her dosage and sure enough, her symptoms got better. Later I discovered that digoxin is known to sometimes cause angina. It was turning out to be good news that we couldn't work with Dr. D.
From more Medline study I discovered that people who have MVA generally are ‘insulin resistant'. Insulin is the body's sugar messenger: when there's too much sugar in the blood stream the pancreas makes more insulin to move it into muscles to provide energy and into the liver where it is stored for the future. However in about 25% of people this process doesn't work well. The pancreas solves that problem by making even more insulin(so blood sugar stays roughly normal) but the constant high insulin level can damage the heart and blood vessels throughout the body. Some people with IR will eventually get diabetes as their ability to make very large amounts of insulin declines with age.
In other words insulin resistance can be the first stage of ‘adult onset diabetes', also called ‘type II diabetes'. A tendency to AOD can be inherited and Sharyn had a grandmother who died from the disease. People who are overweight are more likely to be insulin resistant, particularly if most of the weight is on the abdomen. Sharyn had a few extra pounds on her abdomen. When women get MVA it usually shows up around or after menopause and Sharyn was 48. High insulin levels can make you warm and Sharyn had been bothered by heat for several years.
Sharyn had CSX with some symptoms and several facts in her history that fit MVA, which is often associated with and may be caused by insulin resistance. She had no symptoms which could be caused by just cardiomyopathy. Since insulin resistance and MVA are treatable we had to be sure that they were considered by her doctors; if they were ruled out we had to understand why. Meanwhile we had to do as much as we could on our own: Sharyn began to lose weight and eat less of foods containing sugar.
We had now seen two cardiologists who said Sharyn had a mostly untreatable condition which didn't explain her symptoms and for which there was no convincing evidence; neither doctor had explained why he rejected another diagnosis for which there seemed to be considerable evidence. Our FP didn't know what to think; he suspected a combination of some smoking damage (though that had been ruled out by the lung tests), some deconditioning (though Sharyn had gotten worse when she exercised), and psychogenic disease ("It's all in your head, dearie").
We spent a couple of weeks figuring out what to do next. Finally I wrote a long note with Sharyn's history and our concerns to the head of the cardiology department at the medical school and asked his help in locating someone interested in CSX. He answered with a suggestion of a test which he said might reassure me that there would be no ongoing damage and the name of a doctor in the department.
Reassure me? We just wanted the facts. But Sharyn made an appointment with Dr. S.
A few days before Sharyn's appointment it was rescheduled to a month away because the doctor had been called out of town. When we did see Dr. S. he did a careful exam, talked in detail about what the tests to that point showed, and told us that CSX was generally benign and that it was unlikely in Sharyn's case because it rarely caused heart damage. The test suggested by the head of department was ‘inappropriate'. He scheduled her for another test of her heart's pumping ability and a treadmill test to see if there were signs that her heart wasn't getting enough blood when she exercised. The pumping test showed that her heart was still working well but like many CSX patients she had to stop the treadmill test early because of chest pain so there was no information about the blood supply to her heart. We had been told to see the doctor afterwards but he was too busy that day and we scheduled an appointment. Shortly before that appointment we were called with the news that he was going on vacation and Sharyn's appointment was rescheduled for another month away.
I wrote Dr. S in detail about my concerns, urging him to study the literature on CSX if he had not already done so. He answered that we would discuss my thoughts at the appointment.
I went back to searching the web and found that a more distant medical school had a small group devoted to the prevention of heart disease that also treated cases of chest pain with normal heart arteries; although the name wasn't used, that would include CSX. The nearly five hour drive would be worth it if Sharyn could be seen by someone familiar with CSX. We took the first available appointment, nearly a month away. Since the appointment was early in the morning we planned to drive up the previous day and stay at a motel; we arranged a sitter for our dogs. Two days beforehand we called to get directions and found that the doctor had gone on vacation and our appointment had been rescheduled for three weeks in the future.
We went to Sharyn's rescheduled visit with Dr. S. He was sure that Sharyn had had a viral illness causing cardiomyopathy in the recent past and that the reason her heart had improved so much was that she had gotten over the virus. "We see a lot of patients like this". I asked why, if that was the problem, she had had increasing shortness of breath for five years and he said he didn't know. Why had her pain and shortness of breath gotten worse while her heart got better? He didn't know, in fact he hadn't heard Sharyn mention chest pain at the first appointment. He prescribed vigorous walking: "You can walk through the pain". (Back to "It's all in your head, Dearie"!) Why was it okay for her to walk vigorously now when she had gotten worse the first time that was prescribed? "That was before she got over the virus." He told Sharyn she could stop the digoxin but reassured us that the drugs prescribed by Dr. D. had been perfectly standard treatment for her condition.
So much for Dr. S. Now we had seen three cardiologists who would neither consider CSX nor explain why it couldn't be the problem. Basically they seemed to pick a diagnosis they liked and ignore the fact that it didn't fit Sharyn's history or explain her symptoms. It was clear that seeing doctors who hadn't previously diagnosed patients with CSX was a dangerous waste of time. After looking up the doctors at the new university medical center on the ‘net, we called and changed Sharyn's appointment to the one who seemed likely to be the most experienced with CSX. The cost was another two weeks of delay.
Sharyn had never been a morning person; she generally felt ‘washed out' and her thinking wasn't clear when she first got up. The one exception to reducing her eating of sugary foods had been a small bowl of ice cream we often enjoyed together an hour or so before bed time. Now she stopped the ice cream and it seemed that she felt better in the morning. People who are insulin resistant often have ‘reactive hypoglycemia' -- their blood sugar dips too low a few hours after eating sweets. Could this have been the reason she felt crummy in the morning? One evening she again had ice cream and the following morning she was almost too weak to get out of bed.
We went back to our FP. He ordered a couple of tests that would show if Sharyn had the type of damage to her kidneys that could be caused by diabetes and also looked for evidence that she had a high average level of blood sugar.
Sharyn's chest pain had continued to become more like typical anginal pain -- a feeling of pressure. He prescribed a drug that relieves angina caused by clogged arteries and which (I knew from reading) sometimes helps CSX victims and at last we got a break: the drug worked. Not only did it stop the chest pain but it made her shortness of breath much better. It wasn't the whole answer because the drug gave her a nasty headache and only treated the problem of restricted blood flow, not the insulin resistance. We had to deal with that too; if it was causing her MVA then she could get even worse flow restrictions. But the drug made her symptoms better by actually increasing blood flow so she could safely get more exercise which would help her lose weight. And mild exercise is also extremely good for the heart.
Equally important, since Sharyn's coronary arteries were clear, only MVA could cause both chest pain and shortness of breath which were both relieved by this drug: unofficially at least we had a diagnosis. Our FP still wasn't buying it ("Too empirical for me" he said) but we felt we were on the right track. The tests showed no kidney damage and an average blood sugar at the high edge of normal: both would fit insulin resistance. At the FP's direction we started taking and writing down Sharyn's blood sugar level once in a while so we could tell if she started to slip into diabetes.
The secretary for the fifth cardiologist, Dr. J., called and rescheduled Sharyn's appointment back two weeks; her boss was going on vacation. It was clear she was surprised when I yelled at her.
I continued research on the web and found a report of a case of a man who had shortness of breath as the first symptom and whose basic problem was insulin resistance. At last we knew of another case like Sharyn's. And I found a book by a well-known insulin resistance researcher on dietary management of the disease. ‘Miracle diets' are dangerous, especially if you're sick, but this doctor had thirty years of professional research behind him and many papers published in medical journals. This was advice we could trust and we began changing Sharyn's diet to reduce carbohydrates and partly replace the calories with ‘good' fats.
A hobby friend turned out to be an ex-cardiac nurse with a close friend who is a cardiologist. She asked her friend where in this country the best work on CSX is being done and the answer was ‘NIH'. Looking up the National Institutes of Health on the web I had no problem finding mention of microvascular angina; there was even a cardiac diagnostic study which mentioned it. We had a struggle with the gatekeeper person, who wanted to keep Sharyn out because she didn't have a diagnosis ("We do research, not diagnosis"), then wanted to keep her out because her diagnosis (still officially cardiomyopathy) didn't fit the studies.
We finally passed but when we talked to the screening nurse working on the study we found that basically NIH is studying only the cardiac side of the problem, not its metabolic causes. And (as nearly as we can tell) they treat all cases as ‘benign', mainly using medication to control the pain. So much for the National Institutes of Health.
We saw cardiologist number five. Obviously very bright Dr. J. seemed also to be very shy and said almost nothing during our visit. He prescribed an increase in one of Sharyn's heart drugs and added a second one. We intended to continue seeing him but appointments were repeatedly rescheduled by his office and with the distance and complicated arrangements for an overnight trip, we eventually gave it up.
I continued to get questionnaires from the university asking very detailed questions about our satisfaction with Sharyn's care. Was parking convenient? Was she seen on time for appointments? After two or three serious medical errors and the university doctors' insistence on a diagnosis which did not explain the facts, those things were completely unimportant to us. When they kept sending questionnaires, I wrote a letter evaluating our experience in detail: smooth procedures, caring support staff, but lousy medicine. About the quality of the medicine I said:
"Bluntly, those guys in cardiology are lawsuit bait. Not only could they not figure out the right diagnosis but they insisted on a wrong one (viral or perhaps idiopathic cardiomyopathy) and shouted down the correct one (microvascular angina) when it was handed to them. If it turns out that my wife was significantly harmed by their errors and/or the time we pissed away seeing them, you'll hear from her lawyer. It is a crummy way to do quality control and seek redress, but it is all we have.
"I hope you will follow up on this ... [but] follow up will be difficult because the cardiologists we talked to were so ignorant of chest pain with normal coronaries that they couldn't talk about the subject and so sure they were right that they wouldn't look at the literature. I thought the following was a joke when I first heard it:
Q. What's the difference between a cardiologist and God?
A. God knows he's not a cardiologist."
The questionnaires stopped coming. After a few weeks we got a letter saying that the university always appreciated clients taking the time to write about their experiences. Profiles in courage, wouldn't you say?
We pressed on. While it is unknown whether insulin resistance causes MVA or something else causes both, IR is itself unhealthy. We got a referral to an endocrinologist and made an appointment.
Dr. Z. took Sharyn's history and agreed that IR was a possibility. He did a blood test that showed that she did have more than the normal amount of insulin for the amount of sugar and on the strength of that and the history, started her on a drug which improves insulin sensitivity.
The Medline research continued and one more piece of the puzzle fell into place. We had been assuming that Sharyn's shortness of breath was an ‘anginal equivalent' but did not understand why (if so), some drugs affected her chest pain and shortness of breath differently. I found a paper reporting a study of two groups of patients, one with MVA and the other without. The MVA patients, on average, were less able to get rid of carbon dioxide (CO2) in the blood and since high CO2 is a key trigger for the feeling of shortness of breath they became breathless more quickly when they exercised. The study authors concluded that this was due to changes in blood flow patterns within the lungs causing relatively more blood to flow to areas with less ventilation, and vice-versa, thus making the lungs less efficient.
In other words, Sharyn's shortness of breath was in her lungs; her chest pain was in her heart. But in both cases the explanation was problems with the flow in small blood vessels.
Could the problem in Sharyn's lungs be specifically treated? We took the paper to a third pulmonologist, Dr. B., who did a few tests and prescribed trials of four drugs for one week each. We did the trials but nothing made much difference. At the next appointment he said that diseases of the blood vessels of the lung weren't really his department and we said goodbye.
Sharyn still needed a cardiologist; even if we couldn't find one who could improve her treatment we needed someone for routine followup. We got a recommendation to Dr. N. in the same town as the university. Dr. N., the sixth cardiologist to consider Sharyn's case took the best history of any doctor we had seen, seemed to understand fully, listened to our ideas about the diagnosis. He was surprised that we felt the university was unfamiliar with MVA and mentioned some related research being done there. He offered to read the research paper suggesting that Sharyn's prognosis might be unfavorable and I got his e-mail address. He indicated we could make another appointment at a time of our choosing if we wanted to see him again.
A couple of weeks later I sent him the reference for the paper along with a summary of the key points in our thinking. When we got not even a "Got your email" I wrote again, asking if he had gotten the material. Again, no answer. We guess that he has close ties to some of the doctors Sharyn saw at the university and really did not want to be involved. We won't look again for a doctor in that town.
That brings us to the end of 2001, just about two years from the start of trying to find Sharyn's heart problem. We have what we believe may be the complete diagnosis, we have what we believe is sound basic medical treatment -- an ACE inhibitor, a beta-blocker, and a nitrate for the heart and lung conditions plus Actos for the insulin resistance. The fairly rapid decline in Sharyn's ability to exercise has been slowed, perhaps even stopped. She does still get short of breath after climbing one flight of stairs and two flights done quickly is about what it takes to bring on chest pain. That's better than the month after Dr. P's exercise program but not as good as the month before it.
We don't, however, have a cardiologist and we almost certainly don't have an optimum treatment program. I continue researching her condition on Medline and there are a few new interesting papers per year. We're probably going to try another university medical center soon.
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