Going to Irma’s house isn’t like going to anyone else’s house. She tells me not to wear any deodorant or use any other chemicals.
‘I NEED TO HAVE SOME TEETH EXTRACTED.” IRMA ALWAYS SPEAKS AS THOUGH EACH WORD MIGHT CONSUME THE LAST OF HER LIMITED ENERGY. “BUT I’M ALSO HAVING A PROBLEM WITH MY HEART.”
Irma has never come to my office. For the past 20 years, she has seldom left her house. I acquired her as a patient 8 years ago. She believes she has multiple chemical sensitivity (MCS) syndrome. Most health-care providers approach MCS patients as if the problem is 95 percent psychiatric. Sixty years of efforts to find a biological cause or even a plausible theory to explain the variable and bizarre symptoms that patients with MCS experience have not yielded any helpful clues, and my experience with Irma has not done anything to change my impression that MCS is primarily a psychiatric disorder.
Irma was now explaining to me over the phone that she had several dental abscesses, for which a dentist had prescribed an antibiotic. She was taking the antibiotic in the manner she takes most medicines: she touched a pill to her tongue as often as the label instructed her to swallow one. She believes that this works for her because she is so sensitive to chemicals.
The notion that an antibiotic would work that way is absurd. Antibiotics kill a bacterial species when they achieve the “mean inhibitory concentration” (MIC) in the blood for that particular bacterium. Touching a pill to the tongue does not deliver medicine to the stomach for distribution into the bloodstream. But Irma is so sensitive that, with some medications, she says, she can “take” them by being present in the same room as they are.
Over the years, Irma has constructed a belief system and way of life around the concept that her immune system is unable to handle natural or man-made threats to her body. Crazy people need doctors, too, and I learned the first time I spoke to her that the only way for me to help her was to work within her belief system and not challenge her.
“My heart seems to have two distinct rhythms,” she explained in her soft, slow, breathy tones. “Most of the time, it has a slow, steady beat. At times, I get palpitations, and my heart is beating erratically and much faster, about a hundred times a minute.” She had just given a nice description of paroxysmal atrial fibrillation, a condition where the heart periodically abandons the regular pacemaker-driven sinus rhythm because of intermittent electrical chaos in the left atrium, the heart chamber that houses the pacemaker. This chaos causes the main pump, the left ventricle, to beat rapidly and irregularly. A young heart can beat as quickly as 180 to 200 beats per minute in atrial fibrillation, but Irma’s 67-year-old vital organ could only manage about 100, which was fortunate because one can be quite comfortable with a pulse of 100. Her description prompted my mind to flashback to an early lecture in medical school, during which a venerable professor taught us that if we would just sit back and listen to our patients, “most of the time they will tell you their diagnosis.”
She had two concerns about her heart condition: would she be able to tolerate local anaesthesia for her dental work; and would she be able to move to rural Oregon, as she was planning to do in about three months to get away from chemicals, high property values, and a landlord who was selling her rented house. I arranged a home visit.
I have to fit home visits and long conversations with Irma into a typical 2001 primary-care physician schedule, which is packed with patients and paperwork, but she had more to discuss with me, so I took a few minutes here and there, when I could, to call her and piece together the other elements of her current condition. She was peeing brown urine intermittently and had been doing so for a few months. This had happened, infrequently, on a few occasions, and she had always attributed it to chemicals or medications assaulting her kidneys from time to time. She also had periodic nausea, loose bowel movements, and tightness all across her low abdomen. One of her attendants brought a jar of Irma’s brown urine to us one day, and we found it contained a moderate amount of old blood and a small amount of protein. Something was amiss with Irma’s kidneys.
So I was now facing a new situation: Irma was having some real medical problems, not just something she had invented. A few days later, I went to her house.
Going to Irma’s house isn’t like going to anyone else’s house. She tells me not to wear any deodorant or use any other chemicals, and she has told me many times that she doesn’t get the “buzz” when I enter her house that she gets from so many other people. I haven’t told her that I use Mennen Speed Stick even on the days I see her because I wouldn’t do well with the other 20 patients I have to see the same day if I did not From inside her front door, she hands me plastic bags, into which I place my pager, wallet, and keys. I leave my shoes at the door, and then I’m ready to go through the screen door into her living room.
Irma’s pale face greeted me with the usual smile under her dark brown bangs and white cloth that she wears over her hair. Her wardrobe consists of beige or white (no dyes) wraps made of sheetlike material, and white socks covering her thin legs. She motioned for me to come forward along the narrow path cleared for walking on her beige carpet between plastic containers, covered tables, and a large refrigerator to the corner of the room where she appears to spend most of her time. I recalled how in my younger and more naive days (approximately 1993), I had come there at her request to do a Pap smear on the living room floor because she had not had one in years. In doing so, I exposed her to some latex and plastic to which she had claimed to be deathly allergic and, of course, nothing had happened. She had praised me for my gentleness and for taking precautions on her behalf.
Moments after my entrance, one of Irma’s “servants” arrived. A short, balding man who appeared to be in his 50s, he was Irma’s designated shopper. She excused herself to go to the door to give him a list, and I eavesdropped on their conversation. “I cannot have any filberts that are large because I will react to them. The small ones are fine, but they cannot have any green on them.” Accepting her command, he repeated what she said, went over some other items, and left. She returned her attention to me.
Again I listened to her story about her heart. For some reason, she is not allergic to stethoscopes. My theory is that she will never need to be allergic to anything once it becomes critically important. When I first placed the stethoscope to her chest, she had a normal heartbeat of 66 beats per minute, but after about 15 seconds she said, “There!” as an irregular and more rapid rhythm took over. She had paroxysmal atrial fibrillation. I did not hear any extra whoosh sounds, or murmurs, that would have led me to suspect that she had a leak in her mitral valve, which can cause the left atrium to enlarge and lead to atrial fibrillation. A defective valve would also predispose her to endocarditis, an infection of the heart valve, during dental procedures or other procedures that cause bacteria to circulate in the bloodstream. People with diseased valves have to take antibiotics before invasive dental procedures to prevent endocarditis.
Because her heart rate was reasonable, even while in atrial fibrillation, she did not need any medicine to control her rate. However, it would help to give her a medication to try to keep her in sinus rhythm because atrial fibrillation can cause blood clots to form in the left atrium, which can then circulate and plug up arteries to important organs, such as the brain, kidneys, arms, and legs. If such medicines do not succeed in preventing recurrent atrial fibrillation, we then prescribe a blood thinner to keep the clots from forming.
Irma does not use medicines in doses that work, so we could not do any of these things. “What about aspirin?” she asked. She spends much of her time reading medical literature, so she knows a great deal about what different medicines do. Aspirin is not as effective as warfarin (an anticoagulant) in this situation, but it might help if she would swallow a pill. Instead she proposed to touch it to her tongue. I knew she would do this regardless of my advice, so I acquiesced, with the clear understanding that this was not my recommendation. I did tell her that it was safe for her to have her dental procedure done but that I advised that she have an ultrasound of her heart to see if we could determine why the rhythm disturbance was occurring. She also needed an ultrasound of her kidneys.
Getting these ultrasounds would involve patience and a sense of humor. It would require many phone calls to the technicians for both studies to ensure an environment as chemical-free as possible and even that wouldn’t prevent her body from having an overwhelming reaction to the unfamiliar environment. “I’ll be out of commission for days. Can we possibly do them at the same time?” Different hospital departments do these tests, so this was not possible. Accustomed to obstacles, she sighed in acceptance of the complicated circumstances.
Her shopper returned. “I couldn’t find any small filberts without any green.” I couldn’t help laughing to myself; fortunately, I was out of their view. She looked over the rest of the items and after expressing her satisfaction cheerily dismissed him. I told her my office would call to make the appointments for the tests while she made the environmental arrangements. I then collected my possessions from the plastic bags and left.
About five days later, we were on the phone again. “My dentist gave me some local anaesthetics to try to see how I would react. I really liked what the lidocaine did for my heart.” I knew Irma must have read that lidocaine is used intravenously in intensive care medicine to stabilize malignant heart rhythms, so that now that she was in the same room as lidocaine, it was having powerful effects on her heart. This belief would help us get her the dental procedure she needed, so I didn’t fight it. I told her that lidocaine sounded like the right anaesthetic for her. A few days later, she had the teeth pulled, and she reported later that she could not do anything for several days after that.
A couple of weeks later, she had her kidney ultrasound. I read the report, which confirmed that she had normal appearing kidney tissue without any suspicious tumors, and there was no evidence of damage or obstruction. I relayed the good news to her and faxed her a copy of the report, something she always requests.
The next day, I had a message from her on my desk inquiring about her “missing kidney.” As I picked up the phone to call her and ask what in the world she was talking about, I read the report again and realized to my horror that I had skipped an entire paragraph when I read it the first time. There it was: “...the left kidney has been replaced by a cyst...” I hung up the phone to self-administer a mental whipping for my oversight and to think. People do fine with a solitary kidney. Kidneys often develop cysts, and some people have polycystic kidney disease, in which cysts replace portions of the kidney, making the kidney larger and less proficient, but 1 had never heard of a cyst replacing an entire kidney. I called John, a local nephrologist, and he had never heard of that either. It seemed much more likely that she had always had just one kidney. I explained the other oddities of her case, and he agreed to look at her ultrasound.
I called Irma, apologized, and outlined the mystery, emphasizing that the remaining kidney appeared to be fine. We would need her to collect her urine in a plastic container for 24 hours and get a blood test; together, they would allow us to calculate her creatinine clearance, the best measurement of kidney function. Over the next two weeks, she made the necessary arrangements to get a “chemical free” nurse for the blood draw and urine collection, the products of which then went to the lab for analysis. In the meantime, John called me with good news: Irma was born with one kidney, and it looked normal.
Two days later, the lab faxed us separate results for the urine and blood tests and reported the creatinine clearance as “unavailable” because they said they didn’t have the blood test. After two days and four phone calls to three people at the lab, I found someone capable of finding and combining the two results to give us the final answer. Her creatinine clearance was 41, about half of what we would expect for a woman her age, but well above 10, which is the neighborhood in which dialysis would become a consideration. She had negligible amounts of protein and blood in the urine and therefore did not have any signs of active kidney disease. Her urine contained much more oxalate than normal, and this explained her pain and intermittent brown urine: she was passing calcium oxalate stones. If you give calcium supplements to people who form calcium oxalate kidney stones, the calcium binds oxalate in the digestive tract before it enters the blood through the intestines and therefore keeps oxalate from entering the kidney and thereby prevents stones. John prescribed calcium and cleared her to move to Oregon.
I could not give that permission until we had finished evaluating her heart. When her ultrasound report came, I read every word and, once again, we had a surprise: a small fluid collection around the heart, what we call a pericardial effusion. Irma did not show any new signs of tuberculosis, lupus, or thyroid hormone deficiency, conditions for which we had tested her in the past and all of which can cause a pericardial effusion. We knew she did not have kidney failure, another possible cause. I called Dick, a local cardiologist, who looked at the ultrasound and called me back.
“It’s a small effusion, Jim, but it’s definitely real. It’s not large enough to affect heart function.” It was therefore improbable that it had anything to do with her atrial fibrillation. I explained to him that I did not have an explanation for her effusion. “Sometimes we just find these,” he reassured me. “I would just do another echocardiogram in three months.” Easy for him to say, but when I relayed the news to Irma, to my surprise, she seemed satisfied and willing. Her landlord had given her a reprieve, and she had decided she might not need to move to Oregon at all, or at least not for several years, which would give us the time we needed to resolve her current problems. Perhaps surviving these tests will give her enough reassurance to take liberties with her self-imposed exile and misery, something that would help her more than anything any of us in the medical profession has done for her to this point.