“A rheumatologist is that doctor who lives most easily with uncertainty.” – Dr. Lockshin
If you believe the popular press and social media, most things in medicine are both solvable and known. This belief may be true for acute illnesses (those that begin and end quickly), but for chronic illnesses (those that begin slowly and may not end), and especially for lupus, some degree of uncertainty is common. The best doctors include uncertainty in their planning and make decisions with it in mind. They discuss uncertainty and negotiate priorities with their patients. They make medical care a partnership. This article discusses how uncertainty occurs and how it should be managed.
Although people think that the diagnoses of lupus, rheumatoid arthritis (RA), scleroderma, or other autoimmune diseases are plainly defined, these diseases are not so clearly separated. Here are the three most common reasons for uncertainty in diagnosing lupus:
With lupus, a diagnosis is not always “yes” or “no.” Lupus and lupus-like illnesses lie on a spectrum from antibody only to UCTD to typical lupus to overlap disease. The name for the diagnosis is less important than whether it needs treatment, and, if so, how and when.
In an acute illness, like appendicitis or pneumonia, symptoms start quickly, treatment results are quickly known, and, when it is over, no further treatment is required. Results of treatment trials for acute illnesses are definitive and certain. In chronic illnesses, like lupus and RA, the following may occur:
Here is an example: A patient I met in the late 1970s had unequivocal, very severe lupus. She went into full remission, had several successful pregnancies, and stayed well. In the early 2000s she developed a clear diagnosis of RA — typical symptoms and laboratory findings — and no signs of lupus at all. She went into remission again. In 2016 laboratory signs of lupus returned. The lesson is that the body does not care which names doctors give to diagnoses; the illness will do what it will do. Doctors and patients must be open to the idea that the diagnosis named a decade ago may have a different name today.
When doctors think of chronic illness, they have to consider four categories of time.
Doctors and patients must plan for all time frames at every doctor visit. Patients must tell the doctor their priorities. A medicine that works very quickly may have disastrous side effects a decade from now. Do I want a better chance for cure today, perhaps at the cost of my fertility, or should I take my chances with a less powerful drug, but preserve my ability to have a child? Patients must ask questions and must say “no” if the doctor’s answer does not meet their own priorities. The patient makes the final decision.
When will I have a flare-up? Lupus is often unpredictable. While doctors do know something about what causes flare-ups — heavy sun exposure or allergic reactions to drugs — they cannot predict most flare-ups. Perhaps the best way for patients doing well to cope with the idea of flare-up is to live their lives normally but always have a withdrawal plan if a flare-up does occur.
Doctors use somewhat inflexible “criteria” to diagnose lupus. Those criteria describe typical lupus, not lupus diagnosed by rare symptoms. Patients can still have lupus, even if they do not meet criteria. Whether or not symptoms fit criteria the doctor can still act. The patient’s question to their doctor should not be, “Do I have lupus?” but “Do you have a treatment plan?”
There are several kinds of criteria:
Atypical forms of illness are not included in research studies. Insurance billing codes have no way of listing atypical patients. The above criteria work for “classical” diseases, but cannot work for atypical, overlapping, or forme fruste diseases, complicating a doctor’s ability to follow treatment guidelines, and impacting insurance approvals. Codes, indexes, criteria, and guidelines force doctors to make decisions appropriate for atypical patients. This is a problem in medicine that doctors are trying to correct.
When doctors and patients think about their goals, they use different timelines. Patients want to feel normal right now. Doctors think ahead 20, 30, even 40 years: Will the prescription I write today have side-effects that will make my patient’s life worse later on? These are topics that should be discussed between the patient and doctor.
Although decisions for lupus involve uncertainty, uncertainty is not a cause for frustration or despair. Rather, it is a call for open-mindedness — on the parts of both the doctor and the patient — to the possibilities that may emerge. Uncertainty is an opportunity for patients to partner with their doctors in reaching for their goals.