At the February 15, 2007 meeting of the HSS Myositis Support Group, Lawrence J. Kagen, MD spoke about a doctor’s perspective in treating those with myositis. This discussion offered the group an opportunity to hear from a doctor about what it is like to work with myositis patients. Further, group members had the benefit of learning from Dr. Kagen’s extensive knowledge regarding the needs of patients with the disease.
To begin, group members introduced themselves by giving a short description of their type of myositis, when they were diagnosed, and their current treatment and health. Upon hearing the individual experiences shared by our group members, Dr. Kagen chose to guide the discussion by focusing on these real-life examples.
Dr. Kagen pointed out that although only three types of myositis had been mentioned among group members (dermatomyositis, inclusion body myositis (IBM), and polymyositis), many different medications were being used to treat myositis symptoms; members shared that they had experience with Prednisone, IVIG, Methotrexate, Enbrel, and several other medications.
Since myositis is such a rare disease, the medical community does not have a standard approach to treating the illness. Thus, it can be a challenge for doctors to decide how best to address the symptoms. Also, because myositis affects individuals differently, no one type of medication will work for all patients. Though many doctors would greatly benefit from additional information about medications that treat myositis, the ability of scientists to study effective treatments for the illness is limited by the small number of patients that participate in rare disease studies.
Dr. Kagen discussed the importance of myositis studies as learning tools; yet, he noted that the results are often based on the feedback of only a handful of patients. Research studies conducted on those with other diseases, such as lupus or rheumatoid arthritis, may include hundreds or even thousands of patients. In order for the Food and Drug Administration (FDA) to approve a drug, a proven track record clearly illustrating patient benefit must be present. Therefore, identifying medications that work for rare diseases takes a greater amount of time than for more prevalent diseases.
When making a decision on how to treat a patient, Dr. Kagen mentioned the need to measure the degree of illness with the degree of risk. For example, it is still not exactly known how intravenous immunoglobulin (IVIG) really works but it has proven to be generally effective in treating patients with myositis. Choosing a treatment option with fewer side effects can sometimes be more beneficial to the patient rather than trying a more aggressive approach.
When treating patients, doctors can learn about how a particular disease affects people in unique ways. They may also learn about the progression of the disease from their patients. One group member discussed her illness, describing her current remission. She said that at the time her myositis began to improve, she was being treated for another medical condition. Dr. Kagen asked her to discuss her experience further in order to gain a better understanding of what led her into remission.
Real life experiences with patients can also be a way that a doctor learns how to make treatment decisions. Since myositis is a rare disease, most doctors have few, if any, myositis patients in their practice and have less experience to draw upon when making decisions regarding treatment. Some patients do their own research on myositis and bring it to the attention of their doctor. An informed patient can be helpful, allowing them to prepare for the onset of potential symptoms while considering a variety of options for treating those symptoms.
When asked how Dr. Kagen became interested in myositis, he said that initially he trained in rheumatology and had patients with the disease. He wondered why they had it and if there was a way to help them improve. He said that it was just an area of rheumatology that he gravitated toward; he felt determined to learn more about such a rare disease. He says he still is not sure why his treatments have been successful, but that many people have been helped.
Doctors often look at the creatine phosphokinase (CPK) enzyme level of a patient because it correlates well with disease course. Patients are often interested in their CPK level and can become emotionally invested in the result. When they hear that their CPK is elevated, many people feel terrible and wonder why the number would rise despite their taking their medications faithfully and following their doctor’s recommendations. Dr. Kagen’s experience with patients has shown that where CPK response follows a patient closely and correctly indicates how a person is feeling, the CPK level will generally change before the patient experiences the change. For instance, when he sees an elevated CPK level and the patient is doing well, he knows it is important to look at how the patient is doing three weeks from then.
Dr. Kagen talked about how doctors share some of the challenging experiences of having a chronic condition. For example, it can be frustrating when a doctor decides on a treatment they believe will be effective, but the patient experiences side effects. A physician may try several treatment variations before he/she can figure out whether it makes sense to continue the treatment or switch to a new one. Doctors and patients often share this frustration together, as they are both invested in finding the path that improves the patient’s health and well-being.
Dr. Kagen gave an example, describing how one of his patients had a debilitating headache after IVIG, but found that it benefited him greatly because it enabled him to step out of his wheelchair and walk. The patient chose to continue IVIG treatments, despite the headaches, and eventually, no longer experienced headaches after receiving IVIG. The doctor and patient have to negotiate on the best course of treatment, which may not always be easy.
Just as patients experience frustrations with insurance companies, doctors often have to consider insurance coverage when choosing a method of treatment for a patient’s medical condition. Doctors find it frustrating to treat two patients with the same disease differently because their insurance plans often do not cover the same interventions. One part of a doctor’s decision is limited by patient risk, while another is actually limited by insurance coverage.
When dealing with insurance companies, doctors often have to act as an advocate for their patients. Each insurance company has its own guidelines for coverage and Dr. Kagen mentioned that he has heard about cases of private companies not wanting to cover expenses that the government (Medicare/Medicaid) chooses not to cover in their plans. It can be confusing to decide where to put forth the effort to advocate.
Many factors impact the relationship between a doctor and a patient. Dr. Kagen stressed the importance of words when a patient describes how they are feeling. Weakness can easily be misinterpreted when a patient says they are feeling tired or fatigued; older patients, for instance, often attribute their weakness to aging and believe it is a natural occurrence.
Doctors may have difficulty asking questions in the right way to elicit a true experience of weakness. Dr. Kagen said examples often help, such as when a patient describes how they cannot take a container of milk out of the fridge or get out of a seat or climb a curb. When a doctor and patient experience communication difficulties, it can lead to frustration and misinterpretation on both parts. Many members of our group have shared that it took months - sometimes even years - to be properly diagnosed with myositis.
Patients often talk about how they are confused when doctors have different opinions. Doctors can also find it challenging when their patients receive conflicting advice from another doctor they are seeing. Dr. Kagen, for example, believes that individuals with myositis should avoid alcohol. He asserts that alcohol is not good because it can participate in muscle damage and raise a patient’s CPK level. It can be difficult to share this advice with a patient when they have heard from other doctors that alcohol does not impact myositis patients in a harmful way.
When asked about how one’s attitude can impact coping with myositis, Dr. Kagen said that it is important to look at how your disease affects your attitude and how your attitude affects your disease. A doctor may have a much easier time learning information about a patient when that information is openly shared. However, Dr. Kagen stressed that patients always presenting a true representation of their feelings is of the utmost importance because it is helpful in figuring out if the patient’s condition has improved or not.
Additionally, he recognized that finding ways to maintain a positive outlook can be beneficial. He claimed that a positive outlook allows people to carry on when they are faced with challenging circumstances. Dr. Kagen emphasized that doctors are only human and experience ups and downs alongside their patients. Ultimately, doctors want to know that the treatments they choose are helping their patients improve. He then noted that there are heroes in the world who remain optimistic regardless of difficulties. We could take comfort in their strength.
Dr. Kagen has spent many years working with patients with myositis. When asked about how the treatments for myositis have changed over the years, Dr. Kagen replied that there have been no extraordinary changes in the treatment methods; many patients continue to use anti-inflammatory medications.
That being the case, we have seen progress in understanding the illness. Further, our ability to treat many other diseases has improved, which raises hope that we will one day be able to identify the cause and cure for myositis. The outcomes for patients with myositis have also improved. Many years ago, the disease was described in most medical literature as terrible and basically life ending. Now, patients with myositis are being diagnosed earlier and more accurately; many cases have been found to be much milder than previously anticipated..
Patients often ask for statistics regarding how common myositis is in order to know they are not alone. Today, a much more precise figure on the number of people living with myositis is available. As a myositis specialist Dr. Kagen tends to see a greater number of myositis patients than many other doctors, but generally, he said, population surveys show that it may be close to five new patients per million in the population per year.
A group member asked how gender may play a role in the diagnosis of myositis. Dr. Kagen mentioned that other diseases clearly affect one gender more often than the other. However, he asserted that the impact of gender on myositis is not quite as pronounced Some literature suggests that women are diagnosed with myositis more often, but this may be because of the larger number of women that have overlapping syndromes, such as lupus, scleroderma, or rheumatoid arthritis, along with myositis.
We are continuing to learn more about who myositis affects and why it affects them; when studying such a rare disease, however, answers seem to come slowly over time.
Doctors and patients are teammates in determining the treatment plan that is best for the patient’s emotional, physical, financial, and practical needs. For rare diseases like myositis, doctors have additional concerns to address outside of the symptoms expressed by patients; they may have limited amount of knowledge about the disease, limited experience treating patients with it, and may face constraints in treatments covered by insurance plans.
As a final point, Dr. Kagen noted that both doctors and patients share a common goal – for the patient to feel better – and it is something that only be accomplished by the doctor and patient working together.
Learn more about the Myositis Support Group, a free support and education group held monthly at HSS.
Summary by Angela Hunter, LMSW, Myositis Support Group Coordinator