Maybe you are looking for answers as to why your feet feel as if they are on fire.

These flares may be intermittent or constant, but one thing is for sure- the intense burning pain is really messing with your quality of life. Even a short shower can set you off. Exercise now feels like an impossibility. A walk can leave you is misery, lying with your feet up a wall in search of relief. Wearing warm socks may be a thing of the past. Your feet find themselves peeking out of bedcovers even in the midst of a Minnesota winter. Cocktails are no more, as alcohol can send you on a downward spiral. Nighttime is the worst. Even the subtle increased body temperature caused by entering a warm room can begin a tailspin. Your life is now calculated in the distance you must travel on burning red feet that seem to be betraying you. And the pain—the pain—it’s hard to describe. Brutal. Torturous. Horrific.  Isolating… even those that love you best just don’t get it, try as they might.

The way Dr. Edith Eger describes pain brought tears of recognition to my eyes-

“For more than a year, I have not had the luxury to think about what hurts or doesn’t hurt. I am a circuitry of pain, a signal that feeds back on itself” [1].

Maybe, like me, you saw a doctor who patted you on the head and assured you whatever this was—probably a pulled muscle—it would go away. He didn’t listen when you told him how you could no longer stand to do your job or cook dinner. He didn’t take your claims of pain and redness seriously. Maybe you saw a second or third doctor who was well meaning but incorrectly diagnosed you with Complex Regional Pain Syndrome (CRPS) formerly called Reflex Sympathetic Dystrophy (RSD) [1]. She did this due to the discoloration of your skin. This misdiagnosis created fear and sent you tumbling down the wrong rabbit hole. Maybe your symptoms were initially written off as somatization, meaning that your care provider chalks your problem up as being “all in your head” instead of acting with the humility of one who knows they do not know.

Maybe you now have an official diagnosis. Maybe you don’t.

The dots are connected for each of us in different ways.

I was lucky. The fifth doctor I saw listened to me describe my symptoms with kind eyes. He asked me to lie on my back with my feet in the air. The discoloration of my feet immediately improved. He said he thought I might have erythromelalgia, which is nicknamed “EM”. He has seen it once before. It was the immediate improvement in discoloration with elevating flaring feet that tipped him off. The good news was my malady now had a name. The bad news was that he informed me he did not know how to treat it. Frustratingly, as Eger says, “A diagnosis is not a cure” [1].

Erythromelalgia is a rare disease—so much so it is called an orphan disease—leading to difficulty in diagnosis. A 2008 study by the Mayo Clinic estimated 1.3 people per 100,000 are affected by EM [2]. That means you are much more likely to be struck by lightning in your lifetime than be diagnosed with EM. This is not a common chronic pain condition.

Erythromelalgia’s name comes from three Greek words combined together—“erythro” means redness, “melos” translates to limb, and “algia” meaning pain [2]. The National Organization for Rare Disorders (NORD) describes EM as a chronic painful condition characterized by severe burning pain that “primarily affects the feet and less commonly the hands” [2]. It is thought to result from vascular abnormalities with constriction and dilation of certain blood vessels—small arteries called arterioles—leading to abnormalities of blood flow to the extremities [2]. Symptoms may include itchiness of affected body parts as well [2] [3]. A hallmark of EM is heat intolerance or worsening of symptoms usually triggered by exposure to heat. Affected individuals may also be triggered by exertion [2]. Another cardinal characteristic of EM is relief from symptoms with cooling. Excessive cooling, such as constantly submerging feet in cold water, can lead to a whole new host of problems like ulcerations and even gangrene [2].

 I was diagnosed with Raynaud’s disease in my early 20’s when an earlobe went white as a sheet and wouldn’t perk back up. The same ghost-like appearance happened periodically with my fingers, especially in winter. My cure was increased skin temperature achieved by placing my hands under running warm water. It was easy enough. Autoimmune disease runs in my family. My mom had Raynaud’s phenomenon and assured me that if all else failed a glass of wine would always do the trick. If you had to have an affliction I thought, this one seemed preferable. In my mid 30’s, I would travel to New Zealand, and after a chilly rainstorm, would develop chilblains. Chilblains were by far worse than Raynaud’s. Supremely painful but they would pass. In hindsight, this was vascular dysfunction, a prelude of what was to come.   

The doctor that identified my EM prescribed some supplements and dietary changes that surprisingly and dramatically diminished the inflammation. The flare was no longer unrelenting. Living was more bearable most days, but I was desirous of a life much larger than simply surviving. As a medical professional, I did what we do when faced with our own failing bodies—I threw myself into research. I discovered that the Mayo Clinic housed the only erythromelalgia specialty clinic in the United States [3]. Hope swelled! I had my doctors send over referrals and records. I called to check on the status and was told of a wait list extending forward for at least 2 years. I had already lost so much life. I was suffering.

I could not wait two years.

I found ways forward. I had no other choice. I used socks with cool gel pack inserts to manage flares. I was only 36 years old, yet I sat on a shower chair to bathe myself, using a second chair to prop up my legs, and rinsed with lukewarm water. The muscles that once belonged to a marathon runner withered under my new sedentary lifestyle. I used a knee scooter when I could not stand. I never wore socks, not even in the dead of winter. I avoided the heat of summer—forgoing long walks I used to enjoy with friends, now running errands early in the day, and declining invitations to board a neighbor’s prized sailboat. An avid painter, I lost yet another thing when EM invaded my hands.

My world was growing smaller. I was losing the things I loved.

The capable caregiver, the hospice nurse, could no longer stand. I spent so much of my life caring for others. Now I needed care. Accepting help was equivalent to swallowing a bitter pill. When runners strode past me, I felt anger. My husband wanted a sympathetic response when he reported that it is too cold to go for a jog, but I felt only rage. Those that live in the land of the able-bodied have no idea the gift they so often take for granted.

It would take me many years to understand that we can never fully appreciate something while we are holding it in our hands.

I learned that there were two types of erythromelalgia—primary EM and secondary EM. Primary erythromelalgia, also called Weir-Mitchell disease, is deemed familial meaning that it is an inherited erythromelalgia [4]. There are two subtypes of inherited EM—the more common “early-onset inherited erythromelalgia” which appears in the first decade of life and the less common “late-onset inherited erythromelalgia” occurring in the second decade of life or later [5]. SCN9A mutations define primary erythromelalgia [1]. I asked for genetic testing specific to SCN9A mutations. If this problem was caused by a faulty gene, I didn’t want to pass it along to a future child. Those that test positive for primary erythromelalgia may submit results to the Genetic Testing Registry (GTR) in hopes of advancing EM research [6]. My SCN9A test was negative, meaning I did not inherit erythromelalgia. Sporadic or secondary EM makes up the majority of cases of EM, and the cause is unknown. What is presently understood is that secondary EM can present itself before a person develops myeloproliferative disease—more specifically polycythemia vera or essential thrombocytosis—so blood cell counts should be monitored [5]. This knowledge added a whole new layer of anxiety into my already worried world. Secondary erythromelalgia may also occur in association with underlying diseases such as autoimmune diseases like systemic lupus erythematosus or multiple sclerosis or with rheumatological disease such as rheumatoid arthritis. Less commonly the development of secondary EM is related to the patient’s use of some drugs like nifedipine or bromocriptine [6]. What we know about EM is overshadowed by what we do not know.

Regardless of whether you have primary or secondary erythromelalgia, mutations are found in sodium channels in the affected area [6]. NaV1.7 sodium channels are found in nerves that transmit electrical signals to the brain and spinal cord [6]. When mutations cause these channels to open more easily or stay open longer, nerve cells transmit pain signals [7]. For you, this equals suffering.

Years ago while a hospice nurse, I stood in a dying man’s living room while an old country doctor educated me on pain caused by nerve damage. He said something that stuck with me despite the years – “It is the most brutal type of pain, because it doesn’t even respond to narcotics”.

It sounded like a never-ending nightmare. Now nerve pain was part of my reality.     

I finally did end up at the Mayo Clinic, and was incredibly lucky to meet with Dr. Mark Davis, a world renowned erythromelalgia expert who has published a plethora of papers on EM. I was soon scheduled to strip down and crawl into what felt like a large oven while sprinkling myself with purple powder. I kid you not. This thermoregulatory sweat testing would measure the anhidrosis that accompanies EM, a fancy word for the impaired ability to sweat [6] [7]. Before getting started, I asked if I could place my hand in the “oven” and within seconds emphatically declined the test knowing it would send me into a flare that would last days or weeks… if I could even withstand the heat for the required 15-20 minutes for an accurate test result. The person performing the test was understanding, saying it was not uncommon for people with EM to refuse the test.

I also underwent nerve testing. There is increasing evidence that peripheral neuropathy—damage to nerves—are strongly associated with erythromelalgia [2]. What is not clear is what comes first—the chicken or the egg—the nerve damage or the EM. After lots of expensive tests to confirm a diagnosis, Dr. Davis prescribed a cream to apply during flares which provided some minimal relief [8]. In addition, I was given prescription grade lidocaine patches that could be applied with pain or prior to exercise [9]. They aim to use topical therapies before moving to oral medications in hopes of diminishing the chance of side effects. It’s important to note that for affected individuals, especially those who have or will develop a myeloproliferative disorder, a simple aspirin may improve or relieve symptoms. This was not the case for me.

In all honestly, the holy grail of Mayo prescriptions seemed to be less effective than the supplements and dietary changes my functional medicine doctor had recommended years earlier.

Dr. Davis encouraged me to live as normal a life as possible and to avoid reading scary stuff about EM on the internet. Take heed: Avoiding frightful internet spirals is helpful. Dr. Davis provided me with a list of other prescription interventions to try; I was to pass this along to my primary care provider who could prescribe them as I tried them one by one to see if they might relieve symptoms.

I am grateful for those who have dedicated their valuable time to researching rare disorders. I acknowledge that we are unique as human beings—our results to treatment can be starkly different even when we possess the same disease—and yet…

I had been to mecca and left disappointed.

The medications were mildly helpful, but the treatment options didn’t offer me the entry back to a feeling of vitality I was hoping for. I could no longer deny the painful truth:

No one was coming to save me. It was time to save myself.

Spontaneous remissions of erythromelalgia have been known to occur [5]. As a nurse who worked for a decade with the dying, the idea of spontaneous remissions seems a misnomer to me. Disease must disappear for a reason. In our arrogance, we call the remission “spontaneous” or say the patient was perhaps misdiagnosed instead of sitting with the discomfort of our not knowing.

I couldn’t get rid of the nagging feeling that a house I had lived in was contributing to my illness. About a year into living in that home, dark mold started presenting itself on many of the walls. I spoke to my sweet 94-year-old landlord about my concerns. “Oh, yes dear”, she said unconcerned, “The walls were covered in mold before you moved in. I had them Clorox’ed and then painted over it.” I began to connect the excruciating headaches and repeated “sinus infections” to the environment I was living in. I moved out. I should say that most people are not “lucky” enough to see mold in their environment—this is extreme. Clues that mold is present are things like water spots on the ceiling, condensation collecting on windows, a history of a leaky pipe or roof, past flooding, or a musty odor produced by volatile organic compounds (VOCs) the mold releases.

Over the past few years, I had mentioned this mold concern to many doctors who had dismissed my gut feeling. More empowered to trust myself, I turned to my inner voice and began researching exposure to toxic mold. Mycotoxin exposure could affect the nervous system [10]. Dr. Ritchie Shoemaker had produced a large amount of underappreciated research on the subject. He had named the resulting multisystem illness “chronic inflammatory response syndrome” (CIRS) and had a protocol for treatment and was getting good results. I sought out treatment and experienced deep healing and eventually remission. From a functional medicine perspective, I had found my root or underlying cause!

I had inhaled toxins that my immune system could not tag or remove so instead they circulated in my blood for five years creating massive inflammation.

I believe these toxins pulled the proverbial trigger on my erythromelalgia. I always had the propensity to develop EM much like all of us are more inclined to some diseases than others. The gun was always loaded for erythromelalgia, but it was the mold and resulting CIRS that pulled the trigger.

Now that I knew the root cause, I wondered how treatment of CIRS would influence my EM.

The good news is the appropriate answer is “How did it not?” Getting at the root cause which greatly diminished flares combined with later heat desensitization was my ticket out of EM hell. I am in complete remission. The girl that could no longer wear socks pads around the house in wool-lined slippers. My feet are routinely covered with bedsheets. I enjoy a hot shower almost as much as a long soak in the bath. I waved goodbye to the shower chair days and have never looked back. I occasionally enjoy a glass of wine or a cocktail. I stand to cook dinner. I can again enjoy walks with friends and bike rides in the desert. Flaring of hands is a distant memory. I’ve even enjoyed a 180-degree sauna with no fear of hell to pay. I am able to be the wife, therapist, nurse practitioner, and friend that I desire to be when my extremities—and my life—are not engulfed by pain. This is not to say that I am what I used to be before the health challenges, I am no doubt changed: Life is brighter, deeper, more expansive.

Only one who has once walked with pain can truly appreciate walking without it.

I share my story to offer you hope. I share my story to tell you that a complete remission is possible, and there is plenty you can do to tip the scales in your favor.

The Erythromelalgia Association makes a wonderful guide to erythromelalgia that you can carry to your doctor to educate them on your disease process if they are not familiar with EM—most will not be, and coming with information in hand can be helpful [11]. In addition to treatment by a primary care provider, I have designed an erythromelalgia home study course to share my hard-won wisdom with you in an affordable way. I pass along tips, tricks, education, and materials that have been helpful to me as I’ve walked the road toward healing. Due to my training as a therapist, I am also comfortable addressing the very real psychological effects of living with chronic pain conditions and the accompanying grief associated with all of the untold losses. EM can also be hell for those who love us. They don’t know how to support us well. And how could they? To meet this need, I’ve created an easy education course to give loved ones access to tools that empower them to offer support in a way that actually feels supportive. With this training, it is possible for loved ones to put less effort into supporting us yet be way more effective when they do offer support! It is my pleasure to walk this road to healing with you, cooling socks in tow—until they are no longer needed—and you toss them in the wind.