Secondary Erythromelalgia diagnosis journey

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October 5, 2016 – “Secondary” Erythromelalgia or EM makes my hands and fingers so red that they BURN like Frostbite. EM is also referred to as “The Man on Fire Syndrome” because it literally feels as if your hands and fingers are on FIRE. It is extremely RARE and INCURABLE – at least by traditional means. Lovely.

October 7, 2016 – I have not been able to post much lately because from June, 2016 through September, 2016 I was busy trying to be diagnosed and treated for what started out as “pins and needles” in my fingers but quickly turned into increasingly SEVERE Frostbite-type burning pain in my hands, fingers, feet and toes.  I started with a Pain Management Doctor thinking it was the beginning of an orthopedic problem (likely involving my Neck) but also simultaneously consulting with my inimitable longtime gastroenterologist Dr. Mark L. Chapman because he had “successfully” treated my extremely complex case of Crohn’s Disease  ever since I was finally correctly diagnosed in 1984.
      Just like the “Kardashians” are to blame for people becoming seemingly credible career celebrities with no discernible talents other than their licentious proclivities, Crohn’s Disease has been the “sex tape” which launched and fueled my career as a chronic patient and patient advocate. My very complex case of Crohn’s Disease and its many peripheral manifestations often presented mystifying and even life-threatening issues which confounded doctors all over the world except one (1) located on the Upper East Side of Manhattan. Dr. Mark Chapman’s proven track record in solving ALL of these byzantine medical problems since 1984 made him my obvious choice to quarterback what I suspected was going to be a protracted “diagnosis journey” with these extreme symptoms.

 

This picture is from September, 2016 and shows how Erythromelalgia or EM affects my feet. The red-hot feelings dominate my toes, much like they do my fingertips, and they stab the soles of my feet and make my heels feel like I am walking on such badly blown-out metal-on-metal wheels that sparks fly every time my heels touch the ground.

This picture is from September, 20, 2016 and shows how Erythromelalgia or EM affects my feet. The red-hot feelings dominate my toes, much like they do my fingertips, and they stab the soles of my feet and make my heels feel like I am walking on such badly blown-out metal-on-metal wheels that sparks fly every time my heels touch the ground.  Exaggeration for effect.

      But the business of healthcare has changed and now doctors are limited to opining on only their myopic specialty as if my incurable, autoimmune disease and the potent steroids and biologics I had to take to thwart it are as inconsequential to the accuracy of my future medical diagnoses as the size of Kim Kardashian’s derriere is to her continued popularity. But Dr. Chapman has always been a “problem-solving” doctor and his genuine care for me has always “eclipsed”  (sorry, the more accurate choice of word is “trumped” but …) the short-sighted objectives imposed upon him by the understandably profit-driven, policy-making Suits. To that end, Dr. Chapman patiently listened to my symptom descriptions as he observed me with a Google-like mind of medical knowledge from all he had seen in his 60+ years of experience treating and solving the most severe and complex Inflammatory Bowel Diseases (IBD) cases.  I think for the first time since I was a scared kid diagnosed with an incurable disease I couldn’t even SPELL, he saw me in a uncontrollable state of pain exhibiting borderline fright.  The seriousness of my situation was palpable in the Exam Room and neither of us needed to articulate it especially in light of our mutual reference point of “seriousness” being established sometime during the 200+ hospitalizations and 20-25 surgeries we had been through together over the past 32 years.
      Consistent with his mandate as my gastroenterologist, Dr. Chapman explained that his participation could only be very limited because Crohn’s Disease did not appear to be on the menu.  I understood but for a moment after he spoke I had an unfamiliar feeling of hopelessness.  It sucked. He must have seen the color deflate from my face with a pace only Tom Brady could have matched so he spent a few minutes explaining to me what likely lied ahead given my symptoms. Perhaps most importantly, he reiterated what he had joked about at the onset of the exam that something very strange was going on inside my body. His re-emphasis was our “code” for me to continue trusting what my body was trying to tell me, as I always did, much to the dismay of most other doctors who never considered even for a nanosecond that I might know more about MY BODY than they.
     But the diagnostic journey Dr. Chapman described necessitated pursuing my fate with Neurologists and Rheumatologists which made me very uncomfortable as my past experience had proven neither medical specialty to be populated with “problem-solving” physicians like Dr. Chapman. But to their credit they proved to be very helpful during this diagnosis journey. But as is usually the case with extreme symptoms, I also encountered a few doctors who simply refused to believe the severity of my unrelenting and increasingly debilitating nerve pain which I could only somewhat mitigate by putting absolutely no muscular intentions into the “positioning” of my hands.  But unless I chose a career as a hand model, this pain management strategy always came undone “at hello” so …

 

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September 9, 2016 – This picture highlights the pale and dark color changes in my right palm which belies the excruciatingly painful interactions of my palm and any object such as a car steering wheel when I attempt to securely affix my hands to it. Please also take note of the “tight skin” which actually shows the “ridges and valleys” you’d typically only see in a Fingerprint. Thank you Erythromelalgia. 🙂

      As Dr. Chapman had told me, the diagnostic endgame was a Small Tissue Fiber biopsy so I established a relationship with a New Jersey neurologist hoping he would proceed in that exact manner. He was very accommodating as he empathized with my extreme discomfort and pressing need to be diagnosed and treated. He performed an EMG Test during my first appointment and set up the Biopsy for a few weeks later. Then he told me the Biopsy results would arrive approximately 2 weeks after that. Feeling as if the most promiscuous of Fire Ant colonies had Summer Share Rentals in my fingertips, toes and soles of my feet, I kept hearing in super-slow motion the word “weeks” emanating from my very compassionate physician’s mouth as I almost panicked at the thought of being absolutely clueless for at least another month as to the cause of the most debilitating pain I had ever experienced. But it was toward the end of the summer and my doctor did the best he could to accommodate me around what I figured were vacation schedules of a variety of dedicated healthcare professionals working with, and for, him and eventually on my behalf.
      Tom Petty nailed it when he sang, “the waiting is the hardest part,” and the next few weeks were some of the darker days of my life. The pain continued to intensify and expand into my feet and toes but what really scared me was not knowing why. But Doctor “Google” was at the ready with alacrity and I must have typed my symptoms into its search box 50 different times until my carefree ability to type was no longer. The closest I came to a logically sounding diagnosis was Raynaud’s Syndrome but no one described it as involving “Frostbite-type” or “Burning-feeling” pain and I just knew these keywords would most certainly appear somewhere within the description of whatever was causing me to lie on a couch all day during beautiful 90-degree late summer days with tears in my eyes each time I had to use my hands or fingers to operate the TV remote control.
      The first depreciation of my Rock-Star-Lifestyle became apparent when I couldn’t type at my computer without piercing pain because depressing any key on my computer keyboard with my “Frostbitten” fingertips made each stroke feel like I was pushing my fingers through glass and making them bleed uncontrollably. I also began dreading having to shower and shampoo my hair because each time my palms touched even a strand of my hair to apply the shampoo it felt as if someone with a hammer had driven a rusty nail through the palm of my hand.  If it were only a finger, each encounter with a strand of my hair felt like a coordinated attack from sharp needles which wouldn’t quit until they caused my fingers to bleed uncontrollably. This same exaggerated neurological response occurred during the rote of daily life whenever I involuntarily rubbed my chin and my fingers were met with the steely knifes of a 2-day growth.

 

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October 1, 2016 – Along with the other bizarre symptoms, Erythromelalgia or EM has created this “white area of demarcation” from my mid-knuckles to my fingertips IN BOTH HANDS. When I first noticed this “pattern” I thought I might have been bitten by some venomous spider. I showed this to several doctors during two (2) trips to a very prominent New York City hospital Emergency Room (ER) and not one of them had ever seen it before but that didn’t stop them from treating me like I was the ER Circus Freak despite being in excruciating pain. Sadly for traditional medical professionals, when I posted pictures of this amongst the Erythromelalgia online community, I received empathetic messages almost instantly as this was a very common sight to THEM.

      The Biopsy was conducted before Labor Day with results to follow in a few weeks thereafter.  However, I knew something was seriously wrong when the typing, shampooing and stubble pain I had half-joked about with my friends had become REAL.  It felt like I was in a relationship with the pain and I never took its severity seriously enough but now was forced to because it began “seeing other people.”  I also began feeling incredibly intense discomfort from not having enough skin to cover my hands and fingers.  I know, I had to say that one several times to myself out loud before I told anyone else.  With the color of my hands, toes and feet changing colors, I thought I was losing my f’n mind. But this was no figment of my imagination as this pain tripled at nighttime when my “digits” began to swell from rubbing the tips of my Frostbitten fingertips against the small liquid crystal touch screen of my cell phone so I wouldn’t have to confront the traumatic terror ingrained in the part of my mind which knew the Fire Ants recklessly fornicating in the infinite number of cells and tiny nerves in my fingertips would not be too pleased if I interrupted them by going old school computer keyboard on them.
      The swelling seemed to stretch the skin even more to the point where I felt as if I was walking around with two (2) Yogi Berra-sized catcher’s mitts as hands. The skin was so tight that I felt like “The Michelin Man” whenever I tried to hold a fork with my portly fingers. Driving all of the pain, I thought, had to be those dark blue, purple and black super-sensitive veins and nerves running through my hands, fingers and feet which for some reason, apparently unbeknownst to Dr. Google, had become as aggravated as Donald Trump tweeting at 3 AM some time in 2016 after learning that the Girl From Ipanema who had won his 1997 Miss Universe Title was now an American citizen set to tell the Press the truth behind her unique-looking brown-skinned son with the orange hair.

 

August, 2016 - My right hand as Erythromelalgia or EM makes my Nerves incredibly more sensitive.

August, 2016 – This is my right hand as Erythromelalgia or EM makes my nerves/blood vessels incredibly more sensitive. In fact, if you look closely I think the New York City Subway System Map is embedded in the palm of my hand!

      The saving grace, as usual whenever the seriousness of my health issues got to Defcon 1, was Dr. Mark L. Chapman.  I regularly communicated with him via email since early June when I had initially sought his medical care for this “Frostbite/Burning” severe pain problem. I figured everything was tied to my Crohn’s Disease in one way or another so therefore he was clearly the man to see.  But, by his own admission, he was not and  I had to see a Neurologist and a Rheumatologist to best solve my problem. While I did do that, it was Dr. Chapman’s almost weekly “supportive” emails which kept me afloat and eventually provided me with the perspective I needed to confront the perpetrator of all my pain. This is a very special man because time and time again over the past 32 years he has stayed engaged with me, wherever I lived at the time, e.g., New Jersey, Boston or Santa Monica, and in every hospital in which I had to do time, even at the prestigious Mayo Clinic in Rochester, Minnesota when he answered my SOS call with: “What can we possibly do for you here at Mt. Sinai Hospital that they can’t do for you at The Mayo Clinic?”
      I couldn’t answer his question out loud because I didn’t want to be disrespectful to the doctors taking care of me at The Mayo Clinic but the answer really was: “Mt. Sinai had YOU and they don’t have one of those here at The Mayo Clinic.”  When he reads this I know he will laugh out loud but it turned out to be DEAD-ON. Sure, my relationship with this man is predicated on the Doctor-Patient Relationship but after 32 years and now the onset of this very challenging nerve/blood vessel problem I now understand that he somehow always instinctively knew he might be among the few medical practitioners who could provide lifestyle-saving answers to the often extremely complex medical problems which were to plague me ever since I was diagnosed with Crohn’s Disease in 1984.
     In this instance, I somehow knew to keep him involved and all summer I sent him updates and pictures of the color changes I thought were diagnostically significant and I tried as best I could to vividly describe the pain and different debilitating sensations hoping something I wrote would trigger a diagnosis or a helpful thought from a man who has gotten me out of several life-threatening jams when other doctors had literally given up and even we had exhausted all of the plays in the Playbook.  It was then when he calmly would call an “audible” or draw up a play in the dirt and just like that I was back in law school, back at work or smiling with my friends as if I never had to worry about choosing my quarterback when time was running out, everything was on the line and we had to go the length of the field to pull out a victory.
      The day before Labor Day Weekend in 2016 Dr. Chapman emailed me with what he believed was my diagnosis. He modestly wrote that his diagnosis was purely speculative and that I needed to have it confirmed by a Neurologist and/or a Rheumatologist but he was confident I had a rare disease he had never seen in his sixty (60+) plus years practicing medicine but it was one he had read about. He wrote that he thought I had “Erythromelalgia” or “EM,” which is also known as “Man on Fire Syndrome.”  This diagnosis was subsequently confirmed and the Small Tissue Fiber biopsy came back ABNORMAL.  My quarterback had come through again but given the diagnosis he is not going to be able to lead me to victory this time.  That is on me but I’ve been coached by a VERY special man.

 

Dr. Mark L. Chapman and I after one of the many Videos we made together during which I attempted to share with the world his inimitable experience and knowledge treating the most complex of Inflammatory Bowel DIsease (IBD) cases.

Dr. Mark L. Chapman and I after one of the many Videos we made together during which I attempted to share with the world his inimitable experience and knowledge treating the most complex of Inflammatory Bowel Disease (IBD) cases.

How I plan to battle Erythromelalgia (“EM”) is explained in a short video which is presently being edited.  I will post it here on my Blog as soon as it is completed. I anticipate that to be before Wednesday, October 12, 2016.  I appreciate you reading my writing and just ask for your patience as these days it’s a bit harder for me to “put pen to paper” but as you will see in the EM Video, I view this as an opportunity of a lifetime. Thank you. 

Please feel free to constructively comment or to share any relevant thoughts or ideas. Thank you.

Please feel free to constructively comment or to share any relevant thoughts or ideas. Thank you.

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