TALK ABOUT IT TUESDAY! A “Short” Version of My “Behcet’s Story”

I really want to get this “blog” really up and running – there is so much “misinformation” and so little “awareness” about Behcet’s, ESPECIALLY moderate-to-severe Behcet’s (i.e., “systemic involvement”) and it is incredibly important to me for this “blog” to be a source of “reliable” information as well as a place where people with Behcet’s and all other chronic illnesses and disabilities (especially “invisible illnesses” and severe illnesses) can go to see that none of us are “alone” in this terribly lonely fight. I haven’t really “shared” my Behcet’s story all at once, so I’m going to try to write this up as concisely as I can. I have the difficulty that throughout my life I have had Behcet’s “symptoms;” however, I was also born with a primary immune deficiency (Common Variable Immune Deficiency, aka hypogammaglobulanemia) – an immune deficiency that is NOT contagious but “congenital,” thus the “primary.” This makes the story more complicated because I was not diagnosed with my immune deficiency until I was in my very early 20’s despite having fought life-threatening infections REGULARLY since the age of 2 months. Some of the “symptoms” here might overlap; I will try to stick to the Behcet’s side of things, but please bear with me! (I will also post my “CVID Journey” story at some point, I promise!)

As I mentioned above, I was sick from the age of 2 months. Incredibly severe infections, often of unknown origin with INCREDIBLY high fevers; when I was a kid, I remember lying on the sofa too sick to move and our family doctor had instructed my mother to sit with me and take my temperature regularly – if it hit 105, we were instructed to get to the ER STAT. My preschool / elementary school years were plagued with repeated and resistant bouts of strep; that first severe infection at 2 months ended up being a systemic strep infection – I mention this “chain” of infections because I sincerely believe the countless episodes of strep “triggered” the HLA-B51 “allele” for which I tested positive when I was “formally diagnosed” around the age of 17-18.

I have had mouth ulcers as long as I can remember. Honestly, I never really thought much of them – my mother got them and my sister got them and although they were not as severe as mine were, it never occurred to me that it was anything “unusual.” Uncomfortable, yes, but symptomatic of a much more serious condition? Not at all. And here I do not mean I would get like a mouth ulcer a couple of times a year – as long as I can remember, I would get multiple, sometimes dozens of mouth ulcers in my mouth (sometimes down into my throat and sometimes starting up my nasal passageway) at a time – it was uncomfortable, but my family also had them (albeit to a more minor degree), so I didn’t really think much of them – we all kind of chalked it up to having viruses or strep in my case. I have also had skin rashes basically all over my body throughout my life – unknown bouts of hives and sores that we just assumed were “normal.” I also had TERRIBLE joint pain in my fingers and hands as a young child (through the present!); I never thought it was arthritis or anything like that – if anything, I thought I might have fibromyalgia like my mother (I do, but that was not the cause of the joint swelling and pain, &c.) or the swelling and pain in my hands was just an unavoidable result of playing the piano, violin, cello, and at that time, the flute and the harp. I used my fingers HARD so it didn’t surprise me that they hurt and would swell up and get really hot. My mother remembers very well waking me up for school in the morning (or on weekends) and my groaning to her – “My hands hurt so bad – can you please rub them?” It’s kind of a funny story, because in “ye olde days” before Behcet’s was anything anyone ever talked about, Dr. Yazici actually questioned my diagnosis … until I sat across the desk from him in his old basement office and he literally watched my fingers and hands swell and get red and hot and angry while we were talking with him.

At any rate, when I was around 13-14, my sister and I received the chicken pox vaccine. It did not turn out well for either of us. My sister eventually developed shingles and I ended up with severe sciatica that resulted in receiving nerve blocks regularly at CHOP (Children’s Hospital of Philadelphia). *I WANT TO NOTE HERE THAT ALTHOUGH BOTH OF US HAD BAD REACTIONS TO THIS PARTICULAR VACCINE – AND BOTH OF US HAD BAD REACTIONS TO OTHER VACCINES – I AM BY NO MEANS AGAINST VACCINATION! IN FACT, I FIRMLY BELIEVE IN VACCINATIONS! THEY ARE NOT ONLY FOR THE HEALTH OF THE PERSON RECEIVING THEM, BUT FOR THE PROTECTION OF VULNERABLE INDIVIDUALS LIKE ME AND MANY OTHERS WHO ARE EVEN MORE ILL! THIS IS A CLASSIC TYPE/TOKEN OR CAUSATION/CORRELATION FALLACY! A WHOLE POPULATION IS NOT “ENDANGERED” BY VACCINATIONS JUST BECAUSE SOME INDIVIDUALS WITHIN THAT POPULATION HAD BAD REACTIONS TO SAID VACCINATIONS! AS FOR THE AUTISM BIT, I HAVE DISCUSSED THAT ELSEWHERE AND I WILL DO SO AGAIN HERE LATER! THE POINT I WANT TO CONVEY IN ALL OF THIS IS THAT ALTHOUGH WE BOTH HAD A BAD REACTION TO THIS PARTICULAR VACCINE, IT IS CRUCIAL TO SPEAK WITH YOUR PHYSICIAN IF YOU HAVE ANY CONCERNS AND TO “DO YOUR HOMEWORK” ABOUT FACTUAL INFORMATION REGARDING VACCINATIONS – FOR ME, THAT WOULD HAVE KNOWN THAT THERE WERE BACTERIA ACTUALLY IN THE PNEUMOVAX THAT CAUSED ME TO DEVELOP SEVERE CASES OF CELLULITIS (ONCE MRSA) BOTH TIMES I RECEIVED THAT VACCINE – MOST PEOPLE CAN TOLERATE IT AND FIGHT IT OFF WITHOUT ISSUE – GIVEN MY PAST HISTORY WITH STREP, I AM NOT ONE OF THOSE PEOPLE! THAT DOES NOT MEAN IN ANY WAY THAT I THINK THE PNEUMOVAX IS “BAD” – I THINK EVERYONE WHO CAN GET IT SHOULD GET IT! I WISH I COULD, THAT IS FOR SURE! SO TO END THAT LONG RANT, YOU HAVE TO KNOW WHAT IS IN THE VACCINE YOU’RE GETTING AND IF YOU HAVE A SENSITIVITY TO ANYTHING IN IT, TALK TO YOUR PHYSICIAN – THEY MAKE ALL KINDS OF VACCINATIONS THESE DAYS WITHOUT CERTAIN ADDITIVES OR PRESERVATIVES AND, LIKE THE TETANUS SHOT, THERE ARE MANY VACCINATIONS THAT ARE NOW SINGLED OUT (HERE I AM USING THE EXAMPLE OF THE DPT SHOT) – JUST BE EDUCATED AND DO NOT BE SWAYED BY FEAR! THINK ABOUT THE WELFARE OF YOURSELF, YOUR CHILDREN, AND OTHER VULNERABLE INDIVIDUALS!*

Okay … so yes, at the age of around 13-14 I developed severe sciatica and other nerve problems. A few years later (maybe between 14-16), I started developing genital ulcers. My PCP at the time gave me Valtrex for an unknown amount of time – a long time – without EVER giving me a herpes test. As a young girl who hadn’t even gotten her period yet (I didn’t start menstruating until I was 16; I only note that because I have heard many stories from others with Behcet’s that it has affected them in this sort of way and in other ways AND that their symptoms either first appeared or worsened around the time of puberty) – and as a young girl who had never had sex – I was devastated. It was not until I was 17 (JUST 17, by the way), and ended up in the hospital because I had developed literally so many genital ulcers that I couldn’t go to the bathroom that I was actually TESTED FOR HERPES – repeated tests all came back negative. I was fortunate to have been admitted to the hospital at which I was admitted because an infectious disease specialist saw my case and told my family and me that he would not let me leave the hospital until he knew what was wrong with me. I was hospitalized for about three weeks at that point; I was kind of the “circus exhibit;” none of the doctors who saw me had EVER seen genital ulcers as big and as swollen and as numerous as I had. Finally, after treatment with steroids and careful research by the aforementioned infectious disease specialist, I was diagnosed with Behcet’s. I also had a positive pathergy test and it was also around this time that I had my genetic testing done and I came back HLA-B51+.

I went through the typical drugs that are so frequently used when Behcet’s is diagnosed – colchicine, azathioprine (Imuran), plaquenil, basically every NSAID that was made at the time. I had terrible reactions to all of the drugs mentioned above (I will not go into all that now!). I did manage to get “healthy” for about a 2-3-year-long period (perhaps a little longer; I was not “not sick” as I still caught every infection that was around due to my then-undiagnosed CVID and I still had “flares” but they were limited to ulcers, skin lesions, peripheral nerve problems, and joint issues in my extremities – oh! I have also had Raynaud’s all of my life – I remember in high school my friends would do everything they could think of to get my hands to come back from being blue and purple). During this “healthy period,” I went to Pittsburgh where I attended university. I had a few episodes that landed me in the hospital (infections and severe reactions to medications in conjunction with the Behcet’s) but I had more “healthy” time than “sick” time – I tend to measure one’s disease “severity” not so much by symptoms per se, but by how “functional” one is (perhaps because of lack of symptoms) – at that point in time, I had my “sick times” but I was overall “functional,” going to school at least 2 semesters a year and staying over the summer.

However, when I was around 21-22, just shortly after I had been diagnosed with CVID, the Behcet’s decided to get NASTY – this is actually quite common with this “syndrome;” it is uncommon that mine manifested severely in one way, paused, and then acted up VERY severely – it is more common for systemic disease to present itself fairly close to the manifestation of the other symptoms. At any rate, the disease went into my eyes (this is, by the way, the general “gauge” for the distinction between Bechet’s and, as Dr. Yazici says, “life-threatening Behcet’s” – systemic involvement can occur in other ways, but it is usually ocular involvement which signals the disease is severe and systemic) – I cannot tell you how many bouts of uveitis, iritis, optic neuritis, and just bleeding inside of my eye I have experienced – a LOT. At the same time, the “disease” started affecting my right SI joint and hip – I had never had such horrific pain in my life (even to this day) – my large joints and BONES would actually swell (this was, again, in ye olde days when people said “Behcet’s does not affect the large joints!” We know more now, that’s for sure!). I also started having episodes of aseptic meningitis along with the optic neuritis – the pain was horrific. I also started experiencing severe GI bleeds; one year, I was hospitalized after taking Motrin just to try to control my joint/bone pain – by the time I was admitted to the hospital and an endoscopy could be done, the doctor found a stomach full of dried blood. The skin manifestations increased. I started developing phlebitis and other vascular issues in my extremities. It was at this point that I was fortunate enough to get in to see Dr. Yazici, who, although skeptical, confirmed my diagnosis. It’s kind of funny because as research has continued with the progression of time, my “case” seems to become more and more of a “textbook” Behcet’s case.

That is really MOST of the “outline” of my Behcet’s story – there are many more incidents and symptoms I could talk about, but this post has certainly been longer than I wanted it to be – I told you it was a convoluted story! 😉 I am fortunate now to have a wonderful local rheumatologist and a not-so-local but close-ish PCP who have literally saved my life time and again. I am also extraordinarily fortunate to be one of the few patients Dr. Y has kept seeing. I have seen more quacks and “Gods” than should even exist in the world, but I am very lucky to have the amazing doctors I do have. I have several eye specialists – I am currently working on finding a specific uveitis specialist, given my most recent eye involvement – although I need to find an ophthalmologist who will see me THE DAY OR THE DAY AFTER my eye(s) start(s) acting up. I have also gone through a terribly long list of medications and they have mostly failed or been medications to which I have been allergic; thankfully, my current medications seem to be helping and my labs are better now than they have EVER been (like … in my entire life “EVER!”). I always worry because I do not have a lot of “options” left if these meds do fail – but the hope in that is that there is INCREDIBLE research being done on these sorts of illnesses and every year (or 6 months) I see Dr. Y I learn about new medications. Despite my “minor” flares (they are fairly major in discomfort and the eye issues, but not “MAJOR” because I have not been hospitalized), things seem to be heading in the right direction after over 16 years since my initial diagnosis.

I apologize that turned out to be so long, but I hope that gives you a general idea of “my Behcet’s story.” I have so much more to talk about just in that “general story!” I would love to hear from you – if you have Behcet’s or if you have some other illness or disability and what YOUR “story” is! I hope you are all having a happy and healthy day wherever you are in the world! Thank you for taking the time to read this and I will be back soon!

❤ Always, Beth