Primary Sclerosing Cholangitis

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Primary Sclerosing Cholangitis
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    Community Voices

    “Cursed and Gifted with AS”

    March 1, 2018—Yay! I do have a chronic, debilitating, painful, and incurable disease! My lifelong nightmare was over when I was diagnosed with Ankylosing Spondylitis and prescribed medication to help prevent, instead of just treat, my symptoms. This happened 37 years after my first symptom as a teenager, and almost 30 years after I began having chronic pain and other symptoms in parts of the body known to be affected by AS. Although some of my pain began after very minor injuries, I believe the chronic inflammation caused by AS was at the root of my health problems.

    It’s genetic, man: My father and youngest brother, both named Rubin, had AS. My father’s symptoms began at age 18 with his first bout of uveitis. He soon developed unexplained knee and shoulder pain. In later years, he had GERD, a hip replacement, aortic valve disease, and colon cancer. His spine fused completely when he was in his 50s, and when he was 76, he fell and broke his neck in five places. He died at age 84 of a subdural hematoma.

    “Little” Rubin’s battle with AS began with ulcerative colitis, which was initially misdiagnosed as irritable bowel syndrome (IBS). He also had chronic back and neck pain with very limited flexibility; he appeared to be fused like our father. Little Rubin eventually developed primary sclerosing cholangitis and bile duct cancer that I believe were complications of his AS-related colitis, and he passed away at age 50—just six months after our father died.

    My story is similar to theirs, but like all AS patients, it’s different in its own way. The following is a timeline of my journey with AS:

    Fall 1981—First shot fired: I was an active junior in high school when I began having unexplained fatigue. I could hardly get out of bed and had low energy during the day. My father, who besides having severe AS was an amputee with one leg, accused his able-bodied daughter of being lazy. I soon found that nicotine and caffeine helped me “manage” my fatigue, but after I gave them up many years later, my fatigue got progressively worse.

    May 1989—Release the Kraken: I enlisted in the navy, and during boot camp I began having severe stomach pain and chronic constipation that was diagnosed as irritable bowel syndrome (IBS). After boot camp, I twisted my back slightly but developed persistent, severe pain that was diagnosed as chronic mid-back strain. Just two months after I injured my back, I hyperextended my knee and was diagnosed with chronic right knee strain. Why I had sudden, persistent pain was never investigated while I was in the service. I have dealt with chronic bowel, back, and knee pain ever since.

    After having two major injuries in my first six months of active duty, I was placed on permanent light duty. Additionally, I was considered unfit for ship duty, so my seafaring dreams were crushed. At age 25, this was just the first of many disappointments caused by AS.

    December 1992—Say hello to a new pain: One month after my son was born, I felt a sharp pain in my left hip that shot down to my knee. I was diagnosed with sciatica, which the doctor said was likely caused by the trauma of childbirth. Regular stretching helps, but I still get flares several times a year that hamper my mobility.

    1998—Ah, my neck! I was rear-ended, and although the damage to my car was very minimal, I developed severe neck pain. I’d had no previous neck injuries, but I had herniated discs in three places. The natural curvature had straightened as well. The pain from that injury resolved, but I still have unexplained, extensive damage to my cervical spine and recurrent neck pain.

    1999—Brace yourself: I started to get numbness, tingling, and pain in both wrists and hands that was diagnosed as carpal tunnel syndrome. Shortly after, I was diagnosed with GERD (GERD), and years later, with chronic gastritis. I reduced my digestive symptoms with medication and dietary changes, but they have persisted.

    May 2000: Missed it by that much: After driving cross country, my back pain flared, and the x-ray showed “sclerosis” of my sacroiliac joint. I later learned that sclerosis (thickening or hardening of bone) is how sacroiliitis, an AS marker, can appear on an x-ray. The doctor didn’t mention this, but he tested me for the HLA-B27 gene. When it was negative, he diagnosed me with sacroiliac joint dysfunction. I received the same treatment that I’d gotten up until that point: physical therapy, NSAIDS, and painkillers.

    January 2005—…and broke my sacroiliac: I bent to lift a bench when I suddenly felt such a sharp pain near my tailbone that I couldn’t stand back up. I was diagnosed with facet joint arthropathy and low back sprain, and received my first spinal injections. I was off work for five months, and at age 40 I began to realize that the biggest struggle of my life was going to be living in my own body.

    2008-2010—Deterioration: My life became a pattern of manageable pain periodically interrupted by very painful flares. Now a single mother, I started working full time as an auto claims adjuster. Within months of driving hundreds of miles a week, my neck, back, and wrist pain flared up worse than they ever had. I was on disability for almost two years this time, during which my father and brother died. After their deaths, I became afraid I had AS, so I saw another doctor. This time, the x-ray of my sacroiliac joint was “unremarkable”, and I was still HLA-B27 negative, so he told me I didn’t have AS. He explained that my entire spine simply had degenerative disc disease.

    2010-2017—Voodoo doll: During these years, I developed several new recurring symptoms: tenderness and pain in my chest and outer hips and thighs; pain in my thumb, heel, and elbow; shortness of breath; and excess protein and trace blood in my urine. I became depressed dealing with all this pain, and I’m sure my emotional state worsened my physical symptoms. I switched to a desk job, but sitting all day seemed to aggravate my symptoms even more.

    In this 8-year period, I managed my pain with seven joint surgeries and numerous injections into ten different joints. I endured these procedures because I couldn’t take NSAIDS by mouth anymore. I also spent a small fortune on massages, heating pads, ice packs, braces, and seat cushions, and Biofreeze. I did get diagnosed with fibromyalgia in 2014, but the treatment for it had no effect on my joint and digestive symptoms.

    Not surprisingly, working full-time became impossible, so I retired on disability in 2014. Fortunately, I managed to earn my master’s degree in 2010, and started teaching English as a Second Language to adults part-time after I retired. I love what I do, and it’s been a great diversion.

    2018—Sick and tired of being sick and tired: After nearly 30 years of chronic pain and digestive issues, I’d gotten used to living with regular discomfort. However, I was growing weary of the flares, which were occurring more often and lasting longer (and now included debilitating fatigue and feverishness). Even the stiffness in my back in the morning, which used to be sporadic, had become a daily occurrence. It was like the first day of the flu every single day. When I could take it no longer, my only option was to go to the doctor for a Toradol (an NSAID) shot.

    I was also an emotional wreck. Relieving stress was difficult. I had no energy for exercise, and leisure activities I’d previously enjoyed now increased my pain. I avoided social activities because of anticipated pain, but I was also tired of being housebound “socializing” through social media. Furthermore, I was angry that I although I was well-educated and experienced, my health had killed my career and stifled my earning potential. Worst of all, my disease had put a strain on my marriage and revealed how unsupportive some of my friends and family were. I was even called a hypochondriac. I became despondent, and occasionally suicidal.

    Fortunately, when I reached the end of my rope, there was…Google. It started when I had a CT scan for an unrelated condition. The report said I had sacroiliitis and osteitis pubis—two diseases I’d never heard of, but had been suffering from for years. I googled their causes, and Ankylosing Spondylitis was all over the search results. I also discovered that my other recurrent conditions (costochondritis, trochanter bursitis, Achilles tendonitis, tennis and golfer’s elbow, De Quervain’s tenosynovitis) were possibly AS-related enthesitis.

    I was convinced now that I had AS, but I remembered the HLA-B27 gene issue, so I did some research. I’m a black female, and I was surprised to learn that about half of African Americans with AS don’t have the gene. This wasn’t new information—it was discovered in the 1970s. Unfortunately, the doctors I’d seen up until then believed AS only happened to folks who were HLA-B27 positive, regardless of their symptoms.

    Today— Acceptance and renewal: Armed with knowledge about AS and determined to have a thorough workup this time, I was fortunate to find a rheumatologist who really listened. He took my full history and ordered imaging studies of my entire spine, which showed active sacroiliitis and bone marrow edema (inflammation in the bone), two AS diagnostic criteria. He diagnosed me with AS when he got the test results and established a treatment plan. I was so relieved that I just sat in my car after the appointment and cried like a baby.

    My first treatment was the biologic, Humira, which stopped working after several months. Now I’m on Enbrel, which helped give me my life back. I also manage my disease with regular stretching, exercise and massages, and I’ve found new leisure activities to relieve stress. Although I still live with daily pain and fatigue and occasional major flares, I’ve accepted my new life and no longer feel hopeless about my situation.

    Lastly, I’ve also learned to forgive the people who haven’t been there for me and truly appreciate the angels who have. AS has been a curse and a gift in my life. It’s broken me physically over the years, but it’s also strengthened my resolve to live life to the fullest. Today, I’m the best I’ve ever been.

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    Community Voices

    Based on the Mighty's list of rare diseases, Primary Sclerosing Cholangitis isn't one of them. It's a rare disease.  My GI at the Mayo Clinic told me it's rare and often not detected until it's too late to do anything about it. I was lucky/fortunate/blessed
    to be at the right place at the right time and went on an experimental protocol of medication. That to me says rare disease. Thankfully the medications worked and the disease is in remission. I've seen medical folks who don't know what PSC is. In a nutshell a degenerative liver disease. Who decides the criteria to determine if something is rare or not? I don't see anyone posting about liver diseases which indicate to me that people don't know about them making them rare. PSC is a even when it's not listed as a . Who determines what is and isn't on the list? I look forward to the response from "The Mighty."
    #RareDisease #liver

    4 people are talking about this
    Community Voices

    Everyday is a new struggle and a different battle and I never feel like I have control. At any moment I could have an attack of severe cramping from Ulcerative Colitis or I could spend hours sitting on the floor of my bathroom scratching my body all over because of Primary Sclerosing Cholangitis or at any second I could get a call to go and have my second liver transplant. It is so frustrating to never know how your day is going to go, how do you help to cope with all the uncertainty that comes with being sick?

    6 people are talking about this
    Allie Hublou

    Raising Awareness of Primary Sclerosing Cholangitis (PSC)

    I woke up, climbed out of my cot, quickly got my boots on and hurried out to the line for the latrine. I was going to puke and hoped I would make it. Luckily the person inside finished their business quickly. This wasn’t the first time I’d thrown up during my basic training, but it was the most intense, and I had a pain in my right side as well. It was warrior week, as the air force calls their most intense week of training. Fifth week, out of six. We were doing field training and I didn’t feel right. After I was sick I had gone to the training instructors and they had me checked out, only to think I had gallstones and have an ambulance pick me up and take me out to the hospital. That day I threw up a couple more times before they released me, and I went to stay in a neighboring flight’s dorm while I waiting for a transport back to mine the next day. Only, about 20 minutes after I laid down, I had to run to the bathroom and be sick again and get rushed back to the hospital yet again. The nurse actually told me she thought I was faking until I puked uncontrollably right in front of her, and only just missed her shoes. It was then that they started to think I had gallstones, and a day later I was in surgery getting my gallbladder removed.   It was a scary time for me. I’d never really been sick much, except for the occasional throwing up. I was too young to know that was a sign of something more severe at 16 though, and it continued on up until I was admitted and rushed into surgery shortly after my 18th birthday. I was alone, but I thought I would be OK after it was taken out. I was left on med hold after my surgery, and I wasn’t recovering as the doctors thought I should. I was called down to the Squadron’s Commander’s office. I was terrified I was in trouble but I went without delay. I was asked some basic questions about my training, what I had missed with warrior week while I was sick and a few other things. He then brought up that he had a call from my doctor and they wanted to do another test. I agreed it would be fine, thinking nothing of it. Everything was a blur after that up until the next thing I remember clearly. Laying in the recovery room after my first MRCP, my throat sore and groggy from the anesthetic. The doctor was standing in front of me, explaining that they’d found strictures in my bile ducts, and showed me the images they took while they did the procedure. He explained that I had five to 10 years before I would have to have a transplant, or I would die. I felt… broken… in shock… alone… and all I could do was break down into tears and sob in front of this man I didn’t even know. My house came crashing down around my ears while I was still in it, and I didn’t know what to do. That is the day my world changed forever, and the single worst day of my entire existence. Up until that point my whole life’s plan had been to stay in the military for many, many years, and then maybe go civilian and transfer to a civilian government agency. I had it all worked out. I was going to cross train into another job, because I knew the skills I would learn would be highly useful where I wanted to go, but I was just handed a diagnosis telling me that life as I knew it was at an end. That I would have to return to the home of my abusive mother, and deal with the fact that she wouldn’t let me sleep there even one night after I was discharged. It felt as if my heart had been broken into a million pieces. Today, I’d like to think there aren’t others like me who are sitting alone with a doctor standing before them getting a horrible diagnosis. I’d like to believe that their family will be there for them no matter what. What’s worse, my best friends at the time didn’t believe me, or understand why I was different. They actually ostracized me and stopped being my friends because of this stuff. They had never even really asked, or let me cry with them over the loss. I was grieving. Grieving the loss of what my life might have been. I was grieving the loss of being able to see the world and live a normal life. I was grieving being able to live at all, because I thought I was going to die in five years, and while I don’t really remember, I probably pushed them away and pushed the limits of what they knew how to cope with. I’m not really sure. I just know that my head was in a bad, dark place, and emotionally I was completely broken, even though I tried to hide it from everyone. I don’t think I did a very good job of that though. I am sharing this story because I know there are others out there who feel like me and I know they need to know their world isn’t over. Just as I found out being told this horrible thing wasn’t the end for me, I hope I help someone else find the strength to reach out and talk to those they love. Life goes on, and the world keeps turning, no matter how we feel. We just have to learn to adapt to it. I may have PSC, but it doesn’t have me, as my friends in the PSC Partners Seeking a Cure online support group like to say. I found them, and I dearly hope you do too. Even if I silently lurk most of the time I am so glad for these ladies and gentlemen. They understand, and they are not alone, and now neither am I. It’s now been 15 years since that horrible day, and I’ve learned through reading and through life experiences. I’ve had a beautiful daughter whom I love dearly and a husband who means the world to me, even as he’s sleeping in the bed next to the computer while I write this at 4 a.m. My life hasn’t stopped just because I was given what felt like a death sentence – in fact, I’ve done far better than my original prognosis (though it really hasn’t been all sunshine and roses). PSC is a silent disease, and unless someone has jaundice (yellow skin and eyes), you wouldn’t even know there’s something going on with their health. Some people are able to go their entire lives without a transplant, and yet I know at least three people online who have had multiple, because PSC can come back and affect the new liver too. It can affect more than the liver – in fact, my pancreas is currently being affected too. The biggest thing to know about this is that the only treatment that can cure it is transplantation. Unfortunately, there is also an organ shortage. Some patients can even take partial live donor transplants and take up to 70 percent of the  donor’s liver, and the organ actually regenerates too. Talk to your family, make your wishes known. I encourage you to become an organ donor. We want to hear your story. Become a Mighty contributor here.

    Allie Hublou

    Primary Sclerosing Cholangitis: Distracting Yourself From Chronic Pain

    I was laying in bed the other day when I was awoken by a horrible pain in my gut once again. All I could do was roll onto my side, curl up clutching my belly and cry because it hurt so badly. I stayed that way for a little while, but my sobbing was making the pain so much worse, and I could hardly breathe because it was so severe. My friends, when I posted to vent on social media, didn’t understand at all. “Go to the hospital and call your doctor,” I was told. Sometimes it’s just not that easy. I’d felt like this before, and when I went to the doctor there was nothing they could do but give me pain pills that could further damage my already failing liver. Honestly, if you know what I’m talking about, I’m so, so sorry. But we don’t have to focus on that pain. There are other ways I’ve found to get through those days, and I hope you have too. Eventually I finally got off the bed, drug my pillow and fuzzy blanket to the living room where my cat, Nicodemus, tried to help me catch the blanket as it drug across the floor. I smiled, and felt a little bit better. I actually found myself using my three cats as they played rambunctiously around the living room and kitchen and after a while I was slowly able to find a measure of breathing that didn’t cause me as much pain. Nicodemus was VERY interested in getting all my attention and figuring out what I was doing. I started by propping myself up on my sectional with my memory foam pillow behind me, cradling me to lessen my pain a little, and found other things to distract myself from hurting so badly too. Spamming all over Facebook sometimes helped, zoning off into a good Netflix series also did, and so did roleplaying on a discord chat I belong to in the SWTOR community on my phone, because I could stay in a sitting position to play the game on my computer with my online friends. Also, I found gaming to be a huge help on days I could stand to sit for a little while at a time. (For those who are wondering, SWTOR stands for Star Wars: the Old Republic and I do find it is very great for helping me distract myself.) Either way, distracting myself made me feel better, and even if it was only a placebo effect, it still helped me emotionally so I didn’t start crying and make myself worse all over again. It didn’t really matter what I did to distract myself from that pain, only that I had a distraction. Before I realized this I would just lay in bed for days wallowing in self-pity and misery, only making things worse for myself. I would get mean, and by mean I mean I would snap at anyone around me in a very toxic manner. I was my own worst enemy in that state, and by the time I realized it I would only end up crying more which inevitably made me feel worse physically too. I was sabotaging my own relationships with my husband and my child unintentionally. I am mighty, hear me roar… or yawn… So please, find something you love that isn’t physically taxing and see if distracting and forcing yourself to think about other things helps you, like it did me. If you have pets, spend hours watching them do silly things and laugh in your heart. Skype with people you love and joke around with them, or look at old photo albums. If it makes you happy and helps you as a healthy coping mechanism, do it. It doesn’t make the pain go away, but it makes our lives better. We want to hear your story. Become a Mighty contributor here.

    Allie Hublou

    Primary Sclerosing Cholangitis: Asking for Help With Chronic Illness

    Some days are easier to get through than others. Some days leave me curled up in a bed bemoaning my own pain. Some days are various levels between. It feels like I’m playing Russian roulette sometimes when I go from feeling great one day to curled up in a ball crying on my bed the next, but I’ve come to accept the good with the bad. People don’t always realize what it’s like to live with chronic illness, but that’s OK. I just hope they don’t end up sick like me, because not even my worst enemies deserve to feel like this.   I know it may sound silly, but sometimes what gets me through a bad day is telling myself I’ve felt worse, and this will pass, though I found myself a year and a half ago wondering if that was really true. I couldn’t get out of bed on my own, or shower without my husband helping me. I couldn’t cook, or even make it to the bathroom without assistance, but I made it through. I made it through the care of family and friends helping me when I needed it and driving me to see my doctors, even when they were four hours away, and even refusing gas money when they took me. I don’t like to think of that time in my life because I was in a very dark place mentally. I didn’t think I was going to make it, and was so sick I lost most of my weight. What I’ve learned today is it’s OK to have those feelings as long as I don’t bottle them up and hide them from those I love, because they were scared too and we needed each other too. My little girl needed her mom, but I didn’t know then what I know now – to talk to her about these things. It’s OK to need and receive help. If I hadn’t I probably would not have made it through the massive infection I had gotten in the bile ducts of my liver due to surgery to check for cancer and to open them up, because they had strictures and were swelling shut. It’s also OK to be scared, but if you are scared, your family is probably scared too. Take comfort with them rather than pushing them away to try and save their feelings. It isn’t just you, and if you can’t do it alone, good. Isolation as I see it is the enemy of the chronically ill and even thinking back on how I was isolating myself I can’t help but shed a few tears. It hurt my family and my baby girl later told me she was terrified I was going to die, too. It just made me want to hold her and cry for hours and fight to get better once and for all. In the end, those few I did let in are some of the most special people in my life. They cared for me, helped with chores about the house, cooked me food and helped me shower or use the toilet. Friends that hadn’t had any medical training and were not even 21 yet came over while my husband had to work and helped me, and I’m still grateful for their help, even when they could have been out doing fun things. It all came down to asking for that help so they could offer, and forging a stronger bond of friendship then I’d ever had before, a stronger relationship with my husband than I had had before. Asking for help saved me, not only physically but emotionally, and I can never be happy enough that I did because of what I gained from it. There are still good people out there, if you give them the chance. We want to hear your story. Become a Mighty contributor here.