"No, it's *autoimmune* hepatitis, and I can't pass it on to you no matter how much I want to right now."

Wherein I give the timeline of developments leading up to this still-possibly-poor-decision of a blog.


Late this past March I started to get sore and tired.  This was surprising because in general at that point in the year I'm noticing an uptick in my energy and general gung-ho-ness.  By mid-April I was dealing with constant joint pain in my feet and ankles that at best made walking uncomfortable and at worst made it physically difficult to get out of bed in the morning - for the hour or so after I woke up, the pain was akin to any major injury I've had over the years to those joints.  I had started playing in an intramural kickball league in Philly and generally assumed that the soreness was the result of actually engaging in some physical activity after the long winter hibernation (which, this year, was made even more extreme by the completely batshit amounts of snow that fell on Philly and its surrounding area).  I assumed, given time, it would improve.

It didn't.
 The joint pain spread to my wrists, elbows, knees and fingers.  On the fingers and elbows especially I began to notice swelling that was tender to the touch and lent to the joints some odd, pseudo-deformity angles.  Along with the increase in pain came complete physical and mental exhaustion.  I wasn't sleeping well, but I was sleeping a lot.  If I went out on one weekend night I was wrecked, energy-wise, for the next few days.  I had started to drop a significant amount of weight - at any other time, a welcome symptom, but kind of alarming given how little activity I was undertaking and the fact that it accompanied a near-complete loss of appetite.  I lost a ton of strength, and found myself struggling to lift and move things that would have been easy for me to chuck around when I was in middle school.  This became apparent, and was mostly relevant, when I was completely useless during our moving process early in the summer.

By mid-July I was, aside from one or two times a week when I would go into the city to see friends, spending pretty much my whole day in bed, moving around as little as possible to avoid irritating my increasingly swollen, at that point very unstable joints.  Clearly, I assumed, I had incurable bone cancer and was going to die at any moment so there was no point in being uncomfortable while I waited it out.

That's when things got bad.  I woke up one night with shooting pain in my right shoulder and abdomen, feeling feverish.  After a few minutes of very shallow breathing and sitting upright it subsided, but not entirely.  It got steadily worse over the next few days, peaking whenever I inhaled, or coughed, or laughed, or... basically did anything involving the expansion of my lungs.  It kept me from sleeping and eventually, accompanying a particularly violent bout of gagging, made me pass out in my dog's puke (the cause, as you may surmise, of the gagging).  This led to the measured decision that it was probably time to go see the doctor again.

Because the pain was mostly centralized in my shoulder I assumed some kind of injury (or tumor - honestly I kind of assumed at that point that I was just riddled with tumors) was causing it.  My doctor, knowing more about medicine than I do, humored my request for an x-ray but because I was severely jaundiced (I hadn't noticed) was more concerned about my liver.  He sent me to the hospital with a prescription for a shoulder x-ray and the first of many blood tests.  That was on a Friday, which ultimately became the last night I would ever have a drink.  Had I known that I probably would have planned ahead, had a nice IPA, maybe a good bourbon.  Instead, I am sad to report that the last alcoholic beverage I ever consumed was a Four Loko on a friend's roof.  This, really, is the greatest tragedy of my circumstances.

Moving on, though.

The following Monday my doctor called me to tell me to go to the emergency room for a liver ultrasound - the blood tests had come back and my liver functions were alarmingly abnormal - in some cases 20-30 times the normal range abnormal.  Go to the emergency room abnormal.  In less alarming news, my shoulder x-ray looked fine.

The Paoli Hospital emergency room was a profoundly unproductive experience.  They drew blood for another series of liver function tests (which, six hours later, were "still very abnormal"), diagnosed me with gall stones and set up an IV in my arm in preparation to admit me to wait for surgery in the morning.  Then, they took the ultrasound, which revealed that I did not have gall stones and would not, in fact, be in need of surgery in the morning.  To add insult to ineptitude, the ultrasound tech didn't even find it funny when I asked if she could tell if it was a boy or a girl.  Moved back to my room, I waited a few more hours for the internist to come down and tell me that I had hepatitis and they would need to test me to determine which one it was (A, B, C, Durr).  Despite my assuring the internist that there was pretty much no way I had contracted any form of viral hepatitis in the time frame that would make me symptomatic at that point, they were pretty confident.  They took the IV tube out of my arm (it had never been used) and sent me home with a brochure about living with viral hepatitis, which their test would of course reveal, I wasn't.

Ultimately stumped when they called me the next morning to explain that their second diagnosis had also been off, they referred me to a gastroenterologist.  I was unable to get an appointment with him until late August and spent the time until then essentially the same way I had spent July - mostly bedridden with occasional bouts of socialization when I could manage it.  When I finally saw him he ordered another battery of blood tests (15 of them, requiring 7 draw tubes and nearly a thousand dollars).  Two weeks later he called to say he was confident I was suffering from one of two things -- hemochromatosis, a disease in which iron builds up in the body's organs and ultimately just ruins the hell out of them, or autoimmune hepatitis, a disease in which your body identifies its own liver as something that must, at all costs, be destroyed.  Hemochromatosis had a certain appeal in that the treatment for it is still, to this day, bloodletting.  It was problematic, however, that it was still even a possibility.  I'd read enough at that point to know that the results of the blood tests that had been ordered could not possibly be ambiguous between those two diagnoses - they would be indicated by completely different factors.

It was mid-September by that point and my condition was not improving.  The pain had become more manageable because I was used to it, but I was severely jaundiced, still dropping weight, had no appetite or energy, and all my bodily fluids were exciting (horrific) shades of dark yellow no matter how much water I managed to drink.  We ended up following some of my dad's incredibly lucky connections and getting an appointment at the hospital at UPenn, having more or less lost faith in the previous line of care we'd been pursuing.  In late September I saw Dr. Reddy at Penn.  He had absolutely no hesitation, hearing my symptoms and then reviewing my test numbers, in telling me that I had autoimmune hepatitis ("you are presenting a classic case").  He scheduled me for a biopsy less than a week later to take some liver tissue samples and check on the extent of the damage, which would give them the approximate amount of time I had had the disease, and began me immediately on treatment with high-dose prednisone, a corticosteroid that would combat the inflammation of my liver causing the abdominal and shoulder pain (caused by the diaphragm contacting the tender, inflamed liver during expansion) and relieve the inflammation and pain in the joints.

Prednisone has some nasty side effects - crazy acne, weight gain, puffy face, neck fat, and over the long term bone loss, glaucoma, and diabetes - but the immediate gains were unbelievable.  Within two days my joint pain was gone - and not just the acute pain that I'd had for the previous months, but all of the soreness and instability I'd assumed was just the result of my weight for years and years.  By the time I got back to Penn for the biopsy I had been relieved of most of the acute symptoms of my disease as well as the fatigue, sleep irregularities, and lack of energy that I had just assumed was my "normal".  My blood pressure had also dropped and my resting pulse rate, which had always been in the 70s-80s, was suddenly in the high 40s.

And then, of course, the biopsy showed that there was a reason those old symptoms had been my normal - based on the extent of damage to my liver (a 3.5 on a scale where 0 is normal and 4 is cirrhosis), I had been quietly living with autoimmune hepatitis for my entire adult and adolescent life: at least 10, probably more like 15 years.  The implications of that fact continue to sort of loom large in my mind, and I'll definitely address it in another post at some point, but the bottom line was - the week since I had started prednisone was in fact the first week of my life, since childhood, that I was feeling almost the way most people feel all the time.

The few months that followed up until this point have been about the progress of the treatment - blood tests every two weeks to track my liver numbers (all of them, as of my last test, are finally either back in the normal range or barely abnormal) and the first of a series of imaging tests to track my bone density and keep an eye on my liver (which, because of the level of cirrhosis, is now a high risk to develop liver cancers).  The most intense part of the process has been being proactive about keeping the side effects of prednisone at bay - my diet has been extremely restricted and I've had to be really careful about maintaining a certain level of activity because every signal the drug sends your body is "store fat!  In your face!  And other places that will make you look like a luck troll!"  I've been mostly successful, avoiding every major side effect except swollen cheeks and forehead acne.  I've started reducing my dose of prednisone and boosting my dose of imuran, a non-steroid immunosuppressant that will ultimately be my maintenance drug for life.  Imuran comes with an increased risk of skin cancer over the long term, but compared to the diabetes/glaucoma/fatness/acne suite of prednisone, its side effects are relatively light.

The way forward is going to be about keeping on a schedule.  Pills and supplements at the same time of day, every day.  Blood draws every two weeks.  Bone and liver scans every few months.  Exercise daily, full night's sleep nightly.  At any time before in my life, the prospect of an indefinitely-extended regimen of responsibility would have been an overwhelming prospect.  But the upside of finally being treated is huge - I have never, in my life, felt the kind of energy I have now on a daily basis.  Truthfully, for the past several years, I wasn't feeling any optimism about my ability to move my life forward at all given the length and consistency of my pattern of burning out, something that I realize now was a symptom of a quiet autoimmune disease that made me susceptible to fatigue, exhaustion, stress-related illness, depression - you know, all the other things I'd individually attributed it to.

And of course, I can't ever drink again - on this measure, I do wish I'd had some more advanced warning so I could have made one last pilgrimage to The Alchemist, or at least given myself time to have a tearful goodbye with some of my more favorite craft brews, but... them's the breaks, I guess.  I really enjoy a good beer, but not as much as I'm enjoying the feeling of really wanting to do stuff and being able to move around without hurting, so the tradeoff seems more than fair.

Beyond all of the above, the next few years are going to be a game of catch-up - finishing school, getting my life and career (whatever that will be) underway to the point it would have been had it all been smooth sailing up to this point, hopefully taking the time to take advantage of opportunities that I never had the energy or inclination to pursue before while I'm still young enough that they exist.

Time's a-wastin', after all.