The last thing Dr. Yoda said to me as he was going out of the country was, “behave yourself.” Seriously, do people not think I like a challenge? I don’t like to hear stats anymore, “only 1% of people have this complication or <1% of people will develop this disorder.” Had he not read the statistics on HLH? My body was up for the challenge. Three days later I was admitted to the hospital for 21 days. That’ll teach you Dr. Yoda.
It was the same story to a slightly more intense beat. FEVER, PAIN. The pain originated in my right hip, but now it was progressing to my left. It scared the bejebers (this is my polite word of choice) out of me. Was HLH and chemo going to take my ability to walk? The pain was 8/10 again (see robust and somewhat inappropriate). My fever wasn’t too worse, but by all means was not better either. Here’s the deal, except for Dr. Yoda and his Jedi master, none of the other hematologists believed that I had HLH. They had not agreed with Dr. Yoda treating me and that made hospitalizations challenging to say the least. So they looked high and low for another diagnosis that “fit” my clinical presentation. Excuse my language, but it was a pissing contest.
Gout- fever from inflammatory process. Hip pain from gout crystals. Problem- what about all the other stuff? As well as unlikelihood of gout being in the hips without abnormal labs or higher levels of uric acid in the hip joint.
Strep vividans– fever from infection. Possible sepsis from infection causing pain. Once, again does not address the core problem that brought me to hospitalization. And cultures were all negative again.
Stills Disease- Adult-onset Still’s disease (AOSD) is a form of Still’s disease, a rare systemic autoinflammatory disease characterized by the classic triad of persistent high spiking fevers, joint pain, and a distinctive salmon-colored bumpy rash. The disease is considered a diagnosis of exclusion. Possibly. But I had no rash, no rheumatologic markers (less reliable) and up til the start of chemo no joint pain, no lymphadenopathy (no swollen lymph nodes).
The effusions had worsened around my hip joints and they were very concerned about septic arthritis (infection of the joint)- BUT you could move my limbs all you wanted to, it wasn’t painful with passive ROM (range of motion) at all- this would be very unusual for a septic joint or I was super woman.
In some respects, these findings were complicated by the fact that I had a clear chest CT in the May hospitalization. Seeming that the HLH nodules that we were concerned about, had vanished; however, all of my current chest xrays showed nodules- they were written off as granulomas (essentially scar tissue). The MRI’s of my hips showed pockets of necrosis and multiple effusions possibly abscesses. None of it made complete sense and according to the attendings, I did not have HLH because I was not sick enough, and according to the lab tests- I didn’t have anything else.
The above part of the scenario took about 2 weeks to muddle through. Meanwhile, I was unable to do anything but stand and pivot to a bedside commode, I had lost sensation in my right leg and my fevers were starting to worsen. Anakinra (an immunosuppressive drug used to treat rheumatologic disorders and widespread inflammation) was started. And low and behold, my condition worsened. I vaguely remember a very late night calling my husband. My fever was spiking to 102 and above, the pain was requiring regular dilaudid to control and essentially I was miserable. It was the worst I had been.
The next morning- a test came back mildly positive. The blastomycosis marker showed a mildly positive reaction.
In the beginning, there were 3 differentials: infection, cancer and autoimmune (Now, when did all this start?). 6 months later there were still 3 differentials. A fungal culture never did grow a fungus (did I tell you they FORGOT to send the spleen for culture?), my fungal markers were only mildly positive (I would hate to treat the poor person who has a high marker)- but was a fungus to blame? I was scheduled for another broncoscopy (yay) and started on Amphotericin (ampho-terrible- an antifungal renowned for it’s awful side effects). And out of spite, I (we – my husband played a big role in this) finally convinced an attending to order a chest CT, which she assured us would be negative (because I don’t have HLH ya know).
Just in case you’ve forgotten, I am a nurse practitioner by trade. I have been in nursing 18 (gulp) years. I have actually given Amphotericin before- and it scared the bejebers out of me to receive it. Bring on the ativan and dilaudid (and tylenol and benadryl), let’s get this bad boy under way. And you know what? I started to get better. The fever lingered, but slowly, I could walk again. I could go to the bathroom by myself- seriously, it’s the small things. Talk of sending me to a rehab hospital dissipated, talk of sending me home finally started. And we waited for the CT results.
This would be my mode of transportation for a while.