Monday morning blood tests revealed my creatine kinase levels have spiked. Tuesday, May 18, my throat ached and swelled. By Thursday evening, congestion made breathing through my nose difficult. Friday morning, I could feel my immune system ripping apart my muscles and inflaming and stretching my skin. I told my sister I felt like I had the flu after being beaten up by a football defensive end.
Fortunately, I never had a cough or any remote respiratory impairment, so whatever this is, it is likely not COVID-19. And my typing this on a Sunday afternoon is proof enough that I am on the road to recovery.
What concerns me, however, are my muscle aches. Though the pain is not debilitating, it is sore—the same way one feels a day after an intense workout. In conjunction with my elevated creatine kinase levels, this means my immune system for the first time in 2 years is back to attacking my muscles—even while taking drugs to suppress it.
It can hardly be coincidence this is happening while I am ill. Though I need to confirm with a doctor, I suspect my suppressed immune system had been struggling to rid of the infection and geared into hyperdrive to destroy whatever virus inhabits my body.
These events come as a setback after more than a year in a drug trial. I messaged the dermatologist running the trial about what has been going on. I can only hope this dermatomyositis flareup soon extinguishes itself, and that the new drugs have not failed.
Autoimmune diseases can leave sufferers feeling alone and vulnerable. To our friends, we sound like strangers spitting out words they have never heard. We try to get someone, anyone, to understand that every day, we walk a tightrope made taut by modern medicine over a 10,000-foot drop to oblivion. If and when we fall, doctors cast us lifelines. Sometimes, we lose our grip. Sometimes, the lines break.
The rarer the condition, the more anxious and more depressed its leaves the afflicted. We visit doctor after doctor and wander the world in search of charlatans who have answers to the darkest of questions: Why me? What caused this? Did I do this to myself?
Seeing talented sports stars, actors, singers, and musicians flourish despite their conditions inspires those of us battling autoimmunity.
We have to remind ourselves daily that diagnosis is not the end. Most autoimmune diseases have been thoroughly studied. Though few, if any have cures, many have multiple treatment options. Most treatments are safer and more effective than they have ever been. And in the twenty-first century, many autoimmune patients live long, productive, even happy lives.
Staying on top of their game: sports stars with autoimmune diseases
Pro golfer Kristy McPherson was told by many doctors she would have to give up sports after being diagnosed with juvenile idiopathic arthritis at age 11. A rheumatologist at the Medical University of South Carolina told her that even with the disease, she could still do whatever she wanted.
Pro golfer Kristy McPherson refused to give up sports after her doctors diagnosed her with juvenile rheumatoid arthritis and told her she would never be able to run or jump competitively again. [Chris McGrath | Getty Images]
“All I needed was that one doctor to tell me that,” McPherson said. “That’s when I went back to playing sports.”
Months after becoming the world’s top tennis player, Caroline Wozniacki was diagnosed with rheumatoid arthritis.
She explained: “You start asking yourself questions: What does this mean? Does it mean I can’t get in as great of shape as I was before?”
Though she initially struggled, she bounced back to win the 2018 China Open.
“[Wining in Being] meant so much to me,” Wozniacki added. “It also gave me the belief that nothing is going to set me back. I’m going to work with this and this is how it is, and I can do anything.”
Both McPherson and Wozniacki now speak with young people about living with the rheumatoid arthritis.
Having an autoimmune disease doesn’t mean you can’t be beautiful
Kim Kardashian’s makeup-free selfies have encouraged many others to acknowledge their psoriasis and helped me feel better about flare-ups on my face.
“The disease can cross all socioeconomic lines,” Randy Beranek, CEO of the National Psoriasis Foundation, told The Atlantic. “If someone as famous and visible as Kim Kardashian can have it, it doesn’t make your disease feel so isolating.”
Last December, Kardashian even polled her fans on Twitter about effective medications.
Celebrated singers and musicians have autoimmune diseases
Actress and pop singer Selena Gomez a few years ago revealed she has lupus and in 2017 informed her fans on Instagram about her life-saving kidney transplant.
Few 24-year-olds probably understand what it’s like to have their bodies threaten their lives. To deal with the psychological pain, Gomez checked into Arizona’s Meadows rehab facility. She then faced backlash from fans and from media, who thought she was being treated for drugs or alcohol addiction.
Like Kardashian, revealing her condition to the world opened up mainstream and social media discussions about lupus: What is it? What does it mean for Gomez’s career and for others living with the disease?
Singer Toni Braxton also has refused to let lupus derail her career or her commitment to her family. One of the most decorated pop singers in American history, she has won seven Grammy Awards, nine Billboard Music Awards, seven American Music Awards, among numerous other accolades.
As a guitarist, I find Shawn Lane’s story most inspiring. Widely regarded as one of the greatest guitar players ever, Lane struggled since he was twelve with psoriatic arthritis. His condition not only caused itchy, painful rashes on his skin, but also stiffness in his joints, making it increasingly difficult to play his instruments.
Guitar player Shawn Lane played his entire career in spite of stiffening joints and skin rashes from psoriatic arthritis. His playing and compositions continue to inspire guitarists even after his untimely death in 2003.
Worse, Lane developed Cushing’s syndrome from his long-term use of cortisone to treat his skin and joints. He had to stop playing guitar and died a few years later after developing breathing problems.
Nonetheless, as readers can find more about here, Shawn Lane lived as full of a life as possible, playing in spite of the pain, and producing some of the most beautiful instrumental music of the last thirty years.
Laurence Olivier and Maria Callas: living with and dying from dermatomyositis
As I mentioned here, heralded actor Laurence Olivier and legendary soprano Maria Callas both battled and eventually died from dermatomyositis. That never stopped either of them from prolific careers.
Olivier has been called the most definitive actor of the twentieth century. He lived to be 82, dominating the British stage and starring in more than fifty films.
Callas had a vocal range just below three octaves and is still one of the best known and influential opera singers of the twentieth century. Despite her disease affecting her voice in her later years, she still performed. Forty-plus years after her death, Callas’s name still sells albums.
I hate the dentist. The mere thought of the pick scraping my teeth and gums drives me wild like a dog forced to endure a high-pitched whistle. The memories of the drill hitting a nerve the dentist thought she numbed causes me to cringe like a child being force-fed once-boiled, week-old brussel sprouts. If ever you put a committee into a room and asked them to think of ways to torture human beings, most would suggest the dentist’s chair–picks, drills, giant needles into the cheek and all.
Taking medications to keep my immune system in check has forced me to return to the dentist at least twice in the next month.
Because of this, I have spent much of my adult life taking care of my teeth, brushing daily, twice yearly cleanings. The best I could ask of myself is to floss more frequently. Between my hygiene and being subjected to numerous municipal fluoride experiments, my teeth have been largely impervious. I’ve had maybe six cavities my entire life–most of which were during my teen years, when my parents paid the dental bills and a toothbrush was as foreign to me as bathroom cleaner.
I returned to the dentist Thursday only to find out I have five cavities, including a nasty deep one that will require removing the tooth’s root. My dentist was so surprised by the decay he asked me: Had I changed my diet? Was I taking some crazy medication? Had I stopped brushing in protest of the Trump administration?
Immunosuppressants and oral health
Six months of immunosuppressants have desiccated my mouth like border patrol to the Arizona deserts and leaving my teeth to the mercy of the bacterial cartels.
Dental plaque (a bacterial mass) loves to hide in the canyons, ridges, and crevasses in between my teeth. A healthy mouth can better fight them. The immunocompromised, which now includes me, have to stay vigilant.
I’m now paying the price for those times I was too tired or too lazy or too busy to floss. The cost: a root canal, a crown, and five fillings. This amounts to over $2,000 in dental work and four hours in the dental torture chamber.
Autoimmune diseases and oral health
Interestingly, this may not be my fault alone. According to Colgate, dermatomyositis itself could be the cause. Their one-pager on autoimmune diseases and oral health points out how these diseases can cause trouble with eating, swallowing, and dry out the mouth and lead to more cavities.
Dermatomyositis dries out the mouth and causes trouble swallowing, leading to more cavities.
That said, as my dentist and hygienist explained, the solution is better oral hygiene. Three or four cleanings per year could also help. That and spending the ides of March in the dentist’s office having my teeth repaired.
Over three weeks ago, after having issues with my methotrexate, my rheumatologist switched me to another immunosuppressant, azathioprine.
Azathioprine is the Mayo Clinic dermatomyositis expert’s drug of choice for treating dermatomyositis and polymyositis.
This drug best work without major side effects. That it starts with the letters A and Z is fitting because after azathioprine, for a largely amyopathic form of dermatomyositis, like mine, my doctors and I are out of realistic options.
Like methotrexate, scientists first developed and used azathioprine for chemotherapy. Scientists later discovered its use in suppressing the body’s formation of antibodies—proteins used by the immune system to neutralize anything that can produce disease. During the 1960s and 1970s, it became widely used for kidney and heart transplants, then later for rheumatoid arthritis.
Azathioprine works by inhibiting purine synthesis, a process in which the body produces organic compounds essential to synthesizing white blood cells. Since some of my white blood cells are attacking my skin (and to a lesser extent, my muscles), inhibiting their proliferation keeps a malfunctioning, overactive immune system like mine in check.
Interestingly, caffeine and theobromine (found in chocolate) are purines. The body specifically relies on purine synthesis to process both. That may explain my heart palpitations after one-too-many cups of coffee.
Azathioprine is finally healing my rashes
After only three weeks, my skin looks better than it has in a months, possibly since last June. The rashes have reduced and stopped itching altogether.
My scalp still itches and flakes in a manner that at times, is almost painful. My hair is also still slowly falling out. To date, the only drug to control these symptoms is prednisone in moderate doses.
I even feel stronger than I did on methotrexate and seem to build muscle more quickly.
As I wrote last week, I now need to control the chest tightness and shortness of breath. As I work up to the full dose, I think these symptoms will also go away.
The recent switch from methotrexate to azathioprine has caused breathing difficulties. Though it’s nothing life threatening, my doctors are not sure why.
For most mammals, breathing is easy. Astronaut Chris Hadfield, on Darren Aronofsky’s “One Strange Rock,” calls it the most natural thing humans do. Aronofsky (of Black Swan fame) then shows a baby inhaling minutes after being born.
Like most healthy people, for most of my life, I took breathing for granted. I swam as a child. I ran cross-country in high school. I hiked four of Colorado’s Fourteeners. I played schoolyard football.
I also took my lungs for granted. I built campfires. I smoked the occasional cigar. Nothing ever bothered them, even after one had to be deflated (then later inflated) so my orthopedic surgeon could access and fuse my spine.
Dermatomyositis and Lung Involvement
Dermatomyositis has changed all that. Without prednisone or hydroxychloroquine, I feel short of breath. My chest feels tight. Breaths become heavy, even if my lungs otherwise function.
Shortness of breath can be a symptom of dermatomyositis because the immune system attacks the chest muscles, restricting breathing. This is most likely the cause of my issues.
As Lundberg et al. write, breathing difficulties are well documented in dermatomyositis and are attributed to several causes, including the disease itself and complications like aspiration pneumonia and interstitial lung disease.
Breathing difficulties could also be the result of aspiration pneumonia: Muscle inflammation causes difficulty swallowing, sending liquids and food down the wrong pipe, eventually causing an infection.
Most concerning, dermatomyositis can lead to interstitial lung disease. In short, the immune system malfunctions as it tries to repair damage to the lungs. It scars and thickens the tissue around the air sacs, making it difficult to breathe and to get enough oxygen into the bloodstream.
Fortunately, my pulmonary function test in September came back normal. So too did my chest x-rays and high-resolution CT scans. Three weeks ago, I also had an EKG indicating my heart is very healthy.
Short-Term Possibilities: Medications
Ironically, the very drugs designed to keep my immune system at bay and help me breath can also cause lung damage. Methotrexate has been known to cause interstitial lung disease. So too have many anti-inflammatory drugs used to control autoimmune diseases, such as rituximab (Rituxan).
Azathioprine, the medication I am currently taking, can cause chest pain and increase your heart rate. Of course, those are also symptoms of dermatomyositis.
I returned to my rheumatologist earlier this week because I thought the drugs were causing the issues. He disagrees and believes my symptoms could be stress and anxiety.
Reversing the Cause and Effects of Breathing Difficulties
Two days later, after experimenting with taking the drugs at different times of day, I’m convinced we’re both wrong. I reversed the cause and effect, leading him to look at my symptoms as drug related rather than effects of the dermatomyositis itself.
I reversed the cause and effect of my breathing difficulties.
Azathioprine seems to clear up my rashes and keep my immune system from attacking my chest and shoulder muscles. But as soon as it wears off, the chest tightness and shortness of breath return. I get headaches. My heart speeds up. I become fatigued and want to head to bed.
In time, I think my doctors and I will fix this. Increasing the dose staved off the breathing difficulties all day, then they start to get better again a few hours after the drugs leave my body. Plus, switching medications is very hard on any body being attacked by its immune system.
Tapering down my corticosteroids and battling a run-of-the-mill cold while on immunosuppressants have left me exhausted this week.
Corticosteroids like prednisone are a first-line treatment for dermatomyositis and other autoimmune diseases but they can have serious side effects, especially when used over multiple months.
Side effects of corticosteroids
No one should be on moderate to high doses prednisone or any corticosteroid for forever. Long-term corticosteroid use has several side effects: cataracts, bone loss, easy bruising, muscle weakness, weight gain, high blood sugar, psychosis, infection, and heart disease. People with healthy immune systems should not use them more than a couple weeks. Not unlike heroin detoxification, anyone who does use them for more than a few weeks has to taper down to lower and lower doses until you ween yourself off them.
Interestingly, even short-term steroid use has positive temporary side effects: namely, steroid euphoria. When you take the drug, you feel happy, positive, like you can conquer the world.
You can also have surpluses of energy, functioning on five to six hours of sleep just fine. I actually started this blog last July, during one of my steroid highs.
Corticosteroids screw with my mental health
As you taper down, each subsequent smaller dose deprives you of that drug-induced happiness. Much like someone weening themselves off opiates, you become physically depressed.
For almost nine months, my doctors have been varying doses of corticosteroids, trying to ween me off, only to put me back on moderate doses when my other medications fail.
This has been a mental and physical health nightmare, causing me to feel, at times, bipolar. One month, I get 30 or 40 mg of prednisone each day. The next, I taper down to 20 mg. At one point, I was on only 12.5 mg per day. Lately, I am down to 17.5 mg, leaving me feeling depressed.
Battling a common cold while suppressing my immune system
I also had a cold this week, my second of 2019 (the last ruined my New Year and my vacation). Just the usual symptoms: nasal congestion, ear and headaches, dehydration, fatigue.
Unlike people with healthy immune systems, my body seems to slow down more when I get sick. Several of my coworkers were still functioning the whole week while infected with the same virus, while I had to take a day and a half off work. Tuesday, I did nothing but watch YouTube videos of chef knife reviews. I have not been to the gym in a week. I ate junk food trying to make myself feel better.
Positive note: Even if immunosuppressants like methotrexate, azathioprine, and hydroxychloroquine leave me down for the count when a cold strikes, prednisone is like Aleve (naproxen) or Advil (ibuprofen) on steroids (pun intended). I barely notice nasal congestion or ear aches after I take prednisone in the morning. At night, however, I struggled to breathe normally.
I feel much better today, but now I have a new concern: a new, unexplained allergic reaction.
Tuesday night, I had what my pharmacist describes as an allergic reaction to methotrexate. Within two hours of injecting it into my thigh, I developed nickel- and quarter-sized hives on my knee and elbow pits. My breath was short. My heart palpitated.
With any other patient, with any order condition, with any other drug, these symptoms would be a classic allergic reaction. Any doctor would tell you to stop taking it. When you’re dealing with autoimmune diseases, this could almost mean anything.
A classic allergic reaction is caused by the immune system’s hypersensitivity to a typically harmless substance. Treatment for such a reaction is usually a drug that mildly suppresses the immune system. For example, diphenhydramine (Benadryl) is an antihistamine that treats all sorts of mild allergy attacks and cold symptoms. It is a common ingredient in NyQuil and other over-the-counter cold medicines. Doctors use another common immune system suppressant, prednisone, to treat asthma and rheumatoid arthritis. I also take it to treat my dermatomyositis.
Methotrexate is a heavy-duty immune system suppressant. The irony of an immune system suppressant causing an allergic reaction was not lost on my pharmacist or my internist.
Such a reaction after being on the medication since October is odd, but according to my pharmacist, very possible. Also odd: shortness of breath is a symptom of untreated dermatomyositis. However, hives are not. And this is the second week I have had them after injecting myself with methotrexate.
Because I only take the drug once a week, I have already discontinued it. I made an appointment with my rheumatologist for next week. I will most likely switch to a different medication, most likely, another immune system suppressant, azathioprine.
Hydroxychloroquine is cheap, relatively safe, and controls the heart and muscle inflammation associated with dermatomyositis.
Hydroxychloroquine controls heart and muscle inflammation
This past weekend, I ran out
of hydroxychloroquine, an antimalarial drug used to treat autoimmune
diseases. By Monday, my heart started racing and palpitating. My
lungs felt constricted. Both felt like they were on fire. My throat
seemed to be closing, as though I had a piece of food stuck in it.
Doctors call these symptoms myocarditis and dysphagia, respectively. Basically, along with my skin and skeletal muscles, my immune system is attacking my heart, diaphragm, and throat muscles. Oddly enough, clinical tests show nothing. My resting heart rate is a healthy 55 beats per minute. My breathing tests were normal.
I also could hardly
concentrate. Much like when you have the flu or are weight lifting,
all you can think about is your body’s stress and pain. Much like
when you feel anxious or drink too many double-shot espressos, your
racing heart makes it tough to read and write.
I forgot all of these symptoms and have not experienced most of them since I started treatment in August. Because I mismanaged how much hydroxychloroquine I had left, they returned within 24 hours of exhausting my supply.
When refilling a prescription is worse than managing a proposal
Trying to understand refill
procedures with my local pharmacy is like trying to communicate with
project managers building the Tower of Babel.
Speaking of the ancient world,
to remedy the situation, the pharmacist sent my rheumatologist a fax
for the refill.
As a Denver Broncos fan, I know all too well the dangers of faxing in the 21st century, so I sent my rheumatologist a message through his online portal Monday. No response. I called the office Tuesday. His medical assistant’s voicemail says she will call back within 24 hours. She did not.
By Tuesday evening, I gave up
and phoned my dermatologist, who is always on top of things. Within
45 minutes, the pharmacy cleared my refill.
Treating autoimmune diseases with hydroxychloroquine
Today, after taking 200
milligrams of hydroxychloroquine last night and this morning, my body
is returning to normal. No more heart and lung issues. My throat
feels less swollen. I will not mismanage my prescription again.
First developed in 1955 for
treating malaria, hydroxychloroquine is the first-line treatment for
dermatomyositis and lupus. Compared to other immunosuppressants, it
has few side effects and is so safe pregnant women can and do take
it. Better still, it costs next to nothing; even without insurance, a
month supply is less than $25.
The only downside is long-term
use of hydroxychloroquine can be toxic to your eyes. To make sure
nothing like this happens, I take the recommended daily maximum dose
and have an ophthalmologist as part of my care team.
For all of the alleged benefits of treating dermatomyositis with Otrexup (subcutaneous methotrexate), Antares Pharma doesn’t make it easy to take. They instruct you to once weekly jam a needle in your stomach or thigh. They ship the drug in this ominous yellow bag warning you the contents are for chemotherapy. Every single injector pen comes with a set of instructions and warnings longer than most college essays. Then, they request you return the cartridges in a biohazardous waste container.
I am calling out Antares for their study claiming 98 percent of patients say Otrexup is easy to use. Is it easy to take off the safety and look at the injector pen? Sure. But stabbing oneself is never easy.
By the time you work up the courage to open an individual box holding the cartridge, you’re so paranoid about the possible damage to your liver or losing your hair that you feel like Eric in the sarin gas chamber in The Rock: “You want me to stick this into my heart? Are you fucking nuts?”
Otrexup: Four Weeks later, I still can’t Stab myself
Four weeks later and I still cannot bring myself to stab myself in the thigh. My girlfriend does it for me. Sometimes, she seems all too happy to do so. No matter how silent she stays, I can hear her evil cackle. I wonder if she is not-so-secretly a sadist.
My blood must also be tested monthly to check for live damage. This wouldn’t be too bad, except that as America’s population continues to age, every blood center in Phoenix is full of grouchy, impatient, lifeless geriatrics. Though my girlfriend will say I will fit right in.
Is Otrexup worth it? It’s too early to tell. My rashes have receded. My nose is less red (with any luck, I won’t be guiding Santa’s sleigh). I do not itch as much. I am breathing easier and getting a bit stronger, but that progress could be attributed to going back on a moderate dose of steroids.
Side note: Kudos to Antares Pharma for providing first-time Otrexup patients with a coupon for no co-pay for a year. Even with the best insurance, subcutaneous methotrexate is pricey. No, they did not pay me to say that. The coupon is available on the Otrexup website to anyone with commercial insurance.
Autoimmune patients taking methotrexate must avoid alcohol, according to most rheumatologists. So too say the makers of Otrexup (subcutaneous methotrexate). I messaged my doctor to double check: He agrees abstinence is best.
Not everyone agrees. Newer studies are changing some rheumatologists’ opinions. Many now say rheumatoid arthritis patients may consume alcohol in extreme moderation; in other words, a couple drinks a week is fine. Psoriasis patients, however, should avoid it.
So, where does that leave me? Since methotrexate is used off-label to treat dermatomyositis, all research on methotrexate and alcohol concerns psoriasis and rheumatoid arthritis patients. I can only trust my doctors and look at anecdotal evidence from other dermatomyositis patients.
Many myositis patients on online bulletin boards ignore doctors’ advice. “Live life!” they say. Others were told by their doctors a couple drinks a week is okay—just not on the same day as your injections.
One current complication for me is I am on prednisone and methotrexate. Both are hard on your liver. In fact, I can feel my liver throughout the day yelling at me. For me, until I get off the corticosteroids, alcohol is probably best avoided.
