Breathing
difficulties have made the last two weeks hell. The one symptom of
dermatomyositis that has largely been dormant for over a year has
returned—weak pectoral and diaphragm muscles. My breaths are once
again shallow. Working out is a chore. I wake up in the middle of
night feeling like my body is not getting enough air, like I’m
panicking, like a demon is sitting on my chest.
John Henry Fuseli’s The Nightmare illustrates what breathing difficulties while lying flat feel like for me.
Has stress inflamed
my condition? Have my medications stopped working? Have I simply
taken them too irregularly and messed up my progress?
Two business trips,
a late-night birthday bash, and attending to doctor appointments have
meant irregular schedules and interrupted circadian rhythms. I
compensate for sleepiness with caffeine. Too much caffeine keeps me
awake, creating a vicious sleep cycle. I struggle to motivate myself
to workout. Both exacerbate my symptoms.
Just doing a mild
abdominal workout took all my strength tonight. Typing out this note
has zapped what little energy I have left.
Chronic fatigue has made weeknights rough lately. Despite ritualistic adherence to my azathioprine and hydroxychloroquine, my autoimmune disease zaps my physical and mental strength before the day ends. The moment my right foot crosses the threshold into our condo, I want to collapse.
Eight-hour workdays leave me feeling like I spent them picking anthracite from the walls of an Appalachian coal mine. My muscles feel like they’re sagging—even though I have gained strength. I gulp ultra-caffeinated preworkout energy boosters to start and finish my days.
Sports injuries have forced me to cut back on gym sessions. Between plantar fasciitis in both feet and extensor tendonitis in my right foot, I’m surprised I can stand. And of course, my inflamed lower spine aches and causes me to slouch.
Admittedly, some of this will get better. Sports injuries can be avoided with proper stretching and icing. I have scheduled monthly massages to repair my muscles and soothe my spine. And I’m only three months into a new job—every day feels like a full load of college classes, everything a lesson.
But for those like me with an overactive immune system, feeling tired and stressed can easily escalate to feeling exhausted and overwhelmed. Since stopping steroids in August, I feel like I’m walking through life perpetually sick, minus the drippy nose and sore throat.
I sometimes feel demotivated and depressed despite being otherwise happy with myself and my life. I can’t concentrate enough to read. I don’t have much energy to write or play guitar. I’m embarrassed to admit it took two hours over two evenings to pen even this.
I finished the latest seasons of Netflix’s best shows (Lucifer, Sex Education, and Derry Girls) months ago. Amazon Originals have never held my interest. The NFL airs the least exciting games of the season on weeknights. I have no energy or focus to start my Disneyland-ride-long cue of Great Courses lectures.
Nonetheless, I’m trying to stay positive. I’m trying to meet my goals. I’m trying to reignite my passion to write. I don’t want to make life one long binge watch.
I recall all the great things that have happened since this disease began—a new condo, a new job, a new guitar, another year with my girlfriend. I even earned the next certification level for proposal management. Come to think of it, while these summer months have been a drag, I’ve actually accomplished a lot in the last 18 months. I just wonder, as I did months ago, how much of it was me, how much was the prednisone. Has chronic fatigue become my new normal?
My dermatomyositis symptoms have flared up again. My forearms are dry and scaly. A tiny bloody rash appeared on my tricep. I have pain in my chest. My breathing once again feels shallow and tight like I’m being squeezed by some desert-loving constrictor–a kingsnake, perhaps.
The darker spot toward the bottom of the photo (not the darkest ones on the right, which are moles) is a bleeding rash from my dermatomyositis. It may have been triggered by increased sunlight as the days grow longer and the clouds are few. Readers will also see the discoloration in my skin,. It has been a permanent feature since last April.
Local temperatures soar. Bodies easily sweat. The cloudless skies bathe the Sonoran landscape in blue shadows and bright reflections requiring sunglasses and white balance corrections. The sun rises so early I found myself awake at 4:45 a.m. Summer is on the Arizona horizon.
I wonder if these events are linked: Increased sun exposure causes my autoimmune condition to flare up.
Doctors will certainly think so, but the flare ups could just as easily be work stress. I had to get two proposals and a notice of intent to propose out the door within six days of each other. I feel behind. I feel exhausted. Despite taking melatonin, I woke up in the middle of the night, wondering if we forgot to upload the correct version of the project description.
What of my medicines? I am now on just 7.5 milligrams of prednisone, the lowest dose I’ve taken since last June when all this turned for the worst.
Thursday, I visit my rheumatologist. Hopefully, we can figure something out. I cannot live through the hell that was last summer: the itching, the burning, the gasps for air as I wait for lab test results and wonder if I truly am months away from death.
I remind myself I’m fighting this. I can fight this. I will fight this. I did it before. I can do it again.
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.
Last week, at my annual check-up, my internist reminded how rare dermatomyositis is. He told me he had not seen a patient with the autoimmune disease in his 30-year-plus career. That includes his stint as a pharmacist.
He also relayed my dermatologist’s reaction when he first diagnosed me: “You won’t believe what this guy you sent me has.”
Like my internist, my dermatologist has been in practice almost 30 years and has not seen more than a couple cases of dermatomyositis. That was why it took him about two months to come up with the correct diagnosis. If not for the woman with him that day, it may have taken him longer.
Interestingly, legendary opera soprano Maria Callas had dermatomyositis. So did four-time Academy Award winner Lawrence Olivier, who many regard among the twentieth-century’s greatest actors. See him in Shakespeare’s Hamlet (1948) above.
An Opinion from a Consulting Dermatologist
The day he diagnosed me in July 2018, another doctor was present. I cannot remember her name or her specialty. My internist last week explained she was some sort of a consulting physician on rare dermatological diseases.
After reviewing my case file, she examined my rashes. I told her and my dermatologist about the trouble breathing and my blood tests, which showed elevated antinuclear antibody. I mentioned my internist’s suspicion I had lupus.
She then asked if I had trouble walking up the stairs. I said no.
She again examined the rashes on my fingers, neck, and shoulders. I now know what she was looking for: Gottron’s papules and a shawl rash–both diagnostic indicators of dermatomyositis.
She then walked back toward the computer and exchanged words I could not hear with my dermatologist. Five seconds later, they approached the front of the exam chair and suggested dermatomyositis.
“What in the world is that?” I asked them.
Too Rare—Even for Dr. House
Being an insatiably curious House, MD fan with an Internet connection, I had heard of most common and obscure autoimmune diseases, from rheumatoid arthritis to sarcoidosis to Behçet’s.
Dermatomyositis is an autoimmune disease in which your immune system attacks your skin and muscles. It is among the rarest autoimmune diseases on the planet, affecting 16,000 people in the United States and as few as five in a million people worldwide. Dermatomyositis with little to no muscle involvement, such as mine, presents in only 5 percent of those cases.
Dermatomyositis makes only one appearance in all eight seasons of House: a passing suggestion from Dr. Lawrence Kutner when the team tries to figure out what killed former team member Dr. Amber Volakis (Season 4, Episode 16, “Wilson’s Heart.”). Of course, Dr. Kutner is wrong. I can’t help, however, thinking the writers missed a great opportunity for a final, correct diagnosis for another episode.
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.
Despite having dermatomyositis—my condition in which my immune system is attacking my skin and muscles—I’m still getting stronger. My skeletal muscle mass and body fat mass is better than I expected.
Despite battling dermatomyositis, my body fat mass is right where it should be and my skeletal muscle mass is above average. I’m even stronger than I was a couple months ago.
Saturday, my girlfriend and I went to the Nutrishop in Tempe to stock up on workout supplements and enter ourselves into a competition. The $1000 shopping spree goes to he or she who loses the most weight and grows their skeletal muscle.
Much to my surprise,
my numbers came back very good. My body fat mass is right where it
should be and my skeletal muscle mass is above average. In short, I’m
stronger and healthier than I sometimes feel.
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.
I have never been the strongest guy in any room. Standing 6’5”, built more like a wide receiver than a linebacker, most of my strength is in my legs. Before tearing and repairing my meniscus, I could leg press over 700 pounds.
But with a high metabolism and a sprinter’s frame, upper body strength has never been natural for me. I worked my ass off in the gym four to five days a week during my twenties just to have a body that halfway looked and felt like a man’s.
My peak strength came in summer 2016, aged 31. Since then, between work, age, and inconsistency, my strength dropped a bit. Since February, it fell off a cliff. By June, one push-up seemed impossible.
February 1, 2018
June 28, 2018
Activity
Reps / Weight (lbs.)
Reps / Weight (lbs.)
Bench Press
10 / 135
10 / 65
Bicep Curls
10 / 110
10 / 65
Lateral Deltoid Raises
12 / 30 (15 each arm)
8 / 20 (10 each arm)
One-Arm Dumbbell Row
12 / 60
10 / 45
Seated Cable Row
10 / 120
10 / 60
Push-ups
20 / 215
1 / 215
Getting Stronger Every Day
With numbers like these, I have to be careful not to give up, to compare myself to the gym rats and roid ragers. I can only be who I can be.
When I look at stronger men at the gym, I remind myself my immune system is attacking my muscles, theirs is not. Worse, most anti-autoimmune drugs also induce muscle weakness. While they do reduce muscle inflammation, their benefits are almost immediately canceled out.
But the people at my gym barely notice. Some are weaker than me. I doubt those stronger care. In fact, two have even helped spot me while I embarrass myself at the bench press. They even offered sympathy for my disease.
Since June, I’ve made progress. I workout four to five days a week. I eat a high-protein, creatine-supplemented diet. I’ve gained strength in several muscle groups. Compared to my numbers in the right column above, I can do more reps with more weight.
June 28, 2018
October 15, 2018
Activity
Reps / Weight (lbs.)
Reps / Weight (lbs.)
Bench Press
10 / 65
10 / 75
Bicep Curls
10 / 65
10 / 85
Lateral Deltoid Raises
8 / 20 (10 each arm)
12 / 20 (10 each arm)
One-Arm Dumbbell Row
10 / 45
12 / 55
Seated Cable Row
10 / 60
10 / 90
Push-ups
1 / 215
5 / 215
Those five push-ups are sloppy, to say the least, but for now, it’s progress.
My nose glows red with a dermatomyositis rash like the mythical reindeer of childhood Christmases past. The moral of that story—at least the CBS claymation version—is that what seems a flaw, what makes one different, what subjects one to ridicule, may be a blessing in disguise. But humans are not reindeer. No bearded, red-suited legend is going to ask me to guide his sleigh come some unforeseen foggy Christmas Eve.
We had a web conference with video at work today. It was all too obvious to me with my nose glowing on the screen, I am not who I was. I don’t feel attractive. I don’t feel positive. I don’t want my girlfriend to touch me. I barely feel human some days.
Doctors tell me in time my hydroxychloroquine will help. It has helped a little, but not enough, not fast enough.
Still, I wait for true relief, for what after six months seems like waiting for a miracle. Still, I taper down my steroids. Still, my nose gets redder. The skin on my jaw itches so much it wakes me in the night.
Am I supposed to find meaning in a red nose? Inspiration? Am I supposed to thank God or curse nature? Am I supposed to search for some metaphorical St. Nick for some purpose in this suffering?
As the philosopher Walter Kaufmann, paraphrasing the philosopher Frederich Nietzsche, observes, nature is too well designed to lack purpose but too ill designed to demonstrate intelligence.
Yet, as intelligent, conscious beings, we think; we feel. Aware of our own fragility in the face of near constant chaos, we agonize; we suffer. Aware of our own imperfections, we criticize; we despair. We seek symmetry, predictability, stability, security, order.
So what do we do with our imperfections? Can we fix them? Should we fix them? Or do we embrace them, stare nature in the face, and tell ourselves we are more than our faults?
I may not have a red nose forever. But right now, looking at myself in a photo or a mirror hurts. I tell myself these rashes are not who I am. But right now, I see only who I used to be. I tell nature I have had enough.
