Category: dermatomyositis

Dermatomyositis makes breathing difficult

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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.

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.
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.

Long-Term Possibilities: Interstitial Lung Disease

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.

Corticosteroids, colds, and mental health

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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.
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.

Being reminded how rare dermatomyositis is

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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.

https://www.youtube.com/watch?v=5ks-NbCHUns
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.


Allergic reaction to methotrexate

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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.

Skeletal muscle mass—still getting stronger, despite dermatomyositis

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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.

body mass index body fat mass skeletal muscle mass dermatomyositis
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.

I don’t need to lose weight, but I am trying to gain muscle. I already work out five days a week, so why not enter? Even if I don’t win, it costs me nothing. Plus, these creatine and stimulant supplements essential for fighting my disease are expensive.

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 staves off dermatomyositis symptoms

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Hydroxychloroquine (Plaquenil) staves off my dermatomyositis symptoms far better than I thought.

Hydroxychloroquine is cheap, relatively safe, and controls the heart and muscle inflammation associated with dermatomyositis.
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.

Taking Otrexup: Stabbing Yourself to Save Your Life

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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.

Autoimmunity, methotrexate, and alcohol

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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.

Time to add methotrexate

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My rheumatologist and dermatologist agree with the Mayo Clinic dermatomyositis guru: After ten weeks on hydroxychloroquine, with few results, I need to add methotrexate and folic acid to my regimen of medications and supplements.

Otrexup: subcutaneous methotrexate
I’ll now be injecting myself with methotrexate in the thigh once a week with a cartridge that looks like a Soviet torture device. And I’ll be taking one milligram of folic acid every day—2.5 times the amount pregnant women take to stave off birth defects.

Methotrexate suppresses the body’s immune system. Developed in the 1940s as a chemotherapy agent to treat cancer, in low doses, studies and clinics have shown it effectively treats many autoimmune disorders. It’s a first-line treatment for rheumatoid arthritis and a second-line treatment for psoriasis. (Interestingly, it also used to induce abortions.) The drug is affordable, generally safe, and well tolerated by autoimmune patients.

Only time will tell whether or not the methotrexate relieves any of my rashes and itching. Medical journals show mixed evidence of its efficacy. In this dermatology study, only 1 of 4 patients with amyopathic dermatomyositis, in this one, only 2 of 3. However, this 1998 study showed it helped all 13 patients, whether they had muscle involvement or not. This 2011 study also found methotrexate reduced skin lesions in 8 of 11 patients.

Risks of Methotrexate

Liver toxicity is a risk of taking methotrexate. Though most of the above studies tout few adverse effects, one cancer-dermatomyositis patient had hair loss. These doctors observed psoriasis and dermatomyositis patients are at higher risk to liver damage than those with rheumatoid arthritis.

That said, methotrexate side effects are much more common in cancer patients. They take much higher doses of it than autoimmune patients. Brain damage is a real possibility for them.
 
I talked to all three of my doctors about side effects and risks. All three say the medication is generally safe and not to worry. Issues listed online and discussed in medical journals mostly affect autoimmune patients also battling obesity, drug or alcohol misuse or abuse, or cancer.
 
I will need regular blood tests to check for early signs of liver toxicity before the drug causes irreparable damage.

 

Gym Results: Evidence of Dermatomyositis

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A few weeks ago, my gym results were the only way I knew dermatomyositis caused my muscles to progressively weaken. Between February and late June, I lost almost half my upper body strength.

Muscle Loss of Strength (Percent)
Triceps 40.9
Biceps 45.8
Deltoids 33.3
Trapezius 45.8
Pectorals 52

My Gym Results

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.