Dermatomyositis makes breathing difficult

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

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.

Depression: Don’t Be Afraid to Talk About It

An estimated 16.1 million Americans struggle with depression, according to the Anxiety and Depression Association of America. Few of those want to admit it. Fewer still want to talk about it.

Trying to explain the anguish and hopelessness inside you to those who have never battled mental illness is like a woman trying to explain the pain of childbirth to a man.

Sometimes people sense those overwhelming feelings of sadness, especially if you seem gloomy or unusually slow or uninterested in much of anything. But for many, such obvious signs are buried behind much more subtle symptoms. Some overeat. Some don’t eat at all. Many lose sleep. Others are always tired and sleep too much. More recently, psychologists have begun recognizing rage, anger, and irritability as symptoms of depression–especially in men, but also in women.

Trying to explain the anguish and hopelessness inside you to those who have never battled mental illness is like a woman trying to explain the pain of childbirth to a man.

Worse still, many turn to alcohol, drugs, work, and other addictions to bury their depression, anxiety, ADHD, or other mental disorders. When addictions cease to smother the pain, suicide becomes the last resort to make it all go away.

Depression and the American rock and metal scene

Among the most famous is Nirvana legend Kurt Cobain. A chronic drug and alcohol user since age 13, in 1986, he started using heroine. By 1990, recreation became addiction. Unlike softer drugs, repeated heroin use changes the brain’s structure and physiology, creating imbalances that are not easily reversed. Cobain’s brain became dependent on the drug, then developed a tolerance that made it almost impossible to ever deliver enough of the drug into his body to stay happy and alert. Eventually, overcome with mental, physical, and chemical depression, he shot himself.

Chris Cornell’s voice on Temple of the Dog’s “Hunger Strike” shows the pain he experienced most of his career that eventually killed him. The song is a favorite of mine and represents the best of grunge and has helped me get through some rough times.

Cobain is hardly alone among rockers and metalheads. Soundgarden and Audioslave frontman Chris Cornell struggled with depression most of his life until hanging himself in a Detroit hotel room at age 52.

Linkin Park’s Chester Bennington hung himself only months after Cornell, on what would have been Cornell’s 53rd birthday. Stone Temple Pilot’s Scott Weiland battled substance abuse for much of his career and a year and a half before Cornell died after overdosing on cocaine, ethanol, and methylenedioxyamphetamine (MDA).

At the core of these singers’ struggles, as a psychologist once taught me, are feelings of hopelessness. Little brings you happiness. Nothing seems to change. You feel trapped, perhaps useless, unwanted, unloved–even if you are adored by millions.

What causes us to feel hopeless?

Exactly what causes those feelings depends on the individual, but for many, myself included, we feel as though we are running from our pasts, from memories we long ago repressed, from demons we thought we exorcised on a therapist’s couch.

Some, like Cobain, have a family history of depression, suicide, and substance abuse, likely indicating an underlying physiological problem with the brain. For others, like Weiland, are simply unable to cope with terrible traumas and unprocessed memories of rape or abuse. Others still suffer from both.

Many have no idea most depressed people are in so much pain– even singers, musicians, writers, and artists whose lyrics, music, themes, and styles can be dramatic, morose, and hint at, if not signal mental anguish. Often, nobody knows until it’s too late. Our culture needs to change that.

Finding inspiration in those who have struggled

The past several months, I was inspired, though not surprised, to find these themes not just pop up, but humbly honored at rock and metal concerts. Dexter Holland of Offspring paid tribute to friends he lost. From Ashes to New dedicated multiple songs to Chester Bennington, citing him as a major influence and calling him a friend. Breaking Benjamin similarly called out struggles with suicide and depression. Five Finger Death Punch discussed substance abuse. Their lead vocalist, Ivan Moody, shared his own struggles and recent decision to stay sober.

Disturbed truly went out of their way, airing the numbers for mental health hotlines as they paid tribute to all that rock and metal have lost over the past few years to suicide or substance abuse as they played their ballad “A Reason to Fight.” While some have been critical of the track, calling it too much of a departure from Disturbed’s iconic twenty-first-century metal sound, I love the song. Hearing it live with candles and a supportive audience that clearly had many battling their own beasts inside.

Depression: You should talk about it. With the help of friends, family, therapy, medications, support groups, and hotlines, you can win the battle. I am proof of that.

In Disturbed’s words:

Don't let it take your soul, 
Look at me, take control
We're going to fight this war
This is nothing worth dying for

Are you ready to begin
This is a battle that we are gonna win


Being reminded how rare dermatomyositis is

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.