My Vicious Headache and Giant Cell Arteritis

My doctor stared at his computer, seeking some reason why the inflammation markers in my blood continued to rise. In January, I got a relentless, vicious headache that never went away. Excedrin barely took off the edge. It was now May, and he was worried. "I don't know what's going on with you," he said.

The headache began after an overlong bout of editing at the computer, so I attributed it to work. I had fiery neck pain, too, but it wasn't the usual musculoskeletal stiffness I knew how to deal with. I could hardly turn my head. The headache drilled what felt like a hole in the back of my skull. It hurt to put my head on a pillow. Pain ricocheted from my feet to my head when I walked on the pavement.

Soon, a cascade of seemingly unrelated symptoms followed. I was highly fatigued. I found myself lying down if I didn't have to work. My range of motion diminished to a fraction of normal, as though an invisible force field prevented me from moving freely by bringing on pain. I leaned on furniture to help me up and down from the floor, something I could previously do with ease.

Then came tenderness in my scalp and the skin over my temple. I awoke with my lower jaw jutting ahead of my upper like an open drawer so my teeth didn't line up when I closed my mouth. Soon I had trouble chewing and swallowing, producing a sour, painful sensation. 

It was getting harder and harder to get through the day. Walking to the grocery store would wear me out. Teaching, working and socializing became an ordeal. All I wanted to do was lie down. Before this, nearly full bottles of painkillers would expire before I used them. Now I bought the large size Excedrin. It helped me get through the day, but barely.

I knew this wasn't a migraine because I've experienced those, and this was different. I also knew it wasn't TMJ because I don't tend to grit my teeth. When I initially saw my primary care physician in March, I asked for a prescription for physical therapy, erroneously assuming the cause was musculoskeletal. That turned into a nightmare because the aggressive manual therapy caused more pain.

The therapists thought I was overly sensitive, even when one discovered a thumb-sized bruise on my neck from a previous PT session. I went back to my doctor. The headache, painful movement and fatigue worried me. He suggested it might be polymyalgia rheumatica, which causes hip and shoulder pain.

That diagnosis would make a lot of sense later on because it's a precursor to my eventual diagnosis of Giant Cell Arteritis (GCA), but when I looked it up, the symptoms didn't seem to apply to me.

I had blood tests every few weeks. My inflammatory markers were elevated and getting worse every time I came back. My doctor ordered every test in the world, but nothing was definitive. In May, he said, "I don't know what's going on with you. Go see the rheumatologist," he said. He was concerned enough to contact her directly to squeeze me in as soon as possible.

A few days later, I sat in the rheumatologist's office. "I have all these weird symptoms, so I'll just tell you everything." I quickly ticked them off. She listened attentively. Then, out of the blue, she asked:

"Have you lost weight?"

"Yes. A little bit," I replied. What does this have to do with anything? I thought.

"Have you had a fever?" she continued.

"Well, for me, yes. I run low, but it's been higher than usual." How did she know? I wondered.

And then, finally: "How is your vision?"

Funny, she should ask. "This morning I was having trouble focusing with my right eye," I replied.

The rheumatologist looked at me with a "Gotcha" expression for a second before she spoke. Then she said: "I know exactly what you have, and you're not going to like it," she announced. "You have Giant Cell Arteritis (also known as Temporal Arteritis), and it can lead to blindness."

My world stood still for a breathless moment. 

"You need to be on prednisone today," she punched the last word. "See your ophthalmologist immediately to see if there's been any damage to your optic nerve. Then you need a CT scan and a temporal artery biopsy. You also need to be on Fosamax because prednisone can lead to bone loss."

I was stunned. Ordinarily, I would avoid steroids, but if it's between that and blindness, I'll take the prednisone. I raced from the doctor's office to the pharmacy to pick up the prednisone and started making emergency appointments with specialists for all the tests.

Giant cell arteritis (GCA) is an inflammation of the lining of the arteries. When viewed under a microscope, some white blood cells clump together to look extra-large, thus the name. 

This swelling can reduce blood flow – which contains oxygen and vital nutrients – to the tissues, including the optic nerve, so you could go blind if the inflammation is not reduced in time. Once that happens, regaining sight is unlikely.

The standard treatment blasts you with a high prednisone dose to knock out the inflammation. According to Medscape, GCA affects approximately .5-27 cases per hundred thousand people aged 50 years or older in the US. It is so rare that few people, including many doctors, know about it. 

Untreated patients may suffer a heart attack, stroke, aortic aneurysm and blindness. Scientists do not understand what causes it, although age and genetics might be factors.

Enter prednisone. I took one pill (a third of the full 60 mg dose) the night I was diagnosed and was up all night with insomnia. That insomnia would last for months. If lucky enough to fall asleep, I would wake up at 1 or 3 a.m. Sometimes I could fall back asleep, but all too often, I couldn't and struggled through the day like a zombie. 

It felt like constant electricity buzzed through my veins. I went from lying inert to being a whirlwind of hyperactivity on an out-of-control seesaw. I was wired and tired at the same time. Other side effects would follow suit: prednisone made my skin reptilian. My hair fell out in alarming clumps. I got dizzy. I had blurry vision. Moonface (a swelling of the face) occurred.

Everyone on prednisone at high doses for a long time gets awful symptoms – and loathes them. But all of this was better than going blind. I just didn't want to be on prednisone a second longer than I had to.

Prednisone was not the only option – there was another drug, Actemra, which was not a corticosteroid but a biologic that would reduce the inflammation. The rheumatologist told me that if I took prednisone for a long time, I was guaranteed bad side effects (among the awful possibilities: cataracts, glaucoma, diabetes and high cholesterol). 

Actemra had its own set of black box warnings (perforated intestine, anyone?). Still, it was generally considered a better option if you could take it. I had to be tested for cancer and hepatitis B before I qualified for Actemra. But even with Actemra, you can't stop taking prednisone without risking bringing back your symptoms. 

"Tapering" (gradually reducing the dosage of) prednisone is the trickiest part. If patients taper too quickly, symptoms can return, and they must increase the prednisone dosage again and start over. This is a scenario I want to avoid. My rheumatologist said it would take a few years to return to normal.

Giant Cell Arteritis has been a roller coaster. Several months in, I learned not to make social plans too far in advance because I never know how I will feel daily. I could be doing all right and suddenly feel fatigued and need to cancel plans, inconveniencing friends. I would work as much as I could and then collapse.

The origins of GCA are not well understood. Still, it mostly happens to white individuals in their 40s who see the doctor with severe headaches and other symptoms of GCA (only to be told they are too young to have it, or worse, misdiagnosed because the doctor is unaware of this disease).

Some of these people lose their vision. But GCA can also happen to men and African Americans. It is considered a medical emergency because of the risk of vision loss. GCA can be confused with other illnesses, but if you have these symptoms (described by the Mayo Clinic), see a good rheumatologist right away:

• Persistent, severe head pain, usually in your temple area
• Scalp tenderness
• Jaw pain when you chew or open your mouth wide
• Fever
• Fatigue
• Unintended weight loss
• Vision loss or double vision, particularly in people who also have jaw pain
• Sudden, permanent loss of vision in one eye