Whooops! The rug is ripped from under my feet...again

If you need to catch up…In 1995 I moved from Seattle suburbs to “my little cabin in the woods” in Hungry Horse, Montana while recovering from injuries as a passenger in a car wreck. I had lost my paralegal job. It took awhile to learn to walk again. I was now able to dress without help, was battling PTSD, some memory loss from two blows to the head and back pain from spinal injury.

“Life seems to operate in cycles. I know this empirically through the cyclical patterns of my own life path. Patterns of learning, self-awareness and personal evolution often appear to be dictated by the return of certain challenges that call me to work on an aspect of myself that needs awareness.”  By Simon Esler

At 5 a.m. the alarm buzzed.  There was a glimmer of light in the east. I hadn’t packed a suitcase because I expected to be home that evening. I put a thermos of coffee, protein bars, dog treats and jugs of water in the car, along with an audio book I had borrowed from the library. Radio reception was spotty to non-existent on the way. The only stops would be potty breaks at rest stops for the three of us.

I dressed quickly and went to the closet for the shoes I planned to wear.  One was missing. Gypsy stood at the bedroom door, my missing shoe in her mouth. It was a game she liked to play…keep-away.  She had great fun dodging away just as I was about to grab the shoe. Her four legs were faster than my two. Her reflexes were quicker. Frustrated, I grabbed a dog treat.

“Trade for a cookie?” I asked.

She stepped forward, dropped the shoe at my feet and took the cookie.

I loaded the dogs into the car, Gypsy in the front seat and Veronica in the back. We were finally on our way.

The drive from Hungry Horse to Spokane took about five hours, mostly because of the time it took to drive around Flathead Lake to I-90. There were no shortcuts. It was almost 240 miles one way that included two mountain passes, the steepest being Lookout Pass on the Montana-Idaho border that crossed the continental divide. The grades were long and deep with a lot of pullouts for runaway eighteen wheelers. The second, Fourth of July pass, was not steep but the multiple S-curves slowed down the drive. It was an easy drive with a good traffic flow. I popped up the sunroof so I could smell the forest and enjoy the warm summer breeze. I got into the rhythm of the S-curves and my mind wandered away from the audio book.

This wasn’t a pleasure trip. I had an appointment with a specialist at Rocky Mountain Medical Center in Spokane. Dr. Sharma was concerned about my liver’s high enzyme levels. She had suspected lupus and sent me to Missoula to a specialist who ruled it out. Now I was on my way to a third specialist in as many months to figure out the cause.

My appointment was at 12:30 p.m. and I arrived with time to spare. I found my way to Dr. Harris’ office. The woman at the check-in desk gave me directions to the lab for some blood work. When I returned, it was a short wait before being shown into a small exam room.

Dr. Harris walked into the room, his head bent, reading my chart. He was tall, young and pleasant. He had a nice smile. 

“Hi McKenzie, I’m Dr. Harris,” he said as he shook my hand. “Dr. Sharma sent you here because she is concerned about your elevated liver enzymes,” he said. “I see that Lupus has been ruled out.”

“Yes,” I said. “And I am exhausted all the time.”

“I am going to run you through a few tests,” he said. “They won’t hurt.  The first one is to test your tear production. It will feel a little strange and it’s important that you don’t blink.”

He dabbed my eye with some kind of tool, looked at it and frowned.

“The next is a saliva test,” he said. Again he frowned.

Then he grabbed my left hand and holding each finger, stared at each fingertip.  He did the same with the right one.

“Do you have trouble swallowing crackers, bread and potatoes?” he asked.

“I never thought about it, but yes. I usually have to drink a lot of water with those foods.”

“That’s not surprising,” said Dr. Harris. “Your blood work shows a high level of antinuclear antibodies. Antibodies are proteins produced by white blood cells and defend against bacteria, viruses, toxins, etc. Most people have antinuclear antibodies, but in small amounts. Your level, or titer as we call it, is very high, over 1:160. Your eyes are very dry, with very little tearing. I am giving you a prescription for a lubricant for your eyes.  It’s important you use it frequently so your eyes don’t dry out and you don’t go blind. In the future, it may be necessary to insert plugs in your tear drains to keep as much fluid in your eyes as possible. Sometimes that does not work and you might need to have the tear drains in each eye cauterized. Also, you will need to use some ointment, a saliva replacement, to keep from losing your teeth and to help you swallow. “

“OK,” I said, nodding.

“With your symptoms and the results of the antinuclear antibody test, it’s pretty clear that you have scleroderma, CREST with Sjogrens syndrome,” Dr. Harris said. “Scleroderma is a rare autoimmune disease.”

He lifted my left hand and pointed to my fingertips. 

“See those little red dots on your fingertips?  That’s caused by widened blood vessels and is called Telangiectasias. CREST is an acronym for Calcinosis – lumps of calcium in the skin, Raynaud’s phenomenon, Esophageal dysfunction, Sclerodactyly and Telangiectasias.”

“I’ve had Raynaud’s phenomenon for a few years. It came on suddenly a few weeks after surgery and a blood transfusion,” I said. “But what do all those other things mean?”

Esophageal dysfunction, or problems swallowing is caused by scarring in the esophagus,” he explained. “Scleroderma means literally scarring of the skin. Sclerodactyly is a tightness and thickening of the skin on your fingers or toes.  Eventually you may have a hard time bending your fingers. Scar tissue will build up on your organs, your lungs.”

I was stunned. I had not expected this. I had never heard of scleroderma.

“How did I get this?” I asked. “It doesn’t run in my family. I have never heard of it.”

“It could have been from the blood transfusion,” said Dr. Harris.

“Is there a cure?” I asked.

“No. It’s rare and there is not a lot of research funding for diseases that few people have. There are things we can do, though.  One of the treatments is methotrexate. You will need to learn to give yourself the injections. Methotrexate suppresses the immune system. There are side effects, but we can slow down the disease by keeping your immune system from working. I am going to give you a pamphlet to read about Sjogrens. That is what causes the dry mouth and dry eyes.”

I left the doctor’s office, my head swimming. I felt numb. I don’t remember getting into my car. I found my way to I-90. Waiting at the stoplight, I read the signs at the entrances to the freeway. I could go left, back to Hungry Horse, or I could take the on-ramp to the right and drive to Seattle, where my kids and my parents were. I went right.

I had a lot to think about.