My younger sister, Karen, and I are three years apart. We typically see each other just two or three times a year, because Karen lives in my home state of Minnesota and I live in Florida — more than 1,300 miles away.
While there’s a great distance between us, Karen and I have always been close.
We share many common interests — traveling, gardening, dogs, cooking, outdoor activities, history and our faith. We have often talked of spending more time together when we retire.
Just a few years ago, we began seeing more of each other after Karen and her husband, John, starting wintering outside Thomasville, Georgia. They wanted to get away from Minnesota’s snow and cold, and they needed a warm climate where they could train their dogs to compete in AKC Retriever Hunt Tests.
(Courtesy of Diane Kortus)
Thomasville, which is just north of Tallahassee, is a four-hour drive from my home in Land O’ Lakes — making it close enough for weekend visits.
The rest of the year, Karen is back in Minnesota.
She has a fulfilling and happy life there with John, their sons, three grandchildren and, of course, their beautiful dogs.
As we all know, though, unexpected things can happen that turn your life upside down.
In my sister’s case, that happened three years ago when she began having trouble with her vision and spatial awareness. When she was dressing, for example, she often put her tops on backwards. When she drove, she found it increasingly difficult to stay inside the proper lane and would sometimes drift onto the shoulder.
Karen assumed that a change in her vision was causing the problems.
So, she had her eyes examined and bought the best pair of lenses available.
The new glasses didn’t help, and Karen’s vision problems persisted. So, she went to another eye doctor for a second opinion.
The second optometrist told her the new glasses were right on the mark, and couldn’t explain why she was still having trouble with her sight.
Karen’s visual spatial and perception problems grew worse, and they became apparent to her husband and other family members.
In October, she saw her family physician and told her about these problems. The doctor referred Karen to a neurologist.
After a series of test to rule out everything from cancer to blood disorders, she underwent a CAT scan.
She was diagnosed in 2015 with Posterior Cortical Atrophy (PCA), described as a variant of Alzheimer’s and also known as Benson’s syndrome. The disease causes shrinkage of the back part of the brain, causing a decline in vision.
The disease usually affects people at an earlier age than typical Alzheimer’s cases, with initial symptoms often experienced in people in their mid-50s or early 60s.
Karen was 56 when she was diagnosed. Most cases of Alzheimer’s disease occur in people age 65 or older.
She was very surprised to learn that her vision was not the source of her problems after all.
Because her disability was getting worse, shortly after her diagnosis she gave up driving and resigned from her position as a registered nurse.
The disease is a progressive disease. Early symptoms include difficulty writing, blurred vision, light sensitivity, and problems with depth perception and navigating through space.
Additional symptoms include apraxia, a disorder of movement planning; alexia, an impaired ability to read; and, visual agnosia, an object recognition disorder.
Some studies have found that about 5 percent of all Alzheimer’s cases are diagnosed with PCA.
Like all Alzheimer’s diseases, there is no cure for PCA.
Medications are available to slow down the degeneration of the brain tissue, and studies show that such drugs can give Karen and other PCA patients 10 years or longer to live.
Instead of being distraught with her terminal diagnosis, Karen is excited about life.
She’s grateful her disease is progressive. She tells me it gives her great peace to be able to plan for the future, knowing how much longer she has to live.
Since her diagnosis, John and Karen have done many things that they had planned to do someday. But now, they do these things today.
It is the rest of the family that worries and mourns about what’s ahead for Karen and John. We marvel at how positive this couple of 33 years is about their future, and admire their attitude of “why wait — let’s do it today.”
It’s a philosophy we all should adopt —whether it’s estate planning and completing your will, buying new carpet and furniture, traveling to someplace you’ve always wanted to visit or reading Moby Dick.
It’s an attitude of living every day as if it was your last, and making sure the people you care about know how much you love them.
When I asked Karen about writing this column, she wanted to make sure I reported that PCA often goes undiagnosed for years, because many eye doctors are not aware of the disease.
Most people diagnosed with PCA first go to their optometrist because they believe they needed stronger glasses. When new glasses or contacts don’t eliminate their visual problems, they often visit other eye doctors — who often are unfamiliar with the symptoms of PCA.
If a PCA patient sees a neurologist sooner, it can be diagnosed and treated earlier, with the potential of slowing down the disease, and saving themselves and their family years of stress and anxiety.
So, next time you see your eye doctor, please ask him or her if he or she is aware of PCA, and if not, to please research the disease to understand how to respond if a patient complains about problems with driving, penmanship and spatial awareness.
Do this for yourself and those you care about.
And, do it for me, and my sister, Karen.
Published April 5, 2017
