The devastation of our lives – how did this happen? I have asked myself this question probably a million times over, but there is no answer.
My wonderful husband and best friend passed away on October 12, 2015, from a dreadful and very rare degenerative brain disease – Creutzfeldt-Jakob. It is a prion disease (a disease that can affect both people and humans), as is Mad Cow Disease and Chronic Wasting Disease. There is no treatment. There is no cure. Occurring in one in one million people, many doctors fail to understand or know what this disease is until an autopsy is performed. Misdiagnosed as severe onset Alzheimer’s, or a form of Palsy, families are left with questions, heartache, and the inability to find answers.
Ron was a gentle giant of a man, humble, loving, a father, a grandfather. He was 66 yrs. old and in great shape physically. Always plagued by the fear of diabetes which was in his family, he always had checkups regularly and always was at the peak of health. The summer of 2015 was joyful, as always. Ron was a driver for Douglas County School District and summers were precious to us both as we had more freedom to spend time together doing what we loved – enjoying the outdoors and hiking as much as we could. July 4, 2015 was the best hiking we had in years, both of us ascending the steep trails out of Ouray, CO. Upper Cascade Falls and Bear Creek are among the most scenic and challenging, and we completed them with very little effort. We talked about how good it felt to do these beautiful hikes, and we looked forward to doing these well into our 80’s. How life can change in an instant.
In August 2015, Ron complained of dizzy spells although not serious, just odd. On August 24th he saw his primary care doctor and was told he had low blood pressure and was not hydrated enough, something that happens when you drive a school bus in hot weather. So we proceeded with hydration and he did feel admittedly better. Over Labor Day, September 5, 6 and 7 we spent a wonderful weekend in Ouray, CO, hiking and enjoying the outdoors. The following weekend he had a severe dizzy spell and was having trouble with his balance. On September 16th we again saw his primary care physician. By that time, I noticed Ron was having some confusion and memory issues. The doctor performed and exam and a memory test. The memory test results were poor, so he advised us to have an MRI done to determine what may be happening.
An MRI was scheduled for October 1, 2015, but by the morning of September 18, Ron could not walk steadily and I took him to the hospital emergency. They performed an MRI and told us that there was inflammation in the grey matter, and more tests would need to be done. A lumbar puncture was to be performed that day, but because of insurance issues and scheduling, it was not completed on that day. During the day Ron continued to be confused and unable to walk very well. His speech was also slow. Late afternoon on September 18, a hospital neurologist told us that a lumbar puncture would be scheduled for the following Monday, September 21st. We proceeded to go home, at which time Ron was very confused about his whereabouts and where he had been during the day. We drove home, and over the weekend I watched him progressively decline in movement and speech, resorting to a cane to help him get around.
The lumbar puncture was performed on September 21st, and the neurologist asked us to return on September 24th for a consultation. By September 24th Ron’s walking was difficult and his confusion had increased. That was the day the doctor told us the diagnosis and the prognosis which was, not treatment, no cure, and fatal – death in six months, truly devastating news. What do you do? The doctor gave me information, a web site to consult, and set us up with Palliative Care. Ron completely understood the news and was not upset about himself, but only about me. That was my true love. Always considerate of me. That day we returned home, told family, and hugged each other for a long time.
On September 25th we visited Palliative Care. By Sunday, September 27th, I called in Hospice as he was declining rapidly. His confusion, his memory and his speech were worsening by the hour, although he could remember things from long past and tell stories about UFO’s and meteor’s, something he had studied prodigiously. The week progressed badly every day, with continuing decline and more symptoms — agitation, hallucinations, memory loss, inability to move around without tremendous difficulty. He became sometimes violent which was SO different from the man he was. It was a horrible thing to watch my loved one melt away before my eyes and I could do nothing.
By Tuesday, October 6, 2015, he was unable to understand anything, was unable to straighten up, or sit down, or basically move. Very rapid progression. Under the advice of the neurologist I attempted to find a safe place for him where he would get care. However, nursing homes and memory care units would not take him because of the violent tendencies and hallucinations. We needed to get symptoms under control so Hospice advised that he should go to a Hospice facility for care. He ate some dinner that evening, which was the last food he ever touched. By the next morning, October 7, at Hospice he was in a semi-coma, unable to eat or swallow. From that time on it was a death watch, keeping him comfortable and watching him disappear before my eyes. On the morning of October 12, 2015, my beloved and wonderful man passed, leaving me shattered forever. Death occurred approximately three weeks from diagnosis.
This disease, known as CJD, is insidious. It is considered an infectious disease, transmitted through surgical instruments and spinal fluid. Therefore, embalming was not an option. I wanted an autopsy of his brain, not only for research, but to determine the origin of the disease. In 15% of the cases it can be familial/genetic, so for the sake of his children we needed to know. And then the drama continued. I worked through the National Prion Foundation and they handled all of the arrangements for the autopsy. Unfortunately, there are only five places in the nation that will handle these cases, with no location in Colorado. His body was transported to Kansas City for an autopsy on October 16th. Results came through in January, not familial but determined to be sporadic CJD, which means no one knows how it occurs. Not very definitive and yet it gave the children peace of mind.
I question myself daily – did I see any signs prior to dizzy spells, anything at all? He had sleep problems and then lots of fatigue over a 2-yr. period, but we both thought that it was the inability to sleep soundly and age. Who knows if that was part of this disease. Maybe, maybe not. I have been told that this disease can be dormant for 30-50 years before it rears its ugly head.
Again, there is no treatment, there is no cure, and it is very rare indeed. I have no explanation. It just happened, and now life is shattered and I am picking up the pieces. I do believe there is hope, hope for all affected by these rare diseases but we must be diligent, passionate and willing to do anything we can to help those affected and their families.