A Shattered Life: The Last Days With Creutzfeldt-Jakob Disease


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.

 

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Global Genes Comments

  1. My beloved brother Jay passed away last month of cjd. He was 63 and in great health. He moved home in mid August to work with his family. We know that that we got a miracle now. God got him home to us because he barely remembered driving to Cincinnati from California. From the moment he stepped foot back here at home he wasn’t right. He was very dizzy at first. Then he couldn’t remember little things. Each day it got worse and worse. It was so rapid just like the other posts. It was the most horrible thing my siblings and I ever witnessed. We never left his side. He got home here on August 15th and died October 14th. They did all the test that were mentioned in other post. We had another miracle when they wheeled him into hospice and unbeknownst to them they put him in the same room where our Mother died twenty years earlier of cancer. God was with us and so was our Mom. We were heart broken and still are. We can’t understand how our brother who was a vegetarian got this dreadful shocking disease. God bless you all who have also gone through this. This is even hard to write. I wish you all peace in the coming holiday season.

  2. My brother, 50 years old has CJD right now.
    Sept 22 he felt dizzy, Oct 12 with ambulance to the hospital. Oct 16 they told us about CJD and there are NO cures for this.
    This is a nightmare, I am with him every single day till 9.00 p.m.
    He can’t talk, can’t walk, eat a little, don’t remember anything, it seems he is angry all the time.
    What do I Do to derserve this?
    So unreal.

  3. My heart goes out to you. Sorry for your loss. My sister and I think our 84 year old father died of this and was misdiagnosed with a stroke(which did happen according to mri) and rapid onset alzheimers. He never before displayed signs of dementia. We did no autopsy as we found this information of this disease after his burial but after reading stories this sounds exactly what happened to him. symptoms started in april and he passed mid august. very rapid declines. so sad to watch. we cared for him at home my mother and 2 sisters. Bless you.

  4. G Haverly says:

    God Bless you. My Father, whose name was also Ron, passed away in late June of 2015. His death occurred exactly 40 days from the day he struggled to tie his tie for church because he couldn’t move his fingers with enough dexterity. He had been complaining of a “dizziness” as well and described the sensation as being similar to what it is like after having two glasses of wine. 23 days after he walked into the Hospital ER with my mother and I, he passed away. Reading your account of your husband’s slow fade brought back a lot of memories from that awful time. We also had an autopsy performed and it was confirmed to be Sporadic CJD. In addition, we authorized the use of his brain for research so that one day stories like yours and mine might become a thing of the past.

  5. Dawn Sardes says:

    My husband is scheduled for his lumbar puncture this Friday, April 7th, 2017. He was a Masters level social worker and therapist who specialized in dual diagnosis clients (both chemically addicted and with a diagnosed mental illness). Last fall, just before the holidays, I noticed that he was making small mistakes, turning left when he should have turned right, missing an exit, forgetting to transfer money to checking. But everyone does these things once in a while, so I joked that he was getting “old-timers” disease. Then, on January 17th, I received a call from a local urgent care center. He had experienced a very bad dizzy spell at work and was very confused. He was taken by ambulance to a nearby ER, given a few tests and released as he was fine again. We were able to get an appointment with his doctor on February 15th. During these two weeks, he was having problems finishing sentences, grasping for words, he began walking oddly, and his voice became raspy. He had to be prompted on how to use an ATM. He could not figure out how to add a $7.00 tip to a $33.75 restaurant tab. The waitress gave him a calculator and he had no idea how to use it. I had to explain to him several times over three days how credit cards work. So, I told his doctor all this and he put him in the hospital for 2 days for a battery of tests. MRI of the brain blood work, ekg, the works, thinking it had something to do with mini strokes because his blood pressure was very high (he has been on HBP meds for over 30 years). But all the tests came out looking fine so he was released and a referral was made to a neurologist. He gets worse every day, he did poorly on the psycho-neurological tests. He gets very agitated now over nearly everything, like crowds, crying babies, and is even becoming paranoid. He is up most of the night. He sleeps on the floor, for the small stretches of time he can sleep. The thought that I am this close to losing him forever is killing me.

  6. Katie Orritt says:

    I would love to be able to reach out to you on a daily basis. My name is Katie Orritt, I am 24 years old. My mom was diagnosed with CJD this last November, and thus far she is almost completely immobile. Its progressively harder each day to hold conversations with her, as her memory is fading increasingly as well. I’d love to speak with you, as we are in the same boat.

  7. Renee Hill says:

    i am so sorry for your loss. my husband battled this horrible disease for 3 years . yes it was long long journey. he finally passed away at 42 years old on December 7 2016 leaving me his wife of 21 years to take care of our beautiful 6 & 9 year old kids. He had Sporadic CJD as well.

  8. Lorenzo Thione says:

    My dad passed away last Christmas of CJD with symptoms and a progression remarkably similar to that of your husband. My heart goes out to you and your family. Thank you for sharing your story, and I wish you strength to live a wonderful and full life with the memories your husband left you with.

  9. Hello, my heart goes out to you. I worked at a University in Neurology and saw two cases of CJD, just as you stated rapid progression and absolutely devastating. God bless you as you learn to go on with your memories of wonderful times you did share getting out and enjoying this beautiful world.

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