MY DAD'S CJD STORY
My father's story begins with the same subtle symptoms as many describe at the beginning of
the diabolical journey through CJD. My dad is 76 years old and still alive, hospitalized, and apparently near the end of life. Just today, April 21, 2005 his primary care physician received confirmation from a spinal tap test performed at the Cleveland Research Center that the prion protein 14-3-3 sequence was present. Not a shock at this point. It has clearly been demonstrated that he is not withering away due to any other possible disease---they've all been systematically ruled out.
There's no way for me to put it in a nutshell---this is how it began. Until March 3, 2005 my dad was an extremely healthy, spry, energetic senior. His overall health was that of a man in his 50's according to his doctor before this hit him with a force I've never seen anyone hit with before, and never hope to see happen again to another living soul! He has the kind of beautiful, olive, wrinkle-free skin many commented on because everyone wanted to look like him when they get older. My dad looks like Robert DeNiro. He had a great, even temperament, expressed his love for wife, kids and grandkids in countless acts of service. He was the guy who never held grudges, tolerated the faults of others with patience and was always there when no one else was there to assist those in need. His family and friends were robbed of a humble, sweet man and the pain will be felt forever, I think.
On 3/3/05 Dad complained first to my mother and brother that he felt "stupid" when he couldn't remember my brother's telephone #, which he frequently called. Mom attributed it to a bad day with "senior moments". My brother took it seriously because our normally calm and stoic dad was bothered by it. He thought his new med. for prostrate problems was at fault.
My brother scheduled a visit to the doctor which would happen in a few days. He never snapped out of his senior moment and seemed actually to be having a lot of word retrieval problems in conversation. My brother accompanied him to the visit. Dad came out and couldn't recall one thing the doctor told him. My brother questioned the doctor when he learned a CT scan and blood work were ordered. At this point our worst fear was that dad suffered a mild stroke---a TIA.
4 days later, on the day of the tests he was already worse. Compared to now, he had a lot of skills. He could carry on a conversation and tell you info if you could fill in the blanks. He was walking fine and could get to and answer the phone quicker than my arthritis stricken mom with a hip replacement. He wouldn't know me or many others, though or remember that we asked for mom in the time it took mom to ask, "Who is it?" His big activity was reading books and the daily news at this point.
These tests reveal he is fine. The doctor knows something has happened to the left side of his brain based on observing him. The first MRI is ordered.
A few days later as this appointment occurs, dad is a bit worse. He is less and less able to demonstrate he understands, seems agitated by the play around him of his 7 yr. old grandson who he normally adores, cannot follow basic commands, seems demented more and more and is losing more expressive language. The MRI is difficult because no one expected him to be so out of touch.
At this point, though, dad is aware he's sick. When given encouragement that we're just taking him for tests because we love him and want him well, or if he's told people are praying for him, he cries. However, he tears up and even laughs at other moments when it seems inappropriate, almost a spontaneuous reactiion. He can still make slight jokes, but seems more and more blank.
We wonder, "Is he having more strokes?" We start pushing his doctor to see him again sooner than expected as a follow-up to the MRI. Easter weekend is approaching and we are so clueless as to what's wrong with my dad, we worry if we'll know what warrants a visit to the emergency room. We are assured that he's not in danger of a stroke, nor did he have one. Yet, our fears are based upon a pattern we noticed emerging (and boy, did we have a crash course in getting on board with observing him!)---every 3-5 days he gets new and worsening symptoms.
At this stage his doctor advises we see a neurologist. Our doctor suspects a couple of treatable conditions. A few days later we see the neurologist. By now my dad is very demented and must be accompanied everywhere he goes. His fine motor is compromised and when asked to sign his name, he produces 3 circles in an attempt to begin his name, Albert.
This specialist takes a look at the extensive background we offered and examines and speaks with my dad for almost a 1/2 hour. I leave the examining room with the doctor as he calls to get his previous test results. He tells me that while he seems demented, he believes my dad has a rapid, degenerative disease. An eeg is ordered. We had to search for a hospital to take him ASAP (which ended up to be the next day) because the neurolgical group we were referred to will make us wait over a week. We are more than wise to the fact that every day counts at this point. We pause to realize that we are watching our father waste away before us with no clear medical answers! The eeg shows abnormal brain wave patterns. Finally, something that backs up what anyone can see! We observe on the order for the eeg that JC disease is to be ruled out, as our neurologist coined what we know as CJD. No one knows what this is. Our research begins. We are stunned with everything we read on the web, and learn of the Cleveland clinic. We come to the spinal tap appointment with the directions for shipping the CSF in hand.
The neurologist sees him again within a few days. He is worse by now. His gross motor is very affected and he's not sure on his feet. He is more confused and gets little out verbally. He performs a spinal tap. Dad is incontinent by now and the nurse has to change his adult diaper while we are there. After the trauma of this procedure, my dad manages to say, "home, home" as we leave. We assure him that's where we're headed when we start to comment on the nice day to avoid tears. He breaks into a song, "Keep On Smiling..." That day is laborious for the family trying to get him to lay flat for the day. He fights getting out of bed to use the bathroom. He can't tell us his need, but kicks at the bed covers furiously. He can get up but is unsteady.
Within days it is harder and harder to get him to the bathroom and he loses trust about getting up and sitting down. Without my brother, using the toilet is an impossibiltiy for my 80 yr. old mom to manage. So is nearly everything else. The family rallies to help with meals, cleaning and mostly care of dad. He can no longer feed himself. He can't read the paper. He makes tremendous noise with it though, as the myoclonus rattles whatever is in his hand. He has severe tremors, hallucinations and gets up on all 4's in bed. Mom loses much sleep because he frequently gets out of bed and she has to monitor him. Dilantin is ordered since he once had a history of epilepsy---they say it will help with the tremors.
We dress him, clean and shave him, put him back in bed, walk him to a chair and attempt to converse with a man who sometimes doesn't recognize us. My brother and I meet with a social worker who volunteers her help along with a Sister from my parish who is an RN. We discuss options for in home nursing care. We begin to see the disasters of not yet having a diagnosis.
We attempt a final appointment with the neurologist and my dad is now so stiff and having such difficulty being mobile, our worst fears are realized---he cannot make the appointment because he makes it down a few stairs and his legs locked. With one of us putting him in a chair we phone the Dr. who said some of the spinal tap results show he has life threatening symptoms and we should admit him to the hospital immediately. A neighbor and friend helps my brother carry my dad in a chair outside. He is able to walk to and get in the car fairly easily. He was admitted Fri, April 8th to a local hospital. Upon admission, his blood pressure is abnormally high 235/94. He is later sent to a room. From the gurney he whispers to me, "It's me."
Another neurologist comes on board who is in the same buidling as the primary physician. Now comes more rapid decline and a series of anxiety producing events. We are told that another spinal tap is ordered. We have become saavy enough that we know how to contact the research center ourself, finding it suspicious that no one is reporting the much awaited 14-3-3 results. They couldn't because they never left the lab in our home town! We are angry and sad. New fluid is sent, which is again delayed. This time when I call to check on its arrival, I discover that it was not tested, although frozen and handled properly, because the hospital's pathology dep't. only included my dad's name. It was tested once I called and gave them what they needed. We are feeling like we are thinking for everyone now. Everyone is stressed and worried and my brother and I feel (no, we know) there is no opportunity to fall apart and lost our ability to plan and think. Funeral arrangements are made. Now is the end of feeding him his meals and pudding snacks.
More MRI's, an MRI, another eeg...The 2nd MRI shows no blood flow to the cortical area on the left side. The MRI reveals there's no blockage. The house Dr. presumes it isn't getting blood because gray matter is simply gone! A feeding tube is ordered after the first week because his previously ravenous appetite is thwarted by the fact that he can no longer swallow. It went in after a few tries and was a horrible ordeal for my dad. They restrained his hand, and yet he pulled it put.
We are waiting for the CSF results, but we tell them to hydrate him, but leave the tube out...we can't put him through it again...we know in our heart he's dying.
This week brought onset of a virus which affected his eye, blisters on head, groin and hands, more hallucinations, bilirubin levels high and dark colored urine in the catheter bag, less mobility of his left side, an ever worsening bed sore, horrible chest conjestion with mucous being suctioned out every 2 hours, gallstones to boot, and MOSTLY the test results we both feared and prayed for.
Tomorrow we go to talk with is doctor about final life stages plans and to emphasize the importance of the autopsy. We are left with an immoblile, speechless, helpless man who I don't think can see us anymore who sleeps or at least retreats into unconsciousness for 95% of the day. We are losing him rapidly and now we pray for that mercy.
I will spend the rest of my days working in my best capacity for this cause. My heart aches for all the other victims' families too. All this in 49 days from start to present day. As much as I'll miss him, I pray the end is near.
Thank you for the opportunity to share my dad's tragic story.
Final Note: Dad passed away on Wed. 4/27/05
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