CJD: The Fastest Killer

I just spent my very first Christmas without my dad. Five months ago, my dad was going to the gym with my mother on an average of three times per week, climbing Stone Mountain and participating in community service activities with a neighborhood association of seniors, performing ministerial duties at a local church and still conducting marriage and premarital counseling well into his retirement from the Army Chaplaincy. But as of December 15^th, he's gone forever.

After a grueling three months of inconclusive tests and what seemed like outright guesswork by neurologists in three medical facilities, both diagnosis and prognosis were made. I still remember the text message that my sister forwarded to me from our older brother; he was with my mom at the hospital: "They say dad has Creutzfeldt-Jakob Disease. There's no treatment and is usually fatal within months."

I was at work waiting for a tardy client. I held it together as long as I could and finally had to excuse myself to go sit in the car and call my sister to confirm what I'd read-and to cry. She confirmed it and, like a big sister would, tried to comfort me by saying that we shouldn't give up on the only hope we had-a miracle. Having seen the condition my dad was in before this point and having already accepted that he would no longer be the way I remember him, her words weren't as comforting as either of us would have wished.

It felt like a dream-surely my dad, the benchmark for healthy living in my family, wouldn't succumb to such a rare disease. He had gradually lost his ability to see, walk and speak over the course of 3 months and the neurologists concluded that the Parkinsons-like tremors were actually petit mal seizures or myoclonic spasms; doctors disagreed about which he was actually having but agreed that the two were not the same. All we wanted was to be able to pinpoint SOMETHING that had happened recently that might explain this rapid degeneration of his entire central and peripheral nervous systems.

THE PROGRESSION OF CJD

Dad's vision was the first obvious sign of problems. We noticed that he'd been a little more forgetful than usual in the few months prior to the visible onset in September, but we didn't think too much of it since he had just had his 72^nd birthday. Dad was an avid reader, writer and loved politics, especially enjoying the idea of this nation seeing and nominating its first African American presidential candidate. Dad watched President-Elect Obama win the Democratic primary and was able to cast his vote for Obama's presidency even after he could no longer see.

Mom's opthalmologist diagnosed cataracts and scheduled surgery. When mom took him there, he'd reported having double vision-not a symptom of cataracts which simply cloud one's vision, not multiply it. The surgeon proceeded with the surgery anyway. Mom took him back to the same opthalmologist several weeks later when his vision had not improved. The new glasses they'd recently purchased were completely useless and no one could figure out why since the surgeon concluded that the surgery was a success.

My brother then found a neurologist for dad to see and mom carted him over there with high hopes. This specialist decided that when dad's eyes rolled up toward the ceiling, he was simply taking advantage of some small sliver of cornea that he could see through. In hindsight, this sounds ridiculous but at the time we were overjoyed. He gave my dad medications for what he thought were Parkinsons-like tremors on dad's right side and for the onset of Alzheimer's Disease, still not 100% sure why the cataract surgery hadn't helped. He would sometimes sit still, staring up into the ceiling and his right arm would slowly begin to raise up. He didn't even realize it was happening. I asked him once, "Daddy, why are you raising your arm?" He said he didn't know and lowered it back down.

After several weeks of frequent doctor's visits and the entire month of October, this doctor ordered an electroencephalogram (EEG) and found 'activity' in the brain that looked like dad was having seizures. Upon hearing this, mom and the rest of us couldn't help feeling guilty about having spent so much time at dad's side and that we didn't know he was having seizures right there with us. I felt overwhelmingly helpless like never before. It became harder and harder for him to articulate any speech and he eventually only used a few words at a time. Soon he would start repeating sounds or words over and over for long periods of time. He had begun to hallucinate. We assessed that he was just hypersensitive to such strong medicine since dad had never taken anything stronger than cough syrup. Even still, if mom started to sing, he would join in with the utmost enthusiasm and powerful voice like he used to. We reassured ourselves that daddy was still in there, even though what was coming out wasn't like him at all.

He became weaker, his body movements more rigid and less frequent, and after needing to be helped to the bathroom had begun to refuse to move from the floor beside their bed. Being naturally a lot heavier than mom, his decreasing mobility made trips to the bathroom and even getting him dressed became increasingly difficult tasks for her. My sister literally had to abandon her home and move herself and her daughter in with them in order to be able to help. A cousin of ours was in town and also spent some time helping mom once dad had gotten to the point where he was wetting the bed and requiring adult pampers. Mom remembers with great sadness how she could sense his embarrassment and when he seemed aware one day that she had put one on him before helping him to bed.

Dad became more beligerent and aggressive. He was refusing to let mom dress him. Mom said she was holding his hands to help him up from bed and he told her that she'd run away from him, not realizing who she was. Mom said he had such a firm grasp on her hands that his fingernails broke the skin on her palm. Minutes later, he apologized for what he'd only partially perceived to have done. Such episodes of dementia seemed to confirm the well-known indication of Alzheimer's. He also said with a fleeting awareness one day that he didn't know what was happening to him and that he was sorry for anything he'd done-that he just couldn't help himself.

HOSPITAL HOPPING

Having moved out of town during the summer, I was not able to be with them for the entire ordeal, but came to visit as often as I could. I came to see dad after he'd been admitted to the ICU at a hospital near their home. He'd become almost completely unresponsive and this was partially because he had been administered two anti-seizure medications that made him very drowsy. The very last time I heard him utter anything inteligible was in the first week of November. When a nurse asked him if he knew who was in the room with him, he simply responded, 'yeah.'

As if the sight of dad bedridden with tubes taped to him and needles protruding from his arms wasn't hard enough to digest, the neurologist who was supposedly treating him had the worst bedside manner I'd ever seen to date. After almost two weeks, he had no answers besides keeping dad doped up; he defiantly dismissed all of our concerns. A nurse described to us how we could tell that dad was having a seizure-if his arm and eyelids began twitching at the same time on the same rhythmic pattern, it was a seizure. Shortly after telling us this, she said he hadn't had any seizures all day even though I personally witnessed one, by her definition. It was clear that they were running out of ideas when the doctor told us that none of the anti-seizure medications were stopping dad's abnormal brain activity and that he was ordering temporary intubation and to put dad on a ventilator along with the administration of an anesthesia drug to induce a coma-like state. The purpose of this was to be able to monitor dad's brain activity with no chance of being affected by external stimuli. This was supposed to only take place over a 24-hour period but dad was intubated and sedated when I left to return to school and work and he would remain that way for days. I knew in my limited medical knowledge and by speaking to a nurse that I know that leaving someone under anesthesia for days is potentially very dangerous. No one was explaining anything to us and now we know without a doubt that they did not know what they were seeing either.

After being frustrated beyond belief, mom and my sister demanded another opinion. In comes another neurologist in this same facility who would present himself as the previous specialist's antithesis. He listened intently to what my mother and sister had to say and even took my sister's suggestion to try a steroid treatment as it wouldn't harm dad even if it did absolutely nothing in the way of reversal of his condition. When I asked one of the attending nurses before I left what they were calling dad's state, she said it could be referred to as an 'aware aphasia', or basically a bit of normal brain activity but no ability to form speech. Still, it was apparent that they were just as clueless as we were.

Now going into the middle of November, more than 2 months since dad began his descent into this nearly vegetative state, the new neurologist who'd taken on dad's case ordered a feeding tube be inserted into dad's stomach to replace the nasal tube and also a tracheostomy with oxygen support to help him to breathe more easily. Dad had also developed pneumonia so was on intravenous antibiotics. It was upon receiving this news that we were forced to come to terms with the fact that dad's condition was worsening instead of improving. This facility's medical professionals still had not found a way to stop his seizures, either. Mom, my sister and I knew that dad would never be the same-if he even came out of this alive.

After more tests, including a second spinal tap to test for the St. Louis virus carried by mosquitoes, mom had had enough guess work and requested that dad be moved to another facility for yet another opinion. We relied upon our own internet research and prior knowledge of a local university hospital near downtown Atlanta known for its cutting-edge research capabilities, hoping that they would finally get to the bottom of this puzzling array of symptoms. He had to wait for almost a week for a free bed there but was finally transferred. To add to the bad news, dad would need to have a blood transfusion because he'd acquired a blood infection. We were all just starving for just a tiny bit of good news. This is not at all what we would received.

FINALLY GETTING UNWANTED ANSWERS

Within 48 hours, this new neuroscience ICU team had stopped dad's seizure-like activity and had done more EEGs, chest x-rays, and blood tests-the team's head was the one who gave the diagnosis. Another neurologist would eventually explain to us that a particular test that illuminates the brain with light to see where it had become sponge-like showed excessive light in three of the four lobes. Dad had little to no normal brain tissue left. These destructive prion proteins characterstic of Creutzfeldt-Jakob Disease had turned him into someone other than the man I knew as a deep thinker, thoughtful advisor, funny and nurturing dad. He lay motionless, save a few moments when his eyelids would involuntarily open and blink. It was the beginning of the end.

So we had an answer. We could finally stop guessing. It was explained to us that only an autopsy would prove which of the several variants of CJD dad had and the three neurologists we spoke with agreed that dad's onset was spontaneous.

Maybe I'm somewhat of a Newtonian, but too many instances in dad's life could have contributed to this onset.

This disease is in no uncertain terms the human version of Mad Cow Disease and I believe that his penchant for beef tripe, part of a cow's stomach that is considered a delicacy in many countries including this one that he'd recently eaten, may have had something to do with all of this. There is also interesting but inconclusive research of this disease being transmitted upon direct contact of medical instruments not cleaned properly after having made contact with an infected person's blood, brain tissue or cerebral fluid and reused on a non-infected person. The only surgery dad underwent was for a hernia over a decade ago. The speculation could go on and on, but with the acquisition and full course of a disease that only lasts a few months, there really isn't a lot one can prove without asking him questions or retracing his steps for the past few years-which is impossible now.

Not long after the diagnosis came, we started to receive visits from social workers and palliative care nurses-they wanted to know if mom wanted to implement a DNR (do-not-resuscitate) order and started to suggest that we consider moving dad to a hospice facility in an effort to keep him as comfortable as possible for however long he would remain with us. I finally knew what it was like to have to be involved in deciding how someone's last days will be spent. Mom was faced with the reality that her husband of 39 years would not get better, that he would never come home again. The lead neurologist on this team even suggested that we allow them to stop feeding him through the tube since he seemed to have long lost his need for and ability to metabolize food or water. His body had become swollen from his chest down as he was literally rejecting sustenance.

Mom had been spending the night by dad's side as often as she could, sometimes sleeping in her clothes and using the toothbrush she kept in her purse. In this facility, there was an adjoining family room that had two convertible chairs so I stayed a few nights with dad and with mom while she stayed with dad. It was the hardest thing to witness. We talked to him as much as we felt the need and kissed him, held his hands, and touched him just in case he might be aware at least that we were in the room. They assured us though, that even when he winced and gagged as the respiratory therapist suctioned secretions clogging the pathway from the trach to his lungs, his blood pressure and heart rate remained within normal ranges so they believed he was not in distress or pain. I don't know if I believe it, but would like to think so.

CHOOSING A FINAL RESTING PLACE

So, the week of Thanksgiving, coming to terms with the fact that he would need round-the-clock nursing care if she were to take dad home helped mom, my sister, brother and I to have dad transferred to a hospice facility. I had just seen a presentation on palliative care and hospice facilities for individuals with terminal illnesses with or without a prognosis and was delighted to know that the hospice facility also followed the grieving family after the death of their loved one to assist in any way they could with the emotional ramifications and whirlwind that usually follows. Dad was transferred the day before Turkey Day and we had our first big dinner and holiday without him (thanks to members of their church preparing an entire meal for us).

Getting mom to leave the hospice facility long enough to get some sleep and to eat a real meal was difficult as she didn't ever want to leave dad's side. What kept my sister and I motivated was the fact that dad, who spent over 30 years as an Army Chaplain, marriage and grief counselor, and who had already experienced great loss in his own life, had taught us the importance of working through grief in the most healthy way possible. I couldn't help but remind mom that we must go on. Knowing that I would miss my dad if these doctors were really right and he wouldn't be with us much longer, it still seemed imperative to try to echo the teachings of my wise and strong father who knew and accepted that none of us is going to live forever.

So after weeks without food or water and only an occasional sigh or a few involuntary moments of having his eyes open, my dad left us on Monday morning, a week and a half before Christmas and the day before mom's birthday. Even though my sister, brother and I had individually whispered to dad that he didn't need to hold on for us if he was tired and needed to rest, we believe he needed to hear it from mom. The hospice facility put together a welcome packet for new residents' families that includes literature explaining the hospice experience. On one sheet, it explained how many residents seem to hold on if they feel that something is left undone or until a certain someone has come to visit. Dad held on for almost a month in this facility and two of his brothers and mom's sister were able to come visit as well as a host of church and neighborhood friends and other family members.

My dad delivered many eulogies at funerals and in many sermons he talked about death being a friend. Even though we watched my dad go through something so unimaginable and I won't miss him any less, I most certainly believe that this friend came at just the right time.