After his diagnosis with Parkinson's disease, my father once said, "One person doesn't get Parkinson's. The entire family gets Parkinson's."
Such insights from Dad were rare, but in this case, dead on target. For both patient and caregivers, it's a disease that is painful, frustrating, infuriating, and scary as hell. It's both chronic and progressive.
At times, it's nearly impossible to cope.
There is no cure.
This is the story of my father's final journey - one that took many years - and the lessons I drew from it.
KosAbility is a community diary series posted at 5 PM ET every Sunday and Wednesday by volunteer diarists. This is a gathering place for people who are living with disabilities, who love someone with a disability, or who want to know more about the issues surrounding this topic. There are two parts to each diary. First, a volunteer diarist will offer their specific knowledge and insight about a topic they know intimately. Then, readers are invited to comment on what they've read and or ask general questions about disabilities, share something they've learned, tell bad jokes, post photos, or rage about the unfairness of their situation. Our only rule is to be kind; trolls will be spayed or neutered.
While Dad's initial diagnosis was "Parkinson's disease," specialists ultimately gave him additional diagnoses of "Lewy body syndrome" and "Parkinson's-related dementia." They're all part and parcel of the same spectrum of conditions, but not all people diagnosed with some form of Parkinsonism develop them.
Parkinsonism: What Is It?
Parkinsonism is a neurological condition that covers a spectrum of specific disorders, symptoms, and side effects. These include Parkinson's disease, for which the spectrum is named; Lewy body syndrome; tardive dyskinesia; dystonia; dyskinesia and/or bradykinesia; forms of tremor; spasticity and clonus; and the forms of dementia that all too often accompany the physical effects.
Dad had every one of these, to one degree or another. Cumulatively, the effects were devastating.
So what exactly is Parkinson's?
According to the National Institute of Neurological Disorders and Stroke (part of the National Institutes of Health, or NIH), "Parkinson's disease (PD) belongs to a group of conditions called motor system disorders." NINDS lists for symptoms necessary for a diagnosis of PD: 1) tremor; 2) rigidity; 3) bradykinesia; and 4) postural instability.
Generally, onset of PD occurs after age 50, although there are both early-onset (think Michael J. Fox) and late-onset versions of the disease. Rarely, however, does diagnosis occur shortly after onset; it's a sneaky and mendacious disease, beginning slowly, building gradually, and exhibiting effects that are easily mistaken for a host of other conditions and disorders.
What Causes Parkinsonism?
Generally speaking, the immediate cause is lack of dopamine production and or reuptake in the brain. Dopamine is sometimes called the "happy chemical": It helps to regulate mood and plays a significant role in memory and cognition. It's released during sexual activity, and is part of what makes an orgasm feel so wonderful. It's also implicated in addiction - and it's the chemical most heightened by methamphetamine, which explains the insidiousness of the drug's addictive properties. Finally, via the neurotransmitter norepinephrine, it helps the entire body to function properly, including the autonomic nervous system, regulating automatic, involuntary functions like breathing, blinking, swallowing, the functions of internal organs, etc.
When the brain either 1) doesn't produce enough dopamine, or 2) is unable to process it efficiently or effectively (reuptake), a number of negative effects result, including depression. When the dopamine shortage reaches a critical stage, however, it begins to affect the neurological system, and eventually, the body's entire ability to function properly begins to break down. The effects are very much like a short circuit: The wires may contact periodically and allow for semi-normal function, but you never know when it's going to short out again - and eventually, the short will be permanent.
The underlying causes are more difficult to determine, and there may never be a definitive answer. For some patients, a genetic tendency clearly exists, particularly for the early-onset (before age 50) version of the disorder and the juvenile-onset (before age 20) version, which seems this far to have been diagnosed mostly in Japan. With regular PD, a study published in early 1999 of white male twins who were WWII veterans seemed to indicate that "[g]enetic factors do not play a significant role in causing the most common form of [PD]." Five years later, however, research had uncovered clear evidence of gene mutations in PD patients that indicate that an "inherited" tendency exists.
Since there do seem to be clear regional differences, researchers are looking at the role environmental factors might play, but so far, the official literature has reached no firm conclusions. However, the risk of developing PD is much higher both in industrialized countries, and among farmers and agricultural workers, both of which indicate the possibility of an environmental link. Parkinson's-like symptoms have been established in victims of at least one form of poisoning - with MPTP, which is sometimes found as a contaminant in street drugs, and which is chemically similar to certain pesticides. Some pesticides and other chemical toxins have been established as a cause of PD in animals. And PD-like motor neuron disorders have been identified in the relatively isolated populations of both Guam and Japan's Kii Peninsula, which researchers speculate may be due to specific environmental factors.
Some experts also believe that certain viral and/or bacterial infections might play a role. During the 1918 flu epidemic, some patients who developed encephalopathy subsequently also developed aggressive, progressive form of Parkinsonism. In Taiwan, some women who contracted the herpes virus later developed temporary PD-like symptoms resulting from an inflammation of the substantia nigra, the part of the brain that controls motor functions and is affected by PD (raising the spectre of yet another role played by the herpes virus, a topic for a later diary). Other PD studies have uncovered. And according to NINDS:
Other studies have found evidence of activated immune cells and the accumulation of inflammation-associated proteins in PD. These changes might be triggered by viruses in some cases.
I have my own theories: not necessarily of definitive causes, but of contributing and aggravating factors - certainly in my father's specific case - discussed below.
My father was never able to walk in two worlds. He felt that he had to choose - and in the end, I have always felt, he chose unwisely. That decision would haunt him, in one form or another, all of his days, and never more so than in the final extraordinarily difficult years of his life.
His early life was fraught with poverty and illness. As his mother regrettably informed him one day in a fit of anger, while pregnant with him, she tried unsuccessfully to induce an abortion by drinking some now-unknown concoction. That would scar him physically and emotionally
his entire life. He was born with a perforated eardrum and compromised immune system; he struggled with what would now be diagnosed as hypoglycemia, a result of their grinding poverty, extended periods of hunger, and attendant malnutrition. He was abused both physically and emotionally by other family members, and then by teachers, whose vicious behavior allowed other children to make him the scapegoat of their one-room schoolhouse. Members of the Klan repeatedly sabotaged his father's equipment, threatened his family, and literally ran them out of one small town.
In other words, Dad's entire childhood and adolescence were filled with terror and stress on a daily basis - and, perhaps unsurprisingly, he grew into a lethally depressed, insecure, fearful, paranoid, jealous, rage-filled, abusive adult. The chemical alterations to the brain and the inflammatory effects on the body that occur with prolonged abuse and such extended stress have only come to light in recent years, and the dynamics are sufficiently complex to deserve their own diary. Suffice it for now to say that I have no doubt that his past laid the foundation for the illness that consumed him as an elder. And with the onset of the Parkinson's, those demons that he had fought his entire life - fear, insecurity, depression, anger, resentment - all came back to torment him with a vengeance.
Symptoms and Side Effects
After my father's initial diagnosis, I flew out to see my parents. The change in my father was gradual, but still noticeable: the weight loss, the unsteadiness, the clumsy gait and coordination. Nonetheless, Mom assured me that they were fine: She was healthy and could help him up when he fell, and I should go back home and live my life.
Four or five years later, Mom suddenly needed a triple bypass. And my life turned upside-down.
I flew out for her surgery (at a hospital 200 miles from their home).
I was shocked by the change in Dad. This short but broad wannabe-tough guy who'd always scared the hell out of me was now gaunt, shrunken, stooped, his color ashen, his ability to walk vastly compromised and his gross and fine motor skills severely affected. He had developed bradykinesia, an extreme slowness of movement and a progressive inability to move spontaneously; akinesia, in which the body "locks up" and refuses to move for a period of time; dyskinesia (also called tardive dyskinesia, or involuntary, uncontrollable movements, often socially inappropriate, that are now seem mostly as a side effect of long-term use of the neuroleptic drugs used to treat PD); dystonia, or extreme muscle contractions (such as clonus) that cause some part of the body to warp itself into an unnatural (and sometimes very painful) position; and muscle fasciculations, or smaller (but also frequently painful) muscle spasms or contractions, which may be caused by the PD itself, certain drugs, or both.
And that was just the immediately-visible part.
It wasn't really even a question; I flew home, quit my job, made a clean break with my life there, packed up my dogs, and drove to New Mexico. And I learned, by being thrown in feet-first, how to be caregiver to two people.
In addition to the more obvious physical effects discussed above, Dad had problems chewing and swallowing food and water, including occasional aspiration. He had difficulty hold eating utensils or a glass, much less using them. Needless to say, he no longer wanted to venture out in public. AS though the tremors weren't enough, he developed akinesia, or "lock-ups" of his limbs, particularly his legs and feet: He'd be walking - or rather, shuffling - along, and suddenly stop, unable to move. He would remain that way, locked in position, until his brain decided to "unlock" things again. The tardive dyskinesia caused by the neuroleptics (powerful antipsychotic drugs that have become a part of the standard treatment protocol for PD) caused other effects that embarrassed him greatly: uncontrollable grimacing, smacking his lips, occasional drooling, a periodic inability to form the proper sounds for words. He suffered periodic incontinence - manifesting both as urgency and as lack of warning.
We already knew that, in addition to the PD, he had what's known as Parkinson's-related dementia. Eventually, he was also diagnosed with Lewy body syndrome, a specific form of progressive dementia marked by the buildup in the brain of proteins known as "Lewy bodies." In addition to its progressive nature, Lewy body syndrome includes periodic fluctuations in lucidity, visual hallucinations, and the physical effects of Parkinsonism.
By now, he had also developed fairly severe cognitive deficits: short-term memory, mood, critical thinking and reasoning skills. He had hallucinations. Visual, auditory, tactile hallucinations in three full dimensions and glorious technicolor. He had both sleeping and waking dreams, which he subsequently remembered as "real" occurrences. He often thought my absent brother or my late sisters were present and talking with him. He regularly saw me, not as I actually existed, but as a child: once, as I sat with him in the living room, he told me that he "saw" three-year-old me across the room, playing under the heavy oak dining room table that from the Michigan farm where we lived decades previously.
Other times, he would see people from his past - long past. His parents, aunts, uncles; teachers or other children who'd tormented him as a child; bosses and co-workers with whom he'd clashed (such as the boss who regularly made passes at Mom and other wives at company Christmas parties in the 1940s). Occasionally, he would carry on conversations with people I couldn't see. More often, he'd hallucinate or dream bad episodes from his past and awaken in fury or terror.
To others, the most striking thing about Parkinson's is the obvious physical effects: . They manifest differently in different people: In Janet Reno, what one sees looks more like essential tremor, a generalized trembling that is most obvious in the hands and head. In Michael J. Fox, it appears as a "fidgety" effect that resembles an extreme version of a hyperactive child unable to sit still. These effects can be caused by the disease itself, by the medications commonly used to treat it, or by both.
Fatigue and stress aggravate these involuntary movements, which exhaust the patient even further, turning the entire process into a vicious circle of increasingly extreme tremor. Dad suffered nearly constant tremors of varying degrees of severity. At best, his hands trembled constantly when he tried to hold a pen, a fork, a coffee cup. As the disease progressed, the involuntary movements became more extreme, with rapid uncontrolled swinging of his lower arms from the elbow: up and down, up and down, so hard that his arms beat a loud and incessant tattoo on the armrest of his chair. Eventually, the sound became part of the background noise of my life as a caregiver, and I (mostly) ceased to notice it.
Treatment
There is no cure for Parkinson's disease, nor for Parkinson's-related dementia or Lewy body syndrome. All are progressive. Although research continues, currently, the best prognosis involves efforts to manage the symptoms and to stave off the worst effects as long as possible.
Pharmacological treatment is usually the first line of defense, and Levodopa-Carbidopa is probably the most commonly prescribed drug. It's a dopamine precursor, a combination drug that attempts to increase help the body convert those substances into dopamine and thereby increase its levels and availability in the brain. Antidepressants, such as selective serotonin reuptake inhibitors (SSRIs, like Prozac or Paxil), or selective norepinephrine reuptake inhibitors (SNRIs, like Effexor), may also be prescribed. During the more advanced stages of the disease, many specialists attempt to treat the dementia with drugs like Aricept, or with neuroleptics, powerful atypical antipsychotics more generally used for illnesses like schizophrenia (such as Seroquel or Risperdal).
If you or a loved one face this disease, beware of the neuroleptic drugs: In at least some states (such as NM), medical professionals are required by law to obtain informed consent from the patient or legal guardian before administering such drugs - but they don't always follow the law. I know of two separate instances of failure to do so, the first involving my father. And some cf them, such as Risperdal, are expressly contraindicated in advanced Parkinson's patients or the elderly. Review all prescription orders at the time they are issued, do you research, and be prepare to take control of your - our your loved one's - care. When I discovered the covert administration of Risperdal, I insisted that it be halted immediately, barred its further use, and required all subsequent prescription changes to be approved by me. I have since seen this pattern recur elsewhere. Why? Because despite the clear danger to the patient, and the aggravation of symptoms, side effects, and pain, it has a sedating effect that makes them much more pliable. The patient, mind you, may not get any rest or relief (and indeed may suffer greatly), but it makes things easier for institutional and private caregivers.
There are a couple of forms of surgical treatment that are currently in use to treat PD. The first involves removal or destruction of specific parts of the brain that are affected: pallidotomy (destruction/removal of the globus pallidus) or thalamotomy (destruction/removal of part of the thalamus). These procedures have been refined in recent years, but because they involve destruction of brain tissue, they are irreversible.
More popular now is deep-brain stimulation, or DBS, which involves implanting electrodes in a specific area of the brain to control misfiring neurons. It is a procedure that has given a great deal of hope to many PD patients, and for which my father actually would have been a candidate - had his doctors not continually delayed referral until his condition was too advanced. There are two specific advantages of DBS: First, it is not irreversible; and second, the technology is now sufficiently advanced that in one form, the stimulation can be controlled remotely by the patient him- or herself.
Complementary therapies run the gamut, and often are a matter of personal preference. These may include standards such as exercise, physical therapy, and speech therapy, or "alternative therapies" such as dietary changes, use of vitamins and other supplements, biofeedback, massage, yoga, acupuncture, and various culturally traditional methods (which, at the end, were what we used).
Because of my father's bullheaded refusal to use his walker, he eventually broke a hip. Thereafter, he declined rapidly, and I lacked both the physical strength and the medical expertise to provide the round-the-clock care that he needed, and we were forced to transfer him to an assisted-living environment. He lingered for almost three more years, his condition deteriorating almost daily. By the end, he was largely able to do nothing except sit in his wheelchair or lie in bed. His vision was by then too compromised for him to read or watch TV; his hearing was too bad to make audio books, CDs, or the radio an option. This man, who had topped out at nearly 200 pounds at his heaviest, dropped to a skeletal 115. He developed opportunistic infections; a bedsore that spawned osteomyelitis; pneumonia. He lost most of the circulation in his lower extremities; hands and feet, now doubled in size, wore "gloves" and "stockings" in angry shades of purple and black.
The dementia was nearly complete: Rarely lucid, he almost never understood the things we patiently repeated to him, hoping they'd sink in at least a little. Half the time, he didn't recognize me. That didn't bother me much, but it on the few occasions that he didn't recognize Mom, I know it hurt her terribly.
Worse, he would hallucinate what had always been his greatest fear: that this woman who loved him more than life, but who he'd always felt was too good for him, would finally agree with that conclusion and leave him for another man. At times like that, he'd accuse my teetotaling, ladylike Christian mother of "running around," of "gettin' liquored up," of having divorced him and married someone else. Or he'd tell me that I needed to put a stop to her doing such things. Normally, I played along with the hallucinations, because he got far less agitated if I didn't try to convince him that what he was seeing didn't exist, and normally, it hurt no one. The only time I put a halt to it was when he launched his attacks on Mom. I don't think he ever quite knew what to make of his youngest daughter, who'd always feared him, issuing orders so sternly. Of course, some of the time, he didn't even know that it was I.
At the end, he was too weak even to lift his head. He lay in bed, his swollen purple fingers clutching the covers in a not-really-metaphorical death grip; his legs locked at rigid 45-degree angles. His breath was labored; his eyes drifted back and forth, confused, unseeing; and still, the fear that had wracked him throughout his life would not release him. Mom tried talking to him, telling him it was okay to go.
I had long since hung a dreamcatcher over the head of his bed in hopes that his sleep would be less tormented, as well as a medicine wheel with offerings attached in a small pouch. A medicine bag containing traditional herbs was tied to the bars of the bed. Now, I "sang" a couple of our traditional end-of-life songs to him - "sang" because I was not fluent enough to sing them in our own language, and had to fall back on English. Still he remained agitated.
I had brought my smudge kit, and finally, we got the nurses to come in and shut the off the oxygen long enough for me to smudge him. Because of the draft from the air conditioner, I could maintain only the tiniest of tendrils of smoke, and only for a brief moment. As I prayed, I realized that I was holding my breath. That tiny tendril wafted under his nose for a split second - and dissipated entirely.
And then, his hands released their grip on the covers. The muscles in his legs relaxed and straightened for the first time all day. I lifted the blankets, and nearly fainted with surprise: In a matter of a second or two, not only had the swelling in his hands and feet entirely disappeared, but the ugly mottled black color had vanished; in its place was healthy, pink-tinged skin that he hadn't had in months, perhaps a year or more. Simultaneously, his breathing became smooth and even, and his eyes rolled my way, focusing directly on mine once, before his lids closed and he drifted off to sleep.
I left him alone, then, with the woman he loved more than anything in the world - and with whom, had he survived six more weeks, he would have celebrated a 60th wedding anniversary.
Mom told to go home, so I headed out for the 300-mile drive. Ten minutes later, he was gone - as we say, walking the Western road.
Or perhaps he was flying it. The next day, as I stood on our deck, Giniw, Golden Eagle, flew up out of the alfalfa perhaps a dozen yards away, a terrorized prairie dog hanging helplessly from its talons. The bird looked me directly in the eye as it soared past.
Golden eagles are rare around here in populated areas, and they avoid humans. But for the remainder of that year, on each of the many occasions that I traveled around the state, at least one golden eagle would appear during every trip, flying directly overhead or standing sentry as I passed.
I always knew who sent them.