Today is Martin Luther King Day, and here’s one of his best quotes, from 1968:

“We must accept finite disappointment, but never lose infinite hope.”

Fast-forward to the 1980s, early in my career as a neurologist mostly for patients with incurable movement disorders.  I rapidly learned that besides objective diagnosis and treatment, my agenda at patient visits should include an old-fashioned pep talk along with an update on research.  Now, I had grown up in a culture where such “touchy-feely,” subjective things were far subservient to scientific thinking, and my medical education was no different.  So, once I was out in the real world of patient care, it was kind of a revelation to discover that a simple, subjective appeal to hope could sometimes alleviate more suffering than any medication, therapy or surgery I could prescribe.

Fast-forward again to 2004, at which point I had been CurePSP’s Clinical and Scientific Director for 14 years, and a new CEO named Richard Zyne arrived.  He was an ordained minister who spent his career mostly with non-sectarian, non-profit organizations.  As a clergyman, he well knew the value of hope in helping people deal with adversity, and he quickly made “Because Hope Matters” CurePSP’s tagline.

I’ll admit I was skeptical at first.  I thought that providing hope was the doctor’s job at an individualized, “retail” level in the exam room and that CurePSP should support research, educate patients and clinicians, and help find ways to bring the best available care to all who need it.  But working with CurePSP showed me the value of a national organization with multiple communication platforms in reassuring patients and families that scientific understanding of PSP is advancing, that similar diseases are slowly yielding to new treatment, that more researchers and journal articles are devoted to PSP than ever, and that a well-run non-profit organization is in their corner. In other words, I again discovered that hope matters, but now at a more “wholesale” level.

The idea for this blog post entered my mind from the proximity of MLK Day and my post from four days ago, where I reported the failure of one PSP drug candidate but offered hope for five others currently in clinical trials.  In fact, regular readers of this blog know that I try to infuse hope into every post rather than merely reporting the news objectively.  For the ability to understand the value in that, I thank my patients, Richard Zyne – and Dr. King.

Long PSP

For several years, one of CurePSP’s public-facing taglines has been, “Because Hope Matters.”  Last week, the CurePSP staff decided to make that advice part of the organization’s actual logo, replacing “Unlocking the Secrets of Brain Disease” at that position. 

In trying to help patients with PSP, CBD and many other still-incurable diseases over four decades, I’ve found that hope really does matter.  It’s not just a bit of quackery arising from medical impotence.  No matter how thin that thread of hope may be, a person’s stress level, and their motivation to work with their physician to do what can be done, are greatly enhanced by some measure of hope:  hope that a cure, or at least a way to halt the disease’s progress, may be found in their lifetime; hope that they will be among the minority spared some of the most disabling symptoms; and hope that their survival will beat the average.

That’s why I was most glad a few days ago to see a report from the Rossy Program for PSP Research at the University of Toronto of four patients with clinical diagnoses of PSP surviving 11, 12, 18 and 20 years after symptom onset.  The average figure is typically reported as between 6.7 and 7.5 years.

Three of the four Toronto patients had confirmation of PSP at autopsy.  The fourth had a very rare condition called pallido-nigro-Luysian atrophy (PNLA), a tauopathy that often mimics PSP and is equally resistant to treatment, but has a much slower course. 

Sidebar:  I first learned about PNLA in 2004, when I was invited by the journal Movement Disorders to discuss a “clinicopathological correlation.”  That’s a teaching exercise where the organizer selects a patient whose diagnosis was difficult or impossible to make during life but whose autopsy gave the answer.  The organizer sends a clinical summary to a recognized outside expert or two with no previous knowledge of the patient.  They each submit a written discussion and a diagnostic conjecture to be published alongside the autopsy results.  The case I was invited to discuss was someone with a clinical picture that looked exactly like PSP except for a 26-year survival.  The other discussant and I each independently concluded that the patient had PSP with a long survival was statistically plausible for that condition.  But the autopsy showed “primary pallidal degeneration,” of which this was the eighth case ever reported in the medical literature.  The pathologist mentioned PNLA as a similarly rare, closely-related condition that he considered as an alternative, but it did not fit the autopsy results quite as well.  Now back to business:   

Whenever I discuss expected survival duration with a person with PSP or their family, I tell them the truth – as gently as possible.  But I also point out that they could beat the average survival figures, especially if they get treatment to help protect from falls, aspiration, infections, malnutrition, and emotional stress.  That measure of hope provides a kind of lifeline to grasp, one now corroborated by the medical literature.  So here are four more patients proven to have beaten the odds – four more reasons for hope.