By
Mark Sauer, The San Diego Union-Tribune
(Conducted
via E-Mail)
October
10, 2000
Dear Dr. Perlman:
I believe Mary Clare Schlesinger has told you I'm
doing a feature for our science page on PPS. She said you graciously agreed to
an interview.
I'd like to do this by e-mail, since it allows you
to not only answer at your convenience but also a bit of time to think about
your answers in relation to my lay audience -- plus I don't have to furiously
scribble (type) your answers.
Right now, I see three basic themes to my story: the
genetic research being done at Salk by Sam Pfaff, the clinical research being
done by you at UCLA and the view of those locally suffering from PPS. I have
written about this subject before and happen to be a polio survivor myself
(contracted in 1958 at age 6, suffered temporary weakness in ankles and lower
legs and was unable to walk for a time; no noticeable lasting effects), so I
have some background.
My audience is the general newspaper reader with an
interest in science.
--Mark Sauer
Mark Sauer (MS)
By way of background, please describe how you got interested in PPS and what
your work in this area entails.
Dr. Perlman (DR)
When I finished my training in general neurology, I began specialty work in
chronic diseases of the neuromuscular system (muscular dystrophy, spinal cord
diseases, cerebral palsy). I had a
multidisciplinary clinic (neurologists, psychologist, social worker, physical
therapist, occupational therapist, brace maker, nutritionist) that focused on
proper diagnosis, rehabilitation treatment, and clinical research into causes
and drug treatments. My chairman was
approached by a couple of members of our local polio support group to see if he
would start a clinic for polio survivors. They brought a stack of
articles with them. He agreed and gave the articles to me with a mandate
to start such a clinic. I knew nothing
about post-polio, but with the help of the patients, quickly learned. My existing clinic was well set up to deal
with their needs of proper diagnosis, rehabilitation treatments, and medication
management, which is what we continue to do.
The clinic itself briefly moved into the Neuro-rehabilitation section of
my department and is now a free-standing clinic, separate from my muscular dystrophy
clinic, but utilizing the same interventions as before. We teach the basics of post-polio to
neurology and rehabilitation residents and see about 200 patients a year, for
evaluation and treatment.
MS Since PPS is not widely recognized by front-line physicians and
is difficult (impossible?) to diagnose precisely, how can we be sure when a
patient has PPS?
DR Post-polio
syndrome is a diagnosis of exclusion. Although the diagnostic criteria are
quite specific, so many other diseases can mimic them that a thorough
evaluation is necessary. Just because a
person survived polio, it doesn't mean they're immune to everything else.
MS What
are the symptoms?
DR Post-polio
is suspected when someone has survived a documented case of acute polio, has
had a period of at least 15 years of stable recovery (at whatever level they
recovered to), and is now experiencing gradual onset of new/recurrent muscle
atrophy, weakness, generalized fatigue or decreased endurance, and secondary
pain in the old polio limb (occasionally in limbs that weren't known to be
affected originally).
MS Who's
at risk?
DR Risk
status corellates with (1) severity of original polio and level of recovery
(more complete paralysis or bulbar polio originally, with either excellent
recovery suggesting a lot of remodelling of nerves or poor recovery suggesting
little reserve), (2) aging, (3) overuse (repeated painful muscle use), and (4)
recent injuries or weight gain. Even
so, only 40% of polio survivors have been found to develop post-polio symptoms. The others may never develop them.
MS Why
do some polio survivors contract it and others do not?
DR The
risk factor profile may be more favorable in some of them.
MS Is
this a problem in other countries?
DR Developed
countries share similar patterns of post-polio. The developing countries (eg.
India) may have much less post-polio, due to a higher mortality rate with the
original infection (only those with very mild cases survived) or due to less
rehabilitation available, so recovery to an artificial "remodelled"
plateau may not have occurred (sparing those patients the future decline from
that plateau, ie. "post-polio").
MS What in your opinion has
constituted important progress (breakthroughs?) in our knowledge of PPS in
recent years?
DR The
biggest breakthroughs are in our understanding of the role of rehabilitation
and in the research on the role of the immune system. The first has already led to better treatments and the latter may
as well. We now know that energy
conservation (not pushing to the point of pain or fatigue, modifying life-style
to avoid this), gentle conditioning exercises, and appropriate equipment when
needed (braces, electric mobility) can stop progression of post-polio and even
give improvement in all but 5% of sufferers.
This is so predictable, that it almost becomes part of the definition of
post-polio. If you can't stabilize
using these techniques, something else may have been missed. There continues to be evidence suggestive of
the role of the immune system in causing post-polio--more research is ongoing
in the US and other countries to nail this down.
MS How
is PPS treated? Therapies, medications, etc.
DR Energy
conservation, lifestyle modification, gentle exercise (stretch, pool, resistive
work, aerobic), appropriate equipment (canes, braces, electric mobility), and
symptomatic medication for pain and fatigue, in that order. Popular anti-fatigue medications for other
neurologic diseases may not work as well in post-polio, but are often tried
(pyridostigmine, amantadine, selegeline, amphetamine-like agents, selective
serotonin drugs like Prozac).
Supplements like creatine and carnitine have helped some patients and
have shown benefits in research trials.
Nerve growth factor and stem cell research may yield additional treatment
options in the future.
MS Is
there any hope for a "cure"?
DR Yes. A cure could come through the nerve growth
factor and stem cell research, but the best way to live with post-polio now is
with rehabilitation.
MS It occurs to me that those of us who contracted polio are well into middle age now. There cannot be much money behind researching a disease that, given a few more years, will go away on its own. Is PPS unique in this regard?
DR Acute
polio is still occurring and will result in a continuing reservoir of
post-polios for a while. Many other
infectious diseases were thought to be phased out, but have continued to plague
mankind (Tb, syphillis). Because of the
relationship of post-polio to aging and the burden it places on those aging
with it (as does rheumatoid arthritis, cerebral palsy, mid-life stroke),
research in aging will continue to provide resources for post-polio
research. The whole area of neural
regeneration directly impinges upon post-polio, post-Guillan Barre, and other
motor nerve diseases, and will also power future research beneficial to polios.
MS Heartless as it sounds, would we be better off
simply comforting those afflicted and invest our research elsewhere?
DR Certainly,
those with symptoms need to be encouraged and treated, even if not cured, but I
would say NO to investing research dollars elsewhere. Post-polio is a model disease for some key questions in neurology
about nerve regrowth, the reaction of the immune system in old injury, viral
diseases of the nervous system. It has
a lot to teach us.
MS Has
society/the medical community at large already done that?
DR I
think the errors of society and the medical field are more of ignorance rather
than rejection. More education is
definitely needed. In the US there are
more polio survivors at risk for post-polio syndrome than there are people with
MS, so the risk of misdiagnosis and non-diagnosis are much greater given the
numbers.