[Music]
I'm dr. Carol Johnson John's it is my
privilege to serve as president of Alpha
Omega Alpha Honor Medical Society I'm
pleased to be able to introduce this
filmed interview one of the series
entitled leaders in American medicine
this series was made possible by a grant
to AOA for the late Professor David
Siegel and his widow dr. Beatrice Siegel
both of them the distinguished
physicians and members of the Faculty of
Columbia University College of
Physicians and Surgeons the idea of
leaders in American medicine is to
capture on film
the spirit and character of some of our
most distinguished and outstanding
physicians and surgeons in each instance
they are interviewed by students or
colleagues who may be equally
distinguished themselves I'm pleased to
present leaders in American medicine
[Music]
there's only been in recent years that
medical genetics can be considered a
full-fledged medical specialty with its
own board its own textbooks an integral
part of the medical school curriculum is
my pleasure to introduce today one of
the founders and currently still one of
the leaders of this field of medical
genetics dr. Victor a McKusick Victor
how did you ever get into genetics so
many years ago well it's a little bit
difficult to recall I think that I
usually say an answer to that question
that it's because I a tentacled twin I'm
not certain that's the case but it makes
a good story at any rate or other people
perhaps put those words into my mouth
that I was interested in genetics in
college and some of my first clinical
investigation in fact my first piece of
clinical investigation was of a genetics
problem at genetics disorder the perch
jaegers syndrome and then one thing led
to another after that I believe people
sort of always say that do you start off
as a cardiologist is that true and not
really true I think that my cardiology
activities and my genetics activities
developed in parallel and it was more a
matter of cardiology being phased down
and genetics being phased but my first
clinical investigation as I said was on
pets Jagga syndrome and my first book
was on heritable disorders of connective
tissue first published in 1956 my
cardiology texts on cardiovascular sound
was published in 1958 how did you get
involved in cardiology sound remember
that is an interesting story
yes well the that was I really old that
to dr. Elliot Newman who called my
attention to a method for analyzing
speech sound that had been developed at
the Bell Laboratories called sound
spectrography are in a more popular vein
called a visible speech and he thought
that this would be useful for analyzing
the Hertz
and I seized on that and modified it and
adapted it to phone a choreography and
in what we call spectral phone a card er
fee because the frequency spectrum of
the sounds were displayed and so
genetics was really your first love and
cardiology and offshoot of that yes I
think you might say that of course
medical genetics did not exist as a
discipline in which one got training and
and pursued in a specific way and that's
why I went into cardiology but then in
cardiology I came up against the Marfan
syndrome and related disorders that have
important cardiovascular involvement and
I was often running again on on a
genetics area heritable disorders of
connective tissue was your first book
and has gone through several editions
and your name is certainly synonymous
with the field how did this evolve in
terms of these connective tissue
disorders well the Marfan syndrome was
the first member of the group that I
started and it seemed that since this
was a single gene disorder that one
could interpret all the manifold
features of the condition the
pleiotropic manifestations of the
disorder on the basis of a single a
defect presumably in connective tissue
and leading from that I looked around
for other disorders that would answer
the same description and of course came
up with ehlers-danlos syndrome and
osteogenesis imperfecta pseudo xanthoma
elastica and the mucopolysaccharides
o'seas you've always had a great talent
of predicting where a basic defect might
live from looking at the clinical
features of a disease how has this
proven to be true in the heritable
disorders of connective tissue well at
it amazes me and grieves me to a
considerable extent that at this point
31 years after the first publication of
heritable disorders of connective tissue
that we still do not know the nature of
the basic defect in the Marfan syndrome
which seems rather
surprising rather amazing as they say I
think that the the mucopolysaccharides
o'seas has certainly panned out very
well this was a very interesting story
of the correlation between phenotypic
differences the classifications of
different types and the definition of
different defects in the Apollo
saccharide degradation one of the skills
you've had have been to bring together
basic scientists with clinicians in
terms of uncovering these details I
think the story in the
mucopolysaccharides
is particularly rewarding in that yes of
course I owe a great deal to people like
Liz Neufeld in that regard and it would
be very difficult to carry the story to
completion without to the collaboration
of people like her from the connective
tissue diseases
did you is this how you got involved in
the little people and little people of
America yes
substantially that is the case of course
the skeletal dysplasias represent one
type of the connective tissue a problem
and a naturally has a great interest in
in those conditions I think however that
it's safe to say that that was a
practically speaking an outgrowth of the
army study is more than it was an
outgrowth of the the studies in
heritable disorders of connective tissue
because the I was invited to the
National Convention of little people of
America first as a result of some
publicity which studies of ours in the
Amish on dwarfism had attracted there
was a story in the medicine section of
Time magazine 1964 on our studies of two
forms of dwarfism in the in the Amish
and as a result of that one of the
members of the LPA who was
the meeting in New Jersey in 1965
invited me to speak on dwarfism in
general at that meeting and context you
got started at that time what has
happened with you and the little people
I understand you were one of the first
honorary members of that organization
yes an honorary life member of little
people of America a matter that I'm very
proud of and I'm also an honorary fellow
of the American Academy of Orthopedic
Surgeons of perhaps similar reasons
because of my work with skeletal
dysplasias and with limb anomalous and
so that I say I'm the only a member of
those two organizations who was more
than six feet tall and doesn't operate
could how did you ever get involved with
the Amish the I'd like to say that the
involvement with the Amish was sort of a
fringe benefit of University committee
work which often is not very rewarding
but I was a member of the faculty
Advisory Committee for the Johns Hopkins
press at the time they got to the in the
fall of 1962 the manuscript for John
Hostetler's book called Amish society
and this was my first serious
introduction to the Amish and I said my
goodness this is just the group that
I've been looking for all these years
because of their a well-defined
nature of the society because of their
good genealogical records and of course
first and foremost because of their
inbreeding and I teamed up with dr.
Hostetler and we were got going on a
very successful studies within the year
after that over your first studies in
the Amish our first studies were
involved studies of dwarfism because we
understood that that was unusually
frequent in the groups and we were able
to substantiate the fact that this was
the case we found many cases
of a previously described although rare
a form of skeletal dysplasia called the
Ellis van crevel syndrome named for the
two pediatricians will describe it
originally or you know might call it
six-fingered dwarfism and then we
discovered a new form of skeletal
dysplasia that had not previously been
recognized as a distinct entity which
was very frequent among the Amish and we
dubbed that cartilage hair hypoplasia
because in addition to the involvement
of cartilage responsible for the
dwarfism there was thin sparse silky
hair as well as some other
manifestations that was only the
beginning of many other diseases and
groups that you studied tell us a little
bit about the other types of studies
that were conducted in the Amish what
that taught us well the studies in the
Amish have been very useful for
illustrating a number of genetic
principles so particularly the principle
of founder effect the effect of
consanguinity it has been from a
clinical and genetic disorder point of
view very useful in the delineation of
quote new entities entities that have
not previously been described they occur
in all of us but because of one reason
or another particularly the effect that
our families are small and that we do
not marry close and so on have not been
recognized as separate entities
previously of course in the as outgrowth
of studies very useful studies of many
types in the Amish are being done and
there has been sort of in recent times
sort of a renaissance of studies among
the Amish as new molecular genetic
techniques are brought to bear on the
populations
someone asked me recently whether
thought the the Amish studies were over
the question was so as all the
information been wrung out of the Amish
that one can get I think know that it's
really just starting because one sees as
the new molecular genetic techniques are
brought to bear that one can get much
more information out of them the
demonstration of a form of of
manic-depressive illness linked to
markers on a short arm of chromosome 11
for example is a case in point and I I
think there are many other studies of
normal traits for example that can be
investigated in this brings us right
around to what has been a long-term
interest but probably your most current
interests and that is mapping the human
genome you tell us how you first got
started and being interested in this
enormous task of mapping the human gene
map yes I think that I've always been
intrigued by genetic maps when in
genetics textbooks when you saw the
genetic maps of Drosophila of a corn
maze one couldn't help but be impressed
by them and of course the genetic maps
however a skimpy they were at the in the
1950s of the mouse that I couldn't help
but to think that that would be very
exciting to be able to map the genes in
in man and we started a study of mapping
the distance between the colorblindness
locust are low so I one should say since
there are two low sorry and g6pd locust
for example in the early 1960s and we're
able to show that they're quite close
together on the X chromosome and then of
course we conducted other linkage
studies including Donahue's
demonstration of the death
blood group being located on chromosome
one which is the first assignment of a
specific gene to a specific one of the
autosomes did you look at this as sort
of a an area of just peculiar interest
or could you foresee that possible uses
of this which seemed to be coming close
today I certainly before I saw that I
believe I don't want to claim to be any
great prophet I I saw it in the first
place as linkage as a and mapping as a
very useful common theme for a a
division of medical genetics a medical
genetics group because one had to have
the the clinical genetics to describe
phenotype precisely one had to have the
biochemical genetics and the immuno
genetics for markers one had to have the
mathematical genetic spec for linkage
analysis it was a marvelous unifying
theme for the research programs of a
division but at the birth defects
meeting in 1969 and The Hague in the
wrap-up lecture at the end I suggested
that that mapping the human genome would
be the way to go to to unravel the the
mystery is about lots of birth defects
and pointed out that by the linkage
principle one could do prenatal
diagnosis pre-symptomatic diagnosis at
that time of course i was not the first
one to suggest that because already in
1956 john edwards in a letter to Lancet
had suggested that the method of
amniotic fluid sampling which was used
at that time for testing for RH problems
could be used for prenatal diagnosis by
the linkage principle and recently I
have been brought a way out by
colleagues of the fact that hald
and the holding wrote about everything
about 30 years before everyone else but
in the late 1920s in an essay indicated
that in the future people would be would
be making diagnosis by linkage to blood
groups for example which was one of the
main markets at that time there's
currently a controversy as to whether a
large amount of money and resources be
put into completely mapping the human
genome by the year 2000 ad are you in
support of this I think this is a very
reasonable and and exciting thing that
should be undertaken
it said entirely within the realm of
possibility and already the the results
of the gene mapping that have been
achieved to this point illustrate how
useful information will be I think that
we will get ahead much faster if we know
what the full genome genetic
Constitution of man is you lay the
groundwork of this number of years ago
with a creation of Mendelian inheritance
and man or BIM as you like to call it
you tell us how this got started and how
its evolved yes Mendelian inheritance
and man is a catalog of genes
essentially the subtitle is catalogs of
autosomal dominant autosomal recessive
and x-linked phenotypes but this is
really an encyclopedia of genes we it
now has something like four thousand two
hundred and fifty interests we do not
create an entry let me say that we
create only one entry per genetic locus
so that if two genes let us say the R we
know different mutations that one in the
same locus we create only one entry for
both of those two disorders
so this is really a a gives you account
the 40 mm 2200 figure gives you a count
of how many loci
some information is available on a
current computer expansion of this is
going to allow far greater immediate use
of the catalog
yes that's already the case we're very
happy to have it a online version now it
the book has gone through seven editions
it has been on the computer from the
beginning the I might tell you the way
it started was as a catalog of x-linked
traits because when we were studying the
X chromosome in about 1960 we asked the
question what genes are on the X
chromosome and one way of getting an
answer to that is to make a list of all
the conditions of traits that are
x-linked and then we started studying
the Amish in late 62 or 63 and we
expected to find new recessive disorders
among the Amish so in order to know when
we had a new one we had to know what the
old ones were so we made a catalog of
the recessive disorders and then for
sake of completeness we did the dominant
disorders and then about 1964 David
bowling my computer man urged me to put
it on the computer this was before the
day of the word processor because to
avoid the terrific problems of updating
and introducing errors every time you
retype it and so it has been on the
computer since 64 the first edition was
published in 66 the seventh the hardcopy
edition was published last year in 86
and we have an online version which of
course is continuously updated
almost daily and and the this is now
available through the Welsh Medical
Library generally by dialing in how do
you keep this enormous amount of
information updated well the it I of
course screen they dozen of more
journals that probably more that come
across my desk regularly and then I
screen current contents regularly from
likely candidates for inclusion and
those articles that seem promising I get
hold of copies of and and abstract them
for incorporation at the same time of
course I keep up the gene map the the
the my version of the gene map is fully
integrated on in the computer version
with the text so that if you're looking
at a given entry let us say tuberous
sclerosis and you want to know has this
gene been mapped and if so where you can
just press M on the keyboard and that
the machine will tell you whether it has
been mapped and if so where it seems to
me that much of this original
information came from your old journal
clubs where everyone had to hand in
their contributions in written form yes
yes indeed and we were having the
journal clubs which we haven't had for a
few years and that was a very valuable
mechanism for keeping me up to date you
seem to have evolved a lot of meetings
since then and have been quite
instrumental in founding a group of
meetings that are still ongoing many
years later I recall the Jackson lab
meetings of the gene mapping meetings
and the birth defects meetings could you
tell us how each of these got started
and what their values meant well first
of these was the medical genetics course
at Bar Harbor and in and that's put on
by a group of us from Johns Hopkins in
conjunction with members of the staff of
The Jackson Laboratory plus a number of
guests lecturers in recent times the
ratio has been about 12 from Hopkins 12
from the Jackson staff and about 12
guest lecturers the that is of course
not a seminar symposium conference and
so on I've always insisted that that was
the case this was first given in 1960 it
was conceived in 1959 when Anna and I
visited at the Jackson Laboratory and
the and it was precisely conceived as I
recall at testa's restaurant they're
well known the restaurant on Main Street
in Bar Harbor on a warm July knew and
when I was having lunch with John fuller
who was the assistant director for
training at the Jackson laboratory and
it seemed to us that the people at the
Jackson laboratory which of course is a
world premiere laboratory for research
in most genetics that it seemed to me
that they do in the mouse the same sorts
of things that we do in man namely
identified mutations determine or let us
say deviant phenotypes that's not a
prejudice the answer to the question of
whether it is inherited or not they
determine whether it is inherited how it
is inherited what the nature of the
basic defect is and so on and I felt
that the people at the Jackson
laboratory had a great deal to teach us
in medicine and vice versa
also at that time the teaching of
genetics in medical schools was in a
woeful state and one of the big
objectives of the course was to upgrade
the teaching of genetics by teaching the
teachers as it were I seem to remember
that you had a lineup of chairpersons of
various
apartments attending the course of
students yes we always had a fair
collection of a department chairman and
even deans attending the course the the
deans would be there because of the
question of what they should be quote
doing about genetics in their
institutions but for personal
professional reasons as well but we had
representatives from hematology
endocrinology cardiology and so on
who it had very valuable uses for
genetics both in their research and in
their teaching and also he had
card-carrying geneticists or
card-carrying geneticists to be a very
large proportion of people in in
genetics in this country and now
attended the course at some stage of
their careers about the birth defects
conferences how did they get started the
birth defects conferences were started
first in 1968 there was a series of
annual conferences here for one week
each between 68 and 72 inclusive these
were conferences which were entitled the
clinical delineation of birth defects
and they had as their objective the the
review of birth defects in particular
sub specialty areas they were aimed to
bring the the biology the basic science
related to these defects in these areas
to bear and to really get people
thinking about the range of disorders in
particular areas in the the first
conference we discussed the skeletal
dysplasias and limb anomalous phenotypic
aspects of chromosomal aberrations and
so on then we went through disorders of
the nervous system muscle disorders
dermatological disorders disorders of
the eye and ear and and so on and a
systematic way the I think the
conference's themselves and the and the
publications that came out of them I
think we're quite useful we had in
addition to presentations by people from
all over the world in relation to
particular problems we had at midday
each day for the five days of the week
as you'll recall case presentations in
which we would present actual patients
and their families and this I think was
a a very fine part of the of the program
really I think everyone appreciated
seeing patients in the flesh as it were
rather than just hearing about them or
even even seeing them as only
photographs this really did serve as the
model for delineating a lot of
heterogeneity in disease and I call the
meetings moved on to other venues after
that but our returning home next year
yes yes they will be here in Baltimore
again in 1988 to 20 years after the
first of these conferences in 1968 the
other major group of meetings that you
helped found were the gene mapping
meetings which are now becoming enormous
ly popular and successful can you tell
us how those started the first of those
was held in New Haven in 1973 and Frank
Ruddle and deserves as much credit as re
starting those and the it was right at
that point that the gene mapping was
really picking up speed because of the
addition of the method of somatic cell
hybridization to the previous family
method for study and and the it started
as a very small
group with aficionados and they feel
getting together to compare their
results and the now the ninth was just
held in Paris a an enormous meeting with
500 or so people there I suppose but
there's still a sort of a hardcore a
group that has been carrying on the work
it it's interesting that the that the
composition of the people coming has
evolved somewhat the the molecular
geneticists have gotten into the act
fortunately a much more so that those
methods are added to the previous
methods tell us how you got to all this
from a small town in Maine yes right i
developed a fixation on going to Johns
Hopkins to medical school that at one
stage and I applied to only one medical
school and it was Johns Hopkins and I
think the the reason was you know small
things that determine what one does so
often about in Time magazine in about
1938 there was a story about dr. Henry
Siegrist who was the professor of the
history of medicine here and somewhat
controversial and a person and in his
left-wing view was in view in the
opinion of many rather extreme but this
was a very exciting at the paper very
exciting account of how he had come here
as the professor of the history of
Medicine on the invitation of that dr.
Welch and there was quite a lot about
dr. Welch in it also and I thought
Hopkins was really
a place that I wanted to go so I think
that really that was the first time I'd
heard about Hopkins and so I came down
here in arriving in Baltimore for the
first time not having been south of New
York before arriving in Baltimore on
Washington's Birthday 1943 to start
medical school the 1st of March and this
was during the war and the medical
school went around the calendar 9 months
at a time and the I'd been here
uninterruptedly since Washington's
birthday 1943 I'd been a member of the
faculty since 1947 so 40 years you're
well known as a history buff and I guess
the history of medicine brought you here
how is the your knowledge and love of
history affected your career and how
you've tried to use precedents doozie
I think that there's a to a large extent
history and genetics a dovetail very
well the genetics is is really the
history of the organism of the history
of the organism is written in our genome
for for example the that they fit in
very well I I've always been interested
in the history of any area that I've
worked in as you know very much
interested in the history of heart
sounds and and have been certainly
interested in the history of medical
genetics did your love of history
helping your decision to become the
Osler chairman of medicine it didn't
hurt any yes no of course I was
tremendously pleased that point the
point of view I am a undoubtedly a
traditionalist and when the proposal was
made
to me I had to watch myself that I would
be unrealistic in jumping at the
opportunity and the certainly I was very
pleased and honored to to be and the
seventh in the succession from from
mostly as a chairman of Medicine you
spent 12 years I believe as chairman the
Department of Medicine what do you
recall as your joys and successes in
that job well I enjoyed the job at least
I like to say that I enjoy about 97% of
it and I could put up with the other 3%
or that and certainly the contacts with
medical students with who are excellent
at Hopkins with the house officers who
are equally excellent with the fellows
with the a junior faculty and all the
faculty for that matter and the
continuing extensive clinical
involvement I enjoyed very much I think
that that was very important as far as
successes I think that the preservation
and the renovation extension expansion
of the tradition of strong clinical
medicine and strong clinical teaching at
Hopkins the firm system for preserving
the the best qualities of the House
staff teaching program I think those
aspects that I'm proudest of in
connection with my tenure teaching has
always been one of your great forte
certainly as one of your former students
as a fellow I remember that as do all of
the many hundreds I think now fellows
that we know how did you get involved in
the training of medical geneticists and
what has been the the interaction that
has occurred as a result of that
that's somewhat difficult question to
answer it's I think yet the when I I
took you see I took over the operation
of what we renamed the more clinic July
1 1957 and there was a training program
there of fellows that dr. Moore had
initiated and I continued and redirected
that program along genetics lines and we
unfortunately had the funding to develop
a very large a fellowship program and it
was an exciting area for that I think
that's the best way I can answer your
question you often been called an
Anglophile and many of your fellows have
been of from Great Britain could you
tell us how this has influenced your
thoughts yes well some of my very most
able fellows came from England I had
previous to that time we had a good many
friends in England and and and I went to
England on a honeymoon as a matter of
fact and we had the exchanges from guys
Hospital to Johns Hopkins had always
been created a link with England and so
when I became involved in the fellowship
program and the more clinic I contacted
friends in England and who informed
young people that they had registrar's
and suchlike of the opportunity in
Baltimore and many of them came to this
country some now very distinguished
people in medicine both in this country
and in
when your wife Ann has been part of the
training program all along and I
certainly remember that not only as your
wife but she stimulated my first paper
written to Johns Hopkins how did you
meet at and how she's helped in building
this huge group of geneticists well
she's been a very important part of it
needless to say we first met when she
first came to Hopkins to medical school
which was just as I was starting to
enter and she was starting medical
school and we I learned about her
existence from my cousin who had worked
with her at Oak Ridge and and then we
were married at the end of her third
year of medical school
she did she have an interest in genetics
she certainly has had an interest in
genetics her main activity and
subspecialty area is Rheumatology but
she's always had a great interest in
genetics when you started in medical
genetics back in 57 wasn't there weren't
many people in clinical departments
doing medical genetics so now we have an
American Board of Medical Genetics and a
full specialty can you tell us a bit
about your involvement as to how you
helped genetics achieve its role now as
a medical specialty and under the aegis
of dr. Harvey who was chairman of the
Department of Medicine and my
predecessor in that role in July on July
1 1957 I started a division of medical
genetics in the Department of Medicine
here it's of interest that precisely at
the same time in Seattle
I know Motel ski he started a division
of medical genetics within the
Department of Medicine there I think
that this was the first these were
really the first divisions full-fledged
honest-to-goodness divisions within a
major clinical departments started in
this country and
yet they really served as models for
bringing genetics into the mainstream of
clinical medicine where do you see
genetics now is a clinical specialty
well I think it's never had never been
stronger of course seems to have evolved
as a private practice specialty as well
is this something that you envisioned
when you started this division no I
really thought that it was likely that
for the foreseeable future for at least
as long as I could foresee which I
thought was a fairly length length of
time that it would be an academic
Medical Center based activity strictly I
couldn't see it as a private practice
adventure but I think I was
short-sighted as far as that was
concerned now that you have returned
from the host their chairs office back
to your original office and the more
clinic what are your major goals for the
next few years
my main interest these days is in gene
mapping and and this is what what my
main images into these days well what is
your ultimate goal in this season
well I'm pushing hard for a complete
mapping within a reasonable length of
time the human genome Victor you
mentioned that you were a twin and that
interest in twins perhaps was one of the
facts that got you into genetics how are
hell stead of being a twin influence
your development we went a different
pair this when we went to college and of
course Vincent went into the law and I
went into medicine
eventually in many ways I think that law
and medicine are not as Farrah Paradis
might on the surface seem to be the case
but I have this
we have followed his career including
his health status very closely and
because identical twins I of course
believe being a geneticist have closed
health experiences and some years ago I
had a kidney stone and a few weeks after
that my brother had a kidney stone and
and in recent weeks we've discovered
that we developed dental root abscesses
at the same time but the we enjoy seeing
each other of course he's in Maine and
I'm down here but we see each other in
the summertime in particular
occasionally other times were you
competitive as students and your careers
I was not aware of any competitiveness
in the early stages we went to very
small schools we were educated in one
room school house from eight years of
Grammar School in the same one-room
schoolhouse and we were in a very small
high school there were 28 and graduating
members of our high school class and so
and Vincent was the valedictorian and I
was the salutatorian for the in the
graduation ceremony so the the I wasn't
really aware of any great
competitiveness but although I suppose
it was there I understand you recently
went under another convocation ceremony
together oh yes I have several honorary
degrees but the one that I feel
particularly pleased with was one from
the University of Maine a few years ago
it said Vincent and he got honorary
degrees at the same time how did that
occur well he he was the commencement
speaker and that I am told on good
authority that the president of the
university announced that the Board of
Trustees meeting that they had arranged
for the Chief Justice of the state
Supreme Court to give the commencement
address and
namely Vincent mcusic and Tibby Russell
from the Jackson Laboratory who was on
the board of trustees said you should
give an honorary degree to Victor also
so I owe it to Elizabeth she'll wrestle
and I got a degree at the same time oh I
know that you've gotten many honorary
degrees not all of them following your
brother's footsteps can you tell us
which ones were most important to you
well I have several from abroad and
several from this country I have one
from the University of Helsinki which is
very interesting because they give you a
sword when you get the honorary degree
as well as a hat with a green band
around it for medicine this was a very
interesting ceremony and and so on I
value an honorary degree from Edinburgh
and from Liverpool and in from Memorial
University in Newfoundland and and from
the University of Rochester and from
Tufts so where I went to college without
a degree without a bachelor's degree I
have not already a doctorate I came down
here at the Medical School without
completing the addition to your many
honorary degrees you've received
numerous awards one of which I believe
is the Allan award of the American
Society of Human Genetics can you tell
us about some of the others I think the
only other one that I think of is a
Gairdner award which I certainly prize
very much actually in terms of your
writings you've certainly put out
numerous papers and books but you've
also a very active editorship role could
you mention some of these to us I took
over a few years ago from dr. Harvey as
editor of Medicine this is medicine of
course is a very distinguished reviewed
journal that were started in 1922 and I
took it over in my 65th year and it was
the 65th year of
of Medicine when I took it over the in
the second place Frank Ruddle and I have
started a new journal called genomics
which the first edition of which the
first issue of which is appearing this
month
this is genomic says is devoted to gene
mapping and nucleotide sequencing of the
human genome and other genomes you think
the last edition will be in the year
2000 I hope the name genomics
incidentally is is a name for this field
of mapping and sequencing and I think
it's a very useful term it was a 10 that
Tom Roderick at the Jackson Laboratory
suggested and I think it might just
catch on one of your major areas in
teaching has been in teaching the
history of the science that you're
dealing with and as well as the history
of the institution understand that one
of your teaching activities was a tour
of the the Johns Hopkins Hospital to
your medical students could tell us a
bit about that I guess what you're
referring to is the is the tutorial that
I have with the medical students who are
taking the owsla clerkship I meet with
them once a week one of the purposes of
the tutorial is to give them some
instruction in biomedical writing which
they don't get elsewhere in the
curriculum but another purpose is to
give them instruction and medical
history both the history of the
institution as you say and and the
history of a clinical medicine in the
Latta connection I have them take a
particular section of an early edition
of aussolas textbook and compare it with
the comparable section in a present-day
textbook because the objective is to
give them perspective on medicine in
general but the another thing we do
which is fun and I think has some
historical value is at the end of the
quarter the tradition is that we go on a
pilgrimage to the administration
building
and we do two things in particular we go
to the room on the second floor where
OWSLA wrote his textbook in 1891 and
then we go up on the dome I tell them
that's the pinnacle of that clinical
experience at Johns Hopkins among your
activities you've been working many
hours for many years and yet have a very
active family life you tell us how you
managed this and I've been blessed with
three children and none of them are
going into medicine but that's alright
they're doing things that we're very
proud of Victor know that you have
contributed a lot to the genetic
knowledge of your fellows but I
understand that one of your fellows
genomes contributed a lot to our
knowledge genetic map did you tell us
about that
I guess you're referring to the Donoghue
chromosome
- Roger Donahue and his involvement in
demonstrating that Duffy blood groups on
chromosome number one Roger was a fellow
with me and like and the in fact he was
a PhD candidate in human genetics and
like all PhD students should do he
studied his own a karyotype and
discovered that he had an unusual
chromosome number one and which was
unusually long and he had both the the
gumption and the wit to do a family
study realizing that possibly linkage
could be found between this particular
chromosome and some blood group or other
marker and indeed that proved to be the
case Duffy blood group being linked to
this particular unusual chromosome this
was a coarse abnormal variation but it
would be very nice if every fellow would
bring some unusual finding like that
along with it
and he came to work with me a victor
among your many books so one was a pure
short textbook of medical genetics that
human genetics excuse me that I said has
been widely published and in other
languages as well in fact I think I
remember having a delicious meal in
Moscow paid for by the honor area of one
of these books
can you tell us how that of all the this
is my book on human entitled human
genetics which was published by prentice
hall the first edition was in 1964 and
the second edition in 1969 the first
edition was translated into Russian in
1965 I believe of perhaps late 60 for
the lysenko finally went out in 1964 and
my book was sort of johnny-on-the-spot
as a as a book up to date on human
genetics and of manageable size the
Russia did not at that time belong to
the copyright agreement so it was
strictly a a it was plagiarized or it
was pirated it was a case of pirating
but they had told me that if when the
author goes to Russia they will often
give him an honorarium and a reference
in in return for the use of his book and
so when I went there in 1969 with my
whole family its dentally the my host
took me he had dr. bara took me to the
publishing house and I got I think at
224 roubles and and the 30 kopeks or
something of this sort and they asked me
if I had any questions and I I are at
least I asked the question as to how
many copies had been published
and they looked into the matter and
found that 25,000 copies had been
published and that there were no books
in trade they had all been sold this was
more than had been published in English
from the English edition so there was a
tremendous interest in the subject in in
Russia that the money had to be used in
Europe and in Russia and before we left
the country I had not used it all up so
I took out a bank account in Leningrad
and at 3% per annum and I called myself
a Soviet capitalist but when we went
back in 1978 that had grown appreciably
and that we took the money out again and
we still had some money when we left
again so I again took out a bank account
which I still have your career is taking
you to many interesting travels abroad
but also some interesting detective work
in this country could you tell us about
some of the interesting experience
you've had tracing down genetic diseases
I think that's one of the fascinating
things about about medical genetics is
tracking down family lines and so on
some years ago we had a great deal of
fun tracing out the haemophilia in early
New England between 1830 and 1817 there
were four families with hemophilia
described in New England and we had
interesting time bringing those
pedigrees up to date and and so on did
you have any stories for account about
your time with the Amish you certainly
have been in their homes and have
introduced a lot of us to hamish culture
yes well I've enjoyed of course my
contacts with them very much indeed I
grew up on a farm so that I think my
farm background that makes me
sympathetic and and
sensitive to their way of life and so on
but lots of interesting and even some
amusing experiences I remember once at
when Anna and I were staying with an
Amish family in Pennsylvania that we had
first arrived at their home and we were
ushered into the living room
by the white bearded patriarch of the
family and by his wife and and I started
to sit down and they were rocking chair
and she hesitated for a moment and said
to say I'm dead while the patriarch oh
is this your chair and he said they're
all my chairs Victor I know that as one
of your former students and that we've
had several gatherings of the clan now
with three and perhaps the fourth
generation of the McKusick scientific
pedigree on behalf of all of them I'd
like to thank you for your teachings and
your many contributions to the field of
genetics thank you
you