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Surgical
Options
for
Alpha-1
There
are
two
major
types
of
surgery
for
patients
with
alpha-1
antitrypsin
deficiency:
Lung
volume
reduction
surgery
(LVRS)
Lung
volume
reduction
consists
of
cutting
out
damaged
portions
of
the
lung,
thereby
reducing
its
size
by
around
20%.
It
may
seem
strange
to
remove
part
of a
lung
when
a
patient
has
problems
breathing,
but
the
basis
behind
it
makes
sense.
With
emphysema,
the
alveoli
in
the
lungs
hyperinflate,
become
enlarged
and
less
efficient
in
assisting
with
the
exchange
of
blood
gases.
As a
result,
the
over-expanded
lung
pushes
against
the
healthier
portions
of
lung,
decreasing
its
effectiveness. It
also
pushes
against
the
diaphragm,
causing
it
to
lose
its
natural
shape
and
effectiveness.
When
damaged,
overinflated
portions
of
the
lungs
are
removed,
more
room
is
created
for
the
diaphragm
to
move
and
for
the
normal
parts
of
the
lungs
to
inflate
and
deflate.
Patients
who
have
this
surgery
performed
often
find
that
their
breathing
becomes
easier,
and
their
PFTs
often
show
a
substantial
improvement.
With
a
bullectomy,
the
surgeon
uses
various
techniques
to
locate
the
large
bullae
(hyperinflated
alveoli)
and
cuts
them
out.
Lung
volume
reduction
takes
out
smaller
damaged
areas
from
around
the
lungs.
Surgeons
have
good
results
using
a
stapling
technique
-
where
the
damaged
sections
are
removed
and
the
remaining
tissue
is
stapled
closed.
Some
other
methods
use
laser
ablation,
and
this
is
also
used
in
conjunction
with
the
stapling.
Use
of
the
laser
in
this
surgery
generally
yields
poorer
results,
and
most
centers
performing
lung
reduction
use
it
sparingly
if
at
all.
While
a
majority
of
lung
reduction
patients
show
an
improvement,
some
do
not.
Standards
have
not
been
established
for
choosing
the
most
suitable
candidates,
mostly
because
studies
have
not
ascertained
the
clinical
reasons
for
success
behind
this
surgery.
Initially
there
was
a
high
mortality
rate
for
this
type
of
surgery,
although
that
is
decreasing.
Air
leaks
that
develop
in
the
lung
tissue
often
create
problems
after
the
surgery.
There
is
also
the
possibility
of
an
infection
developing after
the
lung
volume
reduction.
When
the
procedure
works,
it
seems
to
help
for
at
least
a
year.
The
surgery
is
new
enough
that
long-term
results
can
not
be
measured
at
this
time.
Alpha-1
patients
have
undergone
this
surgery,
with
mixed
results.
Some
have
greatly
improved
their
lung
function,
while
others
note
little
or
no
change.
One
of
the
problems
alphas
face
with
lung
volume
reduction
is
that
their
lung
damage
is
often
quite
diffuse
with
no
relatively
good
lung
to
expand
and
to
have
improved
function
in
response
to
the
surgery.
Although
there
are
many
questions
about
lung
volume
reduction
that
have
yet
to
be
answered,
there
is
enough
promise
in
this
surgery
to
continue
with
it.
There
is
also
a
major
clinical
trial
planned
that
hopefully
will
answer
many
questions
and
provide
safer,
more
effective
methods
of
reducing
lung
volume.
Organ
transplantation
of
lung
or
liver
Lung/liver
transplantation
is
becoming
a
viable
option
for
some
patients.
As
experience
with
new
surgical
techniques
increases
(particularly
single-lung
transplantation),
lung
transplantation
may
become
more
attractive
to
Alpha-1
patients
with
end-stage
lung
disease.
Also,
living-related
liver
transplantation
is
an
option
at
some
transplant
facilities.
Transplantation
is
only
for
patients
with
end-stage
lung
or
liver
disease,
which
means
that
they
are
resistant
to
more
conservative
therapies
(such
as
treating
symptoms
or
augmentation
therapy).
Patients
must
have
extensive
damage
in
the
lungs
or
liver
to
warrant
this
surgical
option.
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