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Drug
Therapy
for
Alpha-1
This
is
among
the
most
important
type
of
medical
therapy
for
the
newly
diagnosed
individual
with
Alpha-1
antitrypsin
deficiency.
Although
these
are
most
relevant
to
lung-affected
individuals,
remember
all
Alphas
have
risks
to
their
lungs.
Some
of
the
therapies
listed
are
relevant
for
all
Alpha-1
patients.
1.
Vaccinations
(influenza/pneumonia)
It's
important
to
have
a
yearly
flu
vaccine
and
a
Pneumovax®
shot
every
five
to
six
years.
Since
your
lungs
are
vulnerable
to
pollutants
and
infections,
the
use
of
these
prophylactic
vaccinations
is
of
the
utmost
importance.
Furthermore,
your
patient
may
find
this
is
the
easiest
and
most
convenient
type
of
therapy
available.
Effective
vaccines
are
available
for
hepatitis
A
and
B.
These
are
especially
important
in
patients
with
established
liver
disease.
Recommendations:
- Annual flu vaccine
- Administration or confirmation of Pneumovax vaccine (every 5 to 6 years)
- Hepatitis A vaccine
- Hepatitis B vaccine
2.
Aggressive
treatment
of
lung
infections
Prompt
and
aggressive
treatment
of
infections
is
recommended
due
to
the
increased
neutrophil
elastase
burden
during
periods
of
infection.
It's
important
to
notify
your
doctor
immediately
when
you
suspect
a
lung
infection.
Here
is a
list
of
symptoms
you
should
watch
for
carefully:
- fever
- increased shortness of breath
- increase coughing (may not be productive)
- chills with fever
- changes in color of phlegm
Because
the
lungs
attract
more
leukocytes
when
an
infection
is
present,
and
the
leukocytes
release
neutrophil
elastase,
it
is
important
to
control
lung
inflammation.
Antibiotics
may
help
to
speed
recovery.
3.
Aggressive
Evaluation
of
Liver
Complications
It
is
important
for
parents,
caregivers
or
significant
others
to
be
aware
and
advised
of
any
indication
of
complications
related
to
liver
disease.
Here
is a
list
of
common
symptoms
that
may
require
therapy:
- increased abdominal swelling or edema of the extremities
- coughing up or vomiting bright red blood
- blood in toilet or diaper
- blackish, purplish or dark-colored stools
- confusion, crankiness, unusual crying, disorientation, lethargy
- little or no urine
- dark (tea- or cola-colored) urine
- lack of energy, easily fatigued
- fever
- no appetite/refusal to eat or drink
- itching or increased itching
- peripheral edema
- change in or the appearance of jaundice
It's
very
important
to
carefully
read
the
labels
on
over-the-counter
medications.
Be
sure
to
inform
your
doctor
or
other
healthcare
provider
if
any
alternative
medicines
(such
as
Milk
Thistle)
or
vitamin
supplements
are
being
taken.
4.
Bronchodilators
Your
doctor
may
prescribe
bronchodilators
to
relive
your
Alpha-1
lung-related
symptoms.
Bronchodilators
relax
the
large
muscles
around
your
airways
so
that
more
air
is
allowed
in
and
out.
There
are
two
main
kinds
of
bronchodilators:
beta-agonists
and
anticholinergics.
An
example
of a
beta-agonist
is
the
quite
common
asthma
medication
albuterol.
Beta-agonists
are
typically
quick-acting
"rescue"
medications.
They
work
in 5
to
15
minutes
and
last
from
4 to
6
hours.
Anticholinergics
are
longer-lasting
than
beta-agonists,
but
still
fall
in
the
quick-acting
category.
An
example
of
this
subclass
is
Atrovent.
5.
Corticosteroids
Inhaled
corticosteroids
can
be
useful
as a
preventive
treatment
for
Alpha-1
lung
disease
and
oral
corticosteroids
may
be
prescribed
by
your
doctor,
particularly
during
flare-ups
(which
are
referred
to
by
healthcare
as
"exacerbations").
6.
Supplemental
Oxygen
Supplemental
oxygen
may
be
needed
for
some
alpha-1
related
lung
disease.
Oxygen
is
important
for
individuals
with
low
blood
oxygen
levels,
during
active
infections
and/or
with
progressive
destruction
of
the
lung
tissue.
Supplemental
oxygen
may
be
needed
during
exercise
or
sleep.
Supplemental
oxygen
is
also
recommended
during
exercise.
For
some
Alphas,
it
is
especially
important
when
traveling
by
air,
because
cabin
pressure
changes
with
altitude.
7.
Augmentation
Therapy
Augmentation
Therapy
[link:
augmentation
therapy]
may
be
recommended
for
certain
patients
with
lung
disease.
It
is
not
used
for
patients
with
liver
disease.
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