Diagnosing AL amyloidosis early,
it relies on increasing awareness.
Once you have unexplained nephrotic syndrome
unexplained congestive heart failure, unintentional weight loss,
peripheral and autonomic neuropathy, and even acquired Factor X deficiency
leading to coagulopathy, the suspicion
for AL amyloidosis should be high.
The therapeutic landscape for systemic AL amyloidosis
has changed and expanded dramatically
in the last 20 years, and that is the span of my career.
So the treatments are twofold.
The first is the definitive treatment
attacking the plasma cell dyscrasia
and the second treatment is the supportive treatment
is based on expertise from subspecialties
to make patients’ quality of life better
by controlling congestive heart failure
and nephrotic syndrome and autonomic neuropathy.
But the treatments that are directed towards
the plasma cell dyscrasia reduces the production
of the amyloidogenic light chains to improve the organ dysfunction
and, hence, improve the outlook and prognosis of patients with AL amyloidosis.
Even before you start treatment,
multidisciplinary collaboration is key in figuring out
if a patient is a candidate for aggressive treatment.
And I think—we do this every Friday at the Amyloidosis Center
here where we sit down, and we discuss every patient.
And Dr. Sanchorawala will ask us as cardiologists
do you think this patient can tolerate the treatment that I’m thinking of?
Do you think this patient can tolerate
aggressive treatment because of their heart involvement?
So, I think the cardiologist takes a really important role
in figuring out if a patient is a candidate
for aggressive anti-plasma cell treatment
or for modifying that treatment
so that they are candidates for treatment.
I’m sort of really impressed by how far we’ve come in this field,
and I think that there is real promise
to take this disease that used to be uniformly fatal
and convert it into more of a chronic disease that we can manage.
Really the next step in this treatment, in this disease,
is to figure out how to prevent
progression of the organs that are involved.
Remember, that you're working on 2 levels here.
You're working at the level of a plasma cell dyscrasia
for bone marrow disorder, but then, this
amyloid protein is depositing in organs and causing organ failure,
heart failure, kidney failure, nervous system problems, GI problems.
I think that’s really where the future is,
and there's a lot of interesting developing things like that
when somebody develops signs of organ damage,
you know, how do you reverse that?
And I think that is going to be the next frontier for us.
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