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00:00:15
Diagnosing AL amyloidosis early,
00:00:18
it relies on increasing awareness.
00:00:22
Once you have unexplained nephrotic syndrome
00:00:25
unexplained congestive heart failure, unintentional weight loss,
00:00:29
peripheral and autonomic neuropathy, and even acquired Factor X deficiency
00:00:35
leading to coagulopathy, the suspicion
00:00:39
for AL amyloidosis should be high.
00:00:47
The therapeutic landscape for systemic AL amyloidosis
00:00:51
has changed and expanded dramatically
00:00:55
in the last 20 years, and that is the span of my career.
00:01:00
So the treatments are twofold.
00:01:01
The first is the definitive treatment
00:01:04
attacking the plasma cell dyscrasia
00:01:07
and the second treatment is the supportive treatment
00:01:10
is based on expertise from subspecialties
00:01:15
to make patients’ quality of life better
00:01:19
by controlling congestive heart failure
00:01:22
and nephrotic syndrome and autonomic neuropathy.
00:01:26
But the treatments that are directed towards
00:01:29
the plasma cell dyscrasia reduces the production
00:01:32
of the amyloidogenic light chains to improve the organ dysfunction
00:01:37
and, hence, improve the outlook and prognosis of patients with AL amyloidosis.
00:01:44
Even before you start treatment,
00:01:46
multidisciplinary collaboration is key in figuring out
00:01:50
if a patient is a candidate for aggressive treatment.
00:01:55
And I think—we do this every Friday at the Amyloidosis Center
00:01:58
here where we sit down, and we discuss every patient.
00:02:00
And Dr. Sanchorawala will ask us as cardiologists
00:02:05
do you think this patient can tolerate the treatment that I’m thinking of?
00:02:08
Do you think this patient can tolerate
00:02:10
aggressive treatment because of their heart involvement?
00:02:13
So, I think the cardiologist takes a really important role
00:02:16
in figuring out if a patient is a candidate
00:02:19
for aggressive anti-plasma cell treatment
00:02:21
or for modifying that treatment
00:02:24
so that they are candidates for treatment.
00:02:32
I’m sort of really impressed by how far we’ve come in this field,
00:02:34
and I think that there is real promise
00:02:37
to take this disease that used to be uniformly fatal
00:02:41
and convert it into more of a chronic disease that we can manage.
00:02:46
Really the next step in this treatment, in this disease,
00:02:48
is to figure out how to prevent
00:02:50
progression of the organs that are involved.
00:02:53
Remember, that you're working on 2 levels here.
00:02:56
You're working at the level of a plasma cell dyscrasia
00:02:59
for bone marrow disorder, but then, this
00:03:02
amyloid protein is depositing in organs and causing organ failure,
00:03:05
heart failure, kidney failure, nervous system problems, GI problems.
00:03:10
I think that’s really where the future is,
00:03:11
and there's a lot of interesting developing things like that
00:03:14
when somebody develops signs of organ damage,
00:03:17
you know, how do you reverse that?
00:03:19
And I think that is going to be the next frontier for us.