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Chapters

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