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