Understanding Amyloidosis.Peter Munk Cardiac Centre

Dr. Diego Delgado, cardiologist, Peter Munk Cardiac Centre and leading North American expert in Amyloidosis answers common questions about the potentially fatal, rare disease.

In June 2014, former NHL player Gino Odjick was diagnosed with AL Amyloidosis, and given only weeks to live, casting the spotlight on this often devestating disease with affects up to 3,000 Americans every year.

Here's what Peter Munk Cardiac Centre cardiologist Dr. Diego Delgado says about AL Amyloidosis:

What is AL Amyloidosis?

It is a very rare blood disorder that is caused by the deposition of a protein,called the amyloid protein, in various organs and tissues.

How is the protein produced?

This protein is usually produced by cells, normal cells, that are in the bone marrow. The protein goes to the circulation and deposits in all these different organs, producing different types of damages in all these organs.

What is the prognosis for a patient with AL Amyloidosis?

Usually, it's not detected in the early stages of the disease. By the time patients go to the consult and this disease is diagnosed, sometimes it's too late. In those patients, particularly if they have multiple organs involved, the prognosis is poor -- maybe one or two years. However, in those patients in which the disease is detected early and the treatment is started early, usually the prognosis is good and they survive more than five years after specific treatment.

Why is it difficult to diagnose AL Amyloidosis early on?

Because this disease usually affects multiple organs and there is usually a range of variable symptoms -- from very non-specific symptoms such as weight-loss, tiredness, shortness of breath and leg swelling to more advanced symptoms. But the patient can be with all these vague symptoms for six months up to one year, going to different tests, going from doctor to doctor until finally they have their specific test done where you can reach the diagnosis.

What are the treatment options for early onset AL Amyloidosis patients?

If the disease is detected in the early stages, most of these patients can receive specific treatment -- which is basically chemotherapy. The treatment, and of course the prognosis depends on how aggressive the disease is, the age and general condition of the patient, how many organs are involved, and how soon the treatment starts. All these factors will help determine which patients will do well in the long term.

What are the treatment options?

Chemotherapy -- and there are different forms of chemotherapy, from oral to intravenous chemotherapy. And in some cases, maybe 10 or 20 per cent of patients, they can receive a stem cell transplantation.

Watch the video for a full version of the Q&A with Dr. Deigo Delgado, a cardiologist at the Peter Munk Cardiac Centre and a leading expert on AL Amyloidosis.

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