The Princess Alexandra Hospital (PAH) Amyloidosis Centre aims to improve the diagnosis and treatment of all patients with amyloidosis. This is being done through a program of co-ordinated clinical care, patient education and support, training of doctors and research into better diagnostic and monitoring tools, as well as clinical trials of new treatments.

PAH Amyloidosis Clinic

The clinic was established in 2009 and offers services to patients with all types of Amyloidosis including:

• Diagnosis of amyloidosis
• Amyloidosis typing
• Assessment of organ and tissue damage
• Treatment advice
• Treatment care
• Referral for genetic testing
• Information about available treatment trials
• Support and education.

The clinic is staffed by a team of health professionals which includes cardiologists, haematologists, neurologists, renal physicians, pathologists, specialised nurses, social workers and a support and education officer. Other specialist services from within the PAH are available depending on individual needs.

You may see one or more or all of these specialist doctors depending on the type of Amyloidosis you have and which of your organs is affected by the amyloid.

What is amyloidosis?

Amyloidosis is a term for a group of devastating, complicated and rare disorders. Amyloidosis is caused by the abnormal folding, aggregation and accumulation of abnormal protein fibrils in organs and tissues of the body. These abnormal protein deposits (amyloid) disrupt normal body function and, without treatment, will eventually lead to death. There are more than 20 different types of amyloidosis. It is not a cancer but is equally serious and in most cases is incurable at this time.

The main types of Amyloidosis are:

• AL Amyloidosis (immunoglobulin light chain amyloidosis) – This systemic disease is usually caused by a bone marrow disorder.
• AA Amyloidosis – This type of amyloidosis is caused by deposition of the serum amyloid A protein (SAA) when SAA is elevated over many years due to long-term inflammatory disease or infection.
• Hereditary/Familial Amyloidosis – There are a number of types of hereditary amyloidosis, caused by an inherited gene mutation, which leads to a lifelong production of the abnormal protein amyloid. The most common types of hereditary amyloidosis are ATTRmut (transthyretin gene mutation) and AFib (fibrinogen alpha chain gene mutation).
• Wild Type Transthyretin Amyloidosis, ATTRwt, previously known as Senile Cardiac Amyloidosis. This type of amyloidosis occurs when transthyretin amyloid deposits in the heart. As opposed to ATTRmut, this type of amyloidosis is NOT an inherited disease.
• Localized Amyloidosis – In this type of amyloidosis the amyloid protein is both produced and deposited in a single organ.

For further information on the types of amyloidosis refer to the National Amyloidosis Centre London, web site. www.ucl.ac.uk/amyloidosis/nac

Diagnosis

The diagnosis of amyloidosis is difficult. Because the symptoms of amyloidosis mimic other diseases it is often not diagnosed early. The presence of amyloidosis can only be confirmed at this time by a tissue biopsy. Further tests are then carried out to establish which of the many different types of amyloidosis a person has and to what degree any organs and tissues are involved.

The PAH Amyloidosis Centre offers high-level amyloid diagnostic and typing services not available elsewhere in Australia. Sometimes this typing is difficult and the results may take some weeks before they are known. However it is essential that your type of amyloidosis is correctly typed, as treatment is different for the different types of amyloidosis.

Support and education

When you first attend the clinic you will receive the booklet “Understanding Amyloidosis”. Reading the whole of this book will give you a general idea about all the types of amyloidosis. However many people prefer to look at the index and read the general section on amyloidosis and then turn to the relevant pages describing the type of amyloidosis you have been diagnosed with.

Once the type of amyloidosis you have has been made you will be offered further information and encouraged to ask all your questions so that you can learn about your treatment and how to live with amyloidosis.

Individual support will be offered at the clinic by the medical staff, specialised nurses, the social workers and the support and education officer.

From time to time the Amyloidosis Centre arranges support and education meetings. View our events page for information on upcoming events.

The Leukaemia Foundation Australia (LFA) conducts:

• Regular education and support meetings in the capital cities.
• Publishes a quarterly newsletter.
• Moderates a closed Facebook support group for people living with amyloidosis in Australia https://www.facebook.com/groups/AMYLFA/
• Runs Australia wide telephone education forums.

For more information about the Leukaemia Foundation programs ring 1800 620 6951 (Free call).

More information

Overseas Newsletters

The National Amyloidosis Centre (NAC) in London publishes an online quarterly newsletter containing medical information and patient stories. The NAC site also contains patient information leaflets and a great deal of other useful information for patients and health professionals. To explore this site go to www.ucl.ac.uk/amyloidosis/nac

Other useful sites

• Amyloidosis Australia
• Amyloidosis Foundation (US)
• Amyloidosis Support Groups (US)
• Kidney Health Australia
• Mayo Clinic Amyloidosis Centre
• Myeloma Australia
• Myeloma UK
• The Heart Foundation
• UK National Amyloidosis Centre

Research

The PAH Amyloidosis Centre has also been a leader in amyloidosis research in Australia.

Together with Pathology Queensland PAH Laboratory and the UQ Diamantina Institute we introduced laser capture microdissection and tandem mass spectrometry analysis to accurately diagnose amyloidosis subtype. This is currently recognised as the gold standard diagnostic technique and the PAH Amyloidosis Centre is the only Australian site to offer this service.

The Centre has also led Australia’s only assessment of existing and new assays to monitor response to therapy in AL amyloidosis, research made possible by the prospective banking of samples from patients who attend the Amyloidosis Clinic.

The Centre, in collaboration with the Departments of Radiology, Nuclear Imaging and Cardiology, has conducted research into new techniques to image cardiac involvement in amyloidosis. These studies have demonstrated the particular usefulness of DPD scanning in senile cardiac amyloidosis and shown, in a world first, that Florbetaben can be used to image cardiac amyloidosis by PET/CT.

The Centre has also been heavily involved in clinical trials of new therapies leading Australia’s only two prospective investigator initiated clinical trials in AL amyloidosis as well as conducting Pharma sponsored studies in AL amyloidosis.

Donate

To help The Amyloidosis Centre click here to Volunteer or Donate via the PA Research Foundation.