Rare diseases community rallying for earlier & accurate diagnoses

The rare diseases community is joining forces today (Rare Disease Day) to call upon medical professionals for earlier and more accurate diagnoses of rare diseases to help improve, prolong and save Aussie lives.

According to Genetics Alliance Australia (GAA) and Gaucher Association Australia & New Zealand (GAANZ), nearly two million Australians are living with a rare disease. More than one-in-two rare diseases primarily affect children. Due to their rarity and complexity, they are difficult to diagnose, forcing those affected to endure up to five years before diagnosis.

“Tragically, up to 30 per cent of rare disease patients will die before the age of five,” said Jan Mumford, Executive Director, GAA, Sydney.

“This significant loss of life is unjustifiable. It is therefore imperative that the medical community act now to offer Australians living with a rare disease the best chance at life.”

Leading geneticist and physician, Clinical Professor Jack Goldblatt AM says reducing the substantial delays in diagnosis of rare diseases is key to improving patient outcomes.

“Rare diseases are complex and progressive disorders which are often life-threatening and incurable. That’s why an earlier and accurate diagnosis is critical to both improving and prolonging a patient’s quality of life.

“The effective management of rare diseases, which requires multiple specialists and the coordination of shared medical information between treating healthcare professionals and clinics, is crucial to optimising patient care,” Prof Goldblatt said.

There are up to 8,000 recognised rare diseases, with new diseases identified each week. Although a single rare disease affects less than 0.05 per cent of the population, collectively rare diseases affect 350 million people worldwide, including up to six per cent of Australians, similar to the prevalence of diabetes and asthma.

Symptoms of rare diseases can appear deceptively common and often mask the true diagnosis. Moreover, rare disease symptoms may go unrecognised by doctors and nurses who are yet to encounter them in clinical practice.

Delayed and inaccurate diagnoses of rare diseases not only place a substantial emotional and financial burden on those affected and their families, but can lead to inappropriate management and potentially devastating, aggressive disease progression.

High-school student and 2019 Young Australian of the Year Nominee, Gidon Goodman, 16, Sydney, was diagnosed with Gaucher’s disease – a rare genetic condition afflicting just 100 Australians – at only three years of age. Together with his supportive mother Karen, he founded GAANZ in 2018 to facilitate community education, advocacy and support of research for those living with Gaucher’s disease.

“My parents and I spent the first three years of my life visiting specialist after specialist in a desperate search for a diagnosis. During this time, I was misdiagnosed with both coeliac disease and leukemia. After an extremely frustrating and heart-wrenching three years, I was finally diagnosed with Gaucher’s,” said Gidon.

“Had I not been diagnosed at such a young age, I would probably be living with the permanent and debilitating effects of Gaucher disease, including stunted growth and bone death.

“It was only because I was then granted fully subsidised access to ongoing treatment through the Federal Government’s Life Saving Drugs Program, that I am here today, leading a normal, healthy life,” Gidon said.

“This Rare Disease Day, together with the broader rare diseases community, I am calling upon the Australian medical community to heighten their understanding of rare diseases in order to drive earlier and more accurate diagnosis, help alleviate unnecessary heartache, and improve patient lives.”

Significantly, up to 80 per cent of rare diseases are genetic. Through improved understanding of rare diseases and continuing advances in diagnostic technology, many of these genetic changes can now be detected through genetic testing.

According to Jan Mumford, genetic services can assist with the testing, diagnosis, management and counselling of those living with a rare disease.

“The complexity of rare, genetic diseases can make them extremely difficult to diagnose, leading to a ‘diagnostic odyssey’ where patients consult with a number of specialists before obtaining a definitive diagnosis,” said Ms Mumford.

“Although use of genetic testing is increasing in Australia, earlier employment of this targeted screening tool for rare diseases could improve overall patient outcomes.

“We are celebrating Rare Disease Day today to raise awareness of the tools and resources available to healthcare professionals to aid in the management of rare diseases. Through inter-disciplinary education and collaboration, we can work together to improve the outcomes of all Australians affected,” Ms Mumford said.

To learn more or to support Rare Disease Day, head to www.geneticalliance.org.au/index.php and www.facebook.com/GaucherANZ