We are thrilled to report that our friend and former trustee Ranga has recently been awarded a Member of the Order of the British Empire (MBE), recognising his tireless work to deliver the first ever licenced therapy for the rare disease Alkaptonuria (AKU). Though Ranga is always at pains to point out the huge contributions of a whole team of people who helped to bring the treatment Nitisinone to Alkaptonuria patients around the world, his leadership, focus and integrity have always been a vital cornerstone of that work.
Ranga is a founding member, medical director and trustee of The AKU Society. He established an NHS Highly Specialised Services and funded the National Alkaptonuria Centre (NAC), which is the world’s first centre for the treatment of AKU
About AKU
AKU, also known as Black Bone Disease, is an ultra-rare genetic condition that affects the bones, cartilage and tissues of those affected. This is caused by the body’s inability to break down the chemical homogentisic acid (HGA), which is naturally produced during the digestion of food. This leads to a build-up of HGA, causing brittle bones and early onset osteoarthritis. Only 1 in 250,000 people worldwide are affected.
About Nitisione
The new treatment, Nitisione, the development of which Ranga has played an instrumental part in developing, blocks the enzyme that makes HGA. This stops HGA from building up in the body and causing harm. Nitisnone is now officially licensed to treat AKU in the European Union.
Our CEO Rick Thompson’s message to Ranga on this well-deserved award:
It has always been a pleasure to work with and talk to Ranga over the last ten years. His drive and determination to deliver the best science and care for those living with AKU has been transformative for that community, and a point of light for all rare diseases. It is wonderful to see all Ranga’s work recognised with an MBE, and I offer him my warmest congratulations!
See Ranga in action at a Beacon event!
Watch Ranga shine delivering “The power of rare disease study and repurposing drugs: the nitisinone story“, a talk at our 2022 Drug Repurposing Conference!
The whole Beacon team extends our congratulations to Ranga, and we would like to thank him again for always being a friend and support to the whole rare disease community.
Interested in learning more about what it’s like to live with AKU or work with the National Alkaptonuria Centre (NAC)?
Listen to Season 2 of our Rarely Heard podcast, which delves more into living with AKU:
Season 2, Episode 11: Alkaptonuria: One man’s rare disease journey from Ukraine to the UK Hear about Kostiantyn’s experience of accessing treatment for AKU in the UK after fleeing his home in Kyiv, Ukraine, in February 2022, once the bombing started.
He talks of:
- How he manages his love of sport and determination to reach his goals while managing the pain caused by AKU
- What it was like to flee with his wife and be told that he was unable to join the Ukrainian military due to his rare disease
- Struggling with his new imposed identity of ‘refugee’
Season 2, Episode 12: Alkaptonuria: The value of working closely to empower patients Listen to Clare Soulsby (senior dietician) and Helen Bygott (nurse specialist), who work at the National Alkaptonuria Centre (NAC) in Liverpool, talk about the new treatment for AKU, Nitisinone, which Kostiantyn was able to access at NAC when he reached the UK.
