Jim Keenan's 22 Great Rides

Famous in NZ shearing circles, “the man who never gave up”, 1986 NZ Fine Wool Merino champion shearer Jim Keenan, is riding more than the length of NZ (a mere 2074Km) by doing 22 of the great NZ cycle rides, to fundraise for Fragile X New Zealand (75% of funds raised) and Rare Disorders NZ (25%).

Jim hasn’t let his 80 years slow him down. He started shearing in 1957, has other individual and team shearing titles in South Africa and Australia in the 1980s, and done many farm and outdoor jobs since. It seems he never stops. He still has a part-time role as a school caretaker.

Now he’s off on a 2345 Km ride, starting on Saturday 25 September in the Waikato, subject to Covid of course, riding for 39 of the next 45 days, in honour of his 3 grandchildren who are affected by Fragile X. All going to plan he will ride back into his hometown of Picton in early November.

80 yrs old. 22 trails. 2345 Km. 39 days. Extraordinary.

What is Fragile X Syndrome?

Fragile X Syndrome (FXS) is the leading inherited genetic cause of intellectual disability worldwide and the most common single gene cause of autism. It often impacts multiple family members and is intergenerational. Characteristics of FXS include mild to severe learning difficulties, high anxiety and autistic like behaviour, speech and communication challenges, sensory sensitivities, ADHD and some physical features.

How is it inherited?

Fragile X is a genetic disorder that affects about one in 4000 males and one in 6000 females. As many as one in 130 women and one in 800 men may be carriers of the FMR1 gene mutation on the X chromosome. CGG repeats in the gene can increase over the generations, and if the mutation has a long series of repeats, the likelihood arises of any child being affected by Fragile X. Female carriers have a 50% chance of passing the gene mutation to their children and male carriers will pass it to 100% of their daughters but none of their sons.

Some carriers may be at risk of developing Fragile X-associated conditions, including early menopause (FXPOI) and a neurodegenerative disorder (FXTAS).

Why is a diagnosis of Fragile X important?

Although there is currently no cure for Fragile X syndrome, a diagnosis can lead to targeted support, intervention and treatment that helps individuals reach their full potential and assist families. The information obtained from the test can help identify other family members carrying the gene change.

About the Fragile X New Zealand Trust (FXNZ).

FXNZ supports individuals, families and communities affected by Fragile X syndrome and Fragile X-associated disorders through a freephone support line, a website and social media sites, and run annual family gatherings and workshops. They also coordinate a nationwide family support network, run conferences, education workshops in schools, an online Parent Education Programme and a Fragile X clinic where families can have their FX child assessed and given specialist support.

See more at: www.fragilex.org.nz

About Rare Disorders New Zealand (RDNZ).

RDNZ is the connector hub for people affected by rare disorders and their families to find essential information and links to rare disorder support groups. RDNZ is the only umbrella group for rare disorders in New Zealand and provides a strong common voice to advocate for an equitable healthcare system that works for the 300,000 Kiwis with a rare condition - around 6% of the population.

RDNZ are calling for acknowledgement and awareness of the common challenges faced by people living with a rare disease, along with a commitment to address these challenges through the development of a New Zealand National Rare Disorder Framework. We want inclusion of patients and clinicians in decision-making processes, consideration of a wider definition of patient quality of life, and impact of rare disorders for carers.

See more at: www.raredisorders.org.nz