Jim Keenan’s 22 Great Rides will support Fragile X and other rare disorders. 80 yrs old. 22 trails. 2345 Km. 39 days. Extraordinary.
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
More reflections from Jim on the South Island tour. 6 November 2021
Here’s some more notes from Jim on his recent epic cycling journey, with heartfelt thanks to all he met and stayed with on the South Island leg of the journey:
“Monday 11 October we left Picton on the journey first calling at my Number one helper and itinerary organiser Peter Forman's nephew and my great nephew Dave Forman of Imagine Signs, Dave supplied us with monogrammed shirts, flour jackets, and signage for Pete's ambulance free of charge which was much appreciated. Thanks Dave and family.
Onto Christchurch where we stayed with my niece Sharron and her husband John Lee. We hadn't seen each other for years so was a great catchup. Spent two nights with them which we thoroughly enjoyed. While in Christchurch caught up with another Fragile X family at their house for coffee and chats. Wednesday we drove to Dunedin. I think was the only day I never did a ride because of vile weather. Arriving at daughter Kim’s and son in law Adrian’s house and was able to catch up with their Daughter Jaimie and son Brad both of whom have Fragile X we spent a couple of enjoyable hours with them. After a ride the next day, travelled on to my second cousin in Clyde, Kevin Boyle, with whom we were to stay for six nights. Kev and I started school together in Milton so were able to talk about the dim past. While I was riding some of the trails in the area Pete and Kev had a pretty good time in the ambulance telling yarns and generally having fun. Kev was a great host and Pete and I thoroughly enjoyed our time with him.
Was good to catch up with old shearing mate and his wife Snow and Lynne Quin and friends Bill Usher and partner Lynn. We moved on to Cromwell and stayed with brother Speed’s widow Marie who made us very welcome although she got a quite a shock when first seeing me as it was the day I face planted and obviously didn't look too flash. Typical Marie, took it in her stride and she and Pete had me looking my handsome self again. Also enjoyed having dinner with nephew Kelly and neice Levena.
Pete and I went through the Lindis Pass to catch up with Kim, Adrian and Brad at the Ruataniwha camping ground where we met up with another nephew on my wife Jean's side, Peter Lyall. We had a great three days there and some good riding, and think I did about 330 Km in that time.
On the Monday morning we set of for Greymouth with me actually riding a fair bit on the road, when we arrived at the motel which Pete Lyall had booked and paid for it was dark and rain forecast for next morning. This was only time on whole journey we never stayed with family. Met up with another Fragile X family next morning after my ride and enjoyed catching up with them though was only for a few short time as it was quite wet.
Then onto Motueka to stay with neice and husband Alana and Ray Joyce. Had a great catchup and good night's sleep and a ride in the morning with Ray. Also met their son Darryl who I had never met before. While in Motueka met and had lunch with a family with a Fragile X son. He was a delightful young guy and enjoyed our time with them. We then went onto Nelson for afternoon tea with Andrea and Dan Lee. Andrea is CEO of Fragile X NZ. We then went to spend the last night of our journey with Pete Lyall and partner Cheryl and family.”
Jim passes on his very grateful thanks to all those who helped along the way, those who donated to the cause, Waikawa Bay school for their wonderful support, and John and Peter, without who’s help it wouldn't have happened.
Cheers to all, Jim