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Long Sutton mother running London Marathon in aid of RNIB to raise funds and awareness for her son, 19, now registered blind





A mother experienced every parent’s worst nightmare when a rare condition robbed her teenage son of his sight - and is now fighting for equal access to revolutionary medication.

Motorcycle enthusiast Jacob Ely, 19, is now no longer able to ride his bike and also lost his job after suddenly going blind due to Leber's hereditary optic neuropathy - which could also affect his siblings.

But there is ‘light at the end of the tunnel’ for the Long Sutton youngster as the Idebenone medication is helping to improve his eyesight.

Since losing his sight Jacob can no longer ride a motorbike and no longer has a driving license due to his condition. PHOTO: SUBMITTED
Since losing his sight Jacob can no longer ride a motorbike and no longer has a driving license due to his condition. PHOTO: SUBMITTED

But a postcode lottery means his parents, Kimberley and James, have to pay for it – despite the drug being freely available in Scotland and Wales.

Kimberley is calling on NICE (National Institute for Health and Care Excellence) to end this unfair situation later this year.

The 42-year-old mother of three, who is taking on the London Marathon to raise money for the Royal National Institute of Blind People (RNIB), said: “Jacob is a young man that had his whole future ahead of him.

James, Nicholas, Jacob, Stephanie and Kimberley Ely. PHOTO: SUBMITTED
James, Nicholas, Jacob, Stephanie and Kimberley Ely. PHOTO: SUBMITTED

“Knowing that there is a drug out that has a huge chance of giving him his sight back is such an enormous light at the end of the tunnel but then we find out it is not about because of where we live.

“We are fortunate that we work hard and are able to fund the medication. However as I coordinate the food larder I am acutely aware that for many households that it would not be an option to be able to help your child in this way.”

In 2022, Jacob started to lose his sight over a four to five week period and has since been diagnosed with Leber's hereditary optic neuropathy - a condition that affects only 35,000 in the world.

Jacob Ely in August 2022 at Busch Gardens, Florida. PHOTO: SUBMITTED
Jacob Ely in August 2022 at Busch Gardens, Florida. PHOTO: SUBMITTED

The condition, which affects males aged between 15 and 35, is caused by a mutation that damages cells in the retina and can lead to blindness.

Idebenone, the medication that Jacob is taking, is an antioxidant that helps restore cell generation and has helped to slightly improve his eyesight.

Kimberley said: “His whole life has been thrown a curve ball.

Nicholas, James and Jacob Ely. PHOTO: SUBMITTED
Nicholas, James and Jacob Ely. PHOTO: SUBMITTED

“He just lost his sight and it was completely out of the blue and he lost his driving licence and job.

“The medicine is free but not in England and it has a 50 per cent chance to get his sight back.

“We do feel there is a slight improvement with his sight over the last few months and can see if it is having an effect.

James, Jacob, Kimberley and Nicholas Ely. PHOTO: SUBMITTED
James, Jacob, Kimberley and Nicholas Ely. PHOTO: SUBMITTED

“It could mean that he can drive again.”

Kimberley said that she felt ‘helpless’ and says that Jacob, who is also a Type 1 diabetic, is taking things day by day in order to keep the routine of normal life.

He has been a motorbike enthusiast since passing his CBT but is now unable to ride and although he can ride pillion with dad James it is not the same.

James Ely with son Jacob aged 15. PHOTO: SUBMITTED
James Ely with son Jacob aged 15. PHOTO: SUBMITTED

Kimberley said: “He is a 19-year-old lad and it is not fair but he is not a ‘pity me’ type and he gets on with life.”

The shock has affected the whole family and Jacob’s siblings Stephanie, 24, and Nicholas, 20 could also develop the condition.

Kimberley said: “My other son could develop it but my boys will not pass it onto their children.

LtoR: Stephanie, Kimberley, Jacob and Nicholas Ely on Mother's Day 2023. PHOTO: SUBMITTED
LtoR: Stephanie, Kimberley, Jacob and Nicholas Ely on Mother's Day 2023. PHOTO: SUBMITTED

“But if Stephanie was to develop it, she could pass it on to her children.”

Mrs Ely is gearing up to take on the famous London Marathon on Sunday April 21, and has so far raised £1,000 for the RNIB.

She said: “I can't give him his sight back but I can raise some money for a charity that will support him in later life.

Jacob Ely has lost his sight due to a rare hereditary condition. PHOTO: SUBMITTED
Jacob Ely has lost his sight due to a rare hereditary condition. PHOTO: SUBMITTED

“I am not a marathon runner not at all but you have to have some drive and family is a big drive.

“My husband James has been an amazing support.”

Another source of support has been Fr Jonathan Sibley and the congregation at St Mary’s church.

He said: “I admire her determination and the whole church has been praying for her son and are very supportive.”

This is not the only case of unfair access to medication to happen in this area.

Last month we reported on nurse Tracy Pratt who fighting for access to a drug which could give her two extra precious years with her family. It is also available in Scotland but not England.

Part of NICE’s role is to make recommendations on the use of new and existing medicines and treatments within the NHS in England and Wales, based on a review of clinical and economic evidence.

A NICE spokesman said: “NICE is currently in the process of appraising the clinical and cost-effectiveness of Idebenone for treating visual impairment in Leber’s hereditary optic neuropathy (LHON) in people aged 12 years and over.

“A first meeting of NICE’s independent appraisal committee was held earlier this month and final draft guidance is expected to be published in the coming weeks, with final guidance expected to publish in June this year.”

If you would like to support Kimberley please click here.

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