Alex TLC patron, Britt Ekland, describes the agony of finding out her grandson has ALD

Former Bond girl Britt Ekland, 77, describes the agony of finding out her darling grandchild has a crippling illness which kills many sufferers before the age of ten

  • Lucas was diagnosed with adrenoleukodystrophy (ALD) at birth through the US Newborn Screening Programme

  • If unidentified ALD can cause sight loss, inability to walk and talk resulting in a semi-vegetative state and early death, usually before the age of ten

  • Early diagnosis and monitoring every 6 months means Lucas could have a bone marrow transplant to halt his ALD and ensuring he has a healthy, normal life


“The younger of her two grandsons, three-year-old Lucas has … adrenoleukodystrophy (ALD), a brain disorder that destroys myelin, the protective sheath that surrounds the brain’s neurons — the nerve cells that allow us to think and to control our muscles — typically leading to sight loss, inability to walk and move, and ultimately premature death, on average before the age of ten.

Practical and resourceful, former Bond girl Britt — most recently on our screens in BBC series The Real Marigold Hotel — does not waste energy on outpourings of emotion, but she clearly adores her grandson Lucas and his brother Cash, seven — sons of Alison and Nicholai Adler, Britt’s son from her relationship with record producer Lou Adler.

Britt was at the hospital in Los Angeles to greet Lucas hours after he was born on April 8, 2017, weighing a healthy 6lb 7oz, and with no signs that anything was amiss.

‘He was just beautiful, wrapped up in a blanket like a little burrito, with masses of dark hair,’ she says. ‘The insidious thing about this illness is that your child is just like any other until symptoms occur. ‘Lucas hit all his milestones. You’d never guess in a million years that anything was wrong with him because there are no visible clues.’

She, however, was unaware that results of screening tests — routine in California but not yet in the UK — which arrived a couple of months after Lucas’s birth, revealed he had ALD. It was not, in fact, until last year that she was told the devastating news.

‘I was staying with Nicholai and Alison in the June and one night, after the children had gone to bed, they said: ‘Mum, we need to speak to you.’ Lucas was two; an extraordinarily happy little boy.

‘He’d always been a little bruiser; so cute and with boundless energy. He’s much more active, in fact, than his big brother, so I had no idea at all that anything was wrong.

‘It’s only when I look back now that I wonder if Alison was a bit more protective of him than she was of Cash. We’re a demonstrative family, but she seemed to kiss and hug Lucas more than ever.

‘And on that evening when she told me the news I could see she’d been crying. I didn’t know what to think. My heart sank.

Then she said, “We didn’t want to talk to you before we’d done all our research, or give you information before we knew everything ourselves.”

‘They’d known for two years before they told me and I can understand why they wanted to be certain of all their facts first.

‘I think they also wanted me to have a happy relationship with Lucas without being too fearful.

‘It was a huge shock. Lucas is such a wild thing, like a little typhoon, and it was so difficult to come to terms with the fact that such a beautiful, entertaining, lovely little boy was facing something so dreadful.

‘I’d just been looking after him — I was working in LA at the time — and when Alison and Nicholai went out for the day I’d take all the cushions off the outdoor furniture and put them on the ground so he could play safely.

‘Looking back, I was glad that my motherly instincts told me to do that in case he fell, because Alison told me that evening: ‘We have to be really careful with his head.’ Apparently, there is evidence that head injuries can bring on symptoms.’

Family photos chart Lucas — mischievous and brown-eyed — riding his scooter, in fancy dress and hugging his big brother.

‘There is no indication, from the energy and vigour with which he attacks life, that he has this deadly condition.

And this is the point: for years the disease can be asymptomatic. But once the symptoms start to develop — typically when a child is four — it is usually too late to do anything to stop the decline into early death.

ALD causes the nerves in the brain to malfunction, so speech, reasoning and mobility are lost.

Once symptoms start to occur, boys with the condition usually live just two to four years because there is no treatment for the disease once it has started to manifest itself.

For Lucas, however, the outlook is promising because he has regular six-monthly MRI scans, and blood tests every three months, which can detect the brain lesions that presage the onset of ALD.

Then, before symptoms even start, a bone marrow transplant is very likely to halt it in its tracks.

It does this by replacing damaged blood-forming cells (stem cells) with healthy ones.

Through these healthy cells, the body is able to break down fat-based substances that cause the brain to malfunction.

Success rates for such transplants, improving all the time, stood at around 95 per cent in 2016.

‘It’s quite remarkable — miraculous, really — how the bone marrow transplant works,’ says Britt, ‘And although it’s not a cure as such, because the progress of the symptoms is stopped the outcomes are incredibly positive.

‘Lucas will be able to have a perfect life. He can become a doctor, a lawyer, a plumber, a motorcycle racer.

Once he’s had the stem cell transplant he can do anything he wants. And that’s all because he had that vital screening at birth,’ she says. ‘But for the boys whose symptoms aren’t caught early through an MRI scan, it’s a very different story. These children are dying.’

Britt, as patron of Alex, the UK’s leukodystrophy charity, is campaigning for early diagnosis to be routine in the UK.

‘It’s such a devastating disease it’s hard even to speak about it,’ she says. ‘I’m working on getting through to the Government so they’ll put it on the UK screening list because it’s the difference between life and death.’

Although Lucas’s long-term prognosis is good, his young life is punctuated by hospital visits.

Scans and blood tests are routine and he faces the prospect of months in hospital having a bone marrow transplant.

‘The thought of the transplant is bleak,’ admits Britt. ‘And this is a very nervous week for all of us because tomorrow Lucas is due a routine MRI scan, and as he is approaching his fourth birthday — the age when symptoms of ALD typically start — we’re all holding our breath that the scan does not show lesions on his brain.

‘We pray that he is clear but Alison will call me immediately if something is seriously wrong.

‘If it’s good news, she’ll send me a funny photo of Lucas eating a vegan ice cream or causing some mischief.’

She is resolutely hopeful; determinedly optimistic. ‘I try not to be a miserable, sad, old woman.

‘I have to be upbeat about it, as well as supportive. Nicholai and Alison are amazingly strong.

‘And it’s so fortuitous that Lucas was screened at birth because the routine screening in California had only come in three months earlier. He’s already been through a lot for a little boy.

‘Since he was three months old, he’s had MRI scans every six months. They’re not painful, but it’s disconcerting for him.

‘The machine makes a loud noise and he wears earplugs and has his baby iPad to distract him.

‘And when he has blood taken that’s horrifying. He goes every three months and last week he went Alison said he was amazing. He only whimpered a little bit when the needle went in. He knows he has to do these things to keep him well, and he’s such a strong, happy little chappy.”

Read the full article here

Source – Daily Mail 2nd September 2020

Alex - The Leukodystrophy Charity