About Mum.. 

Juliet Anna Court (born 11th February 1956), a mother of two, wife and sister. Daughter of Eric and Ivy (Kay) Stroud. Mum died of sporadic Creutzfeldt-Jakob disease (CJD) on 6th September 2020 aged just 64.

Juliet was a proud Plymouth girl, a truly selfless, compassionate giving lady with a fantastic zest for fun, music you could sing and dance to, poetry, theatre and a passion for all things creative. From learning a foreign language, pottery, needlework, pastel-work, architectural innovation to landscape design. She loved her garden and conservation, in particular of our seas, marine wildlife (especially otters), Dartmoor (especially its ponies), and elephants. Her heroes were Greenpeace, Daphne Sheldrick, Lawrence Anthony, Gerald Durrell and Sir David Attenborough. Mum also loved to walk, especially with our family dogs, to travel and learn from other cultures.

Like me, Mum was a former student and house captain at Plymouth High School for Girls. Mum also had an incredible memory and skill for English and mathematics. She studied Landscape Architecture at Gloucestershire College of Art & Design and later worked at the Woolwich bank until giving it up to bring up us children whilst Dad worked away. She gave up her spare time to help Dad in his screen printing business, running round us kids and then helped those falling behind in reading and maths at our primary school. She also gained her RHS qualification having gone back to Bicton College to study. 

Mum was the one person you could truly count on, to trust and confide in and give you sound, honest advice. She looked after and was seen as a second “Mum” by so many and to my Dad, brother and I she was also our best friend, so full of life and dreams yet always put our needs first over her own. All the more reason why her diagnosis was so cruel a hand dealt her.

Juliet Court was the bravest, most inspirational and clever woman I know, it is the least we can do to try and spread awareness of CJD and raise funds for research and trials to help find a cure so that her death was not in vain.

Do you know what sporadic Creutzfeldt-Jakob disease (CJD) is? Do you know the symptoms? No? 

Well, neither did we.. it is the most common of the 4 forms of CJD and is believed to be linked to a variant of the more commonly known Bovine Spongiform Encephalopathy (BSE or “Mad Cow Disease”). You can’t catch CJD like you can BSE through eating infected animals but you can inherit it, or, like Mum you are just incredibly unlucky to be 1 of the ‘up to 2 people in a million’ in UK that it ‘just happens to’, to be diagnosed each year with this degenerative disease.

CJD appears to be caused by an abnormal infectious protein called a prion. These prions accumulate at high levels in the brain and cause irreversible damage to nerve cells, resulting in the following main symptoms:

• acute tiredness but insomnia
• loss of intellect and memory 
• changes in personality (acute depression, despair, unusual anxiety, abnormal tearfulness, irritable)
• dizziness, loss of balance and co-ordination 
• Constant aching, pain, numbness or pins and needles in different parts of the body
• Difficulty swallowing 
• slurred speech 
• vision problems  and blindness
• abnormal jerking movements similar to fits with increasing intensity without medication such as sedatives
• progressive loss of brain function and mobility

While the abnormal prions are technically infectious, they're very different from viruses and bacteria. For example, prions aren't destroyed by the extremes of heat and radiation used to kill bacteria and viruses, and antibiotics or antiviral medicines have no effect on them.

Juliet’s journey..

Mum had gone to Derriford Hospital A&E in Plymouth, UK, with a suspected mild stroke as her balance was so awful, she was experiencing trembling and numbness and a “wandering” right arm that she had no control over. 

She had knocked glasses over at home and was increasingly struggling with stairs and uneven ground. Suddenly she couldn’t find the words she needed to say full sentences and she was really struggling to concentrate and add up to do her favourite Suduko puzzles and the cryptic crosswords she loved and did daily in addition to regular exercise, ironically to try and prevent dementia. 

She would get increasingly overly worried, overly paranoid and irritable with things that’s she would have normally taken in her stride or let wash over her. These symptoms we realise now, began around 2 years before her diagnosis but blood tests repeatedly came back clear or scans inconclusive with “nothing sinister to worry about”.. we always blamed her diabetes medication. Her appetite was small but she was increasing feeling bloated and experiencing reflux - this intermittent pain and tiredness increased over the last 12 months - symptoms we now know are associated with CJD.

She was placed on Derriford Hospital’s trauma ward, then the neurological ward with suspected Encephalitis but the treatments had no effect. Due to COVID-19, only Dad could visit daily at a pre-arranged time for an hour at first. 

Mum increasingly lost her appetite because she had developed a heightened sense of smell - her brain was telling her that everything smelt strongly of fish. Her tastebuds were changing too. 

She’d do strange things like drop and leave her phone in the sink and leave the tap or shower running or cupboard doors open and forget she’d done so. 

Then her co-ordination started getting worse with her not being able to get up without assistance, type the buttons on her phone or hold cutlery.

As mums condition rapidly progressed over a fortnight, my husband Rob and I were later permitted to visit once a day in addition to Dad. We felt the air go out the room when the neurologists gently told us their educated suspicion of what was wrong when test after test from blood diagnostics, MRI scans, painful lumbar punctures etc came back negative - 1 in a million, we thought the neurologists had to be wrong, it’s so rare.. sadly not however. We decided it was time to get my brother home from Sydney, Australia, things were not looking good. 

I desperately called, emailed and wrote to everyone we knew, teams of forward thinking medical research teams and frantically tried to find a clinical trial that may be Mum’s only hope of treatment. All led to dead ends. Any potential trials abroad wouldn’t take mum due to the pandemic (she would have been physically unable to travel at this point anyway) and those nearer to home in the UK had either finished trials or were awaiting funding for trials in the distant future. 

It was another couple of weeks before mum was allowed home as yet more and more tests were carried out in the hospital to eliminate anything else sinister going on. CJD didn’t hold back, it swamped Mum seemingly over a fortnight, it was brutal watching and feeling so utterly useless and now also hopeless. 

Mum made her final journey home on the 24th July. She received round the clock care from the awesome team at St.Luke’s Hospice, Marie Curie, myself, my husband Rob, my Dad and brother.

Mums symptoms got worse, her joints stiffening and even an accidental brush on her arm, a gentle breeze or the pleasure of seeing a family friend with a baby triggered her seizures so we realised taking mum to the moor or the beach one last time was impossible. 

Mum was treated with a battery operated intravenous drip, daily community nurse visits and a weekly doctor visit to observe and monitor mums decline and make sure she was in as little pain as possible. 

Bless mum, she continued to try and smile through it all, only her eyes gave her away. The only silver lining was that Mums battle was short.. too much time to think yet not enough time for her to either write down or tell us what she wanted or needed. 

Mum just slowly stopped speaking, moving, eating, then drinking, her eyes shut for longer periods of time as she went blind and then she just drifted slowly away from us with mercifully less and less fits as the medication did its job and she was finally at peace.

My Fundraising Plea..

Please please help us to spread awareness of and find a cure for Creutzfeldt-Jakob disease (CJD). I can’t bare to think of families going through the same traumatic experience that still haunts us today, almost two years on. Other families inevitably will suffer too until a cure can be found and with any luck help solve other similar neurological diseases in the process.

Every day scientists and doctors are getting closer to finding a treatment, however, there is currently no cure and there are no trials happening in the UK largely due to lack of funding from the government as the disease is so rare.

UCLH aims to raise funds to support the use of an antibody drug developed for the treatment of patients with CJD. 

Rob and I would like to help them raise funds by hiking the very hilly but beautiful Cornish coast path from Padstow to Rowan Cove (half a marathon) on the 3rd September 2022. Mum loved visiting and walking this stretch of coastline, walking Otto our German Pointer when we camped at Harlyn Bay in the Summer holidays as kids and later when the weather was wild and waves crashing against the cliffs in the Autumn and Winter with Dad.

Please give as little or as much as you can to this cause and if possible select ‘yes’ to Gift Aid so the tax goes to the charity not the government.

My family and I invite you to raise a glass to Juliet Court on September 6th and remember her as the sun rises, the 2nd Anniversary of her passing. 

Have a thought for those battling CJD and their families.

Many thanks in advance.

Love and warmest wishes,

Amy and Rob Main

The Cure CJD Campaign website is: https://www.curecjd.org

Read more about the symptoms of Creutzfeldt-Jakob disease: https://www.nhs.uk/conditions/creutzfeldt-jakob-disease-cjd/symptoms/ 

Read more on diagnosing Creutzfeldt-Jakob disease: https://www.nhs.uk/conditions/creutzfeldt-jakob-disease-cjd/diagnosis/