Mother with rare spine injury after accident with ceiling fan has surgery after stranger gave £130k

A mother-of-one who was left with a rare spine condition after a freak accident involving a ceiling fan has undergone life-saving surgery in Spain thanks to a stranger’s £130,000 donation.

Rachel Pighills, 35, was left at risk of being ‘internally decapitated’ at any moment following an accident in August 2018.

She had been moving into a new house when she struck her head on a ceiling fan while standing on a bed.

Rachel was left with atlantoaxial instability and basilar invagination – meaning her brain was sinking into her spine and her skull sliding down onto her neck.

It meant she could no longer turn her head the wrong way as each time she did her spine would partially dislocate increasing her risk of paralysis or death.

Rachel Pighills, 35, was able to have life-saving surgery in Barcelona for her rare spinal condition which puts her at risk of internal decapitation after an unknown person donated £130,000

Rachel's symptoms began back in 2017 when she started her vomiting and losing 38kg (6st) in just six weeks, just before her wedding to Guy Pighills.  She is pictured on the big day with her now husband

Rachel’s symptoms began back in 2017 when she started her vomiting and losing 38kg (6st) in just six weeks, just before her wedding to Guy Pighills. She is pictured on the big day with her now husband

Husband Guy said a picture of the surgery looked like Rachel had been 'attacked by a shark'.  Pictured: stitches along Rachel's neck and spine after the surgery

Husband Guy said a picture of the surgery looked like Rachel had been ‘attacked by a shark’. Pictured: stitches along Rachel’s neck and spine after the surgery

To have the surgery, Guy had to drive Rachel 1,000 miles to Barcelona in a second-hand ambulance

To have the surgery, Guy had to drive Rachel 1,000 miles to Barcelona in a second-hand ambulance

Rachel and husband Guy, 41, have spent the last few years trying to raise £350,000 for pioneering treatment that only three surgeons in the world are able to do.

They finally reached the milestone last month thanks to a staggering £130,000 donation from an anonymous beneficiary who had seen her story.

Mum-of-one Rachel set off on the 1,055 mile, 17 hour journey to Barcelona in a second hand ambulance driven by Guy as she was too unwell to fly.

She underwent the 13-and-a-half hour procedure to fuse her neck and skull at Teknon Hospital on May 20 which has left her looking like she had been ‘attacked by a shark.’

Quality inspector Guy said: ‘Initial signs are looking good, but I don’t want to get my hopes up just yet. I’m being as positive as I can be, but I’m just reserving myself.

‘The recovery time for this operation is 12 months minimum.

‘The neurosurgeon Dr Gilete showed us a picture of the operation and it looked like a shark attack, which shows how severe it is

‘Rachel is still in so much physical pain, but she does feel like she has had some slight improvements.’

After multiple visits to her GP and meetings with experts, an MRI brain scan led to her diagnosis.  As well as atlantoaxial instability, Mrs Pighills also has curvature of the spine, a build-up of fluid in the brain and brain stem compression

After multiple visits to her GP and meetings with experts, an MRI brain scan led to her diagnosis. As well as atlantoaxial instability, Mrs Pighills also has curvature of the spine, a build-up of fluid in the brain and brain stem compression

Scan showed an excessive gap, which leads to too much movement between her top vertebrae

Scan showed an excessive gap, which leads to too much movement between her top vertebrae

Rachel had led a very active lifestyle with Guy before her symptoms began

Rachel had led a very active lifestyle with Guy before her symptoms began

Guy said he had also noticed a change in his wife’s voice since the operation and added: ‘She used to have quite a husky voice, almost like a smoker, but now she sounds different.’

The couple now intend to stay in Spain till June 8 before returning to the UK to continue Rachel’s recovery.

Following her accident, doctors told Rachel her neck could no longer support the weight of her head and one wrong move could cause total displacement.

She has been confined to a wheelchair and must wear a neck brace for hours a day to stop her neck from slipping.

Friends and family set up a fundraising page before a mystery benefit from Warwickshire – a woman in her 60s – came forward with the £130,000.

Rachel's three-stage operation in Barcelona lasted for 13-and-a-half hours

Rachel’s three-stage operation in Barcelona lasted for 13-and-a-half hours

Rachel, of Pershore, Worcestershire, said previously: ‘No amount of words can express what it means to us and how grateful we are. She really is our guardian angel.

‘I can’t believe somebody I’ve never even met could do such a thing for us.

‘I’m a determined person and I have to try and do what I can. I know I can’t give up.’

Guy had said the situation had become ‘soul destroying’ for the couple and their 14-year-old daughter.

He said: ‘All she wants is her health and independence back, and to stop living in fear of dying and leaving her family behind.’

Rachel used to be fit enough to go horse riding, but is now on a long road to recovery after her major surgery

Rachel used to be fit enough to go horse riding, but is now on a long road to recovery after her major surgery

Rachel had been fit and healthy until August 2017, which corresponds with when she started a new medication for an overactive immune system.

She began vomiting without warning, which landed her in hospital three times between September and October.

This also caused her to lose 38kg (6st) in just six weeks. This left her thinking she may subconsciously be trying to shed the pounds ahead of her wedding.

Even though she stopped taking the medication almost straight away, the symptoms continued.

After an MRI scan ruled out a brain tumour, she was told she had Addison’s disease in October 2017.

This occurs when the body does not produce enough of the hormones cortisol and aldosterone.

WHAT IS ATLANTOAXIAL INSTABILITY?

Atlantoaxial instability (AAI) is defined as excessive movement at the junction between the spine’s first two vertebrae.

It is unclear how common it is.

Neurological symptoms, such as dizziness, headaches and fatigue, can occur if the spinal cord or its surrounding nerve roots are affected.

In severe cases, AAI can cause spinal compression, which can be deadly.

AAI arises due to an abnormality with the bone or ligaments in the spine.

This can be brought on by conditions that are present at birth.

However, AAI more often occurs due to a traumatic accident or inflammation caused by rheumatoid arthritis.

Infections may also trigger inflammation.

Mild cases are often treated by just believing AAI’s symptoms.

‘Cervical immobilisation’, such as wearing a neck brace, may be required.

In extreme cases, surgery may be needed to stabilize the spine.

She was treated with steroids to help correct her cortisol levels, but it soon became clear the steroids were not easing her symptoms.

‘I was having tremors in my hands, caused by adrenaline, I was exhausted all the time and would just fall asleep at any moment,’ Rachel said in 2019.

‘It got to the point where I could not drive my daughter to school in case I fell asleep at the wheel.’

She was forced to move closer to work and her daughter’s school to cut down her commute.

It was while moving into her new home, she struck her head on a ceiling fan.

‘A week after that I went into adrenal crisis due to water retention and was admitted to hospital,’ Rachel said. ‘It happened again a week later and I was back to hospital.’

An adrenal crisis is a medical emergency that occurs when a person’s cortisol levels fall significantly.

‘After I returned home I was in constant pain,’ she said. ‘My head felt really heavy on my shoulders, and I would get dizzy and lightheaded.’

She paid to have an MRI scan, which came back clear.

With her symptoms continuing, she made additional visits to the GP and was eventually referred to a cardiologist.

The medic carried out an electrocardiogram to test her heart’s rhythm and electrical activity. This revealed her heart was beating faster than normal, which lead to her being diagnosed with PoTS.

After she saw a neurologist, who diagnosed her with Chiari malformation. This occurs when the brain tissue extends into the spinal canal.

‘I posted my MRI scan in a Chiari malformation Facebook group and people were saying Chiari was the least of my problems,’ Mrs Pighills said.

‘The dizziness was getting worse. It felt my head was too heavy for my neck. At work I would have to prop up my head with my hands. It felt like an enormous weight. I couldn’t hold it myself.

‘I was getting really bad headaches. The worst thing at the bottom of my head. It felt like something was pushing and going to pierce through my head. Sitting up would be agony.’

Eventually Rachel was able to see another neurologist, who diagnosed her with platybasia. This is defined as the abnormal flattening at the base of the skull.

It was then she was also told she had basilar invagination, which occurs when the top of the spine pushes into the base of the skull, causing pinching and pressing on the brain stem.

She paid for another private, upright MRI scan.

The images of which she asked to be sent to a Barcelona-based neurosurgeon Dr Gilete, who she came across on the Chiari Facebook group.

The medic was then able to diagnose Rachel with atlantoaxial instability, as well as curvature of the spine, hydrocephalus – build-up of fluid in the brain – and cervical medullary syndrome – brain stem compression.

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