Rachel Pighills was standing on a bed when she was hit by a ceiling fan
A mum-of-one is in a race against time to raise £350,000 for life-saving treatment after a freak ceiling fan accident left her at risk of being "internally decapitated".
Rachel Pighills is trying to raise enough funds for pioneering surgery in America after she was left with a rare condition that could kill her at any moment.
The 35-year-old had been moving into a new house when she struck her head on a ceiling fan while standing on a bed leaving her horrific injuries.
After the accident in August 2018, Rachel was left with atlant-axial instability and basilar invagination - which means her brain is sinking into her spinal canal and her skull is sliding down onto her neck.
It means she can no longer turn her head the wrong way as each time she does her spine partially dislocates increasing her risk of paralysis or death. Husband Guy, 41, says her condition is “deteriorating day-by-day" as the family desperately continues to fundraise to save her life.
"It has been soul-destroying to see her deteriorate. Her breathing is horrendous - she is having to force herself to breathe at the moment," the 41-year-old from Pershore, Worcestershire, said.
"Because of the brain stem compression, the signals are all incorrect. The brain stem subconsciously controls everything.
"Her heart rate can go from 60 to 140 in the click of a finger. The signals are getting confused."
Guy said his wife has no "quality of life" now and is just gasping for breath.
Ideally, he said, Rachel needs non-invasive therapy for her breathing but it is complicated.
"She is suffering from central apnoea's during the day too - sometimes she just stops breathing for a moment.
"She is in constant agony, it is relentless. I genuinely can't remember her having a good day since she had her first surgery.
"It has left me feeling heartbroken and helpless. The surgeries are high risk, but without them Rachel will die."
After receiving £45,000 in donations from well-wishers, the family is looking at different options to help save Rachel’s life, but is still short of their £350,000 target. As of Tuesday afternoon, they had raised just over £48,000.
One option for the family is a complex operation in New York by Dr Paolo Bolognese - the only surgeon in the world prepared to do the procedure.
Guy said that even though the family is continuing to explore their options, travelling abroad could be fraught with difficulty. They will need an Air Ambulance to get there.
The quality inspector said: "The changes in the air pressure and altitude, and she may need oxygen. It could affect her in a dangerous way. We will need medical supervision throughout.
"With that, there is no guarantee that Rachel will survive the surgery - but then again, she might not survive without the surgery."
Guy said the family is not "fooling ourselves that this will be the fairy tale ending".
"We have to prepare ourselves for the worst in surgery, if we get there."
And time is running out: "It is a race against time now, I don't like to think about it but the longer she is like this, without treatment, the worse she is going to get - she will die."
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In January last year, Rachel underwent Occipital Spinal Fusion surgery in the UK which was hoped would improve her quality of life.
Guy explained: “Her skull was fixed to her spine with rods and screws, and a piece of bone from her skull and spine was removed to create more room for her brain.
“The surgeon was new to this procedure and thought that this would relieve the compression from her brainstem.
“Sadly, this has not been the case, and we eventually learnt that Rachel’s surgery had failed.” Doctors said her neck can no longer support the weight of her head and one wrong move could cause total dislocation or "internal decapitation".
Three-and-a-half years on from the freak accident Guy said the family is trying to stay positive for Rachel.
“It is difficult for the family, but we are trying to stay positive for her. Our daughter is dealing with it well.
"I find it really hard sometimes; I try and pretend it is not happening. I try and block it out but have to do all you can to help.”
Speaking previously, Rachel said: “I live in constant fear of paralysis and death. It's hard to describe that feeling. "I’m a determined person and I have to try and do what I can. I know I can’t give up."
You can donate to Rache's surgery fund here.
