A brave woman who suffers from a very rare degenerative illness is preparing for her 25th operation — to stop her body literally falling apart.
Rebecca Hempsell looks like a healthy 40-year-old woman, but has Ehlers-Danlos syndrome affecting connective tissues that support the skin, bones, blood vessels and other organs in her body.
Mum-of-two Rebecca, a former primary school teacher, has undergone 24 operations – many to fuse the bone in parts of her body back together – and is on 40 tablets every day.
Rebecca, from Plymouth, Devon, said: “It’s often thought by someone who knows of Ehlers Danos syndrome that you just dislocate all of the time.
“I do, but I also suffer from multiple internal problems because of it.
“I have pain all the time and there’s days where I don’t want to get up. I’m a very strong person but you can’t always be.
“There’s days I wish I didn’t have this but the reason I have such a positive outlook is because I’ve seen so many people give in and they get worse. I won’t ever give in.”
She added: “When you’re living with an invisible illness, you can be in a wheelchair one day and only with a stick the next.
“It often means people think you’re making it up.
“There are also many other syndromes which run alongside it but these vary greatly from person to person and there lies the great problem.”
Rebecca, who spent almost a year sleeping on her living room floor after several operations to fuse parts of her body in place, says she is literally “bit by bit falling apart”.
As a youngster she was known for her flexibility as a gymnast but had no idea it was due to EDS.
She would dislocate at awkward times and suffer injuries which made life difficult and only discovered she had the syndrome when her oldest daughter Lillie, now 16, was taken ill.
Lillie and her sister Maizie, 14, both have the genetic disorder but find that due to its often invisible symptoms, many dismiss their pain and inability to manage everyday tasks.
Defects in connective tissues cause the signs and symptoms of Ehlers-Danlos syndrome which vary from mildly loose joints to life-threatening complications.
Rebecca, who was misdiagnosed for 30 years before doctors finally realised she had EDS, added: “I was six years old when my first shoulder dislocation happened.
“I thought it was a bit strange but I was a gymnast and known for my absolutely ridiculous flexibility.
“I could put my legs over my head and do the splits to a crazy extreme.
“Everything was so flexible and still is – it didn’t change, which is when I thought this isn’t right, I can still do this and I’m 41 nearly.”
Rebecca took early retirement because of her condition and has undergone three major surgeries in the last year including the fusing of her shoulder, knee and foot.
Her shoulder had been dislocated for FOUR years and she had pins and screws put in her kneecap to stabilise it.
That has stopped her joints from dislocating on a daily basis but the irreversible procedures mean she can no longer move those parts of her body as she once would.
Rebecca also had a disrupted pelvis and underwent two caesarean sections when she had her two children and a hysterectomy.
Her symptoms include joint hyper-mobility, chronic widespread joint pain, brain fog and fatigue, stretchy skin which struggles to heal and bruises easily as well as gastrointestinal dysfunction.
At one point her kidney detached itself and found its way to her abdomen because the tissues surrounding it, as well as the veins and arteries, all stretched leaving it functioning very poorly.
The kidney was unable to be returned to its original place and so was stitched by surgeons underneath Rebecca’s rib cage.
Rebecca is now in the process of being diagnosed with Chiari Malformation, meaning her brain is not being supported by its connective tissue.
It is slowly slipping down and causing her to experience excruciating migraines.
Despite her struggles, Rebecca keeps herself busy with part time 11-plus tuition and holds classes several times a week for youngsters in her back garden.
She added: “Someone might be a relative of someone with Ehlers Danlos and think well she’s fine, she’s just a bit bendy – great gymnast.
“But then they meet me and they think well my daughters got that and she’s fine – well I’m not, you know?”
Her 70-year-old mother Jane, a former midwife who helps Rebecca with housework on a daily basis, said: “We never understood her condition.
“We should have never sent her to gymnastics but she was so good at it – we just thought she was very clever.
“Her two children aren’t allowed to do it.”
EDS is progressive with a poor long-term prognosis.
