A mother-of-two has been undergone 24 operations to fix her dislocating joints due to a rare genetic disorder that even caused her kidney to detach itself.
Rebecca Hempsell’s, 40, from Plymouth, condition affects the connective tissue that supports her skin, bones, blood vessels and other organs.
Ms Hempsell, a former primary school teacher, has undergone 24 operations to fuse her dislocated joints back together and has to take 40 tablets a day.
Although she has dislocated her shoulder, knee, pelvis and feet, Ms Hempsell says many don’t understand her ‘invisible illness’ and accuse her of lying.
Her condition, known as Ehlers-Danos syndrome, affects just one in 5,000 people worldwide.
Rebecca Hempsell, from Plymouth, has undergone 24 operations on her dislocating joints
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WHAT IS EHLERS -DANLOS SYNDROME?
Ehlers-Danlos syndrome (EDS) is the name for a group of rare inherited conditions that affect the connective tissue.
Connective tissues provide support in skin, tendons, ligaments, blood vessels, internal organs and bones.
Different types of EDS are caused by faults in certain genes that make connective tissue weaker.
Depending on the type of EDS, the faulty gene may have been inherited from one paren, or both.
Sometimes the faulty gene isn’t inherited, but occurs spontaneously.
EDS can affect people in different ways. For some the condition is relatively mild, while for others their symptoms can be disabling.
Some of the rare severe types can even be life-threatening.
Source: NHS Direct
Ms Hempsell 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.’
Ms Hempsell, who spent almost a year sleeping on her living room floor after having several operations to fuse her body parts in place, says ‘I’m bit by bit falling apart’.
The condition causes the 40-year-old mother to suffer from painful dislocations that often leave her wheelchair bound. Pictured is Ms Hempsell’s foot following reconstructive surgery
EDS causes the connective tissue to become weak. Pictured is Ms Hempsell’s feet afer surgery
As a youngster she was a talented gymnast, but Ms Hempsell had no idea her flexibility was a result of EDS.
She would dislocate joints at awkward times and suffer life-altering injuries, but doctors misdiagnosed the condition for 30 years.
Ms Hempsell said: ‘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.’
Her 70-year-old mother Jane, a former midwife who helps Ms Hempsell with her housework every day, said: ‘We never understood her condition.
Ms Hempsell was misdiagnosed for 30 years before doctors finally realised she had the debilitating condition. Pictured right is her shoulder following reconstructive surgery
Pictured are Ms Hempsell’s knees after she had major surgery to stop them from dislocating
‘We should have never sent her to gymnastics but she was so good at it – we just thought she was very clever.’
It wasn’t until Ms Hempsell’s daughter Lillie, now 16, was taken ill with the same disorder, that she was finally diagnosed. Her other daughter Maizie, 14, also sufferers from EDS.
In the past year, she has undergone three major surgeries 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.
Although the surgery has stopped her joints from dislocating on a daily basis, the irreversible procedures mean she can no longer move those body parts.
She also suffers with chronic widespread joint pain, brain fog and fatigue, stretchy skin that struggles to heal and bruises easily, and gastrointestinal dysfunction.
At one point the former teacher’s kidney detached itself and found its way to her abdomen. Pictured left is Ms Hempsell following major knee surgery and right after a foot operation
At one point her kidney even detached itself and found its way to her abdomen after the connective tissue surrounding it stretched and sagged.
The kidney was unable to be returned to its original place and had to be stitched beneath her rib cage.
Ms Hempsell is also in the process of being diagnosed with Chiari Malformation, which occurs when the lower part of the brain pushes down in the spinal canal because it is not supported by connective tissue.
This is causing Ms Hempsell to suffer excruciating migraines.
Although she was forced to retire early from her teaching job, Ms Hempsell keeps herself busy with a part time tutor job.
She is also keen to spread awareness of her disorder.
Ms Hempsell said: ‘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?’