I'm being decapitated by my own SKULL: Mother-of-two is given just a month to live because her spine is being crushed by the weight of her head

  • Samantha Smith is being 'internally decapitated' by her own skull due to illness 
  • Ms Smith urgently needs surgery to save her life and has just one month to live 
  • The disease Craniocervical Instability means ligaments in her neck are too weak
  • She aiming to raise £200,000 to have surgery in America, which could save her life

By Isobel Frodsham For Mailonline

Published: 04:45 EST, 3 June 2017 | Updated: 06:10 EST, 5 June 2017

A mother-of-two who is being ‘internally decapitated’ by her own skull is desperately trying to raise funds to have life-saving surgery.

Samantha Smith, 30, is raising a total of £200,000 to have surgery to aid her debilitating condition, which means the weight of her skull is crushing her spine. 

Ms Smith, a psychotherapist, suffers from the genetic condition Ehlers Danlos Syndrome (EDS), a connective tissue disorder, and Craniocervical Instability (CCI), which means the muscles in her neck are too weak to support her head.

Specialists have claimed that due to the severity of CCI, she has just one month to have the surgery before the damage is irreversible and she will die.

Samantha Smith, above with her children Jensen and Brooke. Samantha has been diagnosed with three debilitating conditions

Samantha Smith, above with her children Jensen and Brooke. Samantha has been diagnosed with three debilitating conditions

One of the conditions, Craniocervical Instability (CCI), means her ligaments are too weak to support her head, meaning she is being 'internally decapitated'
Ms Smith is pictured in hospital

One of the conditions, Craniocervical Instability (CCI), means her ligaments are too weak to support her head, meaning she is being 'internally decapitated'

Prior to this, she was diagnosed with genetic condition Ehlers Danlos Syndrome (EDS), a connective tissue disorder

Prior to this, she was diagnosed with genetic condition Ehlers Danlos Syndrome (EDS), a connective tissue disorder

Roughly one in 15 people develop CCI as a result of having EDS, due to the lack of tissue support at the craniocervical junction.

Ms Smith says she was only officially diagnosed with the conditions in August last year after being misdiagnosed eight times.

Speaking to the MailOnline, she said she felt ‘relieved’ after being diagnosed but is now concerned for her future for the sake of her children, Jensen, eight, and Brooke, seven.

What is EDS and CCI? 

Ehlers Danlos Syndrome (EDS) is a a connective tissue disorder which is inherited.

According to the Ehlers-Danlos Society, each EDS subtype has a set of clinical criteria that help guide diagnosis; a patient’s physical signs and symptoms will be matched up to the major and minor criteria to identify the subtype that is the most complete fit.

A rare number of people go on to develop Craniocervical Instability (CCI) as a secondary illness after initially having EDS. 

Craniocervical Instability means the ligaments in her neck are too weakened to support her head

Some people develop it after experiencing an injury, such as whiplash, while others will get it after repetitive movements, such as turning their heads.

Symptoms of CCI include severe headaches, dysautonomia (which can trigger fainting, a rapid heart, chronic fatigue and low blood pressure while standing), neck pain, impaired coordination and paralysis. 

Ms Smith, who is from Littleborough, near Rochdale, Greater Manchester, said: ‘When you suffer for 30 years with multiple conditions, you start to research things. I was seeing six specialists and none of them were joining the dots up as they weren’t communicating with each other.

‘A lot of them were just treating the symptoms rather than the cause of the symptoms.'

Ms Smith said it was a geneticist who finally diagnosed her with EDS – but by the time the diagnosis happened, she was discharged by her rheumatologist.

Her geneticist told her rheumatologist that Ms Smith needed one during the treatment process, but the rheumatologist said there wasn’t a specialist on the NHS who can treat EDS, so Ms Smith needed to go privately.

She added: ‘I was then diagnosed with Craniocervical Instability by Professor Rodney Grahame in London, who told me I needed certain tests and urgent surgery, which aren’t available on the NHS.’

There are only three surgeons in the world who can conduct the life-saving operation Ms Smith needs.

Two are located in America and one is in Barcelona.

After visiting Dr Gilete in Barcelona, who is one of the surgeons capable of performing the complex surgery, Ms Smith was informed she is also suffering from Atlantoaxial Instability (AAI), which means her top two vertebrae in her neck are unstable.

He also told her she has just one month to have the operation, otherwise the damage from the diseases will become irreversible, meaning her body will slowly shut down and she will 'internally decapitate' at any moment.

The single mother said: 'I want to go to America, and Dr Henderson is able fit me in, but I'm concerned about flying the whole way, and the costs are significantly more. I'm worried I won't be able to save the amount before the deadline.'

She said she's desperate to raise enough money to have the operations so she can continue raising her children

She said she's desperate to raise enough money to have the operations so she can continue raising her children

The scan above shows that Samantha's vertebrae have been dislocated as a result of the conditions

The scan above shows that Samantha's vertebrae have been dislocated as a result of the conditions

Dr Gilete also diagnosed Ms Smith with Atlantoaxial Instability (AAI), which means her top two vertebrae in her neck are unstable

Dr Gilete also diagnosed Ms Smith with Atlantoaxial Instability (AAI), which means her top two vertebrae in her neck are unstable

She currently crowdfunding to raise £150,000 for life-saving surgeries in Barcelona, but is hoping to raise an additional £50,000 to have it in America instead

She currently crowdfunding to raise £150,000 for life-saving surgeries in Barcelona, but is hoping to raise an additional £50,000 to have it in America instead

In Barcelona, the £100,000 will fund the for the first two stages of the surgery, and the £50,000 will cover the further surgeries and tests that are needed.

But for America, the total amount needed is £200,000.

This is due to the cost of the surgery being £150,000, plus an additional £50,000 for living costs for Samantha and her two children and the follow-up treatments.

While tearfully explaining her plight, she said: 'It's really difficult as I feel like I'm going to only raise enough money for Barcelona, but my heart is saying to go to America for the benefit for me and my children's future.'  

  • To donate to Samantha's fundraising page, click here.

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