Samantha Smith receives provisional surgery date, but still needs your help

Samantha Smith with her children, Brooke and Jensen

Samantha Smith has received a provisional surgery date, but still needs the public’s help to fund further tests and surgery.

Sam, whose brain and spinal cord are being crushed under the weight of her own skull, flew out to Washington DC last week to meet with her surgeon and has undergone around eight further medical tests already, with a further eight to be carried out before her provisional surgery date on Tuesday 11 July.

Each test so far has cost between $350 (around £270) and $1,500 (around £1,160). Sam is undertaking blood and genetic tests over concerns of a rare blood clotting condition.

She said: “I can pay for some of the surgery afterwards, but I’ll need to consult with the surgeon, anaesthetist, the hospital and there’s the possibility of flying a haematologist in from China as they’re concerned I might have a rare clotting disorder. So I’m having a lot of blood tests for that. I’m having genetic tests too to rule out vascular EDS, because no-one wants that.”

The single mum of two, from Smithy Bridge, was diagnosed with Ehlers Danlos Syndrome (EDS) in December 2016, after ‘a lifetime of pain and eight years of tests, misdiagnoses and ill health’. 

EDS is a genetic condition that means connective tissue holding the body together is faulty and too weak, causing extreme widespread pain, daily dislocations, cardiac abnormalities and multiple other illnesses. It affects around one in 5,000 people worldwide.

The vascular subtype of EDS is the most serious form where blood vessels or organs can rupture spontaneously from the slightest trauma.

Sam also has to wear a hard neck brace daily as her neck ligaments are too stretched and weakened to support her head in a condition known as Craniocervical Instability (CCI). CCI can arise in EDS sufferers due to a lack of connective tissue support at the craniocervical junction (between the skull and first two vertebrae of the spine).

The 30-year-old is also at high risk of internal decapitation every day due to the weakness of her ligaments and the formation of scar tissue which is pressing on her brain stem.

Having deteriorated rapidly since her initial diagnosis, Sam says her ligaments are now virtually non-existent and also requires a new neck brace with more support. She has also received diagnoses including tethered cord syndrome, Atlanto Axial Instability (unstable vertebrae which are easily dislocated).

The surgery in Washington will be carried out in a different way to as reported previously.

Sam explained: “My surgeon will use a different fusion of hardware and tape that works like ligaments as my neck ligaments are virtually non-existent. He’s changed his surgical plan four times since I’ve been out here.

“Some people have had the surgery and their symptoms have improved, but there is only the guarantee I won’t get worse.”

Crushed bone will be mixed with bone marrow from Sam’s hip and used to fill in the space in her neck and cervical spine. Over the course of six months, new bone will form and build over the sites.

She continued: “I’ll need lifelong treatment. I’ll need to be back in a few months’ time. I am concerned because I’m having to spend money on each test. I’ve had eight so far and I’ll need about another eight.

“People have been so amazing, raising the amount they have in such a short period of time. But it is scary when the fundraising slows down, especially as I don’t know how much more I need for tests and surgery.”

Sam will also require surgery for the tethered cord to release her spinal cord from her lower vertebrae as it has become so tight, her back is starting to bend with scoliosis. She currently struggles to stay upright for any period of time and suffers from intermittent paralysis.

She will also require surgery for a dislocated jaw, which is also related to her existing medical conditions.

To donate towards Samantha’s life-saving surgery, visit:

https://www.youcaring.com/samanthasmith-788861