The teen living with one of the world’s most painful conditions

Monday, 22 January 2024

Ella wants to raise awareness of CRPS and other rare diseases.

An Achilles strain from playing rugby aged 13 spiralled into the “worst five years” of Ella Dixon’s life.

The day after the Invercargill teenager was injured in 2018, her foot began to feel like “it was on fire, whilst also being run over by a train”. It turned purple, swollen, and cold.

She was diagnosed with complex regional pain syndrome (CRPS), regarded as one of the most painful of all known diseases.

She says limited awareness and understanding of the rare condition, uneven access to pain services, and slow diagnoses have a major impact on people’s lives – and wants to see change so others “do not have to experience the invalidation I felt”.

CRPS is an uncommon form of chronic pain that typically develops after an injury, surgery, or stroke or heart attack, which tends to affect an extremity: often an arm, leg, hand or foot.

In a widely used measure of self-reported pain, the McGill Pain Questionnaire, CRPS has a mean score of 42 out of a possible 50 – higher than scores of patients who have experienced a limb amputation, or childbirth.

CRPS has turned Ella’s life upside down.

Her mother, Megan Dixon, said it was hard for Ella to be driven in a car given the “unbelievable” level of pain she was in. Wind, and even getting her foot wet caused her agony.

She required multiple hospital admissions and treatment from pain services in Auckland, at the opposite end of the country.

About a year into her CRPS, while in hospital for a ketamine infusion for pain, Dixon experienced loss of function in her arm and right leg.

She was diagnosed with a functional neurological disorder (FND) – which features nervous system symptoms that can't be explained by a neurological disease or other medical condition.

However, its symptoms are real, and cause significant distress or problems functioning, the Mayo Clinic said.

Between CRPS, FND, and regular hospital stays, Ella was unable to continue going to high school, and instead had a teacher from Southern Health School teach her at home, her mum said.

“It’s not been easy,” Megan said.

Ella had to stop all sport, and spent most of the past four years using a wheelchair.

Megan moved to working part-time, so she could be home two days to care for Ella, and they had a carer home for the other three; and she and husband Steve tag-teamed flying back and forth from Invercargill to Auckland for Ella’s treatment.

“At one point, I was doing just about everything for her.”

Ella needed help to be transferred from her bed to her wheelchair; to get onto the toilet; to shower; and to set up her tube feeds.

She underwent intense rehabilitation, including physiotherapy and occupational therapy.

Today, Ella’s CRPS is more manageable, and her mum says she is ‘really well on the road to recovery’. They hope her story can give hope to others living with CRPS.

“I couldn’t do any of the tasks a normal teen [would]. I really struggled with that,” Ella said.

Ella wanted to be out and about, active, and social. She’s independent, and didn’t like having to rely on her parents: “I don’t like this word, but I just wanted to be normal.”

Ella said because they are “invisible illnesses”, few around her had heard of CRPS or FND, and she found some people didn’t believe the pain or symptoms she was experiencing.

She said she found herself having to explain and educate people, which was “disheartening” and added to the burden she already felt.

Ella said better awareness, advocacy and treatment – including more joined-up services – are needed for Kiwis living with rare disorders like CRPS and FND, so those affected are better supported.

Today, Ella’s CRPS is more manageable: she drives, works a part-time job, and can socialise again. She even got to go to her school ball.

CRPS can respond to treatment – especially if started quickly.

Megan said it has been a really tough road, but with a lot of hard work, the right support and the right team, “Ella is really well on the road to recovery”.

They wanted others living with CRPS, or who have a loved one with CRPS, to know that things can improve.

Wayne Pickering has debilitating chronic pain. With no treatment available to him, he lives each day in fear, wondering when his next pain attack will happen.

“There’s hope and there’s a future,” Megan said.

It’s estimated one in five adults in Aotearoa suffer from chronic pain.

Dr Nick Baker, paediatrician and Interim National Chief Medical Officer at Te Whatu Ora, said health agencies are working with Rare Disorders New Zealand to develop a rare disorders strategy, timed for completion in the first half of 2024.

Baker said all Te Whatu Ora districts have pain management services to support adults and children, delivered in a variety of ways across the country, supported by multidisciplinary teams including doctors, nurses, clinical psychologists, occupational therapists and physiotherapists.

While most areas have their own service, support may also be delivered through regional relationships or outsourcing, he said.

Baker said people living with chronic pain waiting to access pain management services is a global problem.

Work is underway on an integrated population-based approach for people with chronic pain in Canterbury that could be utilised in other areas of the country if it proves successful.

This work was consistent with a recent editorial in The Lancet which called for a shift in care of people with chronic pain from tertiary centres to care grounded in the community, supported by primary care, Baker said.