Caernarfon family’s despair over care for son with rare condition

One family says they are at “breaking point” by providing 24-hour care for their son with a rare genetic disorder.

Hari Jones, six, from Caernarfon, Gwynedd, cannot walk or sit and has a life support machine that requires constant monitoring.

His parents said they had been told he would be given a full care package but there were “ongoing problems”.

Betsi Cadwaladr’s health department said it would take time to recruit and train staff due to Hari’s complex needs.

A spokeswoman said the board was recruiting more staff and “continued to work closely” with the family.

Hari, from Caernarfon, Gwynedd, has X-Linked Myotubular Myopathy (XLMTM), which affects approximately one in every 50,000 male births.

Due to the severity of his condition and the constant need for a caregiver, he is unable to attend school. He needs constant monitoring in case the tube becomes blocked in his throat or his breathing stops.

After more than four years at Alder Hey Children’s Hospital in Liverpool, Hari was placed on a care package that included regular home visits, with carers being with the family almost 24 hours a day.

The health department took over from a private company in early 2021. The family said it reduced contact hours and used contract workers who often couldn’t meet the hours.

Hari’s father, Michael Jones, said “bad management” had failed them “as a family” and the lack of an adequate care package was having a negative impact on their physical and mental health as they needed to step up as carers.

Mr Jones also had debilitating health issues and was in a coma for some time in 2018, meaning much of the care falls to Hari’s mother, Ellen.

Mr Jones said: “She’s with him day and night, mainly because I’m still feeling really bad, so basically Ellen’s life is alongside Hari, she can’t leave the house, she’s with him constantly and makes sure he is safe.

“She’s a nurse to him, a doctor, a physical therapist, she manages all his supplies, she does absolutely everything for him day and night – to the point where she doesn’t sleep for days, so it’s a lot of stress for her .

“At worst, Ellen has been working 12 days and 12 nights straight and her base hours are 36 hours with no sleep, so it’s a lot of work.”

Mr Jones added: “There is no company that has accepted the contract and they say there are restrictions because we live in Wales.

“They say they can’t pick grooms out of thin air, they can’t knit grooms.”

“They should just find answers – it’s not something that happened overnight for them.

“They knew this was going to happen and was going to happen since March of last year, so they should have had something ready by now to help us.”

The family said they were offered respite care at a hospice in Chorley, Lancashire, almost three hours from their home.

“We lived with Hari at Alder Hey for four years and it was important for us as a family to come home,” he said.

“They call it respite — we don’t need respite, we need help.”

“I understand there are staffing issues in all areas – in the hospitals and stuff post Covid, but they were forewarned this was going to happen – they’ve had plenty of time to find people, teach them and get them are qualified and on a level to help us now,” he said.

“Certainly this should be advertised far and wide for anyone to try as long as they have the right attitude, are taught to do it and as long as they are safe then anyone can do the job. “

He says: “I hope he still breaks every statistic that any doctor gives him, but the reality is these little boys are precious and they are fragile.

“Maybe I look back and think we wasted all that time fighting the system when all the system should have been was giving us the help we deserve and need and he should be getting, to do with every day to live the way it is in the end because we don’t know what’s going to happen next.”

Neeru Naik of Muscular Dystrophy UK – a charity which supports more than 60 conditions including XLMTM – said there was “definitely inconsistency” over the care packages.

He said after Covid and Brexit, getting a pool of carers relevant to needs was a major difficulty.

Another challenge is that “these are rare diseases that require very complex care”. Finding and training suitable staff “is a much more difficult process”.

dr Carers Trust’s Catrin Edwards said there was “huge pressure” on families and unpaid carers.

“There is no doubt that our health and welfare systems depend entirely on unpaid carers and we must recognize the burden on them.

“We need to remember that caregivers have legal rights and a person must be willing and able to provide care. However, when there are so many gaps in the workforce, many caregivers feel they have no choice but to continue despite the personal strain.”

Betsi Cadwaladr’s health department said Hari’s care needs have been set at 168 hours per week, a plan agreed by a multidisciplinary team and the family.

It said it “compares well with other care packages across the country”.

Liz Fletcher, of the board, said she was “fully committed to providing the care package for Hari and his family as an internal arrangement” and was in touch with the family.

She said: “We made this decision due to the complexity of the package required and the lack of appropriate agencies and care providers nationally that can meet the needs of the family. However, due to Hari’s complex needs, it will take time for us to recruit and train the number of suitably qualified personnel required to fully provide this care package.”

She said that two health care workers and three registered nurses provide regular and consistent care, and are actively recruiting more staff to help Hari to phase it in.

Rocio Cifuentes, Children’s Officer for Wales, said: “For children and young people across Wales who have complex needs, there is often difficulty accessing the help and support they need. We want care to be much more accessible to children and we want it to suit them and their needs.

“Access to robust support at home and regular short breaks are a hugely important part of any care package – so improving access to this for children and families across Wales must be a focus for future services.”

A Welsh Government spokesman said: “We are investing heavily to support the recruitment and retention of social workers in Wales, including paying social workers with real living expenses.

“Local authorities are also working closely with health officials to increase the number of home care workers. We have delivered a record number of NHS staff – a 54% increase over the past 20 years – and more qualified nurses, hospital advisors and ambulance staff than ever before.”