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

A family say they are at "breaking point" giving round the clock care to their son with a rare genetic disorder.

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

His parents said they were told he would get a full care package, but there have been "ongoing issues".

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

A spokeswoman said the board was recruiting more staff and "continuing to work closely" with the family.

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

He cannot attend school due the severity of his condition and the need for a carer at all times. He requires constant supervision in case the tube in his throat blocks or his breathing stops.

Mum Ellen is 'nurse, doctor and a physiotherapist' to Hari

After more than four years on and off at Alder Hey Children's Hospital in Liverpool, Hari was assigned a care package including regular home visits, in which carers would be with the family almost 24 hours a day.

The health board took over from a private company in early 2021. The family said it reduced the contact hours and used agency staff who were often not able to fulfil the hours.

'Hari's mum can't leave the house'

Hari's father Michael also has health conditions which means a lot of the care has fell to Hari's mother Ellen, he says

Hari's father, Michael Jones, said "poor management" has "let them down as a family" and the lack of an adequate care package is having a negative effect on their physical and mental health as they have to step up as carers.

Mr Jones also had debilitating health conditions and was in a coma for a period in 2018, meaning a lot of the care falls to Hari's mother, Ellen.

Mr Jones said: "She's with him day and night, mainly because I'm still really unwell so Ellen's life is basically next to Hari, she can't go out of the house, she's constantly with him and making sure he's safe.

"She's a nurse for him, a doctor, a physiotherapist, she manages all his stock, she does absolutely everything for him day and night - to the point where she doesn't sleep for days and days so it's a lot of stress for her.

"At the worst, Ellen's done 12 days and 12 nights non-stop and her basic hours are 36 hours without sleep, so it's a lot of work."

'Can't pluck carers out of thin air'

Mr Jones added: "There's no company that's taken the contract up and they're saying it's because we live in Wales, that there's limitations.

"They say they can't pluck carers out of thin air, they can't knit carers."

"They should just find answers - it's not something that happened overnight for them.

"They've known that this was going to happen, and happening, since March last year so they should have had something in place to help us by now."

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

"We've lived for four years in Alder Hey with Hari and it's been 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 that there are problems with the staffing in all areas - the hospitals and stuff after Covid but they'd been pre-warned that this was going to happen - they've had enough time to find people, to teach them and get them qualified and up to a standard to help us by now," he said.

"Surely this should be advertised far and wide so anybody can try it, as long as they've got the right attitude, they'd get taught to do it and, as long as they're safe, anybody could do the job."

Hari's father said he had developed a Scouse accent while in hospital in Liverpool on and off for four years

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

"I might be looking back and thinking that we've wasted all that time fighting the system, where the system should have just been giving us the help that we deserve and need, and that he should get, to live every day as it's the last because we don't know what's going to happen next."

'Huge pressure on families'

Neeru Naik, of Muscular Dystrophy UK - a charity which supports more than 60 conditions including XLMTM - said there were "definitely inconsistencies" in terms of care packages.

She said post-Covid and Brexit, a big difficulty was getting a pool of carers relevant for needs.

Another challenge was that "these are rare conditions that have very complex care needs". Finding appropriate staff and training them "is a much more difficult process".

Dr Catrin Edwards from the Carers Trust said there was "huge pressure" on families and unpaid carers.

Hari spent four years in hospital in Liverpool and the family says they just want to be at home

"There's no doubt that our health and social care system is totally dependent on unpaid carers and we have to acknowledge the strain on them.

"We have to remember that carers have legal rights and an individual has to be willing and able to provide care. However, when there are so many gaps existing in the workforce, many carers feel they have no choice but to continue, despite the personal strain."

Betsi Cadwaladr health board said Hari's care needs were set at 168 hours per week, a plan which had been agreed by a multidisciplinary team and the family.

It said it "compares favourably with other packages of care across the country".

Liz Fletcher of the board said it was "fully committed to providing the package of care for Hari and his family as an in-house arrangement," and is liaising with the family.

She said.: "We have taken this decision due to the complexity of the package required and the lack of suitable agencies and care providers nationally that can fulfil the family's needs. However, due to Hari's complex needs, it will take time for us to recruit and train the number of appropriately skilled staff required to deliver this care package in full."

She said two health care support workers and three registered nurses were providing regular consistent care and they are actively recruiting more staff to help Hari, to be gradually introduced.

Rocio Cifuentes, the children's commissioner for Wales, said: "For children and young people throughout Wales who have complex needs, there are often difficulties in accessing the help and support they need. We want care to be much easier to access for children, and we want it to fit around them and their needs.

"Access to robust support at home, and regular short breaks is a hugely important part of any care package - so improving access to this for children and families across Wales must be a focus for services going forward."

The Welsh government said: "We are investing heavily to support the recruitment and retention of social care staff in Wales, including paying social care workers the real living wage.

"Local authorities are also working closely with health boards to increase the number of domiciliary care workers. We've delivered record numbers of NHS staff - a 54% increase over the last 20 years - and more qualified nurses, hospital consultants and ambulance staff than ever before."