Hari Jones, six, from Gwynedd, has X Linked Myotubular Myopathy (XLMTM), which affects about one in every 50,000 male births - he cannot walk or sit up and is connected to a life support machine that needs constant monitoring

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Wales: Boy with rare genetic disorder desperately needs care

A family says they are at a "breaking point" as they wait for a full care package for their son who has a rare genetic disorder.

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

He cannot walk or sit up and is connected to a life support machine that needs constant monitoring.

The parents say they've been told he would get a full care package, but because of "ongoing issues" they have stepped up as Hari's carers while they wait.

Hari is unable to attend school and requires constant supervision in case the tube in his throat blocks or his breathing stops.

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Dad Michael Jones had told the BBC that "poor management" has "let them down as a family" and it is harming their physical and mental health as they have to step up as carers.

He says that he also has health conditions and was in a coma in 2018, which means a lot of care has fallen onto Hari's mum 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.

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"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."

Mr Jones added: "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."

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The health board has said it would need time to recruit and train staff 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.

Hari spent four years on and off at Alder Hey Children's Hospital in Liverpool.

He was then assigned a care package that included carers being with the family almost 24 hours a day.

The health board took over from a private company in early 2021 - and the family say it reduced contact hours and used agency staff who were unable to fulfil the hours.

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

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

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

"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.

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"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 upon by a multidisciplinary team and the family.

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

Liz Fletcher, Assistant Area Director for Childrens Services in the West of Betsi Cadwaladr University Health Board, told The Mirror: We are continuing to work closely with Haris parents, Mr and Mrs Jones, to discuss the plans we have made to ensure that Hari has safe and ongoing care.

We are fully committed to providing the package of care for Hari and his family as an in-house arrangement, rather than via an external provider.

"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 familys needs.

"However, due to Haris 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.

Two Health Care Support Workers and three registered nurses are now providing regular consistent care and we are actively recruiting more staff who will work as a team to support Hari.

"The team will be gradually introduced over a phased period according to a plan that has been discussed and shared with Mr and Mrs Jones.

We recognise there are challenges at times with staffing and when this occurs it is communicated to the family, who are updated regularly throughout the week.

"We do continue to offer meetings with Mr and Mrs Jones to discuss the package of care and any ongoing concerns they have.

We continue to look at all possible ways to support Hari at home recognising the challenges for everyone involved.

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Rocio Cifuentes, the children's commissioner for Wales, told The Mirror: "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."

A Welsh Government spokesperson told The Mirror: "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."

The Mirror contacted the Carers Trust for comment.

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Family at 'breaking point' amid difficulties caring for son with rare genetic disorder - The Mirror

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