Brave young hearts: paediatric heart transplants at the Freeman Hospital

Physiotherapy plays a vital part in one of the UK’s leading paediatric heart transplant units, as Robert Millett discovered.

In 1987 Kaylee Davidson was diagnosed with viral cardiomyopathy, an infection that causes inflammation of the heart muscle. She was only four months old and the damage to her heart was irreparable. Her only hope for survival was an infant heart transplant – a procedure that had yet to be performed successfully in the UK.  
Kaylee was treated at the Freeman hospital, part of Newcastle upon Tyne NHS trust. The hospital became the first in the UK to carry out a successful infant heart transplant and Kaylee, thankfully, went on to make a good recovery. Since then, more than 250 children have received a transplant at the hospital and its surgeons now operate on around 300 babies and children each year.
The hospital’s paediatric intensive care unit (PICU) is run by a multidisciplinary team, which includes physiotherapists, nurses, surgeons, occupational therapists, psychologists, play therapists and teachers.
Karen Caulfield, an advanced paediatric cardiothoracic physiotherapist, has been part of the team for five years. She explains that the unit treats children who need medical or surgical care for any heart or lung condition. ‘The unit is one of only two places in the UK that offers paediatric cardiothoracic transplants, [the other is Great Ormond Street in London],’ she says. ‘So children who have complex cardiac conditions requiring surgery are referred to us from all over the UK.’
Mrs Caulfield and her physiotherapy colleagues’ day-to-day roles involve respiratory and rehabilitation care for the children on PICU and the children’s heart ward. She says working on the unit requires neurological, respiratory and musculoskeletal skills. Physios attend to the children throughout the care pathway, assisting them if they depend on a ventilator, providing pre-and post-operative rehab and linking up with community services after they are discharged.
‘We care for children from newborns to adolescents who have a range of heart, lung and airway conditions,’ says Mrs Caulfield. ‘But most of our children tend to be small, and the majority are under 10.’

Unpredictable pathway

Mrs Caulfield says children come to the unit for a variety of reasons, and their length of stay varies accordingly.
‘A child can be completely fit and well then have a viral infection, end up with cardiomyopathy and deteriorate quickly,’ she says. ‘Or the child might have been born with a complex cardiac problem and require many surgical procedures over a long period.’ 
The element of uncertainty is heightened by that fact that children who require a transplant are on a waiting list, which depends on suitable organs being donated.
Criteria including the size of the child, the maximum donor size acceptable, blood group and antibody status are all deciding factors. Unfortunately, she adds, donor hearts are a scare commodity and there is an increasing number of children on the waiting list for heart transplantation. 
‘It is very unpredictable, as we never know how long patients will have to wait,’ says Mrs Caulfield. ‘Some children wait for months and months before a suitable heart becomes available, while others are very lucky and one becomes available within a couple of days.’

Mechanical assistance

Children who may have previously died while waiting for a heart transplant can now be kept alive thanks to technological advances, such as the support of a mechanical ventricle assist device (VAD). ‘If a child has dilated cardiomyopathy their heart gets larger and larger,’ says Mrs Caulfield. 
And as the heart continues to grow it prevents the lung from fully expanding, causing it to collapse. ‘But the VAD helps to offload the heart and reduce its size a bit,’ she explains.‘And our role is then to help re-inflate the part of the lung that has been squashed.’ 
One disadvantage of this is technique is that mechanical assist devices carry a significant risk of stroke. This is because children need to be on the blood-thinning medication heparin while the device is used, as the attaching cannulae can interrupt blood flow and cause clots. Another potential impact of using a VAD is the psychological effect on parents and families, says Mrs Caulfield. This is because the machine is driven by a pneumatic pump that attaches to the child via a large cannula sewn into the chest and protrudes out of the body.
If a child is supported by a VAD before surgery families can be anxious about their child getting up to exercise, and the children may also feel nervous about moving.
‘When you first see a VAD it is a very scary looking thing and it can be really hard for parents,’ she says. ‘They sometimes think “How do I touch my child with this thing sticking out of their chest?”. But seeing them grow and learn to be able to do that is one of the nice things about the job.’

Rehab and family support

The physios aim to maintain function, keep the children motivated and encourage independence in an age-appropriate way. Mrs Caulfield says the team strives to provide family-centred care, which means looking after both the child and their family. ‘We really try to promote that parents be parents, and provide a normal environment in a semi-abnormal place.’
Improving physically fitness before a transplant can help to minimise contractures, reduce muscle wasting and aid post-operative recovery, says Mrs Caulfield. ‘When they first come in, if they are stable enough and aren’t on a mechanical assist device, we keep them moving as much as possible,’ she says.
‘This can include bed exercises and getting them out of bed for cuddles with their parents. But if they’re on intensive care then we are limited to working within the bed space.’ Children waiting on the ward also have access to a playroom and a sensory room. In addition, because many of the children are very young, rehab is often disguised as a game.
‘If we have goals about getting a child up and mobile then we might use play to do that,’ she explains. ‘And if we are doing some stretches and a child gets distressed a play specialist might come and do some distraction while we do the exercises.’ 
Combating boredom and ensuring children reach their developmental milestones are other issues for patients who are hospitalised for so long, says Mrs Caulfield. ‘We try to have a daily plan for patients so they are not continually being prodded and poked and investigated,’ she says. ‘We work with the family and give advice about how they can get involved and do normal things like washing and nap times.’ 
There are also challenges around motivation and how engaged some of the children are with getting functional and mobile again. ‘It’s quite hard to motivate children who are then going to have a massive operation that will set them back again,’ says Mrs Caulfield. ‘They aren’t rehab-ing to an end goal of getting fit and healthy and out of hospital – you are rehab-ing them to be as good as they can get before a transplant.’
In such cases the physiotherapists often work with the team’s psychologists to find new ways of motivating the children. Mrs Caulfield says no two days are ever the same on the unit and she admits the work can be emotionally taxing. ‘It could be perceived as quite a sad place to work, as we’re working with critically ill children all the time,’ she says. ‘It is emotionally challenging, especially as I have children myself. The journey that the families have is very difficult ... and some children don’t survive.’
In such cases the team benefits from good supervision, she says, and debriefing sessions are held whenever staff have to deal with distressing situations. ‘There are difficult cases, and of course some of the children do pull at your heartstrings,’ she says. ‘But many of them do get better. And when you see a child go home with their family and get back to leading a full life you realise there are so many positive things that come out of this work.’ fl

More information

In 2014, 17 heart transplants were performed on children at the Freeman; ventricle assist device support was given to 16 children, nine of whom went on to have transplants. 
Robert Millett

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