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Better care ahead for infants and parents

Karine Latter has developed national nursing standards for infants born with Pierre Robin sequence (PRS), a rare cleft palate condition that causes breathing and eating difficulties. The standards, based on her own research with PRS families, aim to provide national consistency in nursing care. Ms Latter is the runner-up in the Child Health category of this year’s Nursing Standard Nurse Awards.

Karine Latter with six-month-old Tilly, who has PRS

Picture credit: Tim George

A set of national nursing standards designed to improve the care of children with a rare cleft palate condition has been created by a nurse specialist.

Karine Latter developed the standards for infants born with Pierre Robin sequence (PRS), which results in breathing and feeding difficulties, after her research showed significant variations in care around the UK.

Ms Latter, lead clinical nurse specialist in the Trent regional network for cleft lip and palate, had set out to build an evidence base to improve the outcomes for infants born with PRS and raise nurse specialist practice nationwide, based on parents’ feedback.

‘I’m passionate about qualitative research, exploring patients’ experience of care,’ she says. ‘I did an MSc in advanced nursing practice about parents’ experiences of having a late diagnosis for cleft palate.

‘I became concerned at the lack of evidence on care for infants with PRS and their families – there was no established best practice. So I self-funded and developed a multi-centre qualitative doctorate research study at the University of Nottingham exploring parents’ experience.’

Ms Latter contacted the lead nurses from five national cleft networks and recruited 25 families into the study, conducting in-depth, open-ended interviews in their homes.

‘Only 80 babies a year are born with the condition in the UK so it was a reasonable sample of parents,’ she explains. ‘The study explored parents’ lived experience from six to 18 months following birth, to identify gaps in care provision and inform nursing practice based on expressed needs and perspectives.’

Stephen Hilton, whose son was diagnosed with PRS at birth (see panel), took part. He recalls: ‘The whole experience was pretty traumatic. I had dealt with it mainly by burying my head in the sand without really processing it.

‘However, it was cathartic to be able to talk about all the struggles we went through and to know that they would contribute to making a positive difference.’

Pierre Robin Sequence

Pierre Robin sequence (PRS) is a rare condition with an incidence of approximately 1:15,500 births.

First described in 1923 by Pierre Robin, a French doctor, it is also known as Pierre Robin syndrome, Pierre Robin triad and Pierre Robin anomalad.

In affected children the condition involves a cleft palate, a small lower jaw and a tongue that can sit high up into the cleft palate, causing partial or full airway obstruction and feeding difficulties.

It can occur on its own (isolated PRS) or in conjunction with a syndrome.

PRS presents on a continuum of severity, ranging from mild to severe.

Treatment depends on the severity of the condition, with most cases in the UK being managed by a nasopharyngeal airway (NPA) – a plastic tube inserted via the nose, sitting on the tongue and pressing it down and preventing it from obstructing the airway

The average time the NPA remains in place is seven months.

The cleft palate is repaired approximately between nine and 12 months.

Responsibility

Ms Latter says: ‘It became evident that the care differences across the five regions were huge, with real consequences for infants and families.

‘I needed to use the findings to achieve better consistency in specialist care, more information for parents and the development of minimal nursing standards. There is no point in conducting research and not disseminating the findings. I also felt strongly that I had a responsibility to the parents who had shared their sometimes traumatic experiences with me to use that information to make care better.’

The standards Ms Latter drew up were put out for consultation to national groups, parents of infants with PRS and national clinical nurse specialists in cleft lip and palate. She used the National Clinical Excellence Network and the National Lead Nurse Group for Cleft Lip and Palate, and gave presentations at regional specialist study days for cleft lip and palate.

Ms Latter and Tilly’s sister Isla and parents Rachel and Andrew Johnson

Picture credit: Tim George

‘Getting patient feedback was difficult, given the rarity of the condition and the spread of such a large geographical area, so I posted the standards on the national Facebook page for PRS,’ she says. ‘This generated discussion and feedback from parents nationally.’

Each draft has gone back to the parents for more feedback. They have indicated that the standards would have improved the care their children received.

It took Ms Latter five years to complete her doctoral study while continuing to work full time. The standards took a further two years to develop.

‘It was worth it,’ she says. ‘I am excited that the standards will make a huge difference to the children’s experience of care and their families.’

Her achievement was recognised in this year’s Nursing Standard Nurse Awards, when she was named runner-up in the Child Health category.

Mandie Sunderland, chief nurse at Nottingham University Hospitals (NUH), where Ms Latter is based, is confident her work will improve care. ‘We’re extremely proud of Karine and all that she has achieved,’ says Ms Sunderland. ‘She has helped scores of cleft patients and their families in her time at NUH.’

New resource

The standards were ratified in April at the annual meeting of the National Nursing Clinical Excellence Network for Cleft Lip and Palate, and they will be audited in the first year.

By the end of the year Ms Latter hopes to complete best practice guidelines to sit alongside the standards. Next on her list is the creation of an app, which parents will help to develop. ‘It would have the potential to help parents in other countries as well,’ she says.

Mr Hilton, for one, is keen to be part of that mission, creating better resources for all parents of PRS children. He says: ‘Karine is a driving force for good in our PRS world’.

‘As our son grew, he began to struggle’

Stephen Hilton’s son was born at 39 weeks by elective caesarean section and taken straight to the paediatric intensive care unit (PICU), struggling to breathe.

‘He was diagnosed with Pierre Robin sequence (PRS) the day after his birth by a Trent cleft specialist nurse named Heather,’ says Mr Hilton.

‘We had known something was wrong as his 20-week scan had shown he had a small chin. This started 19 weeks of scans, negative tests, more scans and polyhydramnios (excessive amniotic fluid) from 28 weeks – but we had no definite idea about PRS until Heather told us.

‘I remember it vividly, standing in the strange environment that is the PICU, needing to know what the future held. Heather was great, very matter of fact, breaking it to us gently but firmly that he would need to be NG (nasogastric tube) fed for the time being and his oxygen saturation would need to be monitored.

‘To be honest, we felt a little relieved, as before he was born we were unsure of what his diagnosis would be, but it was still tough.

‘However, we adapted our life and just got on with it – learning to pass an NG tube and feed him the expressed breast milk my wife was spending the majority of her time producing.

‘The nurses were caring, but they didn’t know much about PRS and what we could be faced with, since they had hardly seen any cases. We thought that if we could escape the PICU without any airway support this meant we were home and dry.

‘We managed to do this after three weeks. However, as he grew he started to struggle more and more with his breathing. At six weeks old, Karine had to tell us he needed a nasopharyngeal airway (NPA). This would act like a splint and would prevent his tongue obstructing his airway.

‘We found this development particularly devastating, because as well as having to go into hospital for another week to learn how to manage this, it felt like a huge step backwards. Later we found out this is pretty common in PRS babies, as everything grows at different rates and this can cause issues.

‘We had lots of questions about the future but the medical profession is generally reluctant to be definitive when things are uncertain, as they so often are with PRS. So we did what everyone does now – we turned to Google. Most information was from the United States, where intervention is much more intensive. This often led to a lot of worry about what the future held.

‘It would have been far better to have an official UK source of information that covered all the bases and potential treatments, which we could digest in our own time. This could also help the medical professionals give better guidance when the cleft specialists aren’t around.’

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