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volume 1 issue 2

inside this issue: editor’s welcome editorial panel

2 2-3

wait, watch and wonder 4 hydrotherapy


t yˆ hafan care services


epidermolysis bullosa 10-13

15 years of being there

together for short lives conference


publications of interest and study days


sharing best practice in paediatric palliative care

sharing best practice in paediatric palliative care. volume 1 issue 2

welcome to the second edition of


Mandy Brimble, Editor, Lead Practitioner for Education and Research, T yˆ Hafan I am delighted to say that the response to our first issue was extremely positive. The journal was distributed both locally and nationally, so feedback came from near and far. I hope you will find the range of topics selected by the editorial panel informative and interesting. You can find out more about the editorial panel members in this issue. Following on from Cath Thompson’s article on the ‘Spiritual nature of the child’ in our last issue, we have ‘Watch, wait and wonder’ by Francis Edwards. Francis was appointed as Lead Practitioner for Hospice Palliative Care Services at tyˆ hafan late in 2013 and holds this post jointly with Beth Morgan. He also works as Palliative Care Liaison Nurse at the Royal Bristol Children’s Hospital. In celebration of the opening of our new hydrotherapy pool we also have an article outlining the benefits of this therapy by Alice Dawson, Specialist Paediatric Physiotherapist, Cardiff and Vale UHB, who works in partnership with Tyˆ Hafan. In addition, we have an article on respiratory symptoms in children with Epidermolysis Bullosa (EB) which has been written by Mai Mallari who is a nurse at TyˆŷHafan. This interesting piece is based on a recent assignment completed for the MSc module in Symptom Control in Paediatric Palliative Care at the University of South Wales (details on this course can be found in the ‘course, study days and conferences’ section). We also have a report on the Together for Short Lives Conference from Tracy Jones, Lead Practitioner for Community Palliative Care Services at Tyˆ Hafan. A lot has happened since my last editorial, including our Care Services Professional Development week (more on this later). We are extremely fortunate that T yŷHafan ˆ places such importance on the education, training and professional development of the staff who work for the organisation and this bi-annual event allows valuable time for professional


development which will enhance the care and services offered to the families we serve.

Finally, I would encourage you all to share your experiences, ideas and thoughts with us. Guidelines for submission of articles are included on the back page, or if you wish just send a letter, email or information sharing request. We would be pleased to hear from you.

editorial panel Mandy Brimble - Editor Prior to being appointed as Lead Practitioner for Education and Research at Tyˆ Hafan, I worked as a lecturer in children’s nursing at Cardiff University for nine and a half years. Before this I worked as a Health Visitor and as a

Cath Thompson - Head of Care Services at Tyˆ Hafan RMN, Diploma in Palliative Care, BSc Psychology, MA Healthcare Law and Ethics I qualified as a nurse in 1986, having completed my training in Birmingham and Coventry. I worked in many specialities of psychiatry including child psychiatry. As a ward sister I led a team of nurses working in acute psychiatry in Swansea and later moved to Powys to manage a community team of psychiatric nurses within an integrated mental health team. I moved into the charity sector in 1993, taking up the position of Family Care Officer for the Muscular Dystrophy Group, working with both children and adults with neuromuscular conditions in Wales.

In 1999 I was fortunate to secure the position of Family Support Worker at Tyˆ Hafan. I was instrumental in the development of the Family Support Service and, more recently, the formation of the Community Palliative Care Service. My particular interests are holism, ethics, law and end of life issues. I believe passionately in the philosophy of children’s palliative care and I am fortunate to have been able to play some small part in its development in Wales.

Melda Price - Lecturer and Field Lead for Children’s Nursing Pathway RGN RSCN BSc Child Health Diploma in DN MSc, BN Programme, School of Healthcare Sciences College of Biomedical and Life Sciences, Cardiff University

Another exciting event is our first Research and Innovation Conference which is entitled: Journeys and Destinations: Research and innovation in caring for children and young people with life-limiting and life-threatening conditions. This is being organised in partnership with Cardiff University and will be held at the Vale Hotel and Resort on 14 October 2014. It promises to be a highly informative, engaging and interesting day. Speakers are from a variety of backgrounds and we trust that delegates will find it a very worthwhile day. For those of you who are unable to attend we will include a report in our next issue.

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Children’s Nurse in general medicine, day surgery and outpatient services. In addition to my Nursing Diploma and BSc in Community Health Studies, I am a registered nurse prescriber, hold a post graduate certificate in Education and a Masters in Education. I will be commencing a Doctorate in Advanced Healthcare Practice in October 2014. In addition to my work at Tyˆ Hafan, I am an Honorary Clinical Tutor at Cardiff University. I am a member of the Royal College of Nursing, the Community Practitioners and Health Visitors Association, the Palliative Care Research Society and the National Association of Palliative Care Educators. I am part of the local Paediatric Palliative Care Education and Training Group and I am also involved in the South East Wales Academic Health Science Partnership, which is a research partnership between local academic and healthcare organisations.

I have previous clinical experience of working in primary, secondary and tertiary children’s nursing settings. I was team leader for the Community Children’s Nursing Service (Bromley Primary Care NHS Trust), managing a team of nurses including community children’s nurses, special needs health visitors and special needs school nurses. The team was successful in winning a Nursing Times and 3M award for innovative practice.

educational audits. I am currently a Children’s Nursing Field Leader for the Undergraduate Nursing Programme, School of Healthcare Sciences College of Biomedical and Life Sciences, Cardiff University.

I have expertise in management, leadership, development of services, and care management.

I take a keen interest in all aspects of community care for children and work closely with T yˆ Hafan, a Special needs school and Community Children’s Nursing services providing an educational link for the students and mentors.

Since joining the staff of Cardiff University in 2001, I have developed further expertise in curriculum planning, module management, teaching, assessment annual reviews and

Vera Clement - Palliative Care Clinical Nurse Specialist for Children & Young People 0-25 years I qualified as a State Enrolled Nurse in 1978 and continued to work on an acute paediatric ward until I qualified as a Registered Nurse in 1994 after completing my conversion course. Remaining on the acute unit, I was offered a place to do my Registered Sick Children’s Nursing Diploma at Swansea University and qualified in 1996. In 2001, again at Swansea University, I completed my BSc (Hons) Community Health Studies (Children’s Nursing) and achieved a specialist practitioner qualification through an approved degree programme.

I utilised this qualification to work and help set up a Community Children’s Nursing Team within the now, Cwm Taf UHB. I came into my present post as a Paediatric Palliative Care Nurse in 2010, from Sugar funding. I completed the Palliative Care Course (child module) in May 2013. The goal is to set up this service within our Health board and to bridge gaps and support children and young people who have palliative care needs. This can hopefully be achieved by liaising and working with professionals in the multidisciplinary team.

The aim of Insight is to give professionals clear and intuitive knowledge into published materials, bringing together expert knowledge and is a platform for sharing best practice in paediatric palliative care. I am looking forward to being a member of the editorial panel.


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watch, wait and wonder

members of the family will be at different stages on this very painful spiritual journey. In order to be able to respond to this call, you need to try and create a safe and secure place or what I have come to call a ‘sacred space’, where the child can express their inner suffering and know that it is all right to do so, that they will be heard and taken seriously. You can help them best by just sitting with them, watching with them, waiting with them and just wondering. Take your lead from them, go with them, do not try and direct them, and try and use the language and imagery they use.

Francis Edwards, BSc (Hons), RGN, RSCN. Palliative Care Liaison Nurse, Royal Bristol Children’s Hospital, England & Lead Practitioner for Hospice Palliative Care, Tyˆ Hafan Spirituality and spiritual care are the proper concerns of all who work with life-limited or life-theatened children and their families. It should be recognised that the issues of spirituality and religion are very important. However, they are two different aspects of care. It has been suggested that we all have a spiritual dimension, and some people also have religious needs. It is possible to have spiritual needs independently of religious needs. Religious needs are to do with a shared faith, beliefs, practices and rituals that help a person make a connection with their ‘God’. Spiritual needs are to do with our search for meaning and purpose and a sense of well-being and wholeness. Somebody once said that our spirituality puts us in touch with both our freedom and our destiny, enabling us to find our ‘selves’ and to find our way with others. These two pages are not about answering all the questions a child or parent may have that we often hear as professionals, such as “Why my child?” or “Why our family?”

or “What is the meaning of life?” or “Where the hell is God in all of this?” It’s worth remembering that nobody can give the answers to the profound questions the child or the family seek. What we can do is try and be ‘truly present’ while the questions are being asked and not try and explain them away with “platitudes” or give false hope. The questions need to be asked and that is the important thing. They need to be spoken out loud and that can be therapeutic. Within this short paper, no answers are given, but it is suggested that you do something that is far from easy for anyone to do. That is to sit with the child/ parent or a colleague and try and stay in that difficult place and listen to the questions and hear the fears within the questions. You will not be failing by not knowing the answers to some of the questions you will hear. Not knowing can be a place of strength and may even be reassuring for the child/parent. I once read a book that was called, ‘Failure, the gate way to hope’, which I found very reassuring in itself. We won’t always get it right, so don’t expect to. Don’t go looking for perfection. You will struggle with your own doubts as well as those of the child and family, but the struggle will be worth it.

“in touch with both our freedom and our destiny, enabling us to find our ‘selves’ and to find our way with others”

The next few sections focus on the needs of the child who is ill but they are just as applicable to you as


We need to be open to what children have to teach us. We need to be prepared to learn from them. The skill here, as in other aspects of children’s palliative care, is to be able to understand or ‘crack’ their code. We can start to do this, if we just sit with them, if we learn to watch, wait and wonder with them, if we take our lead from them and become responsive to their needs and not the needs we think they may have or our own needs.

a professional, to parents or to siblings. I would suggest that we all have spiritual needs to which we must attend. Our spirituality is something that cannot be turned on and off at will. It is a part of us and is always present. Your spirituality cannot be isolated from all that makes you who you are. All sick children will at some time think about what is going on, not only in their bodies but also in their inner worlds. On this journey they need you to sit with them, in effect to watch with them, wait with them and let them wonder. They need you to be their companion, their guide and their advocate. However, the most you can do is to prepare and hold that space where they can start to do the work they need to do for the next stage of their journey, where they can explore their inner-world and where the miraculous may happen. You now find yourself on a journey, a journey that you have had very little choice about making, and would perhaps have preferred not to have started. I have suggested that this is about a “journey” to the centre, to the heart of the matter, to our ‘deep centre’, where sometimes we meet our pain and have to name it. Children do come ready equipped for their spiritual journey, in so far as they have an openness and awareness, which in a way is unique to a child’s early years. As we get older this openness and awareness gets pushed to one side or pushed down.

definition Spirituality is what gives a person’s life meaning. It is about how people view the world they find themselves in, and

this may or may not include a God figure or a religious faith. Spirituality is about how we view the world and how we react within it. In talking about spirituality we need to bear in mind that we all come from different social and cultural contexts, that we each have a past and some of us have a future, be that long or short; and it is out of this setting that our spirituality will manifest itself. It is from this background or setting that the questions will flow. Therefore, you may well be the best person to offer this aspect of care, with help and support from others around you. I have found that children with a lifelimiting or theatening illness have a highly developed sense of their own spirituality, though they may not say or show it directly. It may well be deeper and more mature than other children of their age and development. However, they may not always have the words or means of expressing it. Therefore, you as a professional are very important, because you will be able to understand the child’s language and play far better than others.

practicalities If we are to understand children, their spirituality and their needs, we must first reflect on our own spirituality and be prepared to question our own

assumptions about spirituality and religion. How do we see spirituality in our own lives and the psychological influence it may have had on us coming from some distant place in our past? The current situation in which you find yourself will challenge your value systems and notions of spirituality and cause you to reflect deeply. This process of questioning often happens and you need to know that it is not unusual and you should not be wracked with guilt for questioning. In order to offer spiritual care we need to have tried to make sense of these questions for ourselves before we sit down with a child or a parent. That is not to say we share our own spirituality or our own religious beliefs. It is so we are not taken by surprise – the child will pick up on this. Spiritual care is about responding to the uniqueness of the child in front of you and accepting their range of doubts, beliefs and values just as they arise. It means responding to the spoken or unspoken statements from the very core of the child’s being as valid expressions of where they are and who they are. It means being their friend, companion and their advocate in their search for identity on their longest journey and in the particular situation in which they now find themselves. It is to respond to them without being prescriptive, judgmental or dogmatic and without preconditions, acknowledging that the child and other

Never underestimate a child’s understanding of what is going on. You do so at your peril. You may be surprised at how children have an unclouded, clear way of thinking and their take on abstract ideas is often quirky but relentlessly practical. This is the way in which they can help us with our struggle in trying to understand their suffering.

You may have discovered for yourself by now that you cannot fill the hole in a doughnut. As much as you try to fill it, the filling just keeps disappearing out of the back into some ‘black hole’. What you need to remember is that when you are with a child, the spaces or the gaps in the conversation do not need to be filled. This may be the centre of their journey and you just need to hold that space with the child and be present with them. Remember they have chosen you because they trust you and they respect you. Don’t spoil it by trying to fill the space or explain away the mystery. Suffering is not a question that demands an answer from us; it is not a problem that demands a solution; it is a mystery that demands your presence.


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hydrotherapy the benefits of hydrotherapy in children with palliative care needs Alice Dawson, Specialist Paediatric Physiotherapist. For millennia, water has been associated with well-being and healing. Since the earliest Greek civilisations, water has been used to cure a number of illnesses, from fever and constipation to mental illness. In many traditions, people sought out shrines associated with water to bathe at or drink from, believing in the water’s ability to soothe, to heal both physically and spiritually and to refresh. This belief continues today with water taking on miraculous qualities and being associated with many rituals of the great faiths of the world.

individually and as a family unit. It can alleviate many symptoms associated with pain, whether physical, spiritual or psychological. It can be quiet and thoughtful, relaxing and reflective. It can alleviate social isolation and bring families closer together, literally, allowing parents to physically hold their children, no matter how big they may have grown.

There is no doubt that hydrotherapy has many uses in palliative care. It is an effective treatment that creates a sense of well-being and healing for the child and all members of the family,

Of course, water can also just be fun. It can break down barriers, be noisy, exciting and give freedom to those who feel restricted either physically or emotionally. It is even the perfect

what is hydrotherapy?

weightlessness experience’ .

Within this context, hydrotherapy is ‘a therapy programme utilising the properties of water, designed by a suitably qualified physiotherapist specifically for an individual to improve function, carried out by appropriately trained personnel, ideally in a purposebuilt and suitably-heated hydrotherapy pool’.1,2

what are the benefits of hydrotherapy?

how does hydrotherapy work? Hydrotherapy takes advantage of the effects of immersion in water, and exercise. When putting a hydrotherapy programme together, an understanding of hydrostatics, or the behaviour of water at rest and hydrodynamics, the behaviour of water in motion is needed. The therapist will take into account the effects of buoyancy, relative density, centre of gravity, thermal conductivity, resistance and turbulence. The key differences compared to physiotherapy on land are ‘the heat and apparent



There are many reported benefits of hydrotherapy including relief of pain and muscle spasm; maintenance/ increase in joint range of movement; strengthening of weak muscles; increase in exercise tolerance; reduction of swelling; improved circulation; ease of movement; encouraging functional activities; maintenance and improvement of balance, co-ordination and posture and benefits of relaxation and wellbeing. Much of this research is done in adult populations; but the benefits may also be of value to children. The evidence base for these benefits is building, however much of the evidence is anecdotal currently. Hydrotherapy has been reported as the treatment of choice by physiotherapists, parents and children in preference to

environment for exercise, for those who intend it to be so and those who don’t realise it to be so. In the first of a series of articles about the use of hydrotherapy in children’s palliative care, the following article will expand on the benefits of hydrotherapy when used under the direction of a physiotherapist. Cath Thompson - Head of Care Services, Tyˆ Hafan

land-based physiotherapy alone. It has been recognised that there is a lack of empirical evidence to support hydrotherapy in children, particularly in palliative care. However, when reviewing the evidence, three areas of research were found with some relevance to children with palliative care needs cared for by Tyˆŷ Hafan. Evidence can be found for children with cerebral palsy, Retts Syndrome and autism. A 2011 systematic review by Blohm 4 looked at eight studies reporting benefits of hydrotherapy for children with cerebral palsy, including three randomised controlled trials (RCTs). The improvements that were reported in the studies reviewed include ‘greater walking efficiency (three studies); increases in lower-extremity muscle strength (two studies) and balance (two studies); better respiratory function (one study) and reduced spasticity in adolescents (one RCT). Improvements were also reported for range of motion with an

hydrotherapy has been reported as the treatment of choice by physiotherapists increase in passive range of motion of lower-extremity joints (one study), and improved active and passive range of motion (one RCT).’ Of great importance Blohm 4. found that ‘positive impacts on participation were reported in six studies (including one RCT) with improvements in performance, satisfaction, social functioning and self-perception’. Similar benefits were shown in two other systematic reviews. Jorgi5 reported a positive effect on physical fitness and social behaviour in children and adults with cerebral palsy through the use of aquatic therapy. The reviews have also demonstrated that hydrotherapy has a very low risk of

adverse events/harm to patients. Mortimer et al6 have completed a systematic review of literature relating to the effectiveness of hydrotherapy in the treatment of social and behavioural aspects of children with autistic spectrum disorders (ASDs). They concluded that ‘few studies have investigated the effect of hydrotherapy on the social interactions and behaviours of children with ASDs. While there is an increasing body of evidence for hydrotherapy for children with ASDs, this is constrained by small sample size, lack of comparator, crude sampling methods, and the lack

of standardised outcome measures’. Of note they found that ‘hydrotherapy shows potential as a treatment method for social interactions and behaviours in children with ASDs. Bumin et al7 discussed a case study of using hydrotherapy for a young girl with Retts syndrome. They found that after a prolonged course of hydrotherapy (eight weeks) her walking balance had improved, alongside her interaction with the environment. Her hyperactive behaviour and anxiety were found to reduce.


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t yˆ hafan care services professional development week summer 2014

Mandy Brimble, Editor, Lead Practitioner for Education and Research, Tyˆ Hafan At Tyˆ Hafan we are privileged to be allocated two weeks a year for Professional Development. This usually occurs during the third week of January and the end of June / beginning of July and takes place in our excellent educational facilities in the new Care Services Facility at the Sully site. During this time the hospice is closed to all but emergency or crisis admissions. This allows the majority of clinical staff to attend part or all of the week. In a 24 hour / 7 day a week care environment this is the only way that education can be delivered on a ‘block’ basis, to a large number of staff, in a timely fashion.

conclusion Although evidence is limited, it is recognised widely among professionals working with children with complex disabilities that the benefits of working with children in a pool can at times be unmatched. For children that struggle to participate with land-based therapy, hydrotherapy can be ideal as they

can progress quickly, activities are varied and it is possible to disguise that a physiotherapy programme is being conducted. Hydrotherapy can be carried out through simple play activities. Hydrotherapy can help improve compliance with following an exercise programme as activities that

aren’t possible to do on dry land can be performed in the water. Further research into hydrotherapy in children with life-limiting conditions would be beneficial, looking at the benefits for symptom control and the potential benefits of relaxation.


4. Blohm D (2012): ‘Effectiveness of aquatic interventions for children with cerebral palsy: systematic review of the current literature’. Phys Ther. 19(1):19-29.

1. Hydrotherapy Association Chartered Physiotherapists (2006): Guidance on Good Practice in Hydrotherapy

5. Jorgić B & Dimitrijević K et al. (2012): ‘Effects of aquatic programs in children and adolescents with cerebral palsy: systematic review’. Sports Science 5(2):49-56.

2. What are the benefits of hydrotherapy? Retrieved 09/07/14 from:

3. Epps H, Ginnelly L, Utley M, Southwood T, Gallivan S, Sculpher M and Woo P (2005) Is hydrotherapy cost-effective? A randomised controlled trial of combined hydrotherapy programmes compared with physiotherapy land techniques in children with juvenile idiopathic arthritis. Health Technology Assessment 2005; Vol. 9: No. 39


6. Mortimer R, Privopoulos M, Kumar S (2014) The effectiveness of hydrotherapy in the treatment of social and behavioural aspects of children with autism spectrum disorders: a systematic review. Journal of Multidisciplinary Healthcare. Vol 7: 93-104 7. Bumin G, Uyanik M, Yilmaz I, Kayihan H et al (2003) Hydrotherapy for Retts Syndrome. Journal of Rehabilitation Medicine 35 (1): 44-45.

Although this may appear a luxury at the expense of care delivery capacity, at Tyˆ Hafan we see it as an essential and worthwhile investment in our staff. The week is usually a mixture of taught sessions with breakout groups, workshops, discussion forums, case studies, information sharing, a formalised debate on a contentious issue and, of course, mandatory and statutory sessions such as Safeguarding Children and Young People and Statutory Recording and Documentation. There is always a mix of internal and external speakers as we are very conscious to be outward, as well as inward, looking in terms of best practice and educating staff. The week is open to all who work in the hospice building, although some sessions will be more relevant to some roles than others. The week commencing 30 June 2014 started with the usual welcome and introduction together with our ‘rules of engagement’ and was followed by a team working exercise. Other sessions on the first day were ‘Determining needs based care’ delivered by Head of Care Services, Cath Thompson and some excellent Level 1 Safeguarding C&YP Training delivered by Children in Wales.

Tuesday brought a range of discussion forums and an update from physiotherapist Sarah Clements on postural positioning. Workshops and clinical updates from external speakers followed on Wednesday together with an excellent session on supporting bereaved families by Hayley Mason, one of our Family Support Practitioners. For senior clinical and community staff there was Level 2 Safeguarding training all day on Thursday, again delivered by Children in Wales. Many staff found this very challenging and emotive, as you would expect, but it was described by some as the best training they had every received. All other staff engaged in workshop activities on Thursday; sensory activities, memory work, caring for a child with a jejunostomy to name but a few. The week ended with a statutory fire safety update, discussion around adverse events and feedback from discussion forums that had taken place throughout the week. As always the week was brought to a close by Cath Thompson who is skilled at assimilating the outcomes of the week and focusing us on the best way to move these forward into our practice. Ultimately, the aim of the week is to improve knowledge and skills thus enabling Care Services staff to provide evidence-based care for children and families which is of the highest possible standard. All sessions are individually evaluated to ensure that the quality of content and delivery is continuously improved. Evaluations ask the staff

member to identify the most important thing they have learnt from the session and how this will improve their practice. This facilitates a specific action point for each session for each staff member. These small alterations have the potential to add up to big changes. Information from the evaluations and overall feedback on the week is taken forward to the planning of the next professional development week so that we can attend to both group and individual learning needs and comments on the week overall are very positive. As the main organiser and facilitator of this event I am indebted to my colleagues both internally and externally who help to make it such a success. The nicest thing to hear from a staff member is “That was the best PD week we have ever had”, but this then poses a challenge – to make the next one even better.

“the best PD week we have ever had”


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management of respiratory distress in a child with herlitz junctional epidermolysis bullosa receiving palliative care Marlyn Mallari, Nurse, Tyˆ Hafan In paediatric palliative care we are often faced with rare conditions. Herlitz Junctional Epidermolysis Bullosa (H-JEB) is one such hereditary condition1. This article aims to give an overview of the condition focusing primarily on the effects of the disease on the respiratory system. what is Epidermolysis Bullosa (EB)?

completely absent.

EB is described as ‘the worst disease you’ve never heard of’2. Craciun et al3 state that patients with EB live their life filled with pain and distress. EB is an umbrella of genetically determined diseases characterised by the formation of blisters on the skin and mucous membranes as a result of defective structural skin proteins. This complex and hereditary disease is divided into four different types. These are classified based on the manner of inheritance, the location of blistering and epidemiological studies, see Tables 1 and 2. The major classifications are: EB simplex, junctional EB, dystrophic EB and Kindler Syndrome 4. These classifications are further subdivided into major and minor subtypes – see Table 3.5

This causes blister formation at the lamina lucida or the central basal membrane of the skin. The initial symptom is the appearance of blisters which are present at or soon after birth. These patients suffer from lifethreatening respiratory crisis because the underlying disease affects the respiratory mucosa.

herlitz Junctional Epidermolysis Bullosa (H-JEB) The course of the disorder is examined briefly within the parameters of its clinical signs and symptoms. It is necessary to focus on the physiology of the condition as it provides some indication of the complex nature of the physical deterioration of the body. H-JEB is the most severe form of EB. The underlying defects lie in the hemidesmosomes that serve to connect the basal surface of the cell to the basement membrane; these tend to be drastically reduced and sometimes


Non-healing crusted erosions with significant granulated tissue appear around the nose, mouth, ears, fingertips and toes. Injury to the airways can occur spontaneously or can be provoked by crying and coughing. Respiratory tract infection and gastroesophageal reflux, which are common complications of H-JEB can also encourage and aggravate lesions 4. Laryngeal blistering is also a common feature of H-JEB. Repeated blistering and subsequent scarring leads to a severely damaged airway, thereby causing inadequate ventilation and oxygenation6.. EB has been recognised as a distinct entity for over centuries, yet despite considerable interest and research, a definitive cure for the disease remains uncertain5. Yuen1 in his most recent study states that it is largely accepted that H-JEB is untreatable. In addition, Sarkar et al5 also emphasise that as far as systemic treatment is concerned, nothing is proven to be effective in controlling blisters. Therefore, the focus

of intervention is the relief of suffering7. Respiratory difficulty is one of the most common symptoms reported in children nearing the end of life8. Much has been written in the care of the lesions of the skin but there is a dearth of information that focuses specifically in the symptom management of the respiratory distress. management of dyspnoea Dyspnoea or breathlessness is a very distressing symptom that is commonly experienced in palliative care9. Difficulty in breathing associated with progressive respiratory compromise is a crucial concern in clinical practice10.

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studies in the palliation of this symptom11 and it has been argued that it is essential to treat symptoms even if it shortens life16. Palliative management of young children includes managing symptoms in a way that is non-traumatic and is focused on maintaining comfort10. In their most recent study Harlos et al10 confirmed the effectiveness of the use of intranasal fentanyl in the palliative management of newborns and infants. This method proved to be effective, non-distressing with no adverse events noted. This study was a retrospective chart review of children age six months and younger who were referred to paediatric palliative care services in a tertiary hospital. In contrast, Simon et al17 conducted a systematic review of the use of fentanyl to relieve breathlessness and found that there is no evidence to support this claim due to a lack of adequately powered and controlled trials. Anxiolytics are useful in assisting with the anxiety components of breathlessness and along with the management of depression, may improve dyspnoea experience. In addition, Benzodiazepines have been broadly studied and proven to be effective as a single agent or paired with opioids in controlling dyspnoea8. However, pharmacological interventions alone are often not effective in relieving breathlessness. Therefore, care providers need to be updated on the best practice related to non-pharmacological interventions in managing the symptoms effectively in an effort to improve a

patient’s quality of life18. The role of oxygen in the palliation of dyspnoea is unclear. This is established by the study carried out by Currow et al although oxygen remains frequently prescribed for patients with difficulty breathing in palliative care regardless of the patients’ ability to experience or report distress. There is conflicting evidence particularly when breathlessness is not accompanied by hypoxia because oxygen is not without adverse effects and may prolong life without providing comfort if the patient is not experiencing distress13. Another non–pharmacological intervention which has proved effective in minimising breathlessness is a handheld fan directed to the face19. A further intervention for the treatment of respiratory distress associated with chronic laryngotrachial lesions in children is the placement of a tracheostomy4. A tracheostomy is a surgical incision of the neck and placement of tube directly to the trachea to facilitate airway management 20. This may be considered in EB either as an emergency or by choice of the family. However, it can be associated with a number of complications. Each manipulation may readily induce lesions and ulceration in the mucosa and repeated bullae formations can result in stenosis. Wearing tracheal cannula can cause additional skin damage and unnecessary stripping of the already vulnerable skin. In addition, suctioning of secretions from the tracheal cannula will

further damage the tracheal mucosa21. This poses an ethical dilemma for the team managing the patient. Therefore it is disputable whether to perform a tracheostomy6. as it is associated with a number of complications and will bring no improvement In the child’s prognosis21. The advances in technology have accustomed society to believe not only that infants have a chance of an existence, but they will survive the remarkable medical challenges they are faced with22. However, in H-JEB this is not physiologically possible without complications21. conclusion and recommendations Respiratory distress is a symptom of H-JEB resulting from tracheolaryngeal obstruction caused by blisters which are the main feature of this condition. The evidence presented in this article was gathered from many sources, the majority being found within the area of breathlessness at end-of-life in adult and childhood cancers and lung diseases. Literature which explores EB is focused mainly on the epidemiology of the disease with very little information on symptom management particularly on respiratory involvement in a child with H-JEB. In addition, gaps in professional knowledge about the treatment of children with respiratory distress exist because of the inability of this population to self report distress13. The writer hopes that this article will raise the profile of this aspect of paediatric palliative care.

Despite comprehensive studies and research on managing dyspnoea and breathlessness, the incidence and the complexity of the symptom remains a burden even with a high level of evidence of effective intervention8. The use of opioids in palliative care has been advocated for decades in the treatment of dyspnoea11. However, the literature reveals that many clinicians do not prescribe opiods to treat this symptom12, 13, 14 Currow et al15 highlight that failure to treat refractory breathlessness with opiods is an unacceptable level of care ethically and arguably, a violation of human rights. The safe and effective use of opiods in the management of respiratory distress is supported by increasing evidence-based


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table 1 level of blister formation in each major EB type Major EB types

Level of blister formation

EB simplex


Junctional EB

Intra-lamina lucida

Dystrophic EB

Sub-lamina densa

Kindler Syndrome

Multiple levels ( intra-lamina lucida and sub-lamina densa)


Extracted from Fine23. table 2 inheritance and clinical features of the major subtype of epidermolysis bullosa Type


Clinical Features

EB simplex

Autosomal dominant, rarely recessive

Localized blisters or group vesicles, limited mucosal involvement, palm plantar hyperkeratosis, nail dystrophy, normal teeth and hair

Junctional EB

Autosomal recessive

Widespread blistering, scarring, significant granulation tissue, severe mucosal involvement, dental pitting, alopecia, nail dystrophy

Dystrophic EB

Both autosomal dominant and recessive

Hemorrhagic blisters, scarring, milia, pseudosyndactyly, severe mucosal involvement, physical and sexual retardation, significant morbidity and mortality.

Extracted from Sarkar et al5. table 3 classification of inherited epidermolysis bullosa Major EB type

Major EB subtype

Minor EB subtype

Epidermolysis bullosa simplex

Suprabasal EBS Basal EBS

Lethal acantholytic EB Plakophillin deficiency EB superficialis EBS- localised EBS- Dowling Meara EBS- other generalised EBS with mottled pigmentation EBS with muscular dystrophy EBS with pyloric atresia EBS autosomal recessive EBS ogna EBS migratory circinate

Junctional Epidermolysis Bullosa

JEB – Herlitz

JEB, other

JEB non herlitz, generaliSed JEB non herlitz localiSed JEB pyloric atresia JEB inversa JEB late onset LOC syndrome

Dystrophic Epidermolysis Bullosa

Dominant DEB

Recessive DEB

DDEB generalised DDEB acral DDEB pretibial DDEB pruriginosa DDEB nails only DDEB bullous dermolysis of newborn RDEB severe generalised RDEB generalised other RDEB inversa RDEB pretibial RDEB pruriginosa RDEB centripetalis RDEB bullous dermolysis of newborn

1. Yuen WY (2012). Junctional Epidermolysis Bullosa. Available online at media_data/4_DEBRA_International/Documents/ Dissertation_Junctional_EB_-_Yuen_WY.pdf (Accessed 24 July 2014) 2. The Dystrophic Epidermolysis Bullosa Research Association of America (DebRA), (Accessed 23 July 2014). 3. Craciun A., Micle I., Simedria I., et al (2010). Sedation and pain management in epidermolysis bullosa. Presentation of 4 cases. Annals of the Romainian Society for Cell Biology. 15, 1, 185-186. 4. Murat-Susic S., Husar K., Skerlev M et al ( 2011). Inherited Epidermolysis Bullosa – the spectrum of complications. Acta Dermatovenerologica Croatica. 19, 4, 255-263. 5. Sarkar R., Bansal S. and Kumar GV (2011). Epidermolysis Bullosa: where do we stand? Indian Journal of Dermatology, Venereology and Leprology. 77, 4, 431-438. 6. Denyer J (2009). Management of the infant with epidermolysis bullosa. Infant. 5, 6, 168 -171. 7. Yan E., Paris J., Ahluwalia J et al. 2007. Treatment decision – making for patients with herlitz subtype junctional epidermolysis bullosa. Journal of Perinatology. 27, 307-311. 8. Kamal A.,Maguire J., Wheeler J et al (2012). Dyspnea review for the palliative care professional: Treatment goals and therapeutic options. Journal of Palliative Medicine. 5, 1, 106114. 9. Breaden K (2011). Recent advances in the management of breathlessness. Indian Journal of Palliative Care. 17, 4, 2932. 10. H arlos M., Strenekes S., Lambert D., et al (2013). Intranasal fentanyl in the palliative care of newborn and infants. Journal of Pain and Symptom Management . 46, 2, 265-274. 11. Hellenbeck J (2012). Pathophysiology of dyspnea explained: Why might opioids relieve dyspnea and not hasten death? Journal of Palliative Medicine 15, 8, 848-853. 12. Chou R., Ballantyne J., Fanciullo G., et al (2009). Research gaps on use of opioids from chronic non cancer pain: findings from a review of the evidence for an American pain society and American academy of pain medicine clinical practice guideline. The Journal of Pain. 10, 2, 147-159.

13. Campbell M., Templin T. and Walch J (2009). A respiratory distress observation scale for patients unable to self report dyspnea. Journal of Palliative Medicine. 13,3. 285-289. 14. Zerzan J., Lee M and Haverhals L (2011). Exploring physician decisions about end of life opiate prescribing: a qualitative study. Journal of Palliative Medicine 14, 5, 567-572. 15. Currow D.,Abernethy A., Ko D. (2013) . The active identification and management of chronic and refractory breathlessness is a human right. Thorax. 69,4, 393-394 16. Lankan P., Terry P., Delisser H., et al (2008). An official American thoracic society clinical policy statement: palliative care for patients with respiratory disease and critical illnesses. American Journal of Respiratory and Critical Care Medicine .177, 912-927. 17. Simon, S., Koskeroglu, P., Gaertner, J., et al (2013) Fentanyl for the relief of refractory breathlessness: a systematic review. Journal of Pain and Symptom Management. 46, 6, 874-886. 18. Lang D. 2012. Nonpharmacologic interventions for breathlessness in advanced stages of malignant and nonmalignant diseases. Clinical journal of oncology nursing. 16, 3, 320. 19. Galbraith S., Fagan P., Perkins P et al., 2010. Does the use of a hand-held fan improve chronic dyspnea? A randomized, controlled crossover trial. Journal of pain and symptom management. 39, 5, 831-838. 20.Finucane B., Tsui B and Santora A (2011). Principles of airway management 4th ed. New York. Springer. 21. Babic I., Karaman-Ilic M., Pustisek N., et al (2010) Respiratory tract involvement in a child with epidermolysis bullosa simplex with plectin deficiency: a case report. International Journal of Pediatric Otorhinolaryngology. 79, 3, 302-305. 22. Cooper H., Cuthbertson L .and Flemming S. (2013). Neonatal palliative care nursing: working with infants on the cusp of life – a thematic review. Neonatal, Paediatric and Child Health Nursing. 16, 1, 2-7. 23. Fine, JD (2010) Inherited epidermolysis bullosa: past, present and future. Annals of the New York Academy of Sciences. 1194, p213-222

Kindler syndrome EB: Epidermolysis bullosa, EBS: Epidermolysis bullosa simplex, JEB: Junctional epidermolysis bullosa, DEB: Dystrophic epidermolysis bullosa, DDEB: Dominant dystrophic epidermolysis bullosa, RDEB: Recessive dystrophic epidermolysis bullosa. Extracted from Sarkar et al5.



sharing best practice in paediatric palliative care. volume 1 issue 2

T: 029 2053 2245 E:

together for short lives conference

courses, study days and conferences

Tracy Jones, Lead Practitioner Community Palliative Care Services Tyˆ Hafan

courses Cardiff University

The Together for Short Lives (TfSL) conference earlier this year was a useful opportunity for professionals from across the paediatric palliative care profession to think about ‘New Perspectives for Children’s Palliative Care’. The delivery of children’s palliative care is facing many dilemmas as it tackles the challenges faced by ever-increasing demands on service provision and the increasing complexity of the population it aims to support. Conferences such as this provide opportunities for professionals to network with colleagues and share ideas whilst trying to identify better, smarter methods of service delivery across the sector. It was a packed conference programme with some high profile speakers and thought provoking workshops - even the fire alarm and ensuing evacuation provided an opportunity for more networking and idea sharing! The key themes for the day included the ‘demography of children’s palliative care’; the ’evidence base for delivery of children’s palliative care’; the ‘ethics of health and social care rationing’; ‘community engagement and volunteering’; ‘impact measuring in children’s palliative care’ and ‘partnership/collaborative working’. The workshop sessions allowed for more focused thinking around some of these topics and provided opportunities for practitioners to design their own responses to the issues they face in practice. Professor Allan Kellehear gave an engaging and passionate presentation about ‘embracing community participation’ during which he encouraged delegates to think about our communities as an untapped resource in terms of end-of-life and bereavement care. He argued that palliative care needs to be considered, alongside other


health care directives, within the remit of public health. This session provided much to think about, with key messages resonating through to the workshop session on ‘the future of volunteering in children’s palliative care’. Data was presented on the ‘changing population in paediatric palliative care’ during which Dr Lorna Fraser identified the ever growing numbers of children and young people defined as being eligible for paediatric palliative care services. She also identified that current models of service delivery, many of which are based around oncology, are no longer fit for purpose in this ever shifting population. Dr Myra Bluebond-Langer followed this up with a session on why research and data collection is so important and how we, as professionals involved in paediatric palliative care, need to develop an ‘evidence base to guide service development’. She encouraged us to think about having a ‘culture of enquiry’ within our workplaces and posed the question ‘what needs to change?’ to ensure that research is clinically based. She talked about four different approaches to consider in terms of encouraging clinically based research and evidence gathering and identified four key points for our continued consideration: • identify the services we are providing/ hoping to provide • d etermine if the services are meeting the needs of the potential service users

Pain Management (MSc/PGDip/PGCert) via DistanceLearning/ E-Learning. More information available at: University of South Wales Symptom Management for Paediatric Palliative Care 10 credit e-learning MSc Module, managed by Sue Dunlop and Dr Richard Hain. Email for more information: • ask ‘is what we are doing making a difference?’ The morning finished with a lively and impassioned panel debate on the ‘ethics of health and social care rationing’ during which the commissioning inequalities between adult and children’s palliative care services were highlighted. Other topics covered included the withdrawal of services and equipment in the time of financial constraints and how these scarce resources can most equitably be allocated. It was a thought-provoking day with plenty of opportunity for sharing ideas with colleagues from across the sector. Practitioners from all sectors have clearly identified the shared challenges we all face in striving to provide services that are ‘fit for purpose’. It was valuable to have time to collaboratively problem solve and come away feeling invigorated and ready for the changes ahead of us.

conferences Together for short lives conference

International children’s palliative care network

Living Matters for Dying Children, 14 and 15 April 2015, Conference Aston Meeting Suites, Aston University, Birmingham.

Cardiff University – Medicine and Compassion: Tool for the Task - Or Dangerous Distraction? 7th Cardiff International Conference 2015 Paediatric Palliative Care 8 – 10 July 2015 Contact: paaedpalcare-bounces@act. for more information.

More information available at www.togetherforshortlives. dying_children or phone 01179897831, email: conference@

publications of interest Grant, P et al (2014) The significance of end of life dreams and visions. Nursing Times, 100(28), p22-24. Horridge, K A and Wolff, T (2014) Palliative care for disabled children and young people. Paediatrics and Child Health, 24(8), p343-350. Jennings, V and Nicholl, H (2014) Bereavement support used by mothers in Ireland following the death of their child from a life-limiting condition. International Journal of Palliative Nursing.20(4), p 173-178. Lane, C and Mason, J (2014) Meeting the needs of siblings and children with life-limiting illness. Nursing Children and Young People, 26(3), p16-20. Melin-Johanson, C et al (2014) When a child dies: Parents experiences of palliative care – An integrative literature review. Journal of Pediatric Nursing (In press).

Rodriquez, A and King, N (2014) Sharing the care: the keyworking experiences of professionals and parents of life-limited children. International Journal of Palliative Nursing, 20(4), p167171 Smith, H (2014) Giving hope to families in palliative care and implications for practice. Nursing Children and Young People, 26(5), p 21-25. Valdez-Martinez, E et al (2014) When to stop? Decision making when children’s cancer treatment is no longer curative: a mixed method systematic review. BMC Pediatrics 14:124, Whiting, M (2014) Children with disability and complex health needs: the impact on family life. Nursing Children and Young People, 26(3), p26-30. Whiting, M (2014) Support requirements of parents caring for a child with disability and complex health needs. Nursing Children and Young People, 26(4), p24-27.

• e xplore the gaps between service delivery and need


would you like to submit an article? The primary function of Insight is to share best practice in paediatric palliative care. Although Tyˆ Hafan publishes the journal, our aim is to provide a collaborative platform for all those working in this field. Therefore, your contributions are both welcomed and valued. All articles should be submitted electronically to Mandy Brimble, Editor and Lead Practitioner for Education and Research Submissions will be scrutinised by the Editorial Board who may request changes or for the article to be rewritten. The Editorial Team may reject an article, or hold the article on file for a subsequent edition. Articles can be submitted by a single author or co-written. Submissions should be written in Arial 12 and submitted as a Word document. Referencing style is the footnote numbered system. Articles that include details of children and families must be anonymised.

intended to educate and should include condition details and other relevant information. The article may look at a specific palliative need or a specific condition with multiple palliative needs. d ebate (approximately 500-700 words) This article is intended to create debate amongst fellow professionals on a palliative care topic which divides and creates multiple opinions. The article may be contentious and a current ‘hot potato’ as it were. The article will be heavily based on available evidence to support the many arguments, and will actively seek the views of the target audience.

Submitted articles may fall under one of the following categories:

r eflection (approximately 300-700 words)

quality improvement articles (approximately 300-500 words)

This article is intended to highlight an analysis of a particular topic of relevance to children’s palliative care. It may be current or historical.

In this type of article the author wishes to highlight a new or current service or intervention which reflects the principles of children’s palliative care. The article must give a rationale for the service or intervention based on evidence from policy, research or a specialist body of knowledge. case studies (approximately 500-1,000 words) This article is intended to highlight the specific needs of the child and family and the support and interventions required to meet those specific needs. The article is

15 years of being there

regular features Every edition will contain newsworthy or relevant items such as, details of courses and study days, updates of policy and consultations, news from Managed Clinical Network and South Wales Paediatric Palliative Care Forum Information noticeboard

The word count allowances are an approximate guide, longer or shorter pieces will be considered.

If you have any queries about making a submission please contact Mandy Brimble on 029 2053 2245 or email Tyˆ Hafan - Head Office Hayes Road, Sully, CF64 5XX Tel: 029 2053 2245 Fax: 029 2053 2821 Charity no. 1047912

insight - tŷ hafan volume 1 issue 2  

Sharing best practice 15 years of being there in paediatric palliative care

insight - tŷ hafan volume 1 issue 2  

Sharing best practice 15 years of being there in paediatric palliative care