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1 HUNTER SYNDROME CAREGIVER EMOTIONAL SUPPORT

An introduction for nurses

When you’re working with caregivers looking after a child with a rare, long-term condition like Hunter syndrome, you’re a vital part of their support network. You can make a big difference in how caregivers cope.1 Nurses, in their professional capacity, already do a lot to provide care and support for families.

The training and the materials available here, can give you a more structured, in-depth approach to working with caregivers. The training will help you feel more confident that you have a range of skills and techniques to give families the best possible support. And you may find the training will help you manage stressful situations in your own life, too.

You’ll train in proven emotional support techniques that can help you teach caregivers to:

• Create helpful routines • Develop more flexible ways of thinking and solving problems • Cope with stress, anxiety and other painful feelings • Be kind to themselves in difficult situations • Lead a fulfilling life

NURSES GUIDE TO SUPPORTING CAREGIVERS OF PATIENTS LIVING WITH HUNTER SYNDROME (MPS II)

Caregiver Support Modules

This item is developed and funded by Takeda.

This support is exclusively for caregivers of patients with Hunter Syndrome (MPS II).

SectionCopyright Name 2019 | TakedaHunter Pharmaceutical Syndrome Company Caregiver Limited. Support All rights reserved. Takeda and the Takeda Logo are trademarks of Takeda Pharmaceutical Company Limited Page A of 30

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This resource is intended only to provide knowledge of Hunter syndrome health topics. This resource is not a substitute for medical advice, and Copyright 2020 Takeda Pharmaceutical Company should not be used in place of advice from a healthcare professional. Please contact a healthcare professional for advice. This resource is intended Limited. All rights reserved. Takeda and the for an international audience outside of the USA and has been produced by Takeda. This resource has been developed in accordance with industry Takeda Logo are registered trademarks of Takeda and legal standards to provide information for the general public about Hunter syndrome health topics. Takeda makes every reasonable effort to Pharmaceutical Company Limited. include accurate and current information. However, the information provided in this resource is not exhaustive. C-ANPROM/INT//8184 August 2020 2 LEARNING, UNDERSTANDING AND EXPLORING YOUR ROLE IN PROVIDING EMOTIONAL SUPPORT

As you know, Hunter syndrome can result in a range of physical symptoms impacting on many different parts of a child’s body. Their bones and joints, heart, lungs and abdomen are among the parts of the body that can be affected.

And 7 out of 10 people with Hunter syndrome have the neuronopathic form of the condition. This means they also have cognitive symptoms, which can include developmental delay, behavioural problems such as aggression, and neurological issues including seizures.2,3

As a result, caregivers are likely to be living with a range of stressors and difficult feelings. They may experience many challenging emotions, including:

• Stress and anxiety4 • Depression4 • Guilt5 • Financial worries, especially if their work has been affected4 • Relationship challenges4 • A sense of hopelessness4 • Feelings of powerlessness4 • Grief – not only over their child’s reduced life expectancy, but also for the loss of a future they thought their child and family would have4

It won’t surprise you to learn that research shows caregivers of children with Hunter syndrome have even lower quality of life than caregivers of children with other health issues.6 3 LEARNING TO BE AN ACTIVE LISTENER

Whenever you’re working with caregivers, you can practise a technique called active listening.7 Active listening is to give full undivided attention to the other person and to listen with your ears, eyes and heart to both verbal and non-verbal messages.

It’s a simple but powerful way to ensure they feel fully heard. It should make it easier for them to open up to you. And you can use it in lots of situations in your own personal life, both at work and in your relationships. Here are tips to get you started – becoming a skilled active listener takes time and practice.

• Try to create a non-distractive environment7 • Maintain attentive body language and relaxed, natural eye contact7 • Explore issues by using timely and considered questions7 • Allow silence. Allowing for moments of silence in a conversation often encourages people to open up. You could make encouraging sounds, such as ‘Ah’, ‘Hmmm’ or ‘Right’ to show you’re still listening7 • Repeat what the caregiver has said in your own words, to check you’ve understood7 • Try to avoid common “roadblocks” such as judging, suggesting solutions and avoiding concerns raised7 • If you feel the issues raised need to be addressed at a different time or by a different professional, acknowledge the concerns and arrange for an appropriate follow up7 4 CARING FOR YOU

We’ve looked at the importance of emotional support for caregivers. But it’s also vital you look after yourself. All nurses have a demanding job. The work can be physically hard. Mentally, you need to be alert and respond to the needs of patients. And nursing can take an emotional toll, too. There can be lots of joy and many rewards in caring for others. But you may also be in an environment in which people experience pain and sadness. Being around that, and empathising with those feelings, can be difficult.8

As a specialist nurse supporting families of children with Hunter syndrome, you may have to deal with a lot of especially demanding situations. And your wellbeing matters. It’s important to take steps to manage stress for a number of reasons:

• Emotional distress can impact your quality of life • It may affect your physical health, even leading to serious illness in the longer term • You may miss work • It can stop you helping the families effectively8

As you can see, looking after your own emotions helps you stay healthy. It also means you can do your job well and support the caregivers and children you work with. Remember: stress isn’t a sign you’re not coping. It just means you’re having a very human response to difficult situations. Symptoms of stress are simply signs you may need to put a few steps in place to look after your wellbeing.8

The following three sections describe steps you can take to look after yourself: steps to manage stress, build resilience and keep professional boundaries. 5 CONTINUING TO CARE FOR YOU

The following three sections describe steps you can take to look after yourself: steps to manage stress, build resilience and keep professional boundaries.

1. Manage Stress You can’t always control what’s happening in your work or your wider life. But just as with the caregivers you support, taking simple steps to manage stress can make a big difference.

• Make time for leisure activities. It’s important to see friends and family and spend time on hobbies. It helps to give you a work-life balance, which can boost your energy and your mood8 • Look after yourself physically. Try to eat a healthy diet and take regular exercise.8 It may be helpful to prepare meals in advance9 • Plan regular holidays. Even if you can’t go away, make sure you take time off at home8 • Try to prepare mentally before work. A pause can be particularly helpful between meeting with the different families and children you look after. Write down any worries from your previous session and decide to put those to one side for now. Then take six deep breaths and try to let go of any ideas about how your next appointment will be. This can help you go into it fresh9 • And when you’ve finished for the day, try to leave work behind. First, you could think about three things that were difficult, and what you could learn from them. Do you need any extra support, for example? Then, think about three things that went well. Acknowledge how well you supported families in those moments. It may be helpful to finish your working day with a particular symbolic action – like changing your shoes. Once you’ve stopped work, focus your mind on the evening ahead9

6 CONTINUING TO CARE FOR YOU

2. Build your Resilience Resilience is your ability to cope in difficult circumstances, and to recover from setbacks.10 It’s particularly important for you as a specialist nurse because you may experience challenging situations every day. Developing personal resilience can help you cope.10 It can also protect you from becoming overwhelmed in the first place.11 Fortunately, resilience is a skill you can learn.10

The stress management steps above can help. And here are some other more specific ways you can build your ability to cope with stress in your job:

• Try to develop a positive attitude. It can be helpful to find humour in situations where you can, and choose to think positively10 • Build connections with colleagues. Consider identifying a colleague who could be your mentor, giving guidance and sharing their experience10 • Talk to someone. Your work brings you close to a lot of suffering and it’s understandable this may affect you deeply. Find someone you trust to speak to, whether it’s your manager, a friend or family member, or a professional counsellor12 • Work on your confidence. Knowing you’re doing your job well, and trusting you can cope, can help you feel stronger12 7 CONTINUING TO CARE FOR YOU

3. Keeping Boundaries You probably have a good understanding of professional boundaries and why they’re important. You know they keep you and your patients safe, and help you do your job effectively. But when you’re looking after children with a serious condition and supporting their families, it can sometimes be more challenging to maintain boundaries. Understandably, you’re likely to feel close to families and may develop a bond with them. So it can be important to pay special attention to boundaries in these situations.13

To make sure you’re not crossing boundaries, it may be helpful to ask yourself these questions:

• Am I making sure I don’t share personal information or beliefs?14 • Am I staying objective?14 • Am I putting patients’ needs first at all times, and making sure I’m not actually trying to get my own needs met?14 • Am I treating all patients in a similar way, and not giving extra time or attention to certain families?13 • Am I refusing all but small token gifts?14

To stay within boundaries, it can be helpful to:

• Practise self-awareness and reflect on your behaviour THESE THREE STEPS • Have debriefing sessions with peers - MANAGING STRESS, • Go to clinical supervision sessions BUILDING RESILIENCE AND • Speak to your line manager if you have any concerns13 KEEPING PROFESSIONAL BOUNDARIES - SHOULD ALL SUPPORT YOU IN YOUR VERY DEMANDING ROLE. REMEMBER: YOU DESERVE CARE AND ATTENTION TOO. 8 LEARNING ABOUT ACCEPTANCE AND COMMITMENT THERAPY (ACT)

As a specialist nurse, you’re already supporting families. This training builds on what you’re doing now by giving you a structured handbook and lots of simple, practical tools you can use with caregivers. The handbook and caregiver activities use simple techniques from Acceptance and Commitment Therapy (ACT).

ACT has been chosen for the emotional support handbook and activities because it’s for helping people to manage challenging situations. The caregivers you work with probably can’t change very much about their situation. But ACT can help them think and feel differently about it. It’s proven to reduce stress and depressive symptoms in caregivers of children with long-term, serious illnesses.15,16,17

The ACT based activities work by helping people: • Accept difficult thoughts, emotions and sensations. When people don’t have the tools to manage these, it’s natural that they may try to push them away. But that can cause more stress, anxiety and depression. ACT offers a way of learning to be with painful feelings, so they become less overwhelming18,19 • Understand the things that are important to them. This helps caregivers live a more meaningful life, despite their challenges18 • Practise self-compassion. For caregivers, learning to talk to themselves kindly, as they would talk to a friend, can help ease distress and improve wellbeing20 9 LEARNING ABOUT EMOTIONAL TRIGGERS

It's important to be aware caregivers' need for support can change over time. Caregivers may experience different emotions at different points of the journey with Hunter syndrome. And it can be helpful for you to be aware of some of these trigger points. For example, when their child is first diagnosed they may be focused on making sure they have the right treatment. Further down the line, they may be adapting to a possible decline in their child’s condition.

Certain situations or times may trigger painful emotions. These may come in cycles at regular points. For example, the anniversary of a child’s diagnosis may trigger difficult feelings for caregivers.21 Key milestone times can be another trigger. It can be hard for a caregiver to see others their child’s age developing in ways their child is not.22

But effective emotional support delivered by a specialist nurse can make all the difference. With the modules we have developed, you’ll be teaching caregivers skills they can practise themselves. They can make these practices a part of daily family life. The modules can empower caregivers to manage difficult emotions, whenever they come up.15,23 10 NURSE HANDBOOK AND CAREGIVER ACTIVITIES COVERED IN THE TRAINING

NURSES GUIDE TO SUPPORTING CAREGIVERS OF PATIENTS LIVING WITH HUNTER SYNDROME (MPS II)

Caregiver Support Modules

This item is developed and funded by Takeda.

This support is exclusively for caregivers of patients with Hunter Syndrome (MPS II).

There are four different modules that provide structured support activities to use with caregivers:

1. Understanding Stress helps caregivers identify the things that cause the most stress. It includes a problem-solving technique to address the situations that can be changed or improved. For situations a caregiver can’t change, the module introduces mindfulness as a helpful tool.15,17

2. Mindfulness goes into more detail about this strategy. This technique can help stop people worrying about the past or the future by helping them to focus on the present moment and accept whatever is happening.15,20 The module offers some different techniques to develop mindfulness. They’re all very simple, with some practice.

3. Being Kind To Yourself is about self-compassion. It helps caregivers offer themselves empathy and understanding. Self-compassion has been shown to increase positive emotions.17,24

4. Meaningful Living helps caregivers identify the things that are important to them. These are known as values. Understanding and living by your values can help life feel more rewarding and satisfying.15,17,18

The modules have been designed to be simple to work through. You won’t spend a lot of time explaining complex ideas to caregivers. Instead, the modules are practical, based around activities caregivers can try. They can also use many of them with their families. In this way, the emotional support modules empower the whole family to manage difficult feelings. 12 ACTIVITIES CAREGIVERS CAN DOWNLOAD THEMSELVES FROM THE WEBSITE

We’ve also designed a few activities caregivers can use by themselves, with their families. These are based on:

• Mindfulness • Self-compassion • Problem Solving

The activities are easy and practical. They are similar to the activities included in the nurse handbook. However they have been adapted so they are appropriate for caregivers to use by themselves without the support of a nurse. Some are recorded, so caregivers can relax and listen to them, and others are printouts. If you are interested to read them, you can access them HERE.

1 MINDFULNESS FOR DAILY CHORES

We all have to do tasks we do not enjoy. Chores are a good chance to practice Try this activity mindfulness. It can help to focus your attention when difficult thoughts and over the next feelings might distract you from the task at hand. week and see how many Pick a household chore you can use mindfulness with. It should be something simple different chores and repetitive you don't have to concentrate too hard on. Washing1 up, weeding the you can practice garden, sweeping and ironing would all work. Here, let's take ironing as an example. it with. 2 PROBLEM SOLVINGFocus on… Sights KEEPING TRACK OF KINDNESS Notice what you can see as you iron. For example: ● What colour is the thing you are ironing? ● What size is it? When you’re looking after a child with Hunter syndrome, life can probably feel challenging at times. Notes for next time: How I felt afterwards: You can’t always make problems go away. But sometimes● Notice you the can. creases Or you smoothing can improve away a problem, as you iron.so it How I felt before: What I did: becomes easier to live with. Addressing a problem in a step-by-step way can help you feel less stressed 1 Warm. Cared for. Okay The activity below supports youSounds to do this. Down and angry about and more in control. Example: Ran myself a about the emotional this situation Focus on the sounds you can hear. For example: bath before bed and burden on me at the This technique gives you steps to help you work through a situation and come up with a plan to manage it. ● Can you hear a hiss of steam as you iron? calmed my thoughts. moment. ● The sound of the iron moving over the material? It involves: Now, notice what you can feel while you do this chore. For example: ● Understanding what the problem is ● The movement of your arm as you move the iron? ● Thinking of solutions ● Your feet planted firmly on the ground? ● Choosing a solution ● The grip of your hand on the iron handle? ● Putting it into action Smells Choose a difficult situation you think can be changed and work through the steps shown. If you feel stuck, Focus on anything you can smell as you iron. For example: it can help to talk through the steps with someone else. They might help you come up with ideas you haven’t thought of. You could go through this activity● with Are therea nurse, any a smells family from member, the material? or a friend. ● What are the smells like? The difficult situation: You may find you keep getting distracted by difficult thoughts, feelings and urges. If this happens, take a deep Example: I have too many tasks to do in the house.breath in and out and bring your attention1 back to the task at hand. Notice any sights, sounds or sensations that are happening in the present moment.

References 1 Harris, R. (2007). Acceptance and Commitment Therapy (ACT) Introductory Workshop Handout. Psych Med.

to learn more How it is affecting you right now: Visit Huntersyndrome.info Copyright 2020 Takeda Pharmaceutical Company Limited. All rights reserved. Takeda and the Takeda Logo are registered trademarks of Takeda Example: I feel that I am not good enough. This resource is intended only to provide knowledge of Hunter syndrome health topics. This resource is not a substitute for medical advice, and Pharmaceutical Company Limited. should not be used in place of advice from a healthcare professional. Please contact a healthcare professional for advice. This resource is intended C-XXXXXX/XXX//9999 July 2020 for an international audience outside of the USA and has been produced by Takeda. This resource has been developed in accordance with industry and legal standards to provide information for the general public about Hunter syndrome health topics. Takeda makes every reasonable effort to include accurate and current information. However, the information provided in this resource is not exhaustive.

These materials have been developed by a team of Psychologists based at Sprout Behaviour Change Ltd (London, UK). The techniques and methodologies used stem from Acceptance Commitment Therapy: a validated psychological approach used to address emotional, practical and stressful challenges that may be hard to overcome. to learn more Visit Huntersyndrome.info Copyright 2020 Takeda Pharmaceutical Company to learn more Limited. All rights reserved. Takeda and the Takeda Logo are registered trademarks of TakedaVisit Huntersyndrome.info Copyright 2020 Takeda Pharmaceutical Company This resource is intended only to provide knowledge of Hunter syndrome health topics. This resource is not a substitute for medical advice, and Pharmaceutical Company Limited. Limited. All rights reserved. Takeda and the should not be used in place of advice from a healthcare professional. Please contact a healthcare professional for advice. This resource is intended C-XXXXXX/XXX//9999 July 2020 Takeda Logo are registered trademarks of Takeda for an international audience outside of the USA and has been produced by Takeda. This resource has been developed in accordance with industry This resource is intended only to provide knowledge of Hunter syndrome health topics. This resource is not a substitute for medical advice, and Pharmaceutical Company Limited. and legal standards to provide information for the general public about Hunter syndrome health topics. Takeda makes every reasonable effort to should not be used in place of advice from a healthcare professional. Please contact a healthcare professional for advice. This resource is intended C-XXXXXX/XXX//9999 July 2020 include accurate and current information. However, the information provided in this resource is not exhaustive. for an international audience outside of the USA and has been produced by Takeda. This resource has been developed in accordance with industry and legal standards to provide information for the general public about Hunter syndrome health topics. Takeda makes every reasonable effort to include accurate and current information. However, the information provided in this resource is not exhaustive. 13

References 1. Busza J et al. I don't want financial support but verbal support. How do caregivers manage children's access to and retention in HIV care in urban Zimbabwe? J Int AIDS Soc. 2014;17(1):18839. 2. Burton B, Giugliani R. Diagnosing Hunter syndrome in pediatric practice: practical considerations and common pitfalls. Eur J Pediatr. 2012;171(4):631-639. 3. Muenzer J et al. Multidisciplinary management of Hunter syndrome. Pediatrics. 2009;124(6):e1228. 4. Harrington M et al. Assessing the impact on caregivers caring for patients with rare pediatric lysosomal storage diseases: Development of the Caregiver Impact Questionnaire. Journal of Patient-Reported Outcomes. 2019;3(1):44. 5. Somanadhan S, Larkin PJ. Parents’ experiences of living with, and caring for children, adolescents and young adults with Mucopolysaccharidosis (MPS). Orphanet Journal of Rare Diseases. 2016;11(1):138. 6. Guarany NR et al. Mucopolysaccharidosis: Caregiver Quality of Life. Journal of Inborn Errors of Metabolism & Screening. 2015;3:2326409815613804. 7. Robertson K. Active listening: more than just paying attention. Aust Fam Physician. 2005;34(12):1053-1055. 8. Royal College of Nursing. Stress and you: A guide for nursing staff. Publication Code 004 967. Available at: https://www.rcn.org.uk/healthy-workplace. 9. Mind. How to look after your mental health as a nurse. Available at: https://www.mind.org.uk/workplace/coronavirus-and-work/being-a-nurseduring- coronavirus/. 10. Hart PL et al. Resilience in nurses: an integrative review. Journal of Nursing Management, 2014;22(6):720-734. 11. Yilmaz EB. Resilience as a strategy for struggling against challenges related to the nursing profession. Chinese Nursing Research. 2017;4:9-13. 12. Royal College of Nursing. Resilience. Available at: https://rcni.com/hosted-content/rcn/mnd/resilience. 13. Hartlage HN. Exploring Boundaries in Pediatric Nursing. Journal of Pediatric Oncology Nursing. 2012;29(2):109-112. 14. Nursing and Midwifery Board of Australia. A nurses's guide to professional boundaries. 2010. ISBN 978-0-9807515-7-4. 15. Burke K et al. Adapting acceptance and commitment therapy for parents of children with life-threatening illness: pilot study. Fam Syst Health. 2014;32(1):122-127. 16. Graham CD et al. A systematic review of the use of Acceptance and Commitment Therapy (ACT) in chronic disease and long-term conditions. Clin Psychol Rev. 2016;(46):46-58. 17. Whittingham K et al. Parenting Intervention Combined With Acceptance and Commitment Therapy: A Trial With Families of Children With Cerebral Palsy. Journal of Pediatric Psychology. 2016;41(5):531–542. 18. Blackledge JT, Hayes S. Using Acceptance and Commitment Training in the Support of Parents of Children Diagnosed with Autism. Child & Family Behavior Therapy. 2006;28(1):1-18. 19. Sairanen E et al. Psychological inflexibility explains distress in parents whose children have chronic conditions. PloS One. 2018;13(7):e0201155. 20. Neff KD & Faso DJ. Self-Compassion and Well-Being in Parents of Children with Autism. Mindfulness. 2015;6(4):938–947. 21. Nabors LA. Information for Mental Health Providers Working With Children Who Have Chronic Ilnesses. Bentham Science Publishers, 2014. 22. Valentine F & Lowes L. Nursing Care of Children and Young People with Chronic Illness. Wiley-Blackwell, 2007. 23. Barlow JH et al. The effectiveness of the Training and Support Program for parents of children with disabilities: a randomized controlled trial. J Psychosom Res. 2008;64(1):55-62. 24. Wong CCY et al. Self-Compassion: A Potential Buffer Against Affiliate Stigma Experienced by Parents of Children with Autism Spectrum Disorders. Mindfulness. 2016;7(6):1385–1395.