Living with/adherence and PKU

Living with/adherence and PKU Dr Kathryn Bond bondk1@gosh.nhs.uk 0207 405 9200 x 0500

Behaviour Social Development Adherence Chronic Illness Emotional Development Psychological Functioning and PKU Quality of Life Dietary Demands Medical Factors e.g. Phe. levels Cognitive & Neuropsychological Development Family Functioning

Aims for presentation today • Think from a psychological perspective • Strategies • Time for discussion and reflection

Diet Feeling Different Non-adherence Supplements Blood tests PKU….the tricky bits!! Becoming more independent Cognitive Development (for some children) Difficulties for parents: e.g. anxiety, keeping control v promoting independence, guilt/feeling sorry, coping with difficult stages e.g. toddlers and teenagers!

Cognitive Development Increased risk with non-adherence and consistently high blood levels In comparison to siblings slightly lower IQ Executive Functioning: working memory, maths, attention & concentration, executive functioning

The meaning of PKU • What is the meaning of PKU in your family, in your lifestyle, in your cultural system?? ….acknowledge those difficult feelings, where do they come from…are there people undermining PKU management? Involve all your networks in managing PKU so it fits with your lifestyle

Parenting and PKU Some parents can struggle with guilt, feeling ‘sorry’ for their child, wanting to compensate for PKU… Anxiety: diagnosis was probably the most frightening experience of your life as a parent. It can feel as though you are no longer in control and you worry about being able to keep your child safe. Anxiety is transmitted to children. Overprotection: common, normal reaction (can be reinforced by the medical regime). How will your child achieve their best potential in life without becoming more independent and making some mistakes along the way?

Family Functioning and PKU • Chronic health problems like PKU results in extra demands for families • If there are pre-existing difficulties (like relationship problems, parenting difficulties, addiction, abuse, cultural expectations) it can be hard to keep pace with PKU • Talk to your clinic and GP about accessing extra support e.g. social services, CAMHS or AMHS

Control and Chronic Illness If you have a chronic health condition, like PKU, life can be different at times, sometimes it is not fair, there are things you have to do that sometimes you just don’t want to e.g. diet, supplements, medicines, blood tests, weighed and measured, hospital appointments……: FACT How can you give control back? Give choices (but stay in control). Can you work around some expressions of wanting to be in control? Rewards should be based on 75-85% success: do we always achieve 100% success?

Feeling Different • Not PKU per se but feeling different to everyone else • More difficult at secondary school/college…(how do I tell friends, girlfriends/boyfriends) • Set realistic strategies…remember what it was like to be a teenager • Promote well rounded self-esteem • Include good friends, girlfriends/boyfriends….they can be your best allies!! • Sensitive arrangements at school e.g. use of sink to brush teeth • Practice responses together to questions/comments about the diet, supplements, having PKU • Acknowledge, model and talk about how feeling different can be…and can be coped with • Acknowledge, model and talk about how the same issues exist for adults too – e.g. bullying/feeling anxious doesn’t end when you leave school

Becoming more independent • Start as early as possible • Aim: PKU is part of their life not part of parenting • Expect some setbacks & don’t highlight errors • Breakdown every task (e.g. diet, cooking, blood tests, supplements) into tasks and always be working on a step • What will it be like to lose control after so long!!....What long-term outcome do you want??

Blood Tests • What are you transmitting to your child? • How many steps of the blood test can your child do on their own? • Who gets the feedback from the blood tests at home? • Does your child know what the blood test is for and what it is measuring? Can they explain it to a friend?

BREATHING TO HELP RELAXATION Obviously we all know how to breathe… we do it all the time!! There are some ways though, of changing your breathing slightly to help you to be more relaxed. Try to breathe through your nose, using your stomach muscles. When you place your hands on your stomach you will feel the movement if you are breathing properly. Try this out to make sure that you are used to this feeling! Try to breathe slowly and regularly. It is important that you do not take a lot of quick, deep breaths as this can make you feel dizzy or faint and make your anxiety worse. Blowing out slowly when you are breathing out stops this.

SLOW MUSCLE RELAXATION Sit or lie down in a comfortable position. Try to relax as much as you possibly can. Think about your different muscle groups one at a time, and try to learn the difference between tight and relaxed muscles. Try to concentrate on the feeling in the muscle as it goes from tight to loose. Hands and arms Clench your fists and tense your arms. Feel the tightness in your hands and arms and then slowly relax them.See how far them. See how far theywill go, but do not push too hard. Do not hold on at all, let everything go!

YOUR FAVOURITE PLACE Another good way of relaxing/ distracting yourself is try to is to imagine your favourite place. It can be anywhere in the world or Universe, a place where you would most like to be and would be the happiest you have ever been. It could be a beach on a desert island, on a rollercoaster, or anywhere. Now close your eyes and imagine what it’s like… What is there? sounds, smells, noises, feelings, who is there with you? What are you doing?

How worried are you 3 minutes before the blood test? How worried are you 3 minutes after? Did you remember to breathe from your stomach? Yes no Write down the things you did to help yourself. How much did they help? I did my breathing from my stomach. I also used my special place and blocked everything out from my mind.

How anxiety works: a “Worry Cycle” You wake up on a Monday morning and have a job interview…… Thoughts It is going to be awful Everyone will laugh at me People will think I’m rubbish Behaviour you might avoid the interview, pace up and down, go to the toilet.. Feelings Feeling scared Heart pounding Breathing faster Sweating

Maslow’s Hierarchy of Needs

Prochaska & DiClemente, 1983

Thinking about change… • What worries you about your current situation? • How would you like things to be different? • What do you think you might do/be willing to try? • What are the advantages of change? • What makes you think that if you did decide to make a change, you could do it? • What encourages you that you can change if you want to? • Who could offer you helpful support in making this change? • What do you think would work for you, if you decided to change?

Protective Factors • Parents/family can take on the diet but without too much anxiety/control • Learning about your medical condition and understanding the diet early in childhood • Steadily increasing independence and control …especially before teenage years

Thank you for listening!...any questions??

Living with/adherence and PKU