STEPS TEAM Supporting Treatment of Evolving Palliative Symptoms

1. STEPS TEAMSupporting Treatment of Evolving Palliative Symptoms Lynette Thacker Clinical Nurse Specialist Paediatric Palliative Care Paediatric Palliative Care Service November 2011 Disclaimer: Whilst every effort has been made to ensure that the information in this presentation is accurate and referenced the author does not accept any responsibility for the use by any third parties.

2. DEFINITIONS OF PAEDIATRIC PALLIATIVE CARE • Palliative care for children is the active total care of the child's body, mind and spirit, and also involves giving support to the family. • It begins when illness is diagnosed, and continues regardless of whether or not a child receives treatment directed at the disease. • Health providers must evaluate and alleviate a child's physical, psychological, and social distress. • Effective palliative care requires a broad multidisciplinary approach that includes the family and makes use of available community resources; it can be successfully implemented even if resources are limited. • It can be provided in tertiary care facilities, in community health centres and even in children's homes. World Health Organisation 1998

3. OTHER DEFINITIONS • Palliative care is an active and total approach to care, embracing physical, emotional social and spiritual elements. • It focuses on enhancement of quality of life for the child and support for the family and includes the management of distressing symptoms, provision of respite and care following death and bereavement. • It is provided for children for whom curative treatment is no longer an option and may extend over many years. ACT/RCPCH 1997

4. TERMINOLOGY USED IN PALLIATIVE CARE • End of Life Care -This refers to the period when a child with advanced disease lives with the condition from which they will die. It includes those with any chronic, progressive, eventually fatal illness and could be a period of weeks, months or years. • Terminal Care -This refers to care provided when a child is thought to be in the dying phase and usually refers to the last days or hours of life. West Midlands Children’s and Young Peoples Toolkit 2011

5. THE ACT CATEGORIES • Category 1 - This group includes life-threatening conditions for which curative treatment may be feasible but can fail. Here access to palliative care services may be necessary when treatment fails or during an acute crisis, irrespective of the duration of that threat to life. On reaching long-term remission or following successful curative treatment there is no longer a need for palliative care services. Examples: Cancer, irreversible organ failures of heart, liver, kidney.

6. Category 2 - This group includes conditions where premature death is inevitable, but where there may be long periods of intensive treatment aimed at prolonging life and allowing participation in normal activities. Examples: Cystic fibrosis, Duchenne muscular dystrophy.

7. Category 3 - Here progressive conditions without curative treatment options are included, where treatment is exclusively palliative and may commonly extend over many years. Examples: Batten disease, mucopolysaccharidoses.

8. Category 4 -This group includes irreversible but non-progressive conditions causing severe disability leading to susceptibility to health complications and the possibility of premature death. Examples: Severe cerebral palsy, multiple disabilities such as following brain or spinal cord injury, complex health care needs with a high risk of an unpredictable life-threatening event or episode.

9. Case studies • Think about a child from each of the categories. • Write down all the professionals/services involved with each child.

10. WHO PROVIDES PAEDIATRIC PALLIATIVE CARE Children and young people with palliative care needs and their families can access the services they need according to the different stage of the child’s condition A key worker will be responsible for ensuring joined-up and co-ordinated service provision Adapted from Better Care: Better Lives (2008)

11. WHY DO WE NEED PAEDIATRIC PALLIATIVE CARE SERVICES • With medical advances many children and young people with complex conditions are living longer. • There are increasing numbers of adolescents with palliative care needs and problems. • Difficulties are experienced during transition from children to adult services, as neither are suitable to meet many young peoples needs.

12. Palliative Care Symptoms - General • Take a thorough nursing assessment, talk with the family. • Regular reassessment of their symptoms in the same way as any child who is unwell. Generally, symptoms deteriorating every week reassess every 3 weeks, every day reassess every 3 days, every hour reassess every 3 hours. • All symptoms should be explored and addressed as part of a holistic assessment, including physical, psychological, spiritual and social. • Remember that parents know their child well. • Parents observations are key to understanding the child’s symptom progression and it’s impact upon them and the family. • When assessing a child consider: What do we know about this condition, presentation, progression and symptoms in end stage disease?

13. What complications have been evident? • What are the child’s symptoms at present? • What has already been tried and with what effect? • Palliative care emergencies in this child? • Anticipate management and support needed. • Explain the symptoms and their management to the child and their family.

14. Discuss potential complications and management of these. • Discuss a plan to manage the symptoms with them that is acceptable to them and place of care (home, hospital, hospice). • Plan reassessment period. • Ensure that family and staff know how to access care including 24hr advice. • Communicate well between professionals and family, ensuring clear documentation of symptom progression and management. • Don’t be afraid to say that you don’t know and be willing to seek advice from others

15. In terms of treatment: • Keep treatment as simple as possible • Stick to one drug per symptom where possible • Involve the child & family in decisions re treatment choices • Manage with oral preparations where possible • Plan for anticipated symptoms • Listen to the child & family’s account • Ensure management is reviewed in an agreed manner • Regularly review the overall medications being given. Do all remain necessary? Consider rationalising drug use, especially in the terminal stage.

16. Pain Wong-Baker FACES Pain Rating Scale Numeric Rating Scale 0 1 2 3 4 5 6 7 8 9 10 No Pain Mild Moderate Severe Worst Possible Assess using appropriate tool for verbal or non-verbal child.

17. Not all pain can or needs to be controlled with medication. Analgesia can be a combination of non opiate and adjuvant drugs or in combination with opiates. Non-pharmacological approaches can be used instead of and in combination with medication e.g. discussing fears, distraction, positioning and warmth.

18. There may also be more than one source of pain.

19. When Do Children Experience Pain Think about a child you’ve cared for that has a palliative care condition, when have they experience pain and what have you already used to manage this pain.

20. Total pain Spiritual Emotional TOTAL PAIN Physical Social

21. 3 2 1 W.H.O. ANALGESIC LADDER Strong opioid +/- adjuvant Weak opioid Severe Pain Morphine Diamorphine Fentanyl +/- adjuvant Non-opioid Moderate Pain Codeine Transaxmic Acid +/- adjuvant Pain persists or increases Mild Pain Paracetamol NSAID – Ibuprofen

22. Golden Rules

23. Adjuvant Analgesics ‘Adjuvant’ = not primarily analgesic but can improve pain in certain circumstances • Neuropathic - anticonvulsants (carbamazepine, gabapentin), antidepressants (amitriptyline), NMDA receptor antagonists (methadone, ketamine) • Bone - NSAIDs, bisphosphonates, RTx, chemo • Muscle spasm - Benzos, baclofen, tizanidine, botox • Cerebral irritation- Benzos, phenobarb • Inflammatory/Oedema – Steroids • Non-pharmacological - Physio, Psychology…..

24. Initiating strong opioid therapy • What drug? Morphine - short acting formulation (Oramorph, Sevredol) By mouth if possible • What dose? 1mg/kg/day = total daily dose = 30mg 30mg ÷ 6 = 4 hourly dose = 5mg • And for breakthrough pain? • Give the 4 hourly dose (5mg) as required

25. Titration phase Aim – to match the amount of analgesia given with the degree of pain experienced • Add up all doses taken in 24 hours so if 6 doses x 5mg 30mg + 30mg = 60mg 60mg ÷ 6 = 10mg Prescribe 10mg 4hrly and 10mg prn for breakthrough pain

26. Maintenance phase • More convenient opioid preparations • MST • Total daily Oramorph requirement: 60mg • Appropriate MST dose: 30mg bd • Diamorphine SCI • Total Oramorph requirement: 60mg • Appropriate Diamorphine dose: 20mg/24hrs = 60mg/3 as Diamorphine 1/3rd stronger than Oral morphine • Prescribe breakthrough analgesia

27. Calculations: Initiating Phase What dose? Child weight 10kg, 25kg, 50kg total daily dose = 4 hourly dose = Breakthrough pain =

28. Calculations: Titration phase Used 4 breakthrough doses over 24 hours New total daily = New 4 hourly dose = New breakthrough dose =

29. Calculations: Maintenance phase MST Total daily Oramorph requirement= Appropriate MST dose = Diamorphine SCI Appropriate Diamorphine dose=

30. Calculations: Changing to Diamorphine in subcutaneous syringe driver: Maintance Dose of MST 20mg twice daily. Breakthrough dose 6.5mgs used 9 times in past 48 hours. Diamorphine dose for 24 hours = How much Diamorphine is child receiving kg/hour=

31. Think about a child that you have cared for and write down any other symptoms that were difficult: How were they managed:

32. Nausea and Vomiting • Carefully consider the cause of nausea and vomiting. • It may not be appropriate to offer terminally ill children, close to death, enteral feeding. • Most children do not require large amounts of fluid and mouth care alone will help them to remain comfortable. • Parental anxiety around nutrition is very common. Effective control of nausea and vomiting, constipation and mucositis will help to maintain some degree of dietary intake. • Try to give fluids as the child tolerates. Interesting drinks, jellies, ice-lollies and ice cream can all help, and if the child is still eating, offer small portions.

34. Antiemetic receptors Twycross R, Back I. Nausea and vomiting in advanced cancer. European Journal of Palliative Care 1998;5(2):39-45. D2 H1 ACh 5HT2 5HT3 5HT4 Metoclopramide ++ 0 0 0 (+) ++ Domperidone ++ 0 0 0 0 0 Ondansetron 0 0 0 0 +++ 0 Cyclizine 0 ++ ++ 0 0 0 Hyoscine 0 0 +++ 0 0 0 Haloperidol +++ 0 0 0 0 0 Prochlorperazine ++ + 0 0 0 0 Chlorpromazine ++ ++ + 0 0 ? Levomepromazine ++ +++ ++ +++ 0 ?

35. Gastro-Oesophageal Reflux • Lax gastro-oesophageal sphincter D2 blockers (eg domperidone, metoclopramide) • Painful and dangerous acid reflux H2 blockers (eg ranitidine) Proton blockers (eg omeprazole) Gaviscon • Loss of normal reflex motility Change feed timings, D2 blockers • Obstruction Avoid prokinetics if colicky pain Steroids may help if tumour is cause Don’t prescribe prokinetic and anticholinergic together

36. Dyspnoea, Coughing and Secretions • Dyspnoea is a subjective sensation of breathlessness, and a very frightening symptom. • Always assess for a reversible cause of the breathlessness and treat accordingly • Use simple measures first e.g, posture, humidity, fresh air and fan. Anxiety is a major component of breathlessness. • Excess upper airway secretions are common and can be particularly distressing for the child and the family. • Excessive suction should be discouraged as it is unpleasant for the child and may stimulate production of more secretions. • Oxygen may reassure and may not be needed continuously. Oxygen is generally only recommended for children who have benefited from it previously.

37. Remember that not all dyspnoeic patients are hypoxic, that oxygen is a drug and should be prescribed as such, and that oxygen may depress the respiratory drive and therefore be harmful. • In toddlers the equipment can be seen as frightening, causing increased anxiety and worsen the breathlessness. • In palliative care, the monitoring of oxygen saturations is not always recommended. It may be better to look at the child and their condition rather than the numbers. • Dyspnoea is common in neurodegenerative disorders due to weakened respiratory muscles and the inability to clear secretions. Physiotherapy should be done gently. • Thick secretions can be managed with nebulised normal saline. Consider nebulised bronchodilators. • Oral morphine or subcutaneous diamorphine, initially given at half the minimum analgesic dose, can help to settle dyspnoea.

38. Bleeding • The sight of blood is distressing to the child, parent and carer alike. If bleeding is likely to happen, a gentle warning may help to reduce distress and shock for the parents. • It is important to agree a platelet transfusion protocol with the family in advance. Generally only if the child is symptomatic with bleeding that is overt and persistent should platelets be given. • If bleeding does occur the use of red towels and blankets may help minimise visual the shock. • Consider using tranexamic acid orally or topically for oral bleeding.

39. Convulsions & Muscle Spasms • Convulsions and muscle spasms are most commonly seen in the palliative care setting in children with neurodegenerative disorders. • Those with neurodegenerative disorders will often already be on anticonvulsant medications and parents/carers will be knowledgeable about recognising and treating convulsions. For these children convulsions are often variable in type and may become frequent and severe and more difficult to control towards the end of life. • Children may be very distressed when having repeated muscle spasms. • Early involvement from a physiotherapist can be useful and they can give advise on positioning, seating, handling that may prevent positioning that can cause muscle spasm. • An increased muscle tone and spasm may be the only thing that allows the child to sit or stand up. Certain treatments may therefore decrease their mobility, head control, airway management and general posture and medications can cause unnecessary sedation. • In the terminal stages seizures tend to become more severe and frequent. The child may not be able to absorb medications at this stage so subcutaneous midazolam or phenobarbitone may need to be considered.

40. Restlessness/Agitation • Try to nurse in a calm, peaceful, familiar environment. A parent or trusted adult being present may help. Address the fears and remove pain or other symptoms or inadequate positioning. Sedation • It may be neccessay during the final stages of the child’s illness to manage severe distressing agitation. • It is important to first ensure that all other potential contributory underlying symptoms have been addressed and that the potential for respiratory depression has been considered. • Ensure agitation is not pain related (including full bladder) and explore the child’s fears. • Oral diazepam or amitryptyline can be useful, particularly if there is sleep disturbance or an element of depression. • A continuous infusion of Midazolam (sedating and anxiolytic) can be used.

41. Infection • Infection is one of the commonest causes of the terminal event in children with a life threatening condition. Infections should be treated when its effect is contributing to symptoms. • Always discuss and record the course of action that has been taken with the parents and the child when appropriate. • Use of intravenous antibiotics needs to be carefully justified in a terminal setting. • Whatever decision is made ensure the parents are comfortable as possible as it may affect their grieving process. Sometimes antibiotics are necessary e.g. pain relief for an acute ear infection to give symptom relief, when parents have otherwise decided on no more treatment.

42. Constipation • Liaise with parents, as they know their child’s bowel habits best. • There may be a wide variety of causes of constipation, including, inactivity, especially if in a wheelchair long term, neurological conditions gut dysmotility, decreased food intake, fear of opening bowels, medication especially opiates. • If a prophylactic prescription for a laxative is required, consider: • Constipation induced by opiates will require stimulant and stool softener Movicol is often used. • The child may need a suppository or an enema if these do not work or if they refuse to take the medication, but may not be acceptable to them, needing sensitive discussion.

43. Bladder • Do not get too concerned about falling urine output in the terminal days. • Bladder spasms can be treated with Oxybutilin • Obstruction may require catheterisation for comfort • Retention arising from use of opioids may be transient, and simple manoeuvres such as gentle expression, warm baths etc may be sufficient. Fentanyl causes less urinary retention. • The loss of bladder function in a child who has previously been continent can be a source of great distress for themselves and their parents. The use of pads is non-invasive and simple, but needs tact and sensitivity to introduce.

44. Sleeping difficulties • Try to address the child’s fears. Whilst sleep patterns may be very disrupted, try to optimise the bedtime routine. • Consider complimentary therapies to aid relaxation. Try to disturb the child as little as possible overnight, for example, if possible, reschedule medication. • Medication may be required, Melatonin is useful for children who have neurological disorders, sometimes sedation is required. • Psychological • Give the family time and be prepared to listen. Providing honest answers to straight questions can allay fears and anxieties. • In a child manifesting clinical symptoms of anxiety do not be afraid to use medication as an adjuvant to counselling and support. Symptoms may be very different to adults – younger children tend to regress and develop behavioural problems, older children may have nightmares, insomnia or become introspective. Insomnia is a problem for the child and the parents.

45. Oral Care • Good oral/mouth care can enhance the quality of life of children in the palliative care setting. • Signs may include a swollen mouth, ulceration, candida, inability to salivate, painful swallowing, dry tongue and cracked lips. The cause should be identified, discomfort and pain treated. An anti fungal is often needed. • If a child is old enough and able to use a soft toothbrush this should be continued as long as possible. The parents may like to help with this part of their child’s care. A finger tooth brush is often needed in the terminal phase. • If the child has bleeding gums, tranexamic acid may be used as a mouthwash. If the toothbrush is too sore they may like to use cotton swabs soaked in water or mouthwash swabbed around the mouth. • They may also like to use Benzydamine spray or mouthwash as analgesia and Vaseline (unless contraindicated by the use of oxygen) or lip balm for cracked lips. Biotene is a useful saliva replacement gel. It is helpful to start this early, preventatively, before they need it to improve acceptability.

46. Skin care • Good hygiene is important, and attention to hair and nail presentation must not be overlooked. • Children often become immobile and their skin becomes very vulnerable to breakdown with poor subsequent healing. It is important to consider the risks of pressure areas and use pressure-relieving devises when necessary. • Hoists and slings may also be needed especially if caring for a bigger child. • If the skin breaks down advice may be sort from the tissue viability team regarding the appropriate dressings to use.

47. What do we need to consider to care for a child at home:Consider the child’s and family’s understanding of condition.Child’s needs assessed, plan of care developed with child and family.Communication and information provided to child and family appropriate to age and understanding.Advanced care planning should incorporate child’ and family’s wishes.Consider child’s and family’s religious and spiritual needs.

48. Anticipate symptoms and have medication and medication protocols at home. Consider, discuss and decide if any interventions need to be discontinued. Give family contact numbers for emergency, out of hours services. Inform all necessary services of plan. Give family opportunity to discuss plans for after death including who will support them.