STEPS TEAM Supporting Treatment of Evolving Palliative Symptoms

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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.
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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

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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

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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 Childrens and
  Young Peoples Toolkit 2011

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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.

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• 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.

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• 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
  .

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• 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.

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Case studies

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

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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 childs condition
A key worker will be responsible for ensuring
joined-up and co-ordinated service provision
Adapted from Better Care Better Lives (2008)
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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.

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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
  childs symptom progression and its 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?

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• What complications have been evident?
• What are the childs 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.

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• 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.
• Dont be afraid to say that you dont know and be
  willing to seek advice from others

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• 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 familys 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.

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• 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.

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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.

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There may also be more than one source of pain.
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When Do Children Experience Pain

• Think about a child youve cared for that has a
• palliative care condition, when have they
• experience pain and what have you already used
• to manage this pain.

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• Total pain

Spiritual
Emotional
TOTAL PAIN
Physical
Social
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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
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Golden Rules
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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..

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• 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

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• 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

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• 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

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• Calculations
• Initiating Phase
• What dose?
• Child weight 10kg, 25kg, 50kg
• total daily dose
• 4 hourly dose
• Breakthrough pain

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• Calculations
• Titration phase
• Used 4 breakthrough doses over 24 hours
• New total daily
• New 4 hourly dose
• New breakthrough dose

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• Calculations
• Maintenance phase
• MST
• Total daily Oramorph requirement
• Appropriate MST dose
• Diamorphine SCI
• Appropriate Diamorphine dose

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• 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

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

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• 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.

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Antiemetic receptors Twycross R, Back I. Nausea
and vomiting in advanced cancer. European Journal
of Palliative Care 19985(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 ?
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• 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
• Dont prescribe prokinetic and
• 
  anticholinergic together

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• 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.

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• 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.

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• 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.

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• 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.

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• 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 childs 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 childs 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.

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• 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.

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• Constipation
• Liaise with parents, as they know their childs
  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.

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• 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.

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• Sleeping difficulties
• Try to address the childs 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.

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• 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 childs 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.

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• 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.

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What do we need to consider to care for a child
at homeConsider the childs and familys
understanding of condition.Childs 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 familys wishes.Consider
childs and familys religious and spiritual
needs.
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• 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.