Download Symptoms in non-malignant paediatric palliative care I: Cerebral palsy

Symptoms in non-malignant
paediatric palliative care I:
Cerebral palsy
Dr Jo Griffiths
Consultant community child Health, Swansea NHS trust
Honorary Lecturer Paediatric palliative medicine, Cardiff
University
Why consider cerebral palsy
important in paediatric palliative
care?
 The
range of conditions needing
specialist paediatric palliative care is very
wide. The RCPCH in association with the
association for children with LifeThreatening diseases (ACT), has defined
four groups of conditions:-
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Group 1: Life threatening conditions for which curative
treatment may be feasible but can fail. Palliative care may be
feasible but can fail. Palliative care may be necessary during
periods of uncertainty and when treatment fails ( e.g cancer,
cardiac anomalies).
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Group 2: Conditions in which there may be long periods of
intensive treatment aimed at prolonging life and allowing
participation in normal childhood activities but premature death
is still possible ( e.g Cystic fibrosis, muscular dystrophy).
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Group 3: progressive conditions without curative treatment
options, in which treatment is exclusively palliative and may
commonly extend over many years ( Batten’s disease,
mucopolysaccharidosis)
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Group 4: conditions with severe neurological disability
which may cause weakness and susceptibility to health
complications and may deteriorate unpredictably, but are
not considered progressive ( e.g Cerebral palsy).
Learning objectives
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Develop a logical, rational and systematic approach to the diagnosis
and management of symptoms in children suffering from cerebral
palsy and neurodegenerative conditions
Consider the causes of pain in children with cerebral palsy &
neurodegenerative conditions
Recognise the challenges in assessing pain in this group of children
Describe possible approaches to hyper salivation using medication,
surgery or radiotherapy.
Discuss the issues surrounding feeding and nutrition in this group of
children
Consider the wider impact on the family and patient.
Consider the potential impact of new therapies and support on your
future practice.
Cerebral palsy
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A neurological syndrome rather than disease specific. It is
characterized by a group of motor syndromes resulting from
disorders of early brain development.
Often associated with
 Epilepsy
 Abnormalities of speech,
live births
 Abnormalities of vision
 Learning difficulties.
Prevalence
1.5-2.5 / 1000
Although CP has no cure, many palliative measures are
available to aid children in becoming as highly functional as
possible. These may include communication devices,
physiotherapy, bracing, speech therapy etc
Deaths in Cerebral palsy
 Nearly
all deaths from CP during
childhood will occur in children with
Spastic quadriplegia
 Spastic quadriplegia accounts for 20% of
children with CP.
 Pneumonia is the most frequent cause of
death.
Main symptoms in life limiting
cerebral palsy:
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Spasticity & muscle spasm
Gastro-oesophogeal reflux
Pain
Drooling
Cerebral irritability
Convulsions
Sleep disturbance
Constipation
Feeding difficulty
Swallowing difficulties leading to aspiration pneumonia.
Psycho-social difficulties of child and family
Hunt et al; Medical and nursing problems of children
with neurodegenerative disease.
Palliat Med 1995;9 :19-26
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40% of children admitted to helen house over an 11 year period suffered
from neuro-degenerative conditions, chiefly inherited metabolic diseases.
communication disorders and feeding problems were found in over 70% of
children.
Resp infections and dyspnoea were recorded in 38% of the children,
exacerbated by limited mobility, swallowing difficulty, muscular weakness
and kyphoscoliosis.
1/3rd had problems swallowing their own secretions.
Seizures 60%
Constipation 44%
1/3rd were identified as experiencing pain, with the most common cause
being muscle spasm. Other causes included constipation, gastritis and
oesophagitis from reflux.
1/3rd had movement disorders.
1/3rd sleep disorders
Although most were immobile and incontinent, none developed skin
breakdown.
Case 1
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James is 10 years old. His birth was
complicated by hypoxia and he has severe
quadraplegic cerebral palsy.
He has no speech but communicates through
noises and eye contact
He is non-ambulant and has kyphoscoliosis and
deformities of his hips. He has high tone and
severe spasticity making nappy changes difficult.
His weight is below the 0.4th centile, it takes
2hours to feed him and his diet is limited
His mum thinks he is in pain
 1.
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How are you going to assess his pain
What could be the cause or causes of
his pain?
Hunt A, Mastroyannopolou K, Goldman A and Seers K.
(2003) ‘Not knowing – the problem of pain in children
with severe neurological impairment’ International
Journal of Nursing Studies; 40(2) pp171-183
 Qualitative
study aiming to gain an
understanding of pain in children with
neurological disease
Sources of pain
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Four main categories
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* Pains associated with alterations in gut motility such
as gastro-oesophageal reflux, wind and constipation.
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* Pains related to musculo-skeletal problems, particularly
muscle spasm, dislocated hip, joint and back pain and pain
generally associated with the child’s immobility.
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* Co-incidental pains that can also occur in otherwise
well children, for instance, ear and tooth ache.
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* Pain related to poorly fitting aids and equipment.
Pain cues fell largely in to the following
groups:
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* Changes in facial expression.
* Changes in movement and posture.
* Vocal cues such as crying, moaning, groaning or
whimpering.
* Changes in the child’s usual patterns, such as how
well the child slept or tolerated feeds.
* Physiological changes affecting the child’s appearance
such as change in colour or sweating.
* Changes in mood including withdrawal and depression.
knowledge required for comprehensive
pain assessment and management.
Know the
child
Know the
Science
Know the
population
James
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Assessment:
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QUEST approach
Take your time
Flexibility
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Possible causes:
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Muscle spasm
Dislocated hip
Reflux
Constipation
Tooth ache
Poorly fitting chair &
hoist.
Spasticity & Muscle spasm
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Common
PAIN
SPASM
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Spasticity is complex
No single treatment modality is likely to be sufficient
alone
New treatment methods in last decade
Management needs to be specialized and individualized
Challenge: To find the best combination of methods
Requires co-operation across disciplines
Insufficient evidence for most.
Considerations
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Symptomatic - massage, heat, bathing etc
Physiotherapy, splints, casting,
Aids : seating etc
Drugs: Baclofen, Tizanidine, Dantrolene,
benzodiazepines, clonidine
Botox ( reversible chemodenervation)
Intrathecal baclofen pumps
Deep brain stimulation
Surgery - rhizotomy, Neurectomy
Hyperbaric oxygen? / Electrical stimulation?
James 2
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James’ pain improves considerably after a visit
to the dentist, introduction of omeprazole and
use of a new hoist and wheelchair.
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However….. His mum is worried because he’s
not sleeping at night.
Why might this be? What help can you offer?
SLEEP DISTURBANCES
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Profound impact on both children and families.
States of wakefulness are thought to be regulated by
diencephalic and brainstem nuclei and circadian
rhythms.
They require a normal suprachiasmatic nucleus of the
hypothalamus and connections. Therefore children with
midline brain maldevelpment are at high risk of sleep
disorders.
Some portions of the cerebral hemispheres also
contribute to sleep-wake cycles, because children with
hydranencephaly, lacking cerebral hemispheres, but
having an intact brain stem and cerebellum also have
profound sleep disturbances
Factors affecting sleep
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Sleep related breathing disorders can be associated with
anatomic abnormalities.
 Seizures
 Visual impairment.
 Those with LD may have difficulty in interpreting the social
cues families use to promote healthy sleep cycles
 Other symptoms impact on sleep – reflux, colic, hypoxia,
pulmonary oedema, muscle spasm, headaches, movement
disorders.
 Therapeutic drugs used in palliative care can disrupt normal
sleep patterns – opiods, AEDs etc
 Hospitalization and episodic illness interferes with consistent
sleep because of disruption of routine.
 Psychological stressors – fear of pain, fear of dark fear of
death, separation anxiety.
 Negative associations with bed if linked with procedure
Treatment
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Relies on assessing all the previous factors.
 A number of studies have documented the
effectiveness of melatonin in reducing sleep
latency in many children with developmental
disorders. ( ¾ of children are able to fall asleep
faster and may stay asleep longer)
 Hypnotics are less satisfactory – short term
therapy may be indicated.
 Teach and re-inforce basic principles of sleep
hygiene.
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Along with his mother you identify many issues
that may affect his sleeping. Good sleep hygiene
measures alongside with Melatonin improve
things slightly.
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Mum wonders if his drooling and subsequent sore
chin might be affecting his sleep. She tells you
she changes his bib or clothing 10-15 x every day.
His chin is chapped and raw. She’s worried he’ll
choke on his own Saliva.
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How can you manage drooling?
SIALORRHOEA (DROOLING)
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‘ A loss of control over one’s own saliva’
Hypersalivation and ptyalism are sometimes used to
mean similar things.
Secretions that pool in the hypopharynx and contribute
to aspiration can cause choking, dysphagia and
breathing difficulties.
Sailorrhoea is a serious social handicap, it carries
considerable social stigma, can interfere with
communication devices and is a barrier to interpersonal
relationships.
Impacts on caregivers who may have to change the
child's clothing or bib 10-20 times / day.
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Unlikely to cause harm unless the body’s normal
reflex coughing mechanisms are also impaired
in which case it can lead to persistent microaspiration.
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facial chapping
Dental caries
Lip cracking and fissures
As many as 58% of children with cerebral palsy
and 10% of children with other neurological
disorders are faced with severe sialorrhoeas that
requires intervention.
Pathophysiology: excess
production of saliva
 Inability to retain saliva within the mouth
 Problems with swallowing.
 Overproduction
in the absence of
swallowing impairment usually does not
cause sialorrhoeas
Management:
Most treatments are directed at reducing the
volume of saliva produced.
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Behavioral
Pharmacological
Surgical interventions: should be considered in
children with LLC but are not always appropriate.
Invasive techniques should be postponed until
after permanent dentition has appeared as
sailorrhoea may become less of a problem after
this.
PHARMACOTHERAPY
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Medications reduce saliva production and/or alter it’s
consistency
Anticholinergic drugs inhibit salivary secretion by
reversible blockade of the acetylcholine –mediated
activation of muscarinic receptor – e.g. glycopyrolate and
scopolamine ( hyoscine)
Hyoscine can be inhaled, used orally or in transdermal
systems - effectiveness diminishes with time.
Glycopyrolate may have fewer adverse effects and better
effectiveness. Still 20% discontinue it due to S/E
Botulinum toxin A – injection into parotid and
submandibular glands, reliable and well tolerated. Lasts
3 – 8months
Surgery:
First described in 1967. generally reserved for nonprogressive neurological disorders such as CP when
response to medication is insufficient.
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Either – reduce amount of saliva or divert the saliva more
posteriorly.
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remove gland
ligate duct
Section nerves involved
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Results of all three have been disappointing.
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Irradiation of submandibular & sublingual salivary glands:
Risk of secondary malignancy
Cautions
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Published literature and clinical observations suggest that
pharmacotherapy offers only short term solutions, often at
the cost of considerable side effects
Even surgical approaches seem to lose effectiveness with
time.
S/E: excessive dryness of mouth epithelium can
exacerbated existing swallowing difficulties and aggravate
rate of resp infections and breathing difficulties.
Mucus producing respiratory glands are not regulated by
any major nerve supply that can be blocked resulting in
thicker mucus as saliva volume diminishes. This can
accumulate in the back of the throat with a tendency to
block airways or make food stick in the throat.
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Need adequate fluid intake,
Reduce mucosal inflammation
Antihistamines and NSAID may help
Suction
Cough –assist devices
Alternative / additional measures
 posture
control
 dental hygiene
 Address upper aero digestive inflammation
or obstruction.
 oral motor therapy
 behavior modifications
 Biofeedback
 Hypnotherapy
James 3
 You
start James on ¼ hyoscine patch and
increase it up to ½ with good effect.
 You
notice however that his feeding is
becoming more difficult and he’s choking
more on his food.
 Do
you want to intervene? What would
you suggest to mum?
 Whilst
you are arranging a video fluoroscopy
and feeding assessment James aspirates and
needs PICU admission. After a 7 week
admission he is discharged.
 It
has been suggested to mum that he should
have a gastrostomy and fundoplication.
 You
visit them at home to discuss it further
…………..
 Whilst
you are arranging a video fluoroscopy
and feeding assessment James aspirates and
needs PICU admission. After a 7 week
admission he is discharged.
 It
has been suggested to mum that he should
have a gastrostomy and fundoplication.
 You
visit them at home to discuss it further
…………..
Feeding in palliative care..
….brings to mind artificial hydration / nutrition in
the terminal stages but ethical dilemmas
precede this
 Consider Nasogastric tubes / gastrostomy tubes.
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Offering food to a child is one of the most basic
of parental instincts and good nutrition can
improve Q.O.L for child and family.
 But.. artificial nutrition may impose burdens on
the child that outweigh the possible benefits
Simple measures
- Look at position, seating, food offered etc
- Unhurried, frequent, small , appropriate textured
meals.
- Consider feeding equipment
- Oral desensitisation
- Calorie-dense additions e.g. cheese / cream
- Carbohydrate-providing glucose polymers
- Treat reflux or oesophagitis
Supporting families in decision
making:Decisions to increase oral supplements
Enteral / parenteral feeding
Issues of withdrawing / witholding
artificial hydration or nutrition
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Has to be multi-professional.. No individual should
make decisions alone.
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Supplemented by access to information from those
who know the family and child well.
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Nutritional issues rarely occur in isolation; their
impact on prognosis adds weight to the importance
of including the child in decision making if possible.
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Assessment and consequent planning needs to be
made for each child on an individual basis.
Guidance
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RCPCH: Withholding or withdrawing life
sustaining treatment in children. A framework for
practice. Second edition 2004.
 General medical council. Withholding and
withdrawing life-prolonging treatments: good
practice in decision making. London 2002
 BMA: Withholding and withdrawing lifeprolonging treatment. Guidance for decision
making. BMJ, 3rd edition London 2007
RCPCH
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the role of assisted feeding by NG tube or
gastrostomy should be considered very
carefully and discussed fully with the family’
 Emphasis placed on the inclusion of
competent children in decision making.
 Differentiates between the child with a
neurodegenerative disease related
swallowing disorder and a rapidly
progressive, disseminated malignancy.
Managing the feeding of a child
with a life-limiting illness
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Management begins at diagnosis
Includes multi-professional care
Regular reassessment is vital
This in turn paves the way for possible decisionmaking, at the appropriate time.
Optimise oral food intake before embarking on
tube feeding.
Include the family in decision making.
Consider the impact of home enteral feeding on
the entire family
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James has several more chest infections requiring
hospital admissions during one of which NGT
feeds are started. He is felt to be too unwell for a
general anaesthetic and plans for a gastrostomy
are changed.
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James gets very distressed on the ward and his
family are keen to keep him at home. His mother
spends all her time on the ward with him leaving
his father with the other children ( aged 6, 11 and
14). He has needed to give up work to do this.
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Discuss the impact of James’ ill health on the
wider family……………….
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Impact on family dynamics
Parental separation
Family separated : Other children don’t see mum
/ Mum & James don’t see Dad and Siblings
What happens if James dies? Impact on siblings,
parents relationship, mum’s ‘job’
What impact does nursing James at home have
on the family? Storage of equipment /
community nursing teams / home becomes a
hospital.
Financial difficulties
Lack of time for ‘normal activities’…. E.g
shopping / household chores.
Summary
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Many children with Cerebral palsy have life-limiting or life threatening
conditions
Good symptom control is imperative
There can be challenges in assessing symptoms, particularly pain.
Symptoms include:
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Pain from multiple causes
Constipation
Spasticity & muscle spasms
Feeding difficulties and aspiration
Convulsions & movement disorders
Behavioural issues and sleep disorders
Families need to be involved
All symptoms need to be considered alongside psycho-social issues that
the child and family face
Planning for critical illness and end of life care is welcomed by families.