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

Dr Jo GriffithsDr Jo GriffithsConsultant community child Health, Swansea NHS trustConsultant community child Health, Swansea NHS trustHonorary Lecturer Paediatric palliative medicine, Cardiff Honorary Lecturer Paediatric palliative medicine, Cardiff UniversityUniversity

Why consider cerebral palsy Why consider cerebral palsy important in paediatric palliative important in paediatric palliative

care?care?

The range of conditions needing The range of conditions needing specialist paediatric palliative care is very specialist paediatric palliative care is very wide. The RCPCH in association with the wide. The RCPCH in association with the association for children with Life-association for children with Life-Threatening diseases (ACT), has defined Threatening diseases (ACT), has defined four groups of conditions:-four groups of conditions:-

Group 1: Life threatening conditions for which curative Group 1: Life threatening conditions for which curative treatment may be feasible but can fail. Palliative care may be treatment may be feasible but can fail. Palliative care may be feasible but can fail. Palliative care may be necessary during feasible but can fail. Palliative care may be necessary during periods of uncertainty and when treatment fails ( e.g cancer, periods of uncertainty and when treatment fails ( e.g cancer, cardiac anomalies).cardiac anomalies).

Group 2: Conditions in which there may be long periods of Group 2: Conditions in which there may be long periods of intensive treatment aimed at prolonging life and allowing intensive treatment aimed at prolonging life and allowing participation in normal childhood activities but premature death participation in normal childhood activities but premature death is still possible ( e.g Cystic fibrosis, muscular dystrophy).is still possible ( e.g Cystic fibrosis, muscular dystrophy).

Group 3: progressive conditions without curative treatment Group 3: progressive conditions without curative treatment options, in which treatment is exclusively palliative and may options, in which treatment is exclusively palliative and may commonly extend over many years ( Batten’s disease, commonly extend over many years ( Batten’s disease, mucopolysaccharidosis)mucopolysaccharidosis)

Group 4: conditions with severe neurological disability Group 4: conditions with severe neurological disability which may cause weakness and susceptibility to health which may cause weakness and susceptibility to health complications and may deteriorate unpredictably, but are complications and may deteriorate unpredictably, but are not considered progressive ( e.g Cerebral palsy).not considered progressive ( e.g Cerebral palsy).

Learning objectives Learning objectives

Develop a logical, rational and systematic approach to the diagnosis Develop a logical, rational and systematic approach to the diagnosis and management of symptoms in children suffering from cerebral and management of symptoms in children suffering from cerebral palsy and neurodegenerative conditionspalsy and neurodegenerative conditions

Consider the causes of pain in children with cerebral palsy & Consider the causes of pain in children with cerebral palsy & neurodegenerative conditionsneurodegenerative conditions

Recognise the challenges in assessing pain in this group of childrenRecognise the challenges in assessing pain in this group of children Describe possible approaches to hyper salivation using medication, Describe possible approaches to hyper salivation using medication,

surgery or radiotherapy.surgery or radiotherapy. Discuss the issues surrounding feeding and nutrition in this group of Discuss the issues surrounding feeding and nutrition in this group of

childrenchildren Consider the wider impact on the family and patient.Consider the wider impact on the family and patient. Consider the potential impact of new therapies and support on your Consider the potential impact of new therapies and support on your

future practice.future practice.

Cerebral palsyCerebral palsy A neurological syndrome rather than disease specific. It is A neurological syndrome rather than disease specific. It is

characterized by a group of motor syndromes resulting from characterized by a group of motor syndromes resulting from disorders of early brain development.disorders of early brain development.

Often associated withOften associated with

EpilepsyEpilepsy PrevalencePrevalence Abnormalities of speech,Abnormalities of speech, 1.5-2.5 / 1000 live births1.5-2.5 / 1000 live births Abnormalities of visionAbnormalities of vision Learning difficulties.Learning difficulties.

Although CP has no cure, many palliative measures are available to Although CP has no cure, many palliative measures are available to aid children in becoming as highly functional as possible. These aid children in becoming as highly functional as possible. These may include communication devices, physiotherapy, bracing, may include communication devices, physiotherapy, bracing, speech therapy etcspeech therapy etc

Deaths in Cerebral palsyDeaths in Cerebral palsy

Nearly all deaths from CP during Nearly all deaths from CP during childhood will occur in children with childhood will occur in children with Spastic quadriplegiaSpastic quadriplegia

Spastic quadriplegia accounts for 20% of Spastic quadriplegia accounts for 20% of children with CP. children with CP.

Pneumonia is the most frequent cause of Pneumonia is the most frequent cause of death.death.

Main symptoms in life limiting Main symptoms in life limiting cerebral palsy:-cerebral palsy:-

Spasticity & muscle spasmSpasticity & muscle spasm Gastro-oesophogeal refluxGastro-oesophogeal reflux PainPain DroolingDrooling Cerebral irritabilityCerebral irritability ConvulsionsConvulsions Sleep disturbanceSleep disturbance ConstipationConstipation Feeding difficultyFeeding difficulty Swallowing difficulties leading to aspiration pneumonia.Swallowing difficulties leading to aspiration pneumonia. Psycho-social difficulties of child and familyPsycho-social difficulties of child and family

Hunt et al; Medical and nursing problems of children Hunt et al; Medical and nursing problems of children with neurodegenerative disease.with neurodegenerative disease. Palliat Med Palliat Med 1995;9 :19-261995;9 :19-26

40% of children admitted to helen house over an 11 year period suffered 40% of children admitted to helen house over an 11 year period suffered from neuro-degenerative conditions, chiefly inherited metabolic diseases.from neuro-degenerative conditions, chiefly inherited metabolic diseases.

communication disorders and feeding problems were found in over 70% of communication disorders and feeding problems were found in over 70% of children.children.

Resp infections and dyspnoea were recorded in 38% of the children, Resp infections and dyspnoea were recorded in 38% of the children, exacerbated by limited mobility, swallowing difficulty, muscular weakness exacerbated by limited mobility, swallowing difficulty, muscular weakness and kyphoscoliosis.and kyphoscoliosis.

1/3rd had problems swallowing their own secretions.1/3rd had problems swallowing their own secretions. Seizures 60%Seizures 60% Constipation 44%Constipation 44% 1/3rd were identified as experiencing pain, with the most common cause 1/3rd were identified as experiencing pain, with the most common cause

being muscle spasm. Other causes included constipation, gastritis and being muscle spasm. Other causes included constipation, gastritis and oesophagitis from reflux.oesophagitis from reflux.

1/3rd had movement disorders.1/3rd had movement disorders. 1/3rd sleep disorders1/3rd sleep disorders Although most were immobile and incontinent, none developed skin Although most were immobile and incontinent, none developed skin

breakdown.breakdown.

Case 1Case 1 James is 10 years old. His birth was James is 10 years old. His birth was

complicated by hypoxia and he has severe complicated by hypoxia and he has severe quadraplegic cerebral palsy. quadraplegic cerebral palsy.

He has no speech but communicates through He has no speech but communicates through noises and eye contactnoises and eye contact

He is non-ambulant and has kyphoscoliosis and He is non-ambulant and has kyphoscoliosis and deformities of his hips. He has high tone and deformities of his hips. He has high tone and severe spasticity making nappy changes severe spasticity making nappy changes difficult.difficult.

His weight is below the 0.4His weight is below the 0.4thth centile, it takes centile, it takes 2hours to feed him and his diet is limited2hours to feed him and his diet is limited

His mum thinks he is in painHis mum thinks he is in pain

1. How are you going to assess his pain1. How are you going to assess his pain

2. What could be the cause or causes of 2. What could be the cause or causes of his pain?his pain?

Hunt A, Mastroyannopolou K, Goldman A and Seers K. Hunt A, Mastroyannopolou K, Goldman A and Seers K. (2003) ‘Not knowing – the problem of pain in children (2003) ‘Not knowing – the problem of pain in children with severe neurological impairment’ with severe neurological impairment’ International International

Journal of Nursing StudiesJournal of Nursing Studies; 40(2) pp171-183; 40(2) pp171-183

Qualitative study aiming to gain an Qualitative study aiming to gain an understanding of pain in children with understanding of pain in children with neurological diseaseneurological disease

Sources of pain Four main categories

* Pains associated with alterations in gut motility such as gastro-oesophageal reflux, wind and constipation.

* Pains related to musculo-skeletal problems, particularlymuscle spasm, dislocated hip, joint and back pain and pain

generally associated with the child’s immobility.

* Co-incidental pains that can also occur in otherwisewell children, for instance, ear and tooth ache.

* Pain related to poorly fitting aids and equipment.

Pain cues fell largely in to the following groups:

* 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

JamesJames

Assessment:Assessment:

QUEST approachQUEST approach Take your timeTake your time FlexibilityFlexibility

Possible causes:Possible causes:

Muscle spasmMuscle spasm Dislocated hipDislocated hip RefluxReflux ConstipationConstipation Tooth acheTooth ache Poorly fitting chair & Poorly fitting chair &

hoist.hoist.

Spasticity & Muscle spasmSpasticity & Muscle spasm CommonCommon

Spasticity is complexSpasticity is complex No single treatment modality is likely to be sufficient No single treatment modality is likely to be sufficient

alonealone New treatment methods in last decadeNew treatment methods in last decade Management needs to be specialized and individualizedManagement needs to be specialized and individualized Challenge: To find the best combination of methodsChallenge: To find the best combination of methods Requires co-operation across disciplinesRequires co-operation across disciplines Insufficient evidence for most.Insufficient evidence for most.

PAIN

SPASM

ConsiderationsConsiderations

Symptomatic - massage, heat, bathing etcSymptomatic - massage, heat, bathing etc Physiotherapy, splints, casting, Physiotherapy, splints, casting, Aids : seating etcAids : seating etc Drugs: Baclofen, Tizanidine, Dantrolene, Drugs: Baclofen, Tizanidine, Dantrolene,

benzodiazepines, clonidinebenzodiazepines, clonidine Botox ( reversible chemodenervation)Botox ( reversible chemodenervation) Intrathecal baclofen pumpsIntrathecal baclofen pumps Deep brain stimulationDeep brain stimulation Surgery - rhizotomy, NeurectomySurgery - rhizotomy, Neurectomy Hyperbaric oxygen? / Electrical stimulation?Hyperbaric oxygen? / Electrical stimulation?

James 2James 2

James’ pain improves considerably after a visit James’ pain improves considerably after a visit to the dentist, introduction of omeprazole and to the dentist, introduction of omeprazole and use of a new hoist and wheelchair.use of a new hoist and wheelchair.

However….. His mum is worried because he’s However….. His mum is worried because he’s not sleeping at night.not sleeping at night.

Why might this be? What help can you offer?Why might this be? What help can you offer?

SLEEP DISTURBANCESSLEEP DISTURBANCES

Profound impact on both children and families.Profound impact on both children and families. States of wakefulness are thought to be regulated by States of wakefulness are thought to be regulated by

diencephalic and brainstem nuclei and circadian diencephalic and brainstem nuclei and circadian rhythms. rhythms.

They require a normal suprachiasmatic nucleus of the They require a normal suprachiasmatic nucleus of the hypothalamus and connections. Therefore children with hypothalamus and connections. Therefore children with midline brain maldevelpment are at high risk of sleep midline brain maldevelpment are at high risk of sleep disorders. disorders.

Some portions of the cerebral hemispheres also Some portions of the cerebral hemispheres also contribute to sleep-wake cycles, because children with contribute to sleep-wake cycles, because children with hydranencephaly, lacking cerebral hemispheres, but hydranencephaly, lacking cerebral hemispheres, but having an intact brain stem and cerebellum also have having an intact brain stem and cerebellum also have profound sleep disturbances profound sleep disturbances

Factors affecting sleepFactors affecting sleep Sleep related breathing disorders can be associated with Sleep related breathing disorders can be associated with

anatomic abnormalities.anatomic abnormalities. SeizuresSeizures Visual impairment.Visual impairment. Those with LD may have difficulty in interpreting the social Those with LD may have difficulty in interpreting the social

cues families use to promote healthy sleep cyclescues families use to promote healthy sleep cycles Other symptoms impact on sleep – reflux, colic, hypoxia, Other symptoms impact on sleep – reflux, colic, hypoxia,

pulmonary oedema, muscle spasm, headaches, movement pulmonary oedema, muscle spasm, headaches, movement disorders.disorders.

Therapeutic drugs used in palliative care can disrupt normal Therapeutic drugs used in palliative care can disrupt normal sleep patterns – opiods, AEDs etcsleep patterns – opiods, AEDs etc

Hospitalization and episodic illness interferes with consistent Hospitalization and episodic illness interferes with consistent sleep because of disruption of routine.sleep because of disruption of routine.

Psychological stressors – fear of pain, fear of dark fear of Psychological stressors – fear of pain, fear of dark fear of death, separation anxiety.death, separation anxiety.

Negative associations with bed if linked with procedure Negative associations with bed if linked with procedure

TreatmentTreatment

Relies on assessing all the previous factors.Relies on assessing all the previous factors. A number of studies have documented the A number of studies have documented the

effectiveness of melatonin in reducing sleep effectiveness of melatonin in reducing sleep latency in many children with developmental latency in many children with developmental disorders. ( ¾ of children are able to fall asleep disorders. ( ¾ of children are able to fall asleep faster and may stay asleep longer)faster and may stay asleep longer)

Hypnotics are less satisfactory – short term Hypnotics are less satisfactory – short term therapy may be indicated.therapy may be indicated.

Teach and re-inforce basic principles of sleep Teach and re-inforce basic principles of sleep hygiene.hygiene.

Along with his mother you identify many issues Along with his mother you identify many issues that may affect his sleeping. Good sleep hygiene that may affect his sleeping. Good sleep hygiene measures alongside with Melatonin improve measures alongside with Melatonin improve things slightly.things slightly.

Mum wonders if his drooling and subsequent sore Mum wonders if his drooling and subsequent sore chin might be affecting his sleep. She tells you chin might be affecting his sleep. She tells you she changes his bib or clothing 10-15 x every day. she changes his bib or clothing 10-15 x every day. His chin is chapped and raw. She’s worried he’ll His chin is chapped and raw. She’s worried he’ll choke on his own Saliva.choke on his own Saliva.

How can you manage drooling?How can you manage drooling?

SIALORRHOEA (DROOLING)SIALORRHOEA (DROOLING)

‘ ‘ A loss of control over one’s own saliva’A loss of control over one’s own saliva’ Hypersalivation and ptyalism are sometimes used to Hypersalivation and ptyalism are sometimes used to

mean similar things.mean similar things. Secretions that pool in the hypopharynx and contribute Secretions that pool in the hypopharynx and contribute

to aspiration can cause choking, dysphagia and to aspiration can cause choking, dysphagia and breathing difficulties.breathing difficulties.

Sailorrhoea is a serious social handicap, it carries Sailorrhoea is a serious social handicap, it carries considerable social stigma, can interfere with considerable social stigma, can interfere with communication devices and is a barrier to interpersonal communication devices and is a barrier to interpersonal relationships.relationships.

Impacts on caregivers who may have to change the Impacts on caregivers who may have to change the child's clothing or bib 10-20 times / day.child's clothing or bib 10-20 times / day.

Unlikely to cause harm unless the body’s normal Unlikely to cause harm unless the body’s normal reflex coughing mechanisms are also impaired reflex coughing mechanisms are also impaired in which case it can lead to persistent micro-in which case it can lead to persistent micro-aspiration.aspiration. facial chappingfacial chapping Dental cariesDental caries Lip cracking and fissuresLip cracking and fissures

As many as 58% of children with cerebral palsy As many as 58% of children with cerebral palsy and 10% of children with other neurological and 10% of children with other neurological disorders are faced with severe sialorrhoeas that disorders are faced with severe sialorrhoeas that requires intervention.requires intervention.

Pathophysiology:-Pathophysiology:-

excess production of salivaexcess production of saliva Inability to retain saliva within the mouthInability to retain saliva within the mouth Problems with swallowing.Problems with swallowing.

Overproduction in the absence of Overproduction in the absence of swallowing impairment usually does not swallowing impairment usually does not cause sialorrhoeas cause sialorrhoeas

Management:-Management:-

Most treatments are directed at reducing the Most treatments are directed at reducing the volume of saliva produced.volume of saliva produced. BehavioralBehavioral PharmacologicalPharmacological Surgical interventions: should be considered in Surgical interventions: should be considered in

children with LLC but are not always appropriate.children with LLC but are not always appropriate.

Invasive techniques should be postponed until Invasive techniques should be postponed until after permanent dentition has appeared as after permanent dentition has appeared as sailorrhoea may become less of a problem after sailorrhoea may become less of a problem after this.this.

PHARMACOTHERAPYPHARMACOTHERAPY

Medications reduce saliva production and/or alter it’s Medications reduce saliva production and/or alter it’s consistencyconsistency

Anticholinergic drugs inhibit salivary secretion by Anticholinergic drugs inhibit salivary secretion by reversible blockade of the acetylcholine –mediated reversible blockade of the acetylcholine –mediated activation of muscarinic receptor – e.g. glycopyrolate and activation of muscarinic receptor – e.g. glycopyrolate and scopolamine ( hyoscine)scopolamine ( hyoscine)

Hyoscine can be inhaled, used orally or in transdermal Hyoscine can be inhaled, used orally or in transdermal systems - effectiveness diminishes with time.systems - effectiveness diminishes with time.

Glycopyrolate may have fewer adverse effects and better Glycopyrolate may have fewer adverse effects and better effectiveness. Still 20% discontinue it due to S/Eeffectiveness. Still 20% discontinue it due to S/E

Botulinum toxin A – injection into parotid and Botulinum toxin A – injection into parotid and submandibular glands, reliable and well tolerated. Lasts 3 submandibular glands, reliable and well tolerated. Lasts 3 – 8months– 8months

Surgery:-Surgery:-

First described in 1967. generally reserved for non-First described in 1967. generally reserved for non-progressive neurological disorders such as CP when progressive neurological disorders such as CP when response to medication is insufficient.response to medication is insufficient.

Either – reduce amount of saliva or divert the saliva more Either – reduce amount of saliva or divert the saliva more posteriorly.posteriorly.

remove glandremove gland ligate ductligate duct Section nerves involvedSection nerves involved

Results of all three have been disappointing.Results of all three have been disappointing.

Irradiation of submandibular & sublingual salivary glands: Irradiation of submandibular & sublingual salivary glands: Risk of secondary malignancyRisk of secondary malignancy

CautionsCautions Published literature and clinical observations suggest that Published literature and clinical observations suggest that

pharmacotherapy offers only short term solutions, often at pharmacotherapy offers only short term solutions, often at the cost of considerable side effectsthe cost of considerable side effects

Even surgical approaches seem to lose effectiveness with Even surgical approaches seem to lose effectiveness with time.time.

S/E: excessive dryness of mouth epithelium can S/E: excessive dryness of mouth epithelium can exacerbated existing swallowing difficulties and aggravate exacerbated existing swallowing difficulties and aggravate rate of resp infections and breathing difficulties.rate of resp infections and breathing difficulties.

Mucus producing respiratory glands are not regulated by Mucus producing respiratory glands are not regulated by any major nerve supply that can be blocked resulting in any major nerve supply that can be blocked resulting in thicker mucus as saliva volume diminishes. This can thicker mucus as saliva volume diminishes. This can accumulate in the back of the throat with a tendency to accumulate in the back of the throat with a tendency to block airways or make food stick in the throat.block airways or make food stick in the throat.

Need adequate fluid intake,Need adequate fluid intake, Reduce mucosal inflammationReduce mucosal inflammation Antihistamines and NSAID may helpAntihistamines and NSAID may help SuctionSuction Cough –assist devicesCough –assist devices

Alternative / additional measuresAlternative / additional measures

posture controlposture control dental hygienedental hygiene Address upper aero digestive inflammation Address upper aero digestive inflammation

or obstruction.or obstruction. oral motor therapyoral motor therapy behavior modificationsbehavior modifications BiofeedbackBiofeedback HypnotherapyHypnotherapy

James 3James 3

You start James on ¼ hyoscine patch and You start James on ¼ hyoscine patch and increase it up to ½ with good effect.increase it up to ½ with good effect.

You notice however that his feeding is You notice however that his feeding is becoming more difficult and he’s choking becoming more difficult and he’s choking more on his food.more on his food.

Do you want to intervene? What would Do you want to intervene? What would you suggest to mum?you suggest to mum?

Whilst you are arranging a video fluoroscopy Whilst you are arranging a video fluoroscopy and feeding assessment James aspirates and and feeding assessment James aspirates and needs PICU admission. After a 7 week needs PICU admission. After a 7 week admission he is discharged.admission he is discharged.

It has been suggested to mum that he should It has been suggested to mum that he should have a gastrostomy and fundoplication.have a gastrostomy and fundoplication.

You visit them at home to discuss it further You visit them at home to discuss it further …………..…………..

Whilst you are arranging a video fluoroscopy Whilst you are arranging a video fluoroscopy and feeding assessment James aspirates and and feeding assessment James aspirates and needs PICU admission. After a 7 week needs PICU admission. After a 7 week admission he is discharged.admission he is discharged.

It has been suggested to mum that he should It has been suggested to mum that he should have a gastrostomy and fundoplication.have a gastrostomy and fundoplication.

You visit them at home to discuss it further You visit them at home to discuss it further …………..…………..

Feeding in palliative care..Feeding in palliative care..

…….brings to mind artificial hydration / nutrition in .brings to mind artificial hydration / nutrition in the terminal stages but ethical dilemmas the terminal stages but ethical dilemmas precede thisprecede this

Consider Nasogastric tubes / gastrostomy tubes.Consider Nasogastric tubes / gastrostomy tubes.

Offering food to a child is one of the most basic Offering food to a child is one of the most basic of parental instincts and good nutrition can of parental instincts and good nutrition can improve Q.O.L for child and family.improve Q.O.L for child and family.

But.. artificial nutrition may impose burdens on But.. artificial nutrition may impose burdens on the child that outweigh the possible benefitsthe child that outweigh the possible benefits

Simple measuresSimple measures

- Look at position, seating, food offered etc- Look at position, seating, food offered etc- Unhurried, frequent, small , appropriate textured Unhurried, frequent, small , appropriate textured

meals.meals.- Consider feeding equipmentConsider feeding equipment- Oral desensitisationOral desensitisation- Calorie-dense additions e.g. cheese / creamCalorie-dense additions e.g. cheese / cream- Carbohydrate-providing glucose polymersCarbohydrate-providing glucose polymers- Treat reflux or oesophagitisTreat reflux or oesophagitis

Supporting families in decision Supporting families in decision making:-making:-

Decisions to increase oral supplements

Enteral / parenteral feeding

Issues of withdrawing / witholding artificial hydration or nutrition

Has to be multi-professional.. No individual should Has to be multi-professional.. No individual should make decisions alone.make decisions alone.

Supplemented by access to information from those Supplemented by access to information from those who know the family and child well.who know the family and child well.

Nutritional issues rarely occur in isolation; their Nutritional issues rarely occur in isolation; their impact on prognosis adds weight to the importance impact on prognosis adds weight to the importance of including the child in decision making if possible.of including the child in decision making if possible.

Assessment and consequent planning needs to be Assessment and consequent planning needs to be made for each child on an individual basis.made for each child on an individual basis.

GuidanceGuidance

RCPCH: Withholding or withdrawing life RCPCH: Withholding or withdrawing life sustaining treatment in children. A framework for sustaining treatment in children. A framework for practice. Second edition 2004.practice. Second edition 2004.

General medical council. Withholding and General medical council. Withholding and withdrawing life-prolonging treatments: good withdrawing life-prolonging treatments: good practice in decision making. London 2002practice in decision making. London 2002

BMA: Withholding and withdrawing life-BMA: Withholding and withdrawing life-prolonging treatment. Guidance for decision prolonging treatment. Guidance for decision making. BMJ, 3making. BMJ, 3rdrd edition London 2007 edition London 2007

RCPCHRCPCH

‘ ‘ the role of assisted feeding by NG tube or the role of assisted feeding by NG tube or gastrostomy should be considered very gastrostomy should be considered very carefully and discussed fully with the family’carefully and discussed fully with the family’

Emphasis placed on the inclusion of Emphasis placed on the inclusion of competent children in decision making.competent children in decision making.

Differentiates between the child with a Differentiates between the child with a neurodegenerative disease related neurodegenerative disease related swallowing disorder and a rapidly swallowing disorder and a rapidly progressive, disseminated malignancy.progressive, disseminated malignancy.

Managing the feeding of a child Managing the feeding of a child with a life-limiting illnesswith a life-limiting illness

Management begins at diagnosisManagement begins at diagnosis Includes multi-professional careIncludes multi-professional care Regular reassessment is vitalRegular reassessment is vital This in turn paves the way for possible decision-This in turn paves the way for possible decision-

making, at the appropriate time.making, at the appropriate time. Optimise oral food intake before embarking on Optimise oral food intake before embarking on

tube feeding.tube feeding. Include the family in decision making.Include the family in decision making. Consider the impact of home enteral feeding on Consider the impact of home enteral feeding on

the entire familythe entire family

James has several more chest infections requiring James has several more chest infections requiring hospital admissions during one of which NGT hospital admissions during one of which NGT feeds are started. He is felt to be too unwell for a feeds are started. He is felt to be too unwell for a general anaesthetic and plans for a gastrostomy general anaesthetic and plans for a gastrostomy are changed.are changed.

James gets very distressed on the ward and his James gets very distressed on the ward and his family are keen to keep him at home. His mother family are keen to keep him at home. His mother spends all her time on the ward with him leaving spends all her time on the ward with him leaving his father with the other children ( aged 6, 11 and his father with the other children ( aged 6, 11 and 14). He has needed to give up work to do this.14). He has needed to give up work to do this.

Discuss the impact of James’ ill health on the Discuss the impact of James’ ill health on the wider family……………….wider family……………….

Impact on family dynamicsImpact on family dynamics Parental separationParental separation Family separated : Other children don’t see Family separated : Other children don’t see

mum / Mum & James don’t see Dad and Siblingsmum / Mum & James don’t see Dad and Siblings What happens if James dies? Impact on siblings, What happens if James dies? Impact on siblings,

parents relationship, mum’s ‘job’parents relationship, mum’s ‘job’ What impact does nursing James at home have What impact does nursing James at home have

on the family? Storage of equipment / on the family? Storage of equipment / community nursing teams / home becomes a community nursing teams / home becomes a hospital.hospital.

Financial difficultiesFinancial difficulties Lack of time for ‘normal activities’…. E.g Lack of time for ‘normal activities’…. E.g

shopping / household chores.shopping / household chores.

SummarySummary Many children with Cerebral palsy have life-limiting or life threatening Many children with Cerebral palsy have life-limiting or life threatening

conditionsconditions Good symptom control is imperativeGood symptom control is imperative There can be challenges in assessing symptoms, particularly pain.There can be challenges in assessing symptoms, particularly pain. Symptoms include:-Symptoms include:-

Pain from multiple causesPain from multiple causes ConstipationConstipation Spasticity & muscle spasmsSpasticity & muscle spasms Feeding difficulties and aspirationFeeding difficulties and aspiration Convulsions & movement disordersConvulsions & movement disorders Behavioural issues and sleep disorders Behavioural issues and sleep disorders

Families need to be involvedFamilies need to be involved All symptoms need to be considered alongside psycho-social issues All symptoms need to be considered alongside psycho-social issues

that the child and family facethat the child and family face Planning for critical illness and end of life care is welcomed by families.Planning for critical illness and end of life care is welcomed by families.