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Symptoms in non-malignant paediatric palliative care I-Cerebral palsy PowerPoint Presentation

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Description : Check out this medical PowerPoint presentation titled "Symptoms in non-malignant paediatric palliative care I: Cerebral palsy" by Dr Jo Griffiths.
This medical PowerPoint presentation talks about the symptoms experienced by children with cerebral palsy that may require palliative care.

Tags : non-malignant paediatric palliative care, Cerebral palsy, Child Neurology PowerPoint presentation, Neurology PowerPoint presentation

Published on : Mar 21, 2023 | Views : 784 | Downloads : 0

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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
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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 Life-Threatening 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). 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). 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) 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).
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Learning objectives 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.
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Cerebral palsy 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 Prevalence Abnormalities of speech, 1.5-2.5 / 1000 live births Abnormalities of vision Learning difficulties. 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
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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.
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Main symptoms in life limiting cerebral palsy:- 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
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Hunt et al; Medical and nursing problems of children with neurodegenerative disease. Palliat Med 1995;9 :19-26 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.
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Case 1 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
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1. How are you going to assess his pain 2. What could be the cause or causes of his pain?
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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
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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, particularly muscle spasm, dislocated hip, joint and back pain and pain generally associated with the child’s immobility. * Co-incidental pains that can also occur in otherwise well children, for instance, ear and tooth ache. * Pain related to poorly fitting aids and equipment.
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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.
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knowledge required for comprehensive pain assessment and management. Know the child Know the Science Know the population
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James Assessment: QUEST approach Take your time Flexibility Possible causes: Muscle spasm Dislocated hip Reflux Constipation Tooth ache Poorly fitting chair & hoist.
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Spasticity & Muscle spasm Common 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.
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Considerations 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?
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James 2 James’ pain improves considerably after a visit to the dentist, introduction of omeprazole and use of a new hoist and wheelchair. However….. His mum is worried because he’s not sleeping at night. Why might this be? What help can you offer?
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SLEEP DISTURBANCES 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
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Factors affecting sleep 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
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Treatment 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. 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. How can you manage drooling?
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SIALORRHOEA (DROOLING) ‘ 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 micro-aspiration. 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.
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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
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Management:- Most treatments are directed at reducing the volume of saliva produced. 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.
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PHARMACOTHERAPY 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
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Surgery:- First described in 1967. generally reserved for non-progressive neurological disorders such as CP when response to medication is insufficient. Either – reduce amount of saliva or divert the saliva more posteriorly. remove gland ligate duct Section nerves involved Results of all three have been disappointing. Irradiation of submandibular & sublingual salivary glands: Risk of secondary malignancy
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Cautions 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. Need adequate fluid intake, Reduce mucosal inflammation Antihistamines and NSAID may help Suction Cough –assist devices
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Alternative / additional measures posture control dental hygiene Address upper aero digestive inflammation or obstruction. oral motor therapy behavior modifications Biofeedback Hypnotherapy
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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?
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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 …………..
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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 …………..
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Feeding in palliative care.. ….brings to mind artificial hydration / nutrition in the terminal stages but ethical dilemmas precede this Consider Nasogastric tubes / gastrostomy tubes. 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
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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
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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. Supplemented by access to information from those who know the family and child well. Nutritional issues rarely occur in isolation; their impact on prognosis adds weight to the importance of including the child in decision making if possible. Assessment and consequent planning needs to be made for each child on an individual basis.
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Guidance 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 life-prolonging treatment. Guidance for decision making. BMJ, 3rd edition London 2007
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RCPCH ‘ 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.
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Managing the feeding of a child with a life-limiting illness Management begins at diagnosis Includes multi-professional care Regular reassessment is vital This in turn paves the way for possible decision-making, 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. 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. 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.
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Summary 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:- 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.