Session 10: Learning outcomes. Adolescent Palliative Care 9/1/17

Transcription

1 Learning outcomes Session 10: Adolescent Palliative Care By the end of the session participants should: Be able to discuss the effect of a life limiting illness on adolescence. Be able to identify the range of life limiting conditions that occur in adolescence. Recognise the range of responses of adolescent to illness. Recognise the range of responses of the family to adolescent illness. Be able to discuss end-of-life care in adolescents. 2 Life Limiting conditions - Adolescence List of underlying conditions of patients aged >16 years on 01/01/2014 at Martin Category 1: Life threatening may be curable Category 2: Life threatening may not be curable Category 3: Life threatening no treatment Category 4: Life limiting catastrophic event causing progressive complications. 3 4 Number Tasks of Adolescence Tasks of Adolescence A developmental task is a task that arises at or about a certain period in life, unsuccessful achievement of which leads to inability to perform tasks associated with the next period or stage in life. Havighurst R 1930 s 1. Learning to get along with friends of both sexes 2. Accepting one s physical body and keeping it healthy 3. Becoming more self-sufficient 4. Making decisions about marriage and family life 5. Preparing for a job or career 6. Acquiring a set of values to guide behaviour 7. Becoming socially responsible Havighurst R

2 Tasks of Adolescence Age of Adolescence? 1. To learn to look upon girls as women and boys as men; to become an adult among adults; to learn to work with others for a common purpose, disregarding personal feelings; to lead without dominating. 2. To accept one s body, to keep it healthy through good nutrition, exercise, disease prevention, and other health practices 3. To develop affection for parents without dependence upon them; to develop respect for older adults without dependence upon them 4. To explore attitudes towards family life and having children; to acquire the knowledge necessary for home management and, if desired, child rearing. 5. To develop career vocational goals and ways to achieve these goals; to be able to make a living 6. To develop an outlook towards life, based on what is important. 7. To participate as a responsible person with friends at home, and in the community; to develop personal moral values to guide behaviour. Havighurst R 1930 s Suggested Havighurst R 1930 s 7 8 Adolescence On Time events (i.e. with peers) Or Off Time events Can t cure the underlying condition (probably) Don t make the situation worse It s a complex situation so take time (1 long visit) (several short visits) Ask lots of questions they are the experts in them Offer and discuss options i.e. - child birth - dying Give me three goes and then we ll find a proper doctor! Employ a teenager while they still know everything! 9 10 Management of complex health needs Cerebral Palsy as an example of an underlying disorder that is non-progressive. Spiritual, Psychological Person Behavioural Symptoms Pain Respiratory Cardiac Gastrointestinal Muscular Skeletal Renal, Liver Underlying Disorder What is the person and what is the disease? Are they separable? 11 Complex care and condition not initially recognised as life limiting. Prognosis already passed probably more than once! Mental capacity hard to establish. 12 2

3 Management of complex health needs 23 year old with cerebral palsy. Living at home. Mother and home care. Beginning to use a communicator. (using eye pointing) Four limb quadriplegia, fed orally with a gastrostomy as back up. Rare epileptic fits Admitted with pneumonia - aspiration (acute on chronic) weaned off invasive ventilation then relapsed Tracheostomy considered but decided against. Admitted to Hospice for (?terminal) care. History Any problems now? previous schooling previous health care - Access to a specialist clinic? - Membership of a patient support group - Who are the main carers - What interests do you have? Examination - Musculoskeletal - chest - heart - abdomen If it s not a problem now, no need to tackle it now (i.e. hypotension ) Natural History: wearing out (?effect of brain damage) usually gradually worsening respiratory function gastrointestinal failure rare but becoming more common. Life long control of posture? - Lycra suits - spinal rods - rhizotomy - extensive surgery Medication: - GABA inhibitor - baclofen - benzodiazepine - anticonvulsants NB: Look for other irritants Symptoms: 1. Spasms - painful, exhausting - worse when upset or unwell 2. Dislocated hips 3. Scoliosis - painful sitting posture - gastrointestinal problems - back pain Natural History eating and swallowing slowly deteriorate aspiration more common than thought calorie intake high and can be difficult to achieve important to prevent constipation Will need oral feeding -? Access to care food easy to chew, easy to swallow Can be very thin, rarely overweight. Calorie intake individual Routes of Enteral feeding - gastrostomy - trans gastrostomy jejunal feeding - jejunostomy (a. direct through stomach or b. Rouxen-y) Beware of silent aspiration. Wind (swallowed air) Arising issue of Gut failure (failure of peristalsis not absorption) Treatment - manage constipation NB: does gastrostomy lead eventually to gut failure? 17 Parenteral (Intravenous) feeding short term (4 weeks?) only 18 3

4 Natural History: - Lungs chronic infection, aspiration - respiratory wall normal but unco-ordinated - lung volume reduced by scoliosis O 2 uptake reduced CO 2 retention NB. Long term need for oxygen following chest infections is a poor prognostic sign 1. Antibiotics short term for infections What is the role for prophylactic antibiotics? enteral nebulised NB: Pseudomonas has been selected for Consider use of oral azithromycin nebulised colomycin (??Tobramycin) When to start? - How to stop? Oxygen - as needed - don t go more than one concentrator Cough Assist: Stiff chest wall, reflex resistance to inflation so not recommended. Natural History : - Cardiac function normal - blood pressure may be labile - compromised local circulation 4. Non invasive ventilation. Probably won t be tolerated. Discuss option but to discount it. NB. don t anticoagulate 5. What is the place of home monitoring of Oxygen saturation? Check List For Pain Spiritual Gut related - Gastro-oesophageal reflux disease - Constipation - wind Approach as person first (Sport, Computer games, family) Renal or biliary stones Dislocated hips Micro fracture of any bone Severe deficiency - iron - vitamin C - vitamin D blood pressure may be labile Trapped nerve 23 Use of humour Always work to increase the options and opportunities (even if some of them won t be wanted!) Death occurs when you run out of options. 24 4

5 Advanced Care Plan Cerebral Palsy Advanced Care Plan Cerebral Palsy Establish understanding of patient. (Use carers) Establish understanding and fears of family. Establish understanding and fears of extended family. Explain level of health of patient (Respiratory function often over estimated) Explain when decisions will need to be made (only irrevocable when finally HAVE to be made) Establish level of care that will be given. Levels of care: Home based care. Ward based care Respiratory support Intensive Care - oral or IV (once daily) antibiotics - oxygen, suction, chest physiotherapy - subcutaneous medication - as above - IV antibiotics more than once daily + non-invasive ventilation (temporary) + naso-gastric feeding - Invasive ventilation - +/- tracheostomy - +/- parenteral feeding 25 The timing of death on intensive care units is often decided by everyone except the patient 26 Advanced Care Plan Cerebral Palsy Resuscitation: Life threatening events should be predictable. If occur, probably cannot be fully avoided. Only if there is a quick response is any resuscitation likely to be successful. Therefore Invasive resuscitation should not be given - intubation - cardiac stimulants Musculoskeletal System Muscular Dystrophy Natural History: gradually increasing weakness not under control apparent long periods of stability SCOLIOSIS an issue in early teens Fixed flexion deformities No veins, poor circulation, feel the cold. A traumatic resuscitation could be considered an assault Musculoskeletal System Muscular Dystrophy Musculoskeletal System Muscular Dystrophy Scoliosis spinal rods unlikely after teens Good wheel chair support Fixed flexion deformity physiotherapy (different package from paediatrics) Mobility electric chairs are great.?use of standing frames/ standing chairs Tilt in space chairs Mobility CHARGE CHAIRS EACH NIGHT!! positioning, positioning, positioning Moving and Handling Sling and hoist (?)2 carers at least

6 Gastrointestinal System Muscular Dystrophy Respiratory System Muscular Dystrophy Natural History - eating and swallowing well maintained. - important to prevent constipation May need oral feeding -? Access to care food easy to chew, easy to swallow Can be very thin, or very overweight. Calorie intake individual Treatment - manage constipation NB: use of gastrostomy may indicate shorter prognosis Natural History: Headaches Sleepiness NB. Complicated by scoliosis - Lungs normal, respiratory wall weak CO 2 retention O 2 adequate for low requirements Respiratory System Muscular Dystrophy Respiratory System Muscular Dystrophy 1. Ventilatory Support - Non invasive ventilation use overnight - Elective tracheostomy use overnight When to start? - How to stop? - ensure good links with respiratory support team (Sleep service) as long term treatment will be stable on this Prevents chest infections, prolongs life. NB. This is an option. NIPPV video Cough Assist: - Non invasive inflation then rapid expiration effective for clearing weak chests check settings Mechanical devices are relatively expensive 3. Breath stacking devices cheaper NB. This is an option. 34 Cardiovascular System Muscular Dystrophy Other issues Muscular Dystrophy Natural History : - Abnormal ECG - increased risk of arrhythmias (variable) - weak cardiac muscle (variable) - usually asymptomatic hypotension - regular (?yearly) cardiac review - use of β blockers and angiotensin receptor blocker NB. don t anti-coagulate NB. Mother may be have some symptoms (X inactivation) 1. Ear wax 2. Vertigo 3. Skin integrity 4. Upper airway irritation 5. Constipation

7 Spiritual Muscular Dystrophy Is the long term - education, employment a priority? OR Is the short term comfort, family the priority? Perspective: - personal sense of how finite your time on this world is. Advanced Care Plan Muscular Dystrophy Difficult to approach until has had a chest infection. Most want everything done. You would admit any other 17 year old to intensive care why not me? Discuss possible options is tracheostomy (permanent or temporary) acceptable is gastrostomy acceptable Use of humour, discuss the technical options ( What dreadful things doctors can do to you to try and keep you alive ) Accept that the future is unknown. Don t feel sorry for him having muscular dystrophy, but do feel sorry for him supporting Manchester United! Advanced Care Plan Muscular Dystrophy Future: Sudden cardio-respiratory arrest likely after a warning episode after a chest infection So likely to be in Hospital. Risk of a long stay on a Respiratory Unit and then dying before discharge Understand the underlying pathology. Consider the effect on each organ. Consider the effect on the individual. Join in the patient s journey trying to expand opportunities. Recognise the future uncertainties. Never know which is the last chest infection Thank You! 41 7