Complex Care & Palliation Readings

Week 1 - Caring for Persons with Complex & Life Limiting Illness

Quality of life - This concept includes aspects related to the wellbeing of patients. It is more of a personal perception than an objective entity. Improvement in life expectancy is not always linked to an improvement in quality of life. The aim of treatment is not only to remove the disease or relieve symptoms, but to improve quality of life as well, considering the patient’s needs more comprehensively.

Palliative care - Helps people with life-limiting illnesses live as fully and comfortably as they can. It is for people of any age and can be provided in homes, hospitals, hospice facilities or nursing homes.

There are 3 decline trajectories in people with advanced illnesses - rapid decline (cancer), intermittent decline (organ failure), and gradual decline (dementia).

Week 2 - Caring for the Child with a life-limiting illness

Paediatric Palliative Care National Action Plan - Provides guidance and direction on key priorities and actions to improve outcomes for children, young people, and their families living with life-limiting conditions.

• Priority 1: Quality - Facilitate active involvement of the child and their family in their care coordination, designate a care coordinator to support the family unit to receive integrated care from healthcare services, initiate appropriate advance care planning discussions and enable chances for change to be made over time, support alignment with end of life guidelines, implement guidelines for best-practice, holistic care to be delivered to the child and family by healthcare professionals.

• Priority 2: Access - Develop and implement a palliative care pathway including a transition pathway from paediatric to adult palliative care, develop culturally safe pathways for palliative care delivery, develop a national paediatric palliative care workforce strategy, deliver comprehensive and multidisciplinary specialist care across all services, expand to community based capability, explain an outreach education model to support family units in remote and regional areas to access services, up-skill and train healthcare workers to deliver quality care.

• Priority 3: Information sharing/collaboration - Routinely access and discuss relevant health information to children and their families, develop targeted community awareness strategies to build knowledge about issues supporting children and their families accessing palliative care, implement the paediatric palliative care national action plan.

• Priority 4: Data/research - Undertake a focused prevalence study, develop outcome measures and implement benchmarking for paediatric care, identify priorities in research and support research components where a collaborative approach to data collection is warranted.

Palliative care for children - Active total care of the child’s body, mind, and spirit, involves familial support, begins when an illness is diagnosed and continues regardless of whether or not a child receives curative treatment.

• Attributes - Care embraces the whole family, increased familial decision making role, child remains under the care of their primary treating team, care is provided across a wide range of settings, children learn, grow, and develop towards becoming adults even if under palliative care so their emotional, social and spiritual needs will change throughout care, their communication and comprehension abilities will change, education and play therapies are used, siblings and grandparents are vulnerable to reduced psychological wellbeing, conditions can be genetic, and the death of a child can lead to prolonged or lifelong grieving as well.

Palliative care settings:

1. Community - Homes, childcare centers, special/mainstream schools, accommodation for people with disabilities, GP clinics, community palliative care clinics/day centers, paediatric hospices.

2. Hospital based - Critical care units, inpatient palliative care units, other inpatient beds like acute paediatric wards, outpatient services, specialist rooms, ambulatory clinics, emergency departments.

Essential elements for safe and high-quality paediatric end of life care:

1. Processes of care - Relates to the ways paediatric end of life care should be approached and delivered, directed mainly to clinicians working in settings where acute palliative care is provided to children and families.

   • Family centred care (involved in decision-making at all stages)

   • Teamwork (clinicians adopt good teamwork to coordinate care)

   • Goals of care (clear goals are made to improve the quality of end of life care)

   • Using triggers (identifies when children need end of life care)

   • Responding to concerns (clinicians escalate to rapidly respond to the suffering of a child and their family)

2. Organisational pre-requisites - Relates to structural and organisational pre-requisites for effective delivery of safe and high quality end of life care, directed mainly to health service managers and executives who work in health services that provide palliative care to family units.

   • Leadership and governance (appropriate policies/systems in place for end of life care)

   • Education and training (teach clinicians the skills/knowledge to provide quality end of life care)

   • Supervision and support (support clinicians providing care)

   • Evaluation and feedback (measure and improve quality of end of life care provided to family units)

   • Supporting systems (systems to align with NSQHS standards)

Pain assessments - Acute pain (nociception) is associated with tissue damage and an inflammatory response, self limiting of short duration, not involving neural tissue. Pain is multidimensional, so assessments must include intensity, location, duration, description, impact on daily activities, and other factors that may influence the child’s perception of pain (eg. social history/issues, cultural or religious beliefs, past pain experiences, first pain experience, family response). Pain may be self-reported, behavioural, or physiological (clinical observations).

FLACC (2mo - 8yrs) - Face, legs, activity, cry, consolability, each scored on a 0-2 scale adding up to a total score between 0-10. Obtained through observation.

Wong Baker (8yrs) - Visual analogue scale, ask the child to choose the face to best describe what they are feeling, clinicians can also use the faces to estimate the child’s pain score if they are unable to communicate or comprehend the tool.

Physiological indicators - HR increase, RR and pattern may shift from normal, BP increase, SpO2 decrease.

Children’s’ understanding of death:

• Babies - Experience a sense of abandonment which can cause increased crying, disrupted sleep/feeding.

• Under 2 years - No understanding of death, can react to emotional withdrawal from other parent/carer, may search for the person who has died and will need to be reminded that they aren’t coming back. Give cuddles and reassurance and stick to normal routines.

• 2-5 years - Starts to use the word “death” and recognises the difference between being dead and alive, unaware of death’s eternity, still expecting them to return. They may experience separation anxiety, altered sleep and appetite, altered willingness to play may regress in language and toilet training, repeating questions frequently. Give honest answers and be patient with them, giving them small bits of information at a time.

• Primary school age (5-7) - Begins to understand death’s eternity, that it happens to all living things, may become anxious about the prospect of their own death, may think it’s their fault someone died, will need space to ask questions and be told it’s not their fault, be honest and build on their understanding with small bits of information at a time.

• Teenagers - Grief impacts on process of moving to independence, can feel isolating as this experience is different to that of many of their friends, may become withdrawn or act out their distress with risk-taking behaviours, may have an increased need to care for others around them, give them space to still be teenagers and not take on too much responsibility. May seek peer support rather than from families. Keep to your agreed boundaries for usual behaviour to help with stability. They will revisit their grief as their understanding grows over time, meaning they’ll go through periods of worse emotional stability as they realise the implications of the death for the future.

Week 3 - Communication and symptom control

Ascending pain pathway (transmits pain to the brain):

• When tissues are damaged, prostaglandins are produced usually as a swelling response. Sensory nerve fibres respond to prostaglandins and carry the impulse to the dorsal horn of the spinal cord (1st order neuron). Substance P is an important chemical released by the 1st order neuron in the dorsal horn to transmit the impulse to the 2nd order neuron.

• The 1st order neuron synapses and relays the impulse to the 2nd order neuron (crosses over to the other side of the spinal cord to enter the spinothalamic tract), the impulse makes its way to the thalamus.

• In the thalamus, the 2nd order neuron synapses to relay the impulse to the 3rd order neuron in the somatosensory cortex which will bring the impulse to the area of the brain that correlates with the location of the injury to allow for the perception of pain.

Descending pain pathway (controls/inhibits the ascending pathway):

• When not inhibited, the neurons arising from the peri-aqueductal gray matter of the midbrain go down to the nucleus raphe magnus of the medulla and synapse with the 2nd order neuron, continuing to travel down to the dorsal horn of the spinal cord. Its role is to inhibit or control the communication between the 1st and 2nd order neurons of the ascending pathway to help control pain signals going up to the brain.

Gate control theory of pain:

• 1st order neuron enters the dorsal horn (can be called the substantia gelatinosa) and brings in an action potential that simulates vesicles to release their contents (Substance P) into the synaptic cleft.

• Substance P stimulates the 2nd order neuron which will propagate an impulse up to the thalamus through the spinothalamic tract.

• In the descending pathway, the serotonin noradrenaline neuron from the medulla releases both serotonin and noradrenaline. They bind to receptors of the pre-synaptic neuron to inhibit the release of Substance P and they stimulate a small neuron called an intra-neuron in the substantia gelatinosa (opioid neuron), causing it to release an endogenous opioid called encephalin. Encephalin inhibits the pre-synaptic neuron from releasing Substance P, and it inhibits the post-synaptic neuron from depolarizing (stops the continuation of the impulse up to the thalamus), therefore inhibiting the ascending pain pathway

Opioid mechanism of action:

• Opioid receptors are found in the brain and the dorsal horn of the spinal cord. They mediate the analgesic effects and side effects of opioids.

• Mu receptors are responsible for most opioid effects, including analgesia, euphoria, sedation, respiratory depression and decreased gut peristalsis causing constipation.

• Delta receptors have a hallucinogenic effect, located in the gut, causing decreased gastrointestinal secretions.

• Kappa receptors mediate dysphoria by reducing dopamine release.

• Pain is transmitted from the 1st to the 2nd order neuron in the substantia gelatinosa (dorsal horn) of the spinal cord through excitatory neurotransmitters like Substance P and glutamate. These substances are released from the 1st neuron and bind to receptors on the 2nd order neuron to cause excitatory post-synaptic potentials

• Activation of opioid mu receptors in the dorsal horn cause closure of calcium channels leading to a decreased release of excitatory neurotransmitters like Substance P and glutamate from the 1st order neuron (and therefore less excitation of the 2nd order neuron), decreasing the number of pain signals that are sent to the brain.

• Opioids also bind to mu receptors in the membrane of the cell body or dendrites of secondary neurons, which opens potassium channels, causes hyper-polarization of the 2nd order neuron, and a decrease in action potential frequency (less pain signals are sent to the brain).

Pain reliever mechanism of action:

• Nociceptors (nerve cells that detect pain) - They only fire if something happens that could cause or does cause harm to your body.

• Tuning chemicals (prostaglandins) released by damaged cells lower nociceptor thresholds for pain, so that even simple actions that wouldn’t usually cause pain, now do cause pain as the body heals from an event.

• Painkillers block the production of prostaglandins

• When cells are damaged, they release arachidonic acid, then 2 enzymes (Cox-1 and Cox 2) convert this acid into prostaglandin H2 which is then converted into other chemicals that raise your temperature, cause inflammation, and lower the pain threshold.

• Painkillers work at the active sites of Cox-1 and Cox-2 (where arachidonic acid would bind). Aspirin works like a spike; it penetrates at the active site and part of the spike breaks off and part is left behind at the active site so that arachidonic acid can’t bind

• Ibuprofen just sits in the active site of Cox-1 and Cox-2, and while it is there, arachidonic acid can’t bind. The enzymes can remove it from the active site, but as long as the medication’s half life hasn’t yet been reached, ibuprofen will continue to bind at the active site.

Physiology of vomiting:

• The vomiting center is in the medulla oblongata, contains muscarinic receptors which, when stimulated, triggers the vomiting reflex. The chemo-receptor trigger zone (CTZ) near the medulla oblongata contains the dopamine-2 receptors and the serotonin receptors. When the CTZ is stimulated, it stimulates the muscarinic receptors of the vomiting center to cause vomiting to occur.

• The BBB prevents circulating substances in the blood from making contact with the brain and brainstem.

• The CTZ is located outside the BBB (more permeable to circulating substances like cytotoxic agents)

• Motion sickness comes from the labyrinth in the inner ear (bony structure). It’s made up of many areas, one being the vestibule which is important for balance and space. Problems here send signals to the brainstem via the vestibular cochlear nerve, specifically to the vestibular nuclei (in the pons). This nuclei contains histamine 1 and muscarinic receptors. When the nuclei is stimulated in motion/morning sickness, these signals are passed to the CTZ which sends it to the vomiting center

• If emotionally overwhelmed, in severe pain, or they smell/see something really bad, the brain processes this information in its higher centers. The signals from higher brain centers travel down to the vomiting center

• Entero-chromaffin cells in the stomach release serotonin in response to cytotoxic agents which stimulate serotonin receptors on vagal nerve fibres. This stimulus is sent to the vomiting center to trigger vomiting

• When vomiting, the lower oesophageal sphincter relaxes, the diaphragm and abdominal muscles contract to help push food back up. Patients experience tachycardia and increased salivation and peristalsis. The epiglottis also closes to avoid food entering the trachea

Breaking bad news (PLIIE) - Bad news is any information adversely/seriously affecting a person’s view of their future. The PLIIE approach can help minimise the distress of relatives and maintain a therapeutic relationship as well.

• Prepare - Who should be there, know details well, plan the meeting, seek advice if needed, talk to other staff members beforehand, consider what you want to get across and how you are going to do it

• Location - Ensure it’s private, comfortable, appropriate, no distractions, set aside enough time, support people involved if needed

• Introduction - Give your name and role, introduce other staff members, ask family to introduce themselves, use appropriate verbal and body language, find out what the family know already, warn about bad news before it is broken to them

• Information - Use clear and simple language, give information in small bites, no medical jargon, tailor information to the specific family, monitor the pace of information, allow for reflection and silence, listen to them, allow for discussion, convey empathy/respect, check understanding, elicit any concerns, open disclosure of information

• End - Answer all questions, offer support, arrange follow-ups, document

Grief in children:

• They want to be told the truth (may need clear explanations to many questions as they come to an understanding of the situation)

• They want to be reassured that they are cared for (may become clingy and a physical sense of closeness is important for them)

• Embrace the pain of grief (don’t spare them the pain of loss because they will experience the loss and learn that mourning is not right)

• Children manage grief and loss through play which may seem like they are avoiding their grief or not processing all of those emotions

• Acting out is a way of expressing grief (adults need to nurture environments where the child feels safe to express their feelings)

• Sharing stories and memories is important to acknowledge the reality of the loss and continue bonds with the person they have lost

• Grief is unique, some may be expressive and some may not be, siblings will grieve differently and their timelines will be different as well

Week 4 - Care of the Frail, Older Person

Alzheimer’s Disease - Physical brain condition resulting in impaired memory, thinking, and behaviour where one’s abilities deteriorate with time. It causes neuron damage as a plaque of proteins forms on top of them, and other proteins tangle inside the neuron. They can’t communicate with each other as well as before, eventually dying and shrinking brain volume as a result. Risk factors include physical inactivity, lack of mental exercise, smoking, obesity, diabetes, high cholesterol, family history, down syndrome, genetics, and hypertension. Short term memory is affected first but long term memory is affected later too.

Symptoms of Alzheimer’s Disease - Memory lapses, difficulty word finding, forgetting well known people or places, social skill deterioration, loss of enthusiasm for previously enjoyed activity, emotional unpredictability.

Fronto-temporal dementia - Causes progressive damage to either/both the frontal and temporal lobes of the brain. There are 4 types: The behavioural variant, primary progressive aphasia, semantic dementia, and progressive non-fluent aphasia. It usually affects people between 45 and 65 and is incurable, with varied symptoms depending on the type. Changes mainly occur to behaviour, personality, language and movement, with memory only being affected at late stages. Many people have a family history or genetic predisposition to it. Therapies include counselling, occupational therapy, and speech therapy.

• Behavioural variant - Frontal lobe affected first, experiencing personality and behaviour changes as areas controlling conduct, judgement, empathy, and foresight are damaged.

• Primary progressive aphasia - Temporal lobe affected first, experiencing loss of language skills (early), loss of thinking/perception/behaviour (late)

• Progressive non-fluent aphasia - Developed problems speaking, with individuals losing their ability to speak fluently over time.

• Semantic dementia - Loss of ability to assign meaning to words, word-finding issues, can’t name people/objects, behaviour changes, obsession with routines and emotional responses. Most people can do daily activities until late stages, comprehension/expression not really affected.

• Logopenic aphasia - Retained knowledge of word meanings, hard to word-find, takes longer to say things overall, struggling to repeat things that you or someone else just said, long sentences are harder to read.

Lewy Body Dementia - An umbrella term describing both dementia with Lewy Bodies and Parkinson’s disease. Lewy Body dementia causes changes in thinking, movement, behaviour and bodily function. Lewy Bodies are tiny tangles of proteins called alpha-synuclein in brain cells, not understood why they form.

• The most common movement symptoms of Parkinson’s disease are tremors at rest, rigidity or muscle stiffness, bradykinesia, trouble maintaining posture and balance, with symptoms starting on one side before spreading to the other over time. Dementia with Lewy Bodies causes less tremor, more torso stiffness, same balance problems, symptoms occurring on both sides of the body.

Symptoms of Lewy Body Dementia - Lewy bodies deposit in cells in areas of the brain involved in thinking, memory, and movement. Symptoms include visual hallucinations, rigid muscles, slow movement, walking difficulty, and tremors similar to Parkinson’s disease.

Vascular dementia - Damage from restricted bloodflow to the brain, affecting reasoning, planning, judgement, attention and how you function, usually occurring alongside Alzheimer’s disease, and is incurable. Risk factors include hypertension, high cholesterol, diabetes, obesity, smoking, poor diet, physical inactivity, arrhythmias, heart disease, blood vessel disease, large/many strokes. Treat by preventing more strokes, medication use for high blood pressure, high cholesterol, heart disease, diabetes, Alzheimer’s disease, anticoagulants, healthy diet, no smoking, occupational therapy, and counselling support.

• Strategic infarct dementia - Single large stroke can cause vascular dementia depending on its size and location, causing sudden thinking and behaviour changes. If no further strokes, symptoms can stabilise or improve with time. More strokes/vascular diseases worsens symptoms.

• Multi infarct dementia - Caused by multiple strokes. Over time, thinking and functioning are affected, possible depression and mood swings. After each stroke, symptoms worsen and then stabilise for a while.

• Subcortical vascular dementia - When blood vessel disease causes damage deep in the brain, symptoms worsen as damage occurs, causing behavioural changes, inability to control bladder, reduced ability to plan, problem solve, organise, think logically, forgetting things, repeating things, trouble walking or moving.

Arthritis - Group of conditions affecting the joints, over 100 forms of arthritis, some even involving the eyes and skin. Most common forms are osteoarthritis, rheumatoid arthritis, gout, and ankylosing spondylitis. Most types are chronic disorders which are incurable.

• Symptoms include pain (caused by inflammation, joint damage, or muscle tension), stiffness, fatigue, heightened emotions (fear, pain, loss of independence/self-esteem, stress) and more.

• Management includes medications (to relieve pain/swelling, suppress the immune system and reduce joint damage), diet changes, exercise, good pain management (heat/cold packs, massage, relaxation/distract)

Osteoarthritis - Most common form, affects all tissues of the joint including cartilage, the bones themselves, and surrounding tissue like tendons, ligaments, and muscles. Risk factors include being female, older than 45, prior injury in the joint, family history, obesity, participating in sports/jobs with repetitive/excessive joint stress. It is diagnosed using 3 types of information; your symptoms/health history, a physical exam, and medical imaging.

Rheumatoid arthritis - An autoimmune disease where the immune system attacks the tissues, joints, and other organs like the heart, lungs, eyes, and muscles, whereas osteoarthritis only affects the joints. Rheumatoid arthritis tends to cause symptoms in the same joints on both sides of the body (eg. both knees) but osteoarthritis usually involves joint pain on one side but not the other.

Polypharmacy - When people use 5 or more medications at the same time which can increase their risk of medicine-related harm. The more clinicians a person goes to, the less likely that one clinician will have oversight over all their medications. Encourage older people to keep an up to date medication list.

Aging in place - Home based healthcare includes help with ADLs, household chores, meals, money management, healthcare, transportation, safety and more. Ensure to make your home safer by identifying hazards and changes that will impact your ability to move around your home as your mobility changes. Ensure to stay in contact with your social network to avoid isolation, learn about community resources and activities, plan for medical emergencies