Palliative Care for the Medically Complex Child Supplementary cases Melanie Anspacher, MD Neha Shah, MD

Case 1 Develops recurrent ileus and inability to tolerate feeding with each acute illness. 12 year old girl with history of traumatic brain injury Spastic cerebral palsy Developmental delay Seizures Gastrostomy tube fed Tracheostomy Crises

Case 1 Pain with enteral feeding Difficult to manage other symptoms Problem refractory to maximized medical management Quality of life perceived to be poor by family 12 year old girl with history of traumatic brain injury Spastic cerebral palsy Developmental delay Seizures Gastrostomy tube fed Tracheostomy Recurrent ileus, unable to tolerate enteral feeds

Case 1 Burden Benefit 12 year old girl with history of traumatic brain injury Spastic cerebral palsy Developmental delay Seizures Gastrostomy tube fed Tracheostomy Recurrent ileus, unable to tolerate enteral feeds

? Case 1 Withholding Withdrawing 12 year old girl with history of traumatic brain injury Spastic cerebral palsy Developmental delay Seizures Gastrostomy tube fed Tracheostomy Recurrent ileus, unable to tolerate enteral feeds Withdrawing

Case 1 Pain Aspiration Fluid Overload 12 year old girl with history of traumatic brain injury Spastic cerebral palsy Developmental delay Seizures Gastrostomy tube fed Tracheostomy Recurrent ileus, unable to tolerate enteral feeds Parenteral nutrition requires frequent lab monitoring and can cause electrolyte abnormalities, liver disease, and increase risk of infection. Fluid Overload

Case 1 Admitted to inpatient hospice ANH withdrawn Allowed natural death 12 year old girl with history of traumatic brain injury Spastic cerebral palsy Developmental delay Seizures Gastrostomy tube fed Tracheostomy Recurrent ileus, unable to tolerate enteral feeds

Case 1 Key teaching points: Neurologically impaired children are at risk for dysmotility and GI dysfunction that can make feeding challenging and often cause pain and suffering Withdrawal of Artificial Nutrition and Hydration (ANH) is ethically permissible in certain cases ANH can have harmful side effects and benefits don’t always outweigh risks 12 year old girl with history of traumatic brain injury Spastic cerebral palsy Developmental delay Seizures Gastrostomy tube fed Tracheostomy Recurrent ileus, unable to tolerate enteral feeds

Case 2 Diagnosis Delivery of Prognosis 16 month old boy with history of birth asphyxia Spastic cerebral palsy Developmental delay Seizures Gastrostomy tube fed

Case 2 Acceptance of prognosis, focus on symptom control and comfort. Period of Adjustment 16 month old boy with history of birth asphyxia Spastic cerebral palsy Developmental delay Seizures Gastrostomy tube fed Vomiting, poor weight gain

Case 2 Persistent vomiting, weight loss, and breathing difficulties. 16 month old boy with history of birth asphyxia Spastic cerebral palsy Developmental delay Seizures Gastrostomy tube fed Vomiting, poor weight gain Aspiration Crises

Case 2 Benefit Quality of Life Clear 16 month old boy with history of birth asphyxia Spastic cerebral palsy Developmental delay Seizures Gastrostomy tube fed Vomiting, poor weight gain Aspiration Unclear None Suffering Initiative for Pediatric Palliative Care Curriculum.

Case 2 Suffering Pain Secretions Dysautonomia Secretions 16 month old boy with history of birth asphyxia Spastic cerebral palsy Developmental delay Seizures Gastrostomy tube fed Vomiting, poor weight gain Aspiration Suffering Spasticity Dysmotility Seizures Dyspnea

Case 2 Alternative intervention (gastro-jejunal tube) offered Suffering reduced Quality of life improved Palliative care re-introduced to provide continued support for family 16 month old boy with history of birth asphyxia Spastic cerebral palsy Developmental delay Seizures Gastrostomy tube fed Vomiting, poor weight gain Aspiration

Case 2 Key Teaching Points It is often difficult for parents to accept poor neurological prognosis at birth. Goals of care may change over time. Misperceptions of palliative care are common. Education is needed to ensure families can benefit from available services. When benefit/burden of procedures is unclear (such as surgeries) family wishes should be honored. Always ensure that all measures have been taken to make the patient comfortable and relieve suffering. 16 month old boy with history of birth asphyxia Spastic cerebral palsy Developmental delay Seizures Gastrostomy tube fed Vomiting, poor weight gain Aspiration

Case 3 Recurrent pneumonias and respiratory insufficiency lead to tracheostomy and possible ventilator dependence. 9 year old girl born at 24 weeks gestation with Grade 4 IVH Developmental delay Seizures VP shunt Spasticity GJ tube fed New tracheostomy Crises

Case 3 Increased pain / suffering despite maximized medical management Perceived deterioration in quality of life More time in hospital Anticipated escalation in medical interventions needed to sustain life 9 year old girl born at 24 weeks gestation with Grade 4 IVH Developmental delay Seizures VP shunt Spasticity GJ tube fed New tracheostomy Recurrent pneumonia Ventilator dependence

Case 3 HOSPITAL HOME HOSPICE 9 year old girl born at 24 weeks gestation with Grade 4 IVH Developmental delay Seizures VP shunt Spasticity GJ tube fed New tracheostomy Recurrent pneumonia Ventilator dependence HOSPICE

Case 3 Curative 9 year old girl born at 24 weeks gestation with Palliative Curative Concurrent Care 9 year old girl born at 24 weeks gestation with Grade 4 IVH Developmental delay Seizures VP shunt Spasticity GJ tube fed New tracheostomy Recurrent pneumonia Ventilator dependence Curative

Case 3 Family chooses home hospice Private duty nursing services continued Wish for death at home honored Family togetherness maximized 9 year old girl born at 24 weeks gestation with Grade 4 IVH Developmental delay Seizures VP shunt Spasticity GJ tube fed New tracheostomy\ Recurrent pneumonia Ventilator dependence

Case 3 Key Teaching Points Many children ultimately die in a hospital setting. Discharge home with hospice is an alternative that can be considered. The Concurrent Care Act requires that Medicaid support both hospice care and routine or curative care, including home nursing. Barriers continue to exist to providing pediatric hospice care and to implementation of Concurrent Care in practice. 9 year old girl born at 24 weeks gestation with Grade 4 IVH Developmental delay Seizures VP shunt Spasticity GJ tube fed New tracheostomy Recurrent pneumonia Ventilator dependence