Lecture 10a: Hematologic and Oncologic Diseases and Integration of Palliative Care in Children

Define Pediatric Hematologic Disorders.

a group of acute and chronic diseases characterized by disorders of RBCs and WBCs and platelet production, structure, and/or function and coagulation abnormalities, which, if not adequately treated could result in disability or death

Define Sickle Cell Disease.

• chronic disease with acute exacerbations

• caused by mutation in DNA that determines hGB resulting in absence of normal HbA

Describe healthy RBCs.

flexible, biconcave discs with lifespan of 120 days

Describe SCD RBCs.

they polymerize, forming microtubule making cells crescent-shaped and friable with 10-20 days lifespan

By what age is SCD apparent?

6 months

What does misshapen RBCs in SCD lead to?

vaso-occlusion

Management of Sickle Cell Diseases.

• penicillin prophylaxis

• hydroxyurea

• folic acid supplement

• transfusions/chelation

• HSCT

What 3 factors result in vaso-occlusive crisis?

1. Ischemia due to occlusion of vessels by misshaped RBCs

2. Endothelial damage

3. Local inflammation

What are the psychosocial effects on the child in sickle cell disease?

• missed school and social activities

• poorly managed chronic pain

What can unmanaged chronic pain in children with SCD cause?

• increased admissions

• depressive symptom development

• poor working memory

• poor processing speed

• lower reading fluency abilities

What are the psychosocial effects on the caregiver in sickle cell disease?

• missed work

• high parenting stress

• poor mental and physical health of caregivers directly related to worse child outcomes

What can missed school and social activities in children with SCD cause?

higher levels of loneliness

What can missed work in caregivers of SCD children cause?

financial stress, etc.

What can high parenting stress in caregivers of SCD children cause?

elevated risk for physical and psychiatric disorders

What can poor mental and physical health in caregivers of SCD children cause?

• worse child outcomes

  • increased pain intensity

  • higher functional disability

  • worse QoL

Define pediatric cancer.

group of chronic diseases characterized by uncontrolled growth and psread of abnormal cells, which, if not adequately treated, results in death

Fundamentally, what is cancer?

genetic mutation

Define a genetic mutation.

• permanent DNA alteration

• begins in single cell

What do genetic mutation result in?

• lack of differentiation

• loss of contact inhibition

• unregulated growth

• cellular immortality

Two broad categories of childhood cancer?

• hematologic malignancies

• solid tumours

Name 6 different treatment modalities for childhood cancer.

• chemotherapy

• biotherapy

• immunotherapy

• radiation

• surgery

• HSCT

Psychosocial effects in caregivers of children with cancer?

• financial stress

• marital tensions

• psychological distress

  • fear of relapse, recurrence

  • late effects

  • uncertainty

Psychosocial effects in siblings of children with cancer?

• loss, fear, uncertainty

• jealousy, guilt, anxiety

  • “forgotten child”

Psychosocial effects in children with cancer?

• missed school and social activities

  • impact academic performance

• unmet developmental milestones

  • treatment related to neurocognitive effects

• anger, fear, guilt, insomnia, depression

Define palliative care.

a specialized field of care that focuses on the prevention and relief of suffering of a person diagnosed with a life-limiting illness, and their families

Differences in pediatric palliative care from adult pediatric care.

• prognosis, life expectancy, and functional outcome often become unclear

• greater use of intensive disease-modifying or life-sustaining treatments

• focus on growth & development at the same time as death

• greater physical & emotional burden for families

• informational, recreational, and educational needs change as a child grows

• greater number of congenital anomalies of uncertain types of rare genetic conditions

3 main components of pediatric palliative care.

• pain and symptom management

• maximizing quality of life

• end-of-life care

Neurological signs of death in children?

• decreased wakefulness

• decreased sensitivity to touch or stimulus

• increased pain

• increased seizure activity

Cardiac signs of death in children?

• tachycardia

• bradycardia

• decreased circulation

• pale or mottled skin colour

Respiratory signs of death in children?

• dyspnea

• tachypnea

• increased exertion

• increased secretions

GI/GU signs of death in children?

• decreased appetite

• increased nausea

• vomiting

• decreased food tolerance

• decreased urine production

• constipation

• diarrhea

Musculoskeletal signs of death in children?

• increased/decreased muscular tone

• spasms

• incontinence

Name 3 ways to maximize quality of life.

• legacy creation

• memory making

• recreational therapy service

Define grief.

feelings and emotions experienced by a person in response to an actual or perceived loss

Define bereavement.

the journey a person goes through after suffering a loss

What continuum is grief experienced on?

wellness-illness continuum

Name 4 supports for grief and bereavement.

• nursing

• child life

• social work

• palliative care/pain and symptom management teams

Name 4 different types

• normal

• anticipatory

• disenfranchised complicated grief

Physical symptoms of grief in pediatric oncology nurses?

• apathy

• lethargy

• insomnia

• anxiety