|Phase of Trajectory
- Communicating a new vision of hope and a commitment to companioning families throughout the process
- Establishing realistic timelines and expectations for the diagnostic process
- Eliciting parental preferences for communication, decision-making and continuity of care
- Framing the diagnosis as living with the disease rather than focusing on dying
- Anticipating and acknowledging parental grief*
- Disclosing clinicians’ objections to offering or implementing certain treatment options (including enrollment in clinical trial)
- Acknowledging the uncertainty in velocity of progression and severity throughout the disease process
- Appreciating the unique significance of the genetic aspect of the diagnosis on family dynamics such as parental guilt related to mode of transmission, communication of familial risks and family planning decisions.
- Beginning advance care planning by anticipating decision points along the trajectory and assessing families’ priorities for quality of life, and expectations and limits of treatment
- Reframing definition of a “normal” child and honoring the range of bonding attitudes and behaviors
- Encouraging parents/families to begin “meaning-making” activities such as taking pictures, family gatherings, celebrating milestones
- Anticipating technology and therapy needs based on goals of care and values regarding quality of life
- Assessing pain and symptoms to establish patient baseline
|Changes in Functional Status
- Discussing goals and preferences for treatment (resuscitation, ventilation, oxygen, feeding tubes, etc.) at regular intervals and appropriate times as the disease progresses
- with parents
- with affected children and adolescents who have cognitive and developmental capacities
- Re-defining the morally permissible boundaries of treatment including aggressive technological interventions such as tracheostomy, mechanical ventilation, G-tube, etc.
- Re-visiting previous decisions in light of new information and experience, and offering options to continue care or redirect it to focus on palliation or end-of-life care
- Instituting measures to enhance quality of life such as
- optimizing interaction, mobility, positioning
- maximizing educational, social and community integration/access
- Determining eligibility for services such as home nursing care, medical equipment, hospice, etc.
- Frequent monitoring of pain and symptoms including joint and muscle pain, discomfort associated with mobility and positioning, gastrointestinal discomfort due to reflux, dyspnea, etc.
- Honoring child’s resilience and adaptive patterns
- Anticipating grief responses at each loss of function and referring to appropriate specialist.
- Exploring family needs for respite, psychosocial and spiritual support
- Clarifying the meaning of life-threatening events such as progressive respiratory failure, inability to feed orally, infections, pneumonia, etc., acknowledging the likelihood of death and offering psychosocial and spiritual support to: – parents
- affected children and adolescents
- Assessing whether prior preferences for life-sustaining therapies have changed and, if so, document changes in goals of care including use of intensive care, transfer to hospital, home hospice, etc., and, if appropriate, executing DNR or Allow Natural Death orders or other limitations of care and communicating these decisions to relevant entities and personnel including EMS, local hospital, etc.
- Considering if or when to involve ethics consultants or committees if disputes arise
- Re-assessing symptoms, functional status, and comfort
- Re-introducing opportunities for “meaning-making” to
- affected children and adolescents
- Acknowledging the uncertainty and variability in the dying process and offering grief counseling and support
- Anticipating fears and concerns regarding terminal events and care at the end of life/time of death including managing breathing, comfort, impact of analgesia on awareness and interaction
- of parents
- of affected children and adolescents who have cognitive/ developmental capacities
- Completing important life closure activities
- among family members and
- with affected child/adolescent
- Exploring preferences for location of death – home, hospital, hospice, other.
- Assisting with plans for funeral, burial, cremation and care of the body at the time of death
|Death and Bereavement
- Assessing family needs and preferences for bereavement services
- Making referrals for spiritual, emotional and psychological support for the entire family
- Providing opportunities for post-death meeting with the interdisciplinary team to review new findings, autopsy, lingering questions, revisit genetic testing recommendations
- Offering involvement of health care team members in remembrance and memorial services
- Providing bereavement support for 1-2 years after the child’s death