Post on 31-Mar-2015
Moderator: Russell K. Portenoy, MD
Panelists:
John M. Saroyan, MD
Debra F. Fox, APRN, FNP-BC, CHPN
Katherine Leonard, MD
Erica Rosenbaum, LMSW
11 month old femaleEpidermolysis bullosa, junctional typeExtensive skin & gastrointestinal lesionsFailure to thriveSignificant developmental delayPE: Extensive desquamation to arms, legs;
scapulae and sacrum Comfort care; DNR
Pain managementMethadone 0.1 mg po tid +Morphine prnParental concern over “addiction”Principle of double effect
Dressing changesManagement of other symptoms
Pruritis, irritability, dyspneaFeedingsMedications
Formulations with and without feeding tube
Psychosocial support inRecessive autosomal inheritanceWell, “unaffected” siblingMultiple loss bereavement
Home health nursing + palliative careOpportunities for collaboration with industry
and national organizations
10 y/o girlALLs/p standard chemotherapy, not a BMT
candidate, Phase I and II trials exhaustedTransfusion dependentReferred to hospice on discharge from
hospital
Symptom control pain, nausea, dyspnea, fear and anxiety,
insomniaGoals of care
transfusion, feeding, antibiotics, code statusTransition to HomeFamily, Patient and Community Support at
end-of-life and through bereavement
Brief overview
~. State and Federal guidelines
~. Incorporating Pediatric hospice and palliative care within the guidelines of concurrent care
• 6 y/o boy • temperature and blood pressure
abnormalities• dystonic posturing• diaphoresis and erythroderma• severe neurologic impairment following
meningitis/encephalitis• generalized tonic-clonic seizures• Multiple ED visits• ICU admissions over three years with
multiple prolonged intubations, sepsis work-up (including spinal taps), many pneumonia
DiagnosisNeuroirritability
TreatmentClonidineClonazepamDiazepam PR
Goals of Care and Home Services