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