Angel's Trumpet 25F
ADMIT: 3/6/26
TEAM: LAVENDER
CODE: DNR/DNI/CMFT
DX: Advanced Fatal Familial Insomnia
- p/w rapidly progressive neurodegenerative disease consistent with advanced Fatal Familial Insomnia secondary to a Prion Disease.
- previously healthy until 4 months ago, when pt began experiencing: severe insomnia resistant to all sedatives, progressive autonomic dysfunction, cognitive decline, severe confusion and hallucinations, progressive weight loss (~20 lbs)
- BIBA after 3 days of worsening delirium and collapse at home.
- transferred to ICU d/t severe autonomic instability, complete absence of sleep for >10 days, progressive delirium and agitation, refractory tachycardia and hypertension, episodes of hyperthermia
HX: family hx of early neurodegenerative death: Mother died at age 42 from prion disease.
ACTIVITY: Bedrest. Soft Wrist Restraints
DIET: NPO w/Continuous NGT Feed @ 50 mL/hr
IV: R UA PICC, L Wrist arterial line
NEURO: GCS 11 when not sedated w/severe delirium, dysarthria, ataxia, and visual hallucinations. remains intermittently agitated on sedation.
SLEEP STATUS: no physiologic sleep recorded in 96 hours
PULMO: RR 28–32 on high-flow nasal cannula
CARDIAC: Tele: ST
GI: Incontinent
GU: Foley w/Temp probe
SKIN: Pale w/profuse diaphoresis
V/S: HR: 135–150 persistent tachycardia, BP: 170/95 with sudden drops, Temp: 38.9°C episodic hyperthermia
PLAN: Continuous dexmedetomidine infusion, PRN benzodiazepines, Cooling measures, Aggressive fluid support, Nutritional support, Sedation protocols, Q1 Neuro checks
LABS:
- Genetic testing: confirmed PRNP mutation
- MRI Brain: Mild thalamic signal abnormalities
- PET Scan: Hypometabolism in thalamus
- CSF: Elevated 14-3-3 protein
- Chest XR: Right lower lobe consolidation w/patchy infiltrates and air bronchograms