Define the "F"

• ≥37.5°C axillary / ≥38.0°C oral sustained for 1 hour, OR
• A single reading ≥37.8°C axillary / ≥38.3°C oral
• Be suspicious of persistent "low-grade" fevers. If febrile + unwell-looking but not yet neutropenic — they still qualify.

Define the "N"

• ANC < 0.5 ×10⁹/L, or non-severe neutropenia with counts trending down.

History & exam — find a source

• Full physical including CVL site (all lumens), ENT, perineum, feet, skin.
• Stable or unstable? Cap refill, BP, HR.
• Don't be fooled by warm sepsis: wide pulse pressure, bounding/hyperdynamic pulses, flash cap refill.

Workup

• CBC + differential; central cultures from each lumen + peripheral culture (first fever).
• Urine culture; as indicated: CXR, NP swab, others guided by exam.
• Repeat fevers: re-culture centrally with each fever if last culture >24 h ago. Always pull a new culture before adding any new antibiotic.

Empiric antibiotics

• Cover Gram-positive, Gram-negative, and double Pseudomonas coverage. Always cover Pseudomonas.
• Neutropenic (ANC <0.5): start piperacillin-tazobactam.
• Even if not neutropenic — Down syndrome, relapse and AML patients often still need abx; ask fellow (they may want a different choice).
• Septic / unstable / hypotensive: skip pip-tazo → meropenem + vancomycin + amikacin (or upgrade to this).
• Penicillin allergic → discuss with fellow.
• ≥5 straight days of fever → work up for fungal infection.

Supportive

• New febrile patients → run IVF at 1.5× maintenance (usually 0.9% NaCl).
• Unstable / low BP → 20 cc/kg NS bolus, up to ×3, then call CCRT.

Ongoing fever while on antibiotics

• Culture once a day centrally.
• Never assume a positive culture is a contaminant. Gram-positive → add vanco; consider line removal → make NPO.
• Becomes unstable → must upgrade antibiotics.

Hyperleukocytosis = total WBC >100 ×10⁹/L (10% of ALL, up to ~25% of AML, ~100% of CML blast crisis; common in infants). Leukostasis = symptomatic hyperleukocytosis from hyperviscosity — up to 20–40% one-week mortality untreated.

Clinical manifestations

• Respiratory: exertional dyspnea, distress, hypoxemia (pulse oximetry more accurate than ABG; CXR diffuse infiltrates or normal).
• Neuro: confusion, somnolence, stupor, coma, headache, dizziness, gait instability, blurred vision, papilloedema, CN defects. Focal signs may = ICH.
• Hemorrhagic (ICH, GI, pulmonary, DIC, thrombocytopenia), renal failure, TLS.

Management

• ABCs, monitoring; large-bore IV access.
• Bloods: CBC + diff, PTT/INR, type & screen; TLS panel (extended lytes, urate, LDH, BUN, Cr); peripheral blood for flow cytometry.
• Prevent/treat TLS: hyperhydration 2–3× maintenance; rasburicase (or allopurinol).
• Avoid PRBC transfusion (increases viscosity). Only if impending heart failure/death: 2–5 cc/kg very slowly.
• Correct coagulopathy. WBC fragments may be miscounted as platelets → insist on manual platelet count. Platelets & FFP do not increase viscosity.
• Leukocytoreduction: induction chemo; hydroxyurea (CML); leukapheresis.

Metabolic derangements from abrupt lysis of malignant cells releasing intracellular contents. Occurs 3 days before to 7 days after starting treatment — most commonly 12–72 h after cytotoxic chemo.

History & presentation

• Often nonspecific: nausea, vomiting; reduced urine output, edema, fluid overload.
• Hyperkalemia → cramps, paraesthesia, arrhythmia; hypocalcemia → tetany, twitching, seizures, prolonged QT; sudden death.
• Pre-existing renal impairment → upstage the risk category by one level.

Cairo–Bishop laboratory criteria (≥2)

Clinical TLS = lab TLS + a complication: AKI/↑creatinine (≥1.5× ULN), cardiac arrhythmia, seizure, or sudden death.

Who's at risk

• High turnover / large burden: WBC ≥50, LDH ≥2000, bulky/mediastinal mass, HSM.
• Burkitt leukemia/lymphoma, lymphoblastic lymphoma, ALL (NHL 4–42%, ALL ~21%, AML ~15%, solid tumours rare).

Management

• Identify high risk early; frequent monitoring — labs q4–24 h (Ca, PO₄, urate, Cr, LDH, K), strict ins & outs, daily weights.
• Fluids: 0.9% NaCl, often ~2× maintenance (≈200 mL/m²/h). Target urine output 80% of intake; Lasix/mannitol to support UO. No additives — do not put K into the IVF.
• Allopurinol (prophylaxis for all): 200–300 mg/m²/day div BID–QID; continue ≥72 h after starting treatment, until urate normal & stable.
• Rasburicase (extreme cases / high risk / urate >476 / allopurinol failure): 0.2 mg/kg IV q24h (q12h if needed). Send G6PD before giving (haemolysis risk); samples for urate on ice; recheck urate within 4 h.
• Hyperphosphatemia: aluminium hydroxide / sevelamer (PO, rarely used).
• Hypocalcemia: do not treat unless symptomatic.
• Urine alkalinization: no longer recommended.

Presentation

• Headache, behaviour change, nausea/vomiting, lethargy.
• Change in pupil reaction, impaired upward gaze; false-localizing signs (e.g. 6th nerve palsy).
• Seizures, decreased coordination/ataxia, papilloedema.
• Late — Cushing's triad: ↑BP, ↓HR, irregular RR.

Treatment

• Urgent imaging; urgent neurosurgery consult.
• Raise head of bed; intubation; mannitol.
• Cause-directed: steroids, radiation, surgery.

Occurs in 4–25% of children with cancer — often a presenting feature. Commonest causes: extradural sarcomas (rhabdomyosarcoma, Ewing), neuroblastoma, plus CNS tumours and leukaemia/lymphoma. May also arise mid-treatment (metastasis, bleed, or myelitis after intrathecal chemo).

History & presentation

• Back pain is the earliest symptom (often present for a while before diagnosis). In young children may be reported as "tummy pain"; worse with positioning, coughing or sneezing.
• Early: urinary retention, constipation, urinary/faecal incontinence, back pain.
• Late: motor weakness, sensory impairment/loss, paralysis.
• Cauda equina: low back pain, uni-/bilateral sciatica, lower-limb weakness, saddle sensory loss, loss of visceral (bladder/bowel) function.
• In pre-verbal/non-toilet-trained children, test anal sphincter tone and cremasteric reflex. Document level & timeline (determines prognosis).

Workup & management

• Thorough neuro exam; urgent MRI of spine and pelvis (CT if bony involvement suspected).
• Acute symptoms + intraspinal mass → call neurosurgery then oncology for decompression ± tissue sampling.
• Early high-dose steroids reduce swelling and improve recovery — but can obscure/prevent diagnosis (leukaemia/lymphoma), so coordinate timing; taper early.
• If surgery not feasible → radiation oncology; if neither → start chemo (esp. neuroblastoma).

Many children are surprisingly asymptomatic — the mass is found on imaging. A 2-view CXR early drives risk management. Get an echo/ECG the same day.

Stepwise diagnosis (least-invasive first — sedation/positioning ↑ risk)

• CBC (peripheral blasts may be diagnostic) → BMA/biopsy (may need to be done awake, different positioning) → tap pleural/pericardial effusion → peripheral lymph-node excision (not core) → mediastinal mass biopsy (IR or surgery).
• Workup: echo/ECG same day; PFTs per status; anaesthesia consult if sedation needed; CT neck/chest/abdo/pelvis (patient cannot lie flat); TLS bloods; CCRT/PICU if unstable.
• Avoid empiric steroids before tissue diagnosis where possible — they can obscure the diagnosis. If unstable, agree timing of steroids/procedure with PICU + anaesthesia.

SVC / superior mediastinal syndrome — presents with

• Dyspnea, cough, hoarseness, chest pain, headache, vision changes, facial swelling, wheeze, stridor, cyanosis, plethora of face/neck/arms.
• CXR: tracheal deviation/compression, widened mediastinum.

Inflammation of the caecum (± terminal ileum, ascending colon) — bowel-wall thickening, oedema, mucosal ulceration/necrosis. Incidence ~3% (leukaemia/lymphoma), ~2.5% (solid tumours). Can progress to necrosis, hemorrhage, perforation, septicemia. Rapid improvement after neutrophil recovery. Mortality up to ~20%.

History & presentation

• Triad: abdominal pain + RLQ tenderness, fever, neutropenia (ANC <0.5). Also nausea/vomiting, diarrhea, distension; hematochezia ~25%, constipation ~6%; ± hypotension, peritonism. Signs may be blunted by neutropenia/steroids — keep a high index of suspicion.

Workup

• CBC, chemistry (lytes, urea, Cr, albumin, total protein, LFTs); blood cultures (central both lumens + peripheral); stool for C. difficile. Imaging: AXR + US (bowel-wall thickening); reserve CT for inconclusive cases. Cultures positive ~25% (E. coli, Pseudomonas, Klebsiella, viridans strep).

Management (medical, not surgical)

• Bowel rest (NPO until resolution), TPN, fluid resuscitation, NG suction, correct electrolytes, continuous narcotic infusion for pain.
• Broad-spectrum antibiotics per F&N protocol; add metronidazole (anaerobes) ± vancomycin; antifungal (e.g. liposomal amphotericin) if indicated.
• Surgery only for perforation, uncontrolled bleeding, peritonitis, or gangrenous bowel. Call surgery/fellow early.

Thromboembolism affects ~8% of children with cancer (vs ~0.7–1.4/100,000 generally) — multifactorial (cancer + therapy + host).

Presentation by site

• CVL/upper-extremity DVT: arm/neck/facial swelling, distended veins, line dysfunction.
• Right atrial thrombus (RAT): often CVL tip in RA; ~44% symptomatic — respiratory distress (commonest), arrhythmia, new murmur, cyanosis, heart failure. High-risk clots = >2 cm, pedunculated/mobile, snake-shaped → PE risk.
• PE: ~9% fatal; ~70% have a clot elsewhere. Dyspnea, chest pain, hypoxia, tachycardia.
• CSVT (cerebral sinovenous, common in ALL on asparaginase): headache, vomiting, seizures, altered consciousness, focal deficit — may be mistaken for IT-chemo or steroid-HTN headache, so keep a high index of suspicion.

Diagnosis & management

• Confirm objectively: Doppler US (limb/CVL), echo (RAT), CT/CTPA (PE), MRI/MRV (CSVT). If clot at one site, image anatomically-related sites.
• Call the fellow / thrombosis service — anticoagulation choices are weighed against thrombocytopenia, coagulopathy and frequent procedures (LP, BMA, surgery).
• RAT: multidisciplinary (cards/CV-surgery/IR) — high-risk clots may need thrombectomy/thrombolysis; low-risk → anticoagulation ± line removal with serial echo.

Immediate actions

• 20 cc/kg NS bolus (push), up to ×3.
• Call fellow. On the second bolus → consider CCRT.
• If febrile + neutropenic → see Febrile Neutropenia (skip pip-tazo, go to mero/vanco/amikacin if septic).

Stress steroids?

• Consider if recently on prolonged steroid course (e.g. new leukemics post-induction, known adrenal insufficiency).
• No steroids for L&L pre-diagnostic biopsy, or solid-tumour patients on immunotherapy.

Rule out 'easier' causes

• Wrong cuff size; pain/anxiety.
• Fluid overload — check fluid balance, weight, signs of overload → consider Lasix.
• Steroids (common cause in onc patients).

If none of the above

• Consider PRN nifedipine or hydralazine; look for past response to anti-hypertensives.
• First time & not a hypertensive emergency → start at a low dose.
• Consider asking Nephrology for help.

Antibacterial (CID 2020)

• Weak rec for levofloxacin during anticipated prolonged severe neutropenia in AML or relapsed ALL.
• Weak rec against routine systemic antibacterial prophylaxis in other groups (resistance & C. difficile risk).

Antifungal (JCO 2020)

• Strong rec to give systemic antifungal prophylaxis in AML, allogeneic HSCT pre-engraftment, and GVHD on immunosuppression.
• Use a mould-active agent: an echinocandin or a mould-active azole. <13 y → echinocandin, voriconazole, or itraconazole; ≥13 y → posaconazole also an option.
• Strong rec against routine amphotericin for prophylaxis.

Consider in any child with new diarrhea on/after antibiotics. Toxin-producing C. difficile ranges from asymptomatic carriage to pseudomembranous colitis/toxic megacolon. Recurrence ~25%.

Severity (clinical judgment)

Testing — diarrhoeal stool only

CPS treatment by severity

Recurrence (~25%)

• First recurrence: repeat the initial regimen, or vancomycin 40 mg/kg/day PO ÷ QID ×10 d.
• Second+ recurrence: tapered/pulsed oral vancomycin; consult ID. Fidaxomicin (licensed ≥18 y) and FMT have lower-recurrence data in adults, limited paediatric data.

A common, largely preventable healthcare-associated infection in children with CVCs — significant morbidity, cost, and mortality. Onc patients are high-risk (neutropenia, TPN, frequent access).

Four routes of infection

• 1 Extraluminal — skin flora migrate along the outside of the catheter from the insertion site. Early (<1 week) → insertion-related.
• 2 Intraluminal — contamination of the hub with each access, spreading down the lumen. Later → maintenance-related.
• 3 Haematogenous — seeding of the catheter from a distant infection (less common).
• 4 Contaminated infusate — rare.

Definitions — not the same thing

Diagnosis

• Draw paired blood cultures — from each CVC lumen and a peripheral vein, aseptic technique.
• Practise blood-culture stewardship: culture for a clinical indication — avoid reflexively culturing the line (overuse → false CRBSI + antibiotic overuse).

Is it real? Contaminant vs true CRBSI · IDSA 2009

Empiric antibiotics

Remove the line, or salvage it?

* Complicated = severe sepsis/shock, suppurative thrombophlebitis, endocarditis, persistent bacteraemia/fungaemia >72 h on appropriate therapy, tunnel/port abscess, or a metastatic focus.  † High-risk organism = S. aureus, P. aeruginosa, Candida/fungi, mycobacteria (and any short-term line with GNB or enterococci).  ‡ Salvage only: long-term line, uncomplicated, less-virulent organism, limited access.

Duration by organism (after source control)

Prevention bundles

• Examine to rule out thrombosis: head/arm swelling, discoloration, distress.
• Consider CXR to confirm tip placement (SVC–RA junction).
• Use alteplase, up to two attempts (see formulary).
• Unsuccessful → call Vascular Access Nurse or IGT for workup/repair/replacement.

On the onc ward

• New febrile patients → 1.5× maintenance. Ward generally prefers 0.9% NaCl as the main fluid (D5 0.9NS if not eating — check with nursing).
• Certain chemo regimens have hyperhydration protocols and urine-output parameters (e.g. must void 80% of intake — nurses know, can give Lasix).
• Generally don't treat fluid imbalance unless symptomatic — case-by-case.

Maintenance fluid selection (general IV fluid pathway)

• Order labs before fluids: Na, K, glucose, creatinine. Daily lytes if NPO on full maintenance.
• Maintenance [Na⁺] should approximate ½NS — i.e. D5W 0.45% NaCl for most; use D5W 0.9% NaCl if Na low/unknown or severe dehydration.
• Add maintenance potassium (KCl 20 mmol/L) to prevent iatrogenic hypokalemia — only with normal renal function & good urine output (not in TLS-risk / new patients).

Hyperkalemia

• Confirm true value; place on telemetry; watch the TLS trend.
• First step: increase fluids. ECG abnormal → calcium (carbonate/gluconate).
• Very high & real → ventolin; Lasix (slightly slower than ventolin but great); Kayexalate (slow, better for chronic).

Hypokalemia

• Usually don't worry if >3.2. Causes: cisplatin/carboplatin, amphotericin B, diuretics, GI losses, intake.
• Avoid putting K in IVF lines for new / TLS-risk patients.
• If <~2.7 → KCl bolus (Epic order set "Potassium Chloride" for heme/onc): 10–40 mmol in 100 mL, start 10; max rate 0.5 mmol/kg/hr from all sources; usually on monitors. Recheck ~30 min later.
• Hold TPN during bolus (don't permanently change TPN overnight). In-between values → try oral supps.
• On Ambisome → K usually needs to be >3–3.2 before it can be given (clarify with fellow).

Hypomagnesemia

• Don't usually worry unless <0.50. Causes: chemo, antibiotics, cyclosporine.
• IV bolus: order "Magnesium sulfate" — 0.3 mmol/kg to max 10 mmol, infuse slowly (avoid hypotension). Or add to IVF (~4 mmol/L). Oral works but causes diarrhea — start slow.

Hyponatremia

• Common. Check they aren't on hypotonic fluids. Consider SIADH (brain tumours, vincristine, cisplatin, cyclophosphamide).
• Adjust fluids, ?restrict, consider ins/outs ± Lasix; 3% bolus if needed. Talk to fellow.

Phosphate

• Replace if <0.8–0.9. Oral first — sodium phosphate (not potassium phosphate), 1–2 mmol/kg/day div BID. Very low → high-phosphate infusion.

Calcium

• PO: 50–100 mg/kg/day div TID–QID.
• Symptomatic (e.g. seizure): 20 mg/kg CaCl (0.2 mL/kg 10%) or 60 mg/kg Ca-gluconate (0.6 mL/kg 10%).

Urate

• High in new patients starting chemo; most are on allopurinol. No single concerning number, but if rising maximize allopurinol (some consider >375 worrisome — ask).
• Still rising → consider rasburicase (discuss with fellow first; send G6PD).

Sodium: 1–2 above/below reference range is fine.

Red cells

• Transfuse pRBC for Hb <70 g/L, or if symptomatic.
• Dose 10–15 mL/kg over 3–4 h (max 1 unit / 300 mL).
• Anaesthesia/procedures: aim Hb >75–80 (anesthetist-dependent).

Platelet thresholds — transfuse if

• Dose 10 mL/kg over 1 h (max 1 unit / 300 mL).
• BMA/biopsy: no strict minimum — check with anaesthesia/surgeon.
• Brain-tumour thresholds vary (VP shunt/Ommaya 10; gross-total resection on tx 30; residual/subtotal or prior ICH or anti-angiogenesis agent 50).

Anticipated response & rate

• pRBC raises Hb ~2–3 g/dL (Hct ~6–9%); rate ~2.5 mL/kg/h avoids overload (TACO). Monitor vitals at start, 15 min, hourly, end.
• Platelets 5–10 mL/kg raise count ~30–50 ×10⁹/L (over 20–60 min). Poor response → sepsis, fever, splenomegaly, DIC, HLA alloimmunisation, amphotericin. A 10-min–1 h post count detects alloimmunisation.

Standard reaction workup (per SK chart)

• Type & screen + DAT, patient blood cultures, urinalysis, blood-bag cultures.
• CBC, electrolytes, creatinine, bilirubin, LDH, aPTT, INR, fibrinogen, haptoglobin, plasma Hb.
• If dyspneic: chest X-ray, blood gases.
• Suspected anaphylaxis / IgA-related: IgA level / anti-IgA.

Febrile reaction (temp ↑≥1°C and >38°C)

• If ≥39°C, hypotension, tachycardia, rigors, dyspnea, back/chest pain, N/V → possible haemolytic or bacterial contamination: discontinue, sample the product, draw blood cultures.
• Otherwise → likely febrile non-haemolytic: give acetaminophen, continue cautiously. Recurrent → premedicate acetaminophen.

Allergic reaction

• Hypotension, dyspnea/cough, tachycardia, flushing, widespread rash (≥⅔ BSA) → severe/anaphylactic: diphenhydramine + corticosteroid + epinephrine as needed; do NOT restart.
• Mild (urticaria only) → diphenhydramine, continue cautiously. Recurrent → premed ± washed/plasma-depleted products.

For procedures requiring INR, target INR ≤1.5 and PTT <120% of normal. General onc procedure rules also: platelets >50 for LP/CVL insertion; >75 for port insertion; Hb aim >75–80 for anaesthesia.

Principles

• Always check temperature before giving Tylenol (it can mask fever).
• Morphine is fine — watch for constipation (start prophylaxis). Morphine infusions / CADD pumps used liberally.
• Anticipate constipation and prophylax. Liberal Acute Pain Service (APS) consults.
• Assess pain with OPQRSTUV; severity 1–3 mild, 4–6 moderate, 7–10 severe. Neuropathic descriptors (burning, shooting, tingling) → consider adjuvant analgesic.

Opioid equianalgesic (adult reference)

When rotating opioids, reduce the calculated equianalgesic dose by 33–50% (incomplete cross-tolerance) and retitrate. Hydromorphone preferred in renal disease; caution with morphine in severe renal impairment. Avoid meperidine (normeperidine → seizures, accumulates in renal impairment). *Codeine doses shown are table values, not for practice.

Common; worst ~7–10 days post-chemo (mirrors neutrophil nadir). Main complications: pain, decreased oral intake, superinfection (HSV, candida, aerobic/anaerobic bacteria).

Management

• Meticulous oral hygiene; mouthwash QID (sodium bicarbonate or chlorhexidine / "HSC pain mouthwash").
• Morphine boluses — low threshold to start an infusion. IV fluids ± TPN.
• Send swab for Gram stain + viral swab (HSV); treat if positive.
• If bleeding, keep platelets >50.

DDx: hemorrhagic cystitis (cyclophosphamide/ifosfamide), UTI, viral cystitis (rare), thrombocytopenia, other usual paediatric causes.

Approach

• Microscopic vs gross? Microscopic → follow closely; send urinalysis + culture.
• Increase hydration up to 4 L/m²/day if chemo/viral induced → call fellow.
• Ensure platelets ≥50; check coagulation.
• Persistent/severe gross hematuria → call fellow (?hold chemo, ↑Mesna); if obstructive → urology for catheter / bladder irrigation. Watch Hb and platelets.

• Constipation is a major problem — be aggressive with management.
• Anticipate it with opioids and especially vincristine (causes ileus/constipation — treat aggressively).

Biologics/antibodies (ATG, gemtuzumab, ch14.18): high rate of inflammatory/infusion reactions — usually fellow-managed overnight, premeds, fluid boluses, CCRT/ICU as needed.

Match prophylaxis to the chemotherapy's emetogenicity. Acute phase = during chemo to 24 h after the last dose; delayed phase follows. 5HT3RA = ondansetron/granisetron (1st-gen) or palonosetron (2nd-gen).

Highly emetogenic (HEC) — acute

• 5HT3RA + dexamethasone + (fos)aprepitant.
• Can't give aprepitant → palonosetron + dexamethasone. Can't give dexamethasone → palonosetron + (fos)aprepitant. Neither → palonosetron alone.

HEC — delayed

• Prefer palonosetron in the acute phase if high delayed-risk; continue oral aprepitant in delayed phase if started acutely; add dexamethasone if acute drug was granisetron/ondansetron.

Moderately emetogenic (MEC)

• Acute: 5HT3RA + dexamethasone (substitute (fos)aprepitant or palonosetron, or add olanzapine, if dexamethasone can't be given).

Low / minimal

• Low: a 5HT3RA alone. Minimal: no routine prophylaxis.

Call the FELLOW for

• Any chemotherapy issue, methotrexate / antibody patients.
• Non-neutropenic antibiotic decisions, penicillin allergy.
• K replacement in TLS-risk patients; before ordering rasburicase.
• Hyponatremia management; hematuria needing ↑hydration; any significant decline.

Call CCRT / Code Blue for

• Hypotension on/after the 2nd bolus; shock; any patient needing CCRT.
• Refractory status epilepticus or status dystonicus; airway/respiratory compromise.

Other consults

• Neurosurgery — raised ICP, cord compression (call before oncology for cord compression).
• Anaesthesia + ICU — mediastinal mass / SVC. Urology — obstructive hematuria. Nephrology — refractory hypertension. IGT / Vascular Access — line problems.

At onset

• Support ABCs + oxygen; cardiac + SpO₂ monitors; IV access.
• Rapid glucose; consider critical labs (Na, iCa, Mg).
• Watch for loss of airway reflexes, respiratory depression, hypotension, arrhythmia.
• Check drug allergy / drug-induced seizures (phenytoin/fosphenytoin contraindicated in drug-induced seizures).

1st line — benzodiazepine (first 15 min)

• Lorazepam IV/SL/buccal/PR 0.1 mg/kg (max 4 mg) — buccal preferred over SL. Repeat once after 5 min.
• OR Diazepam IV 0.3 mg/kg (IV max <5y 5 mg, ≥5y 10 mg) / PR 0.5 mg/kg (max 20 mg).
• OR Midazolam IN 0.2 mg/kg (max 5 mg/nostril) / buccal 0.5 mg/kg (max 10 mg) / IM 0.3 mg/kg (max 10 mg).

2nd line — seizures >15 min (equivalent efficacy)

• Levetiracetam IV 60 mg/kg (max 4500 mg) over 5–15 min.
• OR Fosphenytoin 20 mg PE/kg (max 1500 mg PE) over 5–10 min (or Phenytoin 20 mg PE/kg in NS over 20–30 min).
• OR Phenobarbital 20 mg/kg over 5–10 min (max 1000 mg) — preferred for refractory febrile seizures <6 mo or drug-induced; MD must monitor.
• OR Valproic acid 30 mg/kg (avoid in liver/mitochondrial disease) / Lacosamide 10 mg/kg over 15 min.
• Repeat with a different 2nd-line agent.

History & exam / triggers

• Underlying etiology & baseline of dystonia, prior episodes/management, recent medication changes, infection signs, painful stimuli, constipation.
• Full vitals + cardiorespiratory monitoring; respiratory status; confirm dystonic movements; skin/line sites; MSK/joint; genitalia (e.g. testicular torsion).
• Common triggers: infection/fever, new meds/changes, constipation or urinary retention, pain or trauma.
• Bloods: CK, renal biochemistry.

Supportive

• Analgesia for fever/pain (Tylenol ± Advil), IV fluids, comfort measures, remove harmful items, minimize handling, full cardiorespiratory + SpO₂ monitoring, IV access.

Pharmacological steps

• Step 1 — Diphenhydramine 1 mg/kg PO/IV/IM (max 50 mg).
• Step 2 — Diazepam 0.1 mg/kg PO/IV (max 5 mg).
• Step 3 — Clonidine 1 mcg/kg PO (max 100 mcg).
• Step 4 — Chloral hydrate 50 mg/kg PO/PR (max 2 g).
• Persists 15 min after each IV / 30 min after each PO-PR dose → next step. Repeat steps 2–4 once.

The complete 23-page paediatric medical emergency reference, viewable within the app.