Patient handout

Sickle Cell Disease (chronic management + acute crisis)

PRODUCTION

1. Your condition

This handout is for sickle cell disease (chronic management + acute crisis). Your care team identified this based on: vaso-occlusive pain crisis (ash 2020; nhlbi 2014).

Other reasons your team may use this plan: fever in scd (functional asplenia — sepsis until proven otherwise) (ash 2020); chest pain / dyspnea / hypoxia → acs workup (ash 2020); priapism >4h (ash 2020).

2. Your medications

Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.

Medication	Starting dose	How	When	What it does
hydroxyurea	—	—	—	MSH NEJM 1995; BABY HUG; ASH 2020; foundation of DMT
l_glutamine	—	—	—	BEACON NEJM 2018
crizanlizumab	—	—	—	SUSTAIN NEJM 2017 — STAND phase 3 FAILED; EU/UK revoked 2023–2024; clinical benefit questioned
exa_cel_casgevy	—	—	—	FDA approval Dec 2023; one-time CRISPR-edited autologous HSC
lovo_cel_lyfgenia	—	—	—	FDA approval Dec 2023; lentiviral β-globin

Plan: Disease-modifying therapy (chronic)

3. Your action plan

Use these zones to know what to do based on how you feel.

GREEN — Stable — feeling well

If you have:

No pain or pain at baseline (ASH 2020)
No fever (ASH 2020)
Eating and drinking well (NHLBI 2014)
Taking hydroxyurea/L-glutamine as prescribed (ASH 2020)

Do this:

Continue hydroxyurea/L-glutamine as prescribed (ASH 2020)
Stay well hydrated (3-4 L/day for adults) (NHLBI 2014)
Avoid extreme cold, high altitude, dehydration (NHLBI 2014)
Continue penicillin (peds) and all vaccinations (NHLBI 2014; NASCC 2024)
Keep all clinic appointments (NASCC 2024)

YELLOW — Caution — early crisis warning

If you have:

Mild pain in usual sites (legs, back, chest, abdomen) (NHLBI 2014)
Fatigue more than usual (NHLBI 2014)
Decreased appetite (NHLBI 2014)
Slight fever (<38.5 C) (ASH 2020)
Mild URI symptoms (ASH 2020)

Do this:

Increase fluids (NHLBI 2014)
Take home pain regimen as prescribed (NSAIDs cautiously, opioids per home plan) (NHLBI 2014)
Continue all baseline medications (ASH 2020)
Use heat packs to affected areas (NHLBI 2014)
Call SCD clinic within 4-8 hours (ASH 2020)
If home regimen ineffective in 4 hours → ED (ASH 2020)

Call your provider if:

Pain not controlled with home regimen (NHLBI 2014)
Persistent fever (ASH 2020)
Cough or shortness of breath (ASH 2020)
Decreased urine output (NHLBI 2014)

RED — Medical alert — go to ED now

If you have:

Severe pain not controlled by home regimen (ASH 2020)
Fever ≥38.5 C (ASH 2020; NHLBI 2014)
Chest pain or trouble breathing (ASH 2020)
Acute neurologic symptoms (weakness, slurred speech, confusion, vision changes) (ASH 2020)
Priapism (sustained erection >2 hours) (ASH 2020)
Severe abdominal pain or sudden splenic enlargement (NHLBI 2014)
Sudden worsening of anemia (severe pallor, dizziness) (NHLBI 2014)

Do this:

Go to ED immediately (ASH 2020)
Transfer criteria — request transfer to SCD-specialized center if local ED limited (ASH 2020)
Bring home medication list and last clinic notes (ASH 2020)
Tell ED you have SCD genotype (HbSS/HbSC/HbS-beta-zero/HbS-beta-plus) (ASH 2020)
Request hematology consult (ASH 2020)

Call your provider if:

Any red zone symptom — go to ED immediately (ASH 2020)

4. When to seek emergency care

Call 911 or go to the nearest emergency room right away if you have:

Temperature ≥38.5°C in SCD patient (ASH 2020; NHLBI 2014)
Fever + cough/dyspnea/hypoxia + new pulmonary infiltrate (ASH 2020)(life-threatening)
Acute neurologic deficit in SCD patient (ASH 2020)(life-threatening)
Sustained priapism >4 hours (ASH 2020)
Acute Hb drop >2 below baseline + splenomegaly + pancytopenia/cytopenia (NHLBI 2014)
Hb drop with absolute reticulocyte count <1% (NHLBI 2014)
Acute organ dysfunction in 2+ systems during VOC (ASH 2020)(life-threatening)

5. Follow-up

Health maintenance per NASCC 2024: vaccinations (penicillin prophylaxis ages <5), TCD, retinopathy screen, baseline echo, contraception/pregnancy planning, cognitive assessment, transition to adult care

6. Sources

Guideline: ASH 2020 SCD Guidelines (multiple Blood Advances) + NHLBI 2014 + NASCC 2024 Health Maintenance + ASH 2025 draft hydroxyurea