Sickle Cell Crisis
1) History of Present Illness
- Mr. R, a 28-year-old male, known case of Sickle Cell Disease (HbSS), presented to the ER with Severe generalized bone pain (especially in long bones, lower back, chest) for 2 days, worsening despite home analgesics.
- Associated symptoms: Fever (38.5 °C), shortness of breath, fatigue, dark urine, poor oral intake. No trauma, no new medication intake, no recent travel.
- Trigger history: Recent dehydration after long hours working outdoors in hot sun.
2) Past Medical & Drug History
- Known Sickle Cell Disease since childhood, multiple previous admissions for vaso-occlusive crisis.
- Medications:
- Hydroxyurea 1g daily → ↑ fetal Hb → prevents sickling.
- Folic Acid 5 mg daily → supports erythropoiesis.
- Occasional NSAIDs at home for pain.
- Previous complication: Splenectomy at age 12 due to autosplenectomy (repeated sequestration crises).
3) Examination on Admission (ER)
- Vitals:
- Temp: 38.5 °C
- HR: 128 bpm (tachycardia)
- BP: 90/60 mmHg (borderline hypotension)
- RR: 32/min, SpO₂: 84% on room air → ↑ with oxygen
- General: Appears very distressed, pale, icteric sclera, dehydrated.
- Systemic findings:
- Respiratory: Bilateral coarse crepitations (suggestive of acute chest syndrome)
- CVS: Tachycardia, no murmurs.
- Abdomen: Mild hepatomegaly, no splenomegaly.
- Extremities: Tender long bones, warm, no swelling
4) Initial Investigations (Day 1 in ER/ICU)
| Investigation | Result | Interpretation |
| CBC | Hb 6.8 g/dL, WBC 16,500, Platelets 180k | Severe anemia, leukocytosis (infection/inflammation) |
| Reticulocyte count | 12% | Bone marrow compensating |
| LDH | ↑ | Hemolysis marker |
| Peripheral smear | Sickled RBCs, Howell–Jollybodies | Sickle crisis + asplenia |
| ABG | pH 7.31, PaO₂ 58 mmHg, HCO₃⁻18 | Metabolic acidosis + hypoxemia |
| Chest X-ray | Bilateral infiltrates | Acute chest syndrome |
| RFT | Creatinine 1.8 mg/dL | Early AKI (pre-renal dehydration hemolysis) |
| LFT | Indirect bilirubin ↑ | Hemolysis |
| Blood culture | Pending | Suspected sepsis (risk ↑ postsplenectomy) |
5) ICU Management (Day-wise)
- Day 1 – ICU Admission
- O₂ therapy via NRBM → SpO₂ improved to 95%.
- IV fluids: cautious hydration (NS @ 80 mL/hr) to prevent worsening pulmonary edema.
- Analgesia: IV Morphine infusion titrated to pain score.
- Antibiotics: IV Ceftriaxone + Azithromycin (cover sepsis + atypical pneumonia in acute chest).
- Blood transfusion: Simple transfusion → 2 units PRBC (target Hb > 9 g/dL).
- Monitoring: Invasive line for CVP & ABG monitoring.
- Day 2
- Fever persisted, tachycardia improved slightly.
- Still requiring high O₂.
- Repeat CBC: Hb 8.2, WBC 14,000.
- Decision → Exchange transfusion (since acute chest + hypoxemia not improving).
- Aim: Reduce HbS < 30%.
- Analgesia continued, IV hydration balanced with CVP.
- Started LMWH prophylaxis (risk of thrombosis).
- Day 3
- Patient showed improvement in oxygenation (SpO₂ 97% with NC O₂).
- Pain score decreased (on morphine taper).
- Hb stabilized at 10.1 g/dL post exchange transfusion.
- Cultures grew Streptococcus pneumoniae → antibiotics switched to IV Ceftriaxone high dose.
- RFT stable, urine output maintained.
- Day 4
- Patient mobilized with physiotherapy.
- O₂ requirement reduced further.
- Analgesics switched to oral Tramadol + Paracetamol.
- IV fluids tapered.
- Started incentive spirometry (prevent pulmonary collapse).
- Day 5 – Step-down to Ward
- Afebrile, hemodynamically stable, pain under control.
- Switched to oral antibiotics + home meds (Hydroxyurea, folic acid).
- Planned discharge after 48 hours of ward observation.
6) Discussion / Learning Points
- Sickle cell crisis in ICU can present with vaso-occlusive pain + acute chest syndrome + hemolysis.
- Exchange transfusion is lifesaving in acute chest syndrome.
- Antibiotic prophylaxis and vaccination are crucial post-splenectomy.
- ICU management focuses on O₂, fluids, pain control, antibiotics, transfusion strategy.
7) Case Storytelling (Memory Hook)
“A young man collapsed from unbearable bone pain after working in the sun. His oxygen dropped, and his X-ray showed cloudy lungs — acute chest syndrome. Despite morphine, oxygen, and simple transfusion, he worsened, until an exchange transfusion flushed out his sickled cells. By Day 3, his lungs cleared, and his life was pulled back from danger.”