Sickle Cell Vaso-Occlusive Crisis
Patient Information
Initials: A.B.
Age: 22 years
Sex: Male
Race/Ethnicity: African American
Source and Reliability:
The patient provided the history. Reliability is good; the patient was alert, oriented, and consistent in reporting symptoms.
Subjective:
Chief Complaint (CC):
“My whole body hurts, especially my back and legs.”
History of Present Illness (HPI):
A.B. is a 22-year-old African American male with a known history of sickle cell disease (HbSS) who presents with severe pain consistent with a vaso-occlusive crisis. Symptoms began approximately 24 hours prior to presentation and have progressively worsened. Pain is deep, throbbing, and aching in quality, primarily localized to the lower back, hips, and bilateral lower extremities, with intermittent radiation to the arms. Associated symptoms include fatigue, nausea, decreased oral intake, and mild shortness of breath. The pain is constant, exacerbated by movement, dehydration, and cold exposure, and minimally relieved by home use of ibuprofen and increased oral fluids. Pain severity is reported as 9/10. The patient reports poor hydration over the past two days due to nausea. He denies fever, cough, chest pain, neurologic deficits, or recent infection. He reports multiple prior hospitalizations for similar pain crises, with the most recent admission occurring six months ago.
Current Medications:
- Hydroxyurea 1,000 mg orally once daily for sickle cell disease
- Folic acid 1 mg orally once daily
- Ibuprofen 600 mg orally every 6 hours as needed for pain
Allergies:
- No known drug allergies
- No food or environmental allergies reported
Past Medical History (PMH):
- Sickle Cell Disease (HbSS), diagnosed in early childhood
- Recurrent vaso-occlusive pain crises
- Acute chest syndrome (2021)
- Chronic anemia
- Immunizations up to date per patient report (including pneumococcal and influenza vaccines)
- Last dental exam: 1 year ago
- Last eye exam: Over 2 years ago
Family History:
- Mother: Sickle cell trait
- Father: Hypertension
- Maternal grandmother: Type 2 diabetes
- Maternal grandfather: Hypertension
- Paternal grandmother: No known medical conditions
- Paternal grandfather: Deceased, cause unknown
Personal and Social History:
- Lives with family
- College student
- Denies tobacco, alcohol, or illicit drug use
- Diet: Poor intake over the past several days due to pain
- Exercise: Limited during pain episodes
- Reports good family support
Review of Systems (ROS):
- General: Reports severe pain and fatigue; denies fever or chills
- HEENT: Denies headache, vision changes, sore throat, or nasal congestion
- Skin: Denies rashes, ulcers, or wounds
- Cardiovascular: Denies chest pain or palpitations
- Respiratory: Reports mild shortness of breath; denies cough or wheezing
- Gastrointestinal: Reports nausea and decreased appetite; denies vomiting or abdominal pain
- Genitourinary: Denies dysuria or hematuria
- Musculoskeletal: Reports severe back, hip, and leg pain
- Neurologic: Denies weakness, numbness, dizziness, or speech changes
- Psychiatric: Reports distress related to uncontrolled pain
- Endocrine: Denies heat or cold intolerance
- Hematologic/Lymphatic: Denies easy bruising or lymphadenopathy
Objective:
Vital Signs:
- Blood Pressure: 128/76 mmHg
- Heart Rate: 102 beats per minute
- Respiratory Rate: 22 breaths per minute
- Temperature: 99.1°F (oral)
- Oxygen Saturation: 94% on room air
- Height: 5 feet 9 inches
- Weight: 155 pounds
- BMI: 22.9 kg/m² (normal)
Physical Examination:
General: Appears uncomfortable, guarding movements due to pain—Alert and oriented ×4.
Head: Normocephalic and atraumatic.
Eyes: Pupils equal, round, and reactive to light; conjunctivae pale.
Ears/Nose/Throat: Mucous membranes slightly dry; oropharynx without lesions.
Neck: Supple; no lymphadenopathy.
Cardiovascular: Tachycardic; regular rhythm; no murmurs, rubs, or gallops.
Respiratory: Breath sounds clear bilaterally; mild tachypnea; no wheezes or crackles.
Gastrointestinal: Abdomen soft, non-tender, non-distended; bowel sounds present.
Genitourinary: Deferred.
Musculoskeletal: Diffuse tenderness over the lumbar spine and bilateral lower extremities; decreased range of motion secondary to pain.
Skin: Warm, dry, intact; no ulcers or rashes noted.
Neurologic: Cranial nerves II–XII intact; strength 5/5 though limited by pain; sensation intact.
Psychiatric: Mood anxious; affect appropriate; thought process logical.
Assessment:
Primary Diagnosis:
Sickle Cell Vaso-Occlusive Crisis
ICD-10 Code: D57.00
Pathophysiology:
Vaso-occlusive crisis results from polymerization of deoxygenated hemoglobin S, leading to red blood cell sickling, microvascular obstruction, ischemia, and inflammation. These processes cause severe pain and can result in organ damage if untreated (Borhade et al., 2024). Common triggers include dehydration, hypoxia, infection, and stress.
Clinical Rationale:
The patient’s known diagnosis of sickle cell disease, acute onset of severe musculoskeletal pain consistent with prior crises, dehydration, tachycardia, and absence of infectious symptoms support the diagnosis of a vaso-occlusive crisis.
Differential Diagnoses (Prioritized):
- Acute Chest Syndrome (D57.01): Considered due to mild shortness of breath but less likely given absence of fever, chest pain, or pulmonary infiltrates (Friend et al., 2023).
- Osteomyelitis (M86.9): Considered but unlikely due to diffuse pain and lack of localized swelling or fever (Nimmana & Savaliya, 2023).
- Deep Vein Thrombosis (I82.409): Less likely given bilateral and diffuse pain without swelling or erythema (Waheed et al., 2023).
Plan:
Each diagnosis listed in the assessment is addressed below through diagnostic evaluation, pharmacologic and nonpharmacologic interventions, patient education, and follow-up planning.
Diagnostics and Laboratory Studies:
- Complete blood count with differential
- Reticulocyte count
- Comprehensive metabolic panel
- Lactate dehydrogenase and total bilirubin
- Chest X-ray if respiratory symptoms worsen
Pharmacologic Treatment:
- IV morphine per institutional pain protocol, titrated to pain control (Brihi & Pathak, 2024)
- IV ketorolac as adjunct analgesia
- IV normal saline for hydration
- Continue hydroxyurea therapy
Nonpharmacologic Interventions:
- Supplemental oxygen to maintain SpO₂ ≥95%
- Warm compresses to painful areas
- Bed rest and limited physical exertion
Patient Education:
- Importance of maintaining adequate hydration
- Medication adherence, including hydroxyurea
- Early recognition of pain crisis symptoms
- Avoidance of known triggers such as dehydration and extreme temperatures (Obeagu & Obeagu, 2024)
Safety and Follow-Up:
- Monitor closely for signs of acute chest syndrome
- Admitted for pain management and observation
- Hematology consultation
- Follow up with the hematologist within one week of discharge
Reflection:
The case highlighted the need to recognize and vigorously handle vaso-occlusive crises to avoid the development of complications such as acute chest syndrome and organ damage. I gained the importance of using pharmacologic pain treatment hand-in-hand with supportive interventions, such as fluid and oxygen therapy. This experience solidified the necessity to pay attention to social determinants of health, including access to hydration and early medical care, when designing an extensive plan of care for patients with sickle cell disease.
References
Brihi, J. E., & Pathak, S. (2024, June 8). Normal and abnormal complete blood count with differential. StatPearls – NCBI Bookshelf. https://www.ncbi.nlm.nih.gov/books/NBK604207/
Borhade, M. B., Patel, P., & Kondamudi, N. P. (2024, February 25). Sickle cell crisis. StatPearls – NCBI Bookshelf. https://www.ncbi.nlm.nih.gov/books/NBK526064/
Friend, A., Settelmeyer, T. P., & Girzadas, D. (2023, November 25). Acute chest syndrome. StatPearls – NCBI Bookshelf. https://www.ncbi.nlm.nih.gov/books/NBK441872/
Nimmana, B. K., & Savaliya, V. (2023, May 31). Osteomyelitis. StatPearls – NCBI Bookshelf. https://www.ncbi.nlm.nih.gov/books/NBK532250/
Obeagu, E. I., & Obeagu, G. U. (2024). Managing emotional and physical stress in sickle cell anemia: a review of effective strategies and approaches. Annals of Medicine and Surgery, 87(3), 1370–1382. https://doi.org/10.1097/ms9.0000000000002748
Waheed, S. M., Kudaravalli, P., & Hotwagner, D. T. (2023, January 19). Deep venous thrombosis. StatPearls – NCBI Bookshelf. https://www.ncbi.nlm.nih.gov/books/NBK507708/