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Sickle Cell Crisis in a Paediatric Patient.

Sickle Cell Crisis in a Paediatric Patient.

Patient details
Patient details.
A, a 9-year-old girl Location: Rural hospital, Northern Nigeria, Sub-Saharan Africa
Patient case fields
Presenting Complaint

Severe abdominal pain and difficulty breathing for the past 24 hours

History

A, a known sickle cell anaemia (HbSS) patient, presented to the emergency department with complaints of severe abdominal pain, joint pain (especially in her knees and elbows), and shortness of breath. The pain started suddenly the previous day and has progressively worsened. She has had multiple similar episodes in the past, usually triggered by infections or dehydration, and was hospitalized twice in the last year for pain crises.

Her mother reports that A had a recent history of fever, but due to limited access to healthcare, she had not received treatment. She has been more fatigued than usual and unable to attend school for the past week.

Examination

Vital Signs:

Temperature: 38.7°C (feverish)

Heart rate: 120 beats per minute (tachycardia)

Respiratory rate: 32 breaths per minute (tachypnea)

Oxygen saturation: 88% on room air (hypoxia)

General: Appears pale, irritable, and in obvious distress due to pain.

Abdominal exam: Diffuse tenderness, most pronounced in the upper right quadrant. Hepatomegaly noted.

Musculoskeletal: Swelling and tenderness in the elbows and knees.

Chest exam: Reduced air entry bilaterally with coarse breath sounds.

Investigations

Full blood count: Haemoglobin 6.2 g/dL (low), reticulocyte count elevated.

Chest X-ray: Bilateral pulmonary infiltrates suggestive of acute chest syndrome.

Blood cultures: Pending, but considering possible bacterial infection.

Diagnosis

A is experiencing an acute sickle cell crisis, complicated by acute chest syndrome and likely bacterial infection (possibly pneumonia). Her crisis was likely triggered by the fever and dehydration due to limited access to clean drinking water and healthcare.

Management Plan
  1. Pain management: Administer IV fluids and morphine for pain control.
  2. Oxygen therapy: Initiate oxygen to maintain SpO2 > 94%.
  3. Antibiotics: Start broad-spectrum antibiotics to cover for potential bacterial infection.
  4. Blood transfusion: Prepare for a transfusion due to severe anaemia and hypoxia.
  5. Hydration: Administer IV fluids to prevent further sickling of red blood cells.
  6. Monitoring: Close monitoring of respiratory status and continuous pain assessment.
Outcome

With prompt treatment, A's pain gradually subsided, and her oxygen saturation improved. She received a blood transfusion, and after several days of hospitalization, her condition stabilized. However, her family was advised on regular follow-ups and the importance of early intervention during future crises. Access to preventive care, such as vaccinations and prophylactic antibiotics, was limited, but community health workers were engaged to ensure closer monitoring in the future.

Discussion
No data was found
Questions
1. What is the most likely trigger for A’s current sickle cell crisis?

a). Trauma

b). Fever and dehydration

c). Excessive physical activity

d). Poor nutrition

2. Which complication of sickle cell anaemia is A most likely experiencing based on her respiratory symptoms and chest X-ray findings?

a). Pneumonia

b). Acute chest syndrome

c). Pulmonary embolism

d). Asthma exacerbation

3. What is the most appropriate initial management for A’s severe pain?

a). Non-steroidal anti-inflammatory drugs (NSAIDs)

b). IV fluids and morphine

c). Oral acetaminophen

d). Cold compresses

4. Why is a blood transfusion recommended for A in this case?

a). To prevent further infections

b). To reduce fever

c). To correct severe anaemia and hypoxia

d). To treat joint pain

Reveal answers

Answers

  1. b) Fever and dehydration
  2. b) Acute chest syndrome
  3. b) IV fluids and morphine
  4. c) To correct severe anaemia and hypoxia