A CHILD WITH CHEST PAIN H
History
Rebekah is a 14-year-old Afro-Caribbean girl who presents to the A&E department complaining of pain in her chest and back. Her pain started this morning and has been worsening throughout the day, despite taking paracetamol, ibuprofen and codeine phosphate. She is finding it difficult to breath deeply and the pain is worse on inspiration. She has HbSS sickle cell disease and has been admitted to hospital three times in the last month with painful crises.
Examination
Rebekah’s temperature is 38.8C, her heart rate is 120 beats/min, blood pressure 135/85 mmHg, respiratory rate 40 breaths/min, and oxygen saturation 91 per cent in air. She is in pain and unable to take a deep breath. There are bronchial breath sounds at both lung bases. Heart sounds are normal. Her abdomen is soft and non-tender and her ears and throat are unremarkable.
INVESTIGATIONS
Normal
Haemoglobin 7.7 g/dL 12.1–15.1 g/dL
White cell count 19.9 109/L 4.5–13 109/L
Platelets 227 109/L 180–430 109/L
Neutrophils 12.8 109/L 1.5–6.0 109/L
Urea and electrolytes/creatinine Normal
Bilirubin 56 mmol/L 1.7–26 mmol/L
Alkaline phosphatase 118 IU/dL 25–125 IU/dL Aspartate aminotransferase 40 IU/dL 10–45 IU/dL
C-reactive protein 127 mg/L 5 mg/L
Chest radiograph – see Figure 50.1
Figure 50.1 Rebekah’s initial chest radiograph
Questions
• What is the most likely cause of the Rebekah’s chest pain?
• How should this be managed?
• What other complications can occur in children with sickle cell disease?
• How can infections be prevented in children with sickle cell disease?
Medicine
Rebekah has acute sickle chest syndrome. Thrombosis, infection and fat embolism to the lung produce a syndrome of pleuritic chest pain, shortness of breath and fever. The pathology often evolves from the lung bases and produces consolidation, which may be clinically apparent before radiographic changes appear. Hypoxia is a frequent feature, and failure to recognize and manage this syndrome aggressively may lead to a rapid deterioration. Compare the initial chest radiograph (which shows just a small patch of consolidation in the left lower lobe behind the heart) with the one taken 2 days later (which shows more extensive bi-basal consolidation – see Fig. 50.2).
Sickling of red blood cells and their sequestration in vessels is exacerbated by dehydration, hypoxia and acidosis, so these factors need to be corrected with hyperhydration and supplemental oxygen. Adequate analgesia (often intravenous morphine) and physiotherapy help the patient to breathe deeply enough to ventilate the affected areas of the lungs. Empirical antibiotic treatment should be started, e.g. co-amoxiclav and clarithromycin. Children with acute sickle chest syndrome should be discussed with a senior paediatrician and a haematologist. If they deteriorate, they may require ventilatory support (continuous positive airways pressure (CPAP) or intubation and ventilation). A top-up or exchange blood transfusion may also be needed to reduce the percentage of sickling red blood cells.
Complications of sickle cell disease in children
• Painful crises, avascular necrosis of the hips and shoulders
• Chest syndrome, abdominal syndrome, girdle syndrome
• Splenic sequestration, aplastic anaemic crisis
• Stroke, retinal vein occlusion
• Priapism, haematuria, enuresis, chronic renal failure
• Pigment gallstones, cholecystitis, biliary colic
• Hyposplenism, sepsis, osteomyelitis
• Delayed puberty
Children with sickle cell disease develop hyposplenism, which makes them particularly vulnerable to infection with encapsulated bacteria such as Haemophilus influenzae and Streptococcus pneumoniae. They should receive all routine immunizations, pneumococcal polysaccharide vaccine, and regular penicillin V prophylaxis. Hepatitis B vaccine is recommended, and if they travel to a malaria-endemic area, it is essential they take adequate prophylaxis.
KEY POINTS
• Sickle chest syndrome is an emergency. Early recognition and treatment can prevent severe consequences.
• Children with sickle cell disease are vulnerable to infections with encapsulated bacteria.
• Complications of sickle cell disease can affect any organ.
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