What preventive measure could have avoided the death of a 9-year-old boy with a history of recurrent abdominal pain, hematuria, hypotension, tachycardia, and severe anemia, who had not received routine vaccinations due to concerns about autism?

From the Guidelines

The patient's death may have been prevented by periodic blood transfusions, which are a crucial component of managing sickle cell disease. This patient likely had sickle cell disease, which would explain his history of recurrent hospitalizations for abdominal pain, hematuria, severe anemia with high reticulocyte count (indicating hemolysis), and rapid clinical deterioration leading to death. Sickle cell disease patients benefit from regular blood transfusions to prevent complications like acute chest syndrome, stroke, and other vaso-occlusive crises. Transfusions increase the proportion of normal hemoglobin, reduce the percentage of sickled cells, improve oxygen delivery to tissues, and decrease blood viscosity. The patient's severe anemia (hematocrit 22%) with high reticulocyte count (12%) indicates his body was trying to compensate for ongoing hemolysis. Folic acid supplementation (option A) is useful in sickle cell disease but wouldn't prevent acute crises. The vaccination options (B, C, D) would be important for preventing specific infections but wouldn't address the underlying hemoglobinopathy that likely caused this patient's death, as noted in guidelines for immunization 1 and treatment of related conditions 1. Regular transfusion therapy, along with comprehensive care, represents the standard of care that could have prevented this tragic outcome. Key points to consider in this case include:

• The patient's history of recurrent hospitalizations for abdominal pain and hematuria
• The presence of severe anemia with a high reticulocyte count
• The importance of regular blood transfusions in managing sickle cell disease
• The role of comprehensive care, including hydroxyurea and infection prophylaxis, in preventing complications.

From the Research

Patient's Condition

The patient is a 9-year-old boy with a history of poorly localized abdominal pain, hematuria, and low hematocrit and reticulocyte count, suggesting a possible diagnosis of sickle cell disease.

Prevention of Death

The patient's death may have been prevented by:

• Vaccination with a conjugate capsular polysaccharide, specifically a pneumococcal conjugate vaccine (PCV) 2, 3, 4, 5
  • PCVs have been shown to be highly effective in preventing invasive pneumococcal disease in children with sickle cell disease
  • The 13-valent PCV (PCV13) has been shown to improve the breadth and magnitude of antibody responses against a large range of pneumococcal serotypes in adults with sickle cell disease 4
  • The use of PCV13 followed by a 23-valent polysaccharide vaccine (PPSV23) has been shown to be effective in preventing invasive pneumococcal disease in children with sickle cell disease 5

Other Options

The other options are not directly supported by the evidence:

• Folic acid supplementation: while important for patients with sickle cell disease, it is not directly related to the prevention of invasive pneumococcal disease
• Vaccination with a live attenuated virus: not relevant to the prevention of invasive pneumococcal disease
• Vaccination with a bacterial toxoid: not relevant to the prevention of invasive pneumococcal disease
• Periodic blood transfusions: while important for managing sickle cell disease, it is not directly related to the prevention of invasive pneumococcal disease