HUS Surveillance Notes—Sarah’s Story

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Hemolytic uremic syndrome (HUS) is a rare disorder characterized by microangiopathic hemolytic anemia, microthrombi, and multiorgan injury. Although infection with Shiga toxin–producing Escherichia coli is the most frequently identified cause, Streptococcus pneumoniae is increasingly recognized as a cause of HUS, and increasing illness and death have been documented for this rare precipitant (1). Cases of pediatric HUS are ascertained prospectively from an active, ongoing national surveillance program (2). Clinicians approach parents to seek consent for study enrollment when they deem the child is clinically stable. Parents of children affected by HUS are given a patient information sheet, a consent form, and a questionnaire if consent is given. As part of the surveillance, parents are asked to answer several open-ended questions at year 0, and 1 and 5 years after enrollment. Questions pertain to the status of the child’s health and whether visits to doctors and ongoing hospital treatments are still needed. The questionnaire also asks parents to assess their child’s illness since the initial visit to the hospital and the effects of this illness on the family. In 2006, a 1-year-old girl was admitted to a pediatric hospital in Glasgow, Scotland, because of microbiologically confirmed pneumococcal infection and superimposed HUS. Such cases are typically characterized by empyema, meningitis, and bacteremia in children, and also by major renal and neurologic injury requiring extensive dialysis (3). Unfortunately, this was true for Sarah. The following account is the child’s story as told by her mother. Year 1 The pneumococcal infection that caused Sarah’s HUS left her severely brain damaged. She has virtually no voluntary movement, cannot walk, talk, speak, or sit unaided, and she cannot feed orally. Sarah attends physiotherapy and speech and language therapy at a hospital for rehabilitation after her brain injury. It is hard to know how much of Sarah’s disability was caused by HUS and how much by the pneumococcal meningitis we also believe she had. Obviously, having a severely disabled child has affected the family. I have given up work to be Sarah’s full-time caregiver. We also lead a different sort of family life from what we had hoped to have, although our second child is due to be born at the end of the month. We are so proud of Sarah for making it through to celebrate her second birthday last week.