Emergency Room Visits in Children with Hemophilia

The emergency room (ER) management of bleeding and other complications of hemophilia constitutes an important component of hemophilia therapy. In this retrospective study, we examined the ER visits of children with hemophilia during a five-year period.

Electronic medical records of all ER visits to our hospital were reviewed for hemophilia patients aged 0–21 years between January 1st, 2006- December 31st, 2010. ER visits were categorized as visits related to injury or bleeding; visits related to fever or a positive blood culture in a patient with central venous catheter (CVC); visits for general pediatric causes unrelated to hemophilia; and visits for clotting factor infusion.

There were 518 ER visits from 79 male patients (71 hemophilia A and 8 hemophilia B) over the 5-year period. Median age was 5 years (range 0–21). Five patients had other chronic conditions in addition to hemophilia: sickle cell anemia in 2 patients and Down syndrome, Crohn disease, and myelomeningocele in one patient each.

The reasons for ER visits were as follows: 60.8% (n=315) were for injury and/or bleeding; 12.2% for either fever in a patient with CVC (n=53) or positive results of a blood culture drawn earlier from a CVC (n=10); 18.0% (n=93) for pediatric causes unrelated to hemophilia; and 9.1% (n=47) for clotting factor infusion. Four of the visits were because of a bleeding episode in patients not yet diagnosed with hemophilia.

Computerized tomographic (CT) examination of the head was undertaken in 85 of the visits: trauma to the head (n=68), face (n=2), nose (n=1), or eyes (n=2); after falling backward (n=1) and motor vehicle accident (n=2) without a reported head injury; other symptoms such as headache (n=6), lethargy (n=1), pallor (n=1), and vomiting (n=1). In 6 patients with head injury, the cranial CT examination was deferred because of a normal neurologic examination. Only 4.7% (n=4) of the cranial CT examinations showed intracranial hemorrhage. These patients had presented with head trauma (n=2), vomiting (n=1), and headache (n=1).

Of the 53 febrile visits in patients with CVC, blood cultures grew an organism in 43.4% (n=23) of the cases. As two of the visits for a positive blood culture were for cultures drawn in the ER the day before, there were a total of 31 visits with a new positive blood culture from a CVC: 14 with single gram-positive bacteria, 9 with single gram-negative bacteria, and 8 with multiple organisms, including two with Candida species.

Children with hemophilia present to the ER mostly for bleeding or injury-related reasons. Head injury or headache may prompt a cranial CT examination in patients with hemophilia; however, only a minority of such patients has intracranial hemorrhage, and a good neurologic assessment should help determine patients who require this investigation. Fever in a hemophiliac patient with CVC is a serious symptom, as almost half of such children might have bacteremia. Although uncommon, undiagnosed hemophilia patients may present with their first hemorrhagic episode to the ER, and appropriate screening tests should be ordered in the event of a clinical suspicion

No relevant conflicts of interest to declare.