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Bleeding Disorder? National Expert Discusses Questions Around Cambridge Baby’s Death

Aisling Brady McCarthy

Aisling Brady McCarthy watches as her attorney addresses the court during a status hearing at Middlesex Superior Court in Woburn in May. McCarthy, a nanny from Ireland, was accused of killing a 1-year-old Massachusetts girl in her care two years ago. (Charles Krupa/AP)

Aisling Brady McCarthy is back in her native Ireland now, after murder charges against her were dropped in the death of Rehma Sabir, a Cambridge infant in her care. But while the case is over, the mystery remains: How did Rehma die?

The Middlesex County district attorney’s office says a review of the case raised the question of whether 1-year-old Rehma had an undiagnosed blood disorder that could have caused her brain hemorrhage. A press release from the office cites these details from the medical examiner:

“Review of Rehma’s coagulation and hematology testing, her history of bruising, the NIH guidelines for diagnosis of von Willebrand disease, and literature on the subject suggest to me that Rehma’s low von Willebrand factor could have made her prone to easy bleeding with relatively minor trauma.

“Given these uncertainties, I am no longer convinced that the subdural hemorrhage in this case could only have been caused by abusive/inflicted head trauma, and I can no longer rule the manner of death as a homicide.  I believe that enough evidence has been presented to raise the possibility that the bleeding could have been related to an accidental injury in a child with a bleeding risk or possibly could have even been a result of an undefined natural disease.  As such I am amending the cause and manner of death to reflect this uncertainty.”

So, then, a bleeding disease might have caused a spontaneous hemorrhage? Or the combination of the disease and a minor head injury led to death? And if so, could it be that quite a few of the contested accusations of baby abuse in recent years could be thus explained away?

Dr. Shannon Carpenter (courtesy)

Dr. Shannon Carpenter (courtesy)

I turned to Dr. Shannon Carpenter, a bleeding disorders specialist who has done research and written American Academy of Pediatrics reports on checking children for bleeding disorders when there’s a question of abuse. She’s the chief of hematology at Children’s Mercy Hospital in Kansas City, Missouri, and the director of the Kansas City Regional Hemophilia Center.

Dr. Carpenter was not involved in the Cambridge case, but speaking generally she says that sadly, no, there’s no kinder explanation here: Abusive head trauma — a better term than “shaken baby syndrome” because abuse can involve more than shaking — is far more common than serious bleeding disorders, and even children with severe disorders are extremely unlikely to have fatal brain bleeds. Our conversation, lightly edited:

Dr. Carpenter, what are we to make of the medical examiner’s mention of Von Willebrand disease — a not-very-rare bleeding disorder — and the suggestion that death could have resulted from disease or “an accidental injury in a child with a bleeding risk”?

One thing I would say is, most patients with von Willebrand disease have a mild disorder, mostly manifested by nosebleeds, bruising, and gum bleeding, and do not seem to have an increased risk for intracranial hemorrhage. Research is ongoing in this area and I think there may be more information coming, but from a clinical perspective, when we see patients with the most common types of von Willebrand disease, we do not have a high concern for intracranial hemorrhage in those patients, certainly not spontaneously.

“Unfortunately, child abuse is more common than bleeding disorders.”

– Dr. Shannon Carpenter

The most severe forms of von Willebrand disease occur in about 1 percent of people with von Willebrand disease, probably less than that. And even in the most severe forms, the risk of having a bleed inside of the head is probably less than 2 percent. So it’s a very rare event. This would be an unusual presentation for von Willebrand disease. I don’t know the specifics of this patient’s case, and I don’t know what her actual von Willebrand factor level was, but even if she was low enough to be diagnosed with the most common form of von Willebrand disease, this kind of bleeding would not be typical for that kind of diagnosis.

But, I suppose, possible?

While anything is possible, you have to look at what’s probable and what other patients have experienced. And patients with bleeding disorders are not immune to trauma, whether it’s inflicted or non-inflicted. I would say if a child came to me with type 1 von Willebrand disease, the most common type, the mild form of von Willebrand disease, with a typical toddler head bump that would not otherwise cause someone to seek medical care, I would not worry about intracranial hemorrhage in that patient.

And if a child had a more severe form of von Willebrand disease?

Then I might be concerned.

But if it were a severe form, wouldn’t it have likely shown up by 1 year of age? Continue reading