Nosebleeds are a common problem experienced by children, and in some families nosebleeds are more prevalent. As pediatric hematologists, there is not a week that passes that we don’t hear, “Everyone in my family had nosebleeds and easy bruising as a kid, but they outgrew it.” Or, “All the women on my mom’s side have really long, heavy periods. That runs in our family.” While these symptoms may be typical for a particular family, they could also indicate that your child has an inherited bleeding disorder.
Pediatric hematologists at the Hemophilia Treatment Center at UHealth - University of Miami Health System always consider von Willebrand Disease, or vWD, when evaluating a child with symptoms like nosebleeds and heavy menstrual cycles. vWD is the most common inherited bleeding disorder in the world, affecting both men and women equally. Although the name sounds frightening, this condition was named after Dr. Eric von Willebrand, who first described it in 1925 after working with a large family in Finland with severe and recurrent bleeding issues.
We now know that the disease is caused by a defect in the von Willebrand protein. If a person doesn’t make enough von Willebrand protein or makes a malfunctioning von Willebrand protein, strong clots are not formed, and affected people will experience easy bruising, nosebleeds, heavy periods, etc. von Willebrand protein is critical to the control of bleeding because it serves as a beacon for sticky platelets to clump together to form the first fragile clot. The protein also protects and attracts clotting proteins to the injured blood vessel to make the fibrin glue that makes the clot stronger.
If you are concerned that your child has vWD, the most important thing is to speak to your pediatrician and be prepared for a referral to pediatric hematology. Try to avoid giving your child aspirin, Advil, Motrin and Naprosyn as all these medications make platelets less sticky. Until you are sure that your child doesn’t have an inherited bleeding tendency, you want to make sure that his or her coagulation system is working as best as it can.
Confirming the diagnosis of vWD may be tricky. General screening tests of the coagulation system may appear normal. Despite a normal result, children with bleeding symptoms need to be referred to a pediatric hematologist. Blood will be taken to assess the amount and functionality of the von Willebrand protein.
Interestingly, these blood tests can be affected by stress. When we are anxious, like when your child panics at the sight of a needle, high adrenaline levels may temporarily increase our level of von Willebrand protein, providing incorrect normal results. Should that happen, the hematologist will likely recommend retesting at a future date.
Upon diagnosis, there will be a discussion about how to best manage and prevent the unusual or bothersome bleeding or bruising. This may include the use of medications to stabilize blood clots and increase the amount of von Willebrand protein in the bloodstream. It is also recommended that siblings and other family members be evaluated, especially if they have or have had vWD symptoms.
Having a vWD diagnosis also enables the patient, family and doctor to plan surgical procedures or dental extractions in a way that avoids excessive bleeding. For teenage girls and young women, knowing the diagnosis will make heavy menstrual periods much easier to manage and control.
Finally, Mom and Dad, relax. The identification of vWD will not only explain why a child is having bleeding issues, but will reassure you that your little one does not have a more serious disorder, such as leukemia or hemophilia. In addition, most people with vWD do not know they have it, and they live with only minor inconveniences caused by bruising or heavy periods. Now that you have a diagnosis, you can follow through with appropriate treatments, knowing that high-risk situations can be prevented through the proper medical care and that your child will grow up happy and healthy.
Fernando F. Corrales-Medina, M.D., is an assistant professor and Joanna A. Davis, M.D., is an associate professor in the Pediatric Comprehensive Hemophilia Treatment Center, Division of Pediatric Hematology/Oncology at UHealth – University of Miami Health System. For more information, visit UHealthSystem.com/patients/pediatrics.