HJAR May/Jun 2021

42 MAY / JUN 2021 I  HEALTHCARE JOURNAL OF ARKANSAS CHILDREN’S HEALTH COLUMN CHILDREN’S HEALTH termine what is “normal”menstrual bleed- ing. It is not uncommon to obtain a history of hysterectomies in several women in a family at an early age due to complications of DUB. Diagnosing von Willebrand’s Disease VonWillebrand’s disease is diagnosed by measuring the amount of VW protein and how it functions as well as the multimers. Once diagnosed, education and treatment plans should be discussed with a patient. Often just trying to minimize sources of the bleed is enough, such as minimizing certain contact sports or prescribing estrogen for girls with DUB. But when surgery is needed or there is amore severe form that results in prolonged or profuse bleeding, VW protein replacement is needed. Currently, the only approvedmedication must be given intravenously. Stimate is EVERYONE has had a bruise or a nose- bleed at one time or another. But when should you become concerned that the bruising and bleeding could be due to an inherited condition? And if it is, is there anything you can do about it? There are several inherited disorders that increase the chances of bleeding and/or bruising. The most common is von Wille- brand’s (VW) disease. The literature states this disorder may be as common as 0.1-1% of the population. For the state of Arkan- sas, that would mean between 30,000 and 300,000 people could be affected. What is von Willebrand’s Disease? When a blood vessel is injured, platelets are activated and need to attach to the in- jured site and to each other to plug the hole in the vessel. The VWprotein forms the net that holds the platelets together. The pro- tein is similar to a folded fishing net that, when needed, is cast out into the blood stream, opens up into a larger net and is able to bind to many platelets to hold them there. If there is an insufficient amount of protein or the protein doesn’t work prop- erly, the platelet plug is unstable or too small and bleeding will recur or continue. The typical presentation is a child with a recently extracted tooth, which bleeds for a while, the bleeding stops, then recurs, stops again and recurs again. This is be- cause the clot is unstable and keeps falling off and allowing the injured site to rebleed. Another typical example is a young girl with dysfunctional uterine bleeding (DUB). This is bleeding that appears to be more severe or of greater duration than what is considered normal. The problem many families and clinicians face is trying to de- Inherited Disorders that Increase the Chances of Bleeding & Bruising