Von Willebrand Disease In Children - All You Need To Know

Von Willebrand Disease In Children

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Does your child suffer from frequent nosebleeds and is she prone to easy bruising? Does she complain of excessive menstrual flow and does it take longer than usual for her wounds to heal? If you said yes, then you should take her to a doctor immediately. It can be Von Willebrand disease in children which is a genetic disorder.

Cuts and scrapes are common occurrences during childhood. Ideally, these wounds are minor and result in some blood loss. Basic first aid and a tight hug are all that your child needs. But for some children, even a small gash can cause serious consequences. So, read on and learn more about Von Willebrand disease- its causes, symptoms, and treatment below.

What is Von Willebrand Disease?

Von Willebrand Disease is the most common bleeding disorder, which affects around 1% of the population. Von Willebrand disease occurs when blood platelets do not stick to the walls of the blood vessels. Ideally, platelets stick to one another and cause clotting in wounds, which prevents further bleeding. Von Willebrand factor (vWD) is the protein that helps them glue together. Children with the Von Willebrand disorder either lack this protein, or the protein does not function properly, it leads to excessive bleeding.

[ Read: Hirschsprung Disease In Children ]

Dr. Eric Von Willebrand described the disorder in 1929. Although Von Willebrand is referred to as a single disease, it is a family of bleeding disorders. There are three different types of the disease:

1. Type I Von Willebrand Disease:

This is a mild and common variety of the disorder and accounts for about 80% of vWD cases. Children with this type of disorder may have normal von Willebrand factor, but it is not enough to help the blood clot. Sometimes, children may be low on another clotting protein called Factor VIII.

2. Type II Von Willebrand Disease:

This type is characterized by an abnormal von Willebrand factor protein composition.

3. Type III Von Willebrand Disease:

This is a severe variety (affects less than 1% of vWD cases3) and is characterized by severe bleeding. Children with this type of disorder have little or zero von Willebrand factor besides very little Factor VIII.

There are four different subtypes of the Type II variety and are offered referred as Type IIA, IIB, IIM, and IIN. Each of these categories has distinct clinical features and needs category-specific treatment. Without prompt diagnosis and effective management, vWD can be life threatening.

[ Read: Crohn’s Disease In Children ]

Causes Of Von Willebrand Disease In Children:

Von Willebrand Disease is caused by an inherited defect in the gene that controls clotting proteins like Von Willebrand in the body. To explain further:

  • Cells in our body are made up of structures called chromosomes. Each cell in the body contains 46 chromosomes arranged in 23 pairs. Von Willebrand disease in children occurs when there’s a defect in chromosome 12- this defect could either be from chromosome 12 from the mother, or the father, or both.
  • It could either be passed from a parent with the defective gene to the child although the parent may not have experienced symptoms of the disease during conception.
  • Sometimes genes undergo changes through a process called genetic mutation. In such cases, both the parents do not carry the genetic disorder, and neither are the parents’ other children likely to inherit the disease.
  • As earlier explained, vWD also occurs when the patient does not have enough of other clotting proteins like factor VIII.
  • And in extremely rare circumstances, vWD can occur later in life probably caused by hypothyroidism or reaction to certain medications like ciprofloxacin and Depakene (1).

Symptoms Of Von Willebrand Disease In Children:

It is difficult to diagnose vWD unless the patient experiences excessive bleeding during an accident or surgery. Similarly, symptoms often vary from person to person. For example, symptoms experienced by patients with Type I vWD are mild, but sometimes a patient with Type I may experience severe bleeding. But some of the commonly described symptoms of von willebrand disease in children include:

  • Nose bleeds frequently and longer than usual.
  • Heavy menstrual periods
  • Easy bruising
  • Bleeding during a dental procedure or when the baby teeth fall off.
  • Bleeding from the gums
  • In some cases, especially among patients with Type II vWD, bleeding can occur right from childbirth when the umbilical cord is cut or during circumcision.

[ Read: Kawasaki Disease In Children ]

Diagnosing Von Willebrand Disease In Children:

Diagnosing vWD is often delayed and difficult. It involves studying the patient’s family history (related to bleeding), analyzing the patient’s bleeding history, and looking for abnormal laboratory findings. Some of the commonly used tests include.

  • PTT- Also called Activated Partial Thromboplastin Time
  • BT- Also called Bleeding Time
  • PFA100- Also called Platelet Function Analyzer
  • Factor VIII C- To check for Factor VIII clotting
  • VWF Antigen: To check for levels of the Von Willebrand factor protein
  • VWF Multimeter- To study the Von Willebrand Factor structure
  • Platelet Test- To check for platelet functioning (2)

[ Read: Blount Disease In Children ]

Treating Von Willebrand Disease In Children:

Timely diagnosing and treatment can prevent life-threatening events among patients with severe vWD. Treatment often depends on the type of disorder. For example, patients with mild vWD may not need treatment except for extra care and precaution during a surgical or dental procedure. Some of the commonly available treatments include:

1. Desmopressin:

Desmopressin is preferred for patients with Type I vWD. This is usually administered before a surgical or dental procedure. Some children do not react well to the drug; hence doctors always administer a test dose to check for allergic reaction. Desmopressin is a synthetic drug that acts as a hormone to release VWF stored in the blood.

2. Cyklokapron and Amicar:

These drugs help the blood keep clots after they have formed by stopping it from producing an enzyme that dissolves blood clots. But they do not help the body form clots and hence cannot be taken independently of Desmopresin.

3. Fibrin Glue:

This process removes the fibrin glue from the child’s blood and applied on the patient’s wound.

4. Blood Transfusion:

Children with severe VWD may need blood transfusion.

[ Read: Treatment For Skin Tags In Kids ]

A Word Of Caution:

Children suspected of VWD should contact a hematologist to identify the type and treatment for the disorder. Such children should avoid certain medications like aspirin or other anti-inflammatory medications.

The above-mentioned treatments and symptoms are based on general observations. Do you have a different experience to narrate? Share your experiences with us. Leave a comment below.

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