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What are bleeding disorders?

Bleeding disorders is a general term to describe medical conditions in which the blood does not clot well.
The process of blood clotting is very complex and things can go wrong at many stages. There are 2 parts of the blood that are required for effective clotting—tiny cells in the blood called platelets and proteins called clotting factors. When blood does not clot, the child may bruise easily, have nosebleeds, or bleed for a long time after being injured or after surgery.
Platelet problems
- Having a low platelet count can interfere with normal clotting. Low platelet counts can be caused by different diseases or as a side effect of certain medications.
- The most common disease causing low platelets is idiopathic thrombocytopenic purpura (ITP).
- There are also rare disorders of platelet function that can have similar bleeding tendencies.
Clotting factor disorders
- The 2 most common of these disorders are von Willebrand disease and hemophilia.
- Von Willebrand disease can affect males and females equally, but hemophilia usually only affects males.

How common are they?

Statistics about von Willebrand disease vary depending on whether very mild cases are included. Some think the condition may be as common as 1% to 2% of the population, but others feel that it affects only 1 in 1,000 people (0.1%).
Hemophilia occurs in 1 in 5,000 male births and affects approximately 18,000 people, mostly male, in the United States.

What are some characteristics of children with bleeding disorders?

Bleeding disorders may cause lumps under the skin (hematomas) or flat collections of pinpoint bleeding in the skin called petechiae.
Spontaneous bleeding or bleeding after trauma can occur in the joints or the intestinal and urinary tracks.
Nosebleeds and bleeding from the gums or teeth may also be common.
Hemophilia
- Deeper and surgical bleeding is more common in hemophilia. Bleeding into the joints can cause problems over time for children with hemophilia; efforts are made to avoid this complication.
- Children with hemophilia are often treated with infusion of the missing clotting factors. In the past, these clotting factors were derived from blood and placed children with hemophilia at risk for blood-borne infections such as hepatitis B and C and HIV, but changes in the way clotting factors are produced have reduced this problem. Since the mid to late 1980s, treatment of these blood-clotting factors as well as the use of genetically engineered clotting factors have essentially eliminated the risk of blood-borne infections.
Von Willebrand disease
- Usually presents with more superficial bleeding such as nose, mouth, and menstrual.
- Can vary in severity, but most forms cause less serious bleeding than hemophilia.

Who is the treatment team?

Children with hemophilia and von Willebrand disease require specialty care. The physicians who care for them are called hematologists and they manage them along with other specialty nurses, physical therapists, and social workers as well as the primary care provider in the medical home.
Children with von Willebrand disease bleed less than children with hemophilia and therefore need less urgent and frequent specialty care, but hematologists are usually involved in the care of their bleeding problems prior to surgery or procedures, or after trauma.

What adaptations may be needed?

Bleeding disorders vary in types and severity, so it is best to get details about the specific child’s needs from parents/ guardians and the child’s specialty doctors.

Medications

A medication called desmopressin acetate (DDAVP) can help prevent or eliminate bleeding in some types of bleeding disorders. It can be given by injection or sprayed into the nose.
Some children with a more severe form of hemophilia will have a long-term intravenous catheter placed so they can get treated with clotting factors more easily.
All children with clotting disorders should avoid nonsteroidal anti-inflammatory drugs such as ibuprofen (eg, Advil, Motrin), naproxen (eg, Aleve), and aspirin.
Acetaminophen (eg, Tylenol) is usually fine to use, but patients with bleeding disorders should discuss any medications to be taken, even over-the-counter medicines, with their hematologist.

Dietary considerations

There is no special diet for bleeding disorders, but it is important that the child not become overweight because that can put more stress on the joints.

Physical environment

Avoiding trauma, especially to the head, can be a big challenge, particularly in the preschool years. Be extra cautious that straps in high chairs are fastened and that children are watched carefully on elevated surfaces such as changing tables and when climbing. Pad any sharp corners. Some children with severe hemophilia may wear helmets to protect their heads, but this is not common and is usually done to reduce the risk of further bleeding in a child who has already suffered a head bleed. Gym activities may need to be adapted for school-aged children.
First aid—cool compresses can be used for bleeding. Popsicles are sometimes helpful for mouth injuries, but don’t allow the child to keep the popsicle in one place for too long to avoid cold injury.
If a nosebleed occurs, pinch the end of the nose below the nasal bone for 10 minutes and have the child stay in a neutral position. Some children with mild von Willebrand disease do not need any special adaptations, just awareness of their condition in case they are injured or require emergency treatment.
Exercises such as bicycle riding, walking, and swimming are good ways to keep muscles strong and joints flexible.
Use standard precautions when dealing with bleeding.

What should be considered an emergency?

Call emergency medical services/911 for
- Head trauma followed by headache, vomiting, change in behavior, or other unusual signs
- Any bleeding that is not easily stopped after 10 minutes, or any vigorous bleeding
Call parents/guardians for
- Swelling of a joint or muscle. Parents/guardians should be notified immediately about any signs or symptoms of bleeding into a muscle or joint such as swelling or inability to move the body part. Older children may recognize the sensation of a joint bleed or notice swelling of their muscle or joint. You should notify parents/guardians immediately.
- Minor episodes of bleeding that are stopped with first aid.

What types of training or policies are advised?

First aid for bleeding
Standard precautions—using gloves, washing hands, and sanitizing surfaces when dealing with blood
Background on hemophilia or von Willebrand disease

What are some related Quick Reference Sheets?

Idiopathic Thrombocytopenic Purpura (page 113)

What are some resources?

National Hemophilia Foundation,www.hemophilia.org
National Heart, Lung, and Blood Institute,www.nhlbi.nih.gov/health/dci/Diseases/vWD/vWD_WhatIs.htmlorhttp://public.nhlbi.nih.gov/newsroom/home/GetPressRelease.aspx?id=2553

Source: Managing Chronic Health Needs in Child Care and Schools: A Quick Reference Guide.

Products are mentioned for informational purposes only. Inclusion in this publication does not imply endorsement by the American Academy of Pediatrics.

Listing of resources does not imply an endorsement by the American Academy of Pediatrics (AAP). The AAP is not responsible for the content of external resources. Information was current at the time of publication.

The information contained in this publication should not be used as a substitute for the medical care and advice of your pediatrician. There may be variations in treatment that your pediatrician may recommend based on individual facts and circumstances.

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Managing Chronic Health Needs in Child Care and Schools—Bleeding Disorders: An Overview