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Course: Pediatric Nursing

Topic: Nursing Care of Child with

Hematologic Disorders Part IV

The Nurses International Community

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Module Goals

Learners will be able to:

Define Von Willebrand Disease and Disseminated Intravascular Coagulation (DIC).
Describe the types of von willebrand disease.
Identify causes of von willebrand disease and DIC.
Identify signs and symptoms of von willebrand disease and DIC.
Describe common diagnostic procedures for the disorders.
Explain medical management of the disorders.
Discuss nursing management of a child with the disorders above.

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Von Willebrand Disease

Bleeding disorders caused by a defect or deficiency of a blood-clotting protein called von Willebrand factor.
The most common inherited bleeding disorder. Found in up to 1% of the U.S. population.
Either have a low level of Von Willebrand factor (VWF) in the blood or the VWF protein doesn’t work the way it should and can lead to heavy bleeding that is hard to control.

CDC, 2021

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Types

Type 1

Most common and mildest form
Has lower-than-normal levels of VWF and might have low levels of factor VIII.
85% of people treated for VWD have Type 1.

Type 2

Body makes normal amounts of the VWF, but the factor does not work the way it should.
Further broken down into four subtypes (2A, 2B, 2M, and 2N) depending on the specific problem with the individual’s VWF.

CDC, 2021

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Types (Continued)

Type 3

The most severe form of VWD, in which an individual has very little or no VWF and low levels of factor VIII.
The rarest type of VWD (Only 3% of people with VWD have Type 3).

CDC, 2021

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Symptoms

Frequent or Hard-to-Stop Nosebleeds

Start without injury (spontaneous)
Occur often, usually five times or more in a year
Lasts more than 10 minutes
Needs packing (gauze placed in the nose) or cautery (a procedure to burn and seal blood vessels) to stop the bleeding

Easy Bruising

Occurs with very little or no trauma or injury
Occurs often (one to four times per month)
Is larger than the size of a quarter
Is not flat and has a raised lump

CDC, 2021

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Symptoms

Blood in the stool or urine
Heavy menstrual bleeding
Heavy bleeding after childbirth
Bleeding into joints or internal organs in severe cases (Type 3 VWD)

CDC, 2021

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Critical Thinking Question

What would be the nurse’s priority concern for a child diagnosed with von Willebrand disease?

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Diagnosis

Personal and family histories of bleeding
Complete blood count (CBC):

Checks the red and white blood cells, platelets, and sometimes reticulocytes, hemoglobin and hematocrit

Prothrombin time (PT) and partial thromboplastin time (PTT):

Checks for bleeding and clotting problems

Von Willebrand factor antigen and activity:

Amount and activity of von Willebrand factor in blood

Platelet function test
Factor VIII clotting activity

Stanford Children’s Hospital, 2022

CDC, 2021

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Treatment

The most common treatments

Desmopressin Acetate Injection milder forms of VWD (mainly Type 1)
Desmopressin Acetate Nasal Spray
Factor Replacement Therapy
Antifibrinolytic Drugs: to help slow or prevent the breakdown of blood clots
Birth Control Pills: can increase the levels of VWF and factor VIII in the blood and reduce menstrual blood loss in heavy menstrual bleeding.

CDC, 2021

(Stanford Children’s Hospital, 2022)

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Prevention

Do not give blood-thinning medicines (aspirin and nonsteroidal anti-inflammatory drugs (NSAIDs).
Discourage child from taking part in contact sports( football, hockey, and weightlifting).
Have a child wear a medical identification (ID) bracelet.
Educate all caregivers, teachers to sports coaches, about the disease and treatment.

Stanford Children’s Hospital, 2022

CDC, 2021

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What Would the Nurse Do?

A nurse is preparing education session for parents and their 13 years old teenager recently diagnosed with Type 3 von Willebrand disease. Which of the following statements are appropriate? (Select all that apply)

“You can play sports like tennis, golf, archery, or rowing.”
“Let others cut the vegetables or fruits in the kitchen.”
“You should never take aspirin for pain, use ibuprofen instead.”
“Acetaminophen can be used for fever and pain relief as well.”
“Always wear identification bracelet for von Willebrand disease.”

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Disseminated Intravascular Coagulation (DIC)

A serious disorder in which the proteins that control blood clotting become overactive.

May lead to both microvascular and macrovascular clotting and compromised blood flow, ultimately resulting in multiple organ dysfunction syndrome.

MedlinePlus, n.d; Costello, & Nehring, 2021

CDC, 2021

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Causes

Usually due to inflammation, infection, or cancer
Estimated to occur in up to 30% to 50% of cases of severe sepsis, which is the most common cause of DIC
Occurs in up to 20% of patients with metastasized adenocarcinoma or lymphoproliferative disease
Other causes

Trauma, pancreatitis, malignancy, snake bites,
Liver disease, transplant rejection, and
Transfusion reactions

Costello, & Nehring, 2021

CDC, 2021

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Risk Factors

Blood transfusion reaction
Cancer, especially certain types of leukemia
Inflammation of the pancreas (pancreatitis)
Infection in the blood, especially by bacteria or fungus
Liver disease
Pregnancy complications (such as parts of the placenta left behind after delivery)
Recent surgery or anesthesia
Severe tissue injury (as in burns and head injury)
Large hemangioma (a blood vessel that is not formed properly)

MedlinePlus, n.d

CDC, 2021

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Sign and Symptoms

DIC that develops suddenly

Bleeding, which may be severe.
Bleeding may occur at the site of an intravenous injection or in the brain, digestive tract, skin, muscles, or cavities of the body.

DIC develops more slowly (with cancer)

Clots in veins (deep venous thrombosis) are more common than bleeding.
May have swelling, redness, or pain in the area.
May be asymptomatic.
A clot may break free and travel (an embolus) to the lungs and develop a pulmonary embolism.

Moake, 2021

CDC, 2021

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Critical Thinking Question

Which of the following statements is true regarding disseminated intravascular coagulation (DIC)? (Select all that apply)

Inflammation may cause DIC
Deep vein thrombosis may occur as a result of DIC
Bacterial or fungal infection of blood may cause DIC
DIC may cause excessive bleeding

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Diagnosis

History, physical exam
Laboratory findings

Increased Prothrombin time (PT) and partial thromboplastin time (PTT)
Reduced overall platelet count and hematocrit level
Fragmented erythrocytes seen on a peripheral blood smear

DIC is confirmed

Abnormally increased quantities of plasma D-dimer and often a low level of fibrinogen.

Moake, 2021

Costello & Nehring, 2021

CDC, 2021

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Treatment

Treatment of the underlying disorder

Antibiotics for severe sepsis, possible delivery for placental abruption, and possible exploratory surgical intervention for trauma.

Platelet and plasma transfusions with active bleeding or a high risk of bleeding.
Heparin may be used to slow the clotting in clients who have more chronic, milder DIC.
Client who is not actively bleeding should receive prophylactic anticoagulation with heparin or low molecular weight heparin (LMWH).

Moake, 2021

Costello & Nehring, 2021

CDC, 2021

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Nursing Management

Assessment of sign and symptoms of DIC
Monitor respiratory status
Identification of clients at risk
Prompt and appropriate follow-up for nursing interventions
Heparin transfusion
Careful teaching
Emotional supportive care

Moake, 2021

Costello & Nehring, 2021

CDC, 2021

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Critical Thinking Question

Reflection/Discussion:

Why should the nurse assess for respiratory status of the child diagnosed with Disseminated Intravascular Coagulation (DIC)?

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Cultural Considerations:

Religion, culture, beliefs, and ethnic customs can influence how families understand and use health concepts:

Health beliefs: In some cultures talking about a possible poor health outcome will cause that outcome to occur.
Health customs: In some cultures family members play a large role in health care decision-making.
Ethnic customs: Differing gender roles may determine who makes decisions about accepting & following treatment recommendations.

AHRQ, 2020

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Cultural Considerations (Continued):

Religion, culture, beliefs, and ethnic customs can influence how families understand and use health concepts:

Religious beliefs: Faith and spiritual beliefs may effect health seeking behavior and willingness to accept treatment.
Dietary customs: Dietary advice may be difficult to follow if it does not fit the foods or cooking methods of the family.
Interpersonal customs: Eye contact or physical touch may be ok in some cultures but inappropriate or offensive in others.

AHRQ, 2020

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References:

CDC. (2021). What is von Willebrand Disease?. https://www.cdc.gov/ncbddd/vwd/facts.html

Costello, R.A., Nehring, S.M. (2021, July 17). Disseminated Intravascular Coagulation. StatPearls Publishing. https://www.ncbi.nlm.nih.gov/books/NBK441834/

Medlineplus. (n.d). Disseminated intravascular coagulation (DIC). https://medlineplus.gov/ency/article/000573.htm

Moake, J. L. (2021). Disseminated Intravascular Coagulation (DIC). MSD Manual. https://www.msdmanuals.com/home/blood-disorders/bleeding-due-to-clotting-disorders/disseminated-intravascular-coagulation-dic

Stanford Children’s Hospital. (2022). Von Willebrand Disease (VWD). https://www.stanfordchildrens.org/en/topic/default?id=von-willebrand-disease-vwd-160-13

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