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Course: Pediatric Nursing

Topic: Nursing care of child with Hematological Disorders Part II

The Nurses International Community

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Module Goals

Learners will be able to:

Define hemophilia
Describe types of hemophilia
Discuss causes of hemophilia
Discuss how hemophilia is inherited
Identify sign and symptoms of hemophilia
Explain common medical management of hemophilia in children
Discuss nursing management of a child with hemophilia

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Hemophilia

A rare bleeding disorder where the blood doesn't clot properly
Usually inherited
More frequent in males
Most individuals are born with it
The frequency of hemophilia is approx. 1 in 10,000 live births, and the number of people worldwide living with hemophilia is about 400,000.

CDC, 2021

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Types of Hemophilia

Hemophilia A:

Most common type
A deficiency in factor VIII
Also known as classic hemophilia

Hemophilia B:

Also known as Christmas disease, occurs because clotting factor IX is missing or severely deficient.

Hemophilia C:

Also known as “factor XI deficiency,”and is a rare form with severe bleeding after dental extractions.

Kahn, 2022

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Critical Thinking Question

Which of the following disorders is a deficiency of factor VIII?

Sickle cell disease
Christmas disease
Hemophilia A
Hemophilia B

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Causes

Caused by an inherited change to a gene.
An individual’s genes provide the instructions about making proteins, such as factor VIII and factor IX.
A mutation in either the gene for the factor VIII protein or the gene for the factor IX protein causes the body to produce too little factor VIII or IX.
Low amounts of either factor VIII (eight) or factor IX (nine) which are key factors responsible for normal blood clotting.

NHS,2020

CDC, 2021

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How Hemophilia Inherited

Both hemophilia A and B are inherited in the same way, both the genes for factor VIII and factor IX are located on the X chromosome.
There are no genes for clotting factors on the Y chromosome.
This means that males only have one allele for factor VIII and one allele for factor IX.
Thus, if a male has a hemophilia allele on his X chromosome, he will have the disorder.

CDC, 2021

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Critical Thinking Question

What Causes Hemophilia?

Genetic mutation
Bacteria
Viruses
Unknown

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How Hemophilia Inherited

A female inherits two copies of the factor VIII or factor IX gene, one from her mother and one from her father.
A female with a hemophilia allele on one X chromosome usually has a normal allele on her other X chromosome that can produce normal clotting factor, so she has some protection against having hemophilia.
A female with one hemophilia allele and one normal allele is called heterozygous or a carrier.

CDC, 2021

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How Hemophilia Inherited

CDC, 2021

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How Hemophilia Inherited

CDC, 2021

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Symptoms

The severity is determined by the level of clotting factors present in blood.

Mild haemophilia: between 5% and 50% of the normal amount of clotting factors.
Moderate haemophilia: between 1% and 5%.
Severe haemophilia: less than 1%.

NHS, 2020

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Symptoms

Mild hemophilia

Asymptomatic for many years.
Usually becomes apparent after a wound or surgery or tooth extraction tooth.
Causes unusually long period of bleeding.

NHS, 2020

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Symptoms

Moderate hemophilia

Same as in mild hemophilia, but also bruise easily.
Possible internal bleeding around joints after a fall.

Usually begins with a tingling feeling and mild pain in the affected joint.
An untreated joint bleed can lead to:

More severe joint pain
Stiffness
Hot, swollen, and tender site

NHS, 2020

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Symptoms of Severe Hemophilia

Joint bleeding is more frequent and severe
Spontaneous bleeding:

Nosebleeds
Bleeding gums
Bleeding in the joints
Bleeding in the muscles

Without treatment a child may develop:
Joint deformity
Soft tissue bleeding
Serious internal bleeding

NHS, 2020

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Critical Thinking Question

What is the main symptom of Hemophilia?

Headache
Fatigue
Bleeding
Muscle Pain

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Management oF acute Bleeding

Hospitalization
Rapid recognition of the location and severity of the bleed.
Immediate administration of high-dose clotting factor concentrate. (CFC) with factor VIII or IX
Dose requirements:

50 IU/kg body weight factor VIII
100 to 120 IU/kg factor IX
70 to 80 IU/kg of prothrombin complex if factor IX unavailable.
May require urgent surgery in cases of intracranial bleed, neck hematoma.

Mehta, & Reddivari, 2021

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Management of acute Bleeding

High-dose clotting factor concentration should be given as required to allow healing after the bleeding stops.
Frequent monitoring of factor levels to assure desired levels are maintained.
Avoid Acetylsalicylic acid (ASA) and non-steroidal anti-inflammatory drugs (NSAIDs).
May use Acetaminophen and certain COX-2 inhibitors.
Avoid all Intramuscular injections if possible.

Mehta, & Reddivari, 2021

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Prophylaxis Treatment

Maintain the factor levels above 1% to 2%

Hemophilia A:

factor VIII infusion of 25 to 40 units/kg of body weight three times per week per Malmo protocol.

Hemophilia B:

factor IX infusion of 25 to 40 units/kg of body weight two times per week per Malmo protocol.

Slow infusion rate of less than 3 ml/minute in adults and less than 100 units/minute in young children.

(Mehta, & Reddivari, 2021)

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Prophylaxis Treatment

Other Pharmacological Options

Desmopressin (DDAVP)

Increases endogenous factor VIII plasma concentrations by 3 to 5 times.
Useful in the prevention of bleeding in hemophilia carrier.
No value in hemophilia B as it does not affect levels of factor IX.
Contraindicated in children less than two years of age who can be at risk of developing seizures due to cerebral edema.

Tranexamic acid and epsilon aminocaproic acid

Useful in preventing: Mucocutaneous bleeds like epistaxis.

Mehta, & Reddivari, 2021

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Pain Management

For acute joint or muscle bleeds:

Rest, compression
Elevation
Cold packs
Immobilization
Crutches s
Splints
Braces or wheelchairs

Avoid intramuscular injections and ASA and NSAIDs,
First-choice drugs for pain are acetaminophen or paracetamol,

NHS, 2020

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Physical Activity in Hemophilia

To ensure physical fitness:

Muscle strengthening
Maintain healthy body weight and bone density

Avoid contact sports:

Soccer
Rugby
Boxing
Racing
Skiing

Encourage non contact sports:

Swimming
Golf
Cycling

Wear protective gear

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What Would the Nurse Do?

Which measures would the nurse avoid when planning care for a child with hemophilia? (Select all that apply)

Frequent blood pressures
Intramuscular injections
Rectal temperatures
Administering aspirin for pain

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Complications of Hemophilia

Joint damage from repetitive bleeding
Deep internal bleeding
Fractures
Neurological problems due to cranial bleeding

NHS, 2020

(Opestax, 2022)

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Nursing Care Management

History
Assessment of the sign of bleeding.
Prevent bleeding

Encourage appropriate exercise.
Safe environment for the motor skills development for infant and toddlers.
Encourage safe physical activities with appropriate safety measure.
Inform parents regarding dental care and regular check-ups at the dentist.
Encourage adolescents to use electric shaver.

Hockenberry and Wilson, 2007

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Nursing Management

Dental care

Water irrigating devices, softening the toothbrush in warm water before brushing.

Recognize and prevent bleeding

Administration of factor replacement therapy as prescribed.
Rest, Ice, Compression and Elevation(RICE).

Prevent crippling effects of bleeding

Joint is elevated and immobilized.
Encourage active range of motion exercise after acute phase.

Hockenberry and Wilson, 2007

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Nursing Management

Support the family and Prepare for Home care

Teach limitations and preventive measures.
Comprehensive team approach.
Parent group discussions with similarly affected families.
Teach children to take responsibilities for their diseases at early age.

Hockenberry and Wilson, 2007

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What Would the Nurse Do?

A child with hemophilia wishes to participate in sports. Which sport should the nurse recommend as the most appropriate for the child?

Basketball
Biking
Baseball
Swimming

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Red Flag

*Nursing Alert:

Passive range of motion exercises should never be part of exercise regimen after an acute episode because the joint capsule could easily be stretched and bleeding could occur.

Hockenberry & Wilson, 2007

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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). How Hemophilia is Inherited. https://www.cdc.gov/ncbddd/hemophilia/inheritance-pattern.html

NHS. (2020). Hemophilia. https://www.nhs.uk/conditions/haemophilia/

Kahn, A. (2022). Hemophilia. https://www.healthline.com/health/hemophilia

Mehta, P., & Reddivari, A. K.R. [Updated 2021 Dec 31]. Hemophilia. StatPearls Publishing. https://www.ncbi.nlm.nih.gov/books/NBK551607/

Hockenberry, M. J. & Wilson, D. (2007). WONG’S Nursing Care of Infants and Children. 8th edi. Mosby

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