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Course: Pediatric Nursing

Topic: Nursing care of child with Hematological Disorders Part I

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Module Goals

Learners will be able to:

Describe the anatomy and physiology of hematologic system
Define thalassemia
Describe the types of thalassemia and their sign and symptoms
Identify causes of thalassemia
Describe common diagnostic procedures for thalassemia
Explain common treatments for children with thalassemia
Discuss nursing management of a child thalassemia

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Characteristics of Blood

A connective tissue.
Made up of cellular elements and an extracellular matrix.
The cellular elements:

Referred to as the formed elements and include:
Red blood cells (RBCs)
White blood cells (WBCs), and
Platelets

The extracellular matrix:

Called plasma, makes blood unique among connective tissues because it is fluid.

Openstax, 2022

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Functions of Blood

The primary function

deliver oxygen and nutrients to cells and remove carries wastes from body cells

Specific functions

Defense
Distribution of heat
Maintenance of homeostasis

Openstax, 2022

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Red Blood Cells (Erythrocytes)

Contain hemoglobin
Primary functions

pick up inhaled oxygen from the lungs and transport it to the body’s tissues.
pick up some (about 24 percent) carbon dioxide waste at the tissues and transport it to the lungs for exhalation from body.

Openstax, 2022

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Hemoglobin

A large molecule made up of proteins and iron
Consists of four folded chains of a protein called globin,

Alpha 1 and 2, and
Beta 1 and 2

Each globin bound to a red pigment molecule heme, which contains an ion of iron.
Each iron ion in the heme can bind to one oxygen molecule and each can transport four oxygen molecules.

Openstax, 2022

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Critical Thinking Question

Which of the following are the extracellular elements of the blood?

Red blood cells
White blood cells
Plasma
Platelets

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Thalassemia

An inherited blood disorder caused where the body doesn’t make. enough of a protein called hemoglobin, an important part of red blood cells.
Typically occurs in individuals from the Middle East, the Mediterranean, African, and Southeast Asia.
Usually diagnosed within the first six months of life.
Can be fatal in early childhood without ongoing treatment.

CDC, 2022

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Types of Thalassemia

Alpha thalassemia:

Occurs when a gene related to the alpha globin protein is missing or mutated.
Most often in found in individuals in Southeast Asia, the Middle East, China, and in those of African descent.

Beta thalassemia:

Occurs when genes related to beta globin defects affect production of the beta globin protein.
Most often in Mediterranean origin.

Medline Plus (n.d)

Better Health, 2019

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Types of Thalassemia

Each type of alpha and beta has different subtypes:

Thalassemia major:

Occurs when an individual has changes in both copies of the affected gene or genes.

Thalassemia minor:

Occurs when an individual has genetic changes in one copy of the HBB gene (beta-thalassaemia) or in one copy of each of the HBA1 and HBA2 genes (alpha-thalassaemia).

Beta thalassaemia major is the most severe type and also called Cooley’s Anemia.

Better Health, Medline Plus

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Critical Thinking Question

Thalassemia is an inherited blood disorder caused when the body doesn’t make enough of a protein called hemoglobin.

True
False

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Symptoms of Thalassemia Major

Symptoms appear in early childhood and include:

Severe anaemia: red blood cells are produced without enough haemoglobin to carry oxygen
Paleness
Sleep difficulties
Poor appetite
Failure to grow and thrive
Enlargement of organs: such as the spleen and liver

Better health, 2019

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Symptoms of Thalassemia Minor

May have mild or no symptoms.
Individual carries the genetic changes and can pass them on to their children.
The World Health Organisation estimates that five to seven per cent of the world’s population has thalassaemia minor.

Better Health, 2019

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Thalassemia Beta

3 types

Beta thalassemia major (Cooley's anemia):

The most severe type of beta Thalassemia
Often found during the first 2 years of life

Beta thalassemia intermedia:

Common throughout the world
Often found later than beta thalassemia major
Transfusions are usually not needed

Thalassemia minor or thalassemia trait:

Carriers of beta thalassemia

Stanford Children’s Hospital, 2022

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Critical Thinking Question

Which is the most severe form of Beta Thalassemia?

Cooley's anemia
Beta thalassemia intermedia
Beta thalassemia minor

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Thalassemia Alpha

Occurs when the body can’t make alpha globin
Two serious types:

Hemoglobin H disease:

Develops as when an individual is missing three alpha globin genes or experiences changes in these genes.
Leads to bone issues (overgrowth of cheeks, forehead and jaw).
May cause jaundice, extremely enlarged spleen and malnutrition.

Holm, 2019

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Thalassemia Alpha

Hydrops fetalis

An extremely severe form of thalassemia that occurs before birth.
Most infants with this condition are either stillborn or die shortly after birth.
This condition develops when all four alpha globin genes are altered or missing.

Holm, 2019

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Causes of Thalassemia

Faulty genes that affect the production of hemoglobin.

Always caused by inherited faulty gene from both parents

If both parents have the faulty gene that causes beta thalassaemia there is a 1 in 4 chance of each child they have being born with the condition.

NHS, 2019

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Critical Thinking Question

Thalassemia is always inherited through faulty genes from the parents.

True
False

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Diagnosis

Blood tests:

Complete blood count (CBC)
Hemoglobin level
Hemoglobin electrophoresis:

a hemoglobin test measures the types of haemoglobin present

Genetic testing:

Looks for gene defects
DNA testing can find alpha thalassemia carriers

Better Health, 2019

Stanford Children’s Hospital, 2022

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Treatment

No cure for thalassaemia major and treatment must continue for life

Main treatments:

Blood transfusion:

Treat and prevent anemia.
Needed once in every month in severe cases.

However, transfusions can lead to a build-up of iron and may cause serious side effects.

NHS, 2019

Better Health,2019

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Critical Thinking Question

The main treatment for thalassemia is_____________.

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Iron Overload in the Body

Too much iron in the body may cause:

Heart problems – including problems affecting the heart muscle (cardiomyopathy), an irregular heartbeat and heart failure
Swelling and scarring of the liver (cirrhosis)
Delayed puberty
Low levels of oestrogen (in women) or testosterone (in men)
Diabetes
Problems with the thyroid gland (hypothyroidism) and parathyroid glands (hypoparathyroidism)

NHS, 2019

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Treatment

Chelation therapy: Medicine (Iron chelators)

Removes the excess iron from the body that builds up as a result of having regular blood transfusions
Individuals may get a build-up of iron even without transfusions
Chelators can be taken orally (by mouth) or injected
3 chelating agents currently available:

Desferrioxamine (DFO): An infusion over 8 to 12 hours (5 or 6 times a week)
Deferiprone (DFP): a tablet or liquid 3 times a day
Deferasirox (DFX): once a day as a tablet

NHS, 2019

Better Health,2019

Contact info: info@nursesinternational.org

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Diet for Thalassemia

A low-fat, plant-based diet is the best choice.
limit iron-rich foods when high iron levels in blood.
Fish and meats are rich in iron and limit these in diet.
Avoid fortified cereals, breads, and juices because they contain high iron levels.
Thalassemia may cause folic acid (folate) deficiencies: 1 mg supplement taken daily.

Holm, 2022

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Treatment

Splenectomy
Bone Marrow Transplant:

The only cure for thalassaemia major.
The odds of finding a compatible donor are about 30% most donors are siblings.
More successful in young children who don’t suffer from iron overload, but graft rejection or even death may still result.

Holm,2022

Better Health,2019

Contact info: info@nursesinternational.org

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What Would the Nurse Do?

When teaching the parents of a child newly diagnosed with beta-thalassemia, which statement made by the parents indicates a need for additional teaching?

“We will give our child daily iron supplements.”
“Our child will need to have blood transfusions.”
“If we want more children, we should have genetic counseling.”
“Swimming is a good activity for our child.”

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Genetic Counseling

If a child or another family member has been diagnosed with thalassaemia, they should speak to a genetic counsellor
If a thalassaemia carrier is thinking of having a child, may decide to have:

Prenatal genetic testing to see whether the child is affected
Other options

For example: conception through in vitro fertilization (IVF) with genetic screening of the embryo before implantation

Health Direct, 2021

Better Health,2019

Contact info: info@nursesinternational.org

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Nursing Care Management

Promote compliance with transfusion and chelation therapy
Assist the child in coping with anxiety
Foster the child and family’s adjustment to chronic illness
Observe for the complication of transfusions
Support the family
Education:

Diet
Infection control

Hockenberry and Wilson, 2007

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Nursing Care Management

Activity:

Assist family and child in planning and prioritizing activities of daily living (ADL).
Assist in developing a schedule for daily activity and rest.
Stress the importance of frequent rest periods.

Infection prevention:

Assess for signs of infection.
Instructions to avoid contact with people with existing infections, eating raw fruits and vegetables and uncooked meat.
Stress the importance of daily hygiene.

Belleza, 2021

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Red Flags

Severe anemia
Failure to grow and thrive
Enlargement of organs – such as the spleen and liver

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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:

Openstax. (2022). An Overview of Blood. Anatomy and Physiology. https://openstax.org/details/books/anatomy-and-physiology-2e

CDC. (2022). What is Thalassemia?. https://www.cdc.gov/ncbddd/thalassemia/facts.html

Better Health. (2019). Thalassemia. https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/thalassaemia

NHS. (2019). Thalassaemia. https://www.nhs.uk/conditions/thalassaemia/

Medlineplus. (n.d.). Thalassemia. https://medlineplus.gov/ency/article/000587.htm#top

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References:

Stanford Children’s Hospital. (2022). Beta Thalassemia in children. https://www.stanfordchildrens.org/en/topic/default?id=beta-thalassemia-cooleys-anemia-in-children-90-P02330

Health Direct. (2021). Thalassaemia. https://www.healthdirect.gov.au/thalassaemia

Holm, G. (2019). Everything You Need to Know About Thalassemia. Healthline. https://www.healthline.com/health/thalassemia

Belleza, M. (2021). Thalassemia. Nurseslabs. https://nurseslabs.com/thalassemia/

Contact info: info@nursesinternational.org

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