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Red Blood Cell and Iron Disorders

Unit 5 Lesson 1

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Student Learning Outcomes

At the end of this lesson, students will be able to:

Describe various red blood cell and iron disorders in children

Identify symptoms and presentation of the above disorders in pediatric clients

Define medical management of the above disorders

Apply nursing management and interventions for children with the above disorders

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Introduction

Sickle cell disease and anemia are complex, often inherited disorders impacting red blood cell production and maintenance.
These conditions cause chronic anemia, recurrent pain, and organ damage.
Sickle cell disease is notable for its crescent-shaped red blood cells, which cause vascular occlusions.
Other types of anemia: iron deficiency, megaloblastic, and hemolytic anemia.
Common symptoms: fatigue, pale skin, and severe complications.

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Sickle Cell Disease

Inherited disorder
Mutation in a hemoglobin-forming gene
Abnormal, crescent-shaped red blood cells
Leads to anemia, pain crises and organ damage

(Stanford Medicine, n.d.)

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Symptoms and Presentation of Sickle Cell Disease

Chronic anemia
Recurrent vaso-occlusive pain crises
Swelling of hands and feet
Jaundice
Delayed growth and puberty
Increased susceptibility to infections
Acute chest syndrome
Stroke
Organ damage

Spleen
Kidneys
Eyes

(Stanford Medicine, n.d.)

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Medical Management of Sickle Cell Disease

Hydroxyurea to reduce pain crises and acute chest syndrome frequency
Pain management

NSAIDs
Opioids
Nonpharmacological techniques

Infection prevention

Vaccines
Penicillin prophylaxis

Blood transfusions for severe anemia and stroke prevention
Stem cell transplantation for selected clients

(Stanford Medicine, n.d.)

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Nursing Management of Sickle Cell Disease

Assess for signs of pain crises
Provide pain management techniques
Monitor for signs of infection and hypoxia
Implement infection prevention measures
Administer oxygen (as needed)
Administer hydroxyurea and monitor for side effects
Educate children and families on hydration, balanced diets and avoiding extreme temperatures

(Stanford Medicine, n.d.)

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Critical Thinking Question

A nurse is caring for a 6-year-old client with sickle cell disease who is experiencing a pain crisis. Which of the following interventions should the nurse prioritize?

A) Administer a pneumococcal vaccine

B) Provide pain management and hydration

C) Initiate iron supplementation

D) Perform a splenectomy

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Anemias

Group of conditions

Characterized by decreased red blood cells and Hemoglobin

Reduced oxygen-carrying capacity

(Boston Children’s Hospital, n.d.b)

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Symptoms and Presentation of Anemias

(Boston Children’s Hospital, n.d.b)

Fatigue
Pale skin
Shortness of breath
Dizziness
Headaches
Cold hands and feet
Chest pain
Irregular heartbeat
Symptoms specific to the underlying cause

Blood loss
Chronic diseases
Nutritional deficiencies

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Medical Management of Anemias

Treatment depends on the underlying cause
Dietary changes

Iron-rich foods
Vitamin B12
Folate

Iron or vitamin supplementation
Blood transfusions for severe anemia
Medications

Erythropoiesis-stimulating agents
Immunosuppressants

Treatment of the underlying condition

Chronic diseases
Infections

(Boston Children’s Hospital, n.d.b)

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Nursing Management of Anemias

Assess for signs and symptoms of anemia
Administer iron or vitamin supplementation
Educate children and families on the importance of a balanced diet

Rich in iron, folate and vitamin B12

Monitor for signs of complications

Heart failure
Hypoxia
Seizures

Report to the healthcare team promptly

(Gallagher, 2022)

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Thalassemia (Type of Anemia)

Inherited blood disorder caused by mutations in genes responsible for hemoglobin production
Leads to abnormal hemoglobin and anemia

(Boston Children’s Hospital, n.d.b)

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Symptoms and Presentation of Thalassemia

(Boston Children’s Hospital, n.d.b)

Chronic anemia
Fatigue
Pale skin
Slow growth
Facial bone deformities
Abdominal swelling
Jaundice
Gallstones
Iron overload leading to organ damage

Heart
Liver
Endocrine glands

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Medical Management of Thalassemia

Regular blood transfusions to maintain adequate hemoglobin levels

Iron chelation therapy to prevent iron overload

Folic acid supplementation and stem cell transplantation for selected children

(Boston Children’s Hospital, n.d.b)

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Nursing Management of Thalassemia

Assess for signs and symptoms of anemia

Monitor for complications like iron overload and organ damage

Administer blood transfusions and monitor for transfusion reactions

Educate children and families on the importance of iron chelation therapy, preventing iron overload and associated complications

Provide emotional support and guidance on coping with a chronic condition

(Boston Children’s Hospital, n.d.b)

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Critical Thinking Question

A nurse is preparing to administer care to a 7-year-old client with thalassemia. Which of the following treatments should the nurse expect to be part of this client's care plan?

A) Phlebotomy

B) Regular blood transfusions

C) Vitamin B12 injections

D) Splenectomy

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Iron Deficiency Anemia

Most common nutritional deficiency worldwide
Causes:

Insufficient dietary iron intake
Poor absorption
Blood loss

(Wang, 2016)

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Symptoms and Presentation of Iron Deficiency Anemia

(Wang, 2016)

Fatigue
Pale skin
Shortness of breath
Headaches
Dizziness
Cold hands and feet
Unusual cravings (pica)
Brittle nails
Inflammation or soreness of the tongue

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Medical Management of Iron Deficiency Anemia

Oral iron supplementation

Dietary changes to include iron-rich foods

Treatment of underlying causes like blood loss and malabsorption

Intravenous iron therapy for severe cases and intolerance to oral supplements

(Wang, 2016)

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Nursing Management of Iron Deficiency Anemia

Assess for signs and symptoms of iron deficiency anemia

Administer iron supplements and monitor for side effects

Gastrointestinal discomfort
Constipation

Provide guidance on managing these symptoms

Educate children and families on proper supplement administration

Encourage a balanced diet rich in iron

(Wang, 2016)

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Critical Thinking Question

A nurse is assessing a 4-year-old client with suspected iron deficiency anemia. Which of the following symptoms should the nurse be most alert for?

A) Jaundice

B) Pica (craving non-food items)

C) Enlarged spleen

D) Neurological symptoms

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Megaloblastic Anemia

Type of macrocytic anemia caused by Vitamin B12 (pernicious anemia) and Folate deficiency

Results in the production of large immature red blood cells

High MCV on the CBC report indicates enlarged red cells

(Boston Children’s Hospital, n.d.a; Wang, 2016)

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Signs and Presentation of Megaloblastic Anemia

Fatigue
Weakness
Constipation
Loss of appetite
Weight loss
Neurological symptoms

Numbness
Tingling
Balance problems (B12 specific)

Glossitis: swollen and smooth tongue
Red blood cell changes in bone marrow and peripheral blood

(Boston Children’s Hospital, n.d.a)

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Medical Management of Megaloblastic Anemia

Vitamin B12 injections

Oral supplementation for vitamin B12 deficiency

Folic acid supplementation for folate deficiency

Treatment of underlying causes of malabsorption, dietary deficiencies and adverse drug reactions

(Boston Children’s Hospital, n.d.a)

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Nursing Management of Megaloblastic Anemia

Assess for signs and symptoms of megaloblastic anemia

Fatigue
Weakness
Neurological symptoms

Administer vitamin B12 injections and oral supplementation as prescribed
Monitor for improvement in symptoms
Educate and emphasize the importance of a balanced diet rich in vitamin B12 and folate
Lifelong treatment for cases of pernicious anemia

(Boston Children’s Hospital, n.d.a)

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What Would the Nurse Do?

A nurse is caring for a 5-year-old client with megaloblastic anemia due to vitamin B12 deficiency. What key assessment should the nurse perform regularly?

A) Check for signs of iron overload

B) Monitor for neurological symptoms

C) Assess for joint pain

D) Look for signs of hemolysis

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Hemolytic Anemia

Characterized by premature destruction of red blood cells
Can be inherited or acquired

(Cedars-Sinai, n.d.)

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Symptoms and Presentation of Hemolytic Anemia

(Cedars-Sinai, n.d.)

Fatigue
Shortness of breath
Rapid heartbeat
Jaundice
Dark urine
Abdominal pain
Splenomegaly

Enlarged spleen

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Medical Management of Hemolytic Anemia

Treatment of underlying cause

Autoimmune disorders
Infections
Inherited conditions

Immunosuppressive drugs

For autoimmune hemolytic anemia

Blood transfusions for severe anemia
Splenectomy (removal of the spleen)

For cases of inherited hemolytic anemia

Folic acid supplementation

Supports increased red blood cell production

(Cedars-Sinai, n.d.)

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Nursing Management of Hemolytic Anemia

Assess for signs and symptoms of hemolytic anemia
Administer medications and monitor for side effects
Prepare children and families for blood transfusions
Monitor for transfusion reactions
Educate on the importance of:

Follow-up care
Prompt treatment of infections in Splenectomy cases

(Cedars-Sinai, n.d.)

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What Would the Nurse Do?

A nurse is preparing to administer a blood transfusion to an 8-year-old client with hemolytic anemia. What is the most important nursing intervention during this procedure?

A) Encourage the client to ambulate frequently

B) Monitor closely for transfusion reactions

C) Administer iron supplements immediately after

D) Apply cold compresses to the abdomen

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Hemochromatosis

Inherited disorder

Characterized by excessive iron absorption and storage

Leads to iron accumulation in various organs

Liver
Heart
Pancreas
Endocrine glands

(Children’s Hospital of Pittsburgh, n.d.)

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Symptoms and Presentation of Hemochromatosis

Fatigue
Abdominal pain
Joint pain

Particularly in the hands

Skin bronzing
Hyperpigmentation
Erectile dysfunction
Decreased libido
Cardiomyopathy

Weakening of the heart muscle

Diabetes mellitus
Liver cirrhosis
Liver cancer

(Children’s Hospital of Pittsburgh, n.d.)

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Medical Management of Hemochromatosis

Phlebotomy

Regular blood removal to reduce iron levels

Iron chelation therapy

For those who cannot tolerate phlebotomy

Dietary changes

Limit iron intake of iron-rich foods
Avoid iron supplements

Treatment of complications

Diabetes
Liver disease
Heart failure

(Children’s Hospital of Pittsburgh, n.d.)

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Nursing Management of Hemochromatosis

Assess for signs and symptoms of Hemochromatosis
Monitor for complications
Educate children and families on the importance of regular phlebotomy and adherence to treatment
Provide guidance on dietary changes to limit iron intake
Monitor for side effects of phlebotomy

Anemia
Hypovolemia

Provide support as needed

(Children’s Hospital of Pittsburgh, n.d.)

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What Would the Nurse Do?

A nurse is educating the family of a 10-year-old client newly diagnosed with hemochromatosis. What dietary advice should the nurse provide?

A) Increase iron-rich foods in the diet

B) Avoid iron-rich foods and vitamin C supplements

C) Increase vitamin B12 intake

D) Avoid all dairy products

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References

Boston Children's Hospital. (n.d.a). Anemia.

https://www.childrenshospital.org/conditions/anemia

Boston Children's Hospital. (n.d.b). Red Blood Cell Disorders.

https://www.childrenshospital.org/conditions/red-blood-cell-disorders

Cedars-Sinai. (n.d.). Anemia in Children.

https://www.cedars-sinai.org/health-library/diseases-and-conditions---pediatrics/a/anemia-in-children.html

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References continued…

Children's Hospital of Pittsburgh. (n.d.). Hemochromatosis in Children

Symptoms and Diagnosis. UPMC.

https://www.chp.edu/our-services/transplant/liver/education/liver-disease-states/hemochromatosis

Gallagher, P. G. (2022). Anemia in the Pediatric Patient. Blood, 140(6),

571–593.

https://doi.org/10.1182/blood.2020006479

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References continued…

Stanford Medicine. (n.d.). Sickle Cell Disease in Children.

https://www.stanfordchildrens.org/en/topic/default?id=sickle-cell-disease-in-children-90-P02327

Wang, M. (2016). Iron Deficiency and Other Types of Anemia in Infants

and Children. American Family Physician, 93(4), 270–278.

https://www.aafp.org/pubs/afp/issues/2016/0215/p270.html

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