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Bone Marrow Failure Syndromes

Unit 5 Lesson 5

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Student Learning Outcomes

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

Describe pediatric bone marrow failure syndromes

Identify symptoms and presentations

Define medical management and nursing management

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Introduction

Bone marrow fails to produce enough healthy blood cells
Types:

Aplastic Anemia
Myelodysplastic Syndrome (MDS)

(Dana-Farber Cancer Institute, n.d.)

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Aplastic Anemia

Rare disorder
Bone marrow fails to make enough blood cells
Can be acquired or inherited
Affects red blood cells, white blood cells and platelets
Mild to severe

(Boston Children’s Hospital, n.d.a;

Dana-Farber Cancer Institute, n.d.)

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Causes of Aplastic Anemia

Acquired:

Autoimmune disorders
Viral infections
Toxin exposure
Radiation/chemotherapy

(Boston Children’s Hospital, n.d.a;

Dana-Farber Cancer Institute, n.d.)

Inherited:

Fanconi anemia
Dyskeratosis congenita
Shwachman-Diamond syndrome

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Symptoms and Presentation of Aplastic Anemia

Fatigue and weakness
Frequent infections
Bruising and bleeding
Pale skin
Rapid heartbeat
Shortness of breath

(Boston Children’s Hospital, n.d.a;

Dana-Farber Cancer Institute, n.d.)

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Critical Thinking Question

A nurse is assessing a 10-year-old client newly diagnosed with aplastic anemia. Which of the following symptoms would the nurse most likely observe?

A) Jaundice

B) Petechiae

C) Enlarged lymph nodes

D) High fever

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Medical Management of Aplastic Anemia

Supportive care:

Blood transfusions
Antibiotics and antifungal medications

Immunosuppressive therapy:

Antithymocyte globulin (ATG)
Cyclosporine
Corticosteroids

Hematopoietic stem cell transplantation (HSCT):

For severe cases or those not responding to immunosuppressive therapy

(Boston Children’s Hospital, n.d.a;

Dana-Farber Cancer Institute, n.d.)

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Nursing Management of Aplastic Anemia

Monitor vital signs and assess for complications

Mouth sores
Nausea
Vomiting
Infections
Tremors
Weight gain
High blood pressure

(Boston Children’s Hospital, n.d.a;

Dana-Farber Cancer Institute, n.d.)

Implement infection prevention measures

Encourage hand hygiene
Limit exposure to people with infections
Clean and disinfect client’s environment

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Nursing Management of Aplastic Anemia continued…

Administer medications and monitor side effects
Provide age-appropriate education to child and parent
Provide emotional support
Use play therapy and art therapy for children
Advise on not missing doses to avoid flare-ups
Help with HSCT preparation and care
Teach about HSCT process, isolation, and complications
Monitor for graft-versus-host disease (GVHD) and other complications after transplant

(Boston Children’s Hospital, n.d.a;

Cleveland Clinic, n.d.a; Cleveland Clinic, n.d.b;

Dana-Farber Cancer Institute, n.d.)

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What Would the Nurse Do?

A 15-year-old client with aplastic anemia is receiving immunosuppressive therapy with antithymocyte globulin (ATG) and cyclosporine.

What key points should the nurse include when educating the client and family about this treatment?

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Myelodysplastic Syndrome (MDS)

Group of disorders with ineffective blood cell production
Can lead to shortage of all blood cell types
May progress to acute myeloid leukemia (AML)

(Boston Children’s Hospital, n.d.b; UW Health, n.d.)

Contact info: info@nursesinternational.org

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Causes of MDS

De novo MDS

No known cause

Secondary MDS

Result of prior cancer treatments

Genetic predisposition

e.g., Down syndrome, Fanconi anemia

(Boston Children’s Hospital, n.d.b;

Johns Hopkins Hospital, n.d.)

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Symptoms and Presentation of MDS

Anemia

Fatigue
Paleness
Shortness of breath
Chest pain

Neutropenia

Frequent or severe infections

(Boston Children’s Hospital, n.d.b)

Thrombocytopenia

Easy bruising
Tiny red spots on skin (petechiae)
Bleeding

Complete blood count (CBC) test checks number and abnormalities of blood cells, infections or other causes

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Critical Thinking Question

A nurse is caring for a 12-year-old client with myelodysplastic syndrome (MDS). The client complains of feeling tired all the time. What is the most likely cause of this symptom?

A) Thrombocytopenia

B) Neutropenia

C) Anemia

D) Hypoglycemia

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Medical Management of MDS

Supportive care:

Blood transfusions
Growth factors
Iron chelation therapy

Disease-modifying therapies:

Lenalidomide
Hypomethylating agents

Intensive chemotherapy (for high-risk MDS)
Hematopoietic stem cell transplantation (HSCT)

(Boston Children’s Hospital, n.d.b;

Johns Hopkins Medicine, n.d.)

Contact info: info@nursesinternational.org

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Nursing Management of MDS

Monitor blood counts and assess for complications
Administer transfusions and medications
Provide oral care and hygiene education

Teach proper oral hygiene

Gentle brushing with soft toothbrush

Encourage regular dental check-ups
Inform dentist about MDS

Educate about chemotherapy side effects and management
Offer emotional support and resources
Facilitate discussions about care planning

(Boston Children’s Hospital, n.d.b;

Johns Hopkins Hospital, n.d.; UW Health, n.d.)

Contact info: info@nursesinternational.org

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What Would the Nurse Do?

A nurse is preparing a 7-year-old client with MDS for a blood transfusion. Which of the following actions should the nurse prioritize?

A) Administer antipyretics

B) Check vital signs and client identification

C) Start intravenous antibiotics

D) Perform a bone marrow biopsy

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References

Boston Children’s Hospital. (n.d.)(a). Aplastic Anemia. https://www.childrenshospital.org/conditions/aplastic-anemia

Boston Children’s Hospital. (n.d.)(b). Myelodysplastic Syndrome in Children. https://www.childrenshospital.org/conditions/myelodysplastic-syndrome

Cleveland Clinic. (n.d.)(a). Graft vs. Host Disease. https://my.clevelandclinic.org/health/diseases/10255-graft-vs-host-disease-an-overview-in-bone-marrow-transplant

Cleveland Clinic. (n.d.)(b). Immunosuppressants. https://my.clevelandclinic.org/health/treatments/10418-immunosuppressants

Contact info: info@nursesinternational.org

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References continued…

Dana-Farber Cancer Institute. (n.d.). Bone Marrow Failure in Children. https://www.dana-farber.org/cancer-care/types/childhood-bone-ma rrow-failure

Fibach, E. & Rachmilewitz, E. A. Iron Overload in Hematological Disorders. La Presse Médicale, 46(12):e296–e305. doi: 10.1016/j.lpm.2017.10.007

Johns Hopkins Medicine. (n.d.). Myelodysplastic Syndrome. https://www.hopkinsmedicine.org/kimmel-cancer-center/cancers-we-treat/pediatric/about-us/myelodysplastic-syndrome

UW Health. (n.d.). Pediatric Acquired Aplastic Anemia. https://www.uwhealth.org/conditions/pediatric-acquired-aplastic-anemia

Contact info: info@nursesinternational.org

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