Hematological, Immunological, and Oncological Disorders in Pediatrics

Hematopoiesis

The process through which the bone marrow produces blood cells and platelets.

Erythropoiesis

Production of the red blood cells (RBCs) with erythropoietin and iron.

Lifespan of RBC

120 days.

Hemolysis

Process of RBCs being destroyed by phagocytes in the spleen and liver.

Complete Blood Count (CBC)

A test that includes WBC, RBC, hemoglobin, hematocrit, and platelets.

CBC differential

The breakdown of the leukocytes or WBCs by type.

Elevated neutrophils

Indicates presence of bacterial infections.

Elevated lymphocytes

Indicates presence of viral infections.

Decreased leukocyte count

Limited defenses and is susceptible to infection.

Low thrombocyte count

Can indicate petechiae, ecchymoses, purpura or spontaneous bleeding.

Anemias

Condition in which mature erythrocytes are decreased in number or volume or impaired in function.

Reticulocytes

Immature RBCs that are not as good at carrying oxygen as mature RBCs.

Iron Deficiency Anemia

Characterized by small, pale RBCs and depleted iron stores, with a subsequent decrease in bone marrow erythropoiesis.

Iron Deficiency Anemia Assessment

Includes screening hemoglobin concentration at 12 month well child check and symptoms like irritability, anorexia, and poor muscle tone.

Diagnostic Tests for Iron Deficiency Anemia

Includes analysis of CBC with RBC's indexes, low serum iron, serum ferritin, total iron-binding capacity, and reticulocyte hemoglobin content.

Nursing Interventions for Iron Deficiency Anemia

Dietary therapeutics with oral elemental iron preparations.

Caregiver Education for Iron Deficiency Anemia

Use commercial iron-fortified formula and provide an iron supplement unless infant is consuming plenty of iron-rich food.

Idiopathic Thrombocytopenic Purpura

Decreased platelet counts due to infections such as rubella, rubeola, varicella or upper respiratory infection.

Idiopathic Thrombocytopenic Purpura Assessment

Includes recent spontaneous bleeding and petechiae or purpura on face and extremities.

Diagnostic Testing for Idiopathic Thrombocytopenic Purpura

Decreased platelet count.

Nursing Interventions for Idiopathic Thrombocytopenic Purpura

Use care with mouth care, monitor stools for blood, and avoid IM infections.

Caregiver Education for Idiopathic Thrombocytopenic Purpura

Teach side effects of steroid therapy and support appropriate activities for child.

Sickle Cell Disease

Genetic condition associated with the presence of an abnormal Hgb gene known as Hgb sickle (Hgb S) gene.

Hgb S gene

Abnormal hemoglobin gene that partially or completely replaces normal hemoglobin A.

Sickle shape

Deformed RBCs change from round to sickle shape and tend to clump together.

Vaso-occlusion crisis

A painful episode with hand-foot syndrome (dactylitis) causing symmetrical infarct in the bones of the hands and feet.

Acute chest syndrome

Characterized by chest pain, fever, cough, tachypnea, wheezing, and hypoxia.

Sequestration crisis

Loss of spleen function from frequent infarcts and pooling of large amounts of blood, leading to spleen enlargement.

CVA

Blocking of the blood vessels in the brain with impaired neurological function, especially in toddlers and preschoolers.

Sickledex

Test that determines if Hgb S is present in 3 minutes.

Hemoglobin electrophoresis

Accurate and rapid test that quantifies the percentages of various types of Hgb S and Hgb A.

Hydroxyurea

Medication approved in 2017 by FDA for treating children with sickle cell disease that increases fetal Hgb levels.

Neutropenia

Adverse effect of hydroxyurea that is reversible when the medication is stopped.

Aplastic Anemia

Condition of complete bone marrow suppression or failure, which may be congenital or acquired.

Pancytopenia

Condition in which all three blood cellular components (WBCs, RBCs, platelets) are low or absent.

Etiology of Aplastic Anemia

Possible insult to the body that destroys the bone marrow by autoimmune response, infection, exposure to chemicals, pharmacological agents, or radiation.

Clinical Presentation for congenital Aplastic Anemia

Red marrow is replaced with a fatty yellow substance, and it is an autosomal recessive disorder with other birth defects.

Sickle Cell Trait

Individuals who have one normal adult Hgb gene (Hgb A) and one Hgb S gene are carriers of the sickle cell trait but do not have the disease.

Complications of Sickle Cell Disease

Includes painful erections (priapism), retinal detachment, and emotional and behavioral problems related to long periods of hospitalization.

Nursing Interventions for Sickle Cell Disease

Key is addressing the triggers of sickling such as stress, infection, alcohol, tobacco, cold, dehydration, and lack of O2.

Preventing Hypoxia

Maintain oxygen saturation levels at 93% or greater.

Caregiver Education for Sickle Cell Disease

Includes genetic testing and resources, signs and symptoms of infection, and when to seek medical care.

Physical Therapy

One of the nursing interventions for managing Sickle Cell Disease.

Complementary Therapy

Another nursing intervention for managing Sickle Cell Disease.

GU issues

Missing kidney, hypospadias, epispadias, cryptorchidism, bladder issues and sexual dysfunction.

Sensory dysfunctions

Deafness, malformation of the eyelid or ear.

Café au lait spots

Skin pigmentation abnormalities associated with various conditions.

Cardiac defects

Congenital or acquired abnormalities of the heart structure.

Short stature

Height significantly below the average for age and sex.

Growth delays

Slower than expected physical development.

Delayed maturation

Postponed development in physical or sexual characteristics.

Microcephaly

Abnormally small head size, often associated with developmental issues.

Intellectual disability

Significantly impaired cognitive functioning and adaptive behavior.

Acquired aplastic anemia

Condition presenting similar to leukemia, characterized by excessive fatigue, weakness, recurrent URIs, febrile episodes.

Aplastic Anemia Assessment

Fatigue, history of illness or injury that does not heal.

Aplastic Anemia Diagnostics

CBC with diff; platelet count; increased % of reticulocytes, bone marrow aspiration.

Aplastic Anemia Treatment

HLA-matched bone marrow or stem cell transplantation is best option for a cure.

Immune system suppression

Treatment goal in aplastic anemia management.

Androgen therapy

May be used as part of the treatment for aplastic anemia.

Nursing Interventions for Aplastic Anemia

Monitor immunosuppressive therapy, blood transfusions and IV infusion access care, prevent infection.

Caregiver Education for Aplastic Anemia

Discuss possibility of bone marrow transplant before receiving multiple transfusions.

Hemophilia

A coagulopathy- bleeding disorder caused by deficiency of factor VIII or factor IX.

Hemophilia A

Classic hemophilia, deficiency of factor VIII, most common form of hemophilia.

Hemophilia B

Christmas disease, deficiency of factor IX.

Clinical Presentation of Hemophilia

Spontaneous or traumatic bleeding, severity ranges from mild to severe.

Hemophilia Assessment

History of bleeding, nosebleeds, and bruising.

Diagnostic Testing for Hemophilia

PTT prolonged, low levels of factor VII or IX coagulant.

Nursing interventions for Hemophilia

Administration of factor VIII concentrate, DDAVP, corticosteroids as ordered.

Lead Poisoning

Caused by chronic ingestion or inhalation of materials that contain lead.

Lead Poisoning Assessment

Perform risk assessment at well child checks and inquire about sources of lead.

Lead Poisoning Diagnostic Testing

Blood lead levels - levels ≥ 3.5 mcg/dL indicates exposure above the reference level.

Immunodeficiency

Occurs when the immune system under functions - increases the body's susceptibility to infections.

Autoimmune disorders

Occurs when the immune system over functions - causing the body to produce antibodies against its own cells.

Primary immunodeficiency disorders (PID)

Tend to present within 6 months of age.

Immunology

The mechanism with which the body defends itself against infectious agents and foreign substances.

Human Immunodeficiency Virus (HIV)

The infectious agent - acquired immunodeficiency disease/syndrome (AIDs) is the disease caused by HIV.

HIV prevalence in children

22% of new HIV cases in the US are children aged 13 to 24 years old (2015).

HIV statistics

In 2020, there were 1.7 million children age 0-14 years living with HIV.

Types of HIV

Two types of HIV: HIV-1 and HIV-2. HIV-1 is the most prevalent.

Vertical transmission of HIV

Occurs from the mother either in utero, during birth or via breast milk.

Horizontal transmission of HIV

Occurs when using nonsterile needles or from sexual contact.

CD4 cell count and AIDS diagnosis

When the CD4 cell count gets below 200 cells/ml - the diagnosis of AIDS is made.

Clinical presentation of HIV in children

May be asymptomatic or may have chronic diarrhea, failure to thrive, developmental delays.

Assessment for HIV in children

Includes lymphadenopathy, mucocutaneous eruptions, FTT and delayed development, hepatosplenomegaly, oral candidiasis, parotitis, chronic or recurrent diarrhea.

Diagnosis of HIV in children

Testing for HIV-2 in children is only done if mother has high risk factors for HIV or known HIV infections or if the child has symptoms.

Treatment for HIV

No cure for HIV infection but treatment has improved survival rates.

Antiretroviral therapy (ART)

Mothers with HIV are started on ART and HAART and these decrease the chances of transmission from mom to baby.

Nursing interventions for HIV

Administration of the HAART or ART combination therapy.

Nutritional management for HIV

High-calorie, nutrient-dense food.

Oncological Disorders

Group of complex diseases with uncontrolled growth and spread of abnormal cells.

Treatment for Oncological Disorders

May include chemotherapy, radiation, surgery, biological therapy medications, and/or bone marrow transplant.

Cancer in Children

Cancer is the leading cause of disease-related mortality in children > 1 years old.

Incidence of Pediatric Cancer

Incidence is still rare - 1% of all cancer cases in the US are pediatric cancers.

Survival Rate Increase

Survival rate for pediatric cancer has increased by 20%.

Leading Cause of Death

Brain tumors are the leading cause of death from cancer in pediatrics.

Survival Rates of Specific Cancers

Cancers with high survival rates: acute lymphocytic leukemia (ALL) at 90%; non-Hodgkin lymphoma (NHL) at 90.6%.

Diagnosis Impact

Diagnosis of cancer is devastating for families.

Cancer Treatment Complexity

Treatment is complex - multiple hospital stays.

Oncogenesis in Children vs Adults

Genes involved in oncogenesis are the same in children and adults.

Etiology of Cancer

Etiology of cancer varies in children and adults; it is largely unknown in children.