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Peds Exam 3
Hematological Disorders of Childhood
| Question | Answer |
|---|---|
| Hematologic system roles | Carry oxygen Fight infection Control hemorrhage |
| Hematologic abnormalities | Increased number of cells – cytosis Decreased number of cells – cytopenia Destruction – lysis Production - poiesis |
| Normal Hematopoiesis | Formed cells in the blood (red cells, white cells, platelets) produced in the bone marrow – all start from a single pluri-potent stem cell and follow individual pathways of maturation |
| RBC function | transport oxygen to the tissues |
| RBC production is regulated by | tissue oxygenation |
| RBC is measured by | hgb, hct, reticulocyte |
| hgb | # of oxygen-carrying protein molecules used often in peds |
| hct | % of RBC’s in whole blood used often in adults usually 3x higher than hgb |
| reticulocyte count | % of progenitor cells in circulating blood |
| Normal life span for RBC | approximately 120 days |
| normal hgb | 11-13g/DL Important to look at pediatric norms for age |
| WBCs protect | the body from invasion of foreign organisms |
| WBCs have multiple | mechanisms for fighting infection |
| components of WBCs | granulocytes monocytes (macrophages) lymphocytes |
| granulocytes are | most important for fighting bacterial infection |
| granulocytes types | neutrophils eosinophils basophils |
| neutrophils | involved in phagocytosis, first to respond |
| eosinophils | control mechanism associated with allergy and parasitic disease |
| basophils | involved in inflammatory reactions during immune response. Also produce histamine, serotonin and heparin that prevents excess clotting |
| monocytes | phagocytosis of bacteria and cellular debris |
| Lymphocytes | stimulate macrophage production (T-lymphocytes) and mobilize antibodies (B-lymphocytes) part of immune response and are specific to each individual |
| normal value of WBCs | 4,500-10,000 mm3 |
| lifespan of WBCs | approx 12-14 hours |
| platelets are | Fragments of megakaryocytes produced in the marrow and stored in the spleen |
| platelets adhere to | the inner surface of a damaged vessel to form a homeostatic plug |
| what initiates the clotting cascade | degradation |
| normal range of platelets | 150- 500,000 mm3 |
| life span of platelets | approximately 5-7 days |
| difference between platelets and RBCs transfusion | may only need blood once a week because they last 120 days and platelets last 2-3 days |
| Alterations in Hematological Function can involve | single cell line or multiple cells lines simultaneously |
| Alterations in Hematological Function can arise from changes in | production - not making enough maturation/morphology - staying in immature state destruction - lysis |
| Alterations in Hematological Function are classified as | primary and secondary |
| primary Alterations in Hematological Function | problem lies within the hematological system |
| secondary Alterations in Hematological Function | alteration in function caused by problem in another organ system (e.g., cardiac, renal, drugs, etc.) |
| Treatment of Alterations in Hematological Function focuses on | managing deficiency correcting underlying problem |
| Assessment of Hematologic Function | Diagnostic studies Historical data Physical assessment findings Child’s energy and activity level Growth patterns |
| diagnostic studies to assess Hematologic Function | Complete blood count (CBC): RBC indices - (MCV, MCH, MCHC) WBC with differential Platelets |
| what type of disorder is anemia | red blood cell disorder |
| anemia is the | most common hematologic disorder of childhood |
| anemia is caused by | Decrease in the number of red blood cells (RBCs) and/or a hemoglobin (Hgb) concentration that is below normal Diminished oxygen-carrying capacity of blood |
| classification of anemia is based on | Etiology Morphology - size, shape, and color |
| symptoms of anemia will look different for | different age and sized children |
| classification of anemia | Decreased RBC production Increased RBC loss Increased RBC destruction |
| Decreased RBC production types | nutritional deficiency bone marrow failure |
| increased RBC loss types | acute blood loss |
| increased RBC destruction types | intracorpuscular extracorpuscular |
| Decreased RBC production symptoms | pallor tachy headache fatigue SOB muscle weakness systolic heart murmur Pica |
| increased RBC loss Symptoms | pallor tachy headache fatigue cool skin muscle weakness decreased peripheral pulses low bp |
| increased RBC destruction symptoms | icteric sclera pallor fatigue headache tachy dark urine splenomegaly hepatomegaly frontal bossing |
| nutritional deficiency anemia due to deficiency in | iron folate vitamin b12 copper chronic disease chronic blood loss |
| there are consequences of Anemia due to | Decrease in the oxygen-carrying capacity of the blood leads to a decreased amount of oxygen available to tissues |
| when anemia develops slowly, | the child adapts |
| consequences of Anemia | Hemodilution Decreased peripheral resistance Increased cardiac circulation and turbulence Several severe and longstanding symptoms |
| severe and longstanding symptoms of anemia | decreased cognitive function behavioral changes delayed growth exercise intolerance impaired immune function |
| Increased cardiac circulation and turbulence | May have murmur May lead to cardiac failure |
| Diagnostic Evaluation of Anemia through | History and physical examination findings CBC Other tests can be done to determine the particular type of anemia |
| History and physical examination findings for anemia | Nutritional history Family history Bowel habits |
| CBC diagnostic evaluation of anemia | Decreased RBCs Decreased hemoglobin and hematocrit (Hct) Typically, the hemoglobin is less than 10 or 11 g/dL |
| nursing considerations for anemia | Prepare the child and family for laboratory tests Decrease oxygen demands Prevent complications Support and educate the family |
| Iron Deficiency Anemia is the | Most preventable nutritional disorder in US |
| Iron Deficiency Anemia is caused by | an inadequate supply of dietary iron |
| inadequate supply of dietary iron can be due to | poor intake (most common) Absorption (seen in chronic diseases like IBS) Demand Synthesis |
| Iron Deficiency Anemia is generally | preventable |
| Iron Deficiency Anemia is | Predictable at developmental periods |
| what periods can Iron Deficiency Anemia be predicted at | Depleted Fetal iron stores At 12 to 36 months, due to ingestion of large amounts of cow’s milk in diet In adolescents, due to rapid growth and poor eating habits |
| Depleted Fetal iron stores | - Premature infants ~2-3 months - Term 5-6 months |
| Iron Deficiency Anemia has | Low MCV – microcytic |
| Therapeutic management - prevention of Iron Deficiency Anemia in infants | Breast milk or iron- fortified formula until 12 months Iron supplement with cereal 4-6 months Iron drops |
| Therapeutic management - prevention of Iron Deficiency Anemia in children | dietary counseling |
| best treatment for Iron Deficiency Anemia | prevention! |
| prognosis of Iron Deficiency Anemia | very good if cut out milk and increase iron and food |
| nursing considerations for Iron Deficiency Anemia | EDUCATION PO Iron given with citrus (NOT MILK)– most soluble state |
| sickle cell anemia inheritance pattern | autosomal recessive if 2 parents are carriers then 1 child has 50% carrier, 1 has 25% affected and 1 has 25% unaffected |
| Sickle Cell Anemia (SCA) incidence | Occurs primarily in African Americans Occurs in 1 in 375 infants born in the United States 1 in 12 has sickle cell trait |
| presence of SCA follows | malarial belt |
| malarial belt | African Caribbean Mediterranean South America Southeast Asia |
| Diagnosis of Sickle Cell Anemia | NB screening Genetic testing to identify carriers and children who have the disease Hemoglobin electrophoresis |
| Genetic testing to identify carriers and children who have SCA | Hgb SS Hgb SC SC trait –only 35-45% total Hgb is HgbS remainder is Hgb A |
| Pathophysiology of SCA crisis pt 1 | Partial or complete replacement of normal HgbA with abnormal hemoglobin S (HgbS) Hgb in the RBCs takes on an elongated “sickle” shape |
| Pathophysiology of SCA crisis pt 2 | Sickled cells are rigid and obstruct capillary bf Microscopic obstructions lead to engorgement Tissue ischemia and cell death |
| lifespan of hgb in SCA crisis | 15 days and normal is 120! |
| patho of SCA crisis: remember it this way | Obstruction…..inflammation……destruction |
| Pathophysiology of SCA: simple | abnormal hgb (hgbS) has triggering event which causes sickle shaped RBCs which leads to increased blood viscosity and increased RBC destruction |
| Sickle Cell Crisis: precipitating factors are essentially | Anything that increases the body’s need for oxygen or alters the transport of oxygen |
| Sickle Cell Crisis: precipitating factors examples | Trauma Fever, infection Physical and emotional stress Increased blood viscosity due to dehydration (so we want to keep them hydrated) Hypoxia |
| hypoxia as a trigger for sickle cell crisis | Results from high altitude, poorly pressurized airplanes, hypoventilation, vasoconstriction due to hypothermia NO ICE and WINTER is very HARD |
| Organs Affected by SCA crisis | every organ! so think about their pain and suffering |
| sickle cell crisis include | Acute exacerbations that will vary in severity and frequency |
| general effects of sickle cell crisis | Chronic Anemia (so will live with decreased hgb) Growth delays Delayed puberty Increase Susceptibility to sepsis (bc of infarcts to spleen so will overtime get aslpenia) |
| sickle cell crisis pts experience | dactylitis: Painful swelling hands in feet in infants |
| type of sickle cell crisis | Vaso-occlusive crisis (VOC) Splenic Sequestration |
| Vaso-occlusive crisis (VOC) | Most common type of crisis and is very painful Stasis of blood with clumping of cells in the microcirculation leads to ischemia and then infarction |
| signs of Vaso-occlusive crisis (VOC) | fever, pain, and tissue engorgement |
| sickle cell crisis: splenic sequestration is most common | between 2months – 5 years |
| sickle cell crisis: splenic sequestration occurs due to the | Trapping of RBC in spleen, abdominal pain, enlargement of spleen, rapid fall in Hgb, & drop in plt count |
| Splenic Sequestration is a | Life-threatening type - death can occur within hours |
| signs of Splenic Sequestration | Profound anemia Hypovolemia Shock |
| in splenic sequestration, | Recurrence is likely – treat with chronic transfusion or splenectomy Hypersplenia |
| Sickle Cell complications: Acute chest syndrome | Acute pulmonary complication |
| Acute pulmonary complication | Pulmonary infiltrate – Vasculature of lungs clogged with sickled cells AND 1 or more of the following: Chest pain Temperature > 38.5°C Tachypnea, wheezing cough or respiratory distress Hypoxemia |
| Sickle Cell complications: Cerebral vascular accidents (CVA) | Neurodevelopmental delay, mental retardation Silent infarct – TCD Change in LOC |
| Sickle Cell complications: chronic organ damage | lungs → pulmonary hypertension kidneys → progressive renal disease spleen → functionally asplenic by ~ 3 years Liver - cirrhosis brain – multiple CVA’s musculoskeletal – avascular necrosis |
| current life expectancy of sickle cell | 50-60 years |
| Medical Management of Sickle Cell Anemia: goals | Prevent sickling phenomena Promptly treat illnesses |
| prevention of sickling | Childhood vaccines - Pneumococcal and meningococcal when possible Possible prophylactic (Penicillin VK) antibiotics in children from 2 months to 5 years of age Diet and nutrition Medications – hydroxurea Good hydration |
| hydroxurea | increases fetal hgb so decreases sickled hgb |
| Medical Management of Sickle Cell Anemia: Prompt treatment of illnesses | Rest Aggressive treatment of infection Fluids – oral and IV Electrolyte replacement Pain management Exchange blood transfusion |
| Medical Management of Sickle Cell Anemia: options | Prompt treatment of illnesses Splenectomy at age 2 if necessary HSCT |
| Nursing Management of Sickle Cell Anemia | Monitor the child’s growth and watch for failure to thrive Carefully do a multisystem assessment Assess and treat pain Observe for the presence of inflammation or possible infection Carefully monitor for signs of shock Promote growth and development |
| Social and medical stigmatization of SCA | Perceived risk of addiction results in inadequate pain management Narcotic use for chronic pain leads to tolerance, dependence |
| Developmental concerns of SCA | Physical: reduced linear growth, delayed pubertal development Cognitive: cognitive deficits, academic disabilities Psychosocial: impact on QOL, peer relationships, self-esteem |
| Psychosocial Needs of SCA | Coping mechanisms Support with genetic counseling Financial needs Caregiver role strain Living with chronic illness in the family |
| Aplastic Anemia is a condition in which | all cell lines are simultaneous depressed - marrow is virtually empty of cellular components - pancytopenia |
| Primary Aplastic Anemia | (congenital) RARE = Fanconi syndrome (autosomal recessive inheritance) |
| Secondary Aplastic Anemia | (acquired) = agent that affects marrow function |
| what agents can cause Secondary Aplastic Anemia | infection (human parvovirus, hepatitis, overwhelming infection) household chemicals (paint remover, shellac, lacquers) medications (chemotherapy agents, anticonvulsants, antibiotics) irradiation |
| Aplastic Anemia is most frequently | idiopathic – unknown cause |
| Aplastic Anemia CMs | Pancytopenia Thrombocytopenia Anemia Neutropenia |
| Aplastic anemia diagnosis | Bone marrow Aspirate/ Biopsy r/o malignancy |
| Aplastic anemia treatment | Matched related transplant Immunosuppressive therapy Matched unrelated transplant |
| Management of Aplastic Anemia | Recognition of underlying disease process Education Support for child and family during prolonged period of uncertainty |
| How to support child and family during prolonged period of uncertainty | Preparation of diagnostic and therapeutic procedures Preventing complications from pancytopenia Emotional support – life threatening outcomes |
| Disorders of homestasis | Hemophilia Immune Thrombocytopenia Purpura (ITP) |
| Hemophilia is a group of | hereditary bleeding disorders that result from deficiencies of specific clotting factors |
| Hemophilia genetic pattern | Typically, an X-linked recessive pattern |
| prognosis of hemophilia | Historically, most patients died by 5 years of age Currently, patients with mild to moderate hemophilia live nearly normal lives |
| hemophilia A | Classic hemophilia (deficiency of factor VIII) Accounts for 80% of cases of hemophilia |
| hemophilia B | Christmas disease (deficiency of factor IX) |
| hemophilia classifications | severe moderate mild |
| severe hemophilia | <2% factor activity spontaneous bleeding without trauma |
| moderate hemophilia | 2-4.9% factor activity bleeding with trauma |
| mild hemophilia | 5-40% factor activity bleeding with severe trauma or surgery |
| Etiology of Hemophilia A | X-linked recessive trait Males are affected Females are the carriers |
| hemophilia A degree of bleeding | depends on the amount of clotting factor present and the severity of a given injury |
| in hemophilia A, up to one third of cases have | no known family history In these cases, disease is caused by a NEW mutation |
| Manifestations of Hemophilia | Bleeding tendencies range from mild to severe Symptoms may not occur until 6 months of age Hemarthrosis Epistaxis Bleeding in the gastrointestinal tract Bleeding after procedures |
| Hemarthrosis is | Bleeding into joint spaces of the knee, ankle, or elbow leads to impaired mobility and, eventually, bony changes and disability |
| symptoms of Hemarthrosis | warmth, pain, bruising, and decreased movement |
| Bleeding after procedures in hemophilia | Minor trauma, tooth extraction, minor surgeries Large subcutaneous and intramuscular hemorrhages may occur Bleeding into the neck, chest, or mouth may compromise the airway Bleeding in the spinal cord may cause paralysis |
| Medical Management of Hemophilia | Replacement of missing clotting factor Always treat if unsure Always give the entire vial Save and document lot # Exercise and physical therapy |
| Nursing Care Management of hemophilia | Prevent bleeding episodes Recognize and control bleeding Support the patient Support the family |
| Support the patient in hemophilia | Pain Mobility Promote growth and development |
| Support the family in hemophilia | Education Prepare for home care |
| Interventions for Hemophilia | Close supervision and safe environment Dental procedures in a controlled situation Shave only with an electric razor IM injections |
| Interventions for Hemophilia: superficial bleeding | apply pressure for at least 15 minutes and ice to promote vasoconstriction |
| Interventions for Hemophilia: if significant bleeding occurs, | transfusion for factor replacement |
| Managing Hemarthrosis | During bleeding episodes, elevate and immobilize the joint Ice Analgesics Range-of-motion exercises after the bleeding stops will help to prevent contractures Physical therapy Avoid obesity to minimize joint stress |
| Von Willebrand Disease is | Deficiency, abnormality, or absence of vWF and factor VIII |
| VWD affects | both males and females (autosomal dominant) pattern |
| Most cases of VWD are | mild and required intervention only for dental and surgical procedures |
| Increased mucous membrane bleeding in VWD | Gingival bleeding Easy bruising Excessive menstrual bleeding |
| treatment for VWD | DDAVP Humate-P |
| Immune (Idiopathic) Thrombocytopenic Purpura (ITP) is an | acquired hemorrhagic disorder |
| ITP is characterized by | Thrombocytopenia (excessive destruction of platelets) Purpura (discoloration caused by petechiae beneath the skin, with no other signs of bleeding) NORMAL bone marrow |
| ITP occurs in two forms | Acute, self-limiting (usually following a viral illness) Chronic (lasting longer than 12 months) |
| ITP diagnostic evaluation | Platelet count is less than 20,000/mm Rule out other diseases and conditions |
| therapeutic management of ITP | Observation only if preceded by viral ilness Steroids IVIG WinRho |
| prognosis of ITP is | good! |
| nursing considerations for ITP | make sure not at high risk for bleeding keep safe |
Created by:
leh195