HEMATOLOGIC DISORDER ASSESSMENT FINDINGS 3. Promote an adequate Intake of iron-rich
Clinical manifestations: foods
Iron deficiency Anemia Pale skin 4. Provide child and family teaching
is caused by inadequate supply of iron Fatigue Emphasize proper
for normal red blood cell (RBC) Pica (eating nonfood items) administration of oral
formation. This results in smaller RBCs, Headaches, dizziness, and supplements
depleted mass, decreased hemoglobin lightheadedness Explain the potential adverse
level, and a decreased oxygen carrying Irritability effects of iron Caution the
capacity of the blood. Slowed thought process, parents about accidental
Iron deficiency anemia is the most decreased attention span, Ingestion of iron
preventable mineral disorder In children. spathy, and depression Discuss measures for
• In children, it typically occurs between ages 6 preventing Infections
mos and 3 years Laboratory and diagnostic study
findings: Sickle Cell Disease
ETIOLOGY Complete blood count will is a group of chronic, severe, genetic,
Inadequate dietary Iron Intake reveal normal to slightly reduced hemolytic diseases associated with
Iron malabsorption RBC's low hemoglobin and hemoglobin (Hb) S, which transform red
Low Iron stores at birth hematocrit, reduced mean blood cells (RBCs) into a sickle
Significant blood loss corpuscular volume (microcytic), (crescent) shape when blood
Excessive demands for iron required for and reduced mean corpuscular oxygenation is decreased.
growth hemoglobin (hypochromic) Sickle cell anemia is the most common
Erythrocyte protoporphyrin level form of sickle cell disease.
PATHOPHYSIOLOGY will be greater than 35 Sickle cell trait is the carrier state of the
Stage 1 - is characterized by depletion Iron tests will reveal low serum disorder. Those affected are usually
of hemosiderin, ferritin, and other iron iron capacity, decreased serum asymptomatic. However, under
storage. Complete blood count ferritin level, and elevated total conditions of extreme or prolonged
compounds in the bone ↳ marrow, liver, iron binding capacity. deoxygenation (for example, riding in an
and spleen. Reticulocyte count may be unpressurized aircraft), persons with
Stage 2 - is characterized by a lack of obtained 10 days after therapy is sickle cell trait may experience splenic
transport iron resulting in the decrease initiated to evaluate sequestration with profound anemia,
of iron saturation of transferrin. effectiveness which can be fatal.
Stage 3 - is characterized by a marked
deficit in transport iron, inhibiting the NURSING MANAGEMENT
normal production of hemoglobin. 1. Assess for fatigue, activity intolerance,
Erythrocyte protoporphyrin increases, and other signs of impaired tissue
and transferrin receptors become more oxygenation.
numerous in response to the iron poor 2. Administer prescribed medications or
environment. therapy
Oral iron
Prepared by: Sosa, Reycel Jean A. BSN2
Parental Iron
transfusions
, ETIOLOGY Stroke 5. Prevent infection
Sickle cell disease is an autosomal Chest Syndrome 6. Foster normal growth and development
recessive disorder, therefore, there is a Overwhelming Infections 7. Support the child and family
25% chance of each child having sickle 8. Provide child and family teaching
cell disease when both parents carry LABORATORY AND DIAGNOSTIC STUDIES:
the trait SICKLEDEX SCREEN, the most widely Hemophilia
used test, will detect the used test, will is a group of hereditary bleeding
detect the presence of Hb S but disorders characterized by a deficiency
ASSESSMENT FINDINGS may yield false negative results before In a blood clotting factor.
Clinical manifestations: ages 4 to 6 months. the two most common forms are
Enlarged spleen from If Sickledex findings are positive, FACTOR 8 DEFICIENCY (classic
congestion with sickled cell • hemoglobin electrophoresis is needed hemophilia, hemophilia A) and FACTOR
Enlarged and tender liver from to distinguish between the sickle cell 1X DEFICIENCY (Christmas disease,
blood stasis trait and the disease. Hemoglobin hemophilia B). The classic form is the
Hematuria electrophoresis should be done at birth most common.
Inability to concentrate urine on all neonates Hemophilia is classified as mild,
Enuresis Chorionic will sampling (CVS) or moderate, or severe, depending on the
Nephrotic syndrome analysis of fetal blood or cells may level of factor the body produces.
(occasionally) reveal sickle cell disease prenatally.
Bone weakness Complete blood count will reveal a ETIOLOGY
Dactylitis decreased RBC and elevated white Hemophilia is an X-linked recessive
Other problems include: blood cell and platelet counts disorder transmitted by females and
Stroke Neonatal screening for sickle cell found predominantly in males.
Myocardial infarction anemia is performed in most of the It may also be caused by a gene
Growth retardation United States. mutation (as many as one third of
Delayed sexual maturation Erythrocyte sedimentation rate will be cases)
Decreased fertility decreased.
Priapism (unwanted, painful Iron tests will reveal an increased serum PATHOPHYSIOLOGY
erection) Iron level. In hemophilia A, there is a deficiency of
Serum RBC survival time will be or a defect in factor VIII (Antihemophilic
SICKLE CELL CRISIS is usually precipitated by decreased. factor), which is necessary for the
infection, but possibly by dehydration, fever, Reticulocyte count will reveal formation of thromboplastin.
cold exposure, hypoxia, strenuous exercise, reticulocytosis. In hemophilia B, there is a defect or
extreme fatigue, or extreme changes in altitude. deficiency of factor IX. Clotting factor
NURSING MANAGEMENT malfunction causes abnormal bleeding
Sickle cell crisis may occur in different forms: 1. Promote tissue oxygenation owing to impaired ability to form a fibrin
Vaso-occlusive crisis 2. Administer appropriate therapeutic clot.
Splenic sequestration measures
Aplastic crisis 3. Relieve pain
Hyper hemolytic crisis 4. Help ensure adequate hydration and a Prepared by: Sosa, Reycel Jean A. BSN2
Megaloblastic anemia nutritionally balanced diet
Clinical manifestations: foods
Iron deficiency Anemia Pale skin 4. Provide child and family teaching
is caused by inadequate supply of iron Fatigue Emphasize proper
for normal red blood cell (RBC) Pica (eating nonfood items) administration of oral
formation. This results in smaller RBCs, Headaches, dizziness, and supplements
depleted mass, decreased hemoglobin lightheadedness Explain the potential adverse
level, and a decreased oxygen carrying Irritability effects of iron Caution the
capacity of the blood. Slowed thought process, parents about accidental
Iron deficiency anemia is the most decreased attention span, Ingestion of iron
preventable mineral disorder In children. spathy, and depression Discuss measures for
• In children, it typically occurs between ages 6 preventing Infections
mos and 3 years Laboratory and diagnostic study
findings: Sickle Cell Disease
ETIOLOGY Complete blood count will is a group of chronic, severe, genetic,
Inadequate dietary Iron Intake reveal normal to slightly reduced hemolytic diseases associated with
Iron malabsorption RBC's low hemoglobin and hemoglobin (Hb) S, which transform red
Low Iron stores at birth hematocrit, reduced mean blood cells (RBCs) into a sickle
Significant blood loss corpuscular volume (microcytic), (crescent) shape when blood
Excessive demands for iron required for and reduced mean corpuscular oxygenation is decreased.
growth hemoglobin (hypochromic) Sickle cell anemia is the most common
Erythrocyte protoporphyrin level form of sickle cell disease.
PATHOPHYSIOLOGY will be greater than 35 Sickle cell trait is the carrier state of the
Stage 1 - is characterized by depletion Iron tests will reveal low serum disorder. Those affected are usually
of hemosiderin, ferritin, and other iron iron capacity, decreased serum asymptomatic. However, under
storage. Complete blood count ferritin level, and elevated total conditions of extreme or prolonged
compounds in the bone ↳ marrow, liver, iron binding capacity. deoxygenation (for example, riding in an
and spleen. Reticulocyte count may be unpressurized aircraft), persons with
Stage 2 - is characterized by a lack of obtained 10 days after therapy is sickle cell trait may experience splenic
transport iron resulting in the decrease initiated to evaluate sequestration with profound anemia,
of iron saturation of transferrin. effectiveness which can be fatal.
Stage 3 - is characterized by a marked
deficit in transport iron, inhibiting the NURSING MANAGEMENT
normal production of hemoglobin. 1. Assess for fatigue, activity intolerance,
Erythrocyte protoporphyrin increases, and other signs of impaired tissue
and transferrin receptors become more oxygenation.
numerous in response to the iron poor 2. Administer prescribed medications or
environment. therapy
Oral iron
Prepared by: Sosa, Reycel Jean A. BSN2
Parental Iron
transfusions
, ETIOLOGY Stroke 5. Prevent infection
Sickle cell disease is an autosomal Chest Syndrome 6. Foster normal growth and development
recessive disorder, therefore, there is a Overwhelming Infections 7. Support the child and family
25% chance of each child having sickle 8. Provide child and family teaching
cell disease when both parents carry LABORATORY AND DIAGNOSTIC STUDIES:
the trait SICKLEDEX SCREEN, the most widely Hemophilia
used test, will detect the used test, will is a group of hereditary bleeding
detect the presence of Hb S but disorders characterized by a deficiency
ASSESSMENT FINDINGS may yield false negative results before In a blood clotting factor.
Clinical manifestations: ages 4 to 6 months. the two most common forms are
Enlarged spleen from If Sickledex findings are positive, FACTOR 8 DEFICIENCY (classic
congestion with sickled cell • hemoglobin electrophoresis is needed hemophilia, hemophilia A) and FACTOR
Enlarged and tender liver from to distinguish between the sickle cell 1X DEFICIENCY (Christmas disease,
blood stasis trait and the disease. Hemoglobin hemophilia B). The classic form is the
Hematuria electrophoresis should be done at birth most common.
Inability to concentrate urine on all neonates Hemophilia is classified as mild,
Enuresis Chorionic will sampling (CVS) or moderate, or severe, depending on the
Nephrotic syndrome analysis of fetal blood or cells may level of factor the body produces.
(occasionally) reveal sickle cell disease prenatally.
Bone weakness Complete blood count will reveal a ETIOLOGY
Dactylitis decreased RBC and elevated white Hemophilia is an X-linked recessive
Other problems include: blood cell and platelet counts disorder transmitted by females and
Stroke Neonatal screening for sickle cell found predominantly in males.
Myocardial infarction anemia is performed in most of the It may also be caused by a gene
Growth retardation United States. mutation (as many as one third of
Delayed sexual maturation Erythrocyte sedimentation rate will be cases)
Decreased fertility decreased.
Priapism (unwanted, painful Iron tests will reveal an increased serum PATHOPHYSIOLOGY
erection) Iron level. In hemophilia A, there is a deficiency of
Serum RBC survival time will be or a defect in factor VIII (Antihemophilic
SICKLE CELL CRISIS is usually precipitated by decreased. factor), which is necessary for the
infection, but possibly by dehydration, fever, Reticulocyte count will reveal formation of thromboplastin.
cold exposure, hypoxia, strenuous exercise, reticulocytosis. In hemophilia B, there is a defect or
extreme fatigue, or extreme changes in altitude. deficiency of factor IX. Clotting factor
NURSING MANAGEMENT malfunction causes abnormal bleeding
Sickle cell crisis may occur in different forms: 1. Promote tissue oxygenation owing to impaired ability to form a fibrin
Vaso-occlusive crisis 2. Administer appropriate therapeutic clot.
Splenic sequestration measures
Aplastic crisis 3. Relieve pain
Hyper hemolytic crisis 4. Help ensure adequate hydration and a Prepared by: Sosa, Reycel Jean A. BSN2
Megaloblastic anemia nutritionally balanced diet
