Transcript 1. Sickle cell disease

Blood Components
• Plasma:
– The liquid part of blood. All the blood cells are suspended
in this liquid.
– Contains dissolved salts (electrolytes) and proteins
• Albumin helps keeps blood vessels from leaking and carries
hormones and drugs to different parts of the body.
• Antibodies (immunoglobulins) that defend the body against
viruses, fungi, and cancer cells
– Serves as a reservoir that can absorb replenish or absorb
water from tissues when necessary.
– Prevents blood vessels from collapsing and clotting by
keeping them filled and circulating
– Plays a role in warming and cooling the body
Blood Components
Red Blood Cells
– Erthrocytes: Make up 40% of the blood’s volume
– Produced in the bone marrow
– Contain hemoglobin, a protein that gives blood its red color and
enables it to carry oxygen.
• White Blood Cells
– Leukocytes: Fewer in number than RBCs (1:660)
– Primary responsibility: Defend the body against infection
• Platelets
– Thrombocytes: cell-like particles smaller than RBCs and WBCs.
– Help with clotting process by gathering at bleeding site and
clumping together to form a plug that helps seal the blood vessel.
Blood cells
Granulocytes
Problems of Erythrocyte Production
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Anemia – reduction of RBC volume or Hgb
concentration below normal
Classifications:
1. Etiology/Pathophysiology –
causes of RBC/Hgb depletion
2. Morphology – changes in RBC
size, shape, and color
Causes of Anemia
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Nutritional deficiency – iron, folate, B12
Increased destruction of RBCs – sickle
cell anemia
Impaired or decreased rate of
production – aplastic anemia
Excessive blood loss - hemophilia
Iron Deficiency Anemia
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Causes
- inadequate supply of iron
- impaired absorption
- blood loss
- excessive demands for iron req’d for
growth
- inability for form Hgb
Iron Deficiency Anemia
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Signs and Symptoms: due to tissue hypoxia >
lack of energy, easy fatigability, pallor
Diagnosis: CBC with diff, red cell indices (MCV,
MCH, MCHC), iron studies, physical exam
Medical Treatment: supplement with ferrous
sulfate (dosages vary with age), dietary
counseling
Nursing Assessment and Interventions
- educate parents about nutrition
- explain laboratory testing
- teach parents proper administration
of iron preparations, caution about
high toxicity of iron
1. Sickle cell disease
In sickle cell anemia (Hgb S cell
disease), an abnormal gene results
in production of an irregular red
blood cell called hemoglobin (Hgb) S
that replaces some of the normal
hemoglobin A.
The red blood cells collapse into a
crescent shape (sickling) when
stressed such as during dehydration,
hypoxemia, or acidosis.
Sickle Cell Disease
Pathophysiology:
- vaso-occlusion from sickled RBCs
, increased RBC destruction leads
to the main manifestations of
the disease, which include:
splenic congestion and
enlargement, hepatomegaly,
liver failure, renal ischemia,
hematuria
• premature death of the cells
(hemolytic anemia)
• increased susceptibility to
infection
Sickle Cell Disease
• The frequency of one
abnormal gene is the
African-American
population is 1 in 12 and
the incidence of sickle cell
anemia is 1 in 650
• The frequency of the gene
is also high in
Mediterranean and
African populations
Sickle Cell Anemia
Signs/Symptoms:
• Exercise intolerance
• Anorexia
• Jaundiced sclera
• Gallstones
• Chronic leg ulcers
• Growth retardation
Sickle Cell Anemia
Diagnosis
• - Sickledex
- Hgb electrophoresis
- Stained blood
smear
• Vaso-occlusive crisis
(resulting in necrosis)
• mild to severe bone
pain
Humeral head infarction and osteonecrosis in a
50 year old female with sickle cell disease
Complications of Sickle Cell Disease
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pain episodes
strokes
increased infections
leg ulcers
bone damage (osteonecrosis)
yellow eyes or
jaundice
early gallstones
lung blockage
• kidney damage and loss
of body water in urine
• painful erections in men
(priapism)
• blood blockage in the
spleen or liver
• eye damage
• anemia
• delayed growth
Treatment of Sickle Cell Disease
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There is no specific treatment for sickle cell
disease, therefore, most therapy is supportive in
treatment of the complications
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Early recognition of infection, administration of
prophylactic antibiotics, and vaccination may
forestall or prevent other complications
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If a painful crisis persists or there is infection of a
major organ (brain, lung, or heart), exchange
transfusion is performed to remove some of the
sickle red cells - the effect is temporary
Treatment of Sickle Cell Disease
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General guidelines
– Taking the vitamin folic acid (folate) daily to help make
new red cells
– Daily penicillin until age six to prevent serious infection
– Drinking plenty of water daily (8-10 glasses for adults)
– Avoiding too hot or too cold temperatures
– Avoiding over exertion and stress
– Getting plenty of rest
– Getting regular check-ups from knowledgeable health
care providers
Prognosis of Sickle Cell Disease
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Prognosis has improved with good supportive care,
and many people with sickle cell disease survive
into middle age
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However, frequent admissions for painful crises, the
complication of sickle cell disease, narcotic use and
abuse due to chronic pain, and absence from school
and work lead to significant psychological and
vocational problems
Sickle Cell Anemia
Nursing care:
• Minimize tissue deoxygenation
• Promote hydration
• Minimize crises
• Pain management
• Administering blood transfusions
• Encourage screening and genetic counseling
• Parent education
Family Teaching
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Teach proactive care to prevent episodes/crisis:
- Adequate fluid intake to prevent dehydration.
- Avoiding infection or early treatment.
- Moderate activity and adequate rest to avoid fatigue and
hypoxia.
Early signs of impending crisis: splenic palpation to detect
sequestration.
Stress need for immediate care if there are signs of crisis.
Genetic testing and counseling:
Explain that SCA is an autosomal recessive condition
requiring the gene from both parents. Encourage testing of
siblings to allow for childbearing planning.
Support child and family with emotional responses,
grieving, and coping.
Hemophilia
• Hemophilia is a group of X-linked recessive
disorders that result in deficiency in one of the
coagulation factors 8 or factor 9 deficiency
• in the blood. X-linked recessive disorders are
transmitted by carrier mothers to their sons,
so usually only males are affected by
hemophilia.
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There are several types of hemophilia, including:
1. Factor VIII deficiency (hemophilia A).
2. Factor IX deficiency or Christmas disease (hemophilia B).
3. Factor XI deficiency (hemophilia C).
The most common type is hemophilia A, occurs when there is a
deficiency of factor VIII in an individual. Factor VIII is essential in the
activation of factor X, which is required for the
• conversion of prothrombin into thrombin resulting in an inability of
the platelets to be used in clot formation.
• Hemophilia is classified according to the severity of the disease,
ranging from mild to severe. The more severe the disease, the more
likely there will be bleeding episodes
• Clinical Manifestation:
• 1. Prolong bleeding any where from the body, with
severe factor deficiencies, circumcision, loss of
deciduous teeth or a result of a slight fall or bruise.
• 2. Subcutaneous or intramuscular hemorrhage is
common.
• 3. Hemarthrosis, bleeding into the joint cavities
especially knee, ankle, elbow.
• 4. Epistaxis (bleeding from the nose)
• 5. Bleeding into the tissue serious if occur into the neck,
mouth, or thorax.
• 6. Hemorrhage anywhere the gastrointestinal tract, can
lead to obstruction.
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Diagnosis:
1. History of bleeding
2. Chromosomal studies.
3. Blood tests, bleeding time, clothing time,
PTT etc.
Nursing care:
• Prevent bleeding
• Recognize and control bleeding
- Rest
- Ice
- Compression
- Elevation
• Prevent crippling effects of bleeding
• Client education
Thalassemia: (Cooley’s anemia)
• Thalassemia: (Cooley’s anemia)
• Thalassemia is an autosomal-recessive
disorder in which the alpha or beta
polypeptide chains in hemoglobin A are
impacted. Refers to those people living near
the Mediterranean sea.
Thalassemia: (Cooley’s anemia)
Types of thalassemia,
alpha and beta.
In alpha-thalassemia, synthesis of the alpha
chain of the hemoglobin protein is affected.
Problems with the beta chain occur more often,
and the condition beta-thalassemia can be
divided into three subcategories based on
severity:
• Thalassemia minor (also called betathalassemia trait): leads to mild microcytic
anemia often no treatment is required.
• 2. Thalassemia intermedia: child requires
blood transfusions to maintain adequate
quality of life.
• 3. Thalassemia major: to survive the child
requires ongoing medical attention, blood
transfusions, and iron removal (chelation
therapy).
• Pathophysiology:
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In beta-thalassemia major, the beta-globulin chain in
hemoglobin synthesis is reduced or entirely absent.
• A large number of unstable globulin chains accumulate,
causing the RBCs to be rigid and hemolyzed easily.
• . An overproduction of RBCs (immature cells) may result
in compensation for the hemolysis.
• The increased activity causes massive bone marrow
expansion and thinning of the bony cortex.
• Growth retardation, pathologic fractures, and skeletal
deformities (frontal and maxillary bossing) result.
• Red blood cells have short life span which is easily destructed. This
lead increase iron level and iron storage in the tissue called
hemosiderosis.
• Hemochromatosis: excessive iron storage in liver, skin, pancreas,
heart, joints and testes resultant cellular damage which cause
fibrosis of vital organs: heart failure, liver cirrhosis, diabetes and
splenomegaly.
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The excess iron is deposited in the body’s tissues, causing bronze
pigmentation of the skin
• If left untreated, beta-thalassemia major is fatal, but the use of
chelation therapy has increased the life expectancy of these children
to beyond their teen years
• Signs and symptoms:
• Thalassemia minor occurs as asymptomatic.
• Thalassemia
intermedia
manifests
with
splenomegaly and moderate to severe anemia.
• Thalassemia major (Cooley anemia) causing
progressive chronic anemia: Hypoxia, headache,
irritability, pallor, fatigue, and poor feeding.
Bronzed complexion: iron-containing pigment may
be noted due to breakdown of RBCs
• If untreated, bone changes such as enlarged
head and other facial changes may be noted.
• Medical treatment:
• keep Hgb > 9.5 g/dL.
• Deferoxamine (Desferal), an iron chelating agent, with oral
vitamin C may be administered to promote iron excretion
• Splenectomy may be done to decrease destruction of blood
cells.
• Bone marrow transplantation may be done in some children.
• After splenectomy, patient is at risk for infection and should
receive vaccines to prevent influenza, meningitis, and
pneumonia in addition to regular immunizations.
Nursing Interventions:
• Support child during illness and distressing treatments. .
• Monitor closely for complications of the condition and
treatment:
• Genetic counseling:
• Encourage testing of siblings to allow for childbearing
planning.
• Explain that each pregnancy when both parents are
carriers presents a 25% chance a child will be born with
the disease and a 50% chance the child will have the
thalassemia trait.
Idiopathic Thrombocytopenic Purpura(ITP)
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Causes: acquired hemorrhagic disorder of unknown
origin, probably an autoimmune response to diseaserelated antigens could be viral infection lead to
produces antiplatelet antibodies. These antibodies
destroy platelets. Excessive destruction of platelets
results in deficiency (thrombocytopenia) leading to
bleeding disorders. Which then leads to the
development of petechiae, purpura, and excessive
bruising. Purpura is a hemorrhagic rash in which
blood collects under the tissues and they are
purplish.
Idiopathic Thrombocytopenic Purpura(ITP)
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Diagnosis: platelet count < 20,000, abnl
bleeding time and clot retraction
Signs and Symptoms: petechiae, bruising, bleeding
from mucous membranes, prolonged bleeding from
abrasions
Complications include severe hemorrhage and
bleeding into vital organs and intracranial
hemorrhage
Medical management:
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Supportive
Steroids [corticosteroides (prednisone)].
, Anti-D antibody
Restriction of child activities.
intravenous gamma globulin increase production of
platelets.
• Platelet transfusions are not indicated unless a lifethreatening condition such as intracranial
hemorrhage is present.
• Splenectomy (after 5 years of age)
Nursing Considerations
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Client/Parent teaching
No contact sports
No aspirin
Prevent infection
Limits activity
Blood Transfusion
Complications:
• Hemolytic reactions
- chills, shaking, fever
- dyspnea
- flank pain
- progressive signs of shock
• Febrile reactions
• Allergic reactions
- urticaria, flushing
- wheezing
• Circulatory overload
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