Alterations in Hematologic Status

NURS 323

Major functions Production of cells Oxygenation Nutrient distribution to cells Immune protection Waste collection from cells heat

production

Common Diagnostic Assessments Family History Child’s health history Physical exam Medication use Dietary pattern Lab studies: CBC w/diff, PT, PTT, Hb, Hct,

iron studies, bone marrow aspiration/bx Prepare child & family for lab tests

Sickle Cell Anemia (SCA)

A global disease

Definition http://www.nhlbi.nih.gov/health/dci/

Diseases/Sca/SCA_WhatIs.html Sickle cell anemia results from the hereditary

presence of abnormal Hgb S in place of Hgb A. When Hgb S becomes deoxygenated (loses oxygen), it tends to form polymers that deform the red blood cells (RBCs) into a sickle shape. Sickled RBCs cannot pass freely through capillaries and cause blockage of small vessels. This can compromise the blood supply to various organs.

Incidence Hereditary hemoglobinopathy;

autosomal recessive; 1 African Americans

Hgb S trait is found in 8 to 10% of the American black population. Sickle Cell Anemia– homozygous form

(HbSS)—most prevalent in US In Western African native blacks, up to

40% of the population carry Hgb S trait.

Basic Defect A defect in the structure of Hemoglobin A—

substitution of 1 amino acid (valine) for another (glutamine) @ the 6th position of the beta polypeptide chain.

Hgb partially/completely replaced by Hgb S When triggered, Hgb S sickles & obstructs

cap. blood flow, s blood viscosity tissue ischemia

Results hemolysis (RBC destruction) & anemia;

Sickling reversible until repeated cycles leads to irreversible sickling

Diagnostic Tests Rarely seen before 4-6 mos. Sickledex: sickle turbidity test; finger or

heel stick; results in 3 min Sickledex does not distinguish between

sickle cell trait and sickle cell disease. Hemoglobin electrophoresis is required to distinguish between these 2 disorders.

Hgb electrophoresis to “fingerprint” protein Hx, PE, CBC CXR if symptoms

Diagnostic Tests (cont’d) Peripheral Blood Smear

The routine peripheral blood smear does not contain sickled red blood cells unless hypoxemia (inadequate levels of oxygen in the blood) or a precipitating factor (such as a red blood cell poison or drug) is present.

Diagnostic Tests (cont’d)

Electrophoresis Both sickle cell disease (homozygous for

hemoglobin S) and sickle cell trait (heterozygous for Hgb S) can be detected by this test.

This test separates different types of hemoglobin based on electrical charge. Hgb S can be differentiated from Hgb A and other types of Hgb.

Diagnostic Tests (cont’d)

Normal Results In hemoglobin electrophoresis, no Hgb S

should be present. Normal hemoglobins in an adult are mostly Hgb A with small amounts of Hgb A2 and Hgb F.

What do abnormal results mean? sickle cell trait sickle cell anemia

Pathophysiology Spleen—enlarged, if untreated,

becomes fibrous & ineffective susceptibility to infection.

Liver—failure & necrosis 2ndary to impaired circulation. Hepatomegaly and tenderness are common sx.

Kidney— perfusion 2ndary to congestion of glomerular capillaries & tubular arterioles hematuria, inability to concentrate urine, enuresis, & possible nephrotic syndrome.

Pathophysiology (cont’d)

Bones— Hyperplasia of bone marrow osteoporosis

weakened bones lordosis and kyphosis or other skeletal deformities

Chronic hypoxia possible osteomyelitis from salmonella

“Hand-foot Syndrome”—occurs in children 6 mos-2yrs. Caused by infarction of short-tubular bones.Sx: swelling & pain of soft tissue of hands & feet. Usually resolves in 2-3 wks.

Pathophysiology (cont’d)

CNS—primarily R/T ischemia MAJOR INFARCT—Stroke or CVA

paralysis MINOR INFARCT—HA, aphasia, weakness,

convulsions, visual changes 2ndary to retinopathy & retinal detachment.

Heart—Decompensation & failure 2ndary to chronic anemia CHF, tachycardia

Pathophysiology (cont’d)

Blood— Rate of destruction of RBC’s > rate

of RBC production RBC’s lifespan is to 10-15 days

(compared to 120 days for nml RBC) Hgb, Hct & RBC count Reticulocyte count, bilirubin &

uric acid

Pathphysiology (cont’d)

Other sx: Generalized weakness Anorexia Joint, back, and abdominal pain Fever Vomiting Possible leg ulcers Failure to thrive, rate of growth &

development

Sickle Cell Crisis Triggers

Acute illness Dehydration Strenous exercise Hypoxic conditions Trauma Respiratory illness

Results: acute pain, cell destruction, infarcts, organ damage

Types of Crises Vaso-occlusive: PAIN, swollen joints,

vomiting, fever, anorexia, dactylitis Acute sequestration: liver & spleen,

HR dyspnea, weakness, pallor, shock

Aplastic crisis: weakness, pallor, dyspnea, tachycardia, shock

Acute chest syndrome: chest pain, cough, fever, abdominal pain

Other complications Megaloblastic anemia Hyperhemolytic crisis Cerebrovascular injury (stroke) Overwhelming infections

TREATMENT Primary Goals:

1. Prevent Crisis (avoid sickling phenomenon)—through adequate oxygenation & hydration in every day life activities. Hydroxyurea may be used prophylactically in adolescents and older adults. Folic Acid 1mg daily is also recommended to restore folate stores needed for erythropoiesis.

2. Treat crisis as an emergency when it occurs—determine etiology quickly and treat appropriately

Nursing Management Adequate hydration (also any electrolyte

replacement if needed) Pain management Promote rest Treat any infections Blood transfusions if needed Monitor O2 – supplement if needed Oral hygiene Maintain nutrition Family support and education

NURSING CARE Pain Control—strive to prevent pain.

Opioids as morphine, oxycodone, hydromorphone & methadone are preferred. Give IV or PO for severe pain round-the-clock. PCA pumps are great! Avoid Demerol (Meperidine) - ↑seizure risk

Tylenol & Ibuprofen may be used after dx is made Use non-pharmacologic methods also Heat (e.g. K-pad)to affected joints may be

soothing by O2 with vasodilation.

NURSING CARE Hydration–

Accurate I & O essential IV/PO fluids up to 150ml/kg/day will promote

hemodilution Note color of urine—sometimes brown

Prevent tissue deoxygenation Avoid strenuous activity Avoid environments with low O2 concentration Avoid extremes in temp- esp. cold environment Avoid emotional stress Avoid known sources of infection

Child/Family Education How to manage & minimize crisis:

avoid dehydration, cold, stress, hypoxia, acute illness, etc.

Signs of infection Importance of immunizations Antibiotic administration if ordered Education & support re: long term

effects Genetic counseling

Thalassemia Major

Autosomal recessive Defective Hgb; RBCs fragile w/short

life span anemia, hemolysis Main Tx: blood transfusions Hemochromatosis – iron overload –

occurs from hemolysis of transfused and defective RBCs

Chelation tx done to eliminate excess Fe

Nursing Considerations Numerous blood transfusions Assess s/s of hemosiderosis Assess s/s infection Folic acid supplementation Iron chelation tx as ordered Family support Possible bone marrow transplant

Aplastic Anemia

Definition: Condition where all formed elements of the blood are depressed.

Pancytopenia or Triad: Anemia (↓ RBC's) Leukopenia (↓ WBC's) Thrombocytopenia (↓ platelets)

Etiology of A.A. Primary (congenital) Secondary (acquired) -- often

secondary to drugs e.g. chemotherapy or other meds that “shut down” bone marrow function

Diagnosis -- Bone marrow aspiration or biopsy

Therapeutic Management -- Goal: Restore bone marrow function Immunosuppressive therapy Bone marrow transplantation ** choice

85% survival rate Nursing Considerations -- Similar to

child with leukemia Prepare family for diagnostic &

therapeutic procedures Preventing complications from severe

pancytopenia Emotionally supporting them in terms

of a potentially fatal outcome

Hemophilia

Deficiency in specific clotting factor X-linked recessive trait; 2 most

common Hemophilia A (classic) Hemophilia B (Christmas Disease)

Uncontrolled bleeding from wounds into joints, muscles, & other body tissues

Spontaneous & severe uncontrolled bleeding in severe form

Common Labs Hx & PE, symptoms PTT prolonged Factor VIII, IX levels Normal: PT, thrombin, bleeding

time, fibrinogen, platelet count May do liver Bx to r/o other causes

Signs & symptoms Excessive bleeding from circumcision Bleeding w/teething Hemarthosis – cardinal sign Bruises in various stages Hematuria Nose bleeds Oral bleeding

Nursing Management

Control localized bleeding: Direct pressure for extended period of time like

10 minutes Ice Elevation of affected limb

Avoid injections when possible No rectal temps Monitor for bleeding (joints, urine, stool, etc) Provide pain relief Monitor neuro status if possible head bleeds

Nursing Management

Administration of Factor replacement as needed

Factor VIII concentrate (Antihemophilia factor {AHF} ), or DDAVP , a synthetic form of vasopressin that is the treatment of choice in mild hemophilia & von Willebrand disease. Should increase Factor VIII activity 3-4 fold.

Teaching Considerations Teach family factor administration/storage DDAVP administration Avoid ASA type products Avoid contact sports Medic alert tag Avoid constipation Good nutrition Good oral hygiene Genetic counseling Protective environment for infants/toddlers

Idiopathic Thrombocytopenia Purpura (ITP)

Excessive destruction of platelets Purpura (from petechiae under skin)

Normal bone marrow Most common bleeding disorder in

children 80% between 2-5 yrs old and usually last

~ 6m Usually after viral infection Symptoms when platelet ct < 20,000

Therapeutic Management

IF medical tx is indicated, then may use one of the following regimens Prednisone IVIG—expensive and takes long to

administer and monitor pt Anti-D antibody—controversial but may

prolong survival of platelets. Can be given in 1 dose over 5-10 minutes.

Nursing Considerations Stress activity restriction Prevention of head injuries; instruct

parents to get med eval if head or abd injury

Assess for bleeding/bruising Oral care support No rectal temps – No ASA products Splenectomy if has for 1 yr and tx not

successful Family support & teach on how to control

bleeds

There you have it!