Blood Disturbances Part Two

Other Anemias

• Thalasemia

• Glucose-6-Phosphate Dehydrogenase Defic

• Hereditary Spherocytosis

• Immune Hemolytic Anemia (remember blood transfusion)

Other RBC conditions

• Hereditary hemochromatosis

• Polycythemia

WBC Conditions

Leukopenia:

• a condition in which there are fewer WBC’s than normal

• results from neutropinia (diminished neutrophils) or lymphopenia (diminished lymphocytes)

• decrease in other WCB’s don’t usually result in total WCB decrease

Lymphopenia

• Lymphocytes less than 1500/mm3

• as a result of ionizing radiation, long-term use of corticosteroids, uremia, some neoplasms such as breast, lung and advanced Hodgkin’s

Neutropenia

• Neutrophils less than 2000/mm3

• results from a decrease in production or an increase in destruction

• essential for preventing and limiting bacterial infection

• pt is at risk for infection

• discovered on routine exam,

• treatment, look for cause

Leukocytosis and Malignancies

• Leukocytosis is an increase in WCB’s, typically one type

• usually neutrophils or lymphocytes

• WCB increased with infection and then decreases normally, abnormal is when there is prolonged or excessive increase

Leukemias

• Unregulated proliferation of WCB’s in the bone marrow (sometimes in liver and spleen)

• cause is generally unknown, may be genetic, viral or a result of benzene exposure

• leukemias are classified by the stem cell line involved ie lymphoid or myeloid

Leukemias

• Also classified as acute or chronic

• Acute Leukemia has an abrupt onset of symptoms. Progresses very fast, death within weeks to months without aggressive treatment

• Chronic, symptoms evolve over a year and WCB’s are mature, progress slowly

Types of Leukemias

• Acute Myeloid Leukemia

• Chronic Myeloid Leukemia

• Acute Lymphocytic Leukemia

• Chronic Lymphocytic Leukemia

Nursing the Pt with Leukemia

• Major goals may include:

• attainment and maintenance of comfort

• attainment and maintenance of adequate nutrition, self care, activity tolerance, ability to cope with the diagnosis and prognosis, positive body image, understanding of the disease process and its treatments, absence of complications

Interventions

Managing mucositis

• oral care

• decrease bacteria in mouth, maintain moisture, provide comfort

• due to antibiotics, chemo,

Interventions

Improving Nutritional Intake

• pt has increased metabolic rate and therefore increased nutritional requirements

• intake may be reduced due to pain, mucositis, nausea

• small frequent feedings (soft)

Intervention

Easing Pain and Discomfort

• frequent fevers are common

• myalgias and arthralgias common

• sweating common

• sponging, mouth care, tylenol

• skin care, massages

Intervention

Decreasing Fatigue and Reconditioning

• assist pt with activity-rest balance

• physical therapy is a good thing

Interventions

Improving Self-care

• encourage pt to do as much as possible for themselves to preserve mobility and function as well as self-esteem

• assist with self care after treatments

• teach about things such as Hickman Line

Interventions

Encouraging Spiritual Well-being

• offer support to enhance the patients spiritual well-being

• spiritual and religious practices should be assessed

• allow for hope

Interventions

Monitoring and Managing Potential Complications

Malignant Lymphomas and Multiple myeloma

• Lymphomas are neoplasms of cells of the lymphoid origin

• usually start in the lymph nodes but can involve lymphoid tissue in the spleen, the GI tract, the liver or the bone marrow

• classified according to degree of cell differentiation and the origin of the predominant malignant cell

• Hodgkin’s or non Hodgkin's Lymphoma

Hodgkin’s Disease

• A rare malignancy with an impressive cure rate

• more common in men than women

• two peaks of incidence; early 20’s and after 50

• spreads by contiguous extension along the lymphatic system

• cause unknown but 20% have epstein - barr virus

Hodgkin’s

• There is a familial pattern,

• classified into four subgroups based on analysis that reflects the grade of malignancy and the prognosis

• minimal node involvement the prognosis is better

Manifestations

• Usually a painless enlarged lymph node on one side of the neck

• in 20%, severe pain after drinking alcohol usually at the site

• symptoms result from tumor compressing organs

• herpes zoster is common

• Fever, night sweats, wt loss are found in 40% of pts and are more common in advanced disease

• mild anemia, up or down WCB, platelet count normal, unless tumor has invaded bone marrow

Diagnosis

• Biopsy of lymph node

• chest xray, bone marrow, CT scan, blood counts,

Treatment

• Goal is cure

• based on stage of disease

• chemo, radiation

• sterility and other side effects

Non-Hodgkin's

• Spread is unpredictable

• prognosis varies, long term survival is commonly achieved in low-grade lymphomas

Bleeding Disorders

• Can be a result of platelet or coagulation factor abnormalities or, vascular abnormalities.

• Signs and symptoms vary with the cause

• abnormalities of the vascular system give rise to local bleeding usually in the skin

Platelet defects

• Platelets are responsible for stopping bleeding from small vessels, patients with platelet defects develop petechiae, often in clusters on the skin and mucous membranes

• bleeding usually stops with pressure

Coagulation defect

• Do not tend to cause superficial bleeding because the primary hemostatic mechanism are still intact.

• Bleeding occurs deep within the body

• such as subcutaneous or intramuscular hematomas and hemorrhage into t he joint spaces

• external bleeding diminishes slowly and may reoccur

Medical management

• Based on underlying cause

• transfusion of blood products

• specific blood products based on underlying defect

Nursing Management

• Patients must be educated regarding their disease and preventative measures

• they should observe themselves for bleeding on skin, gums and nose

• observe urine, stool, vomit and other drainage for blood

• If bleeding starts, ABC’s

Hemophilia

• 2 hereditary bleeding disorder are clinically indistinguishable and can only be separated by laboratory tests. These are Hemophilia A and hemophilia B

• Hemophilia A is created by a genetic defect causing deficient or defective factor 8

• hemophilia B has defective factor 9

hemophilia

• Hemophilia A occurs in 1:10,000 and is three times more common than Hemophilia B

• Inherited on X linked traits some almost all pts are male, females carry the trait

hemophilia

• Usually diagnosed early in life after an injury

• 75% of hemorrhage occurs in the joints

• knees, elbows, ankles, shoulders, wrists, hips

• this bleeding results in ankylosis and many patients have severe joint limitation by adulthood

hemophilia

• Hematomas are deep or superficial and often peripheral nerves are compressed by the clot which may lead to weakness and atrophy of muscles

• spontaneous bleeding any where: GI, nose, rectal etc, most serious is the cranial bleed

• wound healing is slow

Medical management

• In the past pts received plasma in large volumes which resulted in fluid overload

• now the clotting factors can be extracted and given at the time of bleeding or as a preventative measure before procedures such as dental work or LP’s

• 2 meds to be familiar with are Amicar and DDAVP (desmopressin)

Nursing management

• Often nursed as pediatric age patients

• education re: the disease, genetics, lifestyle, coping etc.

• avoid ASA, nsaids or alcohol

• dental care vital

• avoid injections

• analgesics,

• HIV

Acquired Coagulation Disorders

• Liver disease: hepatic dysfunction can result in diminished amounts of the factors needed for maintaining coagulation and hemostasis

• Vitamin K deficiency: Vit K is required by the body for synthesis of many coagulation factors. Can be given sc or IM

• Anticoagulation Therapy: stop med, give Vit K for warfarin (coumadin), protamine sulfate for heparin

Disseminated Intravascular Coagulopathy

• Referred to as DIC• not a disease but a sign of a serious underlying

disease mechanism and is life-threatening• the normal hemostatic mechanism is distorted

and micro clots form in the microcirculation. These clots use up the platelets and clotting factors and therefore there are none left and pt bleeds out

Causes of DIC

• Trauma, sepsis, premature separation of the placenta, metastatic malignancies, hemolytic transfusion reactions, shock

Blood therapies

• Splenectomy

• therapeutic apheresis

• therapeutic phlebotomy

Have a Great Weekend!