Assessment and Management of Patients With Hematologic Disorders

Diagnostic Studies of the Hematologic System

Diagnostic Studies of the Hematologic System

The nurse should recognize the need to thoroughly explain any diagnostic procedures to the patient. It is common for a patient to be anxious when faced with illness. Therefore instructions must be simple, clear, and repeated when necessary to decrease anxiety and ensure the patients compliance with preparatory protocols.

Laboratory Studies
Complete Blood Count. involves several laboratory tests each of which serves as to assess the three major blood cells formed in the bone marrow. when the entire CBC is suppressed, a condition termed pancytopenia exists. Red Blood Cells. Hemoglobin (Hb)- measurement of gas-carrying capacity of RBC. NORMAL VALUES: Women = 12-16g/dl (120-160g/L) Men = 13.5-18g/dl (135-180g/L) It is reduced in cases of anemia, hemorrhage, and sates of hemodilutiion, such as those that occur when the fluid volume is excessive. Increases in hemoglobin are found in polycythemia or in states of hemoconcentration, which can develop from volume depletion.

Laboratory Studies
Hematocrit (Hct)- measure of packed cell volume of RBC expressed as a percentage of the total blood volume.
NORMAL VALUES:
Women = 38-47% (.38-.47) Men = 40-54% (.40-.54)

It represents the percentage of RBC as compared to the total blood volume. Reduction and elevation of hematocrit value are seen in the same conditions that raise and lower hemoglobin value. The hematocrit value generally equals three times the hemoglobin value.

Laboratory Studies
White Blood Cells- WBC differential is of considerable significance because it is possible for the total WBC count to remain essentially normal despite a marked change in one type of leukocyte.
NORMAL VALUES:
4000-11,000/l (4-11x109/L) Neutrophils: 50-70% Eosinophils: 2-4% Basophils: 0-2% Lymphocytes: 20-40% Monocytes: 4-8%

report WBCs in order of maturity with the less mature forms on the left side of the written report. Consequently the existence of many immature cells is termed a shift to the left.

Laboratory Studies
Platelet Count- measurement of number of platelets available to maintain platelet clotting functions.
NORMAL VALUES:
150,000-400,000/l (150-400x109/L)

bleeding may occur when the platelet count is depressed, which is condition termed thrombocytopenia.

Laboratory Studies
Erythrocyte Sedimentation Rate.
measures the sedimentation or settling of RBCs and is issued as a nonspecific measure of many diseases, especially inflammatory conditions. used as a screening routine procedure. Increased ESRs are common during acute and chronic inflammatory reactions when cell destruction is increased. Also found in persons with malignancy, myocardial infarction, and end-stage renal disease.

Laboratory Studies
Blood Typing and Rh Factor.
blood group antigens (A and B) are found only on RBC membranes and from the basis for the ABO blood typing system. Presence or absence of one or both of the two inherited antigens is the basis for the four blood groups

Laboratory Studies
Lymphangiography
it is a radiologic visualization of the lymph system after the injection of dye. the purpose of this procedure is to assess deep lymph nodes. this test may use in conjunction with the other tests such as CT scan or gallium scans to fully identify lymph node involved with cancer. allergies to shellfish and iodine, and previous reactions to dyes, should be ascertained before doing a lymphangiogram.

Laboratory Studies
PROCEDURE:
Begins with the intracutaneous injection of a blue dye into the webs of the toes. The dye is absorbed by the lymph vessels, making them visible through skin on the dorsum of the foot. Once visible the dorsum of each foot is injected with a local anaesthetic agent, and a small superficial incision is made over the lymph vessels. The lymph vessel is then cannulated with a small needle. When the lymph vessels are cannulated, radiopaque oil is injected slowly by means of an automated pump. (the usual dose of oil for an adult is 7 ml in each foot administered for a duration of 45-60 minutes)

Laboratory Studies
Biopsies
these procedures are done when a diagnosis cannot be established from a peripheral blood smear or when more information about the possible hematologic problem is needed.

Laboratory Studies
Bone Marrow Examination it is important in the evaluation of many hematologic disorders. it involves the aspiration of or biopsy of bone marrow with a syringe and needle. The aspirate is made into smears that are useful to cytologic diagnosis. site of bone marrow aspiration is determined by the age of the patient and the skill of the physician or specially credential nurse.

Laboratory Studies
PROCEDURE:
skin over the puncture site is cleansed with bactericidal agent. The skin, subcutaneous tissue, and periosteum are inflated with a local anaesthetic agent. systemic tranquilizers or analgesics are often administered before the procedure to minimize pain and decrease anxiety. once the area is anesthetized the special marrow needle is inserted through cortex of the bone. The stylet of the needle is then removed, the hub is attached to a 10 ml syringe, and 0.2 to 0.5 ml of the fluid marrow is aspirated. after the marrow aspiration, the needle is removed. Pressure applied over the aspiration site to ensure hemostasis. *if the patient is thrombocytopenic, pressure may be required for 5-10 minutes or longer*

Laboratory Studies
Lymp Node Biopsy it involves obtaining lymph tissue for histologic examination to determine the diagnosis and therapy. may be accomplished by either an open biopsy or a closed (needle) biopsy.

***open biopsy*** An incision is made, and the lymph node and surrounding tissue are dissected whenecer possible. Care must be taken because neoplastic cells can be disseminated during the biopsy procedure if the scalpel passes through tissues containing cancerous cells. It is performed in the operating room using either local or general anesthesi.

Laboratory Studies
***closed biopsy*** May also be performed to analyze lymph tissue. Sterile technique is essential throughout the procedure. Frequent observations of the site for bleeding and monitoring vital signs should be done, especially if the platelet count is low. Sterile dressing should be changed as ordered, and the wound should be inspected for healing and infection. It is important to recognize that if a needle biopsy is negative, it may signify only that the cancer cells were not a part of the tissue in the biopsy specimen.

ANEMIA

What is ANEMIA?

Is a condition in which the hemoglobin concentration is lower than normal;

Normal Values:
Females:12-16 gm/dL or 1.86-2.48 mmol/L Males: 13-18 gm/dL or 2.02-2.79 mmol/L

 It reflects the presence of fewer than normal number of erythrocytes within the circulation As a result, the amount of oxygen delivered to body tissues is also diminished. Anemia is not a specific disease state but a sign of an underlying disorder. It is the most common hematologic condition.

What are the COMPLICATIONS? Heart Failure Delirium Paresthesias Angina

CLASSIFICATIONS OF ANEMIA

HYPOPROLIFERATIVE ANEMIAS
-the deficiency in erythrocytes is caused by a defect in their production

 Results when the intake of dietary iron is inadequate or hemoglobin synthesis. It is the most common type of anemia in all age groups and in the world.

1. IRON DEFICIENCY ANEMIA

 Causes:

1. IRON DEFICIENCY ANEMIA

Ulcers

In men and postmenopausal women:

Bleeding from:

Gastritis
Inlflammatory Bowel Disease GI tumors

In premenopausal women:
Menorrhagia

 Pregnancy with inadequate iron supplementation

1. IRON DEFICIENCY ANEMIA

Chronic Alcoholism
Chronic blood loss from GIT

Iron Malabsorption
Usually after gastrectomy or with celiac disease

 Diagnostics:

1. IRON DEFICIENCY ANEMIA

Bone Marrow Aspiration

Aspirate is stained with iron, which is in low levels

CBC
Hemoglobin Hematocrit RBC

 Clinical Manifestations:

1. IRON DEFICIENCY ANEMIA

If deficiency is severe or prolonged:

Smooth, sore tongue Brittle and ridged nails Angular cheilosis fissuring and scaling of the lips

 Nursing Management:

1. IRON DEFICIENCY ANEMIA

Organ meats (beef, chicken, liver) Beans (black, garbanzo) Green leafy vegetables Raisins Molasses

Encourage increase intake of iron-enriched foods with a source of Vitamin C that enhances the absorption of iron.
Food sources high in iron:

 Encourage the patient to continue iron therapy as long as it is prescribed, although the patient may no longer feel fatigued.

1. IRON DEFICIENCY ANEMIA

Antacids or dairy products should not be taken with iron because it decreases iron absorption. Iron is best absorbed in an empty stomach
Take iron supplement 1hour before meals If there is GI distress: Take iron supplements with meals but it decreases iron absorption by 50%.

 Provide oral hygiene

1. IRON DEFICIENCY ANEMIA

Liquid forms of iron causes staining of the teeth. Inform patient that iron salts may color the stool dark green or black which is just normal. Do not massage the injection site after injecting iron through IM administration.

Use Z-track on IM administration to minimize side effects of iron.

 Medical Management:

1. IRON DEFICIENCY ANEMIA

Iron supplements for 6 to 12 months.

e.g. Ferrous sulfate, Ferous gluconate, Ferous fumarate

In some cases, when oral iron is poorly absorbed, tolerated or when iron supplementation is needed in large amounts, IV or IM administration may be needed.
A small test dose is done to avoid anaphylaxis
If negative after 30 mins., administer the remaining dose

Keep epinephrine at bedside in case of emergency

2. APLASTIC ANEMIA
A rare disease caused by:
Decrease in or damage to marrow stem cells Damage to the microenvironment withn the marrow Replacement of the marrow with fat

2. APLASTIC ANEMIA
Causes:
Unknown T cells mediate in inappropriate attack against the bone marrow resulting in
Bone marrow aplasia markedly reduced hematopoiesis. In additon to severe anemia, significant neutropenia (dec. neutrophils) and thrombocytopenia (dec. platelet) also occurs.

2. APLASTIC ANEMIA
Infections Chemicals
Benzene and benzene derivatives (e.g. airplane glue, paint remover, dry-cleaning solutions) may produce marrow aplasia

Toxic materials
Inorganic arsenic, glycol ethers, plutonium, radon

2. APLASTIC ANEMIA
Diagnostics:
Bone Marrow Aspiration
Extremely hypoplastic or even aplastic (very few to no cells) marrow replaced with fat.

2. APLASTIC ANEMIA
Clinical Manifestations:
Often insidious
Fatigue Pallor Dyspnea Purpura (bruising) Lymphadenopahy Splenomegaly Retinal hemorrhage

2. APLASTIC ANEMIA
Nursing Management:
Assess for signs and symptoms of infection and bleeding.
Patients with aplastic anemia are vulnerable on problems related to erythrocyte, leukocyte and platelet deficiencies.

Monitor for side effects of therapy, particularly hypersensitivity reaction while administering Antithymocyte agent (ATG).

2. APLASTIC ANEMIA
Monitor for long-term effects of cyclosporine therapy like:
Renal or liver dysfunction Hypertension Pruritus Visual impairement Tremor Skin cancer

Advise patient not to abruptly stop their immunosuppresive therapy.

2. APLASTIC ANEMIA
Medical Management:
Immunosuppressive therapy Immunosuppressants prevents the patients lymphocytes from destroying the stem cells.
Cyclosporines or androgens Antithymocyte globulin (ATG) a purified gamma-globulin solution obtained form rabbits or horses immunized with human T lymphocytes.
Side effects: fever and chills Report sudden onset of rashes and bronchospasm.

2. APLASTIC ANEMIA
Surgical Management
Bone Marrow Transplant (BMT)
Donor should be:
Below 60 years old Healthy

Peripheral Blood Stem Cell Transplant

3. MEGALOBLASTIC ANEMIA
The erythrocytes that are produced are abnormally large and are called megaloblastic red cells. Other cells derived from the myeloid stem cell (platelets, non-lymphoid leukocytes) are also abnormal.

3. MEGALOBLASTIC ANEMIA
Causes:
Folic acid deficiency
Folic acid is found in green vegetable and liver. Folate deficiency occurs in people who rarely eat uncooked vegetables. Alcohol increases folic acid requirements.

Vitamin B12 deficiency

Crohns disease, after ileal resection or gastrectomy Absence of intrinsic factor that helps in the absorption o Vit. B12

 Diagnostics:

3. MEGALOBLASTIC ANEMIA
Schilling test
Patient receives a small oral dose of radioactive vitamin B12, followed in few hours by a large, nonradioactive parenteral dose of vitamin B12.
If no radioactivity is present in the urine, the cause is GI malabsorption of the vit. B12

Same procedure is done but this time intrinsic factor is added to the oral radioactive vitamin B B12.
If radioactivity is detected in the urine, the diagnosis of pernicious anemia can be made.

3. MEGALOBLASTIC ANEMIA
Bone marrow analysis
There is hyperplasia (abnormal increase in the number of cells)

3. MEGALOBLASTIC ANEMIA
Clinical Manifestations:
Signs and symptoms of anemia Smooth, sore, red tongue Mild diarrhea Confusion Paresthesias in extremities They lose position sense (propioception)

3. MEGALOBLASTIC ANEMIA
Nursing Management:
Assess patients gait and stability. Provide assistance in managing daily activities. Provide assistive devices such as canes, walkers. Give small amounts of bland and soft foods frequently.

3. MEGALOBLASTIC ANEMIA
Medical Management:
Folic acid supplements Vitamin B12 supplements
IM absence of intrinsic factor Oral prevent or treat deficiency of vit. B12.

HEMOLYTIC ANEMIA
-the erythrocytes have shortened lifespan; thus, their number in the circulation is reduced. Fewer erythrocytes result in decreased available oxygen, causing hypoxia, which in turn stimulates an increase in erythropoietin release from the kidney.

SICKLE CELL ANEMIA

A severe hemolytic anemia tha results from inheritance of the sickle hemoglobin gene.
This gene causes hemoglobin molecule to be defective.

The sickle hemoglobin (HbS) acquires a crystal-like formation when exposed to low oxygen tension.
The oxygen level in venous blood can be low enough to cause this change; consequently, the erythrocyte containing HbS loses its round, pliable, biconcave disk shape and becomes deformed, rigid and sickle shaped.

SICKLE CELL ANEMIA

Diagnostics:
Hemoglobin electrophoresis
Normal: normal hematocrit, normal hemoglobin and normal blood smear. Result: low hematocrit and sickle cells on the blood smear.

SICKLE CELL ANEMIA

Prognosis:
Patients with sickle cell anemia are usually diagnosed in childhood, because they become anemic in infancy ad begin to have sickle cell crises at 1 or 2 years of age. Some children die in the first years of life, typically of infection, but antibiotic use and parrent teaching strategies have greatly improved the outcomes for these children.

SICKLE CELL ANEMIA

Clinical Manifestations:
Anemia Jaundice (particularly in the schlera) Tachycardia Cardiac murmurs Cardiomegaly Dysrhythmias Thrombosis

SICKLE CELL ANEMIA

Other symptoms:
Sickle Cell Crisis 3 types:
Sickle crisis most common and very painful which results form hypoxia and necrosis due to inadequate blood flow to a specific region of tissue or organ. Aplastic crisis results from infection with the human parvovirus. The hemoglobin level falls repidly and the marrow cannot compensate, as evidenced by an absence o reticulocytes.

SICKLE CELL ANEMIA

Sequestration crisis results when other organs pool the sickled cells.

Acute Chest Syndrome

Manifested with cough, fever, tachycardia and infiltrates seen on the chest x-rays. Caused by atypical bacteria such as Chlamydia pneumoniae and Mycoplasma pneumoniae as well as viruses such as respiratory syncytial virus(RSV).

SICKLE CELL ANEMIA

Pulmonary Hypertension
Most common sequela of sicke cell disease and often the cause of death.

SICKLE CELL ANEMIA

Nursing Management:
Managing Pain
Elevate swollen joint until it diminishes. Encouraged relaxation techniques and breathing exercises. Provide whirpool baths and transtcutaeneous electrical nerve stimulation (TENS).

SICKLE CELL ANEMIA

Preventing and Managing Infection
Monitor signs and symptoms of infection. Administer antibiotics as ordered. Encourage patient to complete the entire course o antibiotic therapy.

Monitoring and Managing Potential Complications

Leg ulcers scrupulous aseptic technique is warranted to prevent nosocomial infections.

SICKLE CELL ANEMIA

Priaprism leading to impotence Priaprism is the persistent penile erection that may lead to vascular thrombosis then impotence. Advise patient to empty his bladder at the onsent of the attack, exercise ad take a warm bath. If an episode persists longer than 3 hours, medical attention is recommended.

SICKLE CELL ANEMIA

Medical Management:
Peripheral blood stem cell transplant
PBSCT may cure sickle cell anemia. However, this treatment modality is available to only a small subset of affected patients, because of either the lack of a compatible donor or because severe organ damage that may be already present in the patient is contraindicated for PBSCT.

 Pharmacologic therapy Hydroxyurea (Hydrea) Increases fetal hemoglobin levels in patients with sickle cell anemia, thereby decreasing the formation of sickle cells. Side effects: chronic suppression of leukocyte formation, teratogenesis and potential for later development of a malignancy. Arginine Has antisickling properties and enhances the availability of nitric oxide, the most potent vasodiator, resulting in decreased pulmonary artery pressure.

SICKLE CELL ANEMIA

SICKLE CELL ANEMIA

Transfusion Therapy
RBC transfusion
Prevents severe complications from anesthesia and surgery. Improving the response to infection. Diminishes episodes of sickle cell anemia crisis in pregnant women. Prevents or manages complications from sickle cell disease (stroke, chronic heart failure, pulmonary hypertension)

SICKLE CELL ANEMIA

Risks of complications from transfusion: Iron overload Poor venous access Infections (hepatitis, HIV) Alloimmunization Increased blood viscosity

Polycythemia

Polycythemia
refers to an increase volume of erythrocytes(RBCs) term used when hematocrit is elevated More than 55% in males More than 50% in females

Polycythemia
Risk Factors
chronic hypoxia long-term cigarette smoking familial and genetic predisposition living in high altitudes long-term exposure to carbon monoxide (tunnel workers, car garage attendants, residents of highly polluted cities) Ashkenazi Jewish ancestry (may have increased frequency of polycythemia vera due to genetic susceptibility

Polycythemia
Diagnostics: Patients comprehensive medical history, physical examination, family history, and social and occupational history to evaluate a patient with polycythemia Routine blood work including a compete blood count (CBC), clotting profile, and metabolic panel basic components of laboratory tests in assessing the cause of polycythemia

Polycythemia
Chest X-rays, electrocardiogram (ECG), echocardiogram, hemoglobin analysis, and carbon monoxide measurement
typical tests to determine the potential causes of polycythemia

Bone marrow examinations (bone marrow aspiration or biopsy)

necessary to examine blood cell production in the bone marrow

Checking for the JAK2 gene mutation

diagnostic criterion for polycythemia vera

Classifications of Polycythemia

Classifications of Polycythemia

Polycythemia vera/ Primary polycythemia

Is a proliferative disorder of the myeloid stem cells. Marrow stem cell lines proliferate without an increase EPO stimulus.

Causes
Idiopathic

Classifications of Polycythemia
S/sx
Ruddy complexion Splenomegaly Headache Dizziness Tinnitus Fatigue Paresthesia Blurred vision
Claudication Angina Dyspnea Thrombophlebitis Elevated BP Elevated uric acid Generalized prurits Erythromyalgia

Classifications of Polycythemia
Secondary Polycythemia Caused by excessive production of erythropoietin. Erythropoietin is a hormone secreted in the kidney in response to a reduction in the amount of oxygen reaching the tissues. It increases and controls the rate of RBC production. Causes Heavy smoking Hemoglobinipathies Neoplasms (e.g., renal cell carcinoma) Heart disorders (e.g., cyanotic heart disease) Lung disorders (e.g., COPD) Living at a high altitude

Polycythemia
Pathophysiology:

Polycythemia

Nursing Management:
Educate the client Assess the risk factors for thrombolytic complications, particularly smoking, obesity, and poorly controlled hypertension. Patient should be instructed about signs and symptoms of thrombosis. Sedentary behavior, crossing legs, and wearing tight or restrictive clothing must be discouraged to reduce the likelihood of DVT.

Polycythemia
Patients with a history of significant bleeding are advised to avoid aspirin and aspirin-containing medications, because these medications alter platelet function. Minimizing alcohol intake should also be emphasized to further diminish the risk of bleeding. Instruct patient to avoid iron supplement, including those within multivitamin supplements, because the iron can further stimulate RBC production. For pruritus, encourage patient in bathing in tepid or cool water and avoiding vigorous towelling off after bathing.

Polycythemia
Medical Management:
The objective of management is to reduce the high blood cell mass. For secondary polycythemia, if mild treatment may not be necessary; when treatment is necessary, it involves treating the primary conditions. Phlebotomy Involves removing enough blood (initially 500mL once or twice weekly). Reduce blood viscosity and to deplete the patients iron stores, thereby rendering the patient iron deficient and consequently unable to continue to manufacture erythrocytes excessively.

Polycythemia
Pharmacotherapy Chemotherapeutic agents (e.g., hydroxyurea) used to suppress marrow function, but this may increase risk of leukemia. Anagrelide (agrylin) Inhibits platelet aggregation, can also be useful in controlling the thrombocytosis a/w polycythemia vera. May be hard to tolerate by patients because it can cause significant side effects, including headache, fluid retention, cardiac dysrythmias, and heart failure, furthermore it may be leukomogenic(may cause leukemia)

Polycythemia
Interferon alfa-2b (Intron-A) Most effective treatment for managing pruritus a/w polycythemia vera. May cause frequent side effects like depression and flu like syndrome. Antihistamine Allopurinol(zyloprim) Used to prevent gouty attacks in patients with elevated uric acid concentrations. Aspirin Prevents thrombolytic complications and is also useful in reducing the pain a/w erythromyalgia.

Hemophilia

Hemophilia
Hemophilia is a group of bleeding disorders. It is caused by low amounts of specific clotting factors. These factors help to stop bleeding. If you have hemophilia, you may bleed for a longer time than others after an injury. You also may bleed inside your body (internally), especially in your knees, ankles, and elbows. This bleeding can damage your organs and tissues and may be life threatening.

Hemophilia
The most common types of hemophilia are:
Hemophilia A (classic hemophilia)
accounts for 80% of all hemophilia (1 in 5,000 males), caused by too little factor VIII

Hemophilia B (Christmas disease)

occurs in 1 in 25,000 males, caused by too little factor IX

Hemophilia
Pathophysiology:
In hemophilia A, there is a deficiency of, or a defect in, factor VIII (antihemophilic factor [AHF]), which is necessary for the formation of thromboplastin. In hemophilia B, there is a defect or deficiency of factor IX. Clotting factor malfunction causes abnormal bleeding owing to impaired ability to form a fibrin clot.

Hemophilia
Causes
Hemophilia is caused by a faulty gene inherited from one or both parent. It is called an inherited sex-linked recessive gene. The gene is located on the X chromosome. Females carry two copies of the X chromosome. If the faulty gene is only on one X, the normal gene on the second X will take over. As a result, they will not get the disease. Instead they are carriers of the gene. The risk for their male offspring to inherit the gene is 1 in 2 or 50%. The chance that their female offspring will inherit the gene is also 50%. These offspring will be carriers like their mothers.

Hemophilia
It is possible for a female to have hemophilia. For this to happen she must inherit the faulty gene from both her mother and her father. Males carry only one X chromosome. If they get the faulty gene the disease will develop.

 Genetic Outcome Possibilities

2011 Nucleus Medical Media, Inc.

Hemophilia
Risk Factors
Factors that increase your chance of hemophilia include:
Family members with hemophilia Family history of bleeding disorders
Sex: male

Hemophilia
Diagnostics:
Coagulation studies look at the ability of the blood to clot. These studies involve many tests. They are done if the person tested is the first one in the family with the disorder. Once identified, other family members will need less testing for a diagnosis. The doctor will ask about your symptoms and medical history. A physical exam will be done. Tests may include:
Tests of bleeding time Blood tests to measure the amounts of clotting factors present

Hemophilia
Clinical Manifestations
The severity of symptoms can vary. Severe forms become apparent early on. Bleeding is the main symptom. It may be noticed if an infant is circumcised. Additional bleeding problems appear when the infant becomes mobile. Bleeding can be caused by an injury. It can produce excessive bruising after a minor trauma or cut. It can also cause bleeding after extraction of teeth. Bleeding into joints is a common sign. This is called hemarthrosis. Intestinal bleeding may produce bloody stools. If it occurs in the small intestine it will cause black, tarry stools.

Hemophilia
Mild cases may go unnoticed until later in life. Then they occur in response to surgery or trauma. Symptoms for Hemophilia A and B may include:
Hot, swollen, sore, stiff, and/or deformed joints Bleeding into muscles Bleeding into the intestinal tract Bloody stools Bloody urine

Hemophilia
Difficulty stopping bleeding after minor cuts or bumps Heavy bleeding even after simple surgical or dental procedures

Symptoms for Hemophilia B may include:

Nosebleeds Excessive bleeding after circumcision

Hemophilia
Complications may include:
Bone changes, osteoporosis and muscle atrophy, resulting in crippling deformities as a consequence of hemarthrosis Intracranial bleeding Gastrointestinal hemorrhage, leading to intestinal obstruction Hematomas in the spinal cord, resulting in paralysis Airway obstruction due to bleeding into the neck, mouth or thorax

Hemophilia
Nursing Management:
Assess for acute or chronic bleeding
The skin, joints and muscles are assessment priorities Check vision, hearing, and neurologic development. Check for hematuria and bleeding from the mouth, lips, gums and rectum. Administer the missing clotting factor (ie factor VIII or factor IX concentrate)

Hemophilia
Administer DDAVP (desmopressin) to children with mild to moderate hemophilia A.
DDAVP promotes the release of factor VIII. It is not used in hemophilia B.

Prevent or minimize bleeding

Assess home safety and teach about injury prevention. Consider the childs developmental level to ask specific safety questions. Recommend using a soft toothbrush and point out the need for regular dental checkups:.

Hemophilia
Major bleeding requires hospitalization with nursing management.
1. Monitor for bleeding and its consequences. 2. Provide joint care (exercise).

Control bleeding by applying pressure and cold to the injury site and by elevating and immobilizing the injured area. Observe for swelling and tenderness in the joints, and prevent contractures. Prevent crippling effects of joint degeneration by implementing a physical therapy program. Monitor for signs of hypovolemia

Hemophilia
Provide Support
Foster the childs self-esteem by encouraging him or her to express concerns and feelings and by promoting a positive selfimage. Encourage family members to verbalize their feelings, especially about any guilt they may have due to the genetic nature of the disorder. Assist their coping efforts by providing information about the disease and its management. Refer to the child and family to support groups such as the national hemophilia Foundation.

Hemophilia
Provide the child and family teaching.
Explain how to care for, administer, store and reconstitute the replacement factor. Inform the child and family that superficial injuries are treated with ice and pressure. Identify signs of hemarthrosis and teach parents how to immobilize the joint, pack it in ice, and administer replacement factor.

Hemophilia
Assist the child and parents to recognize signs of major bleeding (central nervous system manifestations such as headache, blurred vision, vomiting, lethargy, confusion and seizures) Explain the possible side effects of therapy. Demonstrate passive ROM exercises. Emphasize avoidance of aspirin and aspirin-containing compounds. Provide diet information because weight increase can impose further stress on joints.

Hemophilia
Medical Management:
The main treatment for hemophilia is replacement therapy giving or replacing the clotting factor that is too low or missing. Concentrates of the clotting factor are infused, or injected, directly into the bloodstream. The specific factors used to treat hemophilia are: Factor VIII for hemophilia A Factor IX for hemophilia B Replacement therapy can be used: To prevent bleeding (prophylactic or preventive therapy) To stop bleeding when it occurs, on an as-needed basis (demand therapy)

Hemophilia
The type of treatment you receive depends on several things, including whether you have mild, moderate or severe hemophilia.
Mild hemophilia. Replacement therapy is usually not needed for mild hemophilia; however, a medicine called desmopressin (DDAVP) is sometimes given to raise the bodys levels of factor VIII. Since the effect wears off with chronic use, it is applied only in certain situations (for example, prior to dental work or participation in sports) to prevent or reduce bleeding. Desmopressin does not help in hemophilia B.

Hemophilia
Moderate hemophilia. You may need treatment only when bleeding occurs. You will need to learn to recognize signs and symptoms of bleeding so that you can get treatment as quickly as possible. You also may have treatment to prevent bleeding that could occur when participating in some activity. Severe hemophilia. You usually need long-term or shorter term preventive therapy to prevent bleeding that could cause permanent damage to your joints, muscles or other parts of the body. Some people with severe hemophilia receive treatment only when bleeding occurs, however.

Complications of Anticoagulant Therapy

Disseminated Intravascular Coagulation

Disseminated Intravascular Coagulation

DIC is not a disease but a sign of an underlying condition. Causes:
Sepsis Trauma Cancer Shock Abruptio Placentae Toxins or allergic reaction

Disseminated Intravascular Coagulation

Pathophysiology:

Disseminated Intravascular Coagulation

Diagnostics:

Disseminated Intravascular Coagulation

Clinical Manifestations:

Disseminated Intravascular Coagulation

Disseminated Intravascular Coagulation

Nursing Management:
Avoid procedures/activities that can increase intracranial pressure (e.g. coughing, straining to have a bowel movement) Monitor vital signs closely, including neurologic checks:
Monitor hemodynamics Monitor abdominal girth Monitor urine output

Avoid medications that interfere with platelet function if possible (e.g. ASA, NSAIDs, beta-lactam antibiotics)

Disseminated Intravascular Coagulation

Avoid rectal probes, rectal medications. Avoid IM injections. Monitor amount of external bleeding carefully. Administer oral hygiene carefully. Reposition carefully.

Disseminated Intravascular Coagulation

Medical Management Treat the underlying cause. Correcting the secondary effects of tissue ischemia Improving oxygenation Replacing fluids Correcting electrolyte imbalances Administering vasopressor medications

Disseminated Intravascular Coagulation

If serious hemorrhage occurs Depleted coagulation factors and platelets may be replaced Cryoprecipitate is given to replace fibrinogen and factors V and VII Fresh-frozen plasma is administered Heparin administration Fibrinolytic inhibitors (e.g. aminocaproic acid) May be used with heparin

Disseminated Intravascular Coagulation

Recombinant activated protein C (e.g. APC, Drotrecogin alfa [Xigris]) Efective in diminishing inflammatory responses on the surface of the vessels as well as having anticouagulant properties.

END