Blood Disorders - EXAM 5

Question 1

What is sickle cell disease?

Answer 1

A group of inherited disorders characterized by the presence of abnormal sickle hemoglobin (Hbs) in the erythrocyte. The abnormal hemoglobin (HbS) causes the erythrocyte to stiffen and elongate taking on a sickle shape in response to low oxygen level.

Question 2

What are sickling episodes?

Answer 2

Common triggers, low O2 tension in blood, most commonly due to infection. Sickled cells cannot pass through capillaries causing vascular occlusion and tissue injury, abnormal cells are hemolyzed, causing anemia

Question 3

What is a sickle cell crisis?

Answer 3

A severe, painful, acute exacerbation of RBC sickling causing a vasoclusive crisis, leading to tissue ischemia, infection, and necrosis.

Question 4

What are the clinical manifestations of sickle cell disease?

Answer 4

Varies greatly. Typically anemic, but asymptomatic except during crisis. Primary symptom associated with sickling is pain.

1. Thrombosis or hemorrhage causing paralysis, sensory deficits, death
2. Acute chest syndrome, pulmonary hypertension, pneumonia
3. Hematuria, renal failure
4. Hand-foot syndrome, osteonecrosis
5. Hepatomegaly, gallstones
6. Priapism
7. Stasis ulcers of hands, ankles, and feet
8. Splenic atrophy (autosplenectomy)
9. Heart failure
10. Hemorrhage, retinal detachment, blindness, retinopathy

Question 5

What is the nursing and collaboartive management of sickle cell disease?

Answer 5

1. Prevention: avoid high altitude, maintain fluid intake and prevent infection
2. Sickle Cell Crises: oxygen, rest, and F+E, treat pain adequately, treat infections
3. Hematopoietic Stem Cell Transplant (HSCTs): replace bone marrow
4. Long term follow up required

Question 6

What is hemophilia A?

Answer 6

A sex-linked recessive genetic disorder caused by a defective deficiency of coagulation factor VIII or less often, factor IX

Question 7

What is the pathophysiology of hemophilia A?

Answer 7

The basic defect of hemophilia A is a deficiency of factor VIII (antihemophilic factor AHF). AHF is produced by the liver and is necessary for the formation of thromboplastin in phase I of blood coagulation. The less AHF found in the blood, the more severe the disease.

Question 8

What are the risk factors for hemophilia A?

Answer 8

Recessive X-linked (transmitted by female carriers, displayed almost exclusively in men)

Question 9

What are the clinical manifestations of hemophilia A?

Answer 9

1. Slow, persistent, prolonged bleeding for minor trauma and small cuts
2. Delayed bleeding after minor injuries (hours or days)
3. Uncontrollable hemorrhage after dental extractions
4. Epistaxis
5. GI bleeding
6. Hematuria
7. Ecchymoses and hematomas
8. Neurological signs and symptoms
9. Hemarthrosis (bleeding into the joint)

Question 10

What is the collaboartive care for hemophilia A?

Answer 10

1. Prevent and treat bleeding VIA replacement therapy
2. Treatment of complications of the disease and its treatment

Question 11

What is the nursing management of hemophilia A?

Answer 11

Assessment of clinical manifestations, determine blood levels of factor VIII, and administration of factor VIII. Assess for allergic reactions to AHF. During acute bleeding:

1. Direct pressure, ice, packing (gelfoam, fibrin foam)
2. Administer factor VIII intravenously
3. Immoblize joint and give analgesics
4. Manage life-threatening problems from hemorrhage (airway, intracranial bleeding)

Question 12

What are the causes of anemia?

Answer 12

1. Decreased RBC Production
2. Blood Loss
3. Increased RBC destruction

Question 13

What is the alteration/etiology for anemia caused by decreased erythrocyte production?

Answer 13

Ingredients for bone marrow recipe for RBCs are lacking.

1. Fe
2. Folic acid
3. Cobalamin B12
4. Erythropoietin

OR the bone marrow is not working

Question 14

What are examples of situations that would cause anemia related to decreased RBC production?

Answer 14

1. Fe deficiency
2. Folic acid deficiency
3. Cobalamin deficiency
4. Pernicious anemia
5. Aplastic anemia
6. Anemia of chronic disease

Question 15

What are the alteration/etiology for anemia caused by blood loss?

Answer 15

1. Acute blood loss
2. Chronic blood loss

Question 16

What are examples of situations that would cause anemia caused by blood loss?

Answer 16

Acute: trauma, gastric ulcer

Chronic: menstrual flow, hemorrhoids

Question 17

What are the alteration/etiology of anemia caused by increased erythrocyte destruction?

Answer 17

1. Two types: Intrinsic hemolytic anemia or extrinsic hemolytic anemia
2. When RBCs are hemolyzed, causes jaundice and hyperkalemia

Question 18

What are examples that could cause anemia caused by increased erythrocyte destruction?

Answer 18

Intrinsic: sickle cell disease

Extrinsic: prosthetic heart valves, extracorporeal circulation, dialysis

Question 19

What are risk factors for iron deficiency anemia?

Answer 19

Poor diet, women in reproductive years (menstration),. Iron deficiency anemia is extremely common. Risk factors include being very young, poor diet, being a woman in the reproductive years. Inadequate Fe intake, malabsorption, blood loss can lead to Fe deficiency iron supplements.

Question 20

What happens to the Hct when a person has rapid blood loss?

Answer 20

When acute hemorrhage occurs, RBCs, Hbg, and plasma are all lost in the same proportions. Hct is an indication of the % of RBCs to plasma and ratio does not change with sudden blood loss, Hct will not change drastically with sudden blood loss, even if it is a large loss. However, the body compensates by pulling in more fluid to reverse hypovolemia or IV fluids may be given which will change the Hct to a decreased value.

Question 21

What are the symptoms of anemia related to chronic blood loss?

Answer 21

1. may or may not be symptomatic
2. Patient adapts and may not complain of acute symptoms
3. A careful history may reveal activity intolerance and dyspnea on exertion which has come on slowly

Question 22

What happens to the building blocks of RBCs when cells hemolyze?

Answer 22

RBCs live for 120 days. Several things can cause them to rupture, lyse, or hemolyze prematurely. When large amount of destruction happens, the potassium inside the cell spills out into the circulating blood, causing an increased potassium level and a potential Fe overload. When hbg is liberated due to hemolysis of any cause, the hbg can clog the renal tubules which causes renal insufficiency due to acute tubular necrosis

Question 23

What are the top two nursing diagnoses for anemia?

Answer 23

Fatigue

Altered Nutrition: less than body requirements

Question 24

What routine assessments should be done for a hemophiliac?

Answer 24

1. Presence/continution of bleeding
2. Amount of blood loss
3. VS
4. Labs: hbg, hct, PTT, hematuria
5. Pain
6. Need for analgesia (no ASA)

Question 25

What activities are safe for a hemophiliac?

Answer 25

Quiet activities that keep injured part immoblized until the bleeding stops.

Question 26

What precautions should be instituted for a child in the hosptial for bleeding that has hemophilia A?

Answer 26

• Total rest of affected area/joint with ice, elevation, and analgesics
• Mobilization after bleeding ceases
• No weight bearing until all swelling has resolved
• Pad siderails to prevent bleeding secondary to trauma
• Sign in room that phlebotomies should be performed only by RNs

Question 27

What kind of pain is there in sickle cell disease?

Answer 27

Hemoglobin S is abnormal. When oxygen is lost, Hbg S changes shape. The result is distored RBCs that are stiff and not squeezable that block the blood vessel. This leads to pain and tissue damage and is associated with interrupted blood supply.

Babies: hands and feet swollen and painful

Older children: arms and legs

Teens and adult: spine or backbone

Treatment: heating pad, warm bath or tylenol after on ehour, may need to go to ED, learn techniques to tolerate

Question 28

What is the issue with infection regarding sickle cell patients?

Answer 28

Serious especially for babies. Spleen begins to be damaged within 1st 4 months of life. Lose ability to fight infections which may be deadly. Peniciliin may be prescribed regularly. Fevers must be treated quickly. At risk for severe infections espeically before age 5, but lifelong.

Question 29

What is chest syndrome in sickle cell patients?

Answer 29

Pneumonia is most common. Symptoms of cough or difficulty breathing should be assessed quickly.

Question 30

What neurological issue are sickle cell patients more at risk for?

Answer 30

stroke. blocked blood supply causes weakness and damage to brain

Question 31

Fatigue r/t inadequate oxygenation of the blood

Answer 31

Energy management.

1. Encourage alternate rest and activity periods to provide activity without tiring.
2. Limit number of visitors and interruptions by visitors
3. Limit environmental stimuli to decrease demands placed on patient
4. Assist with regular physical activities (ambulation, transfers, personal care)
5. Monitor cardioresp. response to activity to evaluate activity intolerance
6. Determine patient’s physical limitations
7. Determine what and how much activity is required to build endurance

Question 32

Altered Nutrition: less than body requirements r/t inadequate nutritional intake and anorexia and by weight loss, low serum albumin, decreased iron levels, vitamin deficiencies.

Answer 32

1. Determine in collaboration with dietician number of calories and type of diet needed to meet nutritional requirement to plan interventions
2. Teach how to keep a food diary to help evaluate nutritonal status
3. Encourage increased intake of protein, iron, and vitamin C to provide nutrients needed for hbg production
4. Provide appropriate information about nutritonal needs and how to meet them to increase intake of essential nutrients needed

Question 33

Ineffectuve Self-Health Management r/t lack of knowledge about appropriate nutrition and medication regimen

Answer 33

Nutritional counseling:

1. Facilitate identification of eating behaviors to be changed. Discuss knowledge of the 4 basic food groups, as well as perceptions of the needed diet modification
2. Discuss food buying habits and budget constraints
3. Use accepted nutritional standards to assist patient in evaluating adequacy of dietary intake
4. Discuss nutriitonal requirements and patient perceptions of prescribed diet
5. Provide referral/consultation with other members of health care team to help patient maintain gains and adjustments throughout recovery
6. Review with patient measurements of fluid I&O, hgb values, BP, weight gains/losses

Teaching:

1. Instruct patient on purpose and action of each med
2. Instruct on dosage, route, and duration of each med to improve compliance
3. Instruct patient on possible adverse effects of every med

Question 34

cyanocobalamin (vitamin B12)

Answer 34

Classification: vitamin

Mechanism of action: required for hematopoiesis (formation of blood)

Route for cyanocobalamin deficiency anemia:PO

Route for pernicious anemia: IM, deep SC, intranasal

Use: Im shots once/month for lifelong treatment of perinicious anemia (since GI absorption is non-existent d/t lack of intrinsic factor), Oral forms (daily) for treatment of cobalamin deficiency anemia, usually due to nutritional deficiency

Side/Adverse Effects: allergic reactions, pulmonary edema

Nursing Implications: Dosage/freq may vary, stinging/burning without injecting common and transitory, patient education on how to administer IM