What is sickle cell disease?
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.
What are sickling episodes?
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
What is a sickle cell crisis?
A severe, painful, acute exacerbation of RBC sickling causing a vasoclusive crisis, leading to tissue ischemia, infection, and necrosis.
What are the clinical manifestations of sickle cell disease?
Varies greatly. Typically anemic, but asymptomatic except during crisis. Primary symptom associated with sickling is pain.
- Thrombosis or hemorrhage causing paralysis, sensory deficits, death
- Acute chest syndrome, pulmonary hypertension, pneumonia
- Hematuria, renal failure
- Hand-foot syndrome, osteonecrosis
- Hepatomegaly, gallstones
- Priapism
- Stasis ulcers of hands, ankles, and feet
- Splenic atrophy (autosplenectomy)
- Heart failure
- Hemorrhage, retinal detachment, blindness, retinopathy
What is the nursing and collaboartive management of sickle cell disease?
- Prevention: avoid high altitude, maintain fluid intake and prevent infection
- Sickle Cell Crises: oxygen, rest, and F+E, treat pain adequately, treat infections
- Hematopoietic Stem Cell Transplant (HSCTs): replace bone marrow
- Long term follow up required
What is hemophilia A?
A sex-linked recessive genetic disorder caused by a defective deficiency of coagulation factor VIII or less often, factor IX
What is the pathophysiology of hemophilia A?
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.
What are the risk factors for hemophilia A?
Recessive X-linked (transmitted by female carriers, displayed almost exclusively in men)
What are the clinical manifestations of hemophilia A?
- Slow, persistent, prolonged bleeding for minor trauma and small cuts
- Delayed bleeding after minor injuries (hours or days)
- Uncontrollable hemorrhage after dental extractions
- Epistaxis
- GI bleeding
- Hematuria
- Ecchymoses and hematomas
- Neurological signs and symptoms
- Hemarthrosis (bleeding into the joint)
What is the collaboartive care for hemophilia A?
- Prevent and treat bleeding VIA replacement therapy
- Treatment of complications of the disease and its treatment
What is the nursing management of hemophilia A?
Assessment of clinical manifestations, determine blood levels of factor VIII, and administration of factor VIII. Assess for allergic reactions to AHF. During acute bleeding:
- Direct pressure, ice, packing (gelfoam, fibrin foam)
- Administer factor VIII intravenously
- Immoblize joint and give analgesics
- Manage life-threatening problems from hemorrhage (airway, intracranial bleeding)
What are the causes of anemia?
- Decreased RBC Production
- Blood Loss
- Increased RBC destruction
What is the alteration/etiology for anemia caused by decreased erythrocyte production?
Ingredients for bone marrow recipe for RBCs are lacking.
- Fe
- Folic acid
- Cobalamin B12
- Erythropoietin
OR the bone marrow is not working
What are examples of situations that would cause anemia related to decreased RBC production?
- Fe deficiency
- Folic acid deficiency
- Cobalamin deficiency
- Pernicious anemia
- Aplastic anemia
- Anemia of chronic disease
What are the alteration/etiology for anemia caused by blood loss?
- Acute blood loss
- Chronic blood loss
What are examples of situations that would cause anemia caused by blood loss?
Acute: trauma, gastric ulcer
Chronic: menstrual flow, hemorrhoids
What are the alteration/etiology of anemia caused by increased erythrocyte destruction?
- Two types: Intrinsic hemolytic anemia or extrinsic hemolytic anemia
- When RBCs are hemolyzed, causes jaundice and hyperkalemia
What are examples that could cause anemia caused by increased erythrocyte destruction?
Intrinsic: sickle cell disease
Extrinsic: prosthetic heart valves, extracorporeal circulation, dialysis
What are risk factors for iron deficiency anemia?
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.
What happens to the Hct when a person has rapid blood loss?
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.
What are the symptoms of anemia related to chronic blood loss?
- may or may not be symptomatic
- Patient adapts and may not complain of acute symptoms
- A careful history may reveal activity intolerance and dyspnea on exertion which has come on slowly
What happens to the building blocks of RBCs when cells hemolyze?
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
What are the top two nursing diagnoses for anemia?
Fatigue
Altered Nutrition: less than body requirements
What routine assessments should be done for a hemophiliac?
- Presence/continution of bleeding
- Amount of blood loss
- VS
- Labs: hbg, hct, PTT, hematuria
- Pain
- Need for analgesia (no ASA)
What activities are safe for a hemophiliac?
Quiet activities that keep injured part immoblized until the bleeding stops.
What precautions should be instituted for a child in the hosptial for bleeding that has hemophilia A?
- 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
What kind of pain is there in sickle cell disease?
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
What is the issue with infection regarding sickle cell patients?
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.
What is chest syndrome in sickle cell patients?
Pneumonia is most common. Symptoms of cough or difficulty breathing should be assessed quickly.
What neurological issue are sickle cell patients more at risk for?
stroke. blocked blood supply causes weakness and damage to brain
