Anemia: Low Rbc and Low Hgb

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Anemia: Low RBC and/or low HgB

Causes:
Hypoproliferative (not producing enough): Deficiencies, Cancer. Hemolytic (destruction of): Disease, hyperslenism, mech. Heart valves. Blood loss.

S/S: Fatigue, weakness, malaise, pallor, jaundice, cardiac and respiratory symptoms, tongue/nail changes, pica, Nail changes, angular cheilosis (cracks at corners of mouth)
PT w/ hypothytoid may be asymptomatic. PT w/ CV or Pulmonary disease may have severe symptoms.
Elderly: More pronounced. Dementia, cognitive, fragility, falls, mobility.

Complications: HF, paresthesias, delirium, angina

TX: Correct cause, PRBC, Diet, supplementation, immunosuppressive, BMT (bone marrow), PBSCT (stem cells)

NI: PMH, exam, Labs, symptoms, nutrition, meds, CV+GI+Neuro assesments, blood loses (menses/GI), manage fatigue, tissue
perfusion, compliance with TX, balance activity/rest, monitor VS, O2, education,

Sickle Cell Disease: Increased HgB S, autosomal recessive.

Trait: One defective gene. Carrier. Rarely has symptoms
Disease: Both defective genes. Has crisis. Low Hct, Low HgB Syndrome: Cell has a C shape instead of S shape. Most common type in AA population. Same problems as Disease. Sickle cell/Beta thalassemia: Mediterranean.

Crisis in SCD is triggered by: hypoxia, temperature extremes, excessive exercise, anesthesia, dehydration, fever, hi altitude, vomiting, pregnancy, EtOH, acidosis, elevated HgB, elevated Reticulocytes.

DX: HgB electrophoresis. Sickle-turbidity test (SickleDex) =>6mths of age. Reticulocyte counts measured regularly

S/S: Acute and chronic manifestations, Pallor, fatigue, jaundice, irritability,Pain,Shortened RBC lifespan, compromised erythropoiesis can lead to profound aplastic anemia

Complications: Splenomegaly, Acute chest syndrome @ 2-4 years of age( infection, infarction, PE, Fat embolism), CVA, Hematuria,
dehydration, Tachycardia, HF, Bone infarction, jaundice, gallstone, hepatomegaly, skin ulcers, dec wound healing, eye
scaring, eye hemorrhage, retina detachment, impotence.
TX: Primarily supportive. HOLP (Heat, O2, Liquids, Pain). Pneumococcal vaccine recommended.
Hydroxyurea: Increases Fetal HgB. Shown to reduce number of crisis’. S/E: dec leukocytes, teratogensesis, malignancy.
PBSCT: Contra in pts. w/ organ damage
PRBC: S/E iron overload, alloimmunization, increased blood visc..
Daily folic acid
Aggressive infection treatments, particularly pneumococcal.
Splenectomy
Genetic counseling
NI: : PMH, exam, Labs, symptoms, nutrition, meds, CV+GI+Neuro assesments, blood loses (menses/GI), manage fatigue, tissue
perfusion, compliance with TX, balance activity/rest, monitor VS, O2, education,

NI: Pain relief, monitor for infections, coping skills, education, monitor for complications (ulcers, priapism leading to impotence, substance abuse, promoting self care.

Delayed growth: Hi protein/calorie diet. Folic acid/vit c. Monitor growth. Fluid maintenance requirements (100mL/Kg for 1st 10, then 50mL/Kg next 10kg, then 20mL/Kg) Normal WBC per book (4500-11000)

HBSS PAIN HRSS
Hemolysis, Bone marrow, Stroke, Skin ulcer, Pain, Anemia, Infections, Nocturia, HF, Renal, Sequestration, Sepsis 1 The nurse is teaching a group of parents whose children have sickle cell anemia. When a parent asks the cause of the symptoms, the nurse responds with which of the following? “The sickled cells mix with normal cells, which causes the immune system to be depressed.” “Sickled cells increase the blood flow through the body, which causes pain.” “Sickled cells clump in the smaller blood vessels and obstruct blood flow.” “Sickled cells cause bone marrow depression.”

2 A child with suspected sickle cell disease (SCD) is in the clinic for laboratory studies. The parents ask the nurse what results will tell the physician that their child has SCD. The nurse responds that which of the following is increased...
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