[Hematology] sickle cell anemia/ sickle cell disease

Sickle cell disease is a hereditary blood disorder characterized by RBCs that become sickle-shaped, rather than oval, when deoxygenated, dehydration, stress(eg. infection), and hypoxia cause sickled RBCs to clump together and obstruct blood flow, causing episodes of acute pain.

Thick and sticky blood.

Severe pain due to impaired capillary blood flow (ie. vasoocclusion) and tissue ischemia. -> may lead irreversible tissue damage (eg. myocardial infarction, limb necrosis, stroke) and death.

 

S+S

Tachycardia, due to hypoxemia, pain, and infection

 Leukocytosis: occurs with inflammation of tissues injured by ischemia

Anemia: RBC destrucion (monitor Hb level and administering blood products as indicated to ensure tissue perfusion)

Increased reticulocyte count: indicative of RBC destruction

Severe bone pain and tendor spots, which can be caused by ischemia, infection, and fractures, all of which are comlications of SCD.

Dehydration (leading amber-colored, concentrated urine)

 

NRS Action

i) Diet: high protein, high-calorie diet in addition to a multivitamin without iron.

ii) Folic acid: Stimulation of erythropoiesis, which is needed due to increased hemolysis.

iii) hydration: aggressive IV and oral hydration to reduce blood viscosity.

iv) infection prevention: vacchination

v) O2

vi) pain control: analgesics scheduled around the clock or continually to prevent breakthrough pain. 

vii)Tissue perfusion: monitor hemoglobin levels and administer blood products as indicated

 

 

 

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5살 아기. Acute pain, SOB(Shortness of breath), neasua, vomitting, generalized body and joint pain. 무엇이 가장 급할까?

(1) enlarged spleen on palpation

(2) hamoglobin level of 9.0

(3) pain rated as 8 on the Wong-Baker FACES Scale

(4) bilateral swelling of the hands and feet

 

*Sickle cell disease 환자

• Acute painful episodes 겪음. : vasoocclusive crisis when sickle-shaped cells block blood flow through the vessels. 
• Tend to have a small spleen due to repeated small splenic infarctions.
  • Acute splenic sequestration crisis is a complication of painful episodes that occurs when a large number of sickle-shaped cells get trapped in the spleen, causing splenomegaly.
  • This is a life-threatening emergency b/c large quantitie of pooling blood in the spleen can lead to severe hypovolemia and shock. The classic assessment finding is a rapidly enlarging spleen and hypotension.
• Vasoocclusive crisis is very painful. The nurse should evaluate the client's pain level using an age-appropriate scale (Wong-Baker FACES pain rating scale for a child) and advocate for effective analgesia, however, the priority is to report signs of splenic sequentration crisis.)
• Swelling of the hands and feet: symptom of sickle cell disease, occurs due to this, blocking blood flow to the hands and feet. This is often detected in infants/children as the first sign of the disease.

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Avoid constrictive clothing and devices (eg, tight trousers, frequent use of blood pressure cuffs), which can worsen vasoocclusion. Clients should wear loose-fitting clothes, maintain neutral positioning (eg. head of the bed at <30 degrees) and avoid flexing the extremities.

Avoid cold compresses, which constrict the blood vessels, worsen the pain, and may result in tissue injury.

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SCD -> pain -> opioid analgesia -> suppresses bowel motility and fluid secretion in the intestina lumen and results in constipation. : stool softeners or laxative (eg, docusate sodium), ambulation, drink adequate fluids.

+related problems (eg, abdominal discomfort, nausea, stool impaction, hemorrhoids, paralytic ileus if left untreated)

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Opioid agonists (eg. oxycodone) cause

(1) miosis (pupillary constriction)

(2) reduced bowel motility and fluid secretions in the intestinal lumen, resulting in constipation.