Types of Sickle Cell Crisis
Overview
Sickle cell anemia is an inherited disorder on the beta chain of the hemoglobin resulting to abnormally shaped red blood cells. RBC’s assume a crescent or C-shape that decreases the cell’s life span and ability to function thereby, producing various complications.
In some cases, these sickled cells may block the flow causing pain and organ damage. A severe attack, known as sickle cell crisis, can cause pain because blood vessels can become blocked or the defective red blood cells can damage organs in the body. Most episodes of sickle cell crises last between five and seven days
Types of Sickle Cell Crisis
A. Vaso-occlusive Crisis – This type of sickle cell crisis is due to the presence of sickle shaped red blood cells that impede capillaries and restrict the blood flow to an organ. This results in ischemia, severe pain, necrosis (tissue death) and most of the time it damages an organ. The frequency, severity, and duration of these crises vary considerably.
Treatment: Pain crises are managed and treated by the following measures.
- Hydration
- Analgesics (Opioid administration at regular intervals until the crisis has settled). For milder crises, NSAIDs such as Diclofenac or Naproxen could be used. For severe crises, intravenous opioids are given such as patient-controlled analgesia (PCA).
- Blood transfusion
- Dipenhydramine. This medication is used for the management of itching associated with the opioid use.
- To minimize the development of atelectasis a technique that encourages deep breathing, incentive spirometry, is recommended.
B. Splenic Sequestration Crisis – This crisis is acute and painful enlargements of the spleen. The spleen is responsible for clearing defective red blood cells. However, because of its narrow vessels, defective RBC’s pools down the spleen that leads to its infarction before the end of childhood in individuals suffering from sickle-cell anemia. During a splenic sequestration crisis the abdomen becomes bloated and very hard. Aside from that, the sinusoids and gates would open at the same time that result to the sudden pooling of the blood into the spleen which leads to a circulatory defects, thus, leading to sudden hypovolemia.
Treatment
Splenic sequestration crisis is considered an emergency condition and abrupt and prompt treatment is required or the patient may die within 1-2 hours because of the circulatory failure.
- Blood transfusion
- Management is supportive
C. Aplastic Crisis – This crisis stimulated by parvovirus B19. This virus directly affects the production of RBCs through the invasion of the red cell precursors. The resulting condition is the multiplication of the virus and the destruction of the RBC’s. The crisis is characterized by acute worsening of the patient’s baseline anemia which produces pallor, tachycardia and fatigue.
Treatment
- Blood transfusion
- Supportive management
D. Hemolytic Crisis – When hemoglobin levels drop, hemolytic crisis is accelerated. Resulting to the speeding up of RBC destruction. Individuals with co-existent G6PD deficiency commonly suffers from this crisis.
Treatment:
- Blood transfusion
- Supportive management
Daisy Jane Antipuesto RN MN
Currently a Nursing Local Board Examination Reviewer. Subjects handled are Pediatric, Obstetric and Psychiatric Nursing. Previous work experiences include: Clinical instructor/lecturer, clinical coordinator (Level II), caregiver instructor/lecturer, NC2 examination reviewer and staff/clinic nurse. Areas of specialization: Emergency room, Orthopedic Ward and Delivery Room. Also an IELTS passer.
