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(solved) Sickle Cell Disease Experiencing Vaso-Occlusive Crisis

(solved) Sickle Cell Disease Experiencing Vaso-Occlusive Crisis

Sickle Cell Disease Experiencing Vaso-Occlusive Crisis


Sickle cell disease (SCD) is the most common inherited hemoglobinopathy in the black population. Vaso-occlusion, chronic hemolysis, and increased erythrocyte adhesiveness to vascular endothelium are pathologic hallmarks of the disease. Ischemic pain caused by vaso-occlusion is a significant clinical feature that manifests as varying degrees of severity, episodic bone pain, or abdominal pain. This episodic event is known as a vaso-occlusive crisis. It is the most common type of crisis in the disease, with anemic crisis comprising hyper-hemolytic, sequestration, and aplastic crises.

The significant morbidity in SCD is a vaso-occlusive crisis or acute painful crisis. Fever appears to be expected even in seemingly uncomplicated painful crises, implying that the symptom is a feature of the crisis rather than a sign of infection. SCD is thought to be a tetrad of pain syndromes, anemia and its sequelae, organ failure (including infection), and comorbid conditions, with pain dominating the clinical picture and occurring either spontaneously or as a result of the other three components of the tetrad. Although the primary therapeutic approach to this crisis is pharmacologic, pain management during each episode has expanded beyond focusing solely on the physical dimension of pain to include psychological, social, and behavioral components. A multifaceted approach to pain management is thus critical to patients’ overall care. Pain research, for example, has shown that cognitive behavioral therapy in SCD is an effective adjunct in treating chronic pain because it reduces psychological distress and boosts the patient’s confidence and resilience. Given the well-established influence of psychological, sociocultural, and spiritual factors on pain perception, a vaso-occlusive crisis may have an impact on the patient in the six fundamental domains of health-related quality of life, which are physical functioning, psychological functioning, social functioning, role activities, overall life satisfaction, and perception of health status.


Nonetheless, according to some recent recommendations, a vaso-occlusive crisis requires immediate and adequate analgesia commensurate with the level of patient-reported pain. Although the pain of vaso-occlusive episodes is nociceptive, quantitative sensory testing has revealed neuropathic pain in SCD patients aged seven years and older (QST). Furthermore, an age-dependent increase in SCD patients with chronic pain has been reported, attributed to chronic inflammation, organ damage, and opioid-induced hyperalgesia. Thus, despite the use of opioids as the primary analgesic therapy, low-dose ketamine (a modulator of opioid tolerance and opioid-induced hyperalgesia) has been recommended for clinical use. Furthermore, despite renewed interest in this treatment modality, evidence for the efficacy of psychological therapies in SCD-associated pain appears to be limited.

Sickle Cell Disease Experiencing Vaso-Occlusive Crisis


Sickle Cell Disease Experiencing Vaso-Occlusive Crisis

Q. 4. A patient with Sickle Cell Disease experiencing Vaso-Occlusive Crisis comes to the ER for evaluation. Which of the following are acute manifestations of this disease that the nurse should expect to see in this patient?

a) Acute leg pain and dactylitis

b) Anaemia and hypotension

c) Tachycardia and jaundice

d) Enuresis and proteinuria

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