Abstract
The majority of hospitalizations of patients with sickle cell disease (SCD) are related to painful vaso-occlusive crises (VOCs). Although the pain of VOC is classically nociceptive, neuropathic pain (NP) has also been demonstrated in SCD patients. The aim of our study is to specify the prevalence of NP during VOCs in SCD children using a dedicated scale and to measure its characteristics. We performed a prospective study that included SCD children hospitalized for an acute VOC. The presence of NP was sought with the DN4 scale on the second and fourth days of hospitalization. A total of 54 SCD children were included in the study. Overall, 41% of the patients (n = 22) experienced neuropathic pain during the VOC, mostly at an early stage (Day 2). The median age, the sex ratio, the location of the pain, and the morphine consumption were similar for patients with and without NP. Our study shows that neuropathic pain is very common during VOCs in SCD children. The absence of identified risk factors should prompt us to be vigilant regardless of the patient’s age, sex, and clinical presentation.
Highlights
Sickle cell disease (SCD) is a genetic hemoglobinopathy resulting from a unique mutation in the β-globin gene characterized by hemolytic anemia, painful vaso-occlusive crisis (VOC), and progressive organ failure
The study was proposed to every sickle cell disease (SCD) patient that was aged 6 to 18 years old and hospitalized for an acute VOC
The diagnosis of neuropathic pain during VOC raises the problem of fine descriptions of the characteristics of the pain, which are sometimes difficult in young children, especially during an intensely painful episode where many sensations are mixed, including anxiety, fear of parental separation, and adverse effects of treatment
Summary
Sickle cell disease (SCD) is a genetic hemoglobinopathy resulting from a unique mutation in the β-globin gene characterized by hemolytic anemia, painful vaso-occlusive crisis (VOC), and progressive organ failure. The pain of vaso-occlusive episodes is classically nociceptive, neuropathic pain (NP) has been demonstrated in SCD patients, both in adults [8] and in children aged 7 years and above, by using quantitative sensory testing (QST) [9]. Neuropathic pain in SCD has essentially been reported as chronic pain, described as numb, tingling, lancinating, spontaneous, shooting, or paroxysmal pain, and is sometimes associated with a sensation of pins and needles, hyperalgesia, and allodynia [12] These pains are typically resistant to classical anti-nociceptive treatments, and can be responsible for a high consumption of opioids, which underlines the importance of their specific diagnosis. The aim of our study is to evaluate the prevalence of NP during VOC in SCD children using the DN4 scale and to specify its risk factors and characteristics in order to better understand and manage them
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