A Chronology of Pain and Comfort in Children With Sickle Cell Disease

Beyer, Judith; Simmons, Larry E.; Woods, Gerald M.; Woods, Patrice M.

doi:10.1001/archpedi.153.9.913

Cited by 35 publications

(27 citation statements)

References 24 publications

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“…In producing this biphasic pain response, this model may be particularly relevant to the peak and resolving phases of painful vaso-occlusive episodes in children with sickle cell disease. 5,8,37 Although significant progress has been made in understanding the cellular and humeral mechanisms of sickle cell disease vaso-occlusion, 4 a gap remains in understanding how vaso-occlusion actually produces debilitating pain. Although ischemia and the resulting tissue damage are clearly mediating factors, 4 the endothelin family is likely to be one transducer of vaso-occlusion into pain.…”

Section: Discussionmentioning

confidence: 99%

Age- and Sex-Specific Nociceptive Response to Endothelin-1

McKelvy

Mark

Sweitzer

2007

The Journal of Pain

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Section: Discussionmentioning

confidence: 99%

Age- and Sex-Specific Nociceptive Response to Endothelin-1

McKelvy

Mark

Sweitzer

2007

The Journal of Pain

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“…However, the significantly lower pain scores on the day of discharge may be partially attributed to the natural progression of the vaso-occlusive episode as the hemolytic and inflammatory processes resolved, rather than the overall relief obtained from analgesic medications. 7,[31][32][33][34] Future studies need to determine whether the administration of therapeutic doses of analgesics would improve pain management and lead to a shorter duration of the vaso-occlusive episode and shorter lengths of stay. Our study was not able to determine whether unrelieved pain is related to inadequate administration of analgesics or to an inherent difference in the pathophysiology of vaso-occlusive pain that is refractory to the currently used analgesics.…”

Section: Discussionmentioning

confidence: 99%

Quantification of Analgesic Use in Children With Sickle Cell Disease

Jacob¹,

Miaskowski

Savedra

et al. 2007

The Clinical Journal of Pain

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“…The phases were called prodromal, initial, established, and resolving phases ( Figure 1). Beyer et al 50 and Jacob et al 51 reported the evolution of painful crises along similar phases in children.…”

Section: The Acute Sickle Cell Painful Crisismentioning

confidence: 94%

Sickle cell pain: a critical reappraisal

Ballas

Gupta

Adams-Graves

2012

Blood

369

427

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Sickle cell pain includes 3 types: acute recurrent painful crises, chronic pain syndromes, and neuropathic pain. The acute painful crisis is the hallmark of the disease and the most common cause of hospitalization and treatment in the emergency department. It evolves through 4 phases: prodromal, initial, established, and resolving. Each acute painful episode is associated with inflammation that worsens with recurrent episodes, often culminating in serious complications and organ damage, such as acute chest syndrome, multiorgan failure, and sudden death. Three pathophysiologic events operate in unison during the prodromal phase of the crisis: vaso-occlusion, inflammation, and nociception. Aborting the acute painful episode at the prodromal phase could potentially prevent or minimize tissue damage. Our hypothesis is that managing these events with hydration, anti-inflammatory drugs, aggressive analgesia, and possibly vasodilators could abort the crisis and prevent or minimize further damage. Chronic pain syndromes are associated with or accompany avascular necrosis and leg ulcers. Neuropathic pain is not well studied in patients with sickle cell disease but has been modeled in the transgenic sickle mouse. Management of sickle cell pain should be based on its own pathophysiologic mechanisms rather than borrowing guidelines from other nonsickle pain syndromes. (Blood. 2012;120(18): 3647-3656) IntroductionSickle cell disease (SCD) has been primarily considered a disease of children with a few patients surviving to adulthood. Between 1910 and 1950, the median survival of patients with SCD was Ͻ 20 years of age. 1 Sudden death occurred in ϳ 41% of the patients, whereas 29% deaths occurred within 24 hours after the onset of painful crises. 2 By 1980, 50% of children survived to 20 years of age; and by 2009, survival to age 20 years increased to 85% of children. 2 However, if we rewind the clock by more than a century when SCD was first described in the United States, we find that the first 4 SCD patients reported were adults with leg ulcers. [3][4][5][6] Before 1910, there were observations among adult African slaves suggestive of SCD with immunity to malaria and high prevalence of leg ulcers. 7 Moreover, autopsy on a slave with history of fever and respiratory illness in 1846 showed absence of the spleen. 8 The inevitable question then is: where were the children with SCD in the United States before 1910? There must have been children with SCD. The patient reported by Cook and Meyer 5 gave family history of brothers and sisters who died in early life of a disease associated with grave anemia. These children must have lived, suffered, and died prematurely, the dark side of the discovery of sickle cell anemia in the United States. 9 It was not until the early 1930s that children with SCD were recognized. Sickle cell pain between 1930 and 1960Between 1930 and 1960, the emphasis was on studying the basic science of SCD. This period witnessed quantum leaps in defining the specific molecular signature of SCD with the advent ...

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A Chronology of Pain and Comfort in Children With Sickle Cell Disease

Cited by 35 publications

References 24 publications

Age- and Sex-Specific Nociceptive Response to Endothelin-1

Age- and Sex-Specific Nociceptive Response to Endothelin-1

Quantification of Analgesic Use in Children With Sickle Cell Disease

Sickle cell pain: a critical reappraisal

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