Home Management of Pain in Sickle Cell Disease: A Daily Diary Study in Children and Adolescents

Dampier, Carlton; Ely, Elizabeth; Brodecki, Darcy; O'Neal, Patricia

doi:10.1097/00043426-200211000-00008

Cited by 120 publications

(112 citation statements)

References 16 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…A similar study documenting the daily pain of older children with SCD found that while 17 % of patients required admission for pain during the 6-month study period, 40 % experienced at least one episode of pain each month with most episodes lasting one day (Dampier et al 2004 ). A striking range in the days of reported pain was also shown by this study group; a few children reported no days of pain versus a few who recorded pain on >90 % of days (Dampier et al 2002b ). These pain diary studies estimate that while young children (age < ~6 years) with SCD rarely experience pain (Dampier et al 2014 ), older children and adolescents (age ~6-19 years) with SCD have pain more frequently, on one out of every six days (Dampier et al 2002a ).…”

Section: Pain Crisissupporting

confidence: 64%

Clinical Manifestations of Sickle Cell Anemia: Infants and Children

Nickel

Hsu

2016

Sickle Cell Anemia

View full text Add to dashboard Cite

Children with sickle cell disease (SCD) have varied clinical problems. The hallmark manifestation of SCD, the pain crisis, typically fi rst occurs in early childhood presenting as dactylitis. Pain increases in frequency and severity as children age, especially during adolescence. Due to functional asplenia, children with SCD are at signifi cantly increased risk for certain infections, most notably Streptococcus pneumoniae. Other infections like parvovirus B19 are also special threats and can trigger an aplastic crisis. Unique, potentially life-threatening acute complications like splenic sequestration and acute chest syndrome occur in these children. They are at risk for neurologic disease, the most serious being stroke. In addition, the chronic hemolysis of SCD causes gallstones, which can lead to biliary tract disease. Children with SCD also frequently face chronic issues that include nocturnal enuresis and decreased growth. Despite these many potential problems, with advances in care including antibiotic treatment, stroke screening, blood transfusions, and hydroxyurea therapy, children with SCD rarely die in childhood and can become productive adults.

show abstract

Section: Pain Crisissupporting

confidence: 64%

Clinical Manifestations of Sickle Cell Anemia: Infants and Children

Nickel

Hsu

2016

Sickle Cell Anemia

View full text Add to dashboard Cite

show abstract

“…According to the Pain In Sickle Cell Epidemiology Study adult patients reported SCD pain at home on ϳ 55% of the 31 017 days, 57 whereas children reported SCD pain at home on ϳ 9% of the1515 days surveyed. 58,59 In MSH, at-home analgesics were used for SCD pain on 40% of diary days and 80% of 2-week follow-up periods, with short-acting oxycodone and acetaminophen being the most frequently used analgesics. 21,60 Descriptors and location of pain at home were similar to those during hospitalization but milder.…”

Section: Consequences Of the Acute Painful Crisismentioning

confidence: 99%

Sickle cell pain: a critical reappraisal

Ballas

Gupta

Adams-Graves

2012

Blood

358

427

View full text Add to dashboard Cite

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 ...

show abstract

“…3 Dampier et al studied children and adolescents (ages 6 -21 years) with SCD for a total of 18,377 days, and found less frequent, but still surprisingly common pain; those studied reported 514 distinct SCD pain episodes occurring over 2592 days and 2326 nights. 4 In a related report, children and adolescents reported SCD pain alone 8.4% of the time (1515 d), whereas other pain occurred 2.7% of the time (490 d), and both SCD and other pain 5.7% of the time (1041 d). 5 These results show that acute pain is definitely the hallmark of SCD, but that chronic pain occurs far more often than was previously realized.…”

mentioning

confidence: 95%

Sickle-Cell Pain: Advances in Epidemiology and Etiology

Smith

Scherer

2010

Hematology

View full text Add to dashboard Cite

New epidemiological findings recast pain in sickle-cell disease (SCD) as being more often a chronic manifestation than was previously thought, although acute pain is still the hallmark of the disease. SCD pain intensity, the number of painful locations, and the frequency of hospitalizations due to SCD pain may worsen with age. In adults and even in children, the quantity and severity of SCD pain may be vastly underestimated, because most of the "iceberg" of SCD pain is "submerged" at home, and only the tip of the iceberg is seen by health care providers when acute SCD care is rendered in emergency rooms and hospitals. Implications of this "iceberg phenomenon" are significant for clinicians, researchers, employers, policy makers, and the public. Nevertheless, both emergency room and hospital utilization for SCD pain remain prevalent. Often, utilization recurs early, perhaps emblematic of poor acute pain management. New data show the protean impacts of SCD pain on health-related quality of life, sleep, and psychological and social health. The relationship of the severity of SCD pain to the severity of underlying sickle vasculopathy is unclear, but epidemiologic evidence and patient descriptors suggest a temporal evolution of pain mechanisms. At first, increasingly worse nociceptive pain from vaso-occlusion and local lesions may evolve over the first two decades of life. Then, in the third and following decades, central neuropathic pain may also evolve due to past and continuing nociceptive stimuli. New findings confirm environmental contributors to SCD pain, including seasonal (colder) temperatures, barometric pressure, and wind speed.

show abstract

Home Management of Pain in Sickle Cell Disease: A Daily Diary Study in Children and Adolescents

Cited by 120 publications

References 16 publications

Clinical Manifestations of Sickle Cell Anemia: Infants and Children

Clinical Manifestations of Sickle Cell Anemia: Infants and Children

Sickle cell pain: a critical reappraisal

Sickle-Cell Pain: Advances in Epidemiology and Etiology

Contact Info

Product

Resources

About