Age of first pain crisis and associated complications in the CASiRe international sickle cell disease cohort

Immacolata Tartaglione, Crawford Strunk, Charles Antwi-Boasiako, Biree Andemariam, Raffaella Colombatti, Eugenia Vicky Asare, Connie M. Piccone, Deepa Manwani, Donna Boruchov, Fitz Tavernier, Fatimah Farooq, Sophia Akatue, Bianca Oteng, Rebekah Urbonya, Samuel Wilson, Ahmed Owda, Rose Bamfo, Gifty Dankwah Boatemaa, Sudha Rao, William ZempskyFredericka Sey, Baba PD Inusa, Silverio Perrotta, Catherine Segbefia, Andrew D. Campbell

Research output: Contribution to journalArticlepeer-review

2 Scopus citations

Abstract

Pain is a hallmark of Sickle Cell Disease (SCD) affecting patients throughout their life; the first pain crisis may occur at any age and is often the first presentation of the disease. Universal newborn screening identifies children with SCD at birth, significantly improving morbidity and mortality. Without early screening, diagnosis is generally made after disease manifestations appear. The Consortium for the Advancement of Sickle Cell Research (CASiRe) is an international collaborative group evaluating the clinical severity of subjects with SCD using a validated questionnaire and medical chart review, standardized across 4 countries (United States, United Kingdom, Italy and Ghana). We investigated the age of first pain crisis in 555 sickle cell subjects, 344 adults and 211 children. Median age of the first crisis in the whole group was 4 years old, 5 years old among adults and 2 years old among children. Patients from the United States generally reported the first crisis earlier than Ghanaians. Experiencing the first pain crisis early in life correlated with the genotype and disease severity. Early recognition of the first pain crisis could be useful to guide counseling and management of the disease.

Original languageEnglish (US)
Article number102531
JournalBlood Cells, Molecules, and Diseases
Volume88
DOIs
StatePublished - May 2021
Externally publishedYes

Keywords

  • Complications
  • Newborn screening
  • Pain crisis
  • Sickle cell disease

ASJC Scopus subject areas

  • Molecular Medicine
  • Molecular Biology
  • Hematology
  • Cell Biology

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