Skip to main content
SpringerLink
Log in

Sickle Cell Pain

  • Chapter
  • First Online:
Handbook of Pediatric Chronic Pain

Abstract

The preceding excerpt is from the preface to a tome that exemplifies integrative medicine, written by the preeminent hematologist and sickle cell researcher, Samir K. Ballas, M.D. Dr. Ballas was one of the first physicians to publish ­objective information on the management problems for patients, healthcare providers, and loved ones dealing with sickle cell pain. By unabashedly using awkward, embarrassing terms such as prejudice, stereotyping, mistreatment and neglect to describe the problems encountered by the older sickle cell patient, Dr. Ballas helped shepherd in the much-needed, multi-disciplinary team approach in managing sickle cell pain and sickle cell disease (Epstein et al. 2006; Ballas 2005; Ballas 1995; Ballas 1990). This chapter is dedicated to Dr. Ballas who has devoted his professional life to sickle cell patients while sharing his time, generosity, and wisdom with any medical specialty interested in studying any aspect of sickle cell disease.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 129.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 169.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  • Amrolia, P. J., Almeida, A., Davies, S. C., & Roberts, I. A. (2003a). Therapeutic challenges in childhood sickle cell disease. Part 2: a problem-orientated approach. British Journal Haematology, 120, 737–743.

    Article  Google Scholar 

  • Amrolia, P. J., Almeida, A., Halsey, C., Roberts, I. A., & Davies, S. C. (2003b). Therapeutic challenges in childhood sickle cell disease. Part 1: current and future treatment options. British Journal Haematology, 120, 725–736.

    Article  Google Scholar 

  • Ballas, S. K. (1990). Treatment of pain in adults with sickle cell disease. American Journal of Hematology, 34, 49–54.

    Article  PubMed  CAS  Google Scholar 

  • Ballas, S. K. (1995). The sickle cell painful crisis in adults: phases and objective signs. Hemoglobin, 19, 323–333.

    Article  PubMed  CAS  Google Scholar 

  • Ballas, S. K. (1997). Management of sickle pain. Current Opinion in Hematology, 4, 104–111.

    Article  PubMed  CAS  Google Scholar 

  • Ballas, S. K. (1998). Sickle cell pain (p. ix). Seattle: International Association for the Study of Pain Press.

    Google Scholar 

  • Ballas, S. K. (2005). Pain management of sickle cell disease. Hematology/Oncology Clinics of North America, 19, 785–802.

    Article  PubMed  Google Scholar 

  • Ballas, S. K. (2008). Meperidine for acute sickle cell pain in the emergency department: revisited controversy. Annals of Emergency Medicine, 51, 217.

    Article  PubMed  Google Scholar 

  • Ballas, S. K., & Marcolina, M. J. (2006). Hyperhemolysis during the evolution of uncomplicated acute painful episodes in patients with sickle cell anemia. Transfusion, 46, 105–110.

    Article  PubMed  Google Scholar 

  • Ballas, S. K., & Mohandas, N. (1996). Pathophysiology of vaso-occlusion. Hematology/Oncology Clinics of North America, 10, 1221–1239.

    Article  PubMed  CAS  Google Scholar 

  • Ballas, S. K., Cai, S. P., Gabuzda, T., & Chehab, F. F. (1997). Molecular basis of asymptomatic beta-­thalassemia major in an African American individual. American Journal of Medical Genetics, 69, 196–199.

    Article  PubMed  CAS  Google Scholar 

  • Ballas, S. K., Barton, F. B., Waclawiw, M. A., Swerdlow, P., Eckman, J. R., Pegelow, C. H., Koshy, M., Barton, B. A., & Bonds, D. R. (2006). Hydroxyurea and sickle cell anemia: effect on quality of life. Health and Quality of Life Outcomes, 4, 59.

    Article  PubMed  Google Scholar 

  • Barakat, L. P., Patterson, C. A., Weinberger, B. S., Simon, K., Gonzalez, E. R., & Dampier, C. (2007). A prospective study of the role of coping and family functioning in health outcomes for adolescents with sickle cell ­disease. Journal of Pediatric Hematology/Oncology, 29, 752–760.

    Article  PubMed  Google Scholar 

  • Bardakdjian-Michau, J. (2003). Neonatal detection of sickle cell disease. Journal de Gynecologie, Obstetrique et Biologie de la Reproduction (Paris), 32(1), S61–S64.

    Google Scholar 

  • Beuzard, Y. (1996). Towards gene therapy of hemoglobinopathies. Seminars in Hematology, 33, 43–52.

    PubMed  CAS  Google Scholar 

  • Blaylock, B. (1996). Sickle cell leg ulcers. Medsurg Nursing, 5, 41–43.

    PubMed  CAS  Google Scholar 

  • Brousseau, D. C., McCarver, D. G., Drendel, A. L., Divakaran, K., & Panepinto, J. A. (2007). The effect of CYP2D6 polymorphisms on the response to pain ­treatment for pediatric sickle cell pain crisis. Jornal de Pediatria, 150, 623–626.

    CAS  Google Scholar 

  • Carvalho, K. S., & Garg, B. P. (2002). Arterial strokes in children. Neurologic Clinics, 20, 1079–1100. vii.

    Article  PubMed  Google Scholar 

  • Charache, S. (1996). Treatment of sickling disorders. Current Opinion in Hematology, 3, 139–144.

    Article  PubMed  CAS  Google Scholar 

  • Chen, S. H., Liang, D. C., Lin, H. C., Cheng, S. Y., Chen, L. J., & Liu, H. C. (2005). Auditory and visual toxicity during deferoxamine therapy in transfusion-dependent patients. Journal of Pediatric Hematology/Oncology, 27, 651–653.

    Article  PubMed  Google Scholar 

  • Chiodo, A. A., Alberti, P. W., Sher, G. D., Francombe, W. H., & Tyler, B. (1997). Desferrioxamine ototoxicity in an adult transfusion-dependent population. The Journal of Otolaryngology, 26, 116–122.

    PubMed  CAS  Google Scholar 

  • Chulamokha, L., Scholand, S. J., Riggio, J. M., Ballas, S. K., Horn, D., & DeSimone, J. A. (2006). Bloodstream infections in hospitalized adults with sickle cell disease: a retrospective analysis. American Journal of Hematology, 81, 723–728.

    Article  PubMed  Google Scholar 

  • Cordner, S., & De, C. K. (2003). Musculoskeletal manifestations of hemoglobinopathies. Current Opinion in Rheumatology, 15, 44–47.

    Article  PubMed  Google Scholar 

  • De Franceschi, L., Saadane, N., Trudel, M., et al. (1994). Treatment with oral clotrimazole blocks Ca2 + ­-mediated K + transport and reverses erythrocyte dehydration in transgenic SAD mice: a model for therapy of sickle cell disease. The Journal of Clinical Investigation, 93, 1670–1676.

    Article  PubMed  Google Scholar 

  • Dickerhoff, R., & von, R. A. (1995). Pain crises in patients with sickle cell diseases. Pathogenesis, clinical aspects, therapy. Klinische Pädiatrie, 207, 321–325.

    Article  PubMed  CAS  Google Scholar 

  • Dimashkieh, H., Choe, J., & Mutema, G. (2003). Renal medullary carcinoma: a report of 2 cases and review of the literature. Archives of Pathology & Laboratory Medicine, 127, e135–e138.

    Google Scholar 

  • Dumaplin, C. A. (2006). Avoiding admission for afebrile pediatric sickle cell pain: pain management methods. Journal of Pediatric Health Care, 20, 115–122.

    Article  PubMed  Google Scholar 

  • Eccleston, C., Yorke, L., Morley, S., Williams, A. C., & Mastroyannopoulou, K. (2003). Psychological therapies for the management of chronic and recurrent pain in children and adolescents. Cochrane Database System Reviews, 99, 157–65. CD003968.

    Google Scholar 

  • Embury, S. H. (1986). The clinical pathophysiology of sickle cell disease. Annual Review of Medicine, 37, 361–376.

    Article  PubMed  CAS  Google Scholar 

  • Epstein, K., Yuen, E., Riggio, J. M., Ballas, S. K., & Moleski, S. M. (2006). Utilization of the office, hospital and emergency department for adult sickle cell patients: a five-year study. Journal of the National Medical Association, 98, 1109–1113.

    PubMed  Google Scholar 

  • Erhan, E., Inal, M. T., Aydinok, Y., Balkan, C., & Yegul, I. (2007). Tramadol infusion for the pain management in sickle cell disease: a case report. Paediatric Anaesthesia, 17, 84–86.

    Article  PubMed  Google Scholar 

  • Fosdal, M. B., & Wojner-Alexandrov, A. W. (2007). Events of hospitalization among children with sickle cell disease. Journal of Pediatric Nursing, 22, 342–346.

    Article  PubMed  Google Scholar 

  • Fung, E. B., Harmatz, P., Milet, M., Ballas, S. K., De, C. L., Hagar, W., Owen, W., Olivieri, N., Smith-Whitley, K., Darbari, D., Wang, W., & Vichinsky, E. (2007). Morbidity and mortality in chronically transfused subjects with thalassemia and sickle cell disease: a report from the multi-center study of iron overload. American Journal of Hematology, 82, 255–265.

    Article  PubMed  Google Scholar 

  • Gaziev, J., & Lucarelli, G. (2003). Stem cell transplantation for hemoglobinopathies. Current Opinion in Pediatrics, 15, 24–31.

    Article  PubMed  Google Scholar 

  • Halsey, C., & Roberts, I. A. (2003). The role of hydroxyurea in sickle cell disease. British Journal Haematology, 120, 177–186.

    Article  CAS  Google Scholar 

  • Hebbel, R. P. (1985). Auto-oxidation and a membrane-associated ‘Fenton reagent’: a possible explanation for development of membrane lesions in sickle erythrocytes. Clinics in Haematology, 14, 129–140.

    PubMed  CAS  Google Scholar 

  • Herrick, J. B. (1910). Peculiar elongated and sickle-shaped red blood corpuscles in a case of severe anemia. Archives of Internal Medicine, 6, 517–521.

    Article  Google Scholar 

  • Huang, J., Zou, Z., Kim-Shapiro, D. B., Ballas, S. K., & King, S. B. (2003). Hydroxyurea analogues as kinetic and mechanistic probes of the nitric oxide producing reactions of hydroxyurea and oxyhemoglobin. Journal of Medicinal Chemistry, 46, 3748–3753.

    Article  PubMed  CAS  Google Scholar 

  • Hungerford, D. S., & Lennox, D. W. (1985). The importance of increased intraosseous pressure in the development of osteonecrosis of the femoral head: implications for treatment. The Orthopedic Clinics of North America, 16, 635–654.

    PubMed  CAS  Google Scholar 

  • Jacob, E., Miaskowski, C., Savedra, M., Beyer, J. E., Treadwell, M., & Styles, L. (2006). Changes in sleep, food intake, and activity levels during acute painful episodes in children with sickle cell disease. Journal of Pediatric Nursing, 21, 23–34.

    Article  PubMed  Google Scholar 

  • Kaul, D. K., Fabry, M. E., & Nagel, R. L. (1996). The pathophysiology of vascular obstruction in the sickle syndromes. Blood Reviews, 10, 29–44.

    Article  PubMed  CAS  Google Scholar 

  • Kerle, K. K., & Nishimura, K. D. (1996). Exertional collapse and sudden death associated with sickle cell trait. American Family Physician, 54, 237–240.

    PubMed  CAS  Google Scholar 

  • Kooy, A., de Heide, L. J., ten Tije, A. J., Mulder, A. H., Tanghe, H. L., Kluytmans, J. A., & Michiels, J. J. (1996). Vertebral bone destruction in sickle cell disease: infection, infarction or both. The Netherlands Journal of Medicine, 48, 227–231.

    Article  PubMed  CAS  Google Scholar 

  • Labie, D., & Elion, J. (1996a). Sickle cell anemia: model of variability in expression of monogenic disease. Archives of Pediatrics, 3, 101–103.

    Article  CAS  Google Scholar 

  • Labie, D., & Elion, J. (1996b). Sequence polymorphisms of potential functional relevance in the beta-globin gene locus. Hemoglobin, 20, 85–101.

    Article  PubMed  CAS  Google Scholar 

  • Laird, L., Dezateux, C., & Anionwu, E. N. (1996). Neonatal screening for sickle cell disorders: what about the carrier infants? British Medical Journal, 313, 407–411.

    Article  PubMed  CAS  Google Scholar 

  • Lane, P. A. (1996). Sickle cell disease. Pediatric Clinics of North America, 43, 639–664.

    Article  PubMed  CAS  Google Scholar 

  • Lefevre-Witier, P. (1985). An anthropological perspective on the epidemiology of hemoglobin defects and ­glucose-6-phosphate dehydrogenase deficiencies in the northern half of the African continent. Progress in Clinical and Biological Research, 194, 367–397.

    PubMed  CAS  Google Scholar 

  • Mankad, V. N. (2001). Exciting new treatment approaches for pathyphysiologic mechanisms of sickle cell disease. Pediatric Pathology & Molecular Medicine, 20, 1–13.

    Article  CAS  Google Scholar 

  • Marchant, W. A., & Walker, I. (2003). Anaesthetic management of the child with sickle cell disease. Paediatric Anaesthesia, 13, 473–489.

    Article  PubMed  Google Scholar 

  • Mitchell, M. J., Lemanek, K., Palermo, T. M., Crosby, L. E., Nichols, A., & Powers, S. W. (2007). Parent perspectives on pain management, coping, and family functioning in pediatric sickle cell disease. Clinical Pediatrics (Philadelphia), 46, 311–319.

    Article  Google Scholar 

  • Molitierno, J. A., Jr. (2003). Carson CC, III: urologic manifestations of hematologic disease sickle cell, leukemia, and thromboembolic disease. The Urologic Clinics of North America, 30, 49–61.

    Article  PubMed  Google Scholar 

  • Moore, C. M., Ehlayel, M., Leiva, L. E., & Sorensen, R. U. (1996). New concepts in the immunology of sickle cell disease. Annals of Allergy, Asthma & Immunology, 76, 385–400.

    Article  CAS  Google Scholar 

  • Murray, M. J., & Evans, P. (1996). Sudden exertional death in a soldier with sickle cell trait. Military Medicine, 161, 303–305.

    PubMed  CAS  Google Scholar 

  • Naffakh, N., & Danos, O. (1996). Gene transfer for erythropoiesis enhancement. Molecular Medicine Today, 2, 343–348.

    Article  PubMed  CAS  Google Scholar 

  • Nowak-Gottl, U., Straeter, R., Sebire, G., & Kirkham, F. (2003). Antithrombotic drug treatment of pediatric patients with ischemic stroke. Paediatric Drugs, 5, 167–175.

    PubMed  Google Scholar 

  • Owusu-Ofori, S., & Riddington, C. (2002). Splenectomy versus conservative management for acute sequestration crises in people with sickle cell disease. Cochrane Database System Reviews, PubMed, CD003425.

    Google Scholar 

  • Pandit, H. M. (1996). Viruses–a conundrum. Physiological Chemistry and Physics and Medical NMR, 28, 29–33.

    PubMed  CAS  Google Scholar 

  • Rao, V. M., Sebes, J. I., Steiner, R. M., & Ballas, S. K. (1991). Noninvasive diagnostic imaging in hemoglobinopathies. Hematology/Oncology Clinics of North America, 5, 517–533.

    PubMed  CAS  Google Scholar 

  • Scheinman, J. I. (2003). Sickle cell disease and the kidney. Seminars in Nephrology, 23, 66–76.

    Article  PubMed  Google Scholar 

  • Serjeant, G. R. (1996). The role of preventive medicine in sickle cell disease. The Watson Smith lecture. Journal of the Royal College of Physicians of London, 30, 37–41.

    PubMed  CAS  Google Scholar 

  • Setty, B. N., Kulkarni, S., Dampier, C. D., & Stuart, M. J. (2001). Fetal hemoglobin in sickle cell anemia: relationship to erythrocyte adhesion markers and adhesion. Blood, 97, 2568–2573.

    Article  PubMed  CAS  Google Scholar 

  • Sing, C. F., Boerwinkle, E., & Moll, P. P. (1985). Strategies for elucidating the phenotypic and genetic heterogeneity of a chronic disease with a complex etiology. Progress in Clinical and Biological Research, 194, 39–66.

    PubMed  CAS  Google Scholar 

  • Smith, T. G., Ayi, K., Serghides, L., Mcallister, C. D., & Kain, K. C. (2002). Innate immunity to malaria caused by Plasmodium falciparum. Clinical and Investigative Medicine, 25, 262–272.

    PubMed  CAS  Google Scholar 

  • Steinberg, M. H. (1996). Modulation of the phenotypic diversity of sickle cell anemia. Hemoglobin, 20, 1–19.

    Article  PubMed  CAS  Google Scholar 

  • Steiner, R. M., Mitchell, D. G., Rao, V. M., Murphy, S., Rifkin, M. D., Burk, D. L., Jr., Ballas, S. K., & Vinitski, S. (1990). Magnetic resonance imaging of bone marrow: diagnostic value in diffuse hematologic disorders. Magnetic Resonance Quarterly, 6, 17–34.

    PubMed  CAS  Google Scholar 

  • Stinson, J., & Naser, B. (2003). Pain management in children with sickle cell disease. Paediatric Drugs, 5, 229–241.

    PubMed  Google Scholar 

  • Styles, L. A., & Vichinsky, E. P. (1996). Ototoxicity in hemoglobinopathy patients chelated with desferrioxamine. Journal of Pediatric Hematology/Oncology, 18, 42–45.

    Article  PubMed  CAS  Google Scholar 

  • Terk, M. R., Zee, C. S., Colletti, P. M., & Haywood, L. J. (1996). The application of magnetic resonance techniques to the evaluation of the patient with sickle-cell disease. Biomedical Instrumentation & Technology, 30, 349–353.

    CAS  Google Scholar 

  • Walco, G. A., & Dampier, C. D. (1987). Chronic pain in adolescent patients. Journal of Pediatric Psychology, 12, 215–225.

    Article  PubMed  CAS  Google Scholar 

  • Wald, E. R. (1985). Risk factors for osteomyelitis. The American Journal of Medicine, 78, 206–212.

    Article  PubMed  CAS  Google Scholar 

  • Weatherall, D. J. (1985). WWJRCJB: The developmental genetics of human hemoglobin. Progress in Clinical and Biological Research, 191, 3–25.

    PubMed  CAS  Google Scholar 

  • Weatherall, D. J., Old, J. M., Thein, S. L., Wainscoat, J. S., & Clegg, J. B. (1985). Prenatal diagnosis of the common haemoglobin disorders. Journal of Medical Genetics, 22, 422–430.

    Article  PubMed  CAS  Google Scholar 

  • Yang, Y. M., & Pace, B. (2001). Pharmacologic induction of fetal hemoglobin synthesis: cellular and molecular mechanisms. Pediatric Pathology & Molecular Medicine, 20, 87–106.

    Article  Google Scholar 

  • Zago, M. A., & Costa, F. F. (1985). Hereditary haemoglobin disorders in Brazil. Transactions of the Royal Society of Tropical Medicine and Hygiene, 79, 385–388.

    Article  PubMed  CAS  Google Scholar 

Download references

Acknowledgments

The authors would like to thank Dr. Samir K. Ballas, Dr. Johnson Haynes, Jr., Director, USA Sickle Center, Univ. of South Alabama College of Medicine, Dr. William D. Morrison, Director of Musculoskeletal (MSK) Radiology, Jefferson Medical College, Philadelphia and Dr. Carlton Dampier, Director, Marian Anderson SCC Hematology/Oncology, St. Christopher’s Hospital for Children, Philadelphia for sharing their expertise and materials in the preparation of this chapter.

Author information

Authors and Affiliations

  1. St. Joseph’s Regional Medical Center, 703 Main Street, Paterson, NJ, 07503, USA

    Valerie E. Armstead

Authors
  1. Valerie E. Armstead
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Genevieve D’Souza
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Valerie E. Armstead .

Editor information

Editors and Affiliations

  1. School of Medicine, Dept. Pathology & Pediatrics, Yale University, Cedar St. 333, New Haven, 06520, Connecticut, USA

    Brenda C. McClain

  2. Dept. Anesthesiology, Children's Memorial Hospital, Children's Plaza 2300, Chicago, 60614, Illinois, USA

    Santhanam Suresh

Rights and permissions

Reprints and permissions

Copyright information

© 2011 Springer Science+Business Media, LLC

About this chapter

Cite this chapter

Armstead, V.E., D’Souza, G. (2011). Sickle Cell Pain. In: McClain, B., Suresh, S. (eds) Handbook of Pediatric Chronic Pain. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-0350-1_11

Download citation

  • DOI: https://doi.org/10.1007/978-1-4419-0350-1_11

  • Published:

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4419-0349-5

  • Online ISBN: 978-1-4419-0350-1

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation