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Sickle Cell Disease is a serious disease that is life-threatening for patients being homozygous for the SS form or heterozygous for the SC or bthal forms. The CHU Brugmann hospital currently regularly treats about 70 homozygous adult patients and this number is in constant augmentation. The age average of the patients is below 30. The hospital developed a close collaboration with the Queen Fabiola Kids University Hospital to optimize the transition of young sickle cell patients from the pediatric to the adult network.
The emergency care of sickle cell patients remains a source of worry. Even with a correct treatment (Hydroxy-urea or exsanguineous transfusions), patients suffer from frequent sickle cell disease crisis when stress or infection cause hemolysis. The pain level is intolerable and causes emergency hospital admission (2 to 3 crisis per patient per year on average). The crisis are more frequent with poor compliance to the treatments.
There are several obstacles to the rapid and optimal management of these patients:
The prognostic value of the lactate dehydrogenase (LDH) level in a vaso-occlusive crisis was recently stressed while activation of the coagulation, translated by the elevation of various parameters including the rate of DD dimers, seemed associated with clinical complications. The deleterious role of increased oxidative stress has also been recently demonstrated in patients with sickle cell disease, opening new therapeutic avenues.
This study aims to prospectively evaluate the management of sickle cell patients being admitted in the emergency department for a vaso-occlusive crisis. The level of satisfaction of the patients will be measured.
The investigators will also evaluate the predictive value of several routine biological parameters regarding the severity of the crisis, including the values of nitrous albumin (PNA) as marker of oxydative stress. This last dosage will be made in collaboration with the team of Dr Wayenberg and Pr Bottari in Grenoble.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Emergency admitted sickle cell disease patients | Sickle Cell Disease Patients admitted inside the Emergency Department of the Brugmann Hospital for a vaso-occlusive crisis. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Satisfaction | Other | Satisfaction questionnaire to be filled in |
|
| Measure | Description | Time Frame |
|---|---|---|
| EVA pain scale | The pain will be evaluated using the EVA scale (Visual Analogic scale) by the patient himself and by the nursing staff (hetero-anamnesis). Time frames will be: at admission within the emergency department, 1 hour after admission, 3h after admission, 6 hours after admission and, if the pain did not disappear by then, every 6 hours until the pain disappears. | 48 hours |
| Measure | Description | Time Frame |
|---|---|---|
| Satisfaction questionnaire | Satisfaction of the patient regarding the management of his/her pain management. | 10 days |
| Morphine amount | Morphine amount administrated within the Emergency Department and during hospitalization length. |
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Inclusion Criteria:
Exclusion Criteria:
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Sickle Cell Disease patients, entering the Emergency Department of the Brugmann Hospital for a vaso-occlusive crisis.
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| Name | Affiliation | Role |
|---|---|---|
| Marie-Agnès Azerad, MD | CHU Brugmann | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| CHU Brugmann | Brussels | 1020 | Belgium |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 1710777 | Background | Platt OS, Thorington BD, Brambilla DJ, Milner PF, Rosse WF, Vichinsky E, Kinney TR. Pain in sickle cell disease. Rates and risk factors. N Engl J Med. 1991 Jul 4;325(1):11-6. doi: 10.1056/NEJM199107043250103. | |
| 17940207 | Background | Solomon LR. Treatment and prevention of pain due to vaso-occlusive crises in adults with sickle cell disease: an educational void. Blood. 2008 Feb 1;111(3):997-1003. doi: 10.1182/blood-2007-07-089144. Epub 2007 Oct 16. |
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| ID | Term |
|---|---|
| D000755 | Anemia, Sickle Cell |
| D000098644 | Vaso-Occlusive Crises |
| ID | Term |
|---|---|
| D000745 | Anemia, Hemolytic, Congenital |
| D000743 | Anemia, Hemolytic |
| D000740 | Anemia |
| D006402 | Hematologic Diseases |
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Not provided
| ID | Term |
|---|---|
| D000096002 | Physician Engagement |
| D059408 | Pain Management |
| ID | Term |
|---|---|
| D000074824 | Work Engagement |
| D010559 | Personnel Management |
| D009934 | Organization and Administration |
| D006298 | Health Services Administration |
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| Biological parameters | Other | Measure of standard routine biological parameters (Hb, GB, Plaq, LDH, CRP, TCA, INR, D Dimers) and dosage of nitrous albumin (PNA). |
|
| Pain management | Other | Validated pain scale questionnaire to be filled in |
|
| 10 days |
| Hospitalisation length | Time needed being hospitalized in order to control the pain level after the initial episode. | 10 days |
| Standard biological parameters | Dosage of the standard biological parameters (Hb, GB, Plaq, LDH, CRP, TCA, INR, D Dimers) and the nitrous albumin levels (PNA) at hospital admission. | at hospital emergency service admission |
| Standard biological parameters | Dosage of the standard biological parameters (Hb, GB, Plaq, LDH, CRP, TCA, INR, D Dimers) and the nitrous albumin levels (PNA) at the end of hospitalisation. | 10 days |
| 1736768 | Background | Brookoff D, Polomano R. Treating sickle cell pain like cancer pain. Ann Intern Med. 1992 Mar 1;116(5):364-8. doi: 10.7326/0003-4819-116-5-364. |
| 19380037 | Background | Payne R. Sickle cell anemia and pain: will data prevail over beliefs? Ann Emerg Med. 2009 May;53(5):596-7. doi: 10.1016/j.annemergmed.2008.10.022. No abstract available. |
| 19996405 | Background | Zempsky WT. Treatment of sickle cell pain: fostering trust and justice. JAMA. 2009 Dec 9;302(22):2479-80. doi: 10.1001/jama.2009.1811. No abstract available. |
| 20370779 | Background | Tanabe P, Artz N, Mark Courtney D, Martinovich Z, Weiss KB, Zvirbulis E, Hafner JW. Adult emergency department patients with sickle cell pain crisis: a learning collaborative model to improve analgesic management. Acad Emerg Med. 2010 Apr;17(4):399-407. doi: 10.1111/j.1553-2712.2010.00693.x. |
| 22506947 | Background | Tanabe P, Hafner JW, Martinovich Z, Artz N. Adult emergency department patients with sickle cell pain crisis: results from a quality improvement learning collaborative model to improve analgesic management. Acad Emerg Med. 2012 Apr;19(4):430-8. doi: 10.1111/j.1553-2712.2012.01330.x. |
| 22761289 | Background | Anie KA, Grocott H, White L, Dzingina M, Rogers G, Cho G. Patient self-assessment of hospital pain, mood and health-related quality of life in adults with sickle cell disease. BMJ Open. 2012 Jul 2;2(4):e001274. doi: 10.1136/bmjopen-2012-001274. Print 2012. |
| 22892192 | Background | Stankovic Stojanovic K, Steichen O, Lefevre G, Bachmeyer C, Avellino V, Grateau G, Girot R, Lionnet F. High lactate dehydrogenase levels at admission for painful vaso-occlusive crisis is associated with severe outcome in adult SCD patients. Clin Biochem. 2012 Dec;45(18):1578-82. doi: 10.1016/j.clinbiochem.2012.07.114. Epub 2012 Aug 8. |
| 22253781 | Background | Ataga KI, Brittain JE, Desai P, May R, Jones S, Delaney J, Strayhorn D, Hinderliter A, Key NS. Association of coagulation activation with clinical complications in sickle cell disease. PLoS One. 2012;7(1):e29786. doi: 10.1371/journal.pone.0029786. Epub 2012 Jan 11. |
| 22122125 | Background | Rees DC, Gibson JS. Biomarkers in sickle cell disease. Br J Haematol. 2012 Feb;156(4):433-45. doi: 10.1111/j.1365-2141.2011.08961.x. Epub 2011 Nov 28. |
| 15599544 | Background | Schnog JB, Teerlink T, van der Dijs FP, Duits AJ, Muskiet FA; CURAMA Study Group. Plasma levels of asymmetric dimethylarginine (ADMA), an endogenous nitric oxide synthase inhibitor, are elevated in sickle cell disease. Ann Hematol. 2005 May;84(5):282-6. doi: 10.1007/s00277-004-0983-3. Epub 2004 Dec 14. |
| 22318468 | Background | Nur E, Brandjes DP, Teerlink T, Otten HM, Oude Elferink RP, Muskiet F, Evers LM, ten Cate H, Biemond BJ, Duits AJ, Schnog JJ; CURAMA study group. N-acetylcysteine reduces oxidative stress in sickle cell patients. Ann Hematol. 2012 Jul;91(7):1097-105. doi: 10.1007/s00277-011-1404-z. Epub 2012 Feb 10. |
| 19591921 | Background | Wayenberg JL, Ransy V, Vermeylen D, Damis E, Bottari SP. Nitrated plasma albumin as a marker of nitrative stress and neonatal encephalopathy in perinatal asphyxia. Free Radic Biol Med. 2009 Oct 1;47(7):975-82. doi: 10.1016/j.freeradbiomed.2009.07.003. Epub 2009 Jul 8. |
| D006425 |
| Hemic and Lymphatic Diseases |
| D006453 | Hemoglobinopathies |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D003695 | Delivery of Health Care |
| D010346 | Patient Care Management |
| D017530 | Health Care Quality, Access, and Evaluation |
| D013812 | Therapeutics |
| D019468 | Disease Management |