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

2021 ◽  
Vol 88 ◽  
pp. 102531
Author(s):  
Immacolata Tartaglione ◽  
Crawford Strunk ◽  
Charles Antwi-Boasiako ◽  
Biree Andemariam ◽  
Raffaella Colombatti ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Pain Crisis ◽  
First Pain

scholarly journals Diagnosis patterns of sickle cell disease in Ghana: a secondary analysis using cohort data

2021 ◽  
Author(s):  
Alexandra M. Sims ◽  
Kwaku Osei Bonsu ◽  
Rebekah Urbonya ◽  
Fatima Farooq ◽  
Fitz Tavernier ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Screening Program ◽  
Severe Disease ◽  
Secondary Analysis ◽  
Sub Saharan Africa ◽  
Cell Disease ◽  
Screening Programs ◽  
Pain Crisis ◽  
First Pain

Abstract Background: Despite having the highest prevalence of sickle cell disease (SCD), Sub-Saharan Africa lacks a robust screening program. We sought to capture the diagnosis patterns of SCD, particularly age and mechanism at SCD and age of first pain crisis, in Accra, Ghana. Methods We administered a survey to parents of offspring with SCD between 2009–2013 in Accra as a part of a larger cohort study and analyzed a subset of the data to determine diagnosis patterns. Univariate analyses were performed on diagnostic patterns; bivariate analyses were conducted to determine if patterns differ by offspring’s age, or their disease severity. Pearson’s chi-squared were calculated. Results Data was collected on 354 unique participants from parents. 44% were diagnosed with SCD by age four; 46% had experienced a pain crisis by the same age. 66% were diagnosed during pain crisis, either in acute (49%) or primary care (17%) settings. Younger (< 18 years) offspring were diagnosed with SCD at an earlier age (74% by four years old); in the adult ( > = 18 years) group, 30% were diagnosed by four years (p < 0.001). Few were diagnosed via newborn screen. Half with severe disease were diagnosed by age four, compared to 31% with mild disease (p = 0.009). Conclusions These findings reflect a reliance on diagnosis in acute settings, and a relative underutilization of systematic screening programs. By understanding current patterns, opportunities remain to more effectively detect and treat SCD in this high prevalence population.

scholarly journals Diagnosis patterns of sickle cell disease in Ghana: a secondary analysis

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Alexandra M. Sims ◽  
Kwaku Osei Bonsu ◽  
Rebekah Urbonya ◽  
Fatimah Farooq ◽  
Fitz Tavernier ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Screening Program ◽  
Medical Center ◽  
Academic Medical Center ◽  
Sub Saharan Africa ◽  
Cell Disease ◽  
Mortality And Morbidity ◽  
Pain Crisis ◽  
First Pain

Abstract Background Despite having the highest prevalence of sickle cell disease (SCD) in the world, no country in Sub-Saharan Africa has a universal screening program for the disease. We sought to capture the diagnosis patterns of SCD (age at SCD diagnosis, method of SCD diagnosis, and age of first pain crisis) in Accra, Ghana. Methods We administered an in-person, voluntary survey to parents of offspring with SCD between 2009 and 2013 in Accra as a part of a larger study and conducted a secondary data analysis to determine diagnosis patterns. This was conducted at a single site: a large academic medical center in the region. Univariate analyses were performed on diagnosis patterns; bivariate analyses were conducted to determine whether patterns differed by participant’s age (children: those < 18 years old whose parents completed a survey about them, compared to adults: those > = 18 years old whose parents completed a survey about them), or their disease severity based on SCD genotype. Pearson’s chi-squared were calculated. Results Data was collected on 354 unique participants from parents. Few were diagnosed via SCD testing in the newborn period. Only 44% were diagnosed with SCD by age four; 46% had experienced a pain crisis by the same age. Most (66%) were diagnosed during pain crisis, either in acute (49%) or primary care (17%) settings. Children were diagnosed with SCD at an earlier age (74% by four years old); among the adults, parents reflected that 30% were diagnosed by four years old (p < 0.001). Half with severe forms of SCD were diagnosed by age four, compared to 31% with mild forms of the disease (p = 0.009). Conclusions The lack of a robust newborn screening program for SCD in Accra, Ghana, leaves children at risk for disease complications and death. People in our sample were diagnosed with SCD in the acute care setting, and in their toddler or school-age years or thereafter, meaning they are likely being excluded from important preventive care. Understanding current SCD diagnosis patterns in the region can inform efforts to improve the timeliness of SCD diagnosis, and improve the mortality and morbidity caused by the disease in this high prevalence population.

Impact of Intravenous Opioid Shortage on Managing Pain Crisis in Sickle Cell Disease

2021 ◽  
pp. 106002802110245
Author(s):  
Jin Han ◽  
Santosh L. Saraf ◽  
Michel Gowhari ◽  
Faiz Ahmed Hussain ◽  
Shivi Jain ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Pain Crisis

scholarly journals P10 An audit to assess the prescribing of analgesia in children who present with pain crisis due to Sickle Cell Disease (SCD)

2020 ◽  
Vol 105 (9) ◽  
pp. e11.1-e11
Author(s):  
Masuma Dhanji
Keyword(s):  
Sickle Cell Disease ◽  
Integrated Care ◽  
Sickle Cell ◽  
Care Pathway ◽  
Electronic Prescribing ◽  
List Type ◽  
Cell Disease ◽  
Paediatric Patients ◽  
Pain Crisis ◽  
Integrated Care Pathway

AimTo assess the prescribing of analgesia to manage pain crises in children with SCD. This was to establish whether the Trust was meeting national and local standards. Prompt pain control is essential to reduce length of stay and further complications.1Standards100% of admissions will be prescribed regular paracetamol and non-steroidal anti-inflammatory drugs (NSAIDs) at the recommended frequency unless contraindicated in accordance with national guidance.2 3100% of admissions will be prescribed appropriate doses of analgesia with consideration to weight and age in accordance with local policy.4MethodThe audit was registered with the Trust’s audit committee. A list of paediatric patients with the diagnosis of SCD was sought from paediatricians with an interest in haematology. A data collection form was created. Data was collected retrospectively over a one-year period. A total of 60 admissions were reviewed to check whether analgesia was prescribed regularly at the recommended frequency, and at the correct dose. Results were analysed using descriptive statistical analysis. Exclusion criteria included patients with hospital admissions under 24 hours.ResultsA total of 55 admissions were included in the final sample. The audit showed the Trust was non-adherent to both standards assessed. A total of 45% (95% CI [31.9%, 58.1%]) of admissions were prescribed regular analgesia. A total of 78% (95% CI [67.9%, 88.9%] of admissions were prescribed appropriate doses of analgesia. Two main reasons were found as to why analgesia was prescribed at the incorrect dose. This was due to incorrect weights recorded on the electronic system (n=4) and doses based on age only (n=8).ConclusionThe results show prescribers are familiar with the correct doses of analgesia but fail to prescribe analgesia regularly. This highlights an opportunity for education and training in the management of pain crisis in SCD. One recommendation includes development of an integrated care pathway booklet for paediatric patients presenting with pain crisis due to SCD. Integrated care pathway booklets have been implemented for other conditions such as cystic fibrosis yielding positive outcomes. The results have highlighted key issues surrounding the electronic prescribing system such as out-of-date weights remaining on the system unless updated, and default treatment protocols. The electronic prescribing system requires refinement for use within paediatrics. One suggestion includes compulsory weight field on admission. Limitations of this audit included small sample size. There was a lack of data to make suggestions based on different ages.ReferencesRees D, Olujohungbe A, Parker N, et al. Guidelines for the management of the acute painful crises in sickle cell disease. Br J Haemato 2003;120:744–752.National Institute for Health and Care Excellence (2012) Sickle cell disease: managing acute painful episodes in hospital. NICE Guideline (CG143).Paediatric Formulary Committee. BNF for Children (2018–2019). London: BMJ Group, Pharmaceutical Press, and RCPCH Publications; (2018).General Hospital (2015) Management of sickle cell disease in paediatric patients (CG377).

Impact of Hydroxyurea on Thrombin Antithrombin Complex and Vaso-Occlusive Crisis in Pediatric Sickle Cell Disease.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 5525-5525
Author(s):  
Mohsen Saleh Elalfy ◽  
Ashraf M. Abdelmonem ◽  
Soha Youssef ◽  
Heba Ismail
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Coagulation System ◽  
P Value ◽  
Cell Disease ◽  
Significant Difference ◽  
Healthy Control ◽  
Pain Crisis

Abstract Background: Several studies suggest that increased activity of the coagulation system may be important in the pathogenesis of vascular occlusion in sickle cell disease. Hydroxyurea (HU) has been shown to reduce the frequency of vaso-occlusive manifestations in both adults and children with sickle cell disease (SCD). Aim: To analyze the effect of HU on Thrombin-Antithrombin (TAT) as a marker of thrombin generation and hypercoagualbility in SCD and to find out the relation between TAT level and vaso-occusive crisis. Subjects and Method: we evaluated 37 child with sickle cell hemoglobinopathy (mean age 10.92±5.39 years) and 15 normal control children (mean age 9.75±6.34 years). Informed consent was obtained from patients and/or guardians and study approval by local IRB was obtained. Twenty-two patients (59.5%) were on HU, 15 (41.5%) patients did not receive HU, 7 (46.7%) of them were transfusion dependant. TAT assay was done in vitro using a sandwich enzyme immunoassay. Results: Mean patients’ age at institution of HU was 8.54± 3.85 years with median treatment duration of 4.5 years. Causes for initiating HU therapy were frequent blood transfusion in 11 patients (50%), frequent pain crisis (≥ 3/year) in 9 patients (41%), severe anemia and parents refusing blood transfusion in 1 patient (4.5%) and stroke in another patient (4.5%). HU dose was 20.82±4.95 mg/kg/day. We measured TAT in all patients and compared them to healthy control. There was significant difference in TAT level in sickle cell patients (198.86±185.7) compared to healthy control 2.91±0.94, [P value < 0.0001]. When the level of TAT was compared between the HU and non-HU groups we found that patients on HU had statistically significant lower TAT level (172.36 vs.225.37) [P=0.039]. There was also a significant negative correlation between HU dose and TAT level (p=0.03). A significant positive correlation between number of vaso-occlusive crisis/year [P=0.03], frequency of pain crisis/year [P=0.04], duration of pain crisis [P=0.03] and TAT level was observed. Conclusion: Hydroxyurea has significant inhibitory effect on thrombogenesis in sickle cell patients, which may be another mechanism for reducing vaso-occlusive crisis. Sickle cell children with higher TAT level had more frequent and severe vaso-occlusive crisis.

Sevuparin for the treatment of acute pain crisis in patients with sickle cell disease: a multicentre, randomised, double-blind, placebo-controlled, phase 2 trial

2021 ◽  
Vol 8 (5) ◽  
pp. e334-e343
Author(s):  
Bart J Biemond ◽  
Anil Tombak ◽  
Yurdanur Kilinc ◽  
Murtadha Al-Khabori ◽  
Miguel Abboud ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Acute Pain ◽  
Phase 2 ◽  
Cell Disease ◽  
Double Blind ◽  
Double Blind Placebo ◽  
Phase 2 Trial ◽  
Pain Crisis

Evidence-Based Mini-Review: Are Systemic Corticosteroids an Effective Treatment for Acute Pain in Sickle Cell Disease?

Hematology ◽  
2010 ◽  
Vol 2010 (1) ◽  
pp. 416-417 ◽  
Author(s):  
L. Vandy Black ◽  
Wally R. Smith
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Adjunctive Treatment ◽  
Cell Disease ◽  
X Ray ◽  
Systemic Corticosteroids ◽  
Potential Efficacy ◽  
History Of ◽  
Chest X Ray ◽  
Pain Crisis

Abstract An 18-year-old African-American male with sickle cell disease (SCD) is admitted to the hospital with a vaso-occlusive pain crisis affecting his chest and right upper extremity. He has a history of asthma but does not have a fever or respiratory symptoms, and a chest X-ray is negative for an infiltrate. He is treated with intravenous fluids and morphine. You are asked about the potential efficacy of systemic corticosteroids as an adjunctive treatment for pain control.

Chronic tonsillitis, tonsillectomy and sickle cell crises

1987 ◽  
Vol 101 (5) ◽  
pp. 467-470 ◽  
Author(s):  
G. T. A. Ijaduola ◽  
O. O. Akinyanju
Keyword(s):  
Sickle Cell Disease ◽  
General Anaesthesia ◽  
Sickle Cell ◽  
Chronic Tonsillitis ◽  
Cell Disease ◽  
Pain Crisis ◽  
The Mean ◽  
One Year ◽  
The One ◽  
Sickle Cell Crises

AbstractA study of the effect of tonsillectomy on the frequency of sickle cell pain crises was carried out on 15 patients with sickle cell disease (Hb SS) who presented with complaints of frequent pain crises and were found to have chronic tonsillitis. They comprised 9 females and 6 males and ranged in age from 6 to 35 years, with a mean of 15 years. Tonsillectomy was performed under general anaesthesia. The mean number of pain crises in the one-year period after tonsillectomy was 1.5 and was significantly less than the mean number 4.7 in the one year preceding the operation (p<0.001). Four patients failed to show a reduced number of crises and these were those whose tonsils at operation showed no pus in their tonsillar crypts. It is thus observed that chronic tonsillitis, symptoms of which may not be volunteered at examination, is a potent inductor of sickle cell pain crisis and that tonsillectomy is an effective mode of treatment, especially when the tonsillar crypts contain pus.

scholarly journals Hematological Changes from Baseline in Children with Sickle Cell Disease Admitted for Acute Chest Syndrome Compared to Acute Pain Crisis

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 4909-4909
Author(s):  
Timothy Klouda ◽  
Nataly Apollonsky ◽  
Deepti Raybagkar ◽  
Bruce Bernstein
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Acute Pain ◽  
Neutrophil Count ◽  
Laboratory Data ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
History Of ◽  
Pain Crisis ◽  
Hematological Changes

Abstract Title: Hematological Changes from Baseline in Children with Sickle Cell Disease Admitted for Acute Chest Syndrome Compared to Acute Pain Crisis Authors: Timothy Klouda1, Deepti. Raybagkar2, Bruce Bernstein1, Nataly Apollonsky2, Institutes:1Pediatrics, St Christopher's Hospital for Children, Philadelphia, PA, United States, 2Hematology, St Christopher's Hospital for Children, Philadelphia, PA, United States, Introduction: Children with Sickle Cell Disease suffer from multiple complications including acute pain crisis (VOC) and acute chest syndrome (ACS). Nearly 30% of children with SCD have had one episode of ACS, with the incidence higher in early childhood. The proposed pathophysiology of ACS is thought to be multi-factorial, with pulmonary fat embolism or infectious etiology being identified in a large number of patients. Increased sickling due to hypoxemia or pain has been shown to place patients at risk for ACS development., Studies have shown an increase in inflammatory markers including leukocytes and neutrophils, along with a decreased hemoglobin in SCD children who developed ACS, but no studies to date have compared laboratory changes during the acute illness to their baseline values. We hypothesized that children with SCD who are admitted for ACS will have a larger decrease in hemoglobin from baseline and a higher increase in white blood cell count from baseline when compared to those admitted for an acute pain crisis. Methods: Through retrospective chart review of patients with SCD admitted to St.Christopher's Hospital for Children we identified 45 patients with ACS. Laboratory data collected on admission from chart review included SCD genotype, age, BMI, hemoglobin, white blood cell count, absolute neutrophil count, absolute eosinophil count, platelets, reticulocyte count, hemoglobin F, vital signs and medication history. All 45 children had laboratory data collected from an acute pain crisis that occurred during a different admission for comparison. Collected data was compared to baseline laboratory data, collected during routine visit at sickle cell clinic within 1 year of admission. Changes in laboratory data from baseline during admission for ACS were compared to changes during admission for uncomplicated VOC. Results: Children with SCD who were admitted or developed ACS during admission had a larger increase in leukocyte count (6.99 vs 4.18, p=0.027) and neutrophil count (6.3 vs 3.74, p=0.04) from baseline compared to those admitted for VOC alone. Patients with ACS development also had a larger decrease in platelets (-124.74 vs -56.21, p=.047) from baseline when compared to VOC admissions. There was no statistically significant change from baseline labs when comparing hemoglobin (p=0.10), eosinophil count (p=.382), reticulocyte count (p=0.754), AST (p=0.061) and ALT (p=0.082) in the ACS and VOC groups. Children with a history of 2 or more lifetime ACS were more likely to have OSA (p=0.021), 3 or more VOCs in the past year (p=0.002), and a history of splenectomy, but it was not found to be statistically significant (p=0.155) Conclusion: Children with SCD who developed or were admitted with ACS had a significant increase in leukocyte and neutrophil count from baseline, and a decrease in platelets when compared to VOC admissions. There was no significant change from baseline in hemoglobin, reticulocyte and eosinophils detected. Future larger and multi-center prospective studies need to be performed to confirm the various changes identified in hematological markers seen in ACS vs VOC. Disclosures No relevant conflicts of interest to declare.

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