scholarly journals Air Drep—A Retrospective Study Evaluating the Influence of Weather Conditions and Viral Epidemics on Vaso-Occlusive Crises in Patients with Sickle Cell Disease Living in French Guiana

Author(s):  
Marie-Claire Parriault ◽  
Claire Cropet ◽  
Aniza Fahrasmane ◽  
Stéphanie Rogier ◽  
Michaël Parisot ◽  
...  

(1) Objectives: French Guiana is the French territory most affected by sickle cell disease (SCD). This study investigates the associations between different environmental factors relative to climate, infectious outbreaks, and emergency visits or weekly hospital admissions for vaso-occlusive crisis (VOC). The identification of risk factors would lead to better patient care and patient management, and more targeted prevention and therapeutic education for patients with SCD in French Guiana. (2) Methods: This study was performed using data collected from the medicalized information system and emergency medical records of Cayenne General Hospital, between 1 January 2010 and 31 December 2016. ARIMA models were used to investigate the potential impact of weather conditions and flu epidemics on VOC occurrence. (3) Results: During the study period, 1739 emergency visits were recorded among 384 patients, of which 856 (49.2%) resulted in hospitalization, 811 (46.6%) resulted in hospital discharge, and 72 (4.2%) in another orientation. Decreased temperature and decreased humidity were both independent factors associated with an increase of VOC cases (p = 0.0128 and p = 0.0004, respectively). When studying severe VOC (leading to hospitalization, with or without prior emergency visit), 2104 hospital admissions were recorded for 326 patients. The only factor associated with severe VOC, in the multivariate analysis, was flu epidemics (p = 0.0148). (4) Conclusions: This study shows a link between climate, flu epidemics, and VOC in French Guiana. Patient’s awareness of risks related to climate and flu epidemics should be encouraged, as home prevention measures can help avoid painful crises. Moreover, physicians should encourage patients to get immunized for influenza every year.

2021 ◽  
Author(s):  
Elena Maria Rincón-López ◽  
María Luisa Navarro Gómez ◽  
Teresa Hernández-Sampelayo Matos ◽  
David Aguilera-Alonso ◽  
Eva Dueñas Moreno ◽  
...  

Abstract Severe bacterial infections (SBI) have become less frequent in children with sickle cell disease (SCD) in the last decades. However, because of their potential risk of SBI, they usually receive empirical therapy with broad-spectrum antibiotics when they develop fever and are hospitalized in many cases. We performed a prospective study including 79 SCD patients with fever [median age 4.1 (1.7–7.5) years, 78.5% males; 17 of the episodes were diagnosed with SBI and 4 of them were confirmed] and developed a risk score for the prediction of SBI. The optimal score included CRP > 3 mg/dl, IL-6 > 125 pg/ml and hypoxemia, with an AUC of 0.91 (0.83–0.96) for the prediction of confirmed SBI and 0.86 (0.77–0.93) for possible SBI. We classified the patients in 3 groups: low, intermediate and high risk of SBI. Our risk-score based management proposal could help to safely minimize antibiotic treatments and hospital admissions in children with SCD at low risk of SBI.


2011 ◽  
Vol 33 (7) ◽  
pp. 491-495 ◽  
Author(s):  
Thomas G. Day ◽  
Swee Lay Thein ◽  
Emma Drasar ◽  
Moira C. Dick ◽  
Susan E. Height ◽  
...  

Haematologica ◽  
2016 ◽  
Vol 102 (4) ◽  
pp. 666-675 ◽  
Author(s):  
Frédéric B. Piel ◽  
Sanjay Tewari ◽  
Valentine Brousse ◽  
Antonis Analitis ◽  
Anna Font ◽  
...  

2016 ◽  
Vol 21 (12) ◽  
pp. 2893-2902 ◽  
Author(s):  
Shan-Estelle Brown ◽  
Daniel F Weisberg ◽  
William H Sledge

This study investigated coping with chronic illness in the adult patient–caregiver relationship for sickle cell disease, marked by debilitating acute and chronic pain. One-on-one interviews ( N = 16) were conducted with eight primary caregivers of eight adults with extremely high hospital use, severe sickle cell disease with hospital admissions several times monthly over successive years. Caregivers were predominantly parents; two were romantic partners. Caregivers attributed disruptions to the disease’s variability, tensions in how much support to give, and adults’ inability to fulfill parental obligations. Both groups expressed fears of patients’ increasing age, declining health, and early death. Targeted counseling and resilience training is recommended.


Author(s):  
Dr. Rajesh Shukla ◽  
Dr. Mehul Gajera

Background: The most predominant form of haemoglobinopathy worldwide is sickle cell disease. The greatest burden of the disease lies in sub-Saharan Africa and Asia5. Objective: To evaluate the effectiveness of HU therapy in sickle cell disease as measured by decrease in crises rate, hospital admissions, days of hospitalization and number of blood transfusions. Methods: the study was conducted on 79 children of 1-16 year age. Out of which in only 75 patients Hydroxyurea therapy was started as they were found to be eligible. 16% of the patients responded to 15 mg/kg/ day of HU, 50.66% responded to 20 mg/kg/ day, 29.33% to 25 mg/kg/ day and only 4% needed a dose escalation to 30 mg/kg/ day for the response. Results: Our study showed a significant reduction in the VOC rate from 243 episodes to 46 episodes (p value <0.001), the number of ACS reduced from 37 episodes to 5 episodes (p value <0.001), also there is a significant decline in the rates of hemolytic crises from 63 episodes to 10 episodes per year, Significant increase in the HbF levels from 15.87±5.50% to 21.77.±4.06% (p value <0.001). There was a definite and significant reduction in the number of hospitalization days from 7.76±4.76 to 3.79±2.29 days and in the number of admissions per year dropped significantly from 4.80 ± 1.41 to 1.42± 0.61 per year. Conclusion: Hydroxyurea reduced the frequency of painful crises and diminished the number of hospitalization, transfusion, and episodes of acute chest syndrome17. Keywords: Haemoglobin (Hb), Hydroxyurea (HU), Mean Corpuscular Volume (MCV), Pletelet Counts, Sickle Cell Disease (SCD), White Blood Cells (WBC).


Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 4466-4466 ◽  
Author(s):  
Pooja Lothe ◽  
Tiffany Pompa ◽  
Maneesh Jain ◽  
Parshva Patel ◽  
Yayu Liang ◽  
...  

Abstract Objective: A comparative cost analysis of sickle cell admissions vs. stem cell transplants in sickle cell patients. Hypothesis: We believe the overall cost of a bone marrow transplant for a sickle cell patient will be less than that of a patient with multiple sickle cell admissions. Background: Sickle cell disease remains an increasing burden to the cost of health care and health care providers. The disease results in a variety of serious organ system complications that can lead to life-long disabilities and/or early death. Despite the advent of hydroxyurea, sickle cell admissions and cost have been increasing over the course of several years. Various contributing factors may include socioeconomic status, complications of sickle cell anemia itself, narcotic dependence and noncompliance with medications. Bone marrow transplants were introduced in 1982 as an option for the treatment of sickle cell anemia, and are currently the only curative option in this disease. A study conducted at the NIH from 2004-2013, found that bone marrow transplant reversed the disease in 26 of 30 patients (87%) (Hseih et al, N Engl J Med 2009; 361:2309-2317). The patients ultimately had a normal hemoglobin, fewer hospitalizations, and lower use of narcotics to treat pain from the disease. However, the underutilization of bone marrow transplant continues to exist and may in part be secondary to a lack of fully matched donors. To overcome this challenge, the Johns Hopkins group developed a nonmyeloablative bone marrow transplantation platform using HLA haploidentical donors for patients using posttransplant cyclophosphamide. As a result, 17 patients were successfully transplanted, 14 from HLA-haploidentical and 3 from HLA-matched related donors (Meade et al, Blood. 2012; 120(22):4285-4291). Due to this, most patients with sickle cell disease have the potential to undergo a successful bone marrow transplant. However, an analysis comparing the cost of admissions vs. transplant has yet to be determined. In order to create an effective cost comparison, we utilized a nationwide database. Methods: US hospital admissions were identified from discharge data from the Nationwide Inpatient Sample (NIS), Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality from 1998 to 2011 using ICD9 codes. Admissions were included if they had an ICD9 code for Sickle cell anemia (282.5). Results: Table 1. Year 1998 1999 2000 2001 2002 2003 2004 Length of Stay (Days) 6.21 6.13 6.37 6.19 6.46 6.31 6.05 Cost ($) 15,724.00 16,465.00 18,654.00 18,750.00 22,587.00 24,501.00 23,743.00 Table 2. Year 2005 2006 2007 2008 2009 2010 2011 Average Length of stay (Days) 6.16 6.13 6.00 5.91 5.62 5.71 5.61 6.04 Cost ($) 25,129.00 27,471.00 28,425.00 27,314.00 29,767.00 29,929.00 31,683.00 24,687.00 The cost per hospitalization has almost doubled since 1998, despite a slight decrease in length of stay from an average of 6.5 days to 5.6 days from 1998-2011. According to the data base from our study, the average cost per patient per hospitalization was $24,687. The average number of admissions per year for a single patient with sickle cell anemia is 6 (Ballas et al, Am J Hem 2009) for an estimated overall cost per year of $148,000. However, this value underestimates the true cost since this does not include emergency room visits, medicine costs, and readmission rates. Conclusion: In the age of cost effective medicine, clinicians struggle to find a balance between low cost and optimal patient care. An underutilized modality of care and even cure for sickle cell disease is bone marrow transplant. The sickle cell information center website estimates the cost of the transplant process for most patients to be $150,000 to $250,000 which includes pre-transplant evaluation, transplant stay, and post-transplant follow-up (https://scinfo.org). We estimate that a bone marrow transplant is approximately equivalent to the cost of ten sickle cell hospital admissions. According to this analysis, undergoing a bone marrow transplant would ultimately prove to be more cost efficient while decreasing the rate of complications associated with this debilitating disease. Disclosures No relevant conflicts of interest to declare.


2021 ◽  
Author(s):  
Alizee Sterlin ◽  
Mariane de Montalembert ◽  
Melissa Taylor ◽  
Sandrine Mensah ◽  
Marie Vandaele ◽  
...  

BACKGROUND Sickle cell disease is a lifelong disease for which health outcomes may be greatly influenced by patient and parent self-care knowledge and skills. Therapeutic education (TPE) is a patient-centered teaching instrument, based on patient adaptative processes and their subjective and objective needs, whether expressed or not. TPE is delivered in individual or group sessions, usually during face-to-face meetings. The COVID-19 pandemic has impacted the modalities of TPE by promoting online training. The RoFSED health network, which operates in the Paris region and is focused on coordination of care and TPE for children with SCD, has implemented online TPE since May 2020. OBJECTIVE To evaluate the accessibility of children with SCD to online TPE. METHODS Characteristics of TPE before and after the onset of the pandemic were compared to evaluate the accessibility of children with SCD to online TPE. RESULTS We observed an increase in the total number of trained children, mostly related to increased participation in individual sessions. The age distribution of participants did not vary. However, participation varied greatly according to the geographical area of residence, with a major drop from 22.4 to 4.9% of attendees living in Seine saint Denis, one of the most socio-economically deprived French departments, contrasting with an increased proportion of attendees living in Paris and Hauts-de-Seine, two of the most favoured French departments. CONCLUSIONS Our results highlight the potential of online TPE tools for patients and families with SCD but also reveal unequal access according to socio-economic status. Future research is needed, in particular on the safety and efficacy of these tools, as well as on improving access for the more vulnerable patients living in socially disadvantaged areas.


Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 3790-3790
Author(s):  
Deborah Yallop ◽  
Edward R. Duncan ◽  
Ellie Norris ◽  
Gary Fuller ◽  
Nikki Thomas ◽  
...  

Abstract The clinical severity of sickle cell disease (SCD) is dependent on genetic and environmental variables. The impact of environmental factors on disease is a major public health issue and air pollution has been consistently correlated with poor health outcomes. Environmental factors in SCD have been poorly studied. We have retrospectively studied the numbers of daily admissions with vaso-occlusive sickle cell pain to King’s College Hospital, London, in relation to local daily air quality measurements. We analysed 1047 patient episodes over 1400 days (1st January 1998 to 31st October 2001). Statistical time series analysis was performed using cross-correlation function (CCF), where the observations of one series are correlated with the observations of another series at various lags and leads, values >0.05 being significant. This showed a significant association between increased numbers of admissions and low levels of nitric oxide (NO) (CCF=0.063), low levels of carbon monoxide (CO) (CCF=0.064) and high levels of ozone (O3) (CCF=0.067). There was no association with sulphur dioxide (SO2), nitric dioxide (NO2) or dust. The significant results were further examined using quartile analysis. This confirmed that increased numbers of hospital admissions were associated with high levels of O3 (oneway ANOVA p=0.039) and low levels of CO (oneway ANOVA p=0.042). Low NO levels were also associated with increased admissions, not however reaching statistical significance on quartile analysis (oneway ANOVA p=0.158). O3 levels show marked seasonal variation, with high levels occurring in summer months in the UK. We have previously shown a trend towards increased admissions in the summer months with acute sickle related pain, whereas other groups, which are primarily based in rural tropical climates, found increased admissions in cold, rainy months. The adverse effect of high O3 levels may explain this difference. There is no direct evidence to explain the mechanism by which high O3 levels leads to vaso-occlusion in SCD but high levels of O3 have been linked to reduced respiratory function, which may in turn precipitate vaso-occlusion. Independent studies have shown high CO levels are linked to increased respiratory and cardiovascular admissions. Paradoxically we found that high CO levels were linked to decreased admission numbers and may be protective against acute pain in SCD. CO may confer benefit by forming carboxyhaemoglobin that cannot polymerise resulting in decreased sickling. Previous studies have shown prolonged red cell survival in vivo following administration of CO to patients with SCD. Our study also suggests higher levels of atmospheric NO are linked to fewer admissions. NO is known to be central in the pathophysiology of vaso-occlusion and sickle cell patients are thought to have functional deficiency of NO. Many groups have reported inhaled NO as beneficial in the treatment of sickle pain. Our study suggests air quality has a significant effect on acute pain in SCD and that patients should be counselled accordingly. Based on these findings it would be appropriate to warn patients that high O3 levels might precipitate complications of SCD. The potential beneficial effect of CO and NO is intriguing and requires further investigation.


Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 4826-4826 ◽  
Author(s):  
Vladimir K. Gotlieb ◽  
Khine Z. Oo

Abstract A 45-year old Jamaican man with sickle cell disease came to our clinic complaining of chronic left ankle ulcer for one year. He stated that when he was a young boy he had had several hospital admissions for sickle cell crisis, usually precipitated by physical activity or infection, once or twice per month. We were surprised to find out that he had never had a crisis since the age of fifteen. We doubted his diagnosis yet his hemoglobin electrophoresis revealed HbS was 94.7%, HbF was 0.6% and HbA2 was 4.7%. He denied taking any medication. However, he admitted smoking 4 to 5 cigarettes of marijuana a day for the past 30 years. Cannabis has been used as a medicine even before the Christian era in Asia, first in China then mainly in India. Its use was later spread to the West. The general indications for marijuana were reported as severe nausea and vomiting, weight loss associated with debilitating illnesses, spasticity, pain syndrome, and glaucoma. Numerous Phase I–III studies in 2000 subjects with exposure of Sativex by GW Pharmaceuticals from United Kingdom demonstrated that the patients attained good sleep quality, which may improve patients’ quality of life in disabling chronic pain syndromes. We reviewed the literature on any report that cannabinoids could change the severity of sickle cell disease. We found the study from West Indies, which investigated the perception that marijuana use ameliorated the complications of sickle cell disease, in year 2000 and 2004. The study concluded that marijuana smoking is common in adults with sickle cell disease but its usage is unrelated to clinical severity of the disease. We also found a report, from Central Middlesex Hospital in London, United Kingdom, which observed 86 young adults with HbSS, HbSC and HbSbetathalassemia disease, median age being 30 years. Results of this study showed that 31 (36%) had used cannabis in the previous 12 months to relieve the symptoms associated with sickle cell disease. Symptoms related to sedation and mood effects were reported in 77% of patients. The main reasons for use of cannabis were to reduce pain in 52% and to induce relaxation or relieve anxiety and depression in 39%. (5) We wondered if any physicians had observed similar effects of marijuana in sickle cell disease patients in their practice. Could ‘Marijuana Use has beneficial effect on Sickle Cell Disease’ be an interesting topic for a broader clinical trial or a retrospective analysis?


Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4841-4841
Author(s):  
Sanjay Tewari ◽  
Fred Piel ◽  
Valentine Brousse ◽  
Baba PD Inusa ◽  
Paul Telfer ◽  
...  

Abstract Background: Sickle cell disease (SCD) is a very variable condition, with some patients being asymptomatic and others admitted frequently to hospital. Genetic factors have been extensively investigated but only explain a small amount of the variability to date. Environmental factors are undoubtedly important, but have not been studied in depth, at least in part because of the difficulty of conducting these studies. We have analysed the role of climatic, environmental and temporal factors in determining the frequency of hospital admissions in children with SCD to 4 large sickle cell centres in London and Paris. Participants and Methods: Clinical data were collected from 1st January 2007 to 31st December 2012. Inclusion criteria were children with SCD (HbSS and HbSC) between the ages of 0 and 17 years, admitted to hospital with acute pain, acute chest syndrome or fever. All children lived within 4 miles radius (London) or 10km (Paris) of the hospital. Data were collected using specific electronic patient records of SCD patients. Data were collected on the reason for admission, date and length of admission. Daily air quality records were collected from sites around Paris and London, including details of black smoke, particulate matter, nitric oxide, carbon monoxide, sulphur dioxide and ozone. Daily meteorological records were obtained from weather stations in London and Paris including wind speed, temperature, rainfall and humidity. Statistical analysis including time series studies were conducted using R software version 3.1.1. Results: There were a total of 2717 admissions over the six year study period. Overall for the London hospitals there was a mean of 0.39 admissions/patient/year, with 1406 admissions for pain, 153 for acute chest syndrome and 417 for fever. The rate of admission/patient/year by cause for HbSS and HbSC across the London hospitals is shown in table below: Table 1. Rates of admission/patient/year by cause Sickle genotype/cause of admission All London hospitals Institution A Institution B Institution C HbSS (Pain) 0.31 0.18 0.40 0.43 HbSS (Fever) 0.09 0.03 0.15 0.11 HbSS (acute chest syndrome) 0.04 0.03 0.04 0.04 HbSC (pain) 0.07 0.03 0.08 0.10 HbSC (fever) 0.03 0.01 0.04 0.05 HbSC (acute chest syndrome) 0.004 0.008 0.002 0.002 Overall admission numbers were significantly higher on Mondays and Tuesdays in London but there was no such variation in Paris (Table 2). Table 2. Mean number of admissions on days of week in Paris (1 hospital) and London (3 hospitals). ** denotes significant difference from mean of other days (P<0.001). London Paris Weekday Monday 0.75** 0.35 Tuesday 0.77** 0.36 Wednesday 0.66 0.36 Thursday 0.64 0.32 Friday 0.60 0.32 Saturday 0.51 0.20 Sunday 0.57 0.27 There was no seasonal variation in admission numbers in London, but significantly higher numbers of patients admitted in Paris during autumn and winter. Table 3. Mean number of seasonal admissions in Paris (1 hospital) and London (3 hospitals). ** denotes significant difference from mean of other days (P<0.001). London Paris Season Autumn 0.70 0.35** Spring 0.60 0.31 Summer 0.64 0.25 Winter 0.62 0.34** Conclusion In London, there is a 2-3 fold variation in admission rates for the same complications between different hospitals. Similarly there is a significant difference on the effects of season and weekday between Paris and London. These results are statistically stronger than many effects which are identified in genetic and therapeutic studies, and show the importance of environmental and cultural factors, which are potentially modifiable. The effect of weather and pollution on hospital admissions is currently being analysed. Disclosures No relevant conflicts of interest to declare.


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