The Impact of Local Air Quality on the Number of Hospital Admissions with Acute Pain in Sickle Cell Disease within an Urban Environment.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 3790-3790
Author(s):  
Deborah Yallop ◽  
Edward R. Duncan ◽  
Ellie Norris ◽  
Gary Fuller ◽  
Nikki Thomas ◽  
...  
Keyword(s):  
Air Quality ◽  
Sickle Cell Disease ◽  
Environmental Factors ◽  
Sickle Cell ◽  
Acute Pain ◽  
Hospital Admissions ◽  
Clinical Severity ◽  
Cell Disease ◽  
Low Levels ◽  
The Impact

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.

scholarly journals The associations between air quality and the number of hospital admissions for acute pain and sickle-cell disease in an urban environment

2007 ◽  
Vol 136 (6) ◽  
pp. 844-848 ◽  
Author(s):  
Deborah Yallop ◽  
Edward R. Duncan ◽  
Elizabeth Norris ◽  
Gary W. Fuller ◽  
Nikki Thomas ◽  
...  
Keyword(s):  
Air Quality ◽  
Sickle Cell Disease ◽  
Urban Environment ◽  
Sickle Cell ◽  
Acute Pain ◽  
Hospital Admissions ◽  
Cell Disease

scholarly journals Associations between environmental factors and hospital admissions for sickle cell disease

Haematologica ◽  
2016 ◽  
Vol 102 (4) ◽  
pp. 666-675 ◽  
Author(s):  
Frédéric B. Piel ◽  
Sanjay Tewari ◽  
Valentine Brousse ◽  
Antonis Analitis ◽  
Anna Font ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Environmental Factors ◽  
Sickle Cell ◽  
Hospital Admissions ◽  
Cell Disease

Marijuana Use and Sickle Cell Disease

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 4826-4826 ◽  
Author(s):  
Vladimir K. Gotlieb ◽  
Khine Z. Oo
Keyword(s):  
United Kingdom ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Hospital Admissions ◽  
Pain Syndrome ◽  
Marijuana Use ◽  
Clinical Severity ◽  
Cell Disease ◽  
Severe Nausea ◽  
One Year

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?

A Multicentre Study of Environmental Factors on the Severity of Sickle Cell Disease

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4841-4841
Author(s):  
Sanjay Tewari ◽  
Fred Piel ◽  
Valentine Brousse ◽  
Baba PD Inusa ◽  
Paul Telfer ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Environmental Factors ◽  
Sickle Cell ◽  
Hospital Admissions ◽  
Conflicts Of Interest ◽  
Acute Chest Syndrome ◽  
Electronic Patient Records ◽  
Cell Disease ◽  
Hospital Data ◽  
Significant Difference

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.

Feritility and Attitudes To Fertility In a Cohort Of Women With Sickle Cell disease In South East London

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 4686-4686
Author(s):  
Rachel Kesse-Adu ◽  
Ronwyn Cartwright ◽  
Nicky Thomas ◽  
Eugene Oteng-ntim ◽  
Jo Howard
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Clinical Severity ◽  
Fertility Treatment ◽  
In Vitro Fertilisation ◽  
Disease Phenotype ◽  
Cell Disease ◽  
The Mean ◽  
The Impact ◽  
Age Of Menarche

Introduction Women with Sickle Cell Disease (SCD) have been reported to have delayed menarche, increased rates of miscarriage & still birth. There is little evidence of the impact of SCD severity on fertility, or of patients’ attitudes towards their fertility. Methods Following IRB approval, an anonymised questionnaire was distributed to female patients over the age of 16 attending an SCD clinic. This included questions on menarche, fertility, reproductive history & clinical severity. We also surveyed attitudes to fertility & the role of fertility treatment. Results 101 women gave informed consent & returned the survey of whom 68 (67%) had sickle cell anaemia (HbSS), 31 (31%) had HbSC & 2 (2%) HbSß0 thalassaemia (HbSß0thal), the group mean age was 38yrs (range 17-74). 46 patients had severe disease phenotype (criteria for severity: ≥3 hospital admissions per year / ≥2 episodes of acute chest crisis/ ≥6 crisis at home per year or on Hydroxycarbamide (HU) therapy), 78% (36/46) had HbSS & 49%(2/46) HbSC. 49% (22/46) were on HU, of whom 19 (86%) had HbSS & 3 (14%) HbSC, the mean duration on HU was 4.7 years (range 2 weeks–15 years). 3 patients with HbSS, were on a chronic transfusion program, 1 as secondary stroke prevention & 2 for disease severity. Mean age of menarche was 14 yrs (range 10-19). HbSC patients’ mean age of menarche was 13yrs (range 10-16), HbSS patients mean age of menarche was 14 yrs (range 10-19). 30% (30/101), (24 with HbSS & 6 with HbSC), had never been pregnant. This group had a mean age of 28yrs (range 17-46), 1 never wanted children, 2 had previously been advised not to conceive, 1 was actively trying for a baby, & 1 patient was awaiting in vitro fertilisation therapy (IVF). There was no difference in the severity of sickle disease in this group compared to the group with pregnancies. 70%(71/101) reported a total of 211 pregnancies, resulting in 111 (52%) successful deliveries (SDs) i.e. a live child/children. Further evaluation of 175 (81%) pregnancies revealed 98 (56%) SDs, (5 sets of twins & 1 early neonatal death equating to 102 live births), 45 (26%) miscarriages, 27 (15%) terminations of pregnancy & 5 (3%) still births (pregnancy loss at > 24 weeks gestation). 52% (50/96) & 63% (47/75) of pregnancies in women with HbSS & HbSC resulted in SDs respectively, as did 25%(1/4) of pregnancies in HbSß0thal women. 74% (53/71) had at least 1 miscarriage (mean 1.7, range 1-7) there was no correlation between miscarriage & sickle genotype or age at pregnancy. 46% (20/46) & 63% (36/55) of patients with severe & milder disease phenotype had miscarriages respectively (p = 0.01). The mean age at termination of pregnancy (26yrs) was lower, compared to 30yrs for women who had SDs or miscarriage & stillbirth (31yrs). In total 41% (72/175) pregnancies were unplanned. 96% (26/27) of terminated pregnancies were unplanned, as were 32% (31/98) of SDs & 28% (15/50) of miscarriages. 28% (28/100) of responders were currently using contraception. 12 (16%) of 71 women reporting pregnancies had no children, they had a total of 21 pregnancies (mean 1.75, range 1-7), 8 (67%) had HbSS, 3 (25%) HbSC. 18% (18/101) thought sickle may have affected their fertility, 93% (93/101) reported having children as being important, this included 28 (93%) of 30 women who had reported no pregnancies. 62% (63/101) would consider prenatal diagnoses (PND) if their partner had sickle cell trait, 59% (60/101) would consider pre implantation diagnosis (PIGD) & 24% ( 24/101) would consider termination of an fetus with SCD. Discussion This study confirms that compared to the normal UK population, women with SCD, have a slightly increased rate of miscarriage & a significantly elevated rate of stillbirth 3% vs 0.005%. Menarche is delayed by a year in patients with HbSS & women with HbSC are more likely to have a successful pregnancy. Although 74% of the women had miscarriage & only 56% of pregnancies resulted in a successful delivery, the majority of women were unaware of the effect of SCD on fertility. Most were not currently using any form of contraception, the rate of unplanned pregnancy was lower at 41% than that in the normal UK population of 50%. These women report having a child as being very important to them, & though majority would consider PND & PIGD only a minority (24%) would terminate a foetus diagnosed with SCD. This study is limited by being retrospective & single centred, further multi-centre prospective studies are required to validate these results. Disclosures: Howard: Sangart: Membership on an entity’s Board of Directors or advisory committees.

Inpatient pain management in sickle cell disease

2019 ◽  
Vol 76 (23) ◽  
pp. 1965-1971 ◽  
Author(s):  
Stefanie M Zassman ◽  
Francis J Zamora ◽  
John D Roberts
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Acute Pain ◽  
Pain Control ◽  
Hospital Admissions ◽  
Healthcare Providers ◽  
Emergency Department Visits ◽  
Cell Disease ◽  
Usual Practice ◽  
Blood Disorder

Abstract Purpose A novel strategy for management of acute pain associated with sickle cell disease (SCD), referred to as the oral tier approach, is described. Summary SCD is an inherited blood disorder characterized by episodic acute pain known as vaso-occlusive crisis (VOC), which is the most common reason for emergency department visits and hospital admissions in patients with SCD; these patients are often treated with parenteral opioids on admission and then transitioned to oral opioids prior to discharge. In this report, experience with use of the oral tier approach in 3 patients with SCD hospitalized for management of VOC is reported. As per usual practice, acute pain was initially managed with parenteral opioids via patient-controlled analgesia (PCA). Once pain control was established, an oral tier was added. The oral tier consisted of 3 orders. The first order was for an oral opioid, to be administered every 3 hours on a scheduled basis; however, the patient could refuse 1 or more of these scheduled doses. Two additional orders specified that the patients could receive additional oral opioids in incremental doses for moderate (grade 4–7) or severe (grade 8–10) pain if appropriate. To facilitate transition to an oral regimen with which the patients might be discharged, they were encouraged to use oral opioids in preference to parenteral opioids. Opioid usage and average daily pain scores for the 3 patients are reported. Conclusion Healthcare providers can use the oral tier approach to facilitate rapid inpatient conversion from i.v. PCA to oral opioids while providing adequate pain control in patients with SCD who develop VOC.

Elective Cholecystectomy Decreases the Morbidity of Cholelithiasis and Cholecystectomy in Pediatric Sickle Cell Disease.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 1544-1544
Author(s):  
Emily F. Goodwin ◽  
Paige M. Ivey ◽  
Jeffrey D. Lebensburger ◽  
Roy P. McDonald ◽  
Thomas H. Howard
Keyword(s):  
Abdominal Pain ◽  
Sickle Cell Disease ◽  
Hospital Admission ◽  
Sickle Cell ◽  
Hospital Admissions ◽  
Conflicts Of Interest ◽  
Cell Disease ◽  
Surgical Preparation ◽  
The Impact ◽  
Elective Cholecystectomy

Abstract Abstract 1544 Poster Board I-567 PURPOSE Cholelithiasis frequently occur in sickle cell disease, are easily diagnosed by ultrasound, and are associated with hospital admissions for abdominal pain. Elective cholecystectomy is controversial in sickle cell disease despite small series which suggest that elective cholecystectomy decreases the morbidity of the procedure. Therefore we examined the impact of cholecystectomy on morbidities associated with cholelithiasis and cholecystectomy in sickle cell disease. METHODS: Records of 191 consecutive pediatric sickle cell patients with cholelithiasis who underwent cholecystectomy between January 1999 and May 2009 were retrospectively reviewed. The cholecystectomies were classified into 3 groups 1) elective: no pre-operative symptoms, cholelithiasis on screening ultrasound, pre-planned surgical preparation; 2) symptomatic: pre-operative symptoms of cholelithiasis on ultrasound, pre-planned surgical preparation; 3) emergent: hospitalization for acute cholecystitis symptoms, cholelithiasis on ultrasound, no pre-planned surgical preparation. We compared the morbidity of cholelithiasis and cholecystectomy by examining pre-operative hospitalizations, cholecystectomy hospitalization, and post-operative hospitalizations. RESULTS: Patients with sickle cell disease underwent a total of 191 cholecystectomies over a ten year period: 51 elective, 110 symptomatic, and 30 emergent. Patients who required an emergent cholecystectomy had a longer post-operative hospitalization time than elective cholecystectomy (7.3 vs 4.3 P< 0.001). Prior to hospitalization for the cholecystectomy, patients needing emergent and elective cholecystectomy had similar number of total of hospital admission days (5.2 vs 5.6 P=0.73). However, the emergent cholecystectomy population required more hospital admission days prior to surgery for abdominal pain than the elective patients (1 vs 0.37, P=0.01). After the cholecystectomy, emergent patients required more total hospital admission days (7.2 vs 2.9, P=0.002) and more admission days for abdominal pain (0.5 vs 0.2, P=0.049) than patients that underwent elective cholecystectomy. In 18 patients with the most severe abdominal pain (>2 inpatient admission days) prior to hospitalization for cholecystectomy, 11 (61%) were not admitted after cholecystectomy for pain. Patients receiving chronic blood transfusions prior to surgery had a reduced need for emergent cholecystectomy as compared to non-transfused patients (8% vs 25%, P= 0.056 by chi-square). Patients receiving hydroxyurea had a similar rate of need for an emergent cholecystectomy as compared to patients not on hydroxyurea (22% vs 13% P= NS). No differences in degree of anemia or reticulocytosis were identified in patients requiring emergent vs. elective cholecystectomy (Hb: 8.7 vs 8.6 g/dL; Reticulocyte percent: 10.9% vs 10.4%). The morbidity of patients in the symptomatic cohort was intermediate between the elective and emergent cohorts without demonstrating statistical significance. CONCLUSIONS: This represents the largest reported series of pediatric cholelithiasis and cholecystectomy in sickle cell disease to date. This data strongly suggests that elective cholecystectomy decreases morbidity associated with cholelithiasis and cholecystectomy in sickle cell disease. Disclosures No relevant conflicts of interest to declare.

Procedures, Pain, and Parents

PEDIATRICS ◽  
1991 ◽  
Vol 87 (4) ◽  
pp. 563-565
Author(s):  
HOWARD BAUCHNER
Keyword(s):  
Sickle Cell Disease ◽  
Lumbar Puncture ◽  
Sickle Cell ◽  
Acute Pain ◽  
Cell Disease ◽  
Emergency Rooms ◽  
Pharmacological Agents ◽  
The Past ◽  
The Subject

During the past decade certain types of pain in children have been the subject of much research and discussion. The pain associated with cancer, sickle cell disease, and the preoperative and post-operative periods have all been extensively studied and reviewed.1-4 Less information is available about acute pain inflicted in emergency rooms. Children commonly undergo procedures such as venipuncture, intravenous cannulation, lumbar puncture, and manipulation of fractures in emergency rooms without the benefit of any analgesia. What techniques are available to reduce the pain and anxiety that children feel when they undergo procedures? Traditionally, physicians have tried to reduce pain by using pharmacological agents.

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