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?

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 Medical Marijuana for Sickle Cell Disease: Results of Two Years of Certification in an Adult Sickle Cell Center

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 858-858 ◽  
Author(s):  
Susanna A Curtis ◽  
Dana Lew ◽  
Jonathan Spodick ◽  
John D. Roberts
Keyword(s):  
Chronic Pain ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Hospital Admissions ◽  
Medical Marijuana ◽  
Marijuana Use ◽  
Cell Disease ◽  
Opioid Use ◽  
Services Utilization ◽  
Admission Rates

Abstract Background: The hallmark of sickle cell disease (SCD) is pain, which can be both acute and chronic. Pain in patients with SCD leads to hospital admissions and diminishes quality of life. Pain in patients with SCD is primarily treated with opioid medications, which are often insufficient. In 2017 the Committee on the Health Effects of Marijuana of the National Academies of Science, Engineering, and Medicine reported that there is "conclusive or substantial evidence that cannabis or cannabinoids are effective for the treatment of chronic pain in adults", but the report made no mention of chronic pain in patients with SCD. Currently, 30 states and Washington D.C. have medical marijuana (MM) laws, and 5 states list SCD as a qualifying condition. SCD was added to the list of qualifying conditions in Connecticut in February 2016. Since then we have offered certification to patients in our program who make regular clinic visits and for whom we think MM will be safe. However, not all patients who are certified go on to complete the state regulatory paperwork and obtain MM. We hypothesized that those who had obtained MM would show an improvement in clinical outcomes compared to those patients who had been certified but not obtained MM. Methods: All patients who requested certification were educated on safety risks of MM. We conducted a review of all certified patients using our electronic medical record and the Connecticut Prescription Monitoring Program, which provides dispensing reports for schedule I-IV medications including MM. Our primary outcome was admissions in the 6 months after compared to the 6 months before obtaining MM, or the date certified for those who did not obtain MM. Our secondary outcomes were acute care services utilization (emergency department and outpatient infusion center visits) for the treatment of acute pain, and opioid use. Baseline hospital admissions, acute services utilization, and daily opioid use were defined as use in the 6 months prior to obtaining MM or certification for those who did not obtain MM. Opioid use was calculated as total oral morphine equivalents (OME) dispensed in a 6-month period and expressed as OME per day. Previous marijuana use was defined as 1 or more urine studies positive for cannabinoids before MM certification. Genotype was divided into more clinically severe (HbSS/Hbβ0) or less severe (HbSC/Hbβ+/Persistent HbF). We compared admissions, acute services, and opioid use for those who obtained MM to those who did not using a difference in differences analysis. Baseline admissions, baseline acute services, baseline opioid use, age, gender, genotype, hydroxyurea use, previous marijuana use, and insurance type of those who obtained MM were compared to those who did not using Student's t tests. Results: 52 patients requested certification, and 50 patients were certified. 2 patients were not certified due to concerns about inappropriate use based on their past history. Twenty-eight patients who were certified obtained MM; 22 did not. Baseline hospital admissions, acute services utilization, and daily opioid were similar between the two groups. Patients who obtained MM were more likely to be genotype HbSS/HbSβ0. Age, gender, hydroxyurea use, previous marijuana use, and insurance type were similar between the two groups. (Table 1). Patients obtained MM a median of 109.5 days (IQR 54.8 - 188.8) after certification. Two patients concurrently started L-Glutamine within 6 months of MM certification, one who obtain MM and one who did not. Obtaining MM was associated with a decrease in admission rates in the next 6 months compared to those who did not obtain MM (-1.1 admissions 95% CI -0.1 - -2.0, p=0.03). Obtaining MM was not associated with a significant change in acute services utilization (0.3 visits 95% CI -1.4 - 1.9, p=0.8) or daily opioid use (-0.1 OME 95% CI -19.6 - 19.3, p= 1.0). Conclusion: Though there was no difference in admission rates between the two groups we examined prior to MM certification, and the only clinical difference was that those who obtained MM were more likely to be in the moresevere genotype, those who obtained MM showed a decrease in admissions in the next 6 months compared to those who did not. We suggest that MM allowed patients to improve their pain relief and thereby reduce admission rates. This is consistent with data showing cannabinoid agonists improve pain in murine models of SCD. Larger, randomized and controlled studies of MM for pain in SCD should be conducted. Table 1. Table 1. Disclosures No relevant conflicts of interest to declare.

scholarly journals Use of Marijuana in Patients with Sickle Cell Disease Increased the Frequency of Hospitalization for Acute Painful Vaso-Occlusive Crises

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 2498-2498
Author(s):  
Samir K. Ballas
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Drug Screening ◽  
Marijuana Use ◽  
Clinical Severity ◽  
Questionnaire Study ◽  
Cell Disease ◽  
Significant Difference ◽  
Urine Drug ◽  
Urine Drug Screening

Introduction : Recently, there has been general interest in using marijuana as an analgesic for various types of pain. Cannabis is known to relieve severe nausea and vomiting. Anecdotally, medical marijuana has analgesic effects in patients with chronic and neuropathic pain other than sickle cell pain, decreases the frequency of migraine headache, has anti-spastic effect in multiple sclerosis, and may relieve some of the signs and symptoms of amyotrophic lateral sclerosis and Crohn's disease. Its role in epilepsy is controversial. On the negative side long-term cannabis use is associated with periodontal disease, maternal marijuana use at 20 weeks gestation was strongly associated with spontaneous pre-term birth independent of maternal cigarette smoking and illegal synthetic cannabinoids (K2/spice) were associated with catatonia and myocardial infarction in adolescents. Studies in the transgenic sickle cell mouse showed that cannabinoid receptor-specific mechanisms ameliorate pain via inhibition of mast cell activation and neurogenic inflammation. A questionnaire study from the United Kingdom showed that 36% of the patients with sickle cell disease (SCD) have used cannabis in the previous 12 months to relieve symptoms. Another longitudinal questionnaire study in Jamaica found that marijuana was not related to the clinical severity of SCD. The objective of this study is to report the epidemiological and clinical features of the patients with SCD who were found to be taking marijuana by using random urine drug screening from 1994 to 2009. The frequency of vaso-occlusive crises (VOCs) in these patients was compared to patents with SCD whose urine drug screening showed no marijuana. The terms marijuana and cannabinoid will be used interchangeably in this Abstract. Patients & Methods: A total of 270 random urine drug screen tests were done on 72 patients representing 10% of our patients with SCD during the period of the study. Written consent was obtained from the patients for enrollment in the study that was approved by the IRB. Types of SCD were determined by routine lab tests. Urine samples, collected randomly, were analyzed for the presence of amphetamine, benzodiazepines, opiates, barbiturate, cannabinoid, propoxyphene, methadone and phencyclidine. Samples were classified as either positive or negative for cannabinoid. Statistical analysis included the two-tailed student's t, Fisher exact and the Chi Square tests. Results: Thirty-seven of the 72 patients tested positive for cannabinoid in their urine and 35 patients tested negative. Table 1 shows the major epidemiological and clinical feature of the patients studied. There was no significant difference in ages of men and women who used or did not use marijuana (p > 0.05). However, males who used marijuana were significantly younger (p < 0.001) than males who did not (Table 1). Moreover, the ages of females who tested positive for cannabinoid were not significantly different from women who tested negative (Table 1). The patients who tested positive for cannabinoids also tested positive for other illicit drugs more often than patients who tested negative. Positivity for benzodiazepines, cocaine and phencyclidine were significantly higher in patients who used marijuana than the nonusers. Notably, there was no significant difference in the use of opioids consumed by users and non-users of marijuana. Similarly, there was no significant difference in the frequency of complications of SCD between users and non-users of marijuana (Table 1). The most important aspect of the study is the effect of marijuana use on the frequency of utilization of medical facilities. Patients in the marijuana cohort were admitted to the hospital for VOCs more frequently (Table 1) than the control group: 2,443 versus1,602 admissions respectively (p < 0.05). Conclusion: Together these data suggest that patients in the marijuana cohort were more often in severe pain that required treatment in the hospital for VOCs than non-users. This difference could be due to the quality of the marijuana used, the neuronal effects of marijuana and the severity of pain in the users of marijuana. The role of marijuana in the treatment of sickle cell pain seems more complex than thought. Further controlled trials are needed to clarify this issue. Table 1 Table 1. Disclosures No relevant conflicts of interest to declare.

scholarly journals Screening for ophthalmic manifestations of sickle cell disease in the United Kingdom

Eye ◽  
1994 ◽  
Vol 8 (6) ◽  
pp. 618-622 ◽  
Author(s):  
D Kent ◽  
R Arya ◽  
W A Aclimandos ◽  
A J Bellingham ◽  
A C Bird
Keyword(s):  
United Kingdom ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Ophthalmic Manifestations ◽  
The United Kingdom

scholarly journals Prevalence and Predisposing Factors of Atrial Fibrillation in a Multi-Ethnic Society: The Impact of Racial Differences in Bahrain

2011 ◽  
Vol 4 ◽  
pp. OJCS.S8032 ◽  
Author(s):  
Taysir Garadah ◽  
Saleh Gabani ◽  
Mohamed Al Alawi ◽  
Ahmed Abu-Taleb
Keyword(s):  
Atrial Fibrillation ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Middle Eastern ◽  
Predisposing Factors ◽  
Cell Disease ◽  
Clinical Events ◽  
History Of ◽  
The Mean ◽  
One Year

Background The prevalence and epidemiological data of atrial fibrillation (AF) among multi-ethnic populations is less well studied worldwide. Aim Evaluation of the prevalence and predisposing factors of AF in patients who were admitted to acute medical emergencies (ER) in Bahrain over the period of one year. Methods Two hundred and fifty three patients with onset of AF were studied. The mean difference of biochemical data and clinical characteristics between Middle Eastern (ME) and sub continental (SC) patients was evaluated. The odds ratio of different predisposing factors for the development of clinical events in AF patients was assessed using multiple logistic regression analysis. Results Out of 7,450 patients that were admitted to ER over one year, 253 had AF based on twelve leads Electrocardiogram (ECG), with prevalence of 3.4%. In the whole study, the mean age was 59.45 ± 18.27 years, with 164 (65%) male. There were 150 ME patients (59%), and 107 (41%) SC, 55 (22%) were Indian (IND) and 48 (19%) were South Asian (SA). In the whole study clinical presentation was of 48% for palpitation, pulmonary edema was of 14%, angina pectoris on rest of 12%, 10% had embolic phenomena, 6% had dizziness, and 7% were asymptomatic. The odds ratio of different variables for occurrence of clinical events in the study was positive of 2.2 for history of hypertension, 1.8 for sickle cell disease, 1.2 for high body mass index (BMI) >30, 1.1 for mitral valve disease. The ME patients, compared with SC, were older, had significantly higher body mass index, higher history of rheumatic valve disease, sickle cell disease with high level of uric acid and lower hemoglobin. The history of hypertension, DM and smoking was higher among the SC patients. The rate of thyroid disease was equal in both groups. Conclusion The prevalence of atrial fibrillation was 3.4% with male predominance of 65%. Patients of sub continental origin were younger with a significantly high history of hypertension and ischemic heart disease. The patients of Middle Eastern origin had significantly high rate of rheumatic heart disease, and sickle cell disease. The history of hypertension was the most important independent clinical predictor of adverse events in patients presented with AF.

scholarly journals Salmonella osteomyelitis in a one year old Child without Sickle Cell Disease: A Case report

2014 ◽  
Vol 8 (2) ◽  
pp. 52-54 ◽  
Author(s):  
Saturveithan C ◽  
Arieff A ◽  
Premganesh G ◽  
Sivapathasundaram N
Keyword(s):  
Case Report ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
One Year ◽  
Salmonella Osteomyelitis

scholarly journals Risk-Score Based Strategy to Minimize Antibiotic Exposure in Children With Sickle Cell Disease and Fever

2021 ◽  
Author(s):  
Elena Maria Rincón-López ◽  
María Luisa Navarro Gómez ◽  
Teresa Hernández-Sampelayo Matos ◽  
David Aguilera-Alonso ◽  
Eva Dueñas Moreno ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Risk Score ◽  
Bacterial Infections ◽  
Hospital Admissions ◽  
Empirical Therapy ◽  
Antibiotic Exposure ◽  
Cell Disease ◽  
Optimal Score ◽  
A Prospective Study

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.

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

2019 ◽  
Vol 16 (15) ◽  
pp. 2724
Author(s):  
Marie-Claire Parriault ◽  
Claire Cropet ◽  
Aniza Fahrasmane ◽  
Stéphanie Rogier ◽  
Michaël Parisot ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
French Guiana ◽  
Hospital Admissions ◽  
Weather Conditions ◽  
Therapeutic Education ◽  
Targeted Prevention ◽  
Prevention Measures ◽  
Cell Disease ◽  
Emergency Visits

(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.

Changing Pattern of Hospital Admissions of Children With Sickle Cell Disease Over the Last 50 Years

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

Severity of Sickle Cell Disease: Modeling Interrelationships among Hemolysis, Pulmonary Hypertension and Risk of Death.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 786-786
Author(s):  
Paola Sebastiani ◽  
Vikki G. Nolan ◽  
Clinton T. Baldwin ◽  
Maria M. Abad-Grau ◽  
Ling Wang ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Sickle Cell Disease ◽  
Disease Severity ◽  
Hemolytic Anemia ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Clinical Severity ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Risk Of Death

Abstract A single point mutation in the β hemoglobin gene causes sickle cell disease (SCD), but patients have extremely variable phenotypes. Hemolysis-related complications include pulmonary hypertension (PHT), priapism, stroke and leg ulceration; blood viscosity and sickle vasoocclusion are associated with painful episodes, acute chest syndrome and osteonecrosis. Predicting who is at highest risk of death would be useful therapeutically and prognostically. Applying Bayesian network modeling that describes complex interactions among many variables by factorizing their joint probability distribution into modules, to data from 3380 SCD patients, we constructed a disease severity score (DSS: 0, least severe; 1, most severe), defining severity as risk of death within 5 years. A network of 24 variables described complex associations among clinical and laboratory complications of SCD. The analysis was validated in 140 patients whose SCD severity was assessed by expert clinicians and 210 adults where severity was also assessed by the echocardiographic diagnosis of PHT and death. Information about PHT allowed a comparison of the DSS with the tricuspid regurgitant jet velocity (TRJV), an objective marker of PHT and an independent risk factor for death. DSS and three indices of clinical severity (severity ranking of individuals by expert clinicians; objective measurement of the presence and severity of PHT; risk of prospective death) were correlated. Among living subjects, the median score was 0.57 in 135 patients without PHT, 0.64 in 40 patients with mild PHT and 0.86 in 15 patients with severe PHT. The difference in average score between living patients with and without PHT is significant. The same increasing trend was noticeable in the subjects who died during follow-up: 0.60 in subjects without PHT; 0.68 in subjects with mild PHT; 0.79 in subjects with severe PHT. The utility of the DSS is also supported by the ability to assign a score to subjects for whom the TRJV cannot be measured. Surprisingly, besides known risk factors like renal insufficiency and leukocytosis, we identified the intensity of hemolytic anemia and clinical events associated with hemolytic anemia as contributing to risk for death. Priapism, an excellent reflection of the hemolytic anemia-related complications of SCD, is associated with PHT and its association with death was unexpected. Laboratory variables predictive of disease severity included LDH and reticulocytes that reflect the intensity of hemolytic anemia. Elevated systolic blood pressure increased the odds of death by 3.4, consistent with hypertension as a marker of early death in SCD. Subjects with sickle cell anemia are at greatest risk compared with subjects with sickle cell anemia-α thalassemia and with subjects with HbSC disease. Our model suggests that the intensity of hemolytic anemia, estimated by LDH, reticulocyte count and AST, and shown previously to be associated with PHT, priapism, leg ulceration and possibly stroke, is an important contributor to death. This model can be used to compute a personalized measure of disease severity that might be useful for guiding therapeutic decisions and designing clinical trials.

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