scholarly journals Migration: The aftershocks to the provision of healthcare

2018 ◽  
Vol 8 (1) ◽  
Author(s):  
Stephan Lobitz
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Forced Migration ◽  
Chronically Ill ◽  
Sudden Increase ◽  
Cell Disease ◽  
Host Countries ◽  
Number Of Patients ◽  
Short Period ◽  
The World

Migration is the “movement of people to a new area or country in order to find work or better living conditions” (Oxford dictionary). The term “migration” summarizes forced, reluctant and voluntary migration. Voluntary migration is a comparatively constant event. But reluctant and, in particular, forced migration have been subject to substantial change during the last years. At the end of 2016, more than 17.2 million refugees (+ 5.3 million Palestinians) were on the run outside their home countries. 55% of them fled from Syria (5.5 million), Afghanistan (2.5 million) and South Sudan (1.4 million), respectively. The top hosting countries were not, in fact, the Southern and Western European or North American, but some of the poorest countries in the world. With the refugees from countries where disorders of haemoglobin are very prevalent, the number of patients in the host countries significantly increased within a very short period of time. The extraordinary circumstances required rapid rethinking and adaption and, therefore, did not only pose a big challenge but, in some countries, also a big chance to improve care for patients suffering from hemoglobinopathies. Although there are certainly several trouble spots in the world, the Middle East crisis was and still is currently the most prominent one. There is a significant prevalence of thalassemia and sickle cell disease among the Syrian and Iraqi population and since the chronically ill were presumably those who left their home countries first, there was a dramatic increase in the prevalence of thalassaemia and sickle cell disease in the host countries. Many patients fled to Western and Northern European countries where hemoglobinopathies were very rare and where the healthcare systems were unable to cope with this sudden increase in patient numbers and complications. For example, disease characteristics were much more pronounced than doctors were used to. Complications occurred that physicians only knew from textbooks. In addition, virtually all families needed significant help in psychosocial matters and many refugees were severely traumatized.

scholarly journals Sickle Cell Anemia Presenting with Vaso-Occlusive Pain: Should We Screen for COVID-19?

2021 ◽  
pp. 1-4
Author(s):  
Mohammad Ali ◽  
Lina Okar ◽  
Nabil E. Omar ◽  
Jabeed Parengal ◽  
Ashraf Soliman ◽  
...  
Keyword(s):  
High Risk ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Signs And Symptoms ◽  
Risk Groups ◽  
Position Statement ◽  
Interesting Case ◽  
International Federation ◽  
Cell Disease ◽  
The World

Despite the widespread of coronavirus disease-19 (CO­VID-19) infection around the world, there are very scarce reported literature about the care of patients with a known diagnosis of hemoglobin disorders such as sickle cell disease (SCD) or thalassemia and confirmed COVID-19 infection. Thalassemia International Federation issued a position statement to include patients with thalassemia and SCD among the high-risk groups of patients. Here, we present an interesting case of a 42-year-old patient know to have SCD presenting with Vaso-occlusive (VOC) pain episode in the absence of COVID-19 signs and symptoms, who tested positive for COVID-19 infection and had a smooth recovery. This case highlights the importance of screening SCD patients presenting with VOC-related events even in the absence of COVID-19 signs and symptoms.

Experience of an Interdisciplinary Pediatric Pain Service

PEDIATRICS ◽  
1991 ◽  
Vol 88 (6) ◽  
pp. 1226-1232
Author(s):  
Barbara S. Shapiro ◽  
David E. Cohen ◽  
Kenneth W. Covelman ◽  
Carol J. Howe ◽  
Sam M. Scott
Keyword(s):  
Sickle Cell Disease ◽  
Patient Care ◽  
Sickle Cell ◽  
Health Care Professionals ◽  
Tertiary Care ◽  
Direct Patient Care ◽  
Team Approach ◽  
Cell Disease ◽  
Number Of Patients ◽  
Physician Time

This article is a report of our experience with an interdisciplinary pain service in a large tertiary care pediatric hospital. During the first 2 years of operation, we received 869 consultations and referrals from more than 19 hospital divisions. Postoperative pain was the most frequent reason for consultation (56% of patients). Patients with pain related to cancer and sickle cell disease comprised 25% of the consultations. The remaining patients had a wide variety of primary diagnoses and causes of pain. We calculated the time spent by pain service physicians in direct patient care. The majority (63%) of physician time was spent with a small number of patients (17%). Most of these patients had pain that was unrelated to surgery, cancer, or sickle cell disease, and many posed dilemmas in diagnosis and treatment. Physician time was correlated directly to the use of psychologic and physical therapies for the pain, involving multiple team members. This experience supports the demand for an interdisciplinary pain service in a tertiary care children's hospital. A significant amount of physician time is necessary to provide patient care and to maintain a team approach, however, and pediatricians and other health care professionals who aim to implement such services should be cognizant of the time required.

scholarly journals Advancing Healthcare Outcomes for Sickle Cell Disease in Nigeria Using Mobile Health Tools

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 2173-2173
Author(s):  
Arwa Fraiwan ◽  
Muhammad Noman Hasan ◽  
Ran An ◽  
Amy J. Rezac ◽  
Nicholas J. Kocmich ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Mobile Health ◽  
Sub Saharan Africa ◽  
Diagnosis And Treatment ◽  
Test Results ◽  
Cell Disease ◽  
The World ◽  
Equity Ownership ◽  
Sub Saharan

Nigeria leads the world in the number of cases of sickle cell disease (SCD). An estimated 150,000 babies are born annually in Nigeria with SCD, a heredity disorder, and 70-90% die before age 5. Only a small portion of affected infants and children in sub Saharan Africa (SSA) reach adolescence. Over 650 children die per day in sub-Saharan Africa from SCD. These dismal statistics are in sharp contrast to outcomes in high-income countries (HICs) where more than 90% of SCD patients reach adulthood. The World Health Organization (WHO) estimates that 70% of deaths could be prevented with a low cost diagnostic and treatment plan. Meaningful preventive care and treatment cannot be implemented without a structured plan for early diagnosis and patient tracking.Early diagnosis requires improved access to parents and guardians of children with SCD, and gaining this access remains a challenge in most of SSA. In 2015, Nigeria's Kano state government, with support from foreign partners, established a community-based program for newborn registration. This platform provides unique access to newborn babies in one of Nigeria's most populous cities, but still lacks a functioning patient testing, tracking, and monitoring system, which we plan to address in our ongoing study. This study will introduce mobile health in a low-income country with low literacy rate and hopefully accustom that segment of the population to more varied mobile health applications that will ultimately improve their health in the long run. Our current operational platform in Kano, Nigeria provides access to a large population with a high prevalence of SCD. We have previously completed pilot testing of 315 subjects for SCD using our microchip electrophoresis test. We are planning to test up to 4,500 additional subjects less than 5 years of age at Murtala Muhammed Specialist Hospital. The hospital staff includes 97 physicians and 415 nurses and outpatient clinics serve about 30,000 patients monthly. The maternity department has a 200-bed capacity and the antenatal clinic performs about 1,000 deliveries and serves an average of 3,000 mothers monthly. Enrollment is planned to start on September 15, 2019 and medical staff are currently being trained to run the tests. Our study is registered in the United States National Library of Medicine's ClinicalTrials.gov (Identifier: NCT03948516). Our technology is uniquely paired with an automatic reader and an Electronic Medical Record (EMR) and patient management solution to record POC test results, register new cases, and track patients for follow-up (Fig. 1). The reader enables automated interpretation of test results, local and remote test data storage, and includes geolocation (Global Positioning System) (Fig. 2). The system will generate reports for all cases of SCD, track hospital visits, appointments, lab tests, and will have mobile and dashboard applications for tracking patients and samples. The application will be installed on mobile devices provided to users. The proposed system will be compliant with the existing privacy standards to handle medical data (e.g., HIPAA in the US and GDPR in the EU). All communications between the parties will be secured via end-to-end encryption as a safeguard. We anticipate that our project will increase the rates of screening, diagnosis and timely treatment of SCD in Kano State of Nigeria. The project's broader impact will likely be the ability to track and monitor screening, disease detection, diagnosis and treatment, which can be scaled up to the whole nation of Nigeria, then to sub-Saharan Africa. The data obtained and analyzed will be the first of their kind and will be used to inform the design of programs to improve access to, and availability of, effective care for this underserved populations. The importance of increased access to diagnosis and treatment should not be underestimated - it is crucial for realizing effective management of people with SCD. The impact can be enhanced by complementing diagnosis and patient tracking with education for the families so they can provide or seek the necessary preventative treatment. Identification of the location of the patients in need would help identify the areas where family, parent, caregiver education should be provided. Disclosures Fraiwan: Hemex Health, Inc.: Equity Ownership, Patents & Royalties. Hasan:Hemex Health, Inc.: Equity Ownership, Patents & Royalties. An:Hemex Health, Inc.: Patents & Royalties. Thota:Hemex Health, Inc.: Employment. Gurkan:Hemex Health, Inc.: Consultancy, Employment, Equity Ownership, Patents & Royalties, Research Funding.

Sickle cell disease

2007 ◽  
pp. 249-252
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Middle Eastern ◽  
Cell Disease ◽  
Equatorial Africa ◽  
The World ◽  
Homozygous Form

Sickle cell disease 250 This is one of the most common inherited conditions in the world and affects predominantly people from equatorial Africa but also those of Mediterranean, Indian, and Middle-Eastern descent. It is recessively inherited and in the homozygous form (HbSS) causes a multi-organ disorder....

Adjuvant interventions with opioids for vaso-occlusive crisis in sickle cell disease: A mixed treatment network meta-analysis of randomized controlled clinical trials

2020 ◽  
Vol 16 (4) ◽  
pp. 267-275
Author(s):  
Kannan Sridharan, MD, DM ◽  
Gowri Sivaramakrishnan, MDS
Keyword(s):  
Nitric Oxide ◽  
Sickle Cell Disease ◽  
Adverse Events ◽  
Magnesium Sulfate ◽  
Sickle Cell ◽  
Meta Analysis ◽  
Favorable Effect ◽  
Secondary Outcome ◽  
Cell Disease ◽  
Number Of Patients

Objective: Vaso-occlusive crisis is the most common clinical feature requiring opioid analgesics in patients with sickle cell disease. We conducted a network meta-analysis to compare the drugs that can be used as add-on with opioids for vaso-occlusive crisis.Design: Network meta-analysis of randomized clinical trials.Patients: Sickle cell disease patients with vaso-occlusive crisis receiving adjuvants to opioids for pain management.Main outcome measures: A number of patients with complete pain relief and pain scores assessed either by visual analog or by a numerical rating scale were the primary outcomes. Adverse events and dose of opioids (in morphine equivalents) for pain alleviation between the treatment arms were the secondary outcome measures.Results: Eleven studies evaluating the addition of ketorolac, magnesium sulfate, ketoprofen, ibuprofen, methadone, inhalational nitric oxide, methylprednisolone, and arginine with morphine were obtained. The pooled analysis showed a favorable effect in the pain reduction for the additions of arginine {–2 [–3.39, –0.61]} and ibuprofen {–1.7 [–3.26, –0.14]} with morphine. Arginine has high probability of being the “best” in the pool followed by ibuprofen. No significant differences were observed in the risk of adverse events {ketoprofen—0.84 [0.42, 1.65]; magnesium sulfate—1.81 [0.64, 5.81]; and arginine—2.08 [0.18, 24.31]}. A significant lower dose of opioid was required when given adjunctive to arginine, inhalational nitric oxide, and methylprednisolone.Conclusion: We observed that arginine and ibuprofen could produce additional analgesic effects when combined with morphine in vaso-occlusive crisis.

Does Treatment with Hydroxyurea and Opioids Affect Age of Death in Sickle Cell Disease Patients?

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 4808-4808
Author(s):  
Charlene M. Flahiff ◽  
Jude C Jonassaint ◽  
Charles R. Jonassaint ◽  
Soheir S. Adam ◽  
Marilyn J. Telen ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Treatment Modality ◽  
Group Analysis ◽  
Treatment Modalities ◽  
Age At Death ◽  
Cell Disease ◽  
Number Of Patients ◽  
Living Group ◽  
Age Of Death

Abstract Hydroxyurea (HU) is used to treat sickle cell disease and has been shown to decrease painful episodes (Charache, 1995) and possibly vaso-occlusive episodes associated morbidity and mortality (Steinberg, 2003). Opioids are often prescribed in adult patients for daily management of their chronic pain. The aim of the current study was to determine the age at death and the effect of treatment with HU and/or opioids prior to death in patients who died from sickle cell disease (SCD) related complications and to compare these parameters to those in our current patients population. Methods: Age, daily treatment with opioids, and HU treatment were determined for 185 patients currently followed at the Duke Comprehensive Sickle Cell Center (DCSCC) and for 50 patients who died between 2002 and 2008 due to SCD complications and who were regularly followed at the DCSCC for their care. The two cohorts, living and deceased patients were divided based on their treatment modality into the following 4 groups: opioid only, HU only, both drugs, and neither drug. Non-parametric chi-square test was performed to determine whether the treatment modality distribution was different in the deceased group compared to the living group. Analysis of variance was done to determine the relationship between treatment group and age at death. Results and Discussion: The distribution of treatment modalities in the deceased group was significantly different than that of the living group. The opioids only group had the largest number of patients in the deceased cohort (44%), and this percentage was almost twice that of the living group (25%). (Fig. 1) Moreover, 72 % of the deceased patients were treated with opioids vs. only 53 % of the living patients, perhaps because the sicker patients are often treated with daily opioids. However, the age of death in the opioids only group was 44 ± 15.5 years. (Fig. 2) In the living group, treatment with both drugs or with no drugs were equivalent (28%), while in the deceased group, more patients were treated with both drugs (28%) compared to no drug treatment (18%). The HU only group had the lowest number of deaths (10%), and the percentage of patients in this group was nearly half that the one of the living group (19%). This group also was the oldest at death (58 ±16 years). Age at death was also significantly higher in this group than in each of the other 3 groups (p<0.05). The low use of HU in the deceased cohort, along with the higher age of death, support the reported effectiveness of this drug in reducing morbidity associated with SCD. The age of the living patients receiving treatment with both drugs was the same as that of the deceased. Similarly the age of the non treated living patients was comparable with that of the deceased group. Interestingly, the interaction of the 2 therapeutic interventions (HU and opiates) had a significant effect on the age at death (p=0.003). We conclude that opioid treatment, either alone or in conjunction with HU, appears to have a significant effect on the age at death and warrants further investigation.

The Complex Relationship Between Sickle Cell Disease and Depression.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 2585-2585 ◽  
Author(s):  
Soheir S. Adam ◽  
Charlene Flahiff ◽  
Mary R Abrams ◽  
Marilyn J. Telen ◽  
Laura M. De Castro
Keyword(s):  
Chronic Pain ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Chronically Ill ◽  
Neurocognitive Function ◽  
Clinical Depression ◽  
Current Therapy ◽  
Cell Disease ◽  
Severity Scores ◽  
Prevalence Of Depression

Abstract Abstract 2585 Poster Board II-561 A high prevalence of depression has been described in both chronic diseases and diseases associated with chronic pain. Sickle cell disease (SCD) is a congenital and lifelong complex hematological illness in which both acute and chronic pain are described as hallmarks. Depression prevalence has also been reported as high in SCD. Clinical depression may lead to decreased compliance with prescribed medical treatments, and thus deteriorating function and health status. Furthermore, depressed patients report more frequent painful episodes. Pain and depression can also both have negative effects on health-related quality of life (QoL) measures in a chronically ill population. Methods: We performed an analytic epidemiologic prospective study to determine the prevalence of depression (as measured by the Beck Depression Inventory [BDI]) in 70 adult SCD patients at baseline (no pain episodes within 30 days) who receive their SCD disease-related care primarily from our clinic. We also assessed the association between the prevalence of depression, QoL, and severity scores measuring end organ damage as previously described (Afenyi-Annan et al. 2008). To measure mental and physical QoL domains, patients were administered the SF36 QoL scale. A short computerized test, “CNS Vital Signs,” was used to assess patients' neurocognitive function. Pain diaries were used to determine the use of short-term and long-term narcotics. Results: The sample included 38 females and 32 males, ages 19 – 76 years (mean 36). Mean severity score was 1.61 (SD 1.1; range 0–4). Nineteen patients (27%) had clinical depression by BDI. Nine of them (47%) were classified as severe. The gender ratio was 2:1 F:M. Patients with depression were significantly older (mean age 39.8 vs. 35.0 yrs, p<0.05). The ratio of hemoglobin SS versus other sickle-related hemoglobinopathies was 1:1 in those with depression versus 2:1 in those without depression. Severity scores were not statistically different between those with or without depression (1.8 ±1.1 vs. 1.5±1.2). Nineteen of 51 patients (37%) without depression had a prior history of central nervous system events, while only 4 of 19 (21%) with depression did; this difference was not statistically significant. Similarly, no statistical difference in neurocognitive function was noted between patients with and without depression, when tested for the following 5 domains: memory, psychomotor speed, reaction time, complex attention, and cognitive flexibility. Both the physical and mental domains of the QoL testing showed significantly lower scores for those patients with depression when compared with those without depression (p=0.007 and p<0.0001, respectively). For the mental domain, there was also a statistically significant inverse correlation between the level of depression and domain score. Lastly, neither current therapy with long-acting narcotics or antidepressive medications showed association with the presence of depression. Summary: We conclude that there is likely a complex and thus far poorly understood interaction between multiple SCD cofactors and the presence of depression in this patient population. QoL rather than disease severity is the measure most strongly related to depression in our study. Disclosures: No relevant conflicts of interest to declare.

POST Marketing Observational Study of Children (6 years or older) Treated with Deferasirox (Exjade ICL 670)-One Year analysis

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 5295-5295
Author(s):  
Nagina Parmar ◽  
Manroop Singh ◽  
Isaac Odame ◽  
Melanie Kirby
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Patient Reported Outcomes ◽  
Chelation Therapy ◽  
Descriptive Statistics ◽  
Cell Disease ◽  
Outcomes Measures ◽  
Short Period ◽  
Patient Reported

Abstract Abstract 5295 In 2006, deferasirox was licensed in Canada for use in children over the age of 6 years. Deferasirox (Exjade â, ICL 670) is an orally active iron chelators which represents the new class of tridentate iron chelator. Deferasirox has been formulated as a dispersible tablet based on technical feasibility, stability, and adequate bioavailability. Deferasirox has been shown to be as effective as deferral (DFO) and has a clinically manageable safety profile (the most common side effects are gastrointestinal disturbances and rashes). Few studies have been conducted to compare the satisfaction, convenience, activity limitations, and patient preferences of deferasirox in comparison with DFO in sickle cell and thalassemia patients. The purpose of the present study was to evaluate the long-term response of deferosirox in iron-overloaded children aged 6 years or older treated with transfusion dependent anemia and secondary objective was to collect and analyze the following parameters such as serum ferrtin, SLT, AST, creatinine, audiology and ophthalmology, liver iron concentrations (LIC) and patient reported outcomes measures over a period of 2 years from the start of deferasirox. The study population comprised of male and female patients aged 6 years or <17.99 with chronic iron overload related to blood transfusions in patients with Thalassemia and Sickle cell disease. Fifty-one patients were enrolled into this study at the Hematology Clinic, Hospital for Sick Children, Toronto, ON Canada. This study was observational and does not impose a therapy protocol, diagnostic/therapeutic interventions or a strict visit schedule. Patients were treated with deferasirox according to the local physician's judgment and in accordance with the local (country-specific) deferasirox prescribing information. Data were collected at study entry (baseline), and at 12 months. Patients were approached for the study after the ethics approval was obtained. All variables were explored and summarized using descriptive statistics such as means, standard deviation, median and ranges, counts and proportions, graphs as appropriate. Patients reported outcomes measures questionnaires were given after the written consent/assent was obtained. Statistical analysis was done by descriptive statistics and paired t-test. Of the 51 subjects, 30 were females, 21 were males, and median age for the patients was 14 ± 5.3. Out of 51 patients, 49 patients had thalassemia and two had sickle cell disease. All of the subjects were on desferal chelation therapy before the start of the deferasirox, Analysis of patient reported outcomes measures showed that majority of the patients (73%) were very satisfied with deferasirox, when they started and after 12 months on the study, however 1 patient who reported dissatisfaction in 2009, reported satisfaction in year 2010. The main reason for the deferasirox, treatment preference were relief of pain associated with injections (30%) and more convenient (35%) in administration. Other reasons given were improved sleep patterns (2%) and less disruption to the family (6%). At baseline and 12 months laboratory evaluations were as follows: Mean Creatinine values (88% of the patients) (baseline- 34.41±11.49, 12 months – 52.41 ± 19.82), Mean ALT (65% of the patients) (baseline- 41.73 ± 7.32, 12 months – 30.94 ± 5.30) and serum ferritin (baseline- 1995± 276, 12 month, 1833 ± 203). Mean LIC (baseline-11.08 ± 1.29, 12 months- 7.87 ± 1.12) evaluations showed the incremental decrease over time with 14/51 showed the values of <3 over a 12 months period. Chelation therapy was held for some patients for a short period to prevent toxicity related to the chelator. Mild (4/51) and moderate (1/51) hearing loss were observed in subjects over period from 2009–2010. Data will be collected after 24 months to evaluate their long-term responses and for comparative analysis. Further, we can conclude that given the high levels of satisfaction, it is likely that the quality of life and adherence to chelation treatment have improved for patients who are receiving the deferasirox treatment compared with previous subcutaneous chelation treatment. Disclosures: No relevant conflicts of interest to declare.

Implementation of a European Cohort to Follow Sickle Cell Children and Adults Treated with Hydroxycarbamide

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 564-564
Author(s):  
Mariane De Montalembert ◽  
Frédéric Galacteros ◽  
Jean Antoine Ribeil ◽  
Uwe Kordes ◽  
Jean Benoit Arlet ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Safety Profile ◽  
Conflicts Of Interest ◽  
Subcutaneous Tissue ◽  
Acute Chest Syndrome ◽  
Control Group ◽  
Cell Disease ◽  
Skin Reactions ◽  
Number Of Patients

Abstract Hydroxycarbamide (HU) is a myelosuppressive drug marketed since 1968 for the treatment of hematological cancer, and authorized since 2007 in Europe as orphan medicinal product for the prevention of recurrent vaso-occlusive crises including acute chest syndrome in adults and children older than 2 years with sickle cell disease (SCD). ESCORT-HU (European Sickle Cell Disease Cohort – Hydroxyurea) is a multicenter prospective non interventional study implemented in Europe to collect more information about the safety profile of HU and morbi-mortality in SCD patients treated with HU. The study responds to EMA (European Medicines Agency) request and has been approved by the Ethical of Necker Enfants Malades Hospital (Paris, France).The ongoing study involves the largest number so far of patients with SCD treated with HU. Primary endpoints of ESCORT HU are to determine frequency of adverse events, and possible consequent changes of HU treatment. Secondary endpoints are to evaluate morbi-mortality of the disease although in the absence of control group. From June 2008 to June 2014, 483 patients (255 females; 228 males) were enrolled from 3 European countries, Greece (24%), Germany (19%), and France (56%). 67% patients were adults, median aged 37.35 yrs (17-83.5) and 33% were children, median aged 11.06 yrs (2.6-16.9). genotypes were HbS/HbS in 71.4% cases, and compound heterozygotous HbS/β-thalassemia in 22.8 % (Table 1). 137 (28.4%) patients experienced 421 events (Table 2). 132 (32.2%) of these events may be attributed to HU. The safety profile is roughly similar in children and adults. As expected the most frequent side effects were firstly blood disorders (n=86 events, 42.4%) such as neutropenia or thrombocytopenia. In all cases, these cytopenias were rapidly resolved with the transitory stop of HU. 71 events related to skin and subcutaneous tissue disorders were observed, mostly cutaneous dryness, skin reactions, alopecia and nails or skin pigmentation; 4 patients had a leg ulcer (34.8%). Most of these events are ongoing or stabilized despit the decrease of HU. No secondary cancer has been reported until now. Even if HU is an old drug with a relatively well-known safety profile, some uncertainties remain in terms of long-term safety as well as tolerance in the youngest people. The main interest of ESCORT HU is to offer the possibility of safety surveillance of hydroxycarbamide in European sickle cell patients. Table 1 Demographic data Adults Children < 17 years old Total Number of patients 322 (67%) 161 (33%) 483 Females/Males 183/139 72/89 255/228 Median age (yrs) (range) 37.35 (17-83.5) 11.06 (2.6-16.9) 28.58 Genotype SS 206 (64%) 139 (86.3%) 345 (71.4%) SC 1 (0.3%) 3 (1.86%) 4 (0.8%) Sβ0 51 (15.8%) 11 (6.8%) 62 (12.8%) Sβ+ 46 (14.2%) 2 (1.2%) 48 (9.9%) Other 18 (5.5%) 6 (3.7%) 24 (4.9%) Treatment with HU before enrollment in ESCORT HU No of pts 232 83 315 (65%) Median duration (range) of HU treatment before ESCORT HU 8.2 yrs (0.5 ans-24 yrs) 3. 1 yrs ( 71 days – 8.9 yrs) 6.85 (71 days-24 years)] HU ESCORT Daily mean dose (mg/kg/d) 16.11 ± 4.79 19.63 ± 4.69 17.32 ± 4.94 Abstract 564. Table 2 The most frequent events of hydroxycarbamide in the two populations of SCD patients ADULTS CHILDREN No ofGerman(%) No of adults No ofEpisodes(%) No of children Total(% /411) Events Related to HU treatment (Siklos®)(%**) Blood and lymphatic system disorders (%) 32 (17.7) 22 54 (31.03) 28 86 (20.9) 56 (65.1) Skin and subcutaneous tissue disorders (%) 42 (23.2) 28 29 (16.7) 19 71 (17.3) 46 (64.8) Nervous system disorders Headache (24), Dizziness/vertigo (14), 32 (17.7) 23 12(6.9) 10 44 (10.7) 11 (25) Gastrointestinal disorders Nausea (14), diarrhea (8), other (14) 20 (11) 17 23 (13.2) 16 43 (10.4) 7 (16.3) Metabolic and nutrition disorders: vit D deficiency (17), weight gain (5) 13 (7) 11 18 (18.3) 18 36 (8.75) 4 (11.1) Fever 11 (6) 10 12(6.9) 7 23 (5.6) 1 (4.3) Cardiac disorders (hypertension, bradycardia, chest pain, cardiomegaly) 4 4 2 2 6 1 (16.6) General disorders : fatigue 5 5 0 0 5 0 Hepatobiliary disorders 2 2 0 0 2 0 Neoplasms benign, malignant and unspecified (incl. cysts and polyps) Harmatoma, benign vulvar sebaceous cyst 2 2 0 0 2 0 Renal & urinary disorders 2 2 0 0 2 0 Reproductive system and breast disorders 3 3 0 0 3 0 Other 13 13 21 14 34 6 (17.1%) 181 80 /181(24.8%) 174 57 / 174(35.4%) 411 132/411 (32.2%) ** compared to the total number of “system organ class” events Disclosures No relevant conflicts of interest to declare.

scholarly journals Improved Utilization Amongst Adult Sickle Cell Disease Patients: A Multi-Disciplinary Medical Home Approach

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 22-23
Author(s):  
Daniel Sop ◽  
Shirley Johnson ◽  
Benjamin Jaworowski ◽  
Wally R Smith
Keyword(s):  
Sickle Cell Disease ◽  
Length Of Stay ◽  
Sickle Cell ◽  
Medical Home ◽  
Research Funding ◽  
Readmission Rate ◽  
Cost Savings ◽  
Multidisciplinary Teams ◽  
Cell Disease ◽  
Number Of Patients

Background Worldwide, Sickle Cell Disease (SCD) is the most common monogenetic disorder. SCD is costly to the American health care system: $460,151 per patient for the 100,000 affected US patients. Biological and behavioral health comorbidities with limited hospital resources contributed to a cost increase in SCD readmissions at the Virginia Commonwealth University Health System (VCUHS) from 2012-2016, after a long history of decline. With increasing census, in FY16 the 30-day SCD readmission rate was 33.7%, up from 22.5% in FY14. The average SCD length of stay was 6.7 days, compared with an expected length of 4.2 days. The number of ED visits for SCD patients in FY17 was projected to be double that of FY14. The percent of ED returns in 3 days was projected to be triple that of FY14. Method The VCU Adult SCD Medical Home (ASCMH) was funded and launched to address this issue, based on the success of a pilot that showed cost savings of $333,000 for 5 patients in 12 months. The SCD ASCMH officially began January 1,2018 with a two-year pilot funding period. The ASCMH was structured and designed using quality improvement (QI) principles and consisted of multidisciplinary teams to coordinate inpatient care, emergency care, and ambulatory care respectively. Evaluation compared utilization during CY 2017 (pre-intervention) versus CY 2019 (2 years post intervention). For all patients we compared the average 30-day readmission rate, the average length of stay (ALOS), the average 3-Day ED return rate, the number of ED discharges, the numbers of inpatient days, inpatient discharges, and outpatient visits, the number of patients who used the ED, and total VCU charges. Results Among 541 (2017) and 592 (2019) SCD adults, including 40.6% males and 59.4% females, comparing pre-intervention to intervention, average utilization and VCU charges were either numerically or statistically significantly reduced. Mean 30-day readmission rates were reduced (31.60% vs 20.40%) ALOS was reduced (5.6 days vs 4.3 days), inpatient days were reduced (5313 days vs 3340 days), and total charges were significantly reduced ($15,664,895 vs $13,939,842). Conclusion At VCU, a multi-disciplinary ASCMH that featured intensive case management (CM) reduced annual utilization and cost savings for adult SCD patients. CM program elements that were most effective should be studied in the future and next steps should include a randomized controlled trial that can demonstrate evidence of their efficacy in strengthening and improving utilization Table Disclosures Smith: Emmaeus Pharmaceuticals, Inc.: Consultancy; GlycoMimetics, Inc.: Consultancy; Novartis, Inc.: Consultancy, Other: Investigator, Research Funding; Shire, Inc.: Other: Investigator, Research Funding; Imara: Research Funding; Novo Nordisk: Consultancy; Ironwood: Consultancy; Pfizer: Consultancy; Incyte: Other: Investigator; Health Resources and Services Administration: Other: Investigator, Research Funding; NHLBI: Research Funding; Patient-Centered Outcomes Research Institute: Other: Investigator, Research Funding; Global Blood Therapeutics, Inc.: Consultancy, Research Funding; Shire: Research Funding.

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