Analysis of utilization of drugs for sickle cell disease in tertiary care hospitals

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

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.


Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 2660-2660
Author(s):  
Katherine Ender ◽  
Jennifer Freed ◽  
John Babineau ◽  
Mary Tresgallo ◽  
William Schechter ◽  
...  

Abstract Abstract 2660 Background: Sickle cell disease causes considerable morbidity and mortality. Three guidelines exist for the management of vaso-occlusive pain, although studies have shown that these guidelines are not well followed and that there is considerable variation in care. Investigations of clinical pathways for pediatric sickle cell pain have not been published to our knowledge, but research in other pediatric conditions has shown that the implementation of clinical pathways can improve medical care. We initiated an investigation of the effects of a pain management pathway for sickle cell vaso-occlusive crisis in the emergency department (ED) with the hypothesis that the introduction of a clinical pathway would improve the efficiency and efficacy of acute pain management. Methods: We conducted a prospective, cohort study from February 2009 to March 2010 in an urban, tertiary care ED. We collected data from patients aged 3–18 years old with sickle cell disease who presented to the ED with vaso-occlusive crisis (VOC) pain. Baseline data was collected for five months prior to the introduction of the clinical pathway, followed by a four-week time interval in which the ED physicians and nurses were in-serviced on the pathway, followed by six months in which data was collected with the clinical pathway in place. Our pathway, which is in checklist format with instructions for triage, monitoring, medication administration, and timing of assessments and interventions, was developed by representatives from the divisions of Pediatric Hematology, Pediatric Emergency Medicine, and Pediatric Pain Medicine, Symptom Management and Palliative Care and based upon current standard-of-care guidelines. Results: Over the eleven month study period, 68 patients were enrolled. Random chart audits revealed a 75% capture rate. Significant improvement was demonstrated in time interval to first analgesic from 74 minutes to 42 minutes (p value 0.02), time interval to first opioid from 94 minutes to 46 minutes (p value < 0.01), and time interval to subsequent assessment of pain score from 110 minutes to 72 minutes (p value 0.02). The percentage of patients who received ketorolac also increased from 57% to 82% (p value 0.03). Change in pain scores was not significantly different, nor was admission rate. Conclusions: Implementation of a pain management pathway for sickle cell VOC led to an improvement in the time interval to administration of first analgesic and time interval to pain re-assessment, bringing these aspects of patient management closer to accepted guidelines. Whether improvement can be made in admission rates, change in pain ratings, and patient satisfaction will require further study. Disclosures: No relevant conflicts of interest to declare.


Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 3679-3679
Author(s):  
Patricia Palcu ◽  
Kevin H. M. Kuo ◽  
Richard Ward ◽  
Nusrat Zaffar ◽  
Ann Kinga Malinowski ◽  
...  

Abstract BACKGROUND: Increasing numbers of women with sickle cell disease (SCD) are reaching reproductive age. There is an array of possible pregnancy outcomes that women with SCD may experience. Many have described adverse maternal and fetal outcomes in pregnant patients with SCD including increased risk of pre-eclampsia, infections, painful vaso-occlusive crises as well as increased risk of intrauterine growth restriction, pre-term delivery and pregnancy loss. Not all women with SCD have complicated pregnancies, however. Limited data are available to categorize the proportion of patients who have uncomplicated pregnancies and protective characteristics for good pregnancy outcomes. Identifying these features potentially informs practice and reduces exposure to blood products as red blood cell (RBC) transfusion is associated with an increased risk of alloimmunization, particularly in pregnancy. OBJECTIVE: To determine the proportion and characteristics of pregnant patients with SCD who have uncomplicated pregnancies. METHODS: We retrospectively reviewed medical records of pregnant women with SCD from 1980 to 2018 at Mount Sinai Hospital, a university affiliated tertiary care centre in Toronto, Canada. We defined a pregnancy to be uncomplicated if the pregnancy was not associated with a hospitalization, RBC transfusion was not administered peripartum and/or the delivery was at term (37 or more weeks gestation). RESULTS: A total of 179 pregnancies were included (Figure 1). Preliminary results show that 57% of pregnancies had hemoglobin SS disease. The median maternal age was 27 years (range 16-43 years) (n=179). Median hemoglobin concentration, platelet count and leukocyte count were 91g/L (n=141), 304 x 10(9)/L (n=140) and 11 x 10(9)/L (n=140), respectively. Hospital admission was required in 61% of pregnancies (n=171) and RBC transfusion in 49% of pregnancies (n=173). The median hemoglobin F level was 0.05 (range 0.01-0.87). Nineteen percent had coexisting alpha gene deletion (s). The median birth weight was 2863 grams (n=155). Analysis is ongoing to determine characteristics associated with the pre-defined pregnancy outcomes. CONCLUSIONS: Women with SCD frequently have adverse maternal and fetal outcomes with pregnancy. However, 39% of pregnancies with SCD in this study did not require hospitalization and 51% did not require RBC transfusion. Additional analysis will attempt to identify protective features so as not to expose all patients to RBC transfusion unless it is required. These initial results suggest that risk scores should be developed for pregnant patients with sickle cell disease to identify who may benefit from RBC transfusion. Disclosures No relevant conflicts of interest to declare.


Author(s):  
Manjusha Shripad Dhawle ◽  
Ashwini Radhakrishan Tangde ◽  
Santosh Govind Rathod ◽  
Rajan S. Bindu

Background: Sickle cell disease (SCD) is well known and is the commonest hereditary hematological disorder which is associated with increased mortality and morbidity.  They are group of inherited haemoglobinopathies caused by the occurrence of hemoglobin S (Hbs) in homozygous or heterozygous form or in combinations of Hbs with another hemoglobin such as Hbsc or beta thalassaemia (Hbs-thal). Sickle cell syndromes are remarkable for their clinical heterogenecity including their presentations as sudden and unexpected death due to sickle cell crises. While doing autopsy in cases of deaths with no apparent cause and physical over activity medical officer must keep in mind the possibility of death due to vasoocclusive crisis in sickle cell disease. Aim of the study was to create awareness among the physicians and relatives / public and to minimize future unexpected death from complications or crisis from SCD.Methods: This is a retrospective study of 10 cases carried in the department of pathology, in tertiary care hospital and covers a period from January 2009 to December 2016. These cases were brought dead to the casualty with a history of sudden death. After post mortem examination, the specimens were sent for histopathological examination.Results: The record of 10 cases was reviewed. Out of our ten cases 7 were male and 3 were females. The youngest person was 17-year female and oldest was 65 years male. In clinical history 3 cases had complains of chest pain (30%), 3 others had complained of breathlessness (30%), 2 had history of unconsciousness (20%), one case had complained of abdominal pain (10%) and one case had a history of fall and injury (10%). Microscopic examination of each organ was carried out.  Organs like lungs, liver, spleen, kidneys, heart and brain showed wide spread congested vessels which were stuffed with RBC.Conclusions: We present this study to emphasize that sickle cell crisis is one of the cause of sudden unexplained death and highlight the importance of considering sickle cell disease as a cause of death in cases with no apparent cause.


Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 3577-3577
Author(s):  
Iman Al Fadhali ◽  
Farah Al-Kindy ◽  
Naema Alshibli ◽  
Salam Alkindi ◽  
Murtadha Al-Khabori ◽  
...  

Background: Sickle cell disease is a common hemaglopinapthy worldwide and in Oman. Avascular necrosis (AVN) of the hip causes significant morbidity to patients with sickle cell disease and has a profound impact on their quality of life. This study aims to identify the prevalence and outcome of AVN among young Omani patients with sickle cell disease. Methods: The is a cross sectional study done in the main tertiary care and referral facility in Oman, Sultan Qaboos University Hospital. Out of 3000 registered patients 85 patients found to have clinical and radiological proven AVN, between June 2017-January 2019. Data obtained included demographics, the affected joint, uni or bilateral), radiological staging by FICAT (MRI), hydroxyurea use, dose and duration as well as laboratory data at time of diagnosis. Results: The prevalence of AVN among SCD Omani children and young adults is 2.8 %. Their age ranges between 5-25 (Mean 14.6 +3.4). Male to female ratio was 1.6:1. Based on FICAT system score, most of the cases (82%) are AVN grade 3 and above at diagnosis. Thirty-six patients (42%) were diagnosed to have stage IV AVN. Regarding the hydroxyurea use, 43 patients (50.6%) were already started on HU before the development of AVN. Their mean duration of HU use was 5.3 years, with an average dose of 15.7 mg/kg/day. Fifteen patients developed AVN and were started on HU as part of their management. The follow up MRI of 4 of them showed improvement of their AVN stage (from 4 to 2). Fifteen patients (17.6%) underwent joint replacement because of chronic pain and disability and almost half of them (7 patients) were on hydroxyurea. Conclusion: Prevalence of AVN in young patients with SCD in the main tertiary care referral facility in Oman is 2.8% which is less than the internationally reported. Patients are diagnosed at later stages (3 and above) indicating a possibility of underdiagnosis of asymptomatic stage one and 2 patients. Hydroxyurea use has improved the severity of the AVN in few patients. Disclosures Al-Khabori: SOBI: Honoraria; AstraZeneca: Honoraria; Novartis: Honoraria, Membership on an entity's Board of Directors or advisory committees; Roche: Membership on an entity's Board of Directors or advisory committees; Amgen: Membership on an entity's Board of Directors or advisory committees; Servier: Membership on an entity's Board of Directors or advisory committees; Shire (Takeda): Membership on an entity's Board of Directors or advisory committees; Abbvie: Membership on an entity's Board of Directors or advisory committees; NovoNardisk: Membership on an entity's Board of Directors or advisory committees. Wali:Sultan Qaboos University Hospital: Employment.


2008 ◽  
Vol 1 (1) ◽  
pp. 1 ◽  
Author(s):  
KC Aggarwal ◽  
PC Goyal ◽  
MS Prasad ◽  
S Saluja ◽  
M Sharma ◽  
...  

Author(s):  
Rahi S. Modi ◽  
Srushti S. Patel ◽  
Dipti A. Modi ◽  
Heena Talesara

Background: Sickle cell disease is a hereditary haematological disorder prevalent in tribal regions of India. Sickle cell disease can increase complications during pregnancy and in turn negatively influence pregnancy outcomes. This study reports the analysis of tribal maternal admissions in the tertiary centre S.S.G. Hospital, Baroda, Gujarat. Hence this study was conducted to assess complications in pregnancy and maternal and perinatal outcome among women with Sickle cell disease.Methods: It was a retrospective observational study including all pregnant women with sickle cell disease after 20 weeks of gestation who delivered at S.S.G. Hospital, Baroda from August 2019 to August 2020.Results: There were 43 antenatal women with Sickle cell disease during the study period. There was increased risk of obstetric complications like gestational hypertension (11.62%), preeclampsia (9.3%), eclampsia (6.97%), HELLP syndrome (4.65%), intrauterine growth retardation (23.25%), and oligohydramnios (11.62%). Medical complications observed were mainly anaemia (53.48%), vaso-occlusive crisis (18.16%), acute chest syndrome (4.65%) and infections like urinary tract infection (6.97%) and pneumonia (4.65%). The incidence of low birth weight babies (56.94%), low APGAR score (11.62%) and neonatal ICU admissions (23.25%) was high. 6.5% cases of maternal mortality and 4.65% cases of perinatal mortality was observed.Conclusions: Pregnancy in Sickle cell disease is associated with an increased maternal morbidity and high perinatal mortality due to obstetric and medical complications.


2019 ◽  
Vol 7 (1) ◽  
pp. 97
Author(s):  
Varsha P. Patel ◽  
Archana U. Gandhi ◽  
Chineen Shah

Background: Sickle cell disorders are structural hemoglobinopathies, rendering red blood cells sickle shaped, less deformable and sticky leading to microvascular vaso-occlusion and premature red blood cells destruction which leads to varied clinical manifestations. It leads to lifelong morbidity thus affecting quality of life and contributes to early mortality thereby reducing the key national resources- the healthy workforce. This study was done to evaluate epidemiological and clinical profile of sickle cell disease attending the centre.Methods: This study was cross-sectional, observational study conducted at tertiary care hospital in Gujarat. After taking ethical clearance patients were enrolled as per inclusion and exclusion criteria and epidemiological and clinical profile of sickle cell disease patients was studied.Results: Mean age of sickle cell disease was 22.58 years. It was found in tribal communities of Gujarat like Rathwa, Baria, Tadvi etc. Commonest symptom was musculoskeletal pain (86.84%), followed by jaundice (71.05%), fever, dyspnoea, abdominal pain and chest pain. Most common systemic manifestation was pain crises (60.66%), followed by hemolytic anemia (31.15%), acute chest syndrome (30%), consolidation (11.67%), hepatopathy (10%) and avascular necrosis of hip. (6.56%).Conclusions: Sickle cell disease is seen in younger patients. In Gujarat mainly tribal communities are affected. Major systemic manifestations of sickle cell disease include pain crisis followed by hemolytic crisis, acute chest syndrome, hepatopathy and AVN of hip.    


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