Family caregiving for adults with sickle cell disease and extremely high hospital use

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

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

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

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


2012 ◽  
Vol 8 (1) ◽  
pp. 42-46 ◽  
Author(s):  
Daniel Weisberg ◽  
Gabriela Balf-Soran ◽  
William Becker ◽  
Shan-Estelle Brown ◽  
William Sledge

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

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


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

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 3804-3804
Author(s):  
Raymond U. Osarogiagbon ◽  
Laura M. McHugh ◽  
Lori E. Kronish ◽  
Elisabeth A. Chismark

Abstract Sickle cell disease is typified by dramatic acute painful episodes, which consume the majority of patients’ and healthcare providers’ attention. However, the cumulative damage to vital organs that occurs in this disease is often the determinant of early death. The kidneys are a major target organ in sickle cell disease and renal failure is a highly morbid event. It is also strongly associated with early death. Glomerular hyperfiltration is often the earliest sign of renal injury. This is followed by return to apparently normal filtration rates, then successively lower rates, culminating in end-stage renal disease. Microalbuminuria follows hyperfiltration and progresses to frank proteinuria. We evaluated the prevalence of renal dysfunction in a cohort of patients with sickle cell disease managed at the University of Tennessee Cancer Institute’s adult sickle cell program. At entry into the program, patients underwent a routine battery of tests, including 24 hour urine collection for measurement of creatinine clearance and urine protein. Our findings are summarized in the table below. By the age of 25 years, almost 80% of patients with sickle cell disease showed significant abnormality of glomerular filtration and 40% had significant proteinuria. This worsens with age. By a median of 37 years, 2 in 3 patients have developed significant proteinuria and hypofiltration has become the predominant pattern of glomerular filtration abnormality. Since renal injury is reversible in the early stages, more emphasis needs to be placed on aggressive early screening, surveillance and intervention. Intervention at the stage of hyperfiltration and microalbuminuria is easier and much more likely to be successful. Patients and their healthcare providers need to be educated on the high prevalence of renal damage. Efforts at education, screening, surveillance and, where necessary, treatment should begin well before adolescence.


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

2016 ◽  
Vol 63 (12) ◽  
pp. 2146-2153 ◽  
Author(s):  
Nancy S. Green ◽  
Deepa Manwani ◽  
Mahvish Qureshi ◽  
Karen Ireland ◽  
Arpan Sinha ◽  
...  

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

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


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

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


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

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


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