scholarly journals Management of Sickle Cell Disease Pain among Adolescent and Pediatric Patients

2019 ◽  
Vol 9 (8) ◽  
pp. 182
Author(s):  
Samar Abdo ◽  
Khawla Q. Nuseir ◽  
Ahmad A. Altarifi ◽  
Moussa Barqawi ◽  
Nehad M. Ayoub ◽  
...  

Management of sickle cell pain in adolescent and pediatric patients is inadequate, and the employment of proper management guidelines and practices are highly variable among different regions and populations. APPT, the multidimensional adolescent pediatric pain tool, promotes optimal pain management and introduces best practical guidelines for pain management. The goal of this study is to assess pain and pain management among young patients diagnosed with sickle cell disease (SCD) by introducing the APPT as a tool for pain management, and analyze factors contributing to pain management. Information relevant to demographic data, SCD characteristics, APPT assessment, and satisfaction of patients regarding pain management were collected using a structured questionnaire. Results showed that SCD is highly associated with gender (p = 0.022), consanguinity (p = 0.012), and number of surgeries (p = 0.013). Most patients (58.9%) indicated the involvement of more than six body areas affected during pain crisis. Severe pain was described by more than half the patients (55.6%), while moderate pain was reported by 31.1%. Most patients described their pain by sensory, affective, and temporal words. The number of painful areas, pain intensity, and use of descriptive pain words was correlated and interpreted by age, BMI, school absence, and number of surgeries. Results of this study could provide guidance to healthcare providers to improve current practices for SCD pain management in order to improve health outcomes and patients’ satisfaction.

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 4846-4846
Author(s):  
Sarah Leonard ◽  
Lindsay Cortright ◽  
Dmitry Tumin ◽  
Lora Joyner ◽  
Sruthipriya Sridhar

Introduction: Individuals with sickle cell disease (SCD) experience both acute vaso-occlusive pain (VOC) and chronic pain, both primarily treated with opioids. However, opioids only address the sensory dimension of pain and repeated use of opioids can cause short and long-term side effects such as respiratory system depression, increased risk of cardiovascular events, and hyperalgesia. Non-pharmacologic approaches to the management of acute and chronic SCD pain, such as cognitive behavioral therapy, biofeedback, acupuncture, hypnosis, and megavitamins, have shown positive effects for pain management and may reduce opioid use. Among non-pharmacologic pain management methods, aquatic therapy has been underutilized and understudied in patients with SCD, despite showing benefits for pain management and quality of life in adults with musculoskeletal conditions. At Vidant Medical Center, aquatic therapy has been introduced for patients with SCD in 2015 and has been increasingly used since then. To describe the use of aquatic therapy and outcomes in pediatric patients with SCD, we retrospectively evaluated the use of this therapy at Vidant Medical Center among children hospitalized for acute VOC pain. Methods: The study was approved by the Institutional Review Board at East Carolina University. Children with SCD between 7-18 years of age at the time of admission for acute VOC pain between the years 2015-2018 were included in the analysis. During the review period, orders for aquatic therapy were placed at the provider's discretion. Our primary outcome was utilization of aquatic therapy during a given hospitalization, determined by retrospective query of the electronic medical record (EMR). Secondary outcomes include length of stay and time to readmission for VOC. Covariates were assessed at the time of the index admission included age, sex, and insurance coverage. Mixed-effects logistic or Poisson regression models were fitted to account for multiple hospitalizations per patient. Results: The analysis included 316 hospitalizations of 87 patients (48% female; median age at the earliest hospitalization, 11 years). The median duration of hospital admissions was 4 days (interquartile range [IQR]: 3, 7). Aquatic therapy was used in 38% of admissions, with a trend of increasing use during the study period (Figure 1). On multivariable logistic regression analysis of aquatic use during a given admission, characteristics associated with greater likelihood of aquatic therapy use included older age and more recent year of admission. Aquatic therapy was associated with 69% longer hospital stays, although this may be related to availability of aquatic therapy services. Among 52 patients with multiple admissions, use of aquatic therapy during a given admission was associated with 26% more days between hospitalizations (incidence rate ratio = 1.26; 95% CI: 1.21, 1.31; p=<0.001). Conclusion: Research into the use of non-pharmacologic pain management strategies for sickle cell disease has recently increased in order to better address the multidimensional aspects of pain. Of these management strategies, aquatic therapy has been very underutilized and under researched. Analysis of the utilization of aquatic therapy in our institution showed improved use over the last 3 years as well as increased duration between hospitalizations after the use of aquatic therapy. These positive results point towards the need for more research into other outcome measures derived from the use of aquatic therapy in pain management of pediatric SCD patients. Figure 1 Disclosures No relevant conflicts of interest to declare.


2019 ◽  
Vol 54 (5) ◽  
pp. 610-619 ◽  
Author(s):  
Azza A. Tantawy ◽  
Amira A. Adly ◽  
Fatma S. E. Ebeid ◽  
Eman A. Ismail ◽  
Mahitab M. Hussein ◽  
...  

2007 ◽  
Vol 46 (4) ◽  
pp. 311-319 ◽  
Author(s):  
Monica J. Mitchell ◽  
Kathleen Lemanek ◽  
Tonya M. Palermo ◽  
Lori E. Crosby ◽  
Alisha Nichols ◽  
...  

Hematology ◽  
2013 ◽  
Vol 2013 (1) ◽  
pp. 433-438 ◽  
Author(s):  
Jean L. Raphael ◽  
Suzette O. Oyeku

Pain is the most common cause for hospitalization and acute morbidity in sickle cell disease (SCD). The consequences of SCD-related pain are substantial, affecting both the individual and the health care system. The emergence of the patient-centered medical home (PCMH) provides new opportunities to align efforts to improve SCD management with innovative and potentially cost-effective models of patient-centered care. The Department of Health and Human Services has designated SCD as a priority area with emphasis on creating PCMHs for affected patients. The question for patients, clinicians, scientists, and policy-makers is how the PCMH can be designed to address pain, the hallmark feature of SCD. This article provides a framework of pain management within the PCMH model. We present an overview of pain and pain management in SCD, gaps in pain management, and current care models used by patients and discuss core PCMH concepts and multidisciplinary team–based PCMH care strategies for SCD pain management.


Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 2106-2106
Author(s):  
Madiha Iqbal ◽  
Tea Reljic ◽  
Ernesto Ayala ◽  
Hemant S. Murthy ◽  
Ambuj Kumar ◽  
...  

Background: Sickle cell disease (SCD) is an inherited hemoglobinopathy which affects over 300,000 children born each year worldwide. In spite of improvement in supportive care in recent years, there is still a lack of effective treatment options. SCD leads to debilitating and cyclic episodes of erythrocyte sickling with progressive organ injury, contributing to lifetime morbidity and shortened life expectancy. Allogeneic HCT (allo-HCT) is a potentially curative therapy for SCD because engraftment is associated with resolution of the clinical phenotype of the disease and abrogation of its complications. Medical literature on allo-HCT for SCD is largely limited to children. Recent studies have evaluated the efficacy of allo-HCT in the adult population. Here, we conduct a systematic review/meta-analysis to assess the totality of evidence pertaining to the efficacy (or lack thereof) of allo-HCT in children and adults. Materials and methods: We performed a comprehensive search of the medical literature using PubMed/Medline, EMBASE and Cochrane library on July 3rd, 2019. We extracted data on clinical outcomes related to benefits (overall [OS] and disease free/event free survival [EFS/DFS]) and harms (non-relapse mortality [NRM] and graft failure [GF]), independently by two authors. Our search strategy identified 1001 references but only 30 studies (n= 1995 patients) were included in this systematic review/meta-analysis. We also performed a sub analysis on clinical outcomes for studies that included only pediatric patients (defined as <18 years) and those in patients ≥18 years of age. Results: Median age for patients enrolled in all the studies was at 10 years. Recurrent veno-occlusive crises represented the most common indication for allo-HCT followed by acute chest syndrome and stroke; nevertheless, most patients had more than one indication. Matched related donors (MRD) were the most common donor source (93%). Bone marrow was the most common source of hematopoietic stem cells (77%). Majority of patients underwent conditioning with myeloablative regimens (77%). Pooled OS rates (n=29 studies, 1681 patients) after allogeneic HCT was 95% (95%CI=93-96%) with low heterogeneity (I2=6.4%) among included studies (Figure 1). Pooled EFS/DFS rates (n=29 studies, 1894 patients) post-allografting was 90% (95%CI=87-93%) with moderate heterogeneity (I2=54%). Pooled NRM rates from 30 studies (1995 patients) was 4% (95%CI=2-6%) with low heterogeneity (I2=29.4%). Pooled GF rates from 28 studies (1851 patients) was 4% (95%CI=2-6%) with moderate heterogeneity (I2=55%). A subset analysis specifically for pediatric patients (n= 11 studies, 1009 patients, median age at 9.7 years) showed a pooled OS rate of 96% (95%CI=94-97%) with low heterogeneity (I2=0%); and for adult patients (n=3 studies, 51 patients, median age at 33.4 years) the pooled OS was 94% (95%CI=80-100%) with moderate heterogeneity (I2=52%). Pooled EFS/DFS for pediatric patients (n= 11 studies, 1009 patients) was at 89 %( 95%CI=84-93%) with moderate heterogeneity (I2=55.1%); and for adult patients (n=2 studies, 30 patients) was at 95% (95%CI=83-100%) with high heterogeneity (I2=96.5%). Pooled NRM from 10 studies with pediatric patients (281 patients) was at 6 % (95%CI=3-10%) with low heterogeneity (I2=0%); and from 3 studies with adult patients (51 patients) was at 1% (95%CI=0-7%) with low heterogeneity (I2=15.1%). Pooled GF from 10 studies with pediatric patients (281 patients) was at 3 % (95%CI=1-7%) with moderate heterogeneity (I2=40%); and from 2 studies with adult patients (30 patients) was at 5% (95%CI=0-17%) with high heterogeneity (I2=95.4%). Conclusions: The results of our systematic review/meta-analysis show excellent OS, EFS/DFS in children and adults undergoing allo-HCT with pooled OS rates exceeding 90%. The main limitation to offering an allo-HCT in SCD remains the availability of a suitable donor as 85% of patients meeting criteria do not have a MRD. We anticipate that with emergence of haploidentical transplantation the number of allo-HCT will increase in the future. GF remains a significant concern in this population and future studies should focus on novel immune suppression strategies to help reduce GF. Disclosures Kharfan-Dabaja: Pharmacyclics: Consultancy; Daiichi Sankyo: Consultancy.


2021 ◽  
Vol 2 (3) ◽  
pp. 9-17
Author(s):  
Dafni Eleftherou ◽  
Aristidis Arhakis ◽  
Sotiria Davidopoulou

Aim: This literature review aims to update the evidence for orofacial manifestations and current treatment recommendations for children and adolescents with sickle cell disease. Background: Sickle cell disease is a frequent hemoglobinopathy and a life-threatening genetic disorder. The lifelong condition is characterized by chronic hemolytic anemia and vaso-occlusive crisis that may occur in a variable range of clinical presentations in different regions of the body, including the oral cavity. Review results: This review explored the most common orofacial alterations of pediatric patients with SCD. Dental caries is a common finding in SCD pediatric patients, especially in those who are socio-economically vulnerable. Moreover, malocclusions occur in high prevalence in SCD pediatric patients. Other oral health complications seen in SCD patients include periodontal inflammation, bone changes, infections, mental nerve neuropathy, facial overgrowth, delayed tooth eruption, dental anomalies, pulp necrosis, soft tissue alterations and salivary changes. Dental infections may trigger a vaso-occlusive crisis leading the patient to a higher probability on arriving in hospital emergency departments and in need for further hospital admission to deal with the correlated complications. Thus, preventive dental care and non-invasive dental procedures are the principal focus in SCD patients in order to avoid possible subsequent complications. Conclusion: The review showed that in pediatric patients with SCD the risk for orofacial manifestations and complications depends not only on the presence of SCD but also on other confounding factors such as oral hygiene, diet habits and social conditions. Moreover, more well-designed epidemiological studies are necessary to assess the real link between SCD disease and its impact on stomatognathic health.


2018 ◽  
Vol 11 (2) ◽  
pp. 75-81 ◽  
Author(s):  
Rasha Abdel-Raouf Afifi ◽  
Dina Kamal ◽  
Riham El. Sayed ◽  
Sherif M.M. Ekladious ◽  
Gehan H. Shaheen ◽  
...  

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