Clinical practice guideline improves the treatment of sickle cell disease vasoocclusive pain

Opioids are a critical component of pain relief strategies for the management of patients with cancer and sickle cell disease. The escalation of opioid addiction and overdose in the United States has led to increased scrutiny of opioid prescribing practices. Multiple reports have revealed that regulatory and coverage policies, intended to curb inappropriate opioid use, have created significant barriers for many patients. The Centers for Disease Control and Prevention, National Comprehensive Cancer Network, and ASCO each publish clinical practice guidelines for the management of chronic pain. A recent JAMA Oncology article highlighted perceived variability in recommendations among these guidelines. In response, leadership from guideline organizations, government representatives, and authors of the original article met to discuss challenges and solutions. The meeting featured remarks by the Commissioner of Food and Drugs, presentations on each clinical practice guideline, an overview of the pain management needs of patients with sickle cell disease, an overview of perceived differences among guidelines, and a discussion of differences and commonalities among the guidelines. The meeting revealed that although each guideline varies in the intended patient population, target audience, and methodology, there is no disagreement among recommendations when applied to the appropriate patient and clinical situation. It was determined that clarification and education are needed regarding the intent, patient population, and scope of each clinical practice guideline, rather than harmonization of guideline recommendations. Clinical practice guidelines can serve as a resource for policymakers and payers to inform policy and coverage determinations.

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Technology use and preferences to support clinical practice guideline awareness and adherence in individuals with sickle cell disease

Journal of the American Medical Informatics Association ◽

10.1093/jamia/ocy036 ◽

2018 ◽

Vol 25 (8) ◽

pp. 976-988 ◽

Cited By ~ 5

Author(s):

Amol Utrankar ◽

Tilicia L Mayo-Gamble ◽

Whitney Allen ◽

Laurie Novak ◽

Adetola A Kassim ◽

...

Keyword(s):

Clinical Practice ◽

Sickle Cell Disease ◽

Focus Groups ◽

Sickle Cell ◽

Practice Guidelines ◽

Clinical Practice Guideline ◽

Practice Guideline ◽

Technology Use ◽

Cell Disease ◽

Patient Centered

Abstract Objective Sickle cell disease (SCD) is a chronic condition affecting over 100 000 individuals in the United States, predominantly from vulnerable populations. Clinical practice guidelines, written for providers, have low adherence. This study explored knowledge about guidelines; desire for guidelines; and how technology could support guideline awareness and adherence, examining current technology uses, and user preferences to inform design of a patient-centered guidelines application in a chronic disease. Methods This cross-sectional mixed-methods study involved semi-structured interviews, surveys, and focus groups of adolescents and adults with SCD. We evaluated interest, preferences, and anticipated benefits or barriers of a patient-centered adaptation of SCD practice guidelines; prospective technology uses for health; and barriers to technology utilization. Results Forty-seven individuals completed surveys and interviews, and 39 participated in three separate focus groups. Most participants (91%) were unaware of SCD guidelines, but almost all (96%) expressed interest in a guidelines application, identifying benefits (knowledge, activation, individualization, and rewards), and barriers (poor information, low motivation, and resource limitations). Current technology health uses included information access, care coordination, and reminders about health-related actions. Prospective technology uses included informational messaging and timely alerts. Barriers to technology use included lack of interest, lack of utility, and preference for direct communication. Conclusions This study’s findings can inform the design of clinical practice guideline applications, suggesting a promising role for technology to engage patients, facilitate care decisions and actions, and improve outcomes.

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Bridging the Gap Among Clinical Practice Guidelines for Pain Management in Cancer and Sickle Cell Disease

Journal of the National Comprehensive Cancer Network ◽

10.6004/jnccn.2019.7379 ◽

2020 ◽

Vol 18 (4) ◽

pp. 392-399 ◽

Cited By ~ 2

Author(s):

Alyssa A. Schatz ◽

Thomas K. Oliver ◽

Robert A. Swarm ◽

Judith A. Paice ◽

Deepika S. Darbari ◽

...

Keyword(s):

Clinical Practice ◽

Pain Management ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Clinical Practice Guidelines ◽

Practice Guidelines ◽

Clinical Practice Guideline ◽

Practice Guideline ◽

Patient Population ◽

Cell Disease

Opioids are a critical component of pain relief strategies for the management of patients with cancer and sickle cell disease. The escalation of opioid addiction and overdose in the United States has led to increased scrutiny of opioid prescribing practices. Multiple reports have revealed that regulatory and coverage policies, intended to curb inappropriate opioid use, have created significant barriers for many patients. The Centers for Disease Control and Prevention, National Comprehensive Cancer Network, and American Society of Clinical Oncology each publish clinical practice guidelines for the management of chronic pain. A recent JAMA Oncology article highlighted perceived variability in recommendations among these guidelines. In response, leadership from guideline organizations, government representatives, and authors of the original article met to discuss challenges and solutions. The meeting featured remarks by the Commissioner of Food and Drugs, presentations on each clinical practice guideline, an overview of the pain management needs of patients with sickle cell disease, an overview of perceived differences among guidelines, and a discussion of differences and commonalities among the guidelines. The meeting revealed that although each guideline varies in the intended patient population, target audience, and methodology, there is no disagreement among recommendations when applied to the appropriate patient and clinical situation. It was determined that clarification and education are needed regarding the intent, patient population, and scope of each clinical practice guideline, rather than harmonization of guideline recommendations. Clinical practice guidelines can serve as a resource for policymakers and payers to inform policy and coverage determinations.

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How Do New Immigrant Families (African Continent) with a Child with Sickle Cell Disease (SCD) Experience the Western Medical System?

Blood ◽

10.1182/blood.v128.22.3529.3529 ◽

2016 ◽

Vol 128 (22) ◽

pp. 3529-3529

Author(s):

Aisha Aiko Bruce ◽

Adrienne D. Witol ◽

Haley Greenslade ◽

Mandeep Plaha ◽

Mary Anne Venner

Keyword(s):

Sickle Cell Disease ◽

Medical Care ◽

Sickle Cell ◽

Practice Guideline ◽

Emotional Support ◽

Extended Family ◽

Immigrant Families ◽

Medical System ◽

Cell Disease ◽

Newcomer Families

Abstract INTRODUCTION: New immigrant families from continental Africa account for an increasing proportion of pediatric patients with Sickle Cell Disease (SCD) in Canada and North America. As families enter the western medical system they face a myriad of tests and medications as well they encounter language barriers, endless forms and large teams. Previous experiences with healthcare also influence families' expectations and adjustment.There is no published data exploring the experiences of these families to help guide practice. Resources such as the Canadian Pediatric Society guide on immigrant health are not specific to SCD. We set out to examine cultural sensitivity methodologically in order to improve delivery of care. Research Questions: What are newcomer families' experiences with SCD in Canada and their home country?What are the prevailing values and beliefs related to SCD that shape the attitude and behaviors of newcomer families?How do newcomer families perceivethe current delivery of medical care (the barriers and the facilitators)? METHODS: Focused ethnography was used to understand the socio-cultural context in which newcomer families from Africa experience their child's SCD; to explore their perspectives, beliefs, how they manage daily life and experience the western medical system. A sample size of12-15 participants was selected to reach saturation.Participants were selected using purposeful and convenience sampling and semi-structured interviews were held with the primary caregiver(s) with use of aninterpreter if needed. Research Ethics Board approved. RESULTS: Saturation was reached at 10 families and 12 were interviewed due to recruiting methods. Demographics:12 caregivers (N=8 females; N=4 males); most were in their forties and from Congo, Nigeria or Liberia. The majority had 3 or more children, were married and employed. The majority did not have extended family within the region. Languages spoken at home were English, French, Yoruba, Swahili orMoorie. They immigrated to Canada between 2002 and 2015 For themes see table 1. CONCLUSIONS: Participants' attitude, perception and knowledge about SCD were profoundly affected by their experiences in their countries of origin. These mostly negative experiences (seeing children suffering without appropriate medical care; observing social stigma, etc.) were deeply embedded and determined their response to SCD in their children. 1. Practice guideline: Allow for sufficient time and provision oftranslation services to explore the families' experience with stigma within country of residence and origin as well as embedded in the healthcare system and the community. Despite the prevalence of SCD in their home countries the diagnosis was a surprise. The path towards acceptance was slow, emotionally convoluted and not linear. Acceptance of the diagnosis is a process and devastating in the context of previous experiences. 2. Practice guideline: Review diagnostic information early and have easily accessible information about SCD available for parents/family network. This information will also need to be reviewed with the child at key developmental time periods. SCD has a dominant impact on life causing renegotiation of all relationships: spousal, family, community, co-workers and school staff. Managing SCD influenced daily routines imposing structure which was disrupted for hospitalizations. Families were reluctant to leave children unattended in the hospital and thus sacrificed personal and employment goals. Social support is limited and families cope alone.Families tend to seek practical support and deny the desire for emotional support. 3. Practice guideline: 3a)Screen for potential isolation and explore whether other caretakers are aware of diagnosis and disease specific care 3b) Given the tendency to deny emotional support needs, lack of nearby extended family and the stigma in the community setting up networks that provide both practical and instrumental support could be meaningful and more likely utilized resources. The life-long complexity of SCD creates anxiety for the child's life expectancy. Families trust in medical expertise, improvements in medical treatments and their faith/religious beliefs are foundations for hope. 4. Practice guideline: HCP working with families should ensure awareness of clinical advances and develop means to easily share knowledge as it will strengthen hope for the future. Table 1 Table 1. Disclosures Bruce: Novartis: Consultancy, Honoraria; Apopharma: Consultancy.

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Sickle cell disease-related organ damage occurs irrespective of pain rate: implications for clinical practice

Haematologica ◽

10.3324/haematol.12152 ◽

2008 ◽

Vol 93 (5) ◽

pp. 757-760 ◽

Cited By ~ 22

Author(s):

E. J. van Beers ◽

C. F.J. van Tuijn ◽

M. R. Mac Gillavry ◽

A. van der Giessen ◽

J.-J. B. Schnog ◽

...

Keyword(s):

Clinical Practice ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Organ Damage ◽

Cell Disease ◽

Pain Rate

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Direct Oral Anticoagulants in Sickle Cell Disease, Where We Stand and Where We Are Heading: A Systematic Review

Blood ◽

10.1182/blood-2021-144694 ◽

2021 ◽

Vol 138 (Supplement 1) ◽

pp. 4188-4188

Author(s):

Waail Rozi Kashgary ◽

Elrazi Awadelkarim Awadelkarim Hamid Ali ◽

Alaa Rahhal ◽

Abdulrahman F Al-Mashdali ◽

Yousef Hailan ◽

...

Keyword(s):

Systematic Review ◽

Clinical Practice ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Observational Studies ◽

Oral Anticoagulants ◽

Direct Oral Anticoagulants ◽

Case Series ◽

Cell Disease ◽

Bleeding Events

Abstract Introduction: Sickle Cell Disease (SCD) is a hemolytic disorder with an increased risk of venous thromboembolism (VTE). By the age of 40 years around 11-12% of sickle cell disease patients will have at least one episode of VTE. VTE among patients with SCD is associated with a two to four times increase in mortality compared to SCD patients without VTE. Nevertheless, the evidence guiding VTE management in SCD, specifically in terms of anticoagulant choice, is scarce. Therefore, we conducted a systematic review that evaluates the effectiveness and safety of direct oral anticoagulants (DOACs) in SCD with VTE. Methods: We performed a systematic review following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. We searched the English literature (PubMed, SCOPUS, and Google Scholar) for randomized controlled trials, observational studies, reviews, case series, and case reports for patients with SCD treated with DOAC for thromboembolic disease. We used the terms in combination: "Sickle cell disease" or "Sickle cell anemia", and "DOAC", "rivaroxaban", "apixaban", "dabigatran" "edoxaban". The search included all articles published up to 20th April 2021. Quality and risk of bias assessment were done by two independent authors for each included study. Results: A total of 7 articles were included; four observational studies, and three case series addressing this matter. Patel A et al. found that the use of DOACs, including rivaroxaban, dabigatran, and apixaban in comparison to vitamin K antagonists (VKAs) and low molecular weight heparin (LMWH) for the treatment of VTE in SCD among adults was associated with similar VTE recurrence rate and a better safety profile in terms of a significant reduction in major bleeding events. Similarly, Roberts MZ et al. reported that the use of DOACs for VTE treatment in SCD compared to VKAs resulted in similar effectiveness in terms of VTE recurrence, but the use of DOACs was associated with a similar safety in comparison to VKAs in contrary to the results reported by Patel A et al. in their retrospective study. With regards to the risk of major hemorrhagic events associated with the use of non-VKAs, Gupta VK et al. showed that among 55 patients with SCD treated with VKAs, DOACs, or injectable anticoagulants, only patients treated with VKAs had major bleeding events. Discussion: The current data demonstrated that the use of DOACs for VTE in SCD has similar effectiveness in the prevention of VTE recurrence in comparison to other anticoagulants, including VKAs and injectable anticoagulants with a better safety profile. However, given the absence of clinical practice guidelines for the treatment of VTE among patients with SCD, the clinical practice guidelines recommendations for VTE treatment can be applied to patients with SCD. According to the latest CHEST guidelines (2016) for the treatment of VTE, the use of DOACs is recommended in patients with VTE over VKAs. Similarly, the latest American Society of Hematology (2020) guidelines for VTE suggest the use of DOACs over VKAs, except among patients with renal insufficiency (creatinine clearance less than 30 mL/min), moderate to severe liver disease, or those with antiphospholipid syndrome. Conclusion: In view of the current evidence and based on the results observed; using DOACs was associated with lesser bleeding incidence and fewer complications comparing to VKAs. We think it is rational to use DOACs for VTE treatment among patients with SCD rather than use VKAs. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.

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Benefits of a Disease Management Program for Sickle Cell Disease in Germany 2011–2019: The Increased Use of Hydroxyurea Correlates with a Reduced Frequency of Acute Chest Syndrome

Journal of Clinical Medicine ◽

10.3390/jcm10194543 ◽

2021 ◽

Vol 10 (19) ◽

pp. 4543

Author(s):

Joachim B. Kunz ◽

Andreas Schlotmann ◽

Andrea Daubenbüchel ◽

Stephan Lobitz ◽

Andrea Jarisch ◽

...

Keyword(s):

Clinical Practice ◽

Sickle Cell Disease ◽

Disease Management ◽

Sickle Cell ◽

Disease Management Program ◽

Treatment Guidelines ◽

Management Program ◽

Acute Chest Syndrome ◽

Insurance Company ◽

Cell Disease

Sickle Cell Disease (SCD) is the most common monogenic disorder globally but qualifies as a rare disease in Germany. In 2012, the German Society for Paediatric Oncology and Haematology (GPOH) mandated a consortium of five university hospitals to develop a disease management program for patients with SCD. Besides other activities, this consortium issued treatment guidelines for SCD that strongly favour the use of hydroxyurea and propagated these guidelines in physician and patient education events. In order to quantify the effect of these recommendations, we made use of claims data that were collected by the research institute (WIdO) of the major German insurance company, the Allgemeine Ortskrankenkasse (AOK), and of publicly accessible data collected by the Federal Statistical Office (Statistisches Bundesamt, Destatis). While the number of patients with SCD in Germany increased from approximately 2200 in 2011 to approximately 3200 in 2019, important components of the recently issued treatment guidelines have been largely implemented. Specifically, the use of hydroxyurea has more than doubled, resulting in a proportion of approximately 44% of all patients with SCD being treated with hydroxyurea in 2019. In strong negative correlation with the use of hydroxyurea, the frequency of acute chest syndromes decreased. Similarly, the proportion of patients who required analgesics and hospitals admissions declined. In sum, these data demonstrate an association between the dissemination of treatment guidelines and changes in clinical practice. The close temporal relationship between the increased use of hydroxyurea and the reduction in the incidence of acute chest syndrome in a representative population-based analysis implies that these changes in clinical practice contributed to an improvement in key measures of disease activity.

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Quality assessment of evidence-based clinical practice guidelines for the management of pregnant women with sickle cell disease using the AGREE II instrument: a systematic review

BMC Pregnancy and Childbirth ◽

10.1186/s12884-020-03241-y ◽

2020 ◽

Vol 20 (1) ◽

Author(s):

Yasser S. Amer ◽

Yasser Sabr ◽

Ghada M. ElGohary ◽

Amer M. Altaki ◽

Osamah T. Khojah ◽

...

Keyword(s):

Clinical Practice ◽

Sickle Cell Disease ◽

Pregnant Women ◽

Sickle Cell ◽

Clinical Practice Guidelines ◽

Practice Guidelines ◽

Evidence Based ◽

Cell Disease ◽

Agree Ii ◽

Agree Ii Instrument

Abstract Background The management of pregnant women with sickle cell disease (SCD) poses a major challenge for maternal healthcare services owing to the potential for complications associated with morbidity and mortality. Trustworthy evidence-based clinical practice guidelines (CPGs) have a major impact on the positive outcomes of appropriate healthcare. The objective of this study was to critically appraise the quality of recent CPGs for SCD in pregnant women. Methods Clinical questions were identified and the relevant CPG and bibliographic databases were searched and screened for eligible CPGs. Each CPG was appraised by four independent appraisers using the AGREE II Instrument. Inter-rater analysis was conducted. Results Four eligible CPGs were appraised: American College of Obstetricians and Gynecologists (ACOG), National Heart, Lung, and Blood Institute (NHLBI), National Institute of Health and Care Excellence (NICE), and Royal College of Obstetricians and Gynaecologists (RCOG). Among them, the overall assessments of three CPGs (NICE, RCOG, NHLBI) scored greater than 70%; these findings were consistent with the high scores in the six domains of AGREE II, including:[1] scope and purpose,[2] stakeholder involvement,[3] rigor of development,[4] clarity of presentation,[5] applicability, and [6] editorial independence domains. Domain [3] scored (90%, 73%, 71%), domain [5] (90%, 46%, 47%), and domain [6] (71%, 77%, 52%) for NICE, RCOG, and NHLBI, respectively. Overall, the clinical recommendations were not significantly different between the included CPGs. Conclusions Three evidence-based CPGs presented superior methodological quality. NICE demonstrated the highest quality followed by RCOG and NHLBI and all three CPGs were recommended for use in practice.

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