Music Therapy: Sickle Cell and Pain Crisis

Background: Hydroxyurea (HU) therapy in sickle cell anemia (SCA) improves health care utilization, slows organ failure, and prolongs life. Implementation of evidence-based, comprehensive care has been shown to improve health-related quality of life (HRQOL). Case management by community health workers (CHWs) is an evidence-based health management strategy. We therefore hypothesized that HU-eligible SCA adults exposed to patient navigators (PN), CHWs specially trained as case managers for SCA, would have improved HRQOL compared with controls. Methods: We enrolled 224 patients eligible for HU into the Start Healing in Patients with Hydroxyurea (SHIP-HU) Randomized Controlled Trial. All patients received care from trained physicians who implemented use of a standardized HU prescribing protocol using NIH guidelines. Pateints were randomized to either PN intervention (which included case management and education through home, telephone, and/or other visits from PNs) plus standard care by their treating physician (Experimental, E), or standard care by their physician alone (Control, C). Study physicians were blinded to study arm. At baseline, 6 and 12 months we assessed 4 psychosocial HRQOL variables-- ASCQ-Me emotional impact (EMOT), social impact (SOCI), PROMIS global mental (GMENT) and satisfaction with social roles (ROLE); 6 Physical HRQOL variables-- ASCQ-Me Sleep impact (SLEP) and Stiffness (STIFF), PROMIS global physical (GPHYS), Physical health (PHYS), Fatigue (FATG), and sleep/Wake disturbance (WAKE), and 4 pain HRQOL variables-- PROMIS pain behavior (PAINB), and ASCQ-ME-Pain crisis frequency (PAINF), Pain crisis severity (PAINS), and Pain impact (PAIN). Main analyses consisted of mixed model analysis of variance of follow-up visits, controlling for site and baseline value of outcome variable. Any missing baseline values for subjects were imputed. Results: 181 of 224 randomized patients had at least one HRQOL measure at follow-up. Patients had mean age 30.3, 45.3% were male, 81.2% were on HU at baseline. No HRQOL measures were different between groups E and C in any domain (Table, variables grouped by domain). Conclusions: In our sample, there were no differences in HRQOL among patients who were exposed to PNs vs those who weren't. These findings require further analyes before firm conclusions can be made about the isolated effect of PNs on HRQOL. PN dose of intervention was likely variable. HU use and adherence has been associated with higher HRQOL, and we did not predict high baseline HU uptake and adherence which may have led to minimal improvement despite adequate PN intervention. PNs were not allowed to work with MDs, nor did they work with the remainder of the health care team to improve HRQOL. Analyses are underway to examine these and other possible influences on HRQOL. Table. Disclosures Smith: Novartis: Consultancy, Honoraria. Villella:Emmaus: Membership on an entity's Board of Directors or advisory committees; Pfizer: Other: Site PI for the Rivipansel Clinical Trial. Liles:Novartis: Other: PI on clinical trial Sickle cell ; Shire: Other: PI on clinical trial Sickle cell ; Imara: Other: PI on Clinical trial- Sickle cell .

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ADULT SICKLE CELL ANAEMIA PATIENTS IN BONE PAIN CRISIS HAVE ELEVATED PRO-INFLAMMATORY CYTOKINES

Mediterranean Journal of Hematology and Infectious Diseases ◽

10.4084/mjhid.2018.017 ◽

2018 ◽

Vol 10 (1) ◽

pp. e2018017 ◽

Cited By ~ 4

Author(s):

Adekunle Emmanuel Alagbe ◽

John Ayodele Olaniyi ◽

Oladapo Wale Aworanti

Keyword(s):

Steady State ◽

Sickle Cell ◽

Plasma Levels ◽

Bone Pain ◽

Sickle Cell Anaemia ◽

Cross Sectional Study ◽

Cross Sectional ◽

Tnf Α ◽

Pain Crisis ◽

State Group

Background and Objective: Inflammatory markers that influence bone pain crisis (BPC) and other complications of sickle cell anaemia (SCA) are numerous and play various roles. This study determined the plasma levels of tumour necrosis factor (TNF) - α, interleukin - 8 (IL-8), and endothelin - 1 (ET-1) in adult SCA patients during BPC and in steady state. In addition, the plasma levels of these cytokines were correlated with the severity of BPC of the patients.Methods and Materials: Sixty adult SCA patients (30 during BPC and 30 during steady state) and 30 haemoglobin A controls were enrolled for this cross-sectional study. The severity of BPC was assessed clinically, and questionnaires were filled. Plasma levels of TNF- α, IL-8 and ET-1 were quantified by ELISA, and haematological parameters were determined using a 5-part auto-analyzer. Plasma levels were correlated with the severity of bone pain crisis. Results were considered statistically significant if p<0.05.Results: Plasma TNF-α, IL-8, and ET-1 were significantly elevated in the BPC group than in the steady state group and the controls. Plasma TNF-α, IL-8 and ET-1 were markedly higher in the severe BPC groups than the steady state and control groups, There was a positive correlation between TNF-α and ET-1 in the bone pain crisis group.Conclusion: Elevated levels of plasma TNF-α, IL-8, and ET-1 further establish the chronic inflammatory state in SCA and equally affirm their significant contribution, not only to pathogenesis but also to the severity of pain in SCA. Keywords: Sickle cell anaemia, Cytokines, Bone pain crisis, Severity, Steady state.

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P10 An audit to assess the prescribing of analgesia in children who present with pain crisis due to Sickle Cell Disease (SCD)

Archives of Disease in Childhood ◽

10.1136/archdischild-2020-nppg.19 ◽

2020 ◽

Vol 105 (9) ◽

pp. e11.1-e11

Author(s):

Masuma Dhanji

Keyword(s):

Sickle Cell Disease ◽

Integrated Care ◽

Sickle Cell ◽

Care Pathway ◽

Electronic Prescribing ◽

List Type ◽

Cell Disease ◽

Paediatric Patients ◽

Pain Crisis ◽

Integrated Care Pathway

AimTo assess the prescribing of analgesia to manage pain crises in children with SCD. This was to establish whether the Trust was meeting national and local standards. Prompt pain control is essential to reduce length of stay and further complications.1Standards100% of admissions will be prescribed regular paracetamol and non-steroidal anti-inflammatory drugs (NSAIDs) at the recommended frequency unless contraindicated in accordance with national guidance.2 3100% of admissions will be prescribed appropriate doses of analgesia with consideration to weight and age in accordance with local policy.4MethodThe audit was registered with the Trust’s audit committee. A list of paediatric patients with the diagnosis of SCD was sought from paediatricians with an interest in haematology. A data collection form was created. Data was collected retrospectively over a one-year period. A total of 60 admissions were reviewed to check whether analgesia was prescribed regularly at the recommended frequency, and at the correct dose. Results were analysed using descriptive statistical analysis. Exclusion criteria included patients with hospital admissions under 24 hours.ResultsA total of 55 admissions were included in the final sample. The audit showed the Trust was non-adherent to both standards assessed. A total of 45% (95% CI [31.9%, 58.1%]) of admissions were prescribed regular analgesia. A total of 78% (95% CI [67.9%, 88.9%] of admissions were prescribed appropriate doses of analgesia. Two main reasons were found as to why analgesia was prescribed at the incorrect dose. This was due to incorrect weights recorded on the electronic system (n=4) and doses based on age only (n=8).ConclusionThe results show prescribers are familiar with the correct doses of analgesia but fail to prescribe analgesia regularly. This highlights an opportunity for education and training in the management of pain crisis in SCD. One recommendation includes development of an integrated care pathway booklet for paediatric patients presenting with pain crisis due to SCD. Integrated care pathway booklets have been implemented for other conditions such as cystic fibrosis yielding positive outcomes. The results have highlighted key issues surrounding the electronic prescribing system such as out-of-date weights remaining on the system unless updated, and default treatment protocols. The electronic prescribing system requires refinement for use within paediatrics. One suggestion includes compulsory weight field on admission. Limitations of this audit included small sample size. There was a lack of data to make suggestions based on different ages.ReferencesRees D, Olujohungbe A, Parker N, et al. Guidelines for the management of the acute painful crises in sickle cell disease. Br J Haemato 2003;120:744–752.National Institute for Health and Care Excellence (2012) Sickle cell disease: managing acute painful episodes in hospital. NICE Guideline (CG143).Paediatric Formulary Committee. BNF for Children (2018–2019). London: BMJ Group, Pharmaceutical Press, and RCPCH Publications; (2018).General Hospital (2015) Management of sickle cell disease in paediatric patients (CG377).

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Impact of Hydroxyurea on Thrombin Antithrombin Complex and Vaso-Occlusive Crisis in Pediatric Sickle Cell Disease.

Blood ◽

10.1182/blood.v108.11.5525.5525 ◽

2006 ◽

Vol 108 (11) ◽

pp. 5525-5525

Author(s):

Mohsen Saleh Elalfy ◽

Ashraf M. Abdelmonem ◽

Soha Youssef ◽

Heba Ismail

Keyword(s):

Sickle Cell Disease ◽

Blood Transfusion ◽

Sickle Cell ◽

Coagulation System ◽

P Value ◽

Cell Disease ◽

Significant Difference ◽

Healthy Control ◽

Pain Crisis

Abstract Background: Several studies suggest that increased activity of the coagulation system may be important in the pathogenesis of vascular occlusion in sickle cell disease. Hydroxyurea (HU) has been shown to reduce the frequency of vaso-occlusive manifestations in both adults and children with sickle cell disease (SCD). Aim: To analyze the effect of HU on Thrombin-Antithrombin (TAT) as a marker of thrombin generation and hypercoagualbility in SCD and to find out the relation between TAT level and vaso-occusive crisis. Subjects and Method: we evaluated 37 child with sickle cell hemoglobinopathy (mean age 10.92±5.39 years) and 15 normal control children (mean age 9.75±6.34 years). Informed consent was obtained from patients and/or guardians and study approval by local IRB was obtained. Twenty-two patients (59.5%) were on HU, 15 (41.5%) patients did not receive HU, 7 (46.7%) of them were transfusion dependant. TAT assay was done in vitro using a sandwich enzyme immunoassay. Results: Mean patients’ age at institution of HU was 8.54± 3.85 years with median treatment duration of 4.5 years. Causes for initiating HU therapy were frequent blood transfusion in 11 patients (50%), frequent pain crisis (≥ 3/year) in 9 patients (41%), severe anemia and parents refusing blood transfusion in 1 patient (4.5%) and stroke in another patient (4.5%). HU dose was 20.82±4.95 mg/kg/day. We measured TAT in all patients and compared them to healthy control. There was significant difference in TAT level in sickle cell patients (198.86±185.7) compared to healthy control 2.91±0.94, [P value < 0.0001]. When the level of TAT was compared between the HU and non-HU groups we found that patients on HU had statistically significant lower TAT level (172.36 vs.225.37) [P=0.039]. There was also a significant negative correlation between HU dose and TAT level (p=0.03). A significant positive correlation between number of vaso-occlusive crisis/year [P=0.03], frequency of pain crisis/year [P=0.04], duration of pain crisis [P=0.03] and TAT level was observed. Conclusion: Hydroxyurea has significant inhibitory effect on thrombogenesis in sickle cell patients, which may be another mechanism for reducing vaso-occlusive crisis. Sickle cell children with higher TAT level had more frequent and severe vaso-occlusive crisis.

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