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Improving care for thalassemia patients in line with best practice standards at a tertiary referral cancer care center

Bongay, Leilani RN, BSN, DipMgt, MAN (c)1; Kynoch, Kathryn RN, BN, MN, PhD2

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JBI Evidence Implementation: June 2022 - Volume 20 - Issue 2 - p 128-133
doi: 10.1097/XEB.0000000000000283
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What is known about the topic?

  • Thalassemia is a chronic disease that requires life-long management.
  • Thalassemia patients are receiving care in a tertiary referral cancer care center.
  • Nurses must understand the patient's condition and the specific complexity of caring for patients with thalassemia to ensure patient-centered quality care is provided.

What does this article add?

  • Patients need an individualized treatment plan, as their medical status and comorbidities vary.
  • The implementation of local guidelines ensures that care is safe and based on the best available evidence.
  • Specialized thalassemia education is needed to support current practice and should be based on recent research and evidence.


Thalassemia describes a group of inherited and autosomal recessive hemolytic anemias, including inadequacies in the unification of either the alpha or the beta-globin chains of hemoglobin (Hb).1 As a result of spontaneous genetic mutation, the globin chain decreases in number or is even eliminated, which results in the abnormal development of red blood cells and, eventually, anemia.2 In populations where malaria is (or was) endemic, the occurrence of Hb disorders, including thalassemia, ranges from 0.3 to 25 per 1000 live births.3 Other reasons for this high number of cases include the practice of kin marriage, accrued maternal age, gene drift, and founder effect.4 For these reasons, thalassemia is most common in South-Eastern, Central and Southern Asia, the Mediterranean, the Middle East, and North and Central Africa. In Australia, thalassemia has risen over the years due to relentless migration.4

Beta-thalassemia major or transfusion-dependent thalassemia (TDT) is the homozygous form of thalasse-mia.5 This mutation results in the total absence of beta-globin chains, which causes severe anemia.5 Patients with this type of thalassemia require life-long treatment with regular blood transfusions to manage anemia.6 Frequent blood transfusions can lead to iron overload and toxicity, causing cardiac siderosis, pulmonary hypertension, and endocrinopathy.7 Iron chelation therapy is necessary to remove excessive iron from the body, preventing iron overload.7

Nurses caring for patients affected with beta-thalas-semia major must be equipped with knowledge and skills to monitor and manage this disease and keep up-to-date with current evidence-based practice.8 As nurses are often delegated to be patient advocates, it is imperative to understand the patient's condition and the specific complexity of caring for patients with thalassemia to ensure patient-centered quality care is provided. However, there were varying levels ofnursing knowledge of best practice thalassemia care in the tertiary referral cancer care center where this project was set.

While there are several well developed international evidence-based guidelines to guide thalassemia patient care,4,9 there were no local guidelines at this project site to guide care for this patient group.


The aim of the project was to improve the care of thalassemia patients in line with evidence-based guidelines.



The current project utilized a clinical audit design and was conducted between June 2019 and June 2020. A pre-implementation audit was carried out following current best practice guidelines for the care of patients with thalassemia. Following the baseline audit, strategies to align clinical practice with best practice were implemented. A post-implementation audit was conducted to determine the success of implementation strategies and changes in clinical practice.


The current project was conducted in a tertiary referral cancer care center within a tertiary hospital. The center caters to both public and private adult patients with various cancer and blood disorders, including hemoglo-binopathy, such as thalassemia. Patients receive specialized in-depth treatment and care from expert hematologists and specialist nurses, including patient diagnoses, assessment, patient management, clinical trials, and survivorship. The center is the referring center for thalassemia patients from other regions and remote geographical locations.


The sample for this project was thalassemia patients and the nurses caring for these patients. The project's total patient sample was nine, and the same patients were audited pre and post-implementation (pre-implementa-tion N= 9; post-implementation N= 9). This sample was determined based on the total number of thalassemia patients currently receiving care at the cancer center. The total number of nursing staff who participated in the project was 48 (pre-implementation N= 22; post-implementation N = 26). This sample was determined based on the number of nursing staff who delivered direct patient care for these patients.

Development of evidence-based audit tool

Audit criteria were developed from the best practice guidelines of the UK Thalassaemia Society.9 The standards were developed to guide the care of patients with TDT or thalassemia major and non-TDT. These guidelines are extensive and cover both the nursing and medical management of patients with thalassemia.

The five audit criteria below were developed from the guidelines as an initial focus to ensure consistency in the care of thalassemia patients:

  • (1) Patient received thalassemia information/education during their annual review appointment.
  • (2) Patient has a documented individualized 12-month treatment plan.
  • (3) Thalassemia clinical guidelines are in place to guide care for staff.
  • (4) Patient received copy of annual treatment plan.
  • (5) Staff received thalassemia education within the last 12 months.

Data collection

Patient data for the pre-implementation audit were collected retrospectively for June 2019, and the postimplementation audit was performed in June 2020. The data collection for each audit occurred over 1 month, during which nine thalassemia patient charts were reviewed against the audit criteria. Data were collected from the patient chart for criteria 1–4; for criterion 5, nurses were asked whether they had received thalasse-mia education in the previous 12-month period. The same audit criteria were utilized during the pre-imple-mentation and post-implementation audits to ensure consistency.

Ethical considerations

No data identifying individual nurses and patients were collected for this project. Ethical exemption was granted by the hospital's Human Research Ethics Committee, and the privacy office approved the project, which aligned to organizational governance requirements.

Identifying barriers and strategies to change practice

Following the evaluation of the pre-implementation audit results, barriers to achieving compliance with the audit criteria were identified. Strategies to improve the care of the patients consistent with the best practice recommendations9 were then implemented. Insufficient thalassemia staff education, the absence of local clinical guidelines, and no individualized annual care plans for patients were the main barriers to best practice. Strategies to overcome these identified barriers were then implemented.


Pre-implementation audit

The pre-implementation audit showed there was low compliance with criteria 1–4. There was no documented evidence that patients had received education during their annual review appointment (criterion 1; N = 0). Furthermore, there was no documented individualized 12-month treatment plan for each patient, and a copy of the annual review consultation including a care plan was not provided (criterion 2; N = 0 and criterion 4; N = 0). There was no local clinical guideline in place to guide care for thalassemia patients in this setting (criterion 3; N = 0).

For criterion 5, only 45% (n = 10) of the nursing staff had received thalassemia education within the last 12 months despite 100% (n = 22) of the staff expressing their interest to learn more about thalassemia. Among the 22 nursing staff, 77% (n = 16) had provided care to thalassemia patients over the past 12 months.

Post-implementation audit

Over 12 months, strategies were implemented to improve staff education using three approaches: creating a staff education folder, the delivery of education sessions for nursing staff, and the creation of thalasse-mia-specific nursing assessment and documentation template.

The project lead (L.B.) collaborated with the hospital's specialist oncology electronic medical record system digital information officers to produce a new and specific template for thalassemia nursing assessment and documentation. The content of the template was reviewed and approved by the senior nursing and medical staff.

The staff education folder was placed in the department's nurses’ station for easy access. The project investigators also delivered two face-to-face education sessions for nurses working in the cancer center. Topics were ‘What is thalassemia, and it's management?’ and ‘How to use the thalassemia template in CHARM?’. Evidence-based learning tools were utilized to deliver these education sessions.

The Senior Medical Officer (SMO) developed the TDT Management Plan form. The form can be accessed electronically via the hospital's internal document center. It can be edited and saved as a portable document format file and then uploaded in the hospital digital document scanning clinical portal for easier access by the multidisciplinary team involved in inpatient care. The management plan contained the following information: individual patient transfusion program, iron chelation dosing, routine monitoring, and attendance to various medical specialties, splenectomized patient plan, recent progress notes/issues, and issues to follow up. After the SMO completed the form, a hard copy was provided to the patient, and an electronic copy was uploaded to the patient's clinical health record electronic portal.

The post-implementation audit demonstrated an increase in adherence across all criteria, particularly for criterion 5: staff education increased from 45 to 92% (Figs. 1 and 2). For criterion 1, 100% (N = 9) of patients received thalassemia information/education during their annual review appointment; this was documented in the progress notes. Patients verbally confirmed that they received information from the medical specialist during their subsequent nursing assessment. There was also 100% (n = 9) compliance with criteria 2 and 4, with all patients having a documented individualized 12-month treatment plan. A copy of the individualized care plan was given to the patients and their respective general practitioners (GPs). For criterion 3, local multidisciplinary clinical guidelines were developed to guide thalassemia patient care.

Figure 1:
Improving thalassemia care.
Figure 2:
Staff education.

Approximately 8–10 nurses attended each of the face-to-face education sessions. Out of 26 nurses, 17 (65%) participated in all sessions and had accessed the education folder. Six months after implementing the thalassemia nursing assessment and documentation template, an audit showed that the template was utilized on 46 (84%) occasions. Out of these 46 occasions, 41 (75%) were complete, whereas five (9%) had missing data.


Overall, this project was successful in improving the care for thalassemia patients in line with best practice guidelines at a tertiary referral cancer care center.

Individualized treatment plan

The project results demonstrate that ensuring an evidence-based approach to care improves the consistency of care provided to thalassemia patients. An identified barrier to best practice from this project was the lack of individualized treatment plans for this patient group. As thalassemia is a chronic life-long disease, treatment needs to cater to individual patient needs.10 Individualizing treatment plans ensures patient preferences are identified and patient wishes are being met.11 In addition, a study conducted by Castella-Creus et al.10 revealed that individualization of care increased nurses’ satisfaction, leading to more involvement in care planning, higher quality patient care and better patient outcomes. This finding is further supported by another previous study whereby individualized care plans resulted in improved quality of life for the patient.12 In this quality improvement project, introducing individualizing care plans increased the patients’ knowledge of their disease and resulted in greater involvement in their care.12 The complexity of care required for these patients should be considered when developing treatment plans, as the provision of these plans kept both the patientand their GP up-to-date with current medical management.10

Clinical guidelines

Multiple international resources were utilized from published standards of care, international guidelines, specialized journals, and numerous well known thalassemia organizations to develop local clinical guidelines for this project. The need for local clinical guidance is recommended by Panteli et al.13 as this helps to ensure consistency in the care that is provided and minimize risk to the patient. However, research from Queen's University, Canada, has shown that clinical guidelines in isolation do not guarantee a change in healthcare professionals’ attitudes or patient outcomes.14 Effective dissemination and implementation strategies are crucial for local guidelines to impact healthcare delivery and outcomes.13 For this project, a multiple disciplinary team, including the hemoglobinopathy medical officer, was involved in developing local guidelines for clinical practice. An expert panel was asked to review the guidelines. The panel consisted of two senior hematologists, three senior hematology nurses, and the quality coordinator for blood and apheresis services. This process for developing and reviewing the guidelines was essential to ensure that the content was relevant and encourage implementation. According to Reich et al.,15 local facilitators must support guideline implementation and include leadership, education, and healthcare providers’ collaboration.

Nurse education

Knowledge of a disease is an essential factor in caring for patients. Therefore, nurses must understand the patient condition to ensure good professional practice.16 In this project, the strategies utilized to improve thalassemia nurses’ knowledge focused on the implementation of educational programs and the development of a staff education folder. In this current project, one-third of the nursing staff did not attend the thalassemia education sessions due to increased clinical work demands, high acuity of hematology and cancer patients in the center, lack of interest in the topic, and day off schedule. Further education sessions are scheduled to overcome some of these barriers.

Overall educational interventions implemented in this project improved nurses’ attendance at thalasse-mia-specific education sessions, as reflected in the postimplementation findings. While this project did not specifically measure nurses’ thalassemia knowledge, the attendance results were similar to a study conducted in Egypt, which reported that educational programs positively impacted nurses’ knowledge and practice related to thalassemia, improving the quality of nursing care, and increased patient satisfaction.17

Nursing assessment and documentation

Thalassemia-specific documentation was used in this project to measure patient compliance, tolerance to side effects, and therapeutic response to iron chelation therapy. This approach has been successful in a previous evidence implementation project conducted by Kelly et al.18

While all thalassemia patients did receive an individualized treatment plan, this project highlighted that a quarter of the nursing staff within the cancer center did not correctly complete the template with all relevant patient information. For example, not all nursing staff asked for current chelation therapy dose and patient's adherence to the treatment plan, as evidence by incomplete documentation in the thalas-semia template. This project's results are supported by previous studies that found that improving nursing documentation can be difficult and require investigator guidance and education to achieve compliance.19 Therefore, consistency in educating the nursing staff and follow-up audits are required to ensure sustained success.


In summary, thalassemia is a chronic disease that requires life-long treatment. As patients receive treatment in a cancer care center, thalassemia-specific strategies are required to ensure high quality, consistent care. This project has highlighted that patients need an individualized treatment plan, as their medical status and comorbidities vary. In addition, the implementation of local guidelines ensures that care is safe and based on the best available evidence. Specialized thalassemia education is needed to support current practice and should be based on recent research and evidence. Finally, further education and training for all staff involved in the care of thalassemia patients are essential.


The authors gratefully acknowledge Dr Mimi Yue, Laura Rodwell, and Claire Kelly for their inputs in the project. The authors would also like to thank Isaac Stiria for his contribution in the project planning, chart review, and implementation of staff education.

Ethics approval and consent to participate: Exemption granted by Mater Misericordiae HREC Office (application: EXMT/MML/54580 (V2); project: 54580).

Consent for publication: All authors consent to publication of the article.

Availability of data and materials: Available on request.

Conflicts of interest

None disclosed.


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clinical guidelines; hemoglobinopathy; individualized treatment plan; nursing; thalassemia

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A video commentary on implementation project titled: How do health professionals prioritise clinical areas for implementation of evidence into practice? The commentary is provided by Andrea Rochon RN, MNSc, Research Assistant, Queen's University, Ontario, Canada