Top Back to top

Report of the Paediatric Diseases Working Party Educational Meeting on Haemoglobinopathies - 10-12 November 2022 - Regensburg

Paediatric Diseases Working Party (PDWP)
Nursing Paediatric Committee

Nurses Group Programme

Summary written by Hilda Mekelenkamp, Nurses Group Paediatric Committee Chair and PDWP Nurse; Marjola Gjergji and  Ida Ophorst-Bremer, Paediatric Committee Members; Trude Minnee, Speaker at the PDWP Educational Meeting; and Judith Timmermans, Paediatric Grant Winner.

We had a great meeting on haemoglobinopathies in Regensburg with an interesting parallel nurses track. Twenty-five nurses attended the meeting with the expectation to learn from each other and to discuss and exchange experiences. Nurses from eleven countries with experience in adult, pediatric, hematology and/or SCT, more or less experienced in HSCT nursing, participated actively in the sessions. Especially the easily accessible and enthusiastic way of discussing all topics together in this international context made this meeting a great success.

Session I (Part 1): Pre-transplant care

The first speaker of this session was Kelly Hennessy from UK. She talked about the “Supportive care in sickle cell disease” starting from introducing the sickle cell disease and going through numerous side effects, transfusion program, iron chelation drugs and outpatient’s support.

The second topic was the “Pain management in sickle cell disease”, explained by Regina Kulzer from Germany. Her talk was focused on how to manage the pain by medical and non-medical therapies like talk therapy and aromatherapy.

Third topic was performed by Lisbeth Andersson Lund from Sweden. She talked about the “Supportive care Thalassemia” bringing the experience from her center how they support and educate thalassemia patients through an educational program. She also explained the therapies used like exjade, desferal and ferripax and the side effects but also the importance of psychology and emotional support of thalassemia patients through specific activities.

The last talk was performed by Lawrence Faulkner from Italy/India who brought up an important issue like the “Infertility” as a crucial argument for all the Thalassemia patients who will undergo transplantation, involving all the attenders in free communication on the topic.

Session I (Part 2): Pre-transplant care

Hilda Mekelenkamp spoke about HSCT decision-making for hemoglobinopathy patients. She highlighted the importance of exploring patient’s preferences, needs, wishes and goals, taking time to discuss these preferences and the importance of including these while making an HSCT decision.

Sandrine Bremathas explained how patients are carefully prepared for HSCT. Several checklists support this preparation and included medical and psycho-social aspects.

Donor choice and donor care was discussed by Daphna Hutt. Choosing the best donor for our patients brings many considerations and a careful weighing of all these aspects.

Session II: Research session

Ida Ophorst-Bremer talked about ‘ Is there evidence to provide the best care’. She explained the definition of evidence-based nursing (EBN) and the three main principles: best research evidence, clinical expertise and patient’s perspectives and preferences. Ida guided us through the 5 steps of EBN and she illustrated this nicely with examples from her own practice.

Hilda Mekelenkamp explained how to write an abstract and why sharing your projects and/ or experiences is important. There are always several excuses for not writing an abstract, but each nurse can be proud of his/her work and it is worth presenting and discussing this with others.

During the last talk of this session Valentina Biagioli presented ‘how to present your research/project’. Valentina explained the importance of sharing your work, like passing on the benefits to others, to give an impact, to influence policy and to draw stakeholders’ attention. Different ways can be used like scientific/newspaper articles, books, presentations, posters, websites and educational sessions.

After these talks we continued with a nice group discussion, some of the attendees were experienced in presenting, where others were less experiences but very keen on doing this in the future. To support the integration of nursing research into our clinical practices, attendees responded that management commitment and time are important.

Session III: Clinical transplant care

Pain management during HSCT by Thaisa Zendath. An interesting talk which showed that the sensation of pain is different for every individual whereby biological (genes), psychological (coping skills, personality) and social factors (culture, religion) play a role. Doing a pain assessment at the start of the treatment is essential to find out the baseline and what could help for each individual patient.

Skin care during HSCT by Judith Timmermans. This presentation started with the introduction of a protocol with guidelines to give advice and care of the patient getting treosulfan/thiotepa. By frequently showering during and after treosulfan/thiotepa, no use of perfumed skin care products, skin problems decreased and less skin biopsies. More studies should be done to the treatment of pruritis (itchy skin). This is a common side effect, but options to relief the itch are not yet sufficient.

The special psychological & neurocognitive situation in SCD and B thalassemia patient by Elisabeth Kuhn-Wolff. Elisabeth showed us a neurocognitive assessment study before HSCT and one year after. The common intelligence was almost stable but it influenced the short-term memory and improved fine skills. The eye hand coordination worsened.

Nursing care in gene therapy by Matteo Amicucci. Matteo shared with us the gene therapy procedure. The history and rebirth of gene therapy in non-malignant diseases. He showed the clinical pathway and nursing and how to prepare and administer gene therapy in the “Italian” practice.

Case presentation gene therapy in Thalassemia by Caroline Aumeier & Ann Katrin Lang. This session was about a patient who got CRISP-CAS instead of a HSCT, because he was not suitable for HSCT. After aferese he got genetically modified bloodcells. In this case the patient had a diversity of side effects/complication like, skin problems, low platelets, mucositis, low oxygen.

Family perspectives on gene therapy for thalassemia (video) by Marjola Gjerjgi. An impressive story about a mother who was looking for therapy options for her two children after years of getting transfusion for their disease. Emanuele and Erica who did want a future without thalassemia. They managed to get gene therapy. They spoke about their anxiety and worries but happily it worked out very well for them and they have a “normal” live after gene therapy treatment.

Session IV: Post-transplant care

This session started with a video presentation from Julia Ruiz about the nurses’ role in late effects after a HSCT. Julia started with showing us data about the amount of transplantations and data about transplant indications. After this, Julia talked about the late effects after HSCT. There is a wide range of late effects with increased burden of serious chronic conditions and impairments involving organ systems and impacts on overall quality of life. Balance between cure-improvement and avoiding risk factors for late effects and toxicity is a challenge in transplant decision in hemoglobinopathies. The complexity of the late effects in HSCT survivors means that patients require life-long assessment guided by protocols. In conclusion, long term follow-up is an integral part of HSCT care, which ensures surveillance and intervention for early and late complications. It is important that long term follow-up consultation is coordinated and led by an nurse, integrated in a multidisciplinary team and to establish a patient-centered care pathway including a transition plan.

After this, Trude Minnee, gave a presentation about transition to adult care. She started with her take home message, which is that a nurse practitioner is a key player in the transition to adult care. She explained the late effects of a HSCT and why it is so important that there is a long-term follow-up. An important part about the long-term follow-up is that there is a good transition to adult care, so the follow-up continues. After this, she explained the core principles of good transition to adult care according to the Dutch guideline, which are patient centered care, teamwork, coordination and continuity of care, self-management and independence. It is important to start talking about the transition with patients that are 12-14 years old. And gradually give them more and more responsibility, so that they are ready for the transition to adult care.

This session was ended by a debate about communication challenges in pediatric nursing led by Hilda Mekelenkamp, Marjola Gjergji and Eugenia Trigoso. We started with the question ‘What makes nurses’ communication unique? Everybody agrees that nurses have a feeling for the needs of the patients and the family, ‘can read the patient’ and can adjust the communication to the level of the patient. After this there was a talk about the barriers in nurses’ communication. 41% of the group thinks that time is the biggest problem, followed by a language barrier. Then, there was a poll about using interpreters when facing language barriers. Every member of the session voted that they use an interpreter very often. Also, everybody agrees that in their hospital the services aligned with cultural differences, for example regarding diets/food habits.

Session V: Nursing Challenges

In the first session Eugenia Trigoso told us about the Global Education Concept (GEC) activities: ‘Reaching out globally through education’. In lower-and middle-income countries (MNIC) nurses faces challenges, including inadequate staffing, lack of support, limited access to education and unsafe practice environments. The success of a hematopoietic stem cell transplantation program relies on appropriately trained and experienced nursing staff. Therefore, the EBMT nursing group’s mission is to enhance and value the nurse’s role globally, supporting and sharing knowledge through communication, advocacy, research, training and education. They organize and coordinate HSCT nursing educational events and activities in MNIC. They work in collaboration with like-minded entities to foster and strengthen the ability of local nurse leaders to develop and sustain educational networks. And they creating a model not solely dependent on EBMT but that becomes part of the global community.

In the second session Eugenia Trigoso told us about the quality management in nursing. She told us all about the comprehensive quality management system ‘JACIE’ and how to apply them within the organization. The main principle in all this is teamwork. The staff-nurse (who is at the heart of the system) plays a vital role in de quality improvement of health care services. Inspectors are the backbone of JACIE, without them there is no accreditation process. Becoming an inspector is a wonderful way to contribute to maintaining global quality standards. Quality management inspectors and apheresis inspectors (nurses) are desperately needed!

In the third session Christoph Bauer & Tanja Kremer told us about the differences in nurses’ training in Europe. They have compared the nurses’ training in Germany with France, Switzerland, Austria and Great Britain. The nurses’ training is different in each country. Due to the new training in Germany, the trainees are used in fewer areas of pediatrics. The operating time in practice is significantly reduced. In order to compensate for this deficit, trained and comprehensive training is required in Germany in order to be able to adequately care for patients and families in pediatric hematology and oncology. There is a training after the three years of apprentices, for 6 months. Training by a qualified practical instruction for nursing one by one. In this time the instructor can change to prevent from unilateral and depending on one person. They also aid a tool: specialized guidelines for pediatric oncologic wards with stem cell transplantation.

An interesting discussion started.

In the last session there was time to debate ethical issues. First, we heard about the norms and values and how they influence our moral values in healthcare. We also learned how a moral value can become a moral conflict. An interesting ethical issue and discussion started based on a case presented by one of the attendees.