ABSTRACT
Background: After two years of COVID in which activities were reduced due to the pandemic and each one's life was affected by restrictions and limitations, the Sickle Cell Disease (SCD) Association in Padova teamed up with the Sickle Cell Group at the Pediatric Hematology Oncology and Bone Marrow Transplant Unit to celebrate the Sickle Cell Disease world day by organizing an online meeting with children/youths and their families. Theme of the meeting was: "My Life with SCD: poems, pictures and writings express our view on disease and care". Aim(s): One of the goals of this meeting was to create an opportunity for individuals with SCD to meet and have a constructive discussion with each other about the disease and express their feelings after two years of pandemic. Method(s): One month before the meeting children, teenager and parents were asked to sharer with the organizing team any drawing, painting, poem, writing, that they felt could express their feelings or experience of the disease itself or how it affected their life, or their experience in the hospital. The materials received were organized in a power point presentation and At the meeting, families were able to see a PowerPoint presentation with the poems, drawings, writings. Each author had the choice to personally share their production or have it read out loud by a member of the team. Free time to comment or share experiences was given. Result(s): 20 children, teenagers and parents participated. Countries of origin (Nigeria, Ghana, Congo, Albania, Italy), religious background (catholic, muslim, no religion, other) were different as well as disease genotype (HbSS, HbSC, HbSBdegree), severity or treatment received (Hydroxyurea, transfusion, Hematopoietic stem cell transplantation -HSCT, none). Drawings and writings regarded experience with the disease (mechanism of action, admissions), feelings experienced (fear, hope, light at the end of the tunnel), aspirations (sports) and gratitude (to the social and medical team, to parents) (Figure 1). Surprisingly, families who had a child having undergone HSCT, reported on the need and importance to talk about this experience for years after the event and made a request of a support goup. Finally, all families underlined the need to meet again soon to discuss together issues related to personal experience with SCD, even via web. of discussion with each other and with the drepanocytosis group;and that throug the online telematics platform it is still possible to involve all families, listening and trying to comfort them on doubts and perplexities about the disease, In conclusion, it can be said that after two years of pandemic, in our setting, online meeting can help patients and families reconnect with each other and activities can be planned to aid experiences and feelings. Patients' associations and Health Care Teams can collaborate in this area.
ABSTRACT
[Formula presented] Background In spite of the improvements in clinical care of children with Sickle Cell Disease (SCD), painful vaso-occlusive (VOC) crises, recurrent admissions and long hospital stays contribute to the disruption of the social and school life of children and adolescents with SCD causing a poor QoL. Limited information is available regarding QoL of children, adolescents and young adults with SCD and their caregivers during standard of care and after bone marrow transplantation in Italy even though Italian patients participating in international meetings or global surveys highlighted the importance to improve QoL (Strunk C. BMC Proc. 2020, Osunkwo I. Am J Hematol. 2021). Moreover, no mention is given to QoL in the current AIEOP Recommendations for the Management of Children with SCD in Italy. The availability of new treatment options for SCD highlights the need to improve QoL evaluation before and after treatments. Our Center decided, therefore, to include QoL evaluation as part of comprehensive care for patients with SCD. This study has the following aims: to describe the QoL of children, adolescents and young adults with SCD undergoing standard care or after disease curative treatments (bone marrow transplantation) and the QoL of their caregivers;to evaluate the correlation of QoL with clinical-haematological and therapeutic variables. Methods Health Related QoL was examined with the Patient-Reported Outcomes Measurement Information System (PROMIS) questionnaires in Italian: Parent Proxy Profile-49 v2.0, Pediatric Profile-49 v2.0 and 57 Profile v2.1, exploring 8 domains: Pain, Fatigue, Anxiety, Depression, Physical Abilities, Peer Relations, Sleep Disorders, Pain Interference. An English version was available for English speaking parents. Patients and caregivers accessing the SCD Clinic starting May 2021 were given the paper version of the questionnaires;due to the COVID pandemic and the limited access to the SCD Outpatient Clinic, a link to a Google online version of the questionnaires was provided to all teenagers and young adults, through their mobile devices. PROMIS Scores were standardized through the Health Measures Scoring Service (healthmeasures.net). For the descriptive analysis, the T-score was obtained, for each patient and for each PROMIS domain (symptom or function), classifying impairment in each domain as normal, mild, moderate, or severe. The Student T Test for comparisons of the means among samples and the Wilcoxon Test for the sum of ranks were used in the statistical analysis of normal and non-normal continuous variables. For the correlation analysis between continuous variables of which at least one is not normal, the Spearman Correlation Test was used. The values with p<0.05 were considered statistically significant. Results All patients and parents approached accepted to perform the questionnaire. The study involved 18 caregivers and 50 patients (25% F, mean age 16.4 years, 74% HbSS, 76% from Africa): 41 undergoing standard care (7 no therapy, 34 Hydroxyurea or chronic transfusion) and 9 who received HSCT. 37 patients (74%) and 8 parents (44%) completed the online Google version of the questionnaire. The standard of care patients displayed mild to severe symptoms in various domains (Figure 1A);in the transplant population there was impairment in QoL, with less severe impairment in most of the domains, especially in the pain domains, than what was in the standard of care group. (Figure 1B). Anxiety levels and depressive symptoms were greatest between the ages of 14-26, compared to younger ages (p 0.018). Parents do not have the same perception of the disease as their children: they appeared to overestimate the domain of pain and fatigue and underestimate anxiety and depression (p <0.001). Sleep quality was impaired in both affected and HSCT patients. The number of hospital admissions in the previous year correlated with worse QoL (p 0.04), while the number of painful VOC showed a tendency towards significance (p 0.07);there was no difference with the other domains. Update results will be presented. Conclusions Our data show the feasibility of evaluating QoL during routine visits and also remotely. Impairment of QoL is already present in a subgroup of young patients. Even after HSCT, QoL is not optimal but personal, social, and economic reasons need to be taken into account to adequately interpret the results. Longitudinal assessment to look at QoL will be important. [Formula presented] Disclosures: Biffi: BlueBirdBio: Consultancy, Other: Advisory Board. Colombatti: Global Blood Therapeutics: Consultancy;BlueBirdBio: Consultancy;NovoNordisk: Consultancy;Novartis: Consultancy;Forma Therapeutics: Consultancy;Addmedica: Consultancy;Global Blood Therapeutics: Research Funding;BlueBirdBio: Research Funding.
ABSTRACT
Background: In the past year the COVID19 Pandemic and the repeated lockdown measures have disrupted people's life. Teenagers with chronic illness, such as Sickle Cell Disease (SCD) present special health care and educational needs during the delicate phase of transition to adulthood compared to their peers. The Veneto Region, in North East Italy, was one of the first red zones areas in Europe since February 26th 2020 with Phase 1 of strict lockdown until April 30th 2020;Phase 2 with progressive reopening of activities during the summer;Phase 3 since September 2020 with initial high school opening but successive implementation of risk zones across the country with regional limitation of movements, economical activities, social life and education according to the diffusion of COVID19 in the area. Since November 2020 high school students were forced to stay at home and perform virtual classes;only individual sports were allowed. Aims: We analyzed the psychological impact of the restrictions and isolation measures on teenagers and young adults with SCD in the Veneto Region of Northern Italy during the 3 different phases of lockdown while offering a dedicated telephone service of psychological support. Methods: A dedicated telephone service was organized since March 2020 by a Psychologist of the SCD Team to contact families and offer support. Adolescents and young adults were contacted directly. Telephone calls occurred during Phase 1 (strict isolation), phase 2 (progressive reopening) and the current phase 3 (alternation of openings and closures) and were characterized by a set of standardized questions tailored to the phase followed by an open dialogue. A qualitative description is presented. Results: 28 adolescents responded to the phone calls and agreed to be called again. Psychological experiences differed according to the phase of the lockdown. In phase 1, in which the limits to personal freedom were high, they felt equal to the general population and felt confident that this painful period would soon end. In phase 2, due to loosen restrictions, greater concerns and anxiety emerged: the need to coexist with the virus and the continuous changing of the rules generated a greater perception that a sudden painful event could break into their life at any time, increasing personal discomfort and undermining the quality of life. Phase 3 showed further different responses. 23/28 stated that they resumed school or work activities, although in different ways (virtual classes or smart working). All reported feeling accustomed to, and therefore less anxious, about covid-19. All claimed to respect the rules, to know them well, and to apply them. 20/28 patients reported spending most of their time at home, were not interested in going out and didn't have social interactions. 6/28 teenagers requested specific psychological support in the form of weekly phone calls or zoom calls with the SCD psychologist, which is still ongoing. Most of them are girls who asked for help in managing their anxiety and the fear that they won't be able to realize their expectation in professional or family life. Summary/Conclusion: The dedicated telephone service for psychological support was well received by teenagers with SCD with a subgroup requesting a repeated intervention. The qualitative results of our experience demonstrate the need for psychological support during the pandemic in patients with chronic disease;remote support has proven to be an effective tool.