Disease burden and quality of life in children with sickle cell disease in Italy: time to be considered a priority

Abstract

The objective of the present article is to highlight the need for attention to Quality of Life of patients with Sickle Cell Disease living in Italy. The transformation of sickle cell disease from a severe life-threatening disease of childhood into a chronic, lifelong condition due to the significant improvements in care and treatment options, imposes increasing new challenges to health care providers and patients. Patients now face physical, psychosocial and emotional challenges throughout their lives. They generally have to receive chronic treatments and regular multidisciplinary monitoring which increase social and emotional burden rendering adherence to treatment sometimes complicated. A chronic disease impacts all aspects of patients’ lives, not only the physical one, but also the social and emotional aspects as well as the educational and working life. The entire “Quality of Life” is affected and recent evidence demonstrates the importance quality of life has for patients with chronic illness. The results of this review focus on emerging data regarding quality of life across the lifespan of patients with Sickle Cell Disease, and highlight the need for more action in this field in Italy, where recent immigration and improved care determine an increasing population of children with sickle cell disease being taken into long term care.

Desire for biological parenthood and patient counseling on the risk of infertility among adolescents and adults with hemoglobinopathies

“…1,3 Consequently, enabling the best possible (health-related) quality of life is a central aspect of patient care, which is increasingly being pursued in current treatment strategies. 4,5 Having (biological) children has been reported as a relevant life goal in a small cohort of patients with SCD. 6 The underlying congenital anemia itself, as well as applied treatments such as regular blood transfusions (RBT) and/or hydroxyurea (HU), have been associated with an increased risk of fertility impairment in both men and women with hemoglobinopathies.…”

Physical wellness among adolescents with hemoglobinopathic disorders

“…A chronic disease affects all parts of a patient's life, not just the physical, but also the social and emotional, educational, and occupational aspects [6] . physical wellness defines as a consist of recognition the need for physical activities, healthy diet and sleeping as well as prevention illness and injury or managing chronic health problems [7] .…”

Application of PRECEDE-PROCEED Planning Model on Quality of Life among Children with Sickle Cell Anemia

“…It is most prevalent in sub Saharan Africa with an estimated 12 -15 million cases (Henrici et al, 2021). Children with sickle cell anemia have vaso-occlusion, chronic hemolytic anemia and vasculopathy that cause both acute and chronic complications, which have a profound impact on the quality of life (Colombatti et al, 2021). Findings of many studies on children with SCA revealed that health related quality of life, in terms of pain, suffering and emotional functioning, significantly declined with increasing age of the child (CDC, 2020).…”