Palliative care needs of adults severely affected by sickle cell disease: A mixed-methods systematic review

Sickle cell disease is one of the most common inherited monogenetic blood disorders. People living with sickle cell disease experience both acute pain and long-lasting complications, leading to co-morbidities which impact their quality of life. Palliative care is both under-reported and under-utilised for people living with sickle cell disease despite the profound impacts of this illness across their lives.

To synthesise published primary evidence on the palliative care needs and experiences of adults severely affected by sickle cell disease.

Mixed method systematic narrative review.

Four databases (Medline, PsycINFO, Embase and CINHAL) were searched from inception to April 2024. Eligibility criteria were adults aged ≥18 years with severe sickle cell disease defined in the disease severity classification system as Class II (moderate severity) and Class III (most severe). Data from qualitative and quantitative studies were analysed using data-based convergent synthesis. A best-fit framework synthesis approach was used with the World Health Organization definition of palliative care as the a priori framework. Results were mapped to Bronfenbrenner Ecological Model to pursue interaction between needs across different environmental systems.

23 studies met eligibility (12 qualitative and 11 quantitative studies). Needs were evident across the five domains of palliative care, and an additional domain titled experiences of using healthcare services: i) Physical needs included distressing symptoms focusing mainly on pain (n = 22 studies). Pain manifested as both acute and chronic and impacted all aspects of a person's life; ii) Psychological needs encompassed depression, suicidal ideation, coping and stigma related to the disease itself and need for opioids for pain management; iii) Social needs included the impact on social life, functioning, personal relationships, employment and education; iv) Spirituality was described in terms of its use when living with a complex chronic condition; v) Family caregiver needs and the support provided included the family feeling unable to support the person at times, and vi) Experiencing access to healthcare services which included lack of trust in healthcare providers.

Synthesised palliative care needs for people severely affected by sickle cell disease are interlinked and impact one another. This is influenced by the unpredictability of the disease itself and its chronic complications such as living with and managing chronic wounds. Stigma associated with the disease impacted the person in multiple ways, notably delaying seeking medical treatment, discrimination in the workplace and social isolation. Sickle cell disease is a multifaceted complex condition. Management requires a dynamic holistic approach putting the person before their disease and emphasis on family involvement. PROSPERO registration: (CRD42023392072).