From the Guidelines
HBSS stands for Homozygous Sickle Cell Disease, which is the most common and severe form of sickle cell disease, where a person has inherited two sickle cell genes (one from each parent), resulting in red blood cells that are primarily hemoglobin S (HbS) rather than normal hemoglobin A. This condition is characterized by the presence of two copies of the sickle hemoglobin gene, causing red blood cells to become rigid and crescent-shaped (sickled) under certain conditions, particularly when oxygen levels are low, leading to pain, organ damage, and other serious complications 1. The management of HBSS typically requires comprehensive care, including:
- Pain management
- Hydration
- Infection prevention with vaccines and sometimes prophylactic antibiotics
- Medications like hydroxyurea or newer therapies like crizanlizumab or voxelotor that help prevent sickling or its complications 1. It is essential to note that patients with HBSS are at increased risk of peri-operative complications, and careful planning, assessment of comorbidities, and multidisciplinary team working are crucial to mitigate these risks 1. In terms of transfusion support, patients with HBSS may require pre-operative transfusion to reduce the risk of sickle-related complications, and the target hemoglobin level should be around 100 g/l to avoid hyperviscosity 1. Overall, the management of HBSS requires a multidisciplinary approach, and patients should be cared for by a team of experts, including hematologists, cardiologists, and other specialists, to optimize their outcomes and improve their quality of life 1.
From the Research
Definition of HBSS
- HBSS stands for Hemoglobin SS, which is a type of sickle cell disease [(2,3)].
- It is characterized by the presence of two copies of the HbS gene, one inherited from each parent [(2,3)].
- HBSS is the most common and severe form of sickle cell disease [(2,3)].
Clinical Features of HBSS
- Individuals with HBSS often experience recurrent episodes of pain, anemia, and increased risk of infections [(2,3)].
- They may also develop complications such as acute chest syndrome, stroke, and organ damage [(2,3)].
- The disease can lead to significant morbidity and mortality if left untreated or poorly managed [(2,3)].
Treatment of HBSS
- Hydroxyurea (hydroxycarbamide) is a commonly used medication to manage HBSS [(2,3)].
- It works by increasing the production of fetal hemoglobin, which can help reduce the frequency and severity of sickle cell crises [(2,3)].
- Other treatments, such as blood transfusions and phlebotomy, may also be used to manage specific complications or to reduce iron overload 4.