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Updated: Apr 27, 2026
A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Treatment for avascular necrosis of bone in people with sickle cell disease
Arturo J Martí-Carvajal1, Ivan Solà, Luis H Agreda-Pérez
1Iberoamerican Cochrane Network, Valencia, Venezuela.
Insights
Hip core decompression did not improve avascular necrosis of bone in sickle cell disease patients when added to physical therapy. Further research is needed to evaluate this treatment for bone complications in sickle cell disease.
Area of Science:
- Orthopedics
- Hematology
- Regenerative Medicine
Background:
- Avascular necrosis of bone is a severe complication in sickle cell disease.
- Current treatment strategies for this condition are not standardized.
Purpose of the Study:
- To evaluate the efficacy and safety of surgical interventions versus other treatments for avascular necrosis of bone in sickle cell disease patients.
Main Methods:
- A systematic search of the Cochrane Haemoglobinopathies Trials Register was conducted.
- One randomized clinical trial involving 46 participants was identified and analyzed.
Main Results:
- Hip core decompression combined with physical therapy showed no significant clinical improvement compared to physical therapy alone after a mean follow-up of three years.
- No significant differences were observed in major complications such as hip pain, vaso-occlusive crises, or acute chest syndrome between the groups.
Conclusions:
- There is currently no evidence to support the use of hip core decompression in addition to physical therapy for avascular necrosis of bone in sickle cell disease.
- High attrition rates in the included trial limit the conclusions, emphasizing the need for further high-quality randomized controlled trials with appropriate endpoints.
Background:
Avascular necrosis of bone is a frequent and severe complication of sickle cell disease and its treatment is not standardised.
Objectives:
To determine the impact of any surgical procedure compared with other surgical interventions or non-surgical procedures, on avascular necrosis of bone in people with sickle cell disease in terms of efficacy and safety.
Search Methods:
We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Trials Register, comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. Additional trials were sought from the reference lists of papers identified by the search strategy.Date of the most recent search of the Group's Haemoglobinopathies Trials Register: 17 March 2014.
Selection Criteria:
Randomised clinical trials comparing specific therapies for avascular necrosis of bone in people with sickle cell disease.
Data Collection And Analysis:
Each author independently extracted data and assessed trial quality. Since only one trial was identified, meta-analysis was not possible.
Main Results:
One trial (46 participants) was eligible for inclusion. After randomisation eight participants were withdrawn, mainly because they declined to participate in the trial. Data were analysed for 38 participants at the end of the trial. After a mean follow up of three years, hip core decompression and physical therapy did not show clinical improvement when compared with physical therapy alone using the score from the original trial (an improvement of 18.1 points for those treated with intervention therapy versus an improvement of 15.7 points with control therapy). There was no significant statistical difference between groups regarding major complications (hip pain, relative risk (RR) 0.95 (95% confidence interval (CI) 0.56 to 1.60; vaso-occlusive crises, RR 1.14 (95% CI 0.72 to 1.80; very low quality of evidence); and acute chest syndrome, RR 1.06 (95% CI 0.44 to 2.56; very low quality of evidence)). This trial did not report results on mortality or quality of life.
Authors' Conclusions:
We found no evidence that adding hip core decompression to physical therapy achieves clinical improvement in people with sickle cell disease with avascular necrosis of bone compared to physical therapy alone. However, we highlight that our conclusion is based on one trial with high attrition rates. Further randomised controlled trials are necessary to evaluate the role of hip-core depression for this clinical condition. Endpoints should focus on participants' subjective experience (e.g. quality of life and pain) as well as more objective 'time-to-event' measures (e.g. mortality, survival, hip longevity). The availability of participants to allow adequate trial power will be a key consideration for endpoint choice.
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