The acute pain crisis in sickle cell disease: What can be done to improve outcomes?

The acute pain crisis (APC) is the commonest complication of sickle cell disease (SCD). Severe episodes may require treatment in hospital with strong opioid analgesic drugs, combined with additional supportive care measures. Guidelines for APC management have been produced over the past two decades gathering evidence from published studies, expert opinion, and patient perspective. Unfortunately, reports from multiple sources indicate that guidelines are often not followed, and that acute care in emergency departments and on acute medical wards is suboptimal. It is important to understand what leads to this breakdown in health care, and to identify evidence-based interventions which could be implemented to improve care. This review focuses on recently published articles as well as information about on-going clinical trials. Aspects of care which could potentially make a difference to patient experience include availability and accessibility of individual care plans agreed between patient and treating specialist, innovative means of delivering initial opioids to reduce time to first analgesia, and availability of a specialist unit away from the ED, where expert care can be delivered in a more compassionate environment. The current evidence of improved outcomes and health economic advantage with these interventions is inadequate, and this is hampering their implementation into health care systems.

What is the environmental impact of a blood transfusion? A life cycle assessment of transfusion services across England.

BACKGROUND: Healthcare activities significantly contribute to greenhouse gas (GHG) emissions. Blood transfusions require complex, interlinked processes to collect, manufacture, and supply. Their contribution to healthcare emissions and avenues for mitigation is unknown. STUDY DESIGN AND METHODS: We performed a life cycle assessment (LCA) for red blood cell (RBC) transfusions across England where 1.36 million units are transfused annually. We defined the process flow with seven categories: donation, transportation, manufacturing, testing, stockholding, hospital transfusion, and disposal. We used direct measurements, manufacturer data, bioengineering databases, and surveys to assess electrical power usage, embodied carbon in disposable materials and reagents, and direct emissions through transportation, refrigerant leakage, and disposal. RESULTS: The central estimate of carbon footprint per unit of RBC transfused was 7.56 kg CO2 equivalent (CO2eq). The largest contribution was from transportation (2.8 kg CO2eq, 36% of total). The second largest was from hospital transfusion processes (1.9 kg CO2eq, 26%), driven mostly by refrigeration. The third largest was donation (1.3 kg CO2eq, 17%) due to the plastic blood packs. Total emissions from RBC transfusion are ~10.3 million kg CO2eq/year. DISCUSSION: This is the first study to estimate GHG emissions attributable to RBC transfusion, quantifying the contributions of each stage of the process. Primary areas for mitigation may include electric vehicles for the blood service fleet, improving the energy efficiency of refrigeration, using renewable sources of electricity, changing the plastic of blood packs, and using methods of disposal other than incineration.

Rate of Dental Extractions in Patients with Sickle Cell Disease.

BACKGROUND: Sickle cell disease is an inherited disorder associated with chronic haemolysis and anaemia, recurrent episodes of pain and potentially multisystem end-organ damage. A lot less is known about the dental health of these patients. AIMS: To explore the incidence of severe dental disease leading to dental extraction in our sickle cell population. PATIENT/METHODS: We undertook an audit looking at the rate of dental extractions, as a composite marker of severe dental disease, among sickle cell patients over a 3-month period. The patients were unselected and approached during routine assessments. We analysed both clinical and laboratory data to look for possible associations between dental disease and sickle cell characteristics. RESULTS: 177 patients were interviewed between February 2022 and April 2022. Overall, 71% of the patients had at least one dental extraction with a median number of teeth extracted of three and a median age at first extraction of 26. More than half of the patients stated that they do not have regular dental check-ups. There were no significant associations with the severity of sickle cell phenotype, baseline Hb or markers of haemolysis. CONCLUSION: A large number of patients with sickle cell disease require dental extractions at a relatively young age. The lack of any correlation with disease severity suggests that poor engagement with dental services and the underestimation of the importance of dental health are the main factors behind the increased prevalence of severe dental disease. Actively enquiring about dental problems should be part of any routine consultation with these patients, both in primary and specialist care.

Therapeutic plasma exchange in the management of acute complications of sickle cell disease: A single centre experience.

Sickle cell disease results in systemic inflammation even at steady state and this is accentuated during acute crises. The plasma of affected patients contains several proinflammatory cytokines as well as adhesion molecules and prothrombotic factors. This environment promotes further red cell sickling while many of these substances can cause direct tissue toxicity and end-organ damage. Even though red cell transfusion, whether simple or exchange, is the mainstay of treatment of severe acute complications, addition of therapeutic plasma exchange could potentially provide additional benefit by removing such harmful substances. Here, we describe two cases where therapeutic plasma exchange was used. The first involved a patient with the acute chest syndrome who despite adequate red cell exchange remained significantly hypoxic and in severe pain. We therefore proceeded to perform plasma exchange; this led to rapid clinical improvement and resolution of his symptoms. The second case involved a patient with intractable chest wall pain and impending acute chest syndrome; this patient also had a past history of hyperhaemolysis. The patient underwent therapeutic plasma exchange with very rapid resolution of the pain, avoidance of any respiratory deterioration and full recovery. We also give a brief summary of our previous experience using plasma exchange in patients with sickle cell disease. Plasma exchange was well tolerated with no adverse events in all cases we have treated, led to rapid resolution of pain irrespective of primary indication and in the majority of cases to a favourable clinical outcome.

The safety of modern intravenous iron infusions in patients with rheumatoid arthritis - a review of the literature.

Objectives: To assess the evidence for the safety of intravenous iron infusions in patients with rheumatoid arthritis.Methods: A systematic literature search was performed in June 2019 on PubMed and Cochrane databases for eligible studies.Results: There is significant evidence of safety and efficacy of intravenous iron in patients with rheumatoid arthritis using newer, less immunogenic iron preparations, such as iron sucrose and low molecular weight iron dextran preparations.Discussion: Iron deficiency anaemia has a significant impact on the quality of life of patients with rheumatoid arthritis, but the use of intravenous iron is generally avoided due to concerns raisedin older studies using high molecular weight iron dextran of exacerbating the disease. However, such concerns have not been confirmed in more recent studies using newer preparations.Conclusion: We find significant evidence of safety and efficacy in more recent studies of larger cohorts of patients using newer, less immunogenic iron preparations.