Patients with sickle cell disease (SCD) suffer from frequent vaso-occlusive crises (VOC), the leading cause of hospitalization and ED visits for these patients. Infusion centers (IC) are alternatives to ED care and may provide patients with a better care experience. We previously demonstrated in a single center that the use of such centers provides more rapid pain control and can decrease the risk of a hospital admission. For the management of VOC the NHLBI’s guidelines recommend that patients receive analgesic therapy within 60 minutes of registration and that pain be reassessed every 15-30 minutes until it is controlled. In the ESCAPED study, we aimed to learn whether the ED or IC more rapidly provides analgesia to patients who present with an uncomplicated VOC. Conclusions: With adjustment for differences between patients treated in the ED and IC, we demonstrated that those treated in an IC have significantly better treatment experiences than subjects treated in an ED: a 50% reduction in time to first dose of pain medication and an almost 6-fold decrease in hospital admission. Increasing access to infusion clinics is essential to improving the quality of care of uncomplicated VOC in adults with SCD. Abstract source

Leg ulcers are a serious complication of Sickle Cell Disease (SCD), often linked to a hemolytic phenotype that includes priapism, pulmonary hypertension (PH) and renal disease. In the SCD pathogenesis, hemolysis and ischemia-reperfusion injuries lead to white blood cell (WBC), platelet and endothelial cell activation, pro-inflammatory cytokine production and subsequent activation of coagulation that tips the balance towards a prothrombotic and inflammatory state. Decreased levels of the naturally occurring anticoagulants (NOACs): protein C (PC), protein S (PS), antithrombin III (ATIII), have been observed in patients with SCD in steady state, with some reports stating no differences between steady state and crisis. This occurs due to decreased production, increased consumption, or a combination of both. However, the definite mechanisms have not yet been elucidated. Factor V Leiden (FVL) and prothrombin G20210A mutations are rare in African Americans and in patients with SCD. We speculated that patients with leg ulcers would have a more pronounced pro-thrombotic phenotype than patients who never developed them. Conclusions: In SCD patients with leg ulcers, PC and PS levels are significantly lower than in SCD patients without leg ulcers. Additionally, in SCD patients with leg ulcers, there is a trend towards decreased levels of AT III and increased LA positivity. FVL and prothrombin gene mutations were rare in our cohort, consistent with the general population. The above findings did not correlate clinically with a history of DVT/PE in the same population. Our results of decreased NOACs confirm previous reports from our group that demonstrated similar findings. Of greater significance, however, is our finding that sickle cell patients with leg ulcers have further decreased levels of the NOACs compared to their counterparts without leg ulcers. This finding lends credence to the hypothesis that sickle cell patients with leg ulcers have a procoagulant phenotype (Am J Hematol. 2011 Aug; 86: 705–8, Am J Hematol. 2014 Jan; 89: 1–6). Whether this translates to an increased risk of thromboembolic events remains uncertain. From our findings, it may not be the case for DVTs and PEs. However, further studies evaluating other clinical manifestations such as priapism, PH, stroke, avascular necrosis, and retinopathy, are still needed. Future directions include further correlation with the above-mentioned manifestations to provide a more comprehensive picture. Abstract source

Background: The hallmark of sickle cell disease (SCD) is pain, which can be both acute and chronic. Pain in patients with SCD leads to hospital admissions and diminishes quality of life. Pain in patients with SCD is primarily treated with opioid medications, which are often insufficient. In 2017 the Committee on the Health Effects of Marijuana of the National Academies of Science, Engineering, and Medicine reported that there is “conclusive or substantial evidence that cannabis or cannabinoids are effective for the treatment of chronic pain in adults”, but the report made no mention of chronic pain in patients with SCD. Currently, 30 states and Washington D.C. have medical marijuana (MM) laws, and 5 states list SCD as a qualifying condition. SCD was added to the list of qualifying conditions in Connecticut in February 2016. Since then we have offered certification to patients in our program who make regular clinic visits and for whom we think MM will be safe. However, not all patients who are certified go on to complete the state regulatory paperwork and obtain MM. We hypothesized that those who had obtained MM would show an improvement in clinical outcomes compared to those patients who had been certified but not obtained MM. Conclusion: Though there was no difference in admission rates between the two groups we examined prior to MM certification, and the only clinical difference was that those who obtained MM were more likely to be in the more severe genotype, those who obtained MM showed a decrease in admissions in the next 6 months compared to those who did not. We suggest that MM allowed patients to improve their pain relief and thereby reduce admission rates. This is consistent with data showing cannabinoid agonists improve pain in murine models of SCD. Larger, randomized and controlled studies of MM for pain in SCD should be conducted. Abstract source

Background: The management of pain in adults with sickle cell disease (SCD) is complex, with the intermingling of both acute vaso-occlusive events and chronic daily pain. Sixty percent of adults with SCD suffer with every day chronic pain. In patients with frequent acute visits we use aggressive disease modifying therapy to decrease the risk of VOC yet there remains a subset of patients who continue to have frequent acute visits for pain. In addition, there are patients maintained on high doses of oral opioids as outpatients who continue to have high levels of daily pain. There is little data that escalating doses of opioids is associated with benefit, yet significant data to support that higher doses are associated with harms. We consider these to be cases of opioid failure. Identifying therapies for these patients that improve quality of life is essential. Buprenorphine is a partial mu opioid receptor agonist and kappa antagonist, and has a very high affinity for the mu receptors, with an elimination half-life of 28-37 hours for the sublingual administration. The reduced risk of overdose, lower risk for misuse, diminished withdrawal symptoms, and blunting of opioid craving make it an appealing alternative to full opioid agonists in a subset of patients with SCD who continue to have significant pain or are unable to wean off of ineffective opioid therapy. The purpose of this report is to describe our experience converting patients with SCD and chronic pain from chronic opioid therapy to buprenorphine. Conclusions: Adults with sickle cell disease on chronic full agonist opioid therapy can be safely converted to buprenorphine. Acute care utilization has dropped significantly for patients post induction. Assessment of patient reported outcomes such as quality of life and pain interference are being collected. Buprenorphine appears to be a safe and effective medication in the management of pain in adults with SCD. Abstract source

Hydroxyurea (HU) is approved in EU and USA for preventing vaso-occlusive crises (VOC) including acute chest syndromes (ACS) in adults, adolescents and children >=2 years with sickle-cell disease (SCD). Further to the double blinded, randomized controlled Multicenter Study of Hydroxyurea administration (MSH) which provided the first data on clinical efficacy of HU (Charache 1995), a few follow-up studies suggested that long-term use of HU resulted in significant clinical benefit on patient outcome. This was reflected by lower mortality rates in HU-treated patients compared to conventionally treated patients (Steinberg 2003 & 2010, Voskaridou 2010, Lê 2015). Sustained hematological and clinical response after several years of follow-up at maximal tolerated dose was further shown in subsequent studies, especially the LaSHS study (Voskaridou 2010). Based on these data, the ESCORT-HU (European Sickle Cell Disease COhoRT – HydroxyUrea) study was launched aiming in the establishment of the safety and the sustainability of hematological and clinical benefits to HU administration in the prevention of sickle-cell vaso-occlusive crises in routine practice. We hereby present preliminary results from a large cohort of patients enrolled in the study between January 2009 and June 2017. Conclusion: In preliminary results from ESCORT-HU in 147 patients treated with HU showed sustained hematological and clinical response while MTD was not targeted, with differences between adults and children which may be attributed in part to reduced compliance in the latter group. Abstract source

Sickle cell disease is a blood disorder caused by an inherited genetic mutation. People with sickle cell develop crescent-shaped red blood cells when there is reduced oxygen levels. These sickled red blood cells become stiff and do not flow easily through small blood vessels. When the sickle cells block the flow of blood through the body, extreme pain occurs, due to blood-starved tissues. People with sickle cell have extreme anemia because of the abnormal hemoglobin in their blood cells. Hemoglobin is a protein present in red blood cells that help to carry oxygen throughout the body.   Sickle Cell and Your Eyes Sickle cell disease can affect many organs in the body, including the eyes. Eye signs can include the following:

• Eye redness caused by comma-shaped blood vessels
• Subconjunctival hemorrhage
• Atrophy or weakening of the iris
• New blood vessel growth (neovascularization) of the iris
• Snake-like shaped blood vessels
• Retinal hemorrhage
• Pigmentations in the retinal periphery

  Abstract source

Your kidneys normally remove waste from your blood. They have many small blood vessels that help do this. The waste becomes part of your urine (pee) and goes out of your body. If sickle cell disease blocks normal blood flow to the kidneys, they do not get enough oxygen. This keeps them from working well. Your child’s St. Jude team might say your child has kidney disease or “renal disease.” Another name for sickle cell kidney disease is “sickle cell nephropathy.” If your child’s sickle cell disease affects their kidneys, your child could have some or all of the conditions below. •  Protein in the urine •  Urine with too much water in it •  Blood in the urine •  High blood pressure Abstract source

Participating in regular physical activity is one of the most important things you can do for your health. This is true for everyone, including those with Sickle Cell Trait (SCT). You just have to be aware of the warning signs and complications of exercise-related illness, listen to your body, and take steps to protect yourself. Below are answers to some commonly asked questions about SCT, participation in sports, exercise related illness, and what to do to stay safe and healthy while engaging in physical activity?   Should people with SCT be allowed to play sports? Absolutely! People with SCT can safely participate in all sports provided they take a few general precautions, such as: • Drinking enough water; • Taking breaks when needed; and • Not overdoing it, especially when starting a new exercise program.   Abstract source

According to the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), the hemoglobin A1C assay may be unreliable for diagnosing and monitoring diabetes and prediabetes in individuals with sickle cell trait (SCT) or other hemoglobinopathies. In order to provide the best possible care for your patients, it is important to determine their SCT status and know whether the A1C test you normally use should be replaced or supplemented with a different test.   Can the hemoglobin A1C test identify SCT? No. The hemoglobin A1C test is not designed to look for SCT, but some test methods for hemoglobin A1C might detect“variant hemoglobin,” which could in some cases be sickle hemoglobin. However, this result should not be considered a diagnostic test for SCT. SCT should be diagnosed only by using a test approved for hemoglobin identification.   Abstract source

If you have sickle cell trait (SCT), the hemoglobin A1C test used to test and monitor diabetes (a potentially lifelong disease in which you have high levels of sugar in your blood) may give false results which can affect the care you receive from your healthcare provider. The good news is other tests and test methods are available that will give you accurate results you can trust. Knowing that you have SCT and letting your doctor know will help your doctor choose the right A1C test for you.   How are the results of the A1C test affected by SCT? If you have SCT, the A1C test might give false results, depending on the A1C test method used. These false results could lead to you being undertreated or overtreated for diabetes.   Abstract source