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Sickle-cell disease: managing comorbidities
Editorial
Dr P Marazzi/Science Photo Library
Self-medicating in the opioid crisis
For the advisory see http://www. fda.gov/safety/medwatch/safety information/safetyalerts forhumanmedicalproducts/ ucm505303.htm
On June 7, the US Food and Drug Administration released a drug safety notice about loperamide, an anti-diarrhoea medicine sold over the counter and under prescription. The drug is being used as a cheap and legally-available high, and to reduce the symptoms of opioid withdrawal. A standard dose for the medicine is 8 mg per day in divided doses, but opioid users have been taking massive doses of the drug, as much as 300 mg or more daily for weeks at a time. Taken in such high quantities, overdoses of loperamide can cause severe heart problems like ventricular arrhythmias, cardiac arrest, and death. Although the number of reported cases is currently small, standard toxicology screenings do not look for loperamide, and it is possible that the rate of loperamide abuse is under-reported. But loperamide abuse is just one aspect of the larger problem of viewing opioid addiction through the lens of the criminal justice system. Although Americans with the means to are able to seek treatment in private facilities, the rural poor—disproportionately affected by the opioid
crisis—are stuck in a cycle of unenviable choices. Opioid users are reticent in seeking help. Pursuing treatment for drug addiction risks incarceration and a criminal drug record. These outcomes can have lasting negative effects, from difficulties in finding employment to the inability to receive social services, as well as potential child custody difficulties. At a recent forum on the US opioid crisis organised by Harvard University, the governors of the six New England states reiterated their commitment to addressing the issue. But Governor Paul LePage of Maine used the opportunity to call for a “tough love” approach, citing drug courts where users could choose between treatment and incarceration. These kinds of mixed messages—where addicts fear receiving plenty of the “tough” and not much of the “love”—will continue to drive users towards using certain drugs counter to their intended use rather than seeking out medically sound treatments, potentially making an already bad public health problem worse. The Lancet
Tim Vernon/Science Photo Library
Sickle-cell disease: managing comorbidities
See Perspectives page 2500 See Series pages 2545, 2554, and 2565
2480
To coincide with World Sickle Cell Day on June 19, The Lancet is publishing its first haematology Series commissioned to review three topical and clinically important aspects of sickle-cell disease. At least 90% of children and adults with sickle-cell anaemia live in sub-Saharan Africa and in south Asia, where the disease remains life threatening, particularly in childhood, and where the burden of the disease is expected to rise. For children living in high-income countries, mortality due to sickle-cell anaemia has been dramatically reduced to less than 2% by 15 years of age, but as children live longer, age-dependent end-organ dysfunction has assumed greater importance in clinical management. Two of the Series papers examine respiratory and cardiac complications and comorbidities. Michael DeBaun and Robert Strunk review asthma and acute chest syndrome in children with sickle-cell anaemia. With their colleagues, DeBaun and Strunk were the first to document that asthma worsens outcomes in sickle-cell disease, as the Obituary of Strunk in this issue describes. Despite overlapping risk factors and symptoms, acute
chest syndrome and asthma are two distinct entities that require specific management strategies. Mark Gladwin reviews the chronic effect of sustained haemolytic anaemia and the episodic vaso-occlusive events that result in pulmonary hypertension, left ventricular diastolic heart disease, dysrhythmia, and sudden death. Data are lacking on interventions that work, and Gladwin calls for more research in larger populations with the disease. Guillaume Lettre and Daniel Bauer review the evidence supporting the disease modifying role of fetal haemoglobin in reducing sickling, and how induction of fetal haemoglobin production has potential in treatment. As this Series paper emphasises, genome editing technology offers hope for new treatments based on fetal haemoglobin, but for most people with the disease in Africa and south Asia, an affordable solution such as hydroxyurea is more practical. The future promises therapeutic strategies and potential cures, but for most with sickle-cell disease, the theme of this year’s World Sickle Cell Day “exhibit courage” is the harsh reality of the pain of sickle-cell crises and the disease’s disabling long-term consequences. The Lancet www.thelancet.com Vol 387 June 18, 2016
Dr P Marazzi/Science Photo Library
Self-medicating in the opioid crisis
For the advisory see http://www. fda.gov/safety/medwatch/safety information/safetyalerts forhumanmedicalproducts/ ucm505303.htm
On June 7, the US Food and Drug Administration released a drug safety notice about loperamide, an anti-diarrhoea medicine sold over the counter and under prescription. The drug is being used as a cheap and legally-available high, and to reduce the symptoms of opioid withdrawal. A standard dose for the medicine is 8 mg per day in divided doses, but opioid users have been taking massive doses of the drug, as much as 300 mg or more daily for weeks at a time. Taken in such high quantities, overdoses of loperamide can cause severe heart problems like ventricular arrhythmias, cardiac arrest, and death. Although the number of reported cases is currently small, standard toxicology screenings do not look for loperamide, and it is possible that the rate of loperamide abuse is under-reported. But loperamide abuse is just one aspect of the larger problem of viewing opioid addiction through the lens of the criminal justice system. Although Americans with the means to are able to seek treatment in private facilities, the rural poor—disproportionately affected by the opioid
crisis—are stuck in a cycle of unenviable choices. Opioid users are reticent in seeking help. Pursuing treatment for drug addiction risks incarceration and a criminal drug record. These outcomes can have lasting negative effects, from difficulties in finding employment to the inability to receive social services, as well as potential child custody difficulties. At a recent forum on the US opioid crisis organised by Harvard University, the governors of the six New England states reiterated their commitment to addressing the issue. But Governor Paul LePage of Maine used the opportunity to call for a “tough love” approach, citing drug courts where users could choose between treatment and incarceration. These kinds of mixed messages—where addicts fear receiving plenty of the “tough” and not much of the “love”—will continue to drive users towards using certain drugs counter to their intended use rather than seeking out medically sound treatments, potentially making an already bad public health problem worse. The Lancet
Tim Vernon/Science Photo Library
Sickle-cell disease: managing comorbidities
See Perspectives page 2500 See Series pages 2545, 2554, and 2565
2480
To coincide with World Sickle Cell Day on June 19, The Lancet is publishing its first haematology Series commissioned to review three topical and clinically important aspects of sickle-cell disease. At least 90% of children and adults with sickle-cell anaemia live in sub-Saharan Africa and in south Asia, where the disease remains life threatening, particularly in childhood, and where the burden of the disease is expected to rise. For children living in high-income countries, mortality due to sickle-cell anaemia has been dramatically reduced to less than 2% by 15 years of age, but as children live longer, age-dependent end-organ dysfunction has assumed greater importance in clinical management. Two of the Series papers examine respiratory and cardiac complications and comorbidities. Michael DeBaun and Robert Strunk review asthma and acute chest syndrome in children with sickle-cell anaemia. With their colleagues, DeBaun and Strunk were the first to document that asthma worsens outcomes in sickle-cell disease, as the Obituary of Strunk in this issue describes. Despite overlapping risk factors and symptoms, acute
chest syndrome and asthma are two distinct entities that require specific management strategies. Mark Gladwin reviews the chronic effect of sustained haemolytic anaemia and the episodic vaso-occlusive events that result in pulmonary hypertension, left ventricular diastolic heart disease, dysrhythmia, and sudden death. Data are lacking on interventions that work, and Gladwin calls for more research in larger populations with the disease. Guillaume Lettre and Daniel Bauer review the evidence supporting the disease modifying role of fetal haemoglobin in reducing sickling, and how induction of fetal haemoglobin production has potential in treatment. As this Series paper emphasises, genome editing technology offers hope for new treatments based on fetal haemoglobin, but for most people with the disease in Africa and south Asia, an affordable solution such as hydroxyurea is more practical. The future promises therapeutic strategies and potential cures, but for most with sickle-cell disease, the theme of this year’s World Sickle Cell Day “exhibit courage” is the harsh reality of the pain of sickle-cell crises and the disease’s disabling long-term consequences. The Lancet www.thelancet.com Vol 387 June 18, 2016