With every beat of your heart, blood carries oxygen from your lungs throughout your body. This life-sustaining process happens automatically, whether you’re awake or asleep.
But for people with sickle cell disease, it often goes awry. People with this disease have an abnormal type of hemoglobin, the oxygen-carrying molecule in red blood cells.
Normally, red blood cells are flexible and shaped like a disc. But the hemoglobin in people with sickle cell disease causes abnormally shaped red blood cells. Most commonly, they’re a crescent (or sickle) shape.
These inflexible, bent cells can stick to the blood vessel walls. The resulting clumps slow or stop the flow of blood. This may lead to pain and organ damage.
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Excerpts from our conversation with Dr. Allison King, an NIH-funded sickle cell specialist at Washington University in St. Louis.
NIHNiH: People with sickle cell disease - even children - have a higher than normal risk of stroke. What other challenges can sickle cell disease pose to the developing brain?
King: Both overt and silent strokes can harm the brain. But we’ve also realized that there are some children who haven’t had a stroke who also have a lot of trouble in school. Now we know that things like lower oxygen levels in the blood can also be associated with cognitive challenges. It’s also very stressful on the central nervous system to be chronically anemic. Plus, the inflammation from the disease can be a challenge to the brain as well.
Related story
The risk of opioid use disorder among patients with sickle cell disease who are treated with chronic opioids remains unclear, but the complications associated with opioid use and overdose can be accentuated in those with sickle cell disease. In this case report, we describe a 13-year-old girl with sickle cell disease who presented to the emergency department after a morphine overdose causing renal infarction, acute kidney injury, acute respiratory distress syndrome, and posterior reversible encephalopathy syndrome.
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Background:
Vaso-occlusive crises (VOCs) are a hallmark of sickle cell disease (SCD) during which patients experience localized pain. The current mainstay of treatment for SCD-related pain syndrome is nonsteroidal anti-inflammatory agents (NSAIDs) and opioid analgesics. Opioids, however, carry a high risk of depression, addiction, and life-threatening hypoventilation. In this study we report weight loss through a multidisciplinary program and bariatric surgery as an effective nonopioid modality to decrease the use of opioids and frequency of VOCs in a patient with SCD.
Case Description:
A 35-year-old woman with SCD and morbid obesity (body mass index [BMI] of 42 kg/m2) required high doses of opioids for pain control [870 morphine milligram equivalents (MME) per day], and experienced monthly hospitalizations for VOCs that precluded her from working or having meaningful interpersonal relationships. Palliative care identified that the patient had no admissions for VOCs when her BMI was <25 kg/m2. Given this observation, it was recommended to the patient that she lose weight. The patient enrolled in a multidisciplinary wellness program and underwent laparoscopic sleeve gastrectomy. She subsequently lost 30 kg (BMI of 29 kg/m2), decreased her basal opioid requirement by 58% (365 MME per day), and experienced no further hospitalizations for VOCs after the intervention, thereby improving her quality of life.
Conclusions:
The pervasive use and overreliance on opioid analgesics in the treatment of VOCs increase morbidity and mortality among patients with SCD. This case study offers compelling evidence for weight loss through a multidisciplinary program as an adjunctive therapy to decrease the use of opioids and frequency of hospitalizations for VOCs.
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Investigators from the University of Maryland Medical Center, Baltimore, and Nationwide Children’s Hospital, Columbus, OH, conducted a randomized controlled trial to assess the effectiveness of warmed IV saline administered to children with vaso-occlusive episodes (VOE) and sickle cell disease (SCD). Children 4–21 years with known SCD who required treatment for uncomplicated VOE at Nationwide Children’s Hospital between November 2012 and June 2014 were eligible. VOE was defined as pain in the back, extremities, abdomen, or chest rated as >3 on the Wong-Baker FACES scale. Study patients were randomized to receive either IV saline warmed to 37.5°C (intervention) or at room temperature (22°C–24°C control). Children in both groups received a 20 ml/kg bolus of IV saline, then provided fluid at a maintenance rate.
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SCD is a life-threatening, inherited blood disorder, affecting more than 100,000 Americans. Painful vaso-occlusive crises, the hallmark of SCD, result in substantial suffering and lead to associated stigma. Without adequate treatment, SCD affects all organs and is associated with decreased quality of life and a shortened life span. Among the dozens of conditions that are screened for in state newborn-screening programs, SCD is the most commonly detected condition, regardless of ethnicity. It is thus important to recognize SCD as a common and important medical condition among Americans, and not “just Black Americans.”
The causative mutation in SCD primarily arose on the African continent because of the protective effect of the carrier state against malaria, so most patients with SCD have a shared African ancestry. Although SCD is a global disorder affecting people of all races, in the United States, as a direct result of the transatlantic slave trade, nearly all patients with SCD are Black. This fact would be mere medical trivia if we did not live in such a highly racialized society. Unfortunately, the social construct of race in America requires the majority of patients with SCD not only to face the consequences of a serious health condition, but also to navigate a society in which the color of their skin is often an unfair disadvantage.
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Sickle cell disease (SCD), an autosomal recessive blood disorder, affects millions of people worldwide. Approximately 80% of all cases are located in Africa.
Objectives:
This cross-national, interdisciplinary, collaborative study investigated provider attitudes about, and practices for, managing (assessing and treating) SCD pain
Methods:
We conducted 111 quantitative surveys and 52 semi-structured interviews with healthcare providers caring for adults and/or children with SCD in Cameroon, Jamaica, and the United States (US)
Results:
Applying Haywood’s scale for assessing SCD provider attitudes, the Jamaica site scored lower on ‘Negative Attitudes’ than the Cameroonian and US sites (p =.03 and < .001, respectively). Providers at the US site scored lower on ‘Positive Attitudes’ than other sites (p <. 001). ‘Red Flag’ scores at the Cameroon sites were lower than at other sites (p < .001). Qualitative results across all three sites describe the current practices for SCD pain management, as well as the challenges surrounding management for health providers, including pain subjectivity, patient-provider and parent-provider relationships, resource availability, perceptions of drug-seeking behavior, and adherence. Providers also spontaneously offered solutions to reported challenges
Conclusion:
Overall, findings reveal that SCD provider attitudes toward their patients differed across sites, yet at all three sites, treating SCD pain is multidimensional.
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