Amputations in Sickle Cell Disease: Case Series and Literature Review.

In this study, we describe four new patients with sickle cell disease who had limb amputations. Two of the patients had sickle cell anemia [Hb S (HBB: c.20A > T) (ß(S)/ß(S))] with refractory leg ulcers that required amputations. The third patient had sickle cell trait with an extensive leg ulcer that was associated with epidermoid carcinoma. The fourth patient had amputations of both forearms and feet due to a misdiagnosis of dactylitis. Review of the literature showed that the indications for amputations in sickle cell disease included three distinct categories: mythical beliefs, therapeutic and malpractice. All therapeutic amputations were for severely painful, large, recalcitrant leg ulcers that failed non-interventional therapies. Amputation resulted in pain relief and better quality of life. Phantom neuropathic pain was not a major issue post-operatively. It was absent, transient or well controlled with antidepressants. Limb function was restored post-amputation with prosthetic artificial limbs, wheelchairs or crutches. Malpractice amputations were due to misdiagnosis or to cryotherapy by exposing the painful limb to ice water resulting in thrombosis, gangrene and amputation. We strongly suggest that leg amputations should be considered in the management of certain patients with severe extensive refractory leg ulcers, and topical cryotherapy should never be used to manage sickle cell pain.

Risk Factors of Pulmonary Hypertension in Brazilian Patients with Sickle Cell Anemia.

This study was a prospective cross-sectional cohort study of 125 patients with sickle cell anemia (SS) between the ages of 16 to 60 years. Enrolled patients were followed-up prospectively for 15 months. Demographic, clinical, hematological and routine biochemical data were obtained on all patients. Six-minute walk test and Doppler Echocardiography were performed on all patients. A tricuspid regurgitant jet velocity (TRJV) < 2.5 m/sec was considered normal, 2.5 ≤ TRJV ≤ 3.0 was considered mild-moderate and > 3.0 m/sec, severe. Patients with abnormal TRJV were significantly older and more anemic, had significantly higher lactate dehydrogenase (LDH) levels, reticulocyte count and incidence of death. The logistic multimodal model implemented for the 125 patients indicated that age was the covariate that influenced the outcome of normal or abnormal TRJV with a cutoff age of thirty-two years. The survival rate for the group of patients with creatinine (Cr) > 1.0 mg/dL was lower than the group with Cr ≤ 1 and normal TRJV. A coefficient matrix showed that the LDH values were weakly correlated with the reticulocyte count but strongly correlated with hemoglobin suggesting that the TRJV values were not correlated with the hemolytic rate but with anemia. Ten patients died during the follow-up of whom 7 had TRJV > 2.5 m/sec. Acute chest syndrome was the most common cause of death followed by sepsis. In conclusion, this study shows that patients with SS older than thirty-two years with high LDH, elevated TRJV, severe anemia and Cr > 1 have poor prognosis and may be at risk of having pulmonary hypertension and should undergo RHC.

Treatment of the acute sickle cell vaso-occlusive crisis in the Emergency Department: a Brazilian method of switching from intravenous to oral morphine.

OBJECTIVES: Describe the treatment of patients with vaso-occlusive crises (VOC) in a Brazilian emergency department (ED) and the successful switch from intravenous to oral morphine. PATIENTS AND METHODS: We analyzed records of 315 patients with sickle cell disease using two different protocols for pain: one in March 2010 prescribing intravenous morphine every 4 h throughout their stay, and another in March 2011 and 2012 prescribing one initial dose of intravenous morphine followed by equianalgesic doses of oral morphine every 4 h. Patients were triaged into three groups: mild, moderate, and severe VOC. The mild group was treated within 1 h after triage, the moderate within 30 min and the severe was treated immediately. Patients whose pain was not relieved within 6 h after the first dose of morphine were transferred into a different holding area of the ED where they continued to receive the same treatment for 48 h after which they were hospitalized if still in pain. RESULTS: The number of patients who stayed <24 h in the ED increased significantly from 63 in 2010 to 87 in 2012, and the number of admissions decreased from 26 in 2010 to 10 in 2012. The incidence of acute chest syndrome decreased from 8.5% in 2010 to 1.9% in 2012. CONCLUSION: Patients treated with oral morphine stayed a shorter time in the ED, had more pain relief, were admitted less frequently, and had less acute chest syndrome. These differences may be due to environmental, cultural, psychological, and pharmacogenetic factors.

The exposure of the nursing profession in online and print media.

OBJECTIVE: to describe the coverage of news concerning the nursing profession in the Portuguese media: informative sites on the Internet and in print media. METHOD: a total of 1,271 health news items were collected in September and October of 2011 (956 online news items and 325 news items originating from the press review of the Portuguese Order of Nurses). Statistical analysis was used to characterize the variables. RESULTS: nurses were the sources of information in 6.6% of cases, suggesting limited media exposure. The health news collected is characterized by a production based on limited information sources, that is, male and official sources, on information disseminated by news agencies focused on economic and political issues in the health field. CONCLUSION: the presence of nurses in the news concerning nursing health is reduced. We suggest that nurses develop public communication skills to disseminate the importance of their profession in society and their relationship with the media.

Leg amputation for an extensive, severe and intractable sickle cell anemia ulcer in a Brazilian patient.

A 35-year-old African Brazilian patient had sickle cell anemia complicated with recurrent vasoocclusive (VOC) crises and refractory painful leg ulcers for 16 years. The ulcers started over both medial malleoli and expanded gradually. The ulcer on the left leg spread from the foot to the knee circumferentially and was refractory to all forms of therapy within the frame work of multi-disciplinary care. The patient agreed to a below the knee amputation of the left leg. He felt much better after the amputation but developed severe neuropathic phantom pain that was well controlled medically. He could differentiate the sickle cell anemia and ulcer pain from the neuropathic pain. About 6 months after the amputation he had dengue fever with fatal outcome. This is the first report of treatment of refractory sickle cell anemia leg ulcer with amputation and probably the first report of a Brazilian patient with sickle cell anemia and dengue fever.

The exposure of the nursing profession in online and print media / Visibilidade da enfermagem nas midias impressa e online / Visibilidad de la enfermeria en la prensa y en la media online

OBJECTIVE: to describe the coverage of news concerning the nursing profession in the Portuguese media: informative sites on the Internet and in print media. METHOD: a total of 1,271 health news items were collected in September and October of 2011 (956 online news items and 325 news items originating from the press review of the Portuguese Order of Nurses). Statistical analysis was used to characterize the variables. RESULTS: nurses were the sources of information in 6.6% of cases, suggesting limited media exposure. The health news collected is characterized by a production based on limited information sources, that is, male and official sources, on information disseminated by news agencies focused on economic and political issues in the health field. CONCLUSION: the presence of nurses in the news concerning nursing health is reduced. We suggest that nurses develop public communication skills to disseminate the importance of their profession in society and their relationship with the media. .

OBJETIVO: descrever a cobertura noticiosa da profissão de enfermagem nas mídias portuguesas - sítios informativos da Internet e imprensa escrita. MÉTODOS: foram colhidas 1.271 notícias de saúde nos meses de setembro e outubro de 2011 (956 notícias online e 325 provenientes da resenha de imprensa da Ordem dos Enfermeiros Portugueses) e recorreu-se à análise estatística para caracterizar as variáveis definidas. RESULTADOS: os enfermeiros constituíram-se como fontes de informação em 6,6% dos casos, o que sugere baixa visibilidade mediática. As notícias de saúde colhidas são caracterizadas por produção noticiosa baseada em fontes de informação pouco variadas, oficiais e de gênero masculino, em informação divulgada por agências noticiosas e focada em questões econômicas e políticas da saúde. CONCLUSãO: a presença dos enfermeiros nas notícias de saúde é reduzida. Sugere-se que esses desenvolvam competências de comunicação pública, que visem a divulgação da importância da profissão para a sociedade e a relação com as mídias. .

OBJETIVO: describir la cobertura noticiosa de la profesión de enfermería en los medios de comunicación portugueses: a) sitios informativos de la Internet e b) prensa escrita. MÉTODOS: fueron recolectadas 1.271 noticias de salud en los meses de Septiembre y Octubre de 2011 (956 noticias online y 325 provenientes de reseñas del periódico de la Orden de los Enfermeros Portugueses); recurrió al análisis estadístico para caracterizar las variables definidas. RESULTADOS: los enfermeros se constituyeron en fuentes de información en 6,6% de los casos, lo que sugiere una baja visibilidad mediática. Las noticias de salud recolectadas se caracterizan por una producción noticiosa basada en fuentes de información poco variadas, oficiales y de género masculino; esta información es divulgada por agencias noticiosas y se enfocan en cuestiones económicas y políticas de la salud. CONCLUSIÓN: la presencia de los enfermeros en las noticias de salud es reducida. Se sugiere que éstos desarrollen competencias de comunicación pública, que objetive la divulgación de la importancia de la profesión para la sociedad y la relación con los medios de comunicación. .

Aplicação de programa educativo multidisciplinar em gestações de alto risco devido a doenças endócrinas / Application of multidisciplinary educational program in high-risk pregnancies due to endocrine diseases

OBJETIVO: avaliar a aplicação de um programa de intervenção multidisciplinar educativo em mulheres com gestação de alto risco devido a doenças endócrinas. MÉTODOS: avaliamos retrospectivamente a aplicação de um programa educativo multidisciplinar em 185 gestantes com doenças endócrinas referenciadas para uma maternidade especializada em gestação de alto risco. As gestantes receberam atenção pré-natal multidisciplinar por times compostos por endocrinologistas, obstetras, ultrassonografistas, enfermeiras e nutricionistas. Informações orais e escritas sobre hábitos saudáveis, cuidados com diabetes, uso de adoçantes artificiais e exercícios na gestação foram passadas na primeira consulta endocrinológica. Plano nutricional individualizado foi feito em primeira visita à nutricionista. Nas reavaliações mensais com nutricionista e quinzenais com endocrinologista, as informações sobre mudanças saudáveis no estilo de vida eram reforçadas e o peso registrado. O grau de aderência à dieta e atividade física foi autorreferido. Comparou-se o peso semanal antes e após a intervenção multidisciplinar, peso fetal ao nascimento, taxa de macrossomia e baixo peso, e a frequência de parto cesário nas quatro categorias de índice de massa corpórea (IMC) pré-gestacional (<18,5; 18,5 a 24,9; 25 a 29,9 e >30 kg/m²). RESULTADOS: a principal patologia de encaminhamento foi o diabetes (84,9 por cento). Um terço das gestantes (31,2 por cento) era composto por portadoras de sobrepeso e 42,5 por cento tinham obesidade pré-gestacional. A maior parte das gestantes foi vista pela primeira vez pela equipe multidisciplinar no terceiro trimestre (64,1 por cento), e 50,5 por cento delas excederam o ganho de peso recomendado para toda a gestação à primeira avaliação. Gestantes obesas excederam o ganho de peso recomendado em 62,5 por cento dos casos. Após a intervenção multidisciplinar, o percentual de gestantes que excedeu o ganho de peso semanal recomendado reduziu em todas ...

PURPOSE: to investigate the application of a multidisciplinary educational program to high-risk pregnancies due to endocrine diseases. METHODS: we retrospectively evaluated the application of a multidisciplinary educational program to 185 pregnant women with endocrine diseases referred to a maternity specialized in high-risk pregnancy. All pregnant women received multidisciplinary prenatal care from a team consisting of endocrinologists, obstetricians, sonographers, nurses and dietitians. Oral and written information about healthy habits, diabetes care, use of artificial sweeteners and exercise during pregnancy was given to all patients at the first endocrine consultation. An individualized nutrition plan was prepared on the occasion of the first visit to the nutritionist. In bi-weekly and monthly endocrine and nutritional visits, respectively, information about healthy changes in lifestyle was emphasized and the weight was recorded. Adherence to physical activity and nutritional counseling was self-reported. We compared the weekly weight before and after the intervention, fetal weight at birth, rate of macrosomia and low birth weight, and frequency of cesarean delivery among the four categories of body mass index (BMI) before pregnancy (<18.5, from 18.5 to 24.9, from 25 to 29.9 and >30 kg/m²). RESULTS: the main disease of referral was diabetes (84.9 percent). One third of the pregnant women (31.2 percent) were overweight and 42.5 percent were obese before pregnancy. Most women was first seen by the multidisciplinary team in the third trimester of pregnancy (64.1 percent) and 50.5 percent exceeded the recommended weight gain at first evaluation. Obese women exceeded the recommended weight gain in 62.5 percent of cases. After the intervention, the percentage of women who exceeded the recommended weekly weight gain was reduced in all categories of pre-pregnancy BMI, although a statistically significant difference was found only in the ...

[Application of multidisciplinary educational program in high-risk pregnancies due to endocrine diseases]. / Aplicação de programa educativo multidisciplinar em gestações de alto risco devido a doenças endócrinas.

PURPOSE: to investigate the application of a multidisciplinary educational program to high-risk pregnancies due to endocrine diseases. METHODS: we retrospectively evaluated the application of a multidisciplinary educational program to 185 pregnant women with endocrine diseases referred to a maternity specialized in high-risk pregnancy. All pregnant women received multidisciplinary prenatal care from a team consisting of endocrinologists, obstetricians, sonographers, nurses and dietitians. Oral and written information about healthy habits, diabetes care, use of artificial sweeteners and exercise during pregnancy was given to all patients at the first endocrine consultation. An individualized nutrition plan was prepared on the occasion of the first visit to the nutritionist. In bi-weekly and monthly endocrine and nutritional visits, respectively, information about healthy changes in lifestyle was emphasized and the weight was recorded. Adherence to physical activity and nutritional counseling was self-reported. We compared the weekly weight before and after the intervention, fetal weight at birth, rate of macrosomia and low birth weight, and frequency of cesarean delivery among the four categories of body mass index (BMI) before pregnancy (<18.5, from 18.5 to 24.9, from 25 to 29.9 and >30 kg/m²). RESULTS: the main disease of referral was diabetes (84.9%). One third of the pregnant women (31.2%) were overweight and 42.5% were obese before pregnancy. Most women was first seen by the multidisciplinary team in the third trimester of pregnancy (64.1%) and 50.5% exceeded the recommended weight gain at first evaluation. Obese women exceeded the recommended weight gain in 62.5% of cases. After the intervention, the percentage of women who exceeded the recommended weekly weight gain was reduced in all categories of pre-pregnancy BMI, although a statistically significant difference was found only in the group with normal pre-pregnancy BMI (40.6 versus 21.9%, p = 0.03). At birth, average fetal weight was similar among the various BMI categories (p=0.277). Macrosomia was more frequent in women who were overweight and obese before pregnancy. Cesarean delivery was the most frequent route of delivery, regardless of pre-pregnancy BMI. CONCLUSIONS: in high-risk pregnancies due to endocrine disorders, a multidisciplinary educational approach limits excessive weekly weight gain despite the advanced gestational age.

Avaliação da analgesia de opioide tópico em úlcera de perna de paciente falcêmico / Evaluation of the topical application of opioid analgesia for a leg ulcer of a sickle cell disease patient

A doença falciforme é caracterizada por apresentar várias alterações clinicas e fisiopatológicas nos pacientes que por ela são acometidos. Uma dessas alterações é presença de úlceras de perna dolorosas e de difícil cicatrização, sendo necessário o apoio de equipe multiprofissional no seu manejo e tratamento. Com o objetivo de reduzir a dor associada a úlcera de perna, o paciente falcêmico faz uso de opioides parenterais e enterais que normalmente estão associados a efeitos colaterais indesejados. Com o objetivo de reduzir o uso desses opioides sistêmicos, avaliamos um gel de morfina, de fácil manipulação e baixo custo, que foi utilizado antes e após o processo de troca de curativo das úlceras de perna dos pacientes falcêmicos atendidos em nossa instituição. Baseados na escala analógica da dor foi avaliado o efeito analgésico do gel em 28 pacientes. Todos apresentavam dor grau 7 ou 8 antes da aplicação do gel. Vinte e quatro pacientes (85,7 por cento) apresentaram total ausência de dor por um período de 24 horas, não sendo necessário o uso de analgésicos sistêmicos. Em três pacientes (10,7 por cento) a ausência de dor durou um periodo de 12horas. Somente um paciente (3,6 por cento) não relatou analgesia apos o uso do gel. Os resultados demonstraram que o gel é altamente eficaz no controle da dor das úlceras de perna de pacientes falcêmicos.

Sickle cell disease is characterized by several clinical and pathophysiological changes including painful leg ulcers. These are difficult to heal and require the support of a multidisciplinary team in their management. The treatment of pain in these patients usually involves the use of opioids. In order to reduce the use of systemic opioids, we evaluated an easy-to-use low-cost morphine gel (0.12 percent) that was applied before and after changing leg ulcer dressings of sickle cell patients treated in Hemorio hospital. Based on the Analogue Pain Scale (APS) we evaluated the analgesic effect of the gel with 28 patients. All presented with a degree of pain of 7 or 8 before applying the gel. A total absence of pain was observed by 24 patients (85.7 percent) within thirty minutes of applying the gel, with the analgesia effect being maintained for a period of 24 hours and thus the use of other analgesics was not requiring. In 3 patients (10.7 percent) no pain was reported for a period of 12 hours. Only 1 patient (3.6 percent) reported no analgesic effect thirty minutes after the application of the gel. Our results indicate that the morphine gel was effective in controlling the pain of leg ulcers in this group of sickle cell patients. A controlled study should be designed to assess this important subject.