【分　 类】 医药卫生
【关 键 词】 镰状细胞性贫血，患儿，护理，疼痛，贫血，感染。
【来 　源】 互联网
【收　 录】 中文学术期刊网

正文：
【摘要】 镰状细胞性贫血在中东是一个常见病。它是一种遗传性红细胞缺陷型的疾病。患儿的红细胞以镰刀状代替了圆形的并极易交织在一起而阻塞小血管，此镰状细胞僵硬不易变形，还易破碎。因脱水而致疼痛发作频繁，急性贫血，感染等等。护理好此类患儿需要医生和护士们的共同努力。阿曼医院对镰状细胞性贫血患儿在治疗上的优点以及思想观念上的落后像近亲结婚，不堕胎。 
 
【关键词】 镰状细胞性贫血，患儿，护理，疼痛，贫血，感染。
 
Take Care of children with Sickle Cell Disease and their Parents’ education
 
Zhanh Caixia  Baoji Central Hospital, Shaanxi Province, P. R. China (721008)
 
【Abstract】 Sickle cell disease is a common disease in Meddle Eastern Countries. Sickle cell disease is a group of inherited red blood cell disorders. The red cell become a sickle shape instead of the round shape, it break and blot small blood vessels easily when dehydrated due to hot whether. The most frequent complications are painful crises, acute anemia, and infections. Giving a good care of these children need doctors, nurses and their parents co-operate well. To do newborn baby screen earlier to give treatment for the sickle cell children on time.      
 
 
【Kay Words】sickle cell disease, children, take care, pain, anemia, infection.
 
 
镰状细胞性贫血是一种单基因遗传性红细胞缺陷型的遍及全球的疾病。此病最初发生在非洲，印度，地中海，中东等国家。我曾经在阿曼的皇家医院的小儿血液科工作过四年。镰状细胞性贫血在那里是一个常见病。因此我想谈一下我对此病的护理体会。
镰状细胞性贫血患儿的红细胞以镰刀状代替了圆形的并极易交织在一起而阻塞小血管，此镰状细胞僵硬不易变形，极易破碎。由于中东地区天气非常炎热使患儿容易出现脱水而致疼痛频繁发作，急性贫血，感染等等。护理好这些患儿需要医生和护士们的共同努力。从新生儿开始筛选及时给予早期治疗和护理。
首先，需要对新生儿进行筛选检查，做到早诊断，早治疗。 镰状细胞性贫血患儿应每年体检一次，例如血细胞计数，肝脏肾脏功能，心脏肝脏超声等。按时接种肺炎球菌，流感，脑膜炎疫苗等。
第二，对于较大的患儿及家长进行支持教育。对某些严重并发症急性发作的患儿要急诊收住监护病房，特别是难治性疼痛及换血治疗。对危险妊娠夫妇提供遗传咨询和产前诊断。
第三，镰状细胞性贫血患儿只有在懂得此疾病知识的家长以及医护人员的共同努力下才能得到很好的护理照顾。患儿的父母要学会测量患儿的体温。因为发热会是一系列感染的信号需要进一步检查。密切观察所有的并发症：疼痛发作，脑梗塞，新出现的发热，腿上出现溃疡，骨头病变，黄疸，胆结石，肺梗塞，肾损害，发育迟缓，贫血等等①。
以下是一些保持患儿健康的一般原则：
按医嘱给予叶酸，维生素C和铁剂口服帮助机体造血；
每天注射青霉素至７岁为止；
每天饮大量的水；
避免过热过冷；
避免过度用力及紧张；
休息充足；
在正规的医疗机构进行规律的体格检查。
研究表明这个基因遗传性疾病危害着成千上万的人们的镰状细胞性贫血总会有一天被治愈的。骨髓移植可以治愈此病。但是找到匹配的骨髓万分困难②。
最后，我想谈一下阿曼医院对镰状细胞性贫血患儿在治疗上的优点以及思想观念上的落后。
对顽固难治性疼痛的处理：从两路给止痛药。一路从静脉由微量注射泵给吗啡24小时，同时给口服一般止痛药2-4小时。常用的是Paracetamol 1片/次，4小时一次。这点不同于我们国家。我们一般给了麻醉止痛药就不再给一般止痛药了。但他们研究认为从两路及时地给患儿止痛药其效果最好，也就是说，不要等患者感到疼痛了再给药，那样疼痛就很难被控制住了。从临床观察及患者的反应也证实他们这种方法效果很好。我曾参加过皇家医院举办的关于《疼痛管理的新方法》的学习班。
再谈一下他们的一些穆斯林阿拉伯国家的人们不正确的思想观念。他们喜欢近亲结婚，认为亲上加亲会更亲。我们大家都知道，近亲结婚在世界上许多国家都被禁止的。因为许多遗传性疾病都是与近亲结婚有关。还有，他们不做人工流产。他们认为胎儿也是人，一个活着的生命。因此在那里像镰状细胞性贫血，血友病，地中海贫血，纤维硬化症等等的遗传性疾病的发病率很高。
 
(1412字)
参考文献：
 ⑴   Managementof sickle cell disease <<镰形细胞病的管理>>
     Marianede Montalembert, paediatrician
〔2〕Nature Biotechnology (January 2006).
〔3〕 Sickle Cell Anemia  Paedidiatric Nursing，Student Curse Book，2009. Sultan of Oman.
 
 
 
 
Management of sickle cell disease
镰形细胞病的管理
Mariane de Montalembert, paediatrician
Summary points
Care for patients with sickle cell disease requires a network of doctors
Patients with sickle cell disease should receive annual follow-up investigations, such as blood counts, hepatic and renal tests, transcranial Doppler ultrasonography for children, heart and liver ultrasonography
Immunisation is needed for pneumococcus, Haemophilus influenzae type B, meningococcus, and influenza virus
The most frequent complications are painful crises, acute anaemia, and infections
Patients sometimes need admission to intensive care, in particular for treatment of refractory pain and for exchange transfusion
Patients and their families need education and support
Genetic counselling and prenatal diagnosis may be offered to at-risk couples
 
 
000000000
thalassaemia saving lives的问题  
文发布者：来源：寻医问药网
关于Screening for sickle cell disease and thalassaemia saving lives的原因,关于Screening for sickle cell disease and thalassaemia saving lives的相关知识。     The NHS programme for screening newborn babies for sickle cell disease and thalassaemia now covers more than half of England and is on target to reach all newborn babies by March 2005.
 
    A mother watches as a sample of blood is taken from her baby's heel
 
    Credit: RUTH JENKINSON/MOTHER AND BABY
 
    The programme, which was included in the NHS Plan in 2000 after years of lobbying from doctors and patient groups, was launched in September 2003. Since then more than 100000 babies have been screened for the two blood disorders and 124 have been diagnosed, giving a detection rate of 1.2 per 1000 babies screened.
 
    Antenatal screening for these diseases in pregnant women is also being rolled out across the country and is due to reach full coverage by March 2006. However, many trusts already offer antenatal screening, and 95% of carriers of the diseases are currently being detected, said Dr Allison Streetly, the programme's director and a consultant in public health in southeast London.
 
    The prevalence of sickle cell disease in England has increased by 60% in the past 10 years. An estimated 125000 now have the disorder, and more than 700 people have thalassaemia.

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