This question is extremely difficult to answer because to my knowledge there are no sound data related to this question. SCD is prevalent in most of the countries of Western Asia (Middle East, Arabic Peninsula) and Southern Asia, most notably India, which is the 3rd contributor to new cases yearly (after Nigeria and the Democratic Republic of Congo). I would suspect that because of extremely heterogenous resources from one country to the other, the different attention payed by Health Authorities and most of all the different level of health care in general, life expectancy will be extremely different from one country to the other. Your question is important and studies on the topic are badly needed.
I agree with Prof. Elion, there is a need of survival rate based on age factor for Asian SCD patients specially from India and Saudi Arabia as this haplotype is different than rest of the African and US and UK patients.
There are few documented studies,
1. Kar et al (1986, Lancet, ii, 1198-201, "Sickle cell disease in Orissa state: India")
2. Gelpi (1979, Clin. Genet. 15, 307-10, "Benign sickle cell disease in Saudi Arabia: survival estimate and population dynamics")
Study indicates that Indian SCD patients survive longer than African haplotypes and an almost 100% survival to adult life in SCD patients from Saudi Arabia.
I carried out most the studies at field level and direct screening of SCD patients.
In one of the large screening of random population survey within less than 100 sq. km area (file attached with this answer), (N=3408, AA= 2829, AS= 526 and SCD=53),
Out of 53 SCD patients with 51 SS patients, age of the oldest male patients was 40 and for female it was 35. But large scale studies are needed to explore more on this. And we will be taking into consideration these issue in our future studies in India.
Poster Sickle cell anaemia carrier prevalence amongst different tri...
Thanks for this useful comments Devendra. The two studies you mention date back from the 80's, i.e. about 35 years ago. In the US as well as in France and the UK, the gain in life expectancy has increased by about 15 years over the past 20 years (Elmariah H, Am J Hematol 2014). This illustrates how much new studies are needed and I wish to congratulate you for your very active SCD research in Maharashtra. Keep your efforts alive!
Concerning Saudi Arabia, the situation is extremely interesting, because on the Eastern part of the country, patients indeed carry the Indian haplotype and tend to have a more benign presentation of the disease, whereas on the Western part of the country, patients carry the Benin haplotype. Saudi Arabia, therefore provides a unique opportunity for the researchers to try to decipher the respective participation of the environment (a single one) and of the genetic background which clearly goes far beyond the globin haplotype only.
Thank you so much professor Jacques and devendra; well iam doing my thesis on "quality if life of adult sickle cell disease patients" in Pakistan. And for that I have interviewed 14 adult patients. The maximum age was 50 for male and 40 for female.
I have some experience of working with some sickle cell anemia. Life expectancy in Bangladesh of SC anemia patient is average 5-7 years less than normal. Still, we need to do proper study.
Thanks Babu for sharing your experience in Bangladesh. I have 3 questions: 1. what is "normal" life expectancy in Bangladesh, 2. is your experience based on a feeling or on a systematic study, and 3. to my knowledge, there is no national newborn screening for SCD in Bangladesh, then how did you appreciate under-five-mortality?
I also have a remark, "SC anemia" can be misleading to many readers outside of the Indian subcontinent because "SC disease" is a specific sickle cell syndrome observed in some patients of African descent due to the cohinheritence of HbS and HbC, the later being commonly observed in Western Africa. SC disease tends to be milder than SS anemia. To be precise the term Sickle Cell Anaemia (SCA) is to be used for patients with the SS genotype and Sickle Cell Disease (SCD) for patients with an other genotype (most often SC or Sb-Thal)
@Jacques Elion
Answers --
Q1 . Around 70 years.
Q2. Systemic study. But people has less enthusiasm to publish in international journal.
Q3. It goes broadly under hemoglobinopathies study. I believe, if some day, my professor intended to publish our work in international journal , i can show you bunch of authentic data.
Best regards,
Babu
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