Talk:Sickle-cell disease

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[edit] older entries

Just as a comment for further discussion, I did hemoglobin research for about 4 and a half years. I recall a conversation (ca 1993 or 1994) with Professor Gary Ackers (at the time, chair of biochemistry and molecular biophysics at Washington University in St Louis Medical School, and one of the leading experts on hemoglobin cooperativity) about a paper, in which they mapped the site of action of the digestive enzyme the malaria parasite used on hemoglobins (as Hb is what they eat). It turns out that in the process of polymerization (HB S now, the sickle cell Hb), the polymerization caps the site of digestion, making Hb a poor meal for the parasite. That, to my understanding, was the way that in heterozygous individuals the mutation had selective pressure against the parasite. It slowed the rate of growth, because they couldn't feed as effectively on heterozygous individuals. Inter-cell death seems to be a poor mechanism for selective pressure, as few cells are sickled in heterozygous individuals. dwmyers

[edit] Amyloid

Someone added the ungrammatical statement that Hb polymerisation was a form of amyloid. It isn't, really. Amyloid is the accumulation of polymerised proteins in end-organs (kidney, heart, brain etc), which generally does not seem to occur in sicklers. JFW | T@lk 10:41, 4 Jul 2004 (UTC)

[edit] Pauling

Not a word about Pauling. Where do we insert this classical reference: L. Pauling, H. A. Itano, S. J. Singer, and I. C. Wells (1949) Sickle Cell Anemia, A Molecular Disease Science 110, 543 - 548. JFW | T@lk 00:47, 23 October 2005 (UTC)


[edit] New Treatments

I have edited this page in order to give new info to people looking for infomation about new treatments becoming available. I have given as much information as i know about what's coming out. Please give me a shout if theres an issue with my post. Also.. I was having poblems making links to the available websites that had more info for more research. I'm at HisNHers2006@yahoo.com I care about the subject and the site and I want to have the best info available for wiki users and people just checking it out. If you can help my post, please do. Also please contact me for updates.

Please don't sign your edits. Also, your addition read like an advertisement. I've changed it to reflect what Niposan actually is (phytomedicine). You are free to add the Cochrane Review, but leave external links for a to-be-written Niposan page. JFW | T@lk 15:54, 26 January 2006 (UTC)

ok gocha, but how about more on the new gene therapies that are being developed? I left a link to a great article ..did you see it? Is that bad wiki etiquette? Also How do wiki users contact each other to collaborate and discuss articles?

Sorry, it must have been an edit conflict (i.e. when I save my page just after you've saved a version). It's probably better not to use the "external links" section for sources. If you have a good source for the gene therapy of HbSS then please cite it. Unauthoratitive sources (i.e. not peer-reviewed) may be less ideal.
Talk pages are used to discuss articles. As for medical articles, there's a lot of physicians and medical students on Wikipedia_talk:WikiProject Clinical medicine, and people interested in medication at Wikipedia:WikiProject Drugs. JFW | T@lk 21:46, 26 January 2006 (UTC)

[edit] Well-known victims

GEORGE BUSH DESERVES TO DIE —Preceding unsigned comment added by 24.34.111.199 (talk) 14:47, 29 April 2008 (UTC)

I removed this list, as it is unclear whether these people are "victims" or simply carry HbS. JFW | T@lk 00:40, 16 February 2006 (UTC)
I am not sure about Davis or Jakcson, but I know that Williams, Watkins, Tillman, and Prodigy the rapper had/have full-blown sickle-cell disease. Tillman in fact died from sickle-cell related complications, and Watkins is a spokesperson for the Sickle Cell Disease Association of America. --FuriousFreddy 01:29, 4 March 2006 (UTC)

[edit] JAMA 1989

Whoever wants to dip into the history of the discovery: PMID 2642320. JFW | T@lk 00:48, 3 May 2006 (UTC)

[edit] Nigerian Treatment

Most of the information for the "Nigerian Treatment" does not belong in this article. I don't see how a US SEC document has anything to do with sickle-cell disease. The same with the comment about the Nigerian ruler. At least the trial results are relevant, although they are better served in notes. Of course, any kind of number like that has to be cited.

I look forward to seeing the citations and making this portion of the article as strong as the rest. TedTalk/Contributions 14:10, 21 July 2006 (UTC)

Couple points/suggestions: Firstly is the drug effective or not - for wikipedia the issue will be whether one can WP:Cite from a WP:Reliable sources to WP:Verify the information being added. This nneds to await the outcome on the phase III trials and the publishing in a peer reviewed journals - until then it repains a comapany/personal hope for the success of the research at confirming its claimed actions - this though is prohibited from wikipedia (for now) under WP:No original research. Secondly any information about the development of the drug, should be placed in a separate article about this drug, not within Sickle-cell disease - I'm just not sure that the drug is (yet) able to claim notability (given the absence of published research) and the article would likely be nominated for deletion (WP:AfD). David Ruben Talk 14:59, 21 July 2006 (UTC)

What what it is worth, here is the link to much of the information:[1]. It is, of course, copyrighted. At least some of the material being added is copied directly from this source. A recent addition also had the words "Back to top" at the bottom of the addition, further suggesting it was cut-and-pasted from some as-yet-unknown website. Most likely, violating copyright. TedTalk/Contributions 20:37, 21 July 2006 (UTC)

Moved - I agree shame that original research not cited as opposed to the company's own selective quotes. However on searching PubMed some validation to the product found (not under current international & US names but its former research name of 'Niprisan'). A Cochrane review cautions that reduced crises episodes reported in a later larger trial was not match by reduced complications or rates of anaemia. However more pressing is that this drug is still being evaluated, yet currently occupies a length in the article greater than other established treatments. I have therefore cut it drastically, in this article, to just a mention under the "Future treatments" section, and moved the rest to a newly creatd article of Nicosan. I've given it a little more structure, started marking up the references with fuller citation details and will continue to add a few more papers to cite (I'ld be grateful if other editors could give me about an hour to add this extra material in). NB I've added the cochrane review as it added balance and thus NPOV to what was previously just a quote from the company's own claims. David Ruben Talk 23:37, 21 July 2006 (UTC)

[edit] STOP trial

The STOP trial has shed some light on the use of transfusion to prevent stroke in children with HbSS and the role of transcranial doppler[2]. JFW | T@lk 20:14, 26 July 2006 (UTC)


[edit] Simple test for sickle cell carriers

I read this in a test on genetics in GCSE Biology. If a sample of a carrier's blood is kept in low oxygen conditions, a few cells with become sickle shaped. This is roughly what the test said:

A couple are worried that they might pass on sickle cell anaemia to their children, so they had blood samples taken for a test. The father's sample developed sickle cells in low oxygen conditions whereas the mother's didn't. What does this mean?

Of course, it meant that the couple's children would a half chance of becoming a carrier, but won't develop the disease. But since I can't obtain the paper, I can't prove that it was there, but I doubt that it's a lie.


[edit] what does this mean?

from the first paragraph: "Once the two mutated genes inherited from each parent link together, the DNA nucleotides are pushed into the bloodstream after the "T" is replaced with a "G". The nucleotide then copies itself in the bloodstream and it eventually makes the proteins inside the bloodcells stretch out and form into a sickle shape. The whole paragraph is really confused and confusing and some of it doesn't make sense (The nucleotide then copies itself in the bloodstream??) Does anyone want to write it better? (I'd like too but I'm not sufficiently informed) Plch 22:34, 17 March 2007 (UTC)

Whoa--that sounds pretty bad. I'll have a look.--Dr.michael.benjamin 03:32, 18 May 2007 (UTC)

[edit] physical appearance

this article lacks that it makes you look pale.

Most patients are black, and pallor is much harder to detect is that ethnic group. The conjunctivae/sclerae may look pale, but will be jaundiced in a crisis. JFW | T@lk 15:05, 12 September 2007 (UTC)

What is "black"? This doesn't sound like a scientific or medical determination. Please re-word to refer to "darker skin" or climatic region. Leon Spencer, Animis Opibusque Parati 00:49, 31 March 2008 (UTC)

This is the talk page. Scientific terminology is not required. --78.86.137.221 (talk) 13:57, 10 April 2008 (UTC)

[edit] Thrombotic risk

There appears to be an increased thrombosis risk. doi:10.1111/j.1365-2141.2007.06740.x addresses this is more detail. JFW | T@lk 15:05, 12 September 2007 (UTC)

[edit] Advances in treatment

This article from The Washington Post (December 7, 2007) gives evidence that there are advances in treating sickle cell anemia underway. Granted it's in mice, but perhaps it should be documented here? I personally don't feel capable of doing anything constructive or encyclopedic with this info, but if anyone has a way to find more references and come up with a good way of introducing the topic, it seems worthy of inclusion. Any thoughts? Isaiah (talk) 13:36, 7 December 2007 (UTC)

[edit] "sickle-cell" or "sickle cell"?

Can someone please standardize the use of the term "sickle-cell" or "sickle cell" throughout the article? I see the term used interchangeably with or without the dash all over the place. I'd do it myself, but I'd prefer that we use the more correct spelling, if there is one, and I don't know which that is. Considering the title of the article I'd go with the one using the dash currently, but I think a more authoritative source is in order. Thanks. -- HiEv 07:55, 16 December 2007 (UTC)

When "sickle cell" is used adjectivally, it should be hyphenated. And when it is used nominally, it should be broken with a space. So, "sickle-cell disease involves sickle cells" is not inconsistent but standard English. 68.188.2.182 (talk) 01:13, 9 February 2008 (UTC)

[edit] Hb F formation

The defect does not lie in the delta chain. So is also the case in Thalessimias. Then why does Hb F predominate in Sickle Cell Disease and Hb A2 predominate in Thalessimias when both should be raised in both the diseases. 59.180.25.104 (talk) 12:16, 28 February 2008 (UTC)Bis

[edit] Duplicate entries

Most information listed under "Vaso-occlusive crisis" is listed again under "Complications". But I'm new here, so I'm not comfortable changing it myself. Someone may have done it on purpose, after all. —Preceding unsigned comment added by 64.198.112.213 (talk) 16:09, 29 February 2008 (UTC)

I think it is also a reasonable edit that needs to be made since information is listed twice.--DavidD4scnrt (talk) 07:48, 16 April 2008 (UTC)

[edit] Mostly Sub-Saharan Africa?

Article cites "Sickle-cell disease occurs more commonly in people (or their descendants) from parts of the world such as sub-Saharan Africa..." This is incorrect. "Sub-Saharan Africa" is a geologicial region - not a group of people. I think you want to say tropical and subtropical climates around the world. Sickle-cell (or the trait) has long been common in people of African descent due to climate - since ancient times.

Yahoo Article:Study shows life was tough for ancient Egyptians

"Studies on the remains of ordinary ancient Egyptians in a cemetery in Tell el-Amarna showed that many of them suffered from anemia, fractured bones, stunted growth and high juvenile mortality rates, according to professors Barry Kemp and Jerome Rose, who led the research."

An article citing the history of sickle-cell:

"Although the disease does not distinguish between the rich and the poor, it does single out those from the tropical and subtropical climates of the Old World...One long-held theory as to why it was so common in the tropics was its association with malaria. In the 1940s, E.A.Beet, a British medical officer stationed in Northern Rhodesia (now Zimbabwe), observed that blood from malaria patients who had sickle cell trait had fewer malarial parasites than blood from patients without the trait. Following this observation, a physician in Zaire reported that there were fewer cases of severe malaria among people with sickle cell trait than among those without it."--Innvista Health
Leon Spencer, Animis Opibusque Parati 00:46, 31 March 2008 (UTC)

[edit] Sildenafil for PAH in SCD

doi:10.1111/j.1365-2141.2005.05625.x JFW | T@lk 15:18, 2 April 2008 (UTC)

Genes that increase risk of PAH - PMID 18187665. JFW | T@lk 14:30, 13 June 2008 (UTC)

[edit] NIH consensus conference

The NIH held a consensus conference on 17–22 September 2007. Hydroxyurea was discussed in extenso.

It's actually fascinating that despite all the reviews and conferences, longer-term outcome trials are still sparse. JFW | T@lk 05:40, 6 May 2008 (UTC)

[edit] Differentiate between disease and trait

People who have the sickle cell trait (one recessive gene) do not have 'sickle cell disease' or 'sickle cell anemia.' They have 'sickle cell trait.' This article should probably reflect that. Woland (talk) 21:03, 20 May 2008 (UTC)

True, although typically will have slightly lower haemoglobin levels than the norm and in extreme conditoins might still have crises (eg high altitude mountaineering, commercial aircraft depressurisation and medical/surgical problems causing hypoxia). But I agree the absence of any mention of trait status in the leadin and just single line in Sickle-cell disease#Heterozygotes section needs correcting. David Ruben Talk 02:12, 21 May 2008 (UTC)
There is an article Sickle cell trait (in a very sorry state with manual reference numbering - I'll fix that too), which has a disambinguation note at its top to this Sickle-cell disease article, I'll reciprocate the same back David Ruben Talk 02:30, 21 May 2008 (UTC)

[edit] Disease vs. illness

The text was just changed, all 'disease' became 'illness'. Not a way. I think it should be changed back. --Dumarest (talk) 11:30, 9 June 2008 (UTC)

No discussion has happened - someone changed the Infobox back to disease - I will revert. Sickle cell is a disease, not just an illness. --Dumarest (talk) 17:34, 10 June 2008 (UTC)