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Sickle Cell Society Tel 020 8961 7795 |
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If you have any questions please email info@sicklecellsociety.org Q. My sister has sickle cell anaemia and is expecting her first baby next month. Her health has been up and down and blood tests showed that her white blood cell count is higher than her red blood cell count but her haemoglobin levels are high. What could this mean? A. It sounds as if your sister has a high white cell count in her blood. This can sometimes be caused by infection, but is very common in sickle cell anaemia and also during pregnancy. The high haemoglobin does not sound too worrying, although it is difficult to know whether it is high in a good sense (i.e. is not low) or a bad sense (i.e. is too high and might be causing problems). Women get pain and other crises more frequently during pregnancy, even if they have had very few problems with their sickle cell anaemia previously. In general, pregnant women with sickle cell disease should be looked after jointly by an obstetrician and a haematologist, both with experience of looking after people with sickle cell anaemia. If you have any concerns about her condition then I am sure it is best for you and your sister to discuss this with the doctors looking after her. Q. If a patient is going through a sickle cell crisis is it normal for the patient to be very agitated with accompanied cerebral agitation, and also slight confused. A. Acute pain in sickle cell disease is very sevrere and this can certainly make people very agitated and behave in what seems to be an irrational manner. Pain killers and other drugs can also cause agitation and confusion. However, if the confusion and agitation are very severe, there may be other explanations related to complications of the sickle cell disease. Low oxygen levels can cause agitation and confusion and can occur if the sickling process is involving the chest, and also if some of the pain killers are making the patient very sleepy. Another possible cause includes sickling affecting the blood supply to the brain. A third possibility involves changes in the sodium levels in the blood which sometimes accompany the use of intravenous fluids. In general, it is obviously best to discuss any concerns you have with the medical team looking after the patient during the crisis. Q. I am a GP am currently trying to do some research for a friend who’s son has Sickle Cell trait. He has been experiencing a lot of joint pain for many years especially when exercising and when it is cold. I have found little published evidence about such symptom in Sickle Cell trait and wondered if you had information. Thank you for your help. A. I am one of the medical advisers to the Sickle Cell Society and your query was passed on to me. Sickle Cell trait is generally a very mild condition associated with few health problems. One of the very rare problems is damage and rupture of the spleen at high altitudes. Other problems are all rare with Sickle Cell trait, and include small amounts of the blood in the urine, and a tendency to urine infections. Sickle Cell trait can also cause problems if there is exposure to low oxygen levels as can sometimes occur during operations. Overall, the vast majority of people with Sickle Cell trait and is important for other causes to be fully investigated, and for people not to assume that this is an inevitable feature of Sickle Cell trait. One thing is to be certain that your friend’s son actually has Sickle Cell trait (HbAS) and not a form of Sickle Cell disease such has HbSC, are told they have Sickle Cell trait. There are also some very rare forms of Sickle Cell disease which look exactly like Sickle Cell trait on routine haemoglobin testing, which can be performed in a few specialist centres such as kings as college hospital of the central Yours sincerely.
Q. I have heard many reports this drug developed by Xechem International is almost ready to launch production. Could you Please provide feedback on the validity of this drug and if it really has the potential to help those with sickle cell anemia. A. Nicosan/niprisan has been around for some time. A small study was published 5 years ago showing little toxicity and possibly some benefit in patients in whether its is of any benefit to people with Sickle Cell disease.
Q.Why do children with Sickle Cell have Strokes? A.The cause of stroke in children and adults with Sickle Cell disease is complicated and not fully understood. In most children the larger blood vessels in the brain get gradually narrowed, and at first the blood flows faster through these narrowing’s to compensate. It is not really understood why there Is this tendency for large blood vessels to narrow, but it probably is related to the gradual damage to the lining of the blood vessels caused by the abnormal sick red blood cells. Eventually the narrowing gets so great that the blood supply to part of the brain is not enough to supply adequate oxygen and that part of the brain is damaged and dies causing a stroke. The part of the body controlled by that part of the brain then stops working properly, typically causing a hemiplegia with weakness of the arm and leg on either the left or right side. Sometimes abnormal, new blood vessels form to try and compensate for the narrowing, and these can leak and bleed in to the brain causing a different sort of stroke. The blood vessels can also become dilated causing aneurysms which can also bleed in to the brain. In general, the process is different to the blocking of blood vessels by sickled red blood cells which is thought to cause pain and some other problems. In general, when part of the brain is damaged by a stroke, that part of the brain does not recover and a scar forms. However, other parts of the brain learn to take over the functions of the damage part and so movement in the affected limbs atc gradually improves. I am not sure there are any films of this actual process. I guess there may be animated diagrammatic forms of the process although I have not seen one. There are certainly various brain scans available showing the abnormal blood vessels and scarring in the brain. I hope this helpful and please let me know if you need any more details.
Q. I am anaesthetist involved in treating children for dental extraction, working in We have differing views expressed as to which of patients should be tested using sickledex as a screening test prior to dental chair general anaesthesia. One view, which could be termed the traditional view, is all of ethnic origin from sub-Saharan Africa, north and suggest the first view as being more correct, but this would exclude if we test too many children we upset some unnecessarily and waste money but if test too few we may do damage to a child. we would value your comments and recommendations. A. I don’t think there is any straightforward answer to your question. The NICE guidance is not particularly helpful. As you say, Sickle Cell disease is most common in people whose families originate in African or Caribbean countries, but it occurs at significant and sometimes high frequencies in many other countries such as Saudi Arabia and other middle Eastern countries, Greece, Turkey and India. In most parts of the There are two options: testing everyone for sickle preoperatively, irrespective of apparent risk - defining groups at high risk based on their family origins and only testing those if strategy is used, then all the above groups need to be identified and there has to some clear way of doing this. Obviously it is not possible to rely on foreign-sounding names or skin colour, and probably the most appropriate strategy is asking if any family origins. The results of this process should be recorde somewhere. To reliably identify someone’s ethnic background such that it can be used to identify if there risk of Sickle Cell disease is quite time consuming and potentially more expensive than testing everyone with a simple sickle solubility test. It is also important that everyone tested is informed of the result and has access to some sort of counselling/information about the condition. Ultimately, the best approach depends on the ethnic mix of the population you are screening. If a lot of people (possibly > 10%) are at high risk of carrying the sickle gene because of their ethnicity then universal tetsing is probably most efficient/effective, whereas if very few people are at risk a more selective blood testing Might work best, based on accurate determination of family origins.
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