Q: I am 24 years old and suffering from sickle-cell anaemia. I weigh 74 kg and I am 5 feet 10 inches tall. For the last 6 years, I am taking a capsule of Hydroxyurea daily. Since then my life has changed for better, with lesser frequency, severity, and duration of pain, illness and crisis. I keep myself hydrated with plenty of water. Is Hydroxyurea safe? How long can continue I with hydroxyurea? Is there any alternative to hydroxyurea? I have recently heard about a medicine called Nicosan (Hemoxin in US). How effective is it, as it claims to have lesser side effects than Hydroxyurea. Is there any sort of exercise, which patients of sickle-cell anaemia should follow?

A:Hydroxyurea increases the production of fetal haemoglobin, which decreases the number of deformed red blood cells. Therefore, it reduces the frequency of sickle cell crises. Not everyone is treated with hydrea and it is given only to patients over age 18 who have had at least three painful crises in the previous year. The ability to father a child may be affected by the drug as the developing fetus may be affected. It is necessary to use effective contraception for up to several months after stopping the drug. The dose of hydrea is 20 mg/kg/d orally and this can be increased by 5 mg/kg/d every 12 weeks to maximal acceptable toxicity, usually considered to be a total leukocyte count (TLC) above 4,000/ƒÝl. Till such time that a stable dose is established, a TLC should be done fortnightly and liver & kidney functions should be monitored every month. Nicosan is a phytochemical i.e. a chemical or nutrient derived from a plant source that is not required for normal functioning of the body but that nonetheless has a beneficial effect on health or an active role in the amelioration of disease. Thus, phytochemicals differ from traditional nutrients in that they are not a necessity for normal metabolism, and their absence will not result in a deficiency disease. It was developed at the Nigerian National Institute for Pharmaceutical Research and Development and they have already conducted Phase I, Phase IIa and IIb clinical trials and are currently conducting Phase III clinical trials in Nigeria. It is made from four botanical species indigenous to Nigeria and consists of extract of Sorghum bicolour leaf, Pterocarpus osun stem, Piper guineense seed and Caryophylli flower. It is currently classified as an orphan drug i.e. a drug used to treat rare diseases whose development may not offer the pharmaceutical industry a return on the research investment. Early trials did show a benefit but the Cochrane Collaboration’s review on phytochemicals for SCD noted that Nicosan "did not appear to affect the risk of severe complications or the level of anaemia". It is as yet premature to say what the impact of this drug will be. Exercise regularly but not so much that you become really tired. When you exercise, drink lots of fluids, at least 8 glasses of water a day, especially during warm weather. Due to anaemia, you may have less stamina than haematologically normal individuals and therefore exercise should be at your own pace. Aerobic exercises like running, swimming, walking and biking are recommended. Avoid swimming in cold water. You should acclimatise gradually to heat and humidity and condition carefully and gradually for several weeks before engaging in strenuous workout. If going to an altitude, acclimatise to it over an appropriate period of time.