Sickle Cell Anemia

When the oxygen carrying capacity of the red blood cells is impaired it results in a condition known as anemia; there are many reasons for anemia all of which describe this deficiency in the oxygen carrying capacity of the red blood cells within the body. Due to a genetic defect some individuals have red blood cells that are sickle or crescent shaped and this impairs the ability of the cells to carry oxygen leading to chronic anemia in a condition called sickle cell anemia-which is an inherited disorder in all individuals who suffer from it. Because of this peculiar shape, the red blood cells often clog the smaller of the blood vessels and this leads to an impairment of the oxygen varying capacity of the blood leading to the appearance of the symptoms typical of sickle cell anemia; thus a physical defect in the morphology of the cell leads to its inability in delivering oxygen.

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Due to this clogging on the blood vessels, there may be a severe deprivation of oxygen in the tissues and this leads to a sickle cell crisis; which is a painful episode physically manifested in the body of the individual. People of European descent rarely have this genetic defect and therefore the disorder or the particular genetic trait occurs mostly in people of African descent, and also in people from the Mediterranean countries, the Middle East and India, and the Middle East; the cause of this illness is genetic and therefore strictly inherited being passed from parent to child.

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Some of the more severe manifestations and symptoms that can appear in such individuals include problems like excruciating pain in the joints and the abdominal region due to clogged vessels, fatigue is another symptom, the person may suffer bouts of irritability, there is also often a yellow discoloration of the skin and the white of the eyes, the legs often develop painful sores, and the person may suffer from gum disease, blindness can occur later in life and the person has frequent bouts of different respiratory infections, another symptom that occurs in males is prolonged and painful erections of the penis. Fever, nausea and difficulty in breathing often accompany episodes of severe pain in the regions of the body such as the arms and the legs, the areas of the chest and the abdomen; these sensations may come and go over a period of time. A person inherits the full fledged form of sickle cell anemia with persistent symptoms from his or her parents only if both the copies of the sickle cell gene is passed down from both parents and not otherwise. Individuals who inherit the genes for the sickle shaped cell only from one parent are affected by what is known as a sickle cell trait and will not display any of the usual symptoms associated with the sickle cell anemia.

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While most of the preliminary studies conducted on patients show that the dietary habits of individuals with sickle cell anemia consists of healthy food and nutrient levels and that all such diets in general meets or exceeds recommendations differing little from the diet of healthy individuals-it is still observed in general that many sickle cell anemia affected individual tend to be nutritionally deficiency in many vital organic nutrients. An increased need for and utilization rate in the bodies of the affected individuals may be a plausible reason for the higher rate of nutrient deficiencies observed in the sickle cell patients in general. This view has not been confirmed, and also studies must still be conducted on the suitability of patients for dietary interventions that aim to supply adequate nutrients to meet these higher demands in individuals suffering from sickle cell anemia. Thus controlled trials are awaited on this particular point.

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Supplements and herbs

A life threatening complication that often occurs in patients with sickle cell anemia is pulmonary hypertension; during one preliminary study, such individuals with pulmonary hypertension were administered supplements of L-arginine in the dosage amounts of 100 mg per 2.2 pounds of body weight per person, these were taken thrice every day for the five days that the trial lasted. A 15% decline in the pulmonary artery systolic pressure was observed in most of the patients and thus this supplemental L-arginine treatment led to a significant improvement in the pulmonary hypertension, as far as the individuals affected with sickle cell anemia in this group were concerned. The results of these preliminary studies still need verification through longer-term studies conducted under stricter regimens and in controlled environments.

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A deficiency of the vitamin B12 is also often observed in patients afflicted with sickle cell anemia. A comparative study that had groups of healthy children alongside children with sickle cell anemia found the children with sickle cell anemia to have a higher incidence of vitamin B12 deficiency in general. Similarly, when compared to healthy adults, the results of trials of 85 adults with sickle cell anemia pointed to a greater incidence of deficiency in the vitamin in the sickle cell affected group as a whole. However, a significant reduction in the symptoms associated with low levels of the vitamin B12 can be achieved through intramuscular injections of 1mg of vitamin B12 taken weekly for 12 weeks by a group of patients affected with deficient levels of vitamin B12 who were all sickle cell affected individuals-this was the result of a preliminary trial and requires confirmation studies. Oral supplementations using vitamin B12 and its ability to help out patients with sickle cell anemia who suffer from a vitamin B12 deficiency have not been investigated by researchers-therefore the efficacy of supplementation as such and oral supplements in particular still have to be studied as far as patients with sickle cell anemia are concerned; all this is of course in regard to perceived or real instances of vitamin B12 deficiency.

Twenty patients suffering from sickle cell anemia took part in a preliminary trial, they were all administered either 1mg of folic acid per day of the trial or folic acid along with 6 grams of aged extracts of garlic, and to this was added 6 grams of the vitamin C, and finally followed with 1,200 mg of vitamin E every day during the six months that the trial lasted. In comparison to the group taking folic acid alone, the patients given the combination formula had a greater and much more significant improvement in their hematocrit-this is an index of anemia and these patients also experienced lesser painful crises than the previous group following the trial.

When compared with healthy individuals most patients with sickle cell anemia were also more likely to have elevated levels of homocysteine in the blood so a report from a preliminary research suggest. This is very relevant to health as one of the risk factors form the development of cardiovascular disease in an individual is the elevation of the levels of blood homocysteine. A higher incidence of stroke in patients suffering from sickle cell anemia in particular, have been linked to the high levels of homocysteine that such patients displayed in their blood. Additionally, the elevation of the homocysteine levels in the blood is contributed by many other nutrient deficiencies; thus a lack of the vitamin B6, the vitamin B12, and nutrients like folic acid are often present with greater regularity in patients suffering from sickle cell anemia compared to normal individuals. This can probably be remedied, as supplements of 2-4 mg of folic acid given every day during a controlled trial led to a reduction in the levels of homocysteine in children-these results also depended on the age group of the child, however; supplementation with the vitamins B6 and B12 did not lead to any significant reduction in the levels of homocysteine across all age groups. Additionally swelling in the legs and the ankles often seen in children with sickle cell anemia disappeared or were considerably reduced in their manifestation during a double-blind trial of children; where they were given 5 mg of folic acid every day, such children reported a reduction in the painful sensations in the hands and the feet compared with those children who had received only a placebo. This supplementation regimen however, did not help clear the other abnormalities in the blood and no effect was observed on the impaired growth rate associated with sickle cell anemia. Supplements of folic acid are typically in amounts of about 1,000 mcg administered daily during the treatment of sickle cell anemia. The state of the vitamin B12 within the body must be assessed by a healthcare professional at all times in order to determine detrimental effects due to supplementation; this step is important because supplementation at this level is very high even if folic acid is just a vitamin.

Particularly where women and children with sickle cell anemia are concerned, the deficiency of the mineral iron is relatively common. A lab based test for serum ferritin is the typical method of checking for iron deficiency in people with sickle cell anemia- this method gives the best diagnosis. However, this method of serum ferritin may not be accurate as a test for iron deficiency during sickle cell crises. It is also unclear if iron supplementation is essential or beneficial for people with sickle cell anemia where a deficiency already exists. Some laboratory test have measured that the severity of the anemia is reduced through the use of iron supplements have-so at least according to some sources such supplements may benefit the patient. Paradoxically, other reports suggest that such supplements may actually go the other way and aggravate the symptoms felt because of sickle cell anemia. Added to this is the demonstrated fact that a body in a state of iron deficiency actually reduces the sickling of the red blood cells where the person concerned is suffering from sickle cell anemia. This is further borne out by the facts reported by a small trial which suggest during iron restriction in patients with sickle cell anemia, the clinical symptoms associated with anemia actually went down and it also led to decreased red blood cell destruction in the blood. For the safest option, all sickle cell anemia sufferers must make sure they consult a doctor before or if they decide to go in for supplements of iron or alternately if they are going in for a diet restricted in iron. In all cases a medical doctor will be in the best position to know exactly what is going on and what needs to be done.

Patients with sickle cell anemia also reportedly have lower than normal concentrations of the essential mineral magnesium in the red blood cells. This lowered level of magnesium supposedly contributes to the dehydration of the red blood cells and is concomitant with an intensification of the symptoms. The painful sensations experienced by patients with sickle cell anemia on a daily basis was dramatically reduced and some of the red blood cell abnormalities associated with sickle cell anemia significantly went down during a preliminary trial, where patients underwent administrations of 540 mg of magnesium every day for the six months that the trial lasted; thus in this case supplementation with magnesium was effective in addressing the problem. Magnesium pidolate was the supplemental form of the mineral used during the trial, this form of the mineral is not utilized in most other supplementation regimens that administer magnesium-the effect of other forms of magnesium used in supplemental form against the same symptoms remains unknown and will require further research and controlled trials.

People with sickle cell anemia also benefited markedly during test tube studies that used vitamin B6, the vitamin supplements were seen to have effective anti-sickling effects on the red blood cells of all the test subjects in the group. This may have a basis in fact, because one research has reported that people who have sickle cell anemia when compared to healthy individuals were much more likely to have a deficiency of the vitamin B6. This vitamin B6 deficiency in all such patients suffering from sickle cell anemia was addressed in five patients and who were all given 50 mg of vitamin B6 two times each day as part of controlled trial. The improvement in the anemia was very little and was not significant at all after the trial; however the vitamin B6 was reversed with the supplement. Since the results are inconclusive at best, the use of supplemental forms of the vitamin B6 in people with sickle cell anemia is not verified and it is probable that no real positive aspects recommend such supplementation.

The damage from free oxygen radicals in the cellular structure of the body is repaired by antioxidant nutrients; these also protect the body against further attack from such free radicals. Low levels of many essential nutrients are seen in the blood of people with sickle cell anemia, though consumption of food groups containing these nutrients may be normal or above normal, all sickle cell anemia affected individuals display deficiencies in the levels of essential antioxidants, nutrients like the carotenoids, and the various vitamins such as the vitamins A, vitamin E, and vitamin C- this situation does not seem to have any reason whatsoever. The fact is that a lowering of nutrients in the blood of patients with sickle cell anemia may have adverse effects on the health of the individuals-thus some researchers have pointed out that the incidence of symptoms in adults is greater when they have low blood levels of the vitamin E, and in children with sickle cell anemia, the deficiency of vitamin E tended to be manifested in the increased number of diseased cells that were present in the body. Six sickle cell anemia patients were given 450 IU of the vitamin E every day for up to 35 weeks as part of a small preliminary trial-there was a 44% decrease in the average number of diseased cells in the body of patients because of this supplemental regime. Moreover, this continued as long as the supplementation was carried out-thus by addressing the deficiency of vitamin B6 in the patients, many of the side effects and symptoms can be resolved.

Supplemental combinations of two supplements were administered during another preliminary trial that took place over seven to eight months on patients with sickle cell anemia. All patients were given an initial first combination supplement that included 109 mg of the mineral zinc, 153 IU of the vitamin E, 600 mg of the vitamin C, along with 400 ml-about 14 ounces of the oil of the soybean containing roughly 11 grams of linoleic acid and about 1.5 grams of alpha linolenic acid-this was the initial dose of the first combination. This initial dose was followed by the second combination supplement that had 140 IU of the vitamin E, along with 600 mg of the vitamin C, accompanied by 20 grams of fish oil equivalent to 6 grams of the omega-3 fatty acids. Only the first protocol or supplemental combination brought about some reduction in the number of diseased cells in the bodies of patients.

Studies on the use of fish oil alone have also been carried out. Thus some patients with sickle cell anemia who took part in a double-blind trial, where supplementation was carried out with menhaden oil, reported a reduction in the frequency and severity of the painful episodes-this was compared to a group given placebos and the improvement in the test subjects was found to be about 45 % over the placebo group-the dosage regimen was 250 mg of the oil per 2.2 pounds of body weight per day for one year that the trial lasted.

Additionally many of the various aspects behind the illness seem to be linked to a deficiency of the essential mineral zinc in the body of patients with sickle cell anemia-this deficiency of zinc therefore has an effect on many complications observed during the condition. To elucidate this point, children with deficient levels of the mineral zinc who were also suffering from sickle cell anemia had very poor growth-this result was observed during preliminary studies done on children with sickle cell anemia. Additionally supplementation with zinc was studied during another preliminary trial, where 12 of the participants all suffering from sickle cell anemia received 25 mg of zinc every four hours for 3 to 18 months that the treatment lasted. Absolute numbers of the red cells that were damaged fell from 28% to 18.6% within the bodies of these participants following the trial. The action of the zinc was not inhibited by the addition of 2 mg of copper per day as a co-supplement. This was done because the supplements of zinc in the absence of copper supplementation at the same time always induce a copper deficiency in the body of an individual. The responses differed however, thus in those patients in whom the damaged cell count was minimal to nil - that is less than 20% irreversibly sickled cells, experienced very little benefit and showed almost no response in marked contrast to patients with a lot of damaged cells who received the greatest benefit and responded well to the supplementation. About 75% of adults who suffer from sickle cell anemia also tend to be affected by chronic leg ulcers. Zinc may have some effect on the reduction of ulcer formation, 88 mg of zinc was supplemented thrice a say during a controlled trial, where the sickle cell patients with low blood levels of zinc and corresponding problems with leg ulcers received supplements every day of the 12 week trial. When a comparison between the placebo group and the zinc supplemented group was made, the healing rate for ulcers was more than three times faster in the group who utilized supplements of zinc.

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