research on sickle cell anaemia

Research on Sickle Cell Anaemia

Introduction

Sickle cell is an inheritable condition in a person that affects the red blood cells. The hemoglobin in the red blood cells is in the form of *S which is an abnormal condition of hemoglobin. At times the red blood cells adopt a crescent/sickle shape which makes it difficult to pass through small blood vessel. The tissues that do not receive enough blood flow are destroyed hence causing the sickle cell disease. To this condition, we have several types of sickle cell disease, the most common being Sickle Cell disease (SS) and Sickle-Hemoglobin (SC), Sickle Beta-Plus Thalassemia and Sickle Beta-Zero Thalassemia. Sickle cell anemia arises when the sickle cells are destroyed rapidly in the body of the people with the disease. (Wang, 2004)

When the flow of blood is blocked to pass through the vessels the following condition arises; acute chest syndrome, pain episodes (arms, chest, legs, abdomen), prolonged erections that are painful, and organs like the kidney, spleen, and liver are damaged. Genetically, sickle cell trait (AS) arises when there is both the production of both the hemoglobin A and S in the red blood cells hence having more A than S. This (AS) condition is not a type of sickle cell but in it is the healthy situation in a person. The sickle cell disease is inherited from parents, whereby the hemoglobin genes one inherits comes in two sets; one from each parent. For instance, if one parent has Sickle Cell Anemia and the other Sickle cell trait, there is 50 percent chance on each pregnancy of having a baby with either Sickle Cell Anemia or Sickle Cell Trait. That is the baby can have the disease of can be free of the disease. Also, if one parent has Sickle Cell Anemia and the other is Normal, all the children will have the sickle cell trait. Lastly, if both parents have Sickle Cell Trait, 1out of 4 of their children will have the disease. Dunlop R and Bennett K (2006)

Discussion

At first, patients of Sickle Cell Anemia were only a narcotic pain killer called meperidine. In real sense, meperidine should not be a first narcotic to be used because it contains chemicals that accumulate in the body and hence become toxic. This causes seizure and agitation in ones body. Another challenge to this drug is that it is supposed to be administered at an interval of two hours which is involving to the patient and expensive. Also, patients with life-long experience of the disease are offered the same dose they started with during their initial stages of the disease. This is annoying to their part because the emergency department staffers believe they are addicts. In addition, the sickle cell patients were started with early diagnosis especially in the newborn babies. They were given folic acid supplements, vaccination against pneumococcus bacterial, and penicillin prophylaxis. (Mehanna, 2001)

All these forms of treatment entail maintaining the patient health.

Secondly, there is treatment involving complication which includes pain management, antibiotics, intravenous fluids, blood transfusion and surgery. All this methods are accompanied by psychosocial support. For instance, the blood transfusion approach reduces repeated occurrence of pain, complications, risk of stroke in the patient with sickle cell anemia. And because the red blood cells contain iron in them, it is important device a way that helps to remove the iron as when it accumulates in the body it becomes toxic. The excess iron can accumulate in the liver, heart and other organs leading to damage. Therefore, patients who use blood transfusion approach should seek treatment that eliminate iron overload. (Wang, 2004)

Newer alternatives have been embraced help in controlling the disease. Such includes bone marrow transplantation which can help in curing the disease and also causes early death, cancer, sterility, neurologic, graft versus host diseases and other complication. Also, hydroxyurea which is a new medicine and is taken orally has reduced the frequency of sickle cell crisis. Hydroxyurea raises fetal hemoglobin levels in sickle cell anemia patients. Fetal hemoglobin is free of the defective hemoglobin because oxygen before we are born. Droxia, which is a prescription form of hydroxyurea which is available in the market for adult patient (Mehanna, 2001)

Other current treatment includes current therapies to eliminate iron overload caused by the iron that has accumulated and also experimental treatment focusing on processes that eliminate red blood cells stop sickling in the first place. To the later, there are three basic modes of treatment. To begin with, there is the treatment which entails the stimulation of production of healthy fetal hemoglobin to stop the red blood cells from sickling. The fetal hemoglobin (HbF) is found in fetus or small infants. Later HbF is replaced by the hemoglobin that is present in adults, but some HbF can persist in someone. Lf the later happens; the HbF is able to prevent the sickling action of the red blood cells in infants that have the sickle cell disease. Adults who have the HbF generally have mild sickle cell disease. To this, there are drugs that stimulate the production of HbF. They include Hydroxyurea, cytidine Analogue, and bytyrates.

Next, there is the mode of blocking dehydration in the cells. This alleviates pain brought about by the sickle cell condition and dehydration by addition of fluids to the body. There are drugs that are used; they include opioids like methadone, oral morphine, codeine, and oxycodones. Anti-inflammatory drugs are drugs that reduce inflammation, epidural anesthesia which is effective to pain that is not responding to the usual therapies. (Mehanna, 2001)

Thirdly, the stem cell or the bone is transplanted from healthy donors so that they is the production of normal hemoglobin and not hemoglobin S. This is a proven sure way to treat sickle cell. When the bone marrow of a sickle cell patient is destroyed and transplanted with a healthy bone marrow from a genetically-matched donor, normal hemoglobin may be produced.

The therapy carried is very important in the treatment of Sickle cell. Therapy in some cases is done regularly to prevent life threatening complications of the disease. It helps improve weight and height in children. There is specific therapy done to specific events. For instance, episodic transfusion is done to alleviate acute chest syndrome, stroke and widespread infection. Chronic transfusion controls heart failure, pulmonary hypertension, and chronic kidney. Iron overload and chelation therapy is done to remove excess in the body.

One of the experiments carried out was done by Robert G. Lerner, M.D on a patient he called Rod. In his condition, he had signs relating to both sickle cell condition and sickle cell trait. At the age of 18 his small arteries were blocked. This led to exchange transfusion to be done. His red blood cells were replaced by normal others. This did not assist him much because he experiences pain and other complications relating to sickle cell condition. When the hydoxyurea drug assisted to treat his condition (Mehanna, 2001)

One of the prominent scientists is Allision Streetly, who was investigating how pain is handled by the patients who get treatment at home and those who get treatment form the hospital. In his work, he established that the patients who handle their pain at home device methods that will not lead them to going to hospital, they educate themselves about the disease and they assert themselves in dealing with their pain. On the other hand the patients that go to hospital exhibit varied characteristics that affect most of them. To these patients, they suffer from luck of being attended to, isolation by the public, luck of trust in the patients, and show a lot of control. On the other hand, form relationships with the nurses, and may tend to go to different hospitals for treatment.

Conclusion

To conclude, Sickle cell Anaemia can be treated and prevented if proper measures are administered. For instance, in fetus and the infants if the fetal hemoglobin is checked and controlled, the patient cannot get the disease.

Reference

Dunlop R and Bennett K (2006) Pain Management for Sickle Cell Disease Cochrane Database

Syst Rev.:CD003350

Mehanna A (2001) Sickle Cell Anaemia and Antisickling Agents, Then and Now. Current

Medicinal Chem

Wang W, (2004) Sickle Cell Anaemia and Other Sickling Syndrome In. Wintrobes Clinical

Haematology 11th ed. Philadelphia

 

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