Sickle cell anemia (Hbs) is the most common form of abnormal hemoglobins. It is so named because the erythrocytes of this patients adopt a sickle shaped (Crescent like).³

Fig -2: Diagrammatic representation of hemoglobin with 2a (alpha) and 2B (beta) shade – heme³

Synonyms of sickle cell anemia are –

. Hemoglobin SS Diseases

. Hbs.

. Sickling disorder due to Hemoglobin S.

. Hemoglobin S diseases.

. Sickle cell disorders

. SCD.

The structure of hemoglobin (as described all ready) contains two alpha and two beta globins chain. In case of sickle of anemia the hemoglobin (Hbs) has two normal alpha globins and two abnormal (mutant) beta globins chain. This is due to difference in a single amino acid. In Hbs, glutamate at sixth position of beta chain is replaced by valine.

(Glu B6 à Val).

Fig -3 - Sickle cell anemia is due to a change (missense - mutation) in the single nucleotide (thymine - adenine) of beta globin gene.

This error causes the formation of altered codon (GUG in place of GAG) which leads to incorporation of valine instead of glutamate at the sixth position in beta chain.³

Sickle cell anemia affects millions of people worldwide. It’s most common people whose families come from Africa, South or Central America (Specially panama) Caribbean islands, Mediterranean countries (Such as Turkey, Greece and Italy) INDIA and Saudi Arabia. In united State Sickle cell anemia affects about 70000 peoples. It mainly affects African, American with the condition occurring in about in every 500 African American births. Hispanic American are also affected, the condition occurs in 1 out of every 1000 to 1400 Hispanic American births. About 2 millions American have sickle cell trait. About in 12 African, American has sickle cell trait.

Signs and symptoms of Sickle cell Anemia – the signs and symptoms of sickle cell anemia are different in each person. Some people have mild symptoms others have very severe symptoms and are often hospitalized for treatment. Although sickle cell anemia is present at birth many infants don’t show any sign until after 4 months of age.

The most sign and symptoms are linked to anemia and pain. Sudden episodes of pain throughout the body are common symptoms of sickle cell anemia and are often referred to as sickle cell crises. Occurs when the Red blood cells sickle and stick together in clumps. The clumps blocked the flow of blood through the small blood vessels in limbs and organs. Sickle crises can cause acute and chronic pain the pain usually (in acute) from hours to a few days’ chronic pains for weeks to months. Many factors can contribute to a sickle cell crises often more than one factor is involved and the exact cause can’t be identified. The most common site affected by sickle cell crises are bones, lungs, abdomen and joints. The blocked blood flow can cause pain and organ damage.

When sickle cell blocked the small blood vessels in hand or feet pain and swelling along with fever can occur swelling usually occurs on the back of the hand and feet and moves in to fingers and toes this may be first sign of sickle cell anemia in infants.

Splenic (Sequestration) Crisis - The spleen is an organ in the abdomen that filters out abnormal red blood cells and helps fight infection. Sometimes, the spleen traps many cells that should be in the bloodstream and it grows large. This causes anemia. Blood transfusions may be needed until the body can make more cells and recover. If the spleen becomes too clogged with sickle cells, it can’t work normally. It begins to shrink and stop working.

Infections - Both children and adults with sickle cell anemia have a hard time fighting infections. Sickle cell anemia can damage the spleen. Infants and young children with a damaged spleen are more likely to get infections that can kill them within hours or days. Pneumonia is the most common cause of death in young children who have sickle cell anemia. Meningitis, influenza, and hepatitis are other infections that are common in people with sickle cell anemia.

Acute Chest Syndrome - Acute chest syndrome is a life-threatening condition linked to sickle cell anemia. It's similar to pneumonia and is caused by an infection or by sickle cells trapped in the lungs. People with this condition usually have chest pain, fever, and an abnormal chest x ray. Over time, lung damage may lead to pulmonary arterial hypertension

Delayed Growth and Puberty in Children - Children with sickle cell anemia often grow more slowly and reach puberty later than other children. A shortage of red blood cells (anemia) causes the slow growth rate. Adults with sickle cell anemia often are slender or small in size.

Stroke - Sickle-shaped red blood cells may stick to the walls of the tiny blood vessels in the brain. This can cause a stroke. This type of stroke occurs mainly in children. The stroke can cause learning disabilities or more severe problems.

Eye Problems -The retina, a thin layer of tissue at the back of the eye, takes the images you see and sends them to your brain. When the retina doesn’t get enough blood, it can weaken. A weak retina can cause serious problems, including blindness.

Priapism - Males with sickle cell anemia may have painful and unwanted erections called Priapism (PRI-a-pizm). This happens because the sickle cells stop blood flow out of an erect penis. Over time, Priapism can damage the penis and lead to impotence.

Gallstones - When red blood cells die, they release their hemoglobin, which the body breaks down into a compound called bilirubin. When there is too much bilirubin in the body, stones can form in the gallbladder. Gallstones can cause steady pain that lasts for 30 minutes or more in the upper right side of the belly, under the right shoulder, or between the shoulder blades. The pain may happen after eating fatty meals. People with gallstones may have nausea, vomiting, fever, sweating, chills, clay-colored stool, or jaundice (yellowish color of the skin or whites of the eyes).

Ulcers on the Legs - Sickle cell ulcers (sores) usually begin as small, raised, crusted sores on the lower third of the leg. Leg sores occur more often in males than in females and usually appear between the ages of 10 and 50. The cause of leg ulcers is not clear. The number of ulcers can vary from one to many. Some heal rapidly, but others persist for years or come back after healing.

Pulmonary Arterial Hypertension (High Blood Pressure) - Damage to the small blood vessels in the lungs makes it hard for the heart to pump blood through the lungs. This causes blood pressure in the lungs to increase. This condition is called pulmonary arterial hypertension. Excessive shortness of breath is an important symptom linked to this problem.

Multiple Organ Failure - Multiple organ failure is rare, but serious. It happens when a person has a sickle cell crisis that causes two out of three major organs (lungs, liver, or kidney) to fail. Symptoms linked to this complication are a fever and changes in mental status such as sudden tiredness and loss of interest in your surroundings ¹

Premature death – Homozygous individual of sickle cell anemia die before they rich adulthood (< 20years).³

Infection – Salmonella infection is found more in this disease.⁶

Let us see the block blood vessels due to Sickle shaped Red Blood Cells.

Fig – 4 – Difference between Normal RBCs and Sickle Cell RBCs

Figure A shows normal red blood cells flowing freely in a blood vessel. The inset image shows a cross-section of a normal red blood cell with normal hemoglobin.

Figure B shows abnormal, sickled red blood cells clumping and blocking the blood flow in a blood vessel. The inset image shows a cross-section of a sickled red blood cell with abnormal strands of hemoglobin¹

Diagnosis of Scikle cell anemia - Early diagnosis of sickle cell anemia is very important

So that children who have the condition can get proper treatment. In the United States, 44 States, the District of Columbia, Puerto Rico, and the U.S. Virgin Islands now test all newborns for sickle cell anemia. In the other six States, you can request a sickle cell test. The test uses blood from the same blood samples used for other routine newborn screening tests. It can show whether the newborn infant has sickle cell anemia or sickle cell trait. If the first test shows some sickle hemoglobin, a second blood test is done to confirm the diagnosis. The second blood test looks at how hemoglobin moves in an electric field, a process called electrophoresis (ee-LEK-tro-for-EE-sis). Sickle hemoglobin moves differently than normal hemoglobin. Electrophoresis is usually used to diagnose older children and adults. It’s also possible to identify sickle cell anemia before birth. This is done using a sample of amniotic fluid or tissue taken from the placenta. (Amniotic fluid is the fluid in the sac surrounding a growing embryo. The placenta is the organ that attaches the umbilical cord to the mother’s womb.) This test can be done as early as the first few months of pregnancy. It identifies the sickle gene, rather than the hemoglobin it makes.¹

1. Sickle test – this is a simple microscopic examination of blood smear prepared by adding reducing agents such as sodium dithionite sickle erythrocytes can be detected under the microscope.

2. Electrophoresis – When subjected to electrophoresis in alkaline medium (pH – 8.6) sickle cell hemoglobin moves slowly towards anode than does adult hemoglobin. The slow mobility of Hbs is due to less negative charge, caused by the absence of glutamate residues that carry negative charge. In case of sickle cell trait, the fast moving HbA and slow moving Hbs are observed. The electrophoresis of hemoglobin obtained from lysed erythrocytes can be routinely used for the diagnosis of sickle cell anemia and sickle cell trait.

Fig – 5 - Electrophoresis of hemoglobin’s at pH 8.6

HbA – Normal adult Hemoglobin; HbS – Sickle cell Hemoglobin.³

Treatment – There is no specific therapy avoid fatigue, exposure to cold, stress infection, fever, dehydration. Folic acid is recommended to counteract the secondary folate deficiency that develops due to rapid red cells turn over. Various methods have been suggested form time to time for the painful crises. Crises are due sludging and vascular occlusion with or without myleo suppressive effects.

It is most unusual for the new born to get crises. This is explained on the basis of high fetal hemoglobin level and low level of HbS. Carbonic anhydrous inhibitors, alkalization with sodium bicarbonate administration conversion of a portion of Hb to oxy Hb. By oxygen administration or by carbon monoxide to carboxy hemoglobin or mat hemoglobin. Has been no benefit. Though this form do not Sickle. Magnesium salts therapeutic defibrination, anti coagulant, expansions of blood volume with low molecular weight. Dextrans have been suggested but are still under investigation. The best is to provide supportive and symptomatic therapy. Splenectomy is of moderate benefit in those subjects where there is a excessive blood destruction. Red Cell survival increases after Splenectomy and the frequency of blood transfusion is decreased. Heme turnover is increased during urea therapy due to increased hemolysis of red cell. Which are prevented from aggregating in the microcirculation.

Recent suggestion is administration of oral or intravenous injections of 10 % sodium citrate, 6G in 400ml. Of water every two hours for 2 days. For 24 hour followed by the same every 2 hours for 2 days and then every 6 hours. Codeine is helpful. Sodium citrate may be given in I.V. 200ml. Of M/6 sodium lactate in 3 hours. Hyper transfusion is also used. Corticosteroids have been beneficial, phenothiazine and oral contraceptive are of no value. Analgesic and antipyretic drugs must be used for comfort of the patient. Intravenous distilled water may interfere with sickling. Antibiotics should be used if infection is present. Blood transfusion is necessary for aplastic crises and in pregnancy. Packed Red Cells are preferable. Transfusion decreases viscosity and is helpful. Repeated transfusion would lead to hemosiderosis and iron over load. According to modified muryamma hypothesis for the molecular mechanism of sickling. Which implicates intertertrameric hydrophobic bond on the basis of this hypothesis intravenous urea in sugar solution is used as an effective chemical agent for desickling. This solution reverses and blocks sickling without hemolysis. A high blood nitrogen gradient of 150 to 200mg. Per 100ml./ is achieved rapidly and maintained until the vascular occlusion is reversed. The relief of pain signals this and the urea is discontinued. The maintenance of hydration is critically important and is achieved by the adertically importance and is achieved by the administration of ringers lactate solution by a central; venous catheter to prevent the complication occasionally caused by 30% urea solution prepared by taking 0.05 to .10M. urea (300 to 600mg./100ml.) in sugar solution in a peripheral vein.

Side effects – sedation that is desirable and diuresis. Oral prophylactic urea reduces the number of crises. 160gm of urea per day divided into four doses are given individualization is necessary. Bon should be maintaining at 40 to 50mg/100ml.

Since cyanate (NCO) is present in urea, it can react with N-terminal residues of protein to form carbimilate derivative. This reaction is essentially irreversible.

Low concentration of cyclamate inhibit Sickling the amount bound cyanate per moil is in range of 0.1 to 1per mole of hemoglobin this not only prevents sickling but also blocks the gelling of hemoglobin no side effect were observed.

Partial exchange perfusion is used for prophylaxis and treatment two units of buffu coat free packed sales in two units of whole blood every 6 to 8 weeks for 1.5 to 6 years. This maintenance from 15 to 40% normal circulating RBCs. There is a melioration of crises in 24 hours. And complete relief in 48 hours.

Frequency of crises is not affected the hospital stay is reduce.

Ref -: Brady j.et al.symptomatic crises of sickle cell anemia treated by limited change transfusion.

AM. Int.med.72:327-330,1970²

New Treatments - Today, research on sickle cell anemia is looking at bone marrow transplants, gene therapy, and new medicines. The hope is that these studies will provide better treatments for sickle cell anemia. Researchers also are looking for a way to predict the severity of the condition.

Bone Marrow Transplant - Bone marrow transplant can be a very effective treatment for sickle cell anemia, but because of its risks, only some patients can or should have this procedure. The bone marrow transplant procedure is risky and could result in serious side effects and even death. It’s usually used only for younger patients with severe sickle cell anemia, but the decision is made on a case-by-case basis. Bone marrow used for a transplant must come from a closely matched donor, usually a close family member, who doesn’t have sickle cell anemia.

Gene Therapy - Gene therapy is being studied as a possible treatment for sickle cell anemia. Researchers are looking to see whether a normal gene can be planted in the bone marrow of a person with sickle cell anemia, and thus cause the body to produce normal red blood cells. Researchers also are studying the possibility of treatment to

“Turn off” the sickle cell gene or “turn on” a gene that makes red blood cells behave normally.

New Medicines -New medicines being studied are:

· Butyric acid. This is a food additive that may increase normal hemoglobin in the blood.

· Clotrimazole. This is used now to treat fungus infections. This medicine helps prevent the loss of water from a red blood cell and can keep the cell from turning into a sickle cell.

· Nitric oxide. This may make sickle cells less sticky and keep blood vessels open. People with sickle cell anemia have low levels of nitric acid in their blood. ¹

Now the question is arises how can sickle cell anemia is prevented?

As we know sickle cell anemia is an inherited condition. Children who inherit the genes for sickle cell anemia can’t prevent developing the condition, although they can take steps to reduce complications. People who are in groups that are at high risk for sickle cell anemia and who are planning to have children may want to consider genetic counseling beforehand. A counselor can help you understand your risk of having a child with the condition and help explain the choices that are open to you. You can get information about genetic counseling from health departments, neighborhood health centers, medical centers, and clinics that care for people with sickle cell anemia.

A procedure called pre-implantation genetic diagnosis can improve the chance that two people with sickle cell trait will have a child with normal hemoglobin. Eggs from the mother and sperm from the father are mixed together in a laboratory dish. The eggs that become fertilized are checked for sickle cell anemia. Fertilized eggs that don’t have sickle cell genes are then implanted in the mother’s womb. This is a complex procedure done only in a few places, and it doesn’t always work. ¹

Relation with other diseases: –

01. Person suffering from sickle cell anemia show an increased resistance to malaria.⁶

Because the life span of the sickle cell is less than the time needed for the malaria parasite to mature inside the cells.⁷

In sickle cell anemia, a lower-than-normal number of red blood cells occurs because sickle cells don’t last very long. Sickle cells die faster than normal red blood cells, usually after only about 10 to 20 days. The bone marrow can’t make new red blood cells fast enough to replace the dying ones. The result is anemia.¹

The above all information are of sickle cell anemia and its treatment was also as in allopathic medicinal system but Homeopathy Medicinal system claim that according to there treating manner if we remove the symptoms we remove the disease Hahnemann steps into say for the first time in all history remove the effects and you remove the disease the cause of effect “ Cessat effectus Cessat causa.”⁵

Now as in the care effected by the removal of the whole of the perceptible signs and symptoms of the disease. The internal alternation of the vital force to which the disease is due consequently the whole of the disease is at the same time removed. It follows that the physician has only to remove the whole of the symptoms in order at same to abrogate and annihilate the internal change that is to say he morbid derangement of the vital force consequently the totality of the disease the disease itself.⁴

As same line we can see according to allopathic medicinal system for treatment of sickle cell anemia – “the best is to provide supportive and symptomatic therapy.”²

In allopathic medicinal system generally physician provide folic acid to sickle cell anemic patient -

Folic acid is recommended to that develops due to rapid cell turn over.² Many patients are prescribed a vitamin called folic acid (folate) to help prevent some of the complications of sickle cell anemia.¹

In homoeopathic medicinal system the drugs which are provided to patient in anemic condition are –

. Picricum Acidum.

. Calcarea Phosphorica.

. Ferrum Iodatum.

. Ferrum Mettallicum.

. Ferrum Phosphoricum.

. Vanadium. Etc.⁹

These drugs are prescribe only on the bases of symptoms. It is not to each patient gives same drug. If the symptoms are different the drug will also different.

Dr.K.D.Sharma. in INDIAN HOMEO REMEDIES manufacture SECALGO. This is available in market. This drug is use in anemia, weakness and in sickling. If any patient who belongs the symptoms to be subject to take doses of secalgo.

Secalgo basically consist of two components – Ferrum mettalicum. And Vanadium.

Ferrum Metallicum – Best adapted to young weakly persons, anemic and chlorotic, with pseudo plethora, who flush easily.⁹

Vanadium – Which increases amount of hemoglobin.⁹

These both are metals.

According to homoeopathic medicinal system = by use of such medicines as are capable of producing in the healthy individual an opposite state to that of the case o be cured.⁴

Means those crude drugs produce the effects in healthy voluntary that are use to treat that particular disease in it dilution form.

E.g. Hahnemann, took four drachms of china (cinchona officinalis, Peruvian bark)⁹ twice a day. He had paroxysms of chill and fever. In his practice as a physician he had seen similar paroxysms of chill and fever. He had cured them with china.⁵

Conclusion and Result :–

Sickle cell anemia is serious condition and not have any permanently treatment Effective treatments are available to help relieve the symptoms and complications of sickle cell anemia, but in most cases there’s no cure¹ but according to reference. Remove the effects and you remove the disease.⁵ the homoeopathy medicinal system is also a subject to treat patient those are suffering from sickle cell anemia.

ACKNOWLEDGMENT:

Author is giving thanks to Dr. K.D. Sharma. For giving Library support to complete the research. A Special thanks to Mrs. Jyoti Kalyandas Sharma. [Chairman of Indian Homeo Remedies] for giving Commercial support to complete the Research.

REFERENCE:

01. http://www.nhlbi.nih.gov/health/dci/Diseases/Sca/SCA_WhatIs.html

02. Text Book of medicine edited by Rustom jal vakil Second edition. Association of physician INDIA. Pg. no. 679 to 681.

03. Biochemistry by Satyanarayana revised reprint. 2004 aruna sen books and Allied (P) Ltd. 8/1 Chintamoni das lane Kolkata 700009 pg.no. 199 to 210

04. Hahnemann’s Organon commentary by B.K.Sarkar reprint 1993, M.Bhattacharyya and Co. (P). Ltd. Homoeopathic chemists and pharmacists 73,. Netaji subhas road culcutta – 700001. pg. no. 122, 98.

05. Organon of Medicine by Samuel Hahnemann, Sixth edition. Hahnemaan’s own written revision. B.jain Publishers Pvt. Ltd. 1921, street no.10^th chuna mandi, paharganj, New Delhi – 110055 pg.no. 8,9.

06. Fundamentals of Biochemistry by Dr.AC.Deb Reprint 1999. Eight editions. New Central Book. Agency (P) Ltd. 8/1 Chintamoni das lane, kolkata 700009. pg. no. 102 to 109.

07. Ross and Wilson Anatomy and Physiology in Health and Illnes, Ninth Edition 2001, chruchill Livingstone Edinburgh London, New York. Philadelphia st. Louis Sydney and Toronto 2001. pg. no. 71.

08. Human Physiology Vol. I by Chandi Charan Chhatarjee. Special reprint edition 20^th April 1994, medical Allied Agency 82/1 Mahatma Gandhi road Calcutta- 9 -700009. pg. no. 153 to 154.

09. Pocket Manual of Homoeopathic Materia medica by willian Boericke, ninth edition. B.Jain Publishers Pvt. Ltd. New Delhi – 110055. Pg. no. 516,152,282,283,286,665.

10. http://en.wikipedia.org/wiki/Hemoglobin

Received on 25.09.2011

Accepted on 20.10.2011

Research J. Pharmacology and Pharmacodynamics. 3(6): Nov.-Dec., 2011, 334-339