Sickle Cell (सिकल सेल) is a group of blood disorders typically inherited from a person’s parents. The most common type is known as Sickle Cell Anemia (सिकल सेल एनीमिया). It results in an abnormality in the oxygen-carrying protein hemoglobin found in red blood cells. This leads to a rigid, sickle-like shape under certain circumstances. Problems in sickle cell disease typically begin around 5 to 6 months of age. A number of health problems may develop, such as attacks of pain, anemia, swelling in the hands and feet, bacterial infections and stroke. Long-term pain may develop as people get older. Read in detail about sickle cell, sickle cell disease, sickle cell anemia in detail and know the symptoms, diagnosis, management, bone marrow transplant, blood transfusion, sickle cell crisis, Ayurveda treatment, etc as Siddha Spirituality of Swami Hardas Life System considers that our readers should know about.
Sickle cell Symptoms (सिकल सेल के लक्षण)
Signs of sickle cell disease usually begin in early childhood. The severity of symptoms can vary from person to person. Sickle cell disease may lead to various acute and chronic complications, several of which have a high mortality rate.
Sickle cell crisis (सिकल सेल संकट)
Sickle cell disease results in anemia and crises that could be of many types including the:
- Vaso-occlusive crisis
- Aplastic crisis
- Sequestration crisis
- Hemolytic crisis
Most episodes of sickle cell crises last between five and seven days. “Although infection, dehydration, and acidosis (all of which favor sickling) can act as triggers, in most instances, no predisposing cause is identified.”
Vaso-occlusive crisis (वासो-अविवेकी संकट)
The vaso-occlusive crisis is caused by sickle-shaped red blood cells that obstruct capillaries and restrict blood flow to an organ resulting in ischemia, pain, necrosis, and often organ damage. The frequency, severity, and duration of these crises vary considerably.
Splenic sequestration crisis (स्प्लेनिक क्रमबद्धता संकट)
Because of its narrow vessels and function in clearing defective red blood cells, the spleen is frequently affected. It is usually infarcted before the end of childhood in individuals suffering from sickle cell anemia. This spleen damage increases the risk of infection from encapsulated organisms. Preventive antibiotics and vaccinations are recommended for those lacking proper spleen function.
Splenic sequestration crises are acute, painful enlargements of the spleen, caused by intrasplenic trapping of red cells and resulting in a precipitous fall in hemoglobin levels with the potential for hypovolemic shock.
If not treated, patients may die within 1–2 hours due to circulatory failure. Management is supportive, sometimes with blood transfusion. These crises are transient, they continue for 3–4 hours and may last for one day.
Acute chest syndrome (तीव्र छाती सिंड्रोम)
Acute chest syndrome (ACS) is defined by at least two of the following signs or symptoms:
- Chest pain
- Pulmonary infiltrates or focal abnormality
- Respiratory symptoms, or hypoxemia
It is the second-most common complication and it accounts for about 25% of deaths in patients with sickle cell disease, the majority of cases present with vaso-occlusive crises then they develop ACS. Nevertheless, about 80% of patients have vaso-occlusive crises during ACS.
Aplastic crisis (अविकासी संकट)
Aplastic crises are acute worsenings of the patient’s baseline anemia, producing the pale appearance, fast heart rate, and fatigue. This crisis is normally triggered by parvovirus B19, which directly affects the production of red blood cells by invading the red cell precursors and multiplying in and destroying them.
Parvovirus infection almost completely prevents red blood cell production for two to three days. In normal individuals, this is of little consequence, but the shortened red cell life of sickle cell disease patients results in an abrupt, life-threatening situation.
Reticulocyte counts drop dramatically during the disease, and the rapid turnover of red cells leads to the drop in hemoglobin. This crisis takes 4 days to one week to disappear.
Hemolytic crisis (हेमोलिटिक संकट)
The red blood cells break down at a faster rate. This is particularly common in patients with coexistent G6PD deficiency. Management is supportive, sometimes with blood transfusions.
Sickle cell Diagnosis (सिकल सेल का निदान)
In HbS, the complete blood count reveals hemoglobin levels in the range of 6–8 g/dl with a high reticulocyte count as the bone marrow compensates for the destruction of sickled cells by producing more red blood cells. In other forms of sickle cell disease, Hb levels tend to be higher. A blood film may show features of hyposplenism.
Sickling of the red blood cells, on a blood film, can be induced by the addition of sodium metabisulfite. A mixture of hemoglobin S (HbS) in a reducing solution gives a turbid appearance, whereas normal Hb gives a clear solution. Therefore, a urinalysis to detect an occult urinary tract infection, and chest X-ray to look for occult pneumonia should be routinely performed.
Sickle cell Treatment (सिकल सेल उपचार)
Treatment involves a number of measures. L-glutamine use was supported by the FDA starting at the age of 5 as it decreases complications.
Folic acid and penicillin (फोलिक एसिड और पेनिसिलिन)
From birth to five years of age, penicillin daily, due to the immature immune system that makes them more prone to early childhood illnesses is recommended. Dietary supplementation of folic acid had been previously recommended by the WHO.
Malaria prevention (मलेरिया की रोकथाम)
The protective effect of sickle cell trait does not apply to people with sickle cell disease; in fact, they are more vulnerable to malaria, since the most common cause of painful crises in malarial countries is infection with malaria. It has therefore been recommended that people with sickle cell disease living in malarial countries should receive lifelong medication for prevention.
Vaso-occlusive crisis (वासो-अविवेकी संकट)
Most people with sickle cell disease have intensely painful episodes called vaso-occlusive crises. However, the frequency, severity, and duration of these crises vary tremendously.
Acute chest crisis (तीव्र छाती का संकट)
Management is similar to the vaso-occlusive crisis, with the addition of antibiotics, oxygen supplementation for hypoxia, and close observation. Should the pulmonary infiltrate worsen or the oxygen requirements increase, simple blood transfusion or exchange transfusion is indicated.
Hydroxyurea for Sickle cell (सिकल सेल के लिए हाइड्रॉक्स्यूरिया)
The first approved drug for the causative treatment of sickle cell anemia, hydroxyurea, was shown to decrease the number and severity of attacks in a study in 1995 and shown to possibly increase survival time in a study in 2003.
Blood transfusion for Sickle cell (सिकल सेल के लिए रक्त आधान)
To prevent complications by decreasing the number of red blood cells (RBC) that can sickle by adding normal red blood cells. In those who have sustained a prior stroke event, it also reduces the risk of recurrent stroke and additional silent strokes.
Bone marrow transplant for Sickle cell (सिकल सेल के लिए अस्थि मज्जा प्रत्यारोपण)
Transplants of bone marrow have proven effective in children. The only known cure for Sickle Cell Disease is a bone marrow transplant.
However, bone marrow transplants are difficult to obtain because of the specific HLA typing necessary. Ideally, a close relative would donate the bone marrow necessary for transplantation.
Avascular necrosis (अविकसित परिगलन)
When treating avascular necrosis of the bone in people with sickle cell disease, the aim of treatment is to reduce or stop the pain and maintain joint mobility. Current treatment options are to rest the joint, physical therapy, pain relief medicine, joint replacement surgery, or bone grafting.
High quality randomized controlled trials are needed to assess the most effective treatment option and determine if a combination of physical therapy and surgery are more effective than physical therapy alone.
Ayurveda for Sickle cell (सिकल सेल के लिए आयुर्वेद)
Yogaraj and Laxadi Guguly are two Ayurvedic medicine have been tried clinically on 12 patients for the maintenance of hemoglobin level and prevention of repeated blood transfusion in sickle cell disorders, the duration of treatment was 3 months. The patients were examined clinically every month to note the effect of reducing fever, weakness, enlargement of spleen and liver which are statistically significant. To read more…
Sickle cell & Free Siddha Energy Remedies (सिकल सेल और नि:शुल्क सिद्ध ऊर्जा उपचार)
Siddha energy remedies to be applied from day one of the sickle cells confirmed, which include:
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