Friday, 11 September 2015

Sickle Cell Disease and its treatment options

Sickle Cell Disease and its treatment 


sickle cell disease and its treatment 


The term sickle cell disease (SCD) describes a group of inherited red blood cell disorders. People with SCD have abnormal hemoglobin, called hemoglobin S or sickle hemoglobin, in their red blood cells.

Hemoglobin is a protein in red blood cells that carries oxygen throughout the body.

“Inherited” means that the disease is passed by genes from parents to their children. SCD is not contagious. A person cannot catch it, like a cold or infection, from someone else.

People who have SCD inherit two abnormal hemoglobin genes, one from each parent. In all forms of SCD, at least one of the two abnormal genes causes a person’s body to make hemoglobin S. When a person has two hemoglobin S genes, Hemoglobin SS, the disease is called sickle cell anemia. This is the most common and often most severe kind of SCD.

Hemoglobin SC disease and hemoglobin Sβ thalassemia (thal-uh-SEE-me-uh) are two other common forms of SCD.

Some Forms of Sickle Cell Disease

    Hemoglobin SS
    Hemoglobin SC
    Hemoglobin Sβ0 thalassemia
    Hemoglobin Sβ+ thalassemia
    Hemoglobin SD
    Hemoglobin SE

Overview

Cells in tissues need a steady supply of oxygen to work well. Normally, hemoglobin in red blood cells takes up oxygen in the lungs and carries it to all the tissues of the body.

Red blood cells that contain normal hemoglobin are disc shaped (like a doughnut without a hole). This shape allows the cells to be flexible so that they can move through large and small blood vessels to deliver oxygen.

Sickle hemoglobin is not like normal hemoglobin. It can form stiff rods within the red cell, changing it into a crescent, or sickle shape.

Sickle-shaped cells are not flexible and can stick to vessel walls, causing a blockage that slows or stops the flow of blood. When this happens, oxygen can’t reach nearby tissues.

The lack of tissue oxygen can cause attacks of sudden, severe pain, called pain crises. These pain attacks can occur without warning, and a person often needs to go to the hospital for effective treatment.

Most children with SCD are pain free between painful crises, but adolescents and adults may also suffer with chronic ongoing pain.

The red cell sickling and poor oxygen delivery can also cause organ damage. Over a lifetime, SCD can harm a person’s spleen, brain, eyes, lungs, liver, heart, kidneys, penis, joints, bones, or skin.

Sickle cells can’t change shape easily, so they tend to burst apart or hemolyze. Normal red blood cells live about 90 to 120 days, but sickle cells last only 10 to 20 days.

The body is always making new red blood cells to replace the old cells; however, in SCD the body may have trouble keeping up with how fast the cells are being destroyed. Because of this, the number of red blood cells is usually lower than normal. This condition, called anemia, can make a person have less energy.


Sickle Cell Disease and its treatment 


Home Treatment

Home treatment for sickle cell disease includes steps to control pain and prevent complications of the disease. If you don't already have a home treatment plan, ask your doctor to help you make one. Use this plan whenever symptoms are present. Your plan may include tips for:

    Managing pain.
    Home treatment for prolonged erection of the penis (priapism).
    Staying healthy.
    Coping with stress and worry.

Managing a child's special needs

You can help your child cope with special needs in school by:

    Making arrangements with teachers or a tutor to help your child keep pace with classmates when illness causes absences from school.
    Explaining to teachers that children with sickle cell disease may need to use the bathroom more often than other kids. They also need more water than the other students. Not drinking enough water can raise the chance of a sickle cell crisis.
    Educating teachers and other school employees about the signs and symptoms of sickle cell disease that need urgent medical care. Written instructions will help school personnel know what to do and who to call in an emergency.

Children with sickle cell disease can usually exercise and play normally if they:

    Drink plenty of fluids before, during, and after exercise. Lack of fluids (dehydration) can cause cells to sickle.
    Get regular rest breaks during vigorous exercise.
    Stay warm. Exposure to cold air, wind, and water can trigger a sickle cell crisis. Dress children in warm layers of clothing for cold-weather activities. Your child should avoid swimming and playing in cold water.

Folic acid supplements are often a necessary part of the diet for people with sickle cell disease, particularly if you aren't eating enough folate-rich leafy vegetables (such as spinach).

Medications

Medicines that treat sickle cell disease include hydroxyurea and various pain medicines. Some of these medicines require a prescription. Others are available over the counter. Pain medicine may work best when combined with pain management skills, such as distraction; guided imagery; deep breathing; relaxation; and positive, encouraging self-talk.

Pain treatment for sickle cell disease pain varies depending on how bad the pain is and how long the pain lasts. Medicines that treat the pain include over-the-counter pain relievers (such as ibuprofen) and prescription opioids (such as codeine). Opiate pain medicines are used under careful medical supervision.


Surgery

Some sickle cell complications are treated with surgery. Surgery options include:

    Removal of the spleen (splenectomy), to prevent the trapping of too many red blood cells in the spleen (splenic sequestration).
    Removal of the gallbladder (cholecystectomy), to prevent problems caused by gallstones.
    Draining fluid from the penis in cases of severe priapism.
    Hip replacement, if the tissue in the hip breaks down and dies because it doesn't get enough blood (osteonecrosis).

Other Treatment

Blood transfusions may be used for sickle cell disease. They are the treatment of choice to prevent strokes and treat other aspects of this disease. They can reduce the risk of some complications and improve symptoms of severe anemia.

Stem cell transplants can cure sickle cell disease. But they are not common.

Herbal Medicine: There are herbal medicines which has been successful in management and cure of Sickle cell. Inbox us for details.


Natural Remedies For Sickle Cell

Get support for sickle cell anemia by focusing on your unique nutritional needs. According to research or other evidence, the following self-care steps may help you manage this kind of chronic anemia:

What You Need To Know:

• Fight back with fish oil
Reduce the frequency of severe pain episodes by taking a daily fish oil supplement providing 33 mg of EPA and 23 mg of DHA for every 2.2 pounds of body weight

• Think zinc
Under the supervision of a doctor, take a daily supplement of 100 mg of zinc, plus 2 mg of copper, to help prevent cell damage and speed healing of leg ulcers associated with sickle cell anemia.


• Fill up on fluids
Drink plenty of water and other fluids to maintain good circulation

• Take a test
Have your blood homocysteine levels checked to find out if daily folic acid supplements of 1 to 4 mg are right for you, or to discover if you have a vitamin B12 deficiency that requires treatment


Sources : Web Md
Blackdoctors
NIH US.

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