Sickle Cell Disease | Cannabis Wiki

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Sickle Cell Disease

In sickle-cell disease, abnormal forms of hemoglobin (oxygen carrying protein) in red blood cells cause the to become or sickle (crescent) shaped. This makes the cells inflexible, and thus they are more likely to block small blood vessels. The cells are also fragile and do not survive for as long as they should. Although sickle-cell disease is inherited and present at birth, symptoms do not usually occur until after four months of age.

Long-Term Symptoms

Most people with sickle-cell disease suffer some degree of chronic anemia, which result in pale skin, fatigue and shortness of breath. Other long-term symptoms may include:

  • Jaundice (yellowing of the skin and eyes)-this may develop because red blood cells break down too quickly.
  • Skin ulcers, particularly on the legs-these arise due to poor oxygen supply (the sickle cells are unable to carry adequate oxygen).
  • Enlargement and deterioration of the spleen-this can compromise the immune system and increase vulnerability to infection, especially pneumococcal and blood-cell-damaging viral infections.
  • Delayed growth and late onset of puberty.
  • Gallstones, caused by high concentrations of red blood cell breakdown products.
  • Impaired kidney function and an enlarged liver and heart.
  • Narrowing of blood vessels and thus increased risk of stroke.
  • Deterioration of retina-can lead to blindness.

Hand and Foot Syndrome

Hand and foot syndrome is common in children with sickle cell disease. The blockage of small blood vessels causes painful swelling of the hands and feet, due to death of areas of bone. This may be the first sign of sickle cell disease in infants.

Crisis Episodes

Some sickle-cell are present at all times, but under certain conditions many more cells are affected. Infection, dehydration or decreased levels of oxygen in the blood can trigger a sickle-cell crisis, in which the following symptoms occur:

  • Pain, particularly in the abdomen or long bones
  • Chest pain
  • Convulsions
  • Fever
  • Priapism (a prolonged and painful erection).

Crisis tend to occur in 70 percent of cases and can last for hours or several days. The frequency of crises varies-sufferers can have 15 or more episodes a year or only one every few years.

Causes

Sickle-cell disease is caused by a defective form of hemoglobin, called hemoglobin S. Hemoglobin S proteins have a tendency to link up into rod-like chains when they are deprived of oxygen, warping red blood cells into a sickle-shape.

When the cells become sickle-shaped, they lose their flexibility and cannot pass through narrow blood vessels, and have a shortened lifespan of 10-20 days as opposed to 120 days for a normal red blood cell.

Pattern of Inheritance

The gene for hemoglobin S is recessive; therefore a child must inherit two copies-one from each parent. A person who has only one copy of the gene is a carrier, and said to have sickle-cell trait. Carriers rarely exhibit any symptoms of the disease, but their children could also be carriers. If two carriers have a child, then there is a 25 percent chance that the child will have sickle-cell trait.

Incidence

The gene for hemoglobin S evolved as a defense against forms of malaria in Africa, and spread through the population of sub-Saharan Africa and parts of the Middle East and Mediterranean. In the West, the disease mainly occurs in people of African descent, although people of Middle Eastern or Mediterranean extraction are also affected.

Sickle-Cell Crisis

Episodes of sickle-cell crisis are extremely painful, and usually require hospital admission. Common treatment methods involve strong painkillers (often opioids and lead to addiction, tolerance, and increased pain), dehydration with intravenous fluids, and oxygen therapy. Antibiotics are given if the crisis has been triggered by an infection.

In animal studies, cannabis has been found to mitigate neurogenic inflammation and hyperalgesia (increased sensitivity to pain) in mice with sickle-cell disease. The study, which was conducted at the University of Minnesota, showed that synthetic cannabinoids were effective in reducing pain; potentially protected organs from hypoxia or reperfusion injury, oxidative stress, and inflammation’ and possibly slowed the progression of the disease.

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