Sickle cell anemia is a transgenerational genetic disorder

Investigation: Randa Gerges
The prevalence of sickle cell disease is among the highest in the region, and the rate of sickle cell disease in the UAE is 1.1% of the population, and it is one of the leading genetic disorders in the world. The infant and child mortality rate is high in the developing world, and 50-90 percent of children born with sickle cell anemia do not reach their fifth birthday. It is expected that there is a 30% growth in the number of people with sickle cell disease by 2050, and in the coming lines a group of experts and specialists will talk about this subject in detail.

Remember dr. Asma Al Olama, head of the Emirates Division of Hematology, said that the most targeted groups for screening for sickle cell anemia are young men and women who are about to get married. As the best way to confront the problem is prevention and not having infected children, and this is what the UAE has implemented by imposing a mandatory pre-marital examination law for more than 20 years.

Scientists indicate that sickle cell anemia affects the health of children in the future if it is inherited from one or both parents; Therefore, the UAE resorted to imposing a pre-marital examination and it became mandatory, and if both parties insisted on marriage, the country provided what is known as IVF to give birth to a child free of disease IVF PGD and diagnosis during pregnancy PND, and despite the high risk, awareness of the disease is still very low ; Therefore, we seek to introduce the prevalence of sickle cell anemia, its symptoms, screening and treatment options available through awareness campaigns, to help patients with this disorder lead a happier life.

Scientists confirm that encouraging premarital examination, diagnosing children before and immediately after birth, and disseminating educational programs about sickle cell anemia for individuals and health care providers, are among the most important early preventive steps that benefit in eliminating this disease, and awareness campaigns also aim to raise awareness about sickle cell disease. Sickle-cell anemia, correctly identifying it and highlighting its prevalence in the United Arab Emirates; It is classified as the second most common beta-globin gene defect in the UAE, after Thalassemia.

sickle cell

Dr. explains. Moza Al-Marshudi, Consultant Internal Medicine and Benign Hematology, said that the sickle cell gene occurs with variable frequency in the Arab Middle East countries. In the past two years, genetic diseases, such as sickle cell disease, have constituted a major cause of chronic health problems, ill health and deaths, and thus have become a major burden on the health care system. Red blood cells, and cause them to stick inside the small blood vessels, and the balls turn from their normal and healthy circular position to unable to move and take the shape of a sickle or similar to a crescent, and thus reduce the flow of blood and oxygen to the organs of the body, the most important of which are the brain, heart, kidneys and joints, which affects the condition health of the patient.

Al-Marshudi pointed out that the symptoms of sickle cell anemia include repeated episodes of pain in different parts of the body, especially in the limbs, joints and bones, chronic infections, malnutrition and slow growth in infants, and the birth of an affected child is usually predicted when one of the parents appears in premarital examinations, or By routine newborn blood test.

Effects

Dr. points out. Mahmoud Marashi, a consultant hematologist, pointed out that sickle cell anemia is a disease that affects all body systems and damages organs. And the feet, and some small damage that accumulates in the tissues of brain cells, and develops with age and leads to a delay in intellectual activities, and microvascular obstruction may cause a stroke.

He adds: Chest syndrome is one of the most serious complications of sickle cell anemia, and it is one of the acute conditions that occur as a result of the lack of blood flow to the lungs, as well as kidney damage that requires repeated dialysis, inflammation of the ligaments and joints, most notably the pelvis, and the patient may need surgical intervention to change the joint. injured.

Dr. Marashi points out that hydroxyurea is one of the most important treatments used to treat sickle cell anemia, as it reduces severe pain and prevents patients from recurring crises and attacks, but there are some cases that do not respond to this drug and are treated by intravenous injection, and scientists are working On the study of gene therapy, it should be noted the need to protect the patient with seasonal vaccines against infectious diseases.

disease management

Sickle cell anemia is a disease that accompanies a person from birth to old age. Therefore, public health experts recommend some methods that contribute to reducing the development of symptoms and complications, the severity of pain and seizures, and the possibility of living with the disease, which are: –

Obligation to conduct a comprehensive medical examination before marriage; It helps reduce the transmission of sickle cell disease between generations.

Psychological comfort and avoidance of daily stress, tension and anxiety.

Exercising regularly reduces the chance of experiencing pain attacks.

Continuing to drink sufficient amounts of water, and avoid dehydration so as not to make the situation worse. Avoid exposure to extreme heat or cold.

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