Africa-Press – Angola. Sickle cell anemia is one of the congenital diseases, whose sharp growth among the population, should place it at the level of other Public Health pathologies, for the doctor Jeremias Agostinho.
This Sunday, the specialist said that in Angola, 20 percent of the population is affected by sickle cell anemia, a congenital disease, with no cure so far and without state subsidies.
The doctor, a specialist in Public Health, reported that, in the country, half of sickling patients die before reaching the age of majority. To mitigate the situation, he said, several Care Centers for Anemic Patients (CADA) and the Pediatric Hematological Institute Victória do Espírito Santo were created in Luanda.
Patients with this pathology, he said, tend to have problems from childhood, although some children have few symptoms and lead a normal life most of the time. “The main signs and symptoms of the disease are painful episodes, called sickle cell crises, which can be very severe and last up to a week, under the increased risk of contracting infections,” he explained.
In anemia, he explained, red blood cells cannot carry enough oxygen through the body, which causes tiredness and shortness of breath for patients. “Some people also experience other problems, such as stunted growth, strokes and lung difficulties.”
Sickle formation, he said, is caused by a gene that affects the development of red blood cells and is very common when both parents are carriers. “In these cases, there is a possibility that one in four children will be born with sickle cell disease”, said the doctor.
“Sometimes, the child’s parents do not have the disease and are only carriers of the sickle cell trait. However, despite this, the disease affects the child and, almost always, is only detected during pregnancy or shortly after birth”, mentioned the specialist.
People with sickling, he said, need lifelong treatment. This is usually done by different healthcare professionals and at a specialized cell center. “It is also essential that people with the disease take care of their own health, with self-care measures”.
Personal Cares
Regular fluid intake is one of the essential self-care for people with the disease, as the doctor explained. The other, he said, is to keep warm to avoid episodes of pain. “And when you’re in pain, you should take Paracetamol or Ibuprofen. But there are cases where it’s best to go to a hospital for the proper treatment, with stronger painkillers.”
The doctor also considers recommended, for some patients, the daily use of antibiotics and some vaccines to reduce the chances of infections. “Hydroxyurea is a drug that helps to reduce the symptoms and crises of sickling,” he said.
“In certain situations, emergency blood transfusions are necessary, if the patient also develops severe anemia. The only cure for sickle cell disease is stem cell or bone marrow transplantation, procedures not yet available in the country and in other countries, more advanced in Medicine, is not a very common practice, due to the risks involved”, he said.
Revolutionary Drug
Hydroxyurea is a drug considered by many to be the drug that changed the history of sickle cell anemia, since its use was discovered in the 1980s. However, it is not a cure for the disease, but a way to improve the quality of life and reduce mortality by reducing painful bone crises, or the need for transfusion, as well as avoiding serious and potentially fatal complications of the disease, such as stroke (Cardio-Vascular Accident) and Acute Thoracic Syndrome. According to studies done so far, hydroxyurea reduces mortality from this disease by 40 percent or more.
The Impact Of The Disease On The Quality Of Life Of Patients
Valdemar Mateus, a specialist in Clinical Pathology and Laboratory Medicine, considers sickle cell disease to be a Public Health problem, due to the impact of the disease on the patient’s quality of life, to the point of causing social repercussions , family members and even the costs of the National Health System.
The epidemiological burden of the disease, he said, should also be assessed by the high prevalence rate of sickle cell disease in southern Africa and in Angola, in particular. “The prevalence of sickle cell anemia in the country is around 1.5 percent, that is, it affects half a million people, not including asymptomatic carriers”.
The specialist added that sickling, as it is a disease transmitted through genes, is almost impossible to cure by conventional means. “In addition to genetic counseling for carriers, the effort has been to reduce mortality and increase patients’ quality of life by reducing seizures,” he said.
Efforts to improve patients’ quality of life, he added, have had an effect worldwide over the last few decades, as more data on the disease continues to be discovered. “40 years ago it was considered a pediatric disease, as patients did not reach adolescence, but today it is not uncommon to find sickling patients in their 40s or even 60s,” she said.
Studies Done In The Country
The routine diagnosis of sickle cell anemia is made by hemoglobin electrophoresis and can be done in the country in some laboratories of tertiary health institutions. More specific studies of a scientific research nature involving Genetics and Molecular Biology techniques were carried out a few years ago in the Genetics Area of the Faculty of Medicine of the Agostinho Neto University, a project currently suspended due to technical constraints and conditions imposed by the Covid-19 pandemic. 19.
In the portfolio, he revealed, there are also some already advanced projects, including sequencing techniques, resulting from partnerships with international research centers, which may, in the future, open the door to a partnership in the diagnosis of blood diseases, including anemia. sickle cell.
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