Early diagnosis is made by the foot test, performed on newborns as part of the newborn screening program. According to the Hospital da Criança de Brasília, for every 1,200 births in the Federal District, at least one newborn is affected by the disease.
Intense pain, medication, hospitalizations and blood transfusions. This is the daily life of more than 2,000 people in the Federal District affected by sickle cell disease. In Brazil, the number of patients is 30,000, according to the Ministry of Health. The chronic, hereditary and genetic disease is characterized by a failure of the hemoglobin structure which, instead of having the shape of a disc, resembles a sickle or a waning moon, impairing the transport of oxygen by the organism.
This Sunday (19) is World Sickle Cell Awareness Day, established in 2008 by the United Nations (UN). The proposal is that knowledge about the disease be disseminated, in order to facilitate early diagnosis and treatment throughout the public network.
Early diagnosis is made by the foot test, performed on newborns as part of the newborn screening program. The child is then followed regularly at the Hospital da Criança de Brasília (HCB), with a specialized team, a special vaccination schedule and folic acid supplementation. The family receives support, advice and self-care techniques.
“It’s not just anemia. With the alteration of the vascularization of red blood cells, we understand that it is a systemic disease, which can harm all the organs of the body “Isis Magalhães, technical director of the Children’s Hospital of Brasilia
According to HCB data, for every 1,200 births in the Federal District, at least one newborn is affected by the disease and, currently, 741 children with sickle cell disease are treated in the unit. Of these, 87 are part of the regular transfusion program to prevent stroke, one of the threats of the disease.
Last Wednesday (15), the Fundação Hemocentro de Brasília (FHB) organized an event to celebrate the day of dissemination of knowledge and information on sickle cell disease, with the participation of patients and health professionals.
Pediatric hematologist Isis Magalhães, technical director of HCB, explains that sickle cell disease directly impacts the routine of patients, with pain attacks, progressive kidney failure and greater susceptibility to infections, in addition to the risk of neurodevelopmental changes. . “It’s not just anemia. With the alteration of the vascularization of red blood cells, we understand that it is a systemic disease, which can harm all the organs of the body”, explains the hematologist.
She stresses the importance of society as a whole understanding that sickle cell disease causes chronic pain and several other health problems. “It is a public health problem that must be known to all and taken care of by all, in the emergency services. This involvement of civil society is very important for the search for public policies that protect and facilitate the lives of patients,” she adds.
The Fundação Hemocentro de Brasília (FHB), as responsible for the management of the blood system, blood components and blood products of the DF, coordinates the policy of comprehensive care for patients with sickle cell disease. Technical training is encouraged for healthcare professionals and students in the region to guide patients with hemoglobinopathies. Multi-professional care at all levels of care is the responsibility of the DF Health Department.
The director-president of the Fundação Hemocentro de Brasília (FHB), Osnei Okumoto, explains that all blood donations undergo laboratory analysis to control the quality of the contribution. But, in the case of phenotyped blood, that transfused to sickle cell patients, caution is even greater.
“In addition to identifying that person’s blood type, we detect several other antigens to identify the best blood for sickle cells. This is an additional match so that these people do not develop resistance and control the disease. becomes more difficult,” says Osnei.
The official adds that the disease cannot be overlooked. “It is an extremely dangerous disease compared to neurological problems of all kinds. It is essential that we can see patients in a different way to provide a better quality of life,” he stresses.
Elvis Magalhães, 54, lived with a severe form of sickle cell disease for 38 years. There were hundreds of trips to and from the hospital, side effects, medications and unsuccessful treatments. Until he had the opportunity to be transplanted with his younger brother’s bone marrow and everything changed, being one of the first people in Brazil to be cured of the disease.
“There is hope for people with sickle cell disease and we are fighting for more people to have access to treatment”Elvis Magalhães, coordinator of the Brazilian Association of People with Sickle Cell Disease
“You know that commitment to illness that I had? Ended. Of course, I continue to take care of myself, but it’s very good to live more peacefully. I no longer had hope that anything would improve, but when the opportunity arose, I was very happy,” he says. Since 2015, more than 150 people with sickle cell disease have received bone marrow transplants in Brazil, six of whom reside in the Federal District.
Currently, Elvis is coordinator of the Brasiliense Association of People with Sickle Cell Disease (Abradfal) and defends, tooth and nail, public policies that help the lives of patients directly affected by the disease. “There is hope for people with sickle cell disease and we are fighting for more people to have access to treatment. The progress we have made in recent years is undeniable, but there is still a long way to go,” he adds.
Civil servant Emerson Silva, 48, was unaware of the disease until his first child, Victor Hugo, was diagnosed at the age of one. Today – the father of another boy who also has sickle cell disease, Rafael, 9 – he understands the symptoms, care and treatment of the disease, in addition to participating in awareness events and conferences.
“My children’s lives have been saved on several occasions, during hospitalizations and transfusions. Humanized treatment and knowledge about the disease saves”Emerson Silva, civil servant
The discovery of the firstborn’s illness came unexpectedly: during a visit the boy paid to his grandmother at work, a local doctor warned that the child had sickle cell disease, after recognizing signs such as swollen hands and feet, and that he needed to be examined urgently. . Even disbelieving the visual diagnosis, transmitted by the child’s grandmother, Emerson took the boy to the doctor and had an unpleasant surprise.
“I knew nothing about the disease, nothing at all. It was only after a lot of suffering that I stopped and studied, because I needed to help my son”, recalls the civil servant. Victor Hugo had to undergo a removal of the spleen in his childhood and underwent further surgeries throughout his life, in addition to hundreds of hospitalizations to control pain attacks.
But, even with the difficulty imposed by illness, he managed to maintain a pleasant life, studying and working. However, on December 21, 2021, while spending the holidays with his grandmother in Rio de Janeiro, Victor Hugo died. He was 23 years old.
“It was the only crisis where I was not there to help, to show the doctors what my son needed,” laments Emerson, who also stresses the importance of medical knowledge and the credibility of the patient’s pain .
Now who lives with the pain is Emerson, who is gathering strength to continue in the fight for disease awareness. “When everyone knows what it is, how they treat it, everything will be better,” he says.
The second son, Rafael, has a milder form of sickle cell disease and receives care, most of the time, at home. When he was born, it was hoped that he would be compatible with the eldest, for the bone marrow transplant, but unfortunately it was not possible. “My children’s lives have been saved on several occasions, during hospitalizations and transfusions. Humanized treatment and knowledge of the disease saves, ”he concludes.