Sickle cell disease: pain crises impair patients’ quality of life  Drauzio Varella

Sickle cell disease: pain crises impair patients’ quality of life Drauzio Varella

The disease causes episodes of acute pain that can lead to a long stay in the hospital.

THE sickle cell disease It is an inherited condition that causes changes in red blood cells and is considered to be the most common inherited disorder in Brazil.

In this way, red blood cells, which have a slightly rounded appearance, are deformed and take on a different shape, similar to the shape of a sickle, hence the name “sickle”.

Due to the change in structure, blood cells are “stuck” and cannot deliver oxygen to organs and tissues of the body. This “small” deformity causes serious damage to the patient, such as anemia, jaundice (yellow eyes), fatigue, in addition to the so-called. health sector), episodes of acute pain that is very debilitating, impairing the quality of life of patients.

Despite the high prevalence, 47% of the population is not aware of this condition, according to a survey conducted by Ibope. There are about 3,500 new cases a year. The frequency is higher in blacks, because the disease is of African origin. Many people have the feature of sickle cells, which means that they do not develop the disease, but can pass the gene on to future generations. In order for a child to be born with the disease, both the father and the mother must have a gene. If only one parent has a gene, a child can be born with that trait, but not the disease.

Diagnosis and multidisciplinary monitoring

Today, sickle cell disease is diagnosed via Foot testmade soon after birth. The patient is monitored by a hematology team at a specialized reference center. Since it is a chronic disease, nutritional and psychological supervision is necessary. Physiotherapy can also be used in treatment.

But, in addition, monitoring by other specialists, such as a neurologist, cardiologist and nephrologist, for example, may still be necessary, as sickle cell disease can cause a number of complications, such as pulmonary hypertension, a higher risk of cerebrovascular infarction (stroke)changes in the kidneys that can lead to renal failure, retinopathy (retinal damage) and ulcers on the lower limbs. Due to the higher risk of infections, it is still important to follow the dental examination, because many infections start in the mouth.

As it is a chronic disease, follow-up is lifelong. “Each country has its own reference service. Often the patient lives far from the referral center and we recommend joint monitoring in the health unit near the house “, explains Dr. Rita de Cássia, hematologist. “From an early age, what changes the history of these patients is the prevention of infectious diseases, so they follow the issue of vaccines in the basic health unit.”

According to doctors, the life expectancy of these patients was 55 years earlier. “But that has changed with the advent of antibiotics, better self-care, because patients are more aware of the disease, in addition to the drugs we have been taking in recent years,” he explains.

Late detection of the disease can have consequences

Social worker Sheila Pereira, 41, was diagnosed only when she was 7 years old, when her mother realized that her difficulty walking and swollen joints could be a sign of something serious. At that time, no tests had yet been done to diagnose the condition in newborns. The lack of knowledge about the disease at the time further aggravated the situation. “The first diagnosis was rheumatism, but the drugs used for rheumatism did me harm.” From there, genetic mapping was done and it was discovered that Sheila’s father and younger sister, at the time of 2 years, also had the disease, while her mother had only the sickle cell trait – which is a genetic change, not a disease.

The difference in these two cases is that sickle cell disease causes a genetic change in hemoglobin, which changes from type A to type S. People with this trait do not develop sickle cell disease and therefore have no symptoms and do not need treatment. see:

  • People who do not have sickle cell disease or characteristics: normal hemoglobin (AA);
  • People with sickle cell disease: Hemoglobin altered in homozygosity or heterozygosity with other variants (SS, SC, SD, SE, S / β0-thalassemia or S / β + -thalassemia);
  • People who have only sickle cell characteristics: Partially altered hemoglobin (AS).

So, both parents must have a gene in order for a child to be born with the disease. If only one parent carries the gene, the child can only inherit that trait. In Sheila’s case, both parents had a gene: a father with the disease and a mother with the trait.

With frequent pain, the consequences in Sheila’s life were numerous, from difficulty playing like other children, and not attending physical education classes, she repeated for two years at school due to long and periodic hospitalizations, which led to the interruption of studies. in adolescence. Sheila’s younger sister died in 2013, at the age of 29, from kidney failure due to sickle cell disease.

The life expectancy of Brazilians is 77 years, but according to a mortality study cited at the beginning of the report, sickle cell disease may be linked to a reduction of up to 37 years of life in Brazil. According to the study, the main causes of death among those suffering from this disease are sepsis (generalized infection) and respiratory failure.

Sheila and her sister had a number of problems that resulted from improper treatment. But today, according to Dr. Rita, sickle cell disease is technically well known. “What is happening is that measuring pain is a very private matter. So sometimes that patient is the one who goes to the same ambulance many times. In other words, everyone already knows him. Sometimes, the team may reduce this understanding of the patient’s symptoms, may not give due importance, may not appreciate the pain. “Our mission is to remove the pain and not make judgments,” he said.

Attacks of pain make it impossible

Sheila says crises start with feelings of discomfort, fatigue and weakness. Then there is a stab of pain (sometimes caused by certain movements) which gradually increases, until it becomes so intense that the person is unable to perform any task. “Even today, there are those pains that I can endure, I can do things slowly, treat them at home. But there are those pains that grow to the point that they become unbearable, to cry. Then you have to go to the hospital, because only with medication in a vein can you feel better “. In some cases, a transfusion may be needed due to a lack of oxygen in the blood.

Prejudice and ignorance of the intensity of the crisis can have a very negative impact on patients’ lives. The pain is very debilitating and causes a person to be absent from work for many days. According to the International Sickle Cell Survey (SWAY), conducted with more than 2,000 patients in 16 countries, patients claim to have missed more than one day of work per week (8.3 hours in 7 days) due to the disease. That is why it is common for a company to have the image of someone who is lazy or relaxed, for example. Even friends and family may find it difficult to understand the situation. Sometimes they think that the patient is making a “soft body”.

The importance of mental health care

With so many difficulties and challenges that are not limited to a person’s health, but affect all spheres of life, it is extremely important to have admission, a support network and psychological care. “Illness brings a few prejudices: you feel incompetent, you always think you’re different from others, you always hear negative comments, you always live with this fear of death,” says Sheila.

“It’s always a new beginning. Things are not waiting for you, the system is not waiting for you. The boss will not wait for that employee to return. The school will not realize that this student was absent because he had limitations. Today, with all the progress, we always tell people, ‘you can’. We can, all within our limits. But for that you have to work on the psychological “, he reports.

Sheila currently chairs the Associação Pró-Falcemics (APROFe) – a non-profit institution whose mission is to expand access to treatments for diseases affecting the black population founded by people with sickle cells and which seeks to help patients with this condition. Sheila says she is always very involved with patients. “I’m there today and I learned to accept myself and live better with the disease,” he says. “My mother did not have this reception, orientation. And today we are able to make it possible for many families, not to be alone. “

This mental health care must be extended to family members of the patient and caregivers, who are most often mothers. Many of them also get sick, and that is the stress and anxiety that the situation causes in their lives. Therefore, it is necessary for these people to have admission and psychological support, in addition to the opportunity to dedicate themselves to their activities, and not to live only for the treatment of their children’s illnesses. Sheila’s mother eventually fell ill and died at the age of 36 after a stroke. “There was no time, no looking at her, no worrying about myself. I see that in mothers today, because they stop taking care of themselves in order to take care of the person. It is necessary for the guardian to have this psychological help “, explains the social worker.

for dr. Rita, the issue of taking care of yourself is very important. “It simply came to our notice then. Remember medications, remember vaccination prevention, cancer prevention, remember hydration (take 2 to 3 liters of fluid a day). You have to take care of your skin, watch your mouth. “Taking care of yourself increases your self-esteem, and by increasing your self-esteem, you will always want to be well, love yourself and feel loved,” he concludes.

See also: What are the most common diseases in the black population?

#eusinto campaign

In order to draw the attention of society and health decision makers to the challenges and prejudices faced by people with sickle cells, to clarify the population about the disease and seek interventions that actually increase and improve the lives of these patients. , APROFe, in partnership with ABHH – ​​the Brazilian Association for Hematology, Chemotherapy and Cell Therapy – and with the support of Collabore com o Futuro and other patient associations, has developed the #EuSinto campaign.

Created to show the population that sickle cell disease is devastating but has no visible signs and is therefore neglected by society, the #EuSinto campaign aims to work on public policies, expanding access to medicines, thus improving the treatment scenario and the quality of life of sickle cell patients. cells.

In addition to facing obstacles to inequality and prejudice, sickle cell patients see that opportunities and hopes for access to innovation in new health technologies are neglected compared to other diseases.

“Given this scenario and with the goal of increasing the visibility of the disease, we want to draw the public’s attention to the cause and make the health of sickle cell patients a priority of the Government to improve, control and implement public policy, coverage. systems and providing care and treatment, Sheila says.

Content developed in partnership with NOVARTIS

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