considered incurable and difficult to treat, Idiopathic Pulmonary Fibrosis (IPF) is classified as an intensive process of damage lung. The disease was the cause of death on Tuesday, March 3. Singer and songwriter Rita de Cássia, 50 years old, who was successful in Forró with the songs Meu Vaqueiro, Meu Peão and Saga de Um Vaqueiro. According to her producer, she was rushed to an intensive care unit (ICU) at a private hospital in Fortaleza, Ceará. as a result of worsening fibrosis, discovered about a year ago when he put his career on hold to begin treatment.
According to the Ministry of HealthIPF is defined as a specific chronic form of progressive fibrosing interstitial pneumonia of unknown origin causing pulmonary fibrosis. These scars cause the lung tissue to harden, making it difficult to breathe. The term idiopathic, which names the disease, is used when the disease has no known cause.
In general, the disease begins to manifest itself in patients in between 50 and 60 years. The average survival time from diagnosis is two to three years. Although medication can help slow the development of the disease, once idiopathic pulmonary fibrosis has developed, it is not possible to reverse the lung damage it has caused.
:quality(80):focal(5x5:5x5)/cloudfrontuseast1.images.arcpublishing.com/estadao/5I4HGA2IU5MI7JMM3Q5L6QPPFQ.jpg "Idiopathic Pulmonary Fibrosis: Understand the Disease That Killed Forró Singer Rita de Cássia at the Age of 50 3")
According to the Department of Health, it’s common for people to feel fatigue from small exertions, a feeling that usually gets worse over time. Photo: Darren Staples/Portal
What is idiopathic pulmonary fibrosis?
THE idiopathic pulmonary fibrosis It is considered an intense process of abnormal lung damage and tissue repair. “This leads to the formation of fibrotic and scarring areas that ultimately cause the lungs to retract, reducing air entry and therefore air exit. So we have a decrease in lung capacity. This also leads to a decrease in pulmonary gas exchange over time, thickening the exchange membrane and causing shortness of breath,” explains José Pereira Rodrigues, pulmonologist at BP Beneficência Portuguesa de São Paulo, Paul.
As the disease progresses, severe shortness of breath occurs and gas exchange can no longer be carried out sufficiently, so that oxygen supplementation becomes necessary. In these cases, the indication for lung transplantation is common. “However, it is important to remember that this is one of the most complex transplants that there is, with very high risks,” adds the pulmonologist.
“Various diseases, including Covid19can lead to fibrotic areas of the lungs, which are abnormally shaped lung scars, but if we talk about it idiopathic pulmonary fibrosis it’s a different situation. This is the deadliest fibrosis we have and the most difficult to treat,” observes the pulmonologist.
Still loud Ministry of Health, in most cases the development of the disease is slow and progressive, leading to severe respiratory failure. There are no studies describing the number of patients affected by the disease in Brazil.
When we talk about idiopathic pulmonary fibrosis, that is smoke is the main cause of the disease.
“This delays the diagnosis as many of the smokers tend to associate the symptoms of idiopathic pulmonary fibrosis with cigarettes, bronchitis or pulmonary emphysema without even going through a pulmonological evaluation or a more attentive family doctor asking for a CT scan and.” notes that the patient has other sounds in the lungs,” says the pulmonologist.
What other people are likely to have the disease?
“We also have papers that talk about pollution and genetic and hereditary syndromes that can lead to idiopathic pulmonary fibrosis, even in people who have never smoked, although this is the minority of cases,” Rodrigues assesses.
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Patients who had someone in their family with idiopathic pulmonary fibrosis can also develop the disease due to the hereditary problem.
What are the main symptoms?
One of the most common symptoms of idiopathic pulmonary fibrosis is shortness of breath. “The patient usually comes into the office with shortness of breath and fatigue, which occurs over the course of a few months. In the course of the disease, it is very common for a predominantly dry cough to appear.”
According to the Department of Health, it’s also common for people to feel fatigue from small exertions, a feeling that tends to get worse over time.
It is a disease with a high risk of death. The average survival time from diagnosis is two to three years.
How is the disease developing?
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It is an extremely heterogeneous disease. “There are people who develop quickly. But there are patients who take years to die from the disease,” adds the specialist.
At what age does the disease usually manifest itself?
The disease usually manifests itself in patients between 50 and 60 years. “Usually the patient has the flu and they have an tomography and we discover that they have some changes in the early stages of the fibrosing lung disease,” says Rodrigues.
How is the diagnosis made?
The main basis of the diagnosis is a surgical biopsy, which can be done by video.
“Although it is a less aggressive procedure, it also has its risks. Precisely for this reason, the biopsy is delayed. The clinical condition of the patient must also be taken into account. We can then take a family inventory, ask about lifestyle habits and find out how long the disease lasts,” explains the pulmonologist.
In more advanced cases, the chest CT can also show certain features that lead to the diagnosis without the need for a biopsy. “However, at this stage the patient is already very weak,” muses the pulmonologist from BP Beneficência Portuguesa de São Paulo
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What care should patients have?
Patients with lung damage are more susceptible to infections. Any time they have an infectious process, be it viral or bacterial, there can be an irreversible loss of lung capacity.
“In this way, we always focus on therapies to avoid exacerbations. We also invest in vaccines and medicines that maintain stable lung function. It is also recommended that the patient avoid contact with sick people,” he affirms.
Oral antifibrotics such as nintedanib and pirfenidone are used to try to slow the progression of the disease. Although the lungs cannot be regenerated, it is possible to slow down the progressive process of organ damage. All of them have side effects and medical monitoring is essential.
A continuous treatment with expensive and difficult to access drugs costs an average of R$ 20,000 per month. “Most of the time it is necessary to legalize the process so that the patient has free access to medicines, either through Unified Health System (SUS) or by the health insurance operator,” concludes Rodrigues.
According to the National Commission for the Incorporation of Technologies in the SUS, the Department of Health still does not have a clinical protocol and therapeutic guidelines (PCDT) for the treatment of IPF. Currently, the treatments available on the public network with antitussives, morphine, corticosteroid therapy and oxygen therapy are used to manage and control the symptoms of the disease. There is also the option of performing a lung transplant.
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