Prof. Gabriel Izbicki, Director of the Pulmonology Institute, Shaare Zedek Medical Center.
(photo credit: Courtesy)
In recent years, IPF patients have received, for the first time, a therapeutic solution for their disease, a rare and deadly pulmonary fibrosis, and yet, many patients are unable to receive the treatment that can slow down the relapse of the disease.
Fibrotic pulmonary diseases are diseases characterized by scarred areas in the lungs that do not function properly and do not fulfill their role – gas exchange. These areas do not participate in the respiratory effort, they detract from the lungs' ability to carry oxygen into the blood vessels and remove carbon dioxide. One of the most serious fibrotic diseases is IPF, idiopathic pulmonary fibrosis, or “pulmonary fibrosis”, the cause of which is unknown in most cases.
The symptoms of the disease are highly common, which can make it difficult to diagnose. Usually they will be manifested as shortness of breath in exertion or a dry cough. One of the disease’s identifying signs, which is fine crackles, called velcro, which sounds a lot like velcro pulled apart, can only be detected through physical examinations. If the attending physician recognizes those crackles, accompanied by a complaint of cough or shortness of breath, he should refer the patient to undergo a lung X-ray. In the presence of abnormal findings, the patient should be referred to a thin section CT for a final diagnosis of the disease.
Sometimes, a sample/biopsy of the lung is also needed, and this is collected through a bronchoscopy. The diagnosis has to be made at a specialized site that consists of multidisciplinary teams such as pulmonologists, radiologists and surgeons, who consult each other regarding the diagnosis and treatment of the disease.
In the past, the disease used to be treated with Cortisone – an anti-inflammatory drug that is part of standard practice for other fibrotic pulmonary diseases as well. However, several years ago, an article was published which contended that not only was this treatment ineffective, it might also harm IPF patients. Treatment with Cortisone was discontinued and many patients remained without a solution. However, approximately three years ago, two new drugs appeared for the treatment of IPF patients, each working through a different mechanism. Although the new drugs cannot cure the patients and have not impact on the symptoms of the disease, such as shortness of breath and cough, they can slow down the relapse of the disease, so they are good news for the patient population. The side effects of the new drugs are predominantly mild, such as nausea, diarrhea and so on.
Both new drugs are included in the Health Basket in Israel, but they are restricted by several limitations of pulmonary functions, despite the fact that the drugs have been tested in studies and have proven to be effective in slowing down the relapse of the disease for different pulmonary functions, exceeding the limitations set in the Basket. Other countries around the world offer the treatments to patients in various settings without restrictions, but in Israel, some patients still remain without a solution.
Pulmonary rehabilitation is also not included in the Health Basket for these patients. This treatment can help IPF patients to cope with shortness of breath and reduced endurance in exertion. These symptoms make daily activities hard for patients, and require them to take multiple breaks for rest. Pulmonary rehabilitation is a treatment that takes place in large centers and is combined with physical therapy. It is part of the available treatment in many countries in Europe and in different parts of the world, and has proven to be highly effective for IPF patients, and that is why it is important to include it in the Health Basket for these patients.
IPF can behave differently in every patient, some will relapse rapidly while others will live with the disease for a long time without symptoms and relapse overnight, but without proper treatment, the chances of survival for IPF patients are poor and mortality rates are high. The new treatments can significantly prolong their lives, and therefore, one of our important tasks is to increase awareness to the disease both in the general population and among family practitioners, who can identify the symptoms and thereby enable earlier diagnosis. At the same time, we must do everything in our power to remove the existing limitations in the Health Basket, both on the drugs and on the pulmonary rehabilitation.Prof. Gabriel Izbicki is Director of the Pulmonology Institute at Shaare Zedek Medical Center in Jerusalem.
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