Advances in pulmonary hypertension allow that it no longer be a Terminal disease.

Presents the first guide for parents in all the world

-the prognosis for a child with pulmonary hypertension is complicated, but there are new treatments

– symptoms are confused with mild disease

Madrid, April 2010.- advances in pulmonary hypertension have led to it cease to be in all cases a terminal illness, according to Dr. Mª Jesús del Cerro, President of the Group of pulmonary hypertension of the Spanish society of Cardiology pediatric and congenital heart disease. This expert has participated in the first guide to support parents of children with pulmonary hypertension in the world, according to Irene Delgado President of the Spanish Association of pulmonary hypertension, which has published the Guide.

Without treatment, the survival of pulmonary hypertension of idiopathic or unknown cause is 10 months, but advances in treatments and multidisciplinary assistance have improved the prognosis. Until 1993 it lacked effective treatments for this disease, so the only possibility was the lung transplant, whose 5-year survival is around 40-50% ” in the Pediatric ” drew Hill Dr. Now already available for specific drugs that manage to improve the quality of life, and delay the need for transplantation, or in some cases, prevent it. Fortunately, research in this field is currently very active, both in terms of the search for new drugs, and the mechanisms that produce disease ”. The treatment of children with pulmonary hypertension must be conducted in specialized units in pediatric pulmonary hypertension with multidisciplinary teams, formed by cardiologists, pulmonologists, intensivistas, geneticists, radiologists, pediatricians, psychologists, etc.

Spanish adult patients with pulmonary hypertension registry includes more than 1,200 people, but it ignores the number of children who may be affected in our country ”, recognizes. Are currently working on a record on hypertension pulmonary Pediatric (REHIPED) to evaluate how many children with this disease there is in our country, which will help us to improve their attendance ”, says the doctor of the Hill. This disease is characterized by an excessive rise in pressure in the pulmonary arteries, which hinders the bloodstream to the lungs and just hurting in serious way to the heart, which has to fight against the increase of pressure that can lead to heart failure and the failure of the right ventricle. The mechanisms by which arrives to develop hypertension pulmonary not always are well known; It can be idiopathic (of no known cause), hereditary, or in connection with congenital heart diseases, respiratory disorders, autoimmune diseases, diseases of the liver, including ”, adds.

Subtle symptoms

The symptoms may be subtle and nonspecific, such as shortness of breath with minimal effort, fatigue, chest pain, dizziness and fainting. So confused at times with other pathologies, such as asthma or pneumonia, so it is often diagnosed late. Two-dimensional echocardiography with doppler is the most useful image if there is suspicion of pulmonary hypertension, but the definitive diagnosis is done through cardiac catheterization.

As includes the Guide, in which participated the psychologists Cristina Solis and Aldana Zavanella, the time of the diagnosis in this chronic disease implies a period which is extremely painful, because life will change in a resounding way. It is a saying goodbye to a previous situation, in which pulmonary arterial hypertension did not exist, giving way to a new reality ”. For this reason, in mourning, it is hard to accept it. Del Cerro Dr. explains that children complain very little, and sometimes, when the symptoms are still little obvious or nonspecific, parents find it difficult to understand the severity of the disease; When the child receives treatment and see how it improves their quality of life, is when better understand the disease ”.

The guide presents tips on how to deal with the various stages of diagnosis and development of pulmonary hypertension from the psychological point of view. It includes recommendations to talk to children about their illness and also with his brothers. The family will be involved in the anguish and stress by the disease. More even when the patient is a child. For all, this is an intolerable reality and that we are trying to escape ”. Denial is usually pass to the rationalization and then to rabies. Begin to appear feelings of anger, wrath, envy and resentment. Emerge all the questions that have to do with the whys, mixed many times, with feelings of guilt (e.g. habré did I something?)”, stands out. The other stages are pain and finally acceptance, which is the only way to spend full support to the child sick, concludes.