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La enfermedad rara de Paula: el sueño se convierte en pesadilla

sindrom3

enero 09, 2021

El norte de Castilla

La niña, de cuatro años y vinculada a Hontalbilla, donde viven sus abuelos, sufre el síndrome de Ondine, que afecta a la respiración.

El doctor Pedro Gómez de Quero, responsable clínico de la Unidad de Diagnóstico Avanzado de Enfermedades Raras Pediátricas de Castilla y León, de reciente creación, calcula que alrededor de un 7% de la población puede tener una enfermedad rara. Cada año se diagnostican unos 2.000 casos en niños. Como el de Paula, una niña de cuatro años que sufre el síndrome de Ondine, una enfermedad rara que puede provocar la muerte cuando los afectados se duermen porque dejan de respirar. Sus abuelos viven en la vecina localidad de Hontalbilla y, tras conocer la historia de Paula, el Ayuntamiento decidió que la iniciativa ‘Cuéllar solidario’, que se celebrará el próximo sábado, 5 de mayo, recaudará fondos para apoyar la lucha contra este síndrome.

Paula y su madre, Silvana Teixeira, estuvieron presentes en la conferencia impartida por Pedro Gómez de Quero, previa a la jornada solidaria. El doctor explicó al público qué es una enfermedad rara, por qué son tan difíciles de diagnosticar y facilitó algunos datos sobre la nueva unidad, que se está empezando a montar. Gómez de Quero diferenció entre enfermedades raras –con una prevalencia menor de un caso entre 2.000 recién nacidos vivos– y ultra-raras, como es el caso del síndrome de Ondine, que afecta a menos de uno de cada 10.000 recién nacidos vivos. Los afectados tienen riesgo de muerte o invalidez crónica.

En Europa hay más de 36 millones de personas con enfermedades raras y en España, alrededor de 2,5 millones. «Pero cuando uno piensa en una enfermedad rara, piensa en una mucho más rara, que son aquellas que no están diagnosticadas, que es lo que crea angustia, sobre todo a los padres. El no tener un diagnóstico es un gran problema», destacó el doctor, que apuntó que con la puesta en marcha de la nueva Unidad de Diagnóstico Avanzado se pueda agilizar el tiempo de diagnóstico, que en ocasiones se prolonga más de cinco o diez años, «y sobre todo se puede dar una respuesta a esos padres sobre si pueden tener más hijos o si pueden ofrecer algún tratamiento a sus pequeños», apuntó. En algunos casos, las enfermedades raras no logran diagnosticarse nunca. Durante su intervención recalcó que las enfermedades raras no afectan únicamente a las personas diagnosticadas, sino también a las familias, amigos, cuidadores y a toda la sociedad. Respecto a la nueva unidad, Gómez de Quero mostró su esperanza en que en el plazo de unos dos meses esté implantada en toda la Comunidad, dado que se ha mantenido una reunión con representantes de todos los hospitales de Castilla y León, tanto de laboratorio como de pediatría, y ya existe un responsable en cada uno de ellos. Apuntó que el camino ha sido lento por los problemas informáticos para permitir la conectividad, pero ya se han desarrollado los primeros proyectos pilotos en Zamora, Ávila, Soria y en las localidades burgalesas de Aranda de Duero y Miranda de Ebro. La previsión es que en las próximas semanas se extienda al resto de los hospitales de la región, de forma que en dos meses puedan estar todos conectados.

La unidad de referencia tiene su sede física Salamanca, también la de diagnóstico genético, y existen tres niveles de asistencia, por lo que se pretenden optimizar todos los recursos que hay en los hospitales de Castilla y León. Por ejemplo, un niño de Soria no tendría que ir directamente a Salamanca, sino que si hubiera que derivarle, su primer filtro sería Burgos. Si fuera necesario derivarle para valoración (el tercer nivel), ya tendría que acudir a la unidad de Salamanca, explicó Pedro Gómez de Quero, quien destacó que esta unidad «es una apuesta muy importante de la Gerencia Regional de Salud». En este sentido, agradeció «el compromiso» del consejero de Sanidad, Antonio María Sáez, y del gerente regional, Rafael López, al tiempo que mostró su confianza en que «se sigan destinando fondos para mantenerla en los próximos años».

Avances

El doctor Gómez de Quero conoce a Paula desde que nació. «Fue un diagnóstico nuestro y la seguimos nosotros en Salamanca», explicó. El de Ondine es un síndrome muy poco frecuente, incurable y potencialmente mortal. Afecta directamente a la respiración de la persona. Los afectados ‘olvidan’ respirar o su respiración es superficial. Dependiendo de su grado de afectación, esto puede ocurrir mientras duermen o 24 horas al día, señaló el facultativo, quien destacó que uno de los principales avances, «el fundamental», respecto a esta enfermedad es la mejora de los respiradores. «Hace años casi todos los niños tenían un respirador a través de una cánula, una traqueostomía, que es lo que tiene Paula ahora. Pero ahora, con la progresión de la ventilación mecánica, a los niños y adolescentes se les quita la traqueostomía y duermen, como muchos adultos, con una mascarilla con un respirador». La mejora de estos «es el gran avance ahora», subrayó.

Ahora la investigación pone la lupa en la terapia génica, puesto que es una enfermedad genética del sistema nervioso autónomo que controla las funciones involuntarias del organismo. «Está identificado el gen y hay que trabajar en ello», aseguró el responsable de la Unidad regional de Enfermedades Raras. Hay esperanza, pero Pedro Gómez de Quero lamentó la escasez de recursos para luchar contra estas enfermedades, «que no interesan a la industria farmacéutica, porque son muy pocos niños y muy pocos casos, y no ganan dinero con ello».

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