International Niemann–Pick Disease Alliance

The First Three Year Old Patient to Receive Enzyme Therapy for Niemann-Pick Type B

A constant smile and strong personality are just two traits of Isadora, a Brazilian girl who has became the first three year old in the world to receive enzyme therapy for Niemann-Pick type B disease. Upon hearing this news Isadora has received affectionate well wishes from not only close friends and family, but also thousands of people across various social media networks – something which gave her a real boost as she began receiving her first infusion at the Research Centre of Hospital de Clínicas, in Porto Alegre. 

Isadora has been kept in high spirits by the positivity passed on by her parents, Luiz Gustavo Rodrigues Prateleira and Betina Bender, as they relay to her each and every message. “She is playing with the team, research coordinator, nurse, and another child patient who is also undergoing the same treatment. As Isadora’s mother it is hard to put into words such momentous joy and excitement!”

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The research will be coordinated by Larissa Pozebon, and headed by Dr. Roberto Giuliani – people who the entire family have every confidence in: “she [Isadora] is very well, and so are we” commented Isadora’s father, who also sends huge thanks for the time, help, and solidarity of all of the people who have aided Isadora in getting to this stage – as without them there may have never been authorisation for the research to be carried out in Brazil.

Isadora’s aunt, Cláubia Bender, has led the campaign from its inception and has even travelled to England to learn more about the disease. She was there at the first procedure in Porto Alegre (pictured on the far left) to offer her support. and later commented on the occasion on the official ‘Niemann-Pick B – RS’ page: “Today we have the realisation of a dream, and with this, the certainty that everything is worthwhile if the soul is not small. It is through these dreams we can later make choices, and now we have chosen to make it happen rather than simply waiting.”

The treatment will take place over 68 weeks, and today (3rd January) is the first of ten infusions that will take place over the next 15 days. Isadora will remain at Hospital de Clínicas for 24 hours after the procedure. Due to the historic nature of the NP-B enzyme therapy, Isadora has been featured on the news numerous times – including the second print edition of the Vale Vision Journal.

On the 15th November three children from São Leopoldo, Isadora, Arthur, and Ygor, all of whom are battling with rare diseases, met together – a meeting which highlighted not only their own issues but also the struggle of parents to treat their children. May the victory of Isadora and her parents give strength to other parents, especially the parents of Arthur and Ygor who are still continuing their fight.

This article is a translation, originally published in Portuguese on visaodovale.com – you can read it here. 

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