Until recently, the diagnosis of spinal muscular atrophy (SMA), which affects the youngest patients, sounded like a verdict. Is it possible to talk about it in a completely different way and educate at the same time? It can - but only if Wolf talks about it.
The organisers of the nationwide educational campaign, under the auspices of the PTND, on spinal muscular atrophy are the SMA Foundation and the Polsat Foundation. It was launched in August - the month of SMA awareness. The campaign is based on the book "Wolf talks about SMA", written by Jacopo Casiraghi a psychologist from the Italian Centro Clinico "Nemo" in Milan.
SMA once sounded like a sentence. Nowhere in the world was there access to pharmacotherapy dedicated to treating this genetic, progressive disease that took the lives of 90 per cent of children before they were two years old. When parents received the diagnosis, the ground parted beneath their feet. Today, the reality is very different, because in 2016, the world's first innovative therapy for the treatment of SMA was registered - nusinersen! A new drug that offers the possibility to stop the disease and hope for a better future for those affected by SMA.
At the time the invention of the drug was being worked on, a well-known Italian psychologist - Jacopo Casiraghi, worked on his own form of therapy for SMA patients and their families. The results of his fifteen years of work are unique and therapeutic fairy tales collected in the book 'Wolf tells the story of SMA', helping patients and their families come to terms with the SMA that has invaded their lives and that they will have to face from the moment of diagnosis. The book has proved to be bestseller in many countries, and currently an animated film is being made in Italy, based on the fairy tales. Thanks to the efforts of the SMA Foundation and with the great support of the Polsat Foundation and the commitment of artists and people struggling with the disease, it will also be possible to listen to these beautiful stories, filled with emotion and written on the basis of true stories, in Poland.
Italian gentleness in approach to seriously ill patient captivates
Maria Koźmińska-Brygoła from the SMA Foundation, who waited for the Italian fairy tales to be transferred to Polish soil, mother of Antek with SMA 1, a psychologist, draws attention to the situational context in which SMA patients lived a few years ago. - Some of the fairy tales were written at a time when the diagnosis was a verdict and the prospect of life for tiny children with severe SMA was limited to a few months, sometimes a few years. Type 1 SMA very quickly took away children's motor functions, breathing and brought death, she explains.
She adds: when I came across Jacopo Casiraghi's 'Fairy Tales' in an Italian centre, I was captivated by the fact that Italians, to these most seriously ill children and their carers, try to offer quality of life, also on a psychological level.
- Such an item has not been available in the world so far, so we, as the SMA Foundation, thought it was worth having it on the Polish market. You have to understand how hard it is for us parents to talk to our children about the disease, treatments and sometimes about the impending passing away. It is insanely difficult. That's why we often run away from these topics, and children are getting older, they are aware of their difference, they ask. "Fairy tales are a tool that facilitates these conversations for us, allowing us to safely tame the world that children and young people have to live in," she emphasises.
"Station at the top of the hill"
Kasia Pedrycz from the SMA Foundation, who reads the fairy tale 'Station at the Top of the Hill' with her husband, explains that the campaign promoting the book in Poland is part of the popularisation of knowledge about what SMA is. - The fairy tales are primarily aimed at patients and serve them as part of their therapy, but at the same time for the general public they are an opportunity to understand our situation. Through the language of the fairy tale, the stories, the emotions of the characters captured in the tales, we can better communicate with society. By presenting 'Fairy Tales' to others, we want to show them who we are as a community of sufferers. We teach that a child who is in a wheelchair, lying down, connected to complex equipment, has similar needs and emotions as healthy people," she explains.
She also explains that the fairy tale she reads, in part, reflects the fate of her family as well. - My child's diagnosis coincided with drug trials of nusinersen. Until then, families receiving the news of their child's illness were prepared for their child's slow passing, we were given hope. An early access to treatment (EAP) programme emerged overseas - and our Benio got in.
We flew first to France, then to Belgium, until we returned to Poland, where reimbursement finally arrived. And it is about this chance for treatment - a long-awaited turn that is not yet available to all patients - that the fairy tale we read," he stresses.
Nusinersen - this drug has changed patients' outlook on life
Today, both patients and their families are in a very different place. Since January 2019, all SMA patients have been receiving nusinersen as part of the 'Treatment of Spinal Muscular Atrophy' programme in Poland. The drug targets the underlying cause of SMA by increasing the full-length motor neuron survival protein (SMN). It is administered intrathecally, directly into the central nervous system where motor neurons are located, to provide treatment where the disease begins.
This is confirmed by Professor Katarzyna Kotulska-Jóźwiak, head of the Department of Neurology and Epileptology at the Children's Memorial Health Institute. - Currently in Poland, there are 738 patients in the drug programme for nusinersen under treatment, who started therapy at different times. None of them have been excluded from the programme due to ineffectiveness of the drug since the implementation of the therapy. All patients benefit from the treatment," the expert notes.
However, the best therapeutic results are obtained in the group of pre-symptomatic patients, i.e. before the first symptoms of the disease appear. This is confirmed by the NURTURE clinical trial, in which 25 children up to six weeks of age were included in the treatment. All of them are breathing on their own, all of them are standing on their own and 96 per cent of them, are able to walk. Now, thanks to the universal screening programme introduced in Poland from April this year, all newborns with a diagnosis of SMA have been given a chance for normal development, emphasises Prof. Kotulska-Jóźwiak. - Last week, with the help of these tests, it was possible to diagnose the first child with SMA in Poland. Thanks to this, this child will have the chance for very early, and therefore the most effective, treatment,' she adds.
Known and loved ones about the campaign: gives hope and raises awareness of SMA
Wojciech Blach, actor:"The Wolf tells the story of SMA" is a tale for children, for teenagers, for parents. A fable about the world of spinal muscular atrophy that not only tells the story of the disease, but also gives hope, because thanks to the SMA Foundation, the clinicians, the support of the Polsat Foundation, there is a drug available to fight the disease and stop its progression. There is hope.
Jerzy Mielewski, journalist: I joined the 'Wolf talks about SMA' campaign because there should be more awareness in society about this disease, as the hardships parents face are enormous. I am a father of two boys, I know what it means to have my own children healthy.
Magdalena Stużyńska, actress: I got involved in this action because it is the action of the Polsat Foundation, and the Polsat Foundation has been supporting and helping children in need very effectively for many years. That is why I believe in the effectiveness and success of this action.
What does the author himself say about the book?
The fairy tales show that we are all different, and that diversity should be a source of richness, not fear. "In the 'Wolf tells the story of SMA' fairy tales, I wanted to show families who have only just heard the diagnosis, as well as people who are unfamiliar with SMA, what it means to have weak muscles," says psychologist Jacopo Casiraghi, author of the book, who works with families of SMA sufferers on a daily basis.
Why did you decide to write fairy tales dedicated to SMA sufferers?
Jacopo Casiraghi: This was determined by many factors. I was often asked by parents of newly diagnosed children with SMA how to tell their child about the disease, how to explain that they would not be able to walk. Since the language of fairy tales makes it possible to create a fantasy-based setting and tell true stories in it, while protecting those involved, I suggested to parents that the explanations given to their children should be dressed up in the form of a fairy tale. I soon realised that not only the children but also the parents themselves needed stories in order to accept their child's illness, to be able to name the emotions that accompany them, to answer the doubts and fears that plague them. In short, the fairy tales proved to be good not only for the children, but especially for the parents. The SMA fairy tales also make it easier to talk about the disease with people who do not know it, they help the integration process. They also show that we are different and that this diversity is a source of richness and should not be a source of fear. Wolf explains: "We are not all the same, we are all different", and this is a valuable asset.
What do fairy tales show and who do they serve?
JC: I wanted to show parents who have only just heard the diagnosis, as well as people who are unfamiliar with SMA, what it means to have weak muscles. What limitations this disease entails, but also what challenges, emotions and hopes families face when living with a child with 'weak muscles'. I wanted to tell the story in a simple, direct, sometimes even raw way, without being pietistic, like in a fairy tale where I talk about death. At other times I appealed to adventure or irony, as life is a mixture of good and bad, pain and hope. These stories show that the world is not only made up of beautiful and easy things, and that sometimes there are difficult moments that allow families with SMA to feel less alone, to help them mourn their sick child, but also to overcome the limitations of the disease.
Did you use the stories of real SMA sufferers and their families to describe the fate of the fairy tale characters?
JC: All the stories I have described are true. They happened to families, couples or children with whom I conducted psychotherapy for fifteen years, on behalf of the SMA Onlus Family Association or the Clinical Centre of the NeMO Hospital in Milan. Sometimes there are whole phrases in the stories, which I have heard many times in the course of my work. Some reflections are the result of therapeutic activities and psychological reinterpretations of what was said and taught to me by parents and children with SMA. There is a theme of hope in the book, of waiting for change, this being the time of the appearance of the first drug for SMA. Its introduction rekindled hope among families with SMA, but also highlighted, more than ever, the importance of comprehensive disease management.
How was your book received in Italy and in other countries where it was published?
JC: I was lucky! In Italy, 25,000 copies of the publication were distributed in the first two weeks of free paper distribution. The book was also published as an ebook and audiobook, read by Italian actors and voiceovers. Many press articles have been written about the book. The fairy tales have not only been translated into Polish. They had previously been released in an English-language version in the UK, and in a Spanish translation they appeared in Argentina. In Italy, an animated film is being made based on the fairy tales. The most beautiful comment I received was from the father of a young child with SMA - he told me that the fairy tale entitled: Skin of the Wolf, is about him and his emotions. It was a very beautiful, touching moment. It all made me decide to write another book about SMA.
During August, you can listen to the following chapters of the tale every Monday/Wednesday/Friday and Sunday at 7pm. All the fairy tales included in the book "The Wolf tells the story of SMA" can be downloaded or read HERE
For more information on the SMA, visit: www.fsma.pl and www.care.togetherinsma.pl
Source: medexpress.co.uk