The excellence of the specialists for the treatment of muscle diseases on the one hand, the experts of emergency medicine on the other and in the middle, in the center, the patients. Thus, from the discussions around a table during an SMA Families congress, the first recommendations for managing SMA in emergency rooms were born. A real revolution in the health care of these patients but also a revolution for the emergency rooms, because they promote a new emergency management model for those living with spinal muscular atrophy, partly the result of the therapeutic innovations that have affected this disease in the last few years.
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New therapies, new challenges
“The arrival of new therapies against SMA has literally changed the history of the disease for these patients – Fabio De Iaco, national president of SIMEU (Italian Society of Emergency and Emergency Medicine) comments to Salute – and it has been an exciting process. This however, but fortunately, also confronts us with new needs from some points of view: some patients become chronic, they need adequate health care and it is necessary to be better equipped to manage any emergencies”. Emergencies can be many and different when it comes to SMA, a rare genetic disease that compromises muscle function, and so walking, sitting, but also breathing and sometimes swallowing. The main reasons why patients with SMA go to the emergency room are respiratory failure and trauma, continues De Iaco, emergency conditions different from those of any other patient: “The evaluation of the person with SMA must be done by measuring the fragility of these patients, even giving them priority with the same urgency code in triage”.
If the evaluation must take into account the fragility of patients with SMA, the emergency and urgency management interventions of the medical and nursing staff must do the same, which in some cases represent a real peculiarity, continues the expert : “For example, one of the recommendations is not to give oxygen in case of low saturation, an indication that goes against the normal management criteria applied in the emergency departments for respiratory crises”. Other useful indications – from the management of secretions, dehydration, to traumas – are summarized in a small handbook in the form of a poster, designed precisely for those critical conditions in which the management of the patient with SMA could be different from that of a person without SMA, explain the authors. The downloadable flyer on the Famiglie SMA and Simeu website is intended for emergency rooms, but not only: “Even patients can print it and take it with them – continues De Iaco – it may seem trivial as an indication, in reality it is extremely revolutionary as a concept, especially considering the state of stress in the emergency departments today”.
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Don’t leave caregivers out
Alongside the practical recommendations for emergency management, the indication to leave free access to the caregiver in the emergency room also stands out: “The caregiver must never be detached from the patient: I understand that it is difficult to pass this information on, especially in crowded emergency rooms , but it is fundamental”, De Iaco points out. “Today more than ever it is even more important to pay attention to the presence of the parent who, in these cases, is also an expert caregiver, able to transfer the correct information to the professionals of the Emergency Department”, added Marika Pane, clinical director of the Center Clinic NeMO Rome.
The challenge of training doctors and nurses
However, being able to draw up the first recommendations – thanks to the joint work of Famiglie SMA, the NeMO Clinical Centers, Simeu and the Italian Society of Pediatric Emergency Medicine (Simeup), with the non-conditioning support of Roche – is not, however, that the first step. “Recognize that a person with spinal muscular atrophy needs specific answers ea
times different from other patients is a long-awaited goal for us”, comments the President of the SMA Families patient association, Anita Pallara, one of the authors of the recommendations, published in the complete version in the scientific journal of Simeu, the Italian Journal of Emergency Medicine : “It’s the first time this has happened in Italy and due to a neuromuscular disease. It is a huge victory for our community and it is only the first step of a precious journey started with Simeu, Simeup and the NeMO Centres. It will guarantee more serene and safer access to emergency rooms and will be able to have a concrete impact on emergency management”.
In fact, the challenge today remains to ensure that these recommendations are implemented: “There are many emergency departments, and patients with SMA are rare – continues De Iaco – and the greatest difficulty is not adhering to these indications, but rather making sure that they become available to all. The real challenge therefore is to inform and train virtually all emergency rooms”. To do this, in addition to promoting awareness activities and the material dissemination of the recommendations, for example through the distribution of posters and communications at congresses, Simeu and the Nemo centers are already working on a package for the distance training of doctors and nurses, video lessons which include also the contribution of patients’ experiences, probably extended to all neuromuscular pathologies. “Emergency rooms must make themselves available, even when it is difficult,” concludes De Iaco.