2023-12-04 10:54:51
by Ruggiero Corcella
On 31 October, Aifa had warned of the quota and then of the shortage of the medicine due to “production problems” until 8 March 2024. In reality, patients are already forced to go abroad. Difficulty also in finding the antiarrhythmic mexiletine
On the sites and social channels of patients with hereditary heart diseases, the tom-tam is incessant: pharmacies are running out of supplies of nadolol, a beta blocker which is a lifesaver in patients with genetic diseases associated with the risk of sudden death, including long QT syndrome and catecholaminergic ventricular tachycardia characterized by a high risk of cardiac rhythm irregularities which can cause syncope (loss of consciousness) and sudden death due to cardiac arrest, especially during conditions of physical and emotional stress.
«We are inundated with requests for information from patients who would like to know where and how to find nadolol» says Professor Silvia Priori, director of the Molecular Cardiology Operational Unit in the Experimental Medicine area, at the Scientific Institutes of Irccs Maugeri in Pavia. «In the vast majority of patients there is a lot of concern about the situation that has been created» echoes Simone Succi, 43 years old from Florence, suffering from catecholaminergic ventricular tachycardia, one of the founding members of the«A family for the heart» association.
What do people suffering from these pathologies ask for? «We want to be sure that nadolol will be available again in March, because otherwise we run a really big risk», is the appeal launched by Simone Succi.
The causes: «production problems»
The reason? «Production problems (lack of the active ingredient)», as the Italian Medicines Agency explains in one informative note of 31 October agreed with the German company from which Italy is supplied and published on the Aifa website. Specifically: «In the hospital channel, the medicine is distributed on a quota basis until 01/01/2024; from 02/01/2024 to 08/03/2024 the medicine will presumably be totally lacking; retail channel: the medicine will presumably be totally lacking until 03/08/2024″. AIFA specifies that the supply shortage is not related to any defect in the quality of the medicine or safety problems.
In short, we are once again faced with a problem of drug shortages. Which also concerns mexiletine, an antiarrhythmic produced for patients with genetic diseases of the heart and skeletal muscle in this case by the Military Chemical Pharmaceutical Plant of Florence (Ministry of Defense). Unfortunately, however, for several months the Military Chemical and Pharmaceutical Plant has also stopped production. In the case of Mexiletina, possible shortages were already announced as early as January 31, 2022 for “adjustments to production plants”. There was an adjustment, but production did not resume. Patients’ anxiety about shortages of medicines is exacerbated by the fact that it is not the first time that nadolol supplies have run out: it also happened in 2009 and in 2014 it was even withdrawn from the market and then returned after protests sparked by patients who without the life-saving drug they feel at risk of arrhythmic death
What is nadolol and for which pathologies is it used
«Nadolol is a beta blocker, that is, it belongs to a class of drugs that reduces the effect of adrenaline on the heart in the cardiological field and this is particularly useful in patients with serious ventricular arrhythmias that are triggered when the patient has high levels of adrenaline — explains Professor Priori who is also Professor of Cardiology – Department of Molecular Medicine at the University of Pavia -. High levels of adrenaline which are typically the result of physical activity, such as sport, training, or emotional stress such as in the case of a scare or a sudden loud noise. We even have patients who suffer from long QT syndrome, a disease particularly susceptible to sudden noises, who can have cardiac arrest because the phone rings in the night.”
«The other disease treated with nadolol is called catecholaminergic ventricular tachycardia, which patients call CPVT, the acronym of the name in English. Even in these patients the arrhythmias are triggered either by emotion or physical activity. Beta blockers actually include many different types of drugs. However, the international guidelines, which for us are those of the European Society of Cardiology of 2022, say that, as the data demonstrate, only non-selective beta blockers are the most suitable for the two pathologies. And these are only two: nadolol and propranolol”, adds the cardiologist.
Theoretically, therefore, would there be an alternative to nadolol? «Aifa says to use propranolol, but it is not the same thing – explains Priori -. We have had patients whose therapy was switched from nadolol to propranolol, not now, who died after the switch.”
Simone’s story
As Simone Succi explains «nadolol is the most effective drug that has been discovered. I have tried many others and none of them work as effectively. I was even hospitalized for 10 days in the coronary unit to try another one, but it wasn’t good. Coupled with another drug I take, flecainide, nadolol ensures that CPVT no longer presents arrhythmias even when making a certain type of effort. So for me it is a life-saving drug.”
Simone, who has an 11-year-old son who also suffers from catecholaminergic ventricular tachycardia, began having the first episodes in 1988. «I was 8 years old and I fainted at school. At that time it was thought to be a manifestation linked to my development. They took me to Meyer hospital, but they found nothing.”
«After less than a year I had a second episode at the seaside and from there I started all the possible checks, but in those years nothing was known about my pathology. Until in ’94, through my cardiologist in Florence, I met the Pavia center and Professor Priori and from there I began all my studies and I was taken into care by them. But the genetic defect was found in 2001. I also have a defibrillator which, together with drugs, gives me protection in cases of cardiac arrest”, he says.
Hunt for supplies
After learning of the AIFA note, he like all the other people with the same pathology or with long QT syndrome began to think about how to stock up at least until March 8 next year, the date by which according to AIFA the production of the drug should regularize. «In recent days I went to Switzerland, because you can still get nadolol there. Here in Italy the local health authorities are bending over backwards and they can’t find it either. They have to order it abroad. Even in France and Germany it is impossible to find it and it is also scarce in Switzerland. I have to take three a day and therefore a box, which contains 30 tablets, doesn’t even last me ten days. So far I have made do by asking some specialized pharmacies, in Florence and also outside the city, to prepare the galenic for me.”
«But in Italy it is increasingly difficult because even the active ingredient is starting to run out. Some pharmacies have told us patients, but nothing is certain, that perhaps in January the active ingredient will be available again: however, not even pharmacists are sure when they will be able to start making galenic preparations again. Then I found a pharmacy in Switzerland that prepares them for me and other patients. But the costs are exorbitant: in Italy a pack of 30 tablets costs around 11-12 euros. In Switzerland, I paid 100 euros for 100 pills. And the same amount is spent on the galenic preparation, considering the shipping costs.”
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December 4, 2023 (modified December 4, 2023 | 09:54)
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