«I was less than 25 years old, it was spring. I looked at my fingers: they were blue, just because I was in the shade». Galetti, now vice president of Gils and Fesca, thus recalls the dawn of his illness. And then the diagnosis, shortness of breath, pulmonary fibrosis, ulcers, fear in the period of Covid and, today, the prospect of a lung transplant. A testimony, on Systemic Sclerosis Day, to talk about a chronic autoimmune disease that affects women 5 times more than men. Unknown causes, even very serious symptoms, early diagnosis is essential

Sti celebrate today, June 29ththe World Systemic Sclerosis Day, also known as Scleroderma. A rare, chronic autoimmune disease of the connective tissue involving the vascular system, skin, and internal organs. A day to raise awareness of the needs of people who are affected by it, with the ambitious goal of improve diagnosis, treatment and quality of life. Because living with this pathology is often a daily struggle, in which it is very important not to be alone. As history demonstrates, very hard but full of life, of Ilaria Galetti, Vice President of Gils (Italian Group Fight against Scleroderma) and of cool (Federation of European Scleroderma Associations), patient with systemic sclerosis.

“My life with scleroderma”. The story of Ilaria Galetti

«It all started with the hands. I was under 25, it was spring. I looked at my fingers: they were blue, just because I was in the shade», recalls Galetti, who is 50 today. He is the so-called Raynaud’s phenomenon, generally the very first symptom of Systemic Sclerosis. The visits begin, the suspicion of an autoimmune disease and finally, within two months, the diagnosis.

«Mine is a rare case, the diagnosis arrived very quickly: this is essential, to start monitoring and therapies as soon as possible, and try to avoid organ damage». At least, try. Ilaria thus begins, with a couple of pills useful for circulation and with a regular follow-up every six months: the so-called pre-clinical phase.

After a year and a half, the stumbling block: «I was young, I was recently with the one who later became my husband and, during a walk in the mountains, I realized that I was out of breath. In a very evident way, on a path taken other times without any effort». CT reveals pulmonary fibrosis. “Since, the lungs have been my Achilles heel».

“We wanted to have a baby. But the therapy sent me into menopause at the age of 30″

Immunosuppressant therapy is postponed, until it can no longer be postponed. «It would send me through menopause, I knew it, and we wanted to have a baby. I was in my early thirties when it happened. But then I had to weigh the desire to have a child and to take care of myself. And then I think nature is very clever: I had already had a miscarriage. My body won’t allow me to have a child».

Not just the lungs. Ilaria’s body suffers at the home of two ulcers on her feet and one in her arm, leading her to surgery. AND the gastroesophageal tract of the intestine is also attacked, while the pulmonary situation worsens. «Let’s move on to another drug, then to yet another, biological: a pharmaceutical category that didn’t even exist at the time of my diagnosis. Well, this drug has an amazing effect on me: I’m fine, wonderful».

But well-being does not last long. “After a while’, the drug no longer works, I get worse again. So let’s try one of the more powerful ones biological immunosuppressants out there, it works, and it’s the same one I still use».

Pulmonary fibrosis and oxygen, 24 hours a day

About 4 years ago a new slap in the face for Ilaria: «I remember the cardiologist, during a cardiopulmonary test. He tells me: “Stop!”. And I try to justify myself, I sketch: “Yes, of course, I’m really out of practice”. “No, Ilaria, the machines say you really can’t do it”». Ilaria’s story with oxygen begins that day. Before, only at night, when the risk, not being conscious, is to go into apnea and send the heart into pain. After a while’ the oxygen tank and nose tubes are prescribed for her 24 hours a day.

Covid, scleroderma and two years without leaving the house

Until, overnight, the world stops turning: Covid arrives, for Ilaria it is absolute terror: if she had taken it she would not have survived. «I remember February 22, 2020, the day of patient zero. I immediately canceled all commitments, and I went into lockdown, alone, right away. My husband also took incredible precautions. In short, I stayed in home for two years, two years in which I only met my husband and the doctors».

With equal and opposite emotion, Ilaria recalls the day when the refrigerated van with vaccines crossed the Italian border: “I was the happiest and most grateful person in the universe”. Naturally, Ilaria gets 5 vaccinations and when finally, last year, she gets infected with the virus, the monoclonal antibodies. But she is forced to stop the immunosuppressant drug that was treating her Scleroderma. «The only possible choice to allow my body to defend itself from the virus».

The perspective of lung transplantation

Today Galetti has returned to taking that drug, and another, and yet another. And it’s being evaluated for a lung transplant: “This requires me to train a lot, a studied diet: I must be healthy enough to be able to receive such precious organs. Those lungs mean that a person is gone and that she was generous enough to donate them: they must be given to a person who can do it». There are many unknowns, from the possibility of rejection to infections, but for Ilaria this is the great hope at this moment.

The disease brought many difficulties as a dowry. Above all oxygen, which you must have a safe supply of at all times, even when you travel. But she also brought her a role that gives her joy: «Representing patients with my disease and trying to improve their lives satisfies me». And a no less great gift: «The ability to appreciate the small and simple things in life, never taking them for granted».

What is Scleroderma (or Systemic Sclerosis)

The term means “hard skin”. It is a pathology that affects women more between the ages of 30 and 50, but it can also occur in children and adolescence. It is a rare disease, with an incidence of between 4 and 20 new cases per 1,000,000 inhabitants each year. In Italy there are therefore about 25,000 people affected by the disease.

Raynaud’s phenomenon

In most patients, the first symptom is the so-called Raynaud’s phenomenon: a clinical condition in which the extremities of the body, more commonly the hands and feet, become icy, numb and change color as soon as it gets cold. They become pale or bluish and then redden: is the result of an excessive response of the organism to environmental stimuli. Scleroderma does not necessarily correspond to the phenomenon, however 98% of those with the disease have this phenomenon.

This first phase can be followed more or less rapidly by the development of the first complications of the disease. And that is skin fibrosis (ie hardening of the skin and other parts of the body). Edema, swelling and ulcers on the fingers. Alterations of the oesophageal or gastrointestinal transit. And also (within the first three years of illness) pulmonary fibrosis. Or that there is heart or kidney involvement. Later is the appearance of other pathologies affecting the lungs, such as pulmonary hypertension and interstitial pulmonary disease, the main cause of death in patients with systemic sclerosis, responsible for about 35% of related deaths.

Early Scleroderma, the importance of an early diagnosis

But, and hence the importance of a early diagnosisto talk about Early Scleroderma the presence of a Raynaud’s phenomenon associated with the presence of abnormalities in tests (such as nail video capillaroscopy, which evaluates blood circulation) is sufficient. And this diagnosis is enough to set up an adequate clinical and therapeutic monitoring plan and thus avoid a worsening of the disease.

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Diagnosis, research and sharing: Gils’ commitment

The commitment of GILS (Italian Group for the Fight against Scleroderma), a founding member of FESCA, has been for 30 years to defend the rights of the patient, to raise awareness of early diagnosisFor support research and young researchers, as well as to promote a new approach to the doctor-patient relationship, increasingly based on listening and personalized assessment of each individual case.

Hence the opening of 15 Scleroderma Unit: network distributed on the national territory for facilitate the stages of diagnosis. The birth of the project ScleroNetwhich aims to improve the quality of care for patients through knowledge sharing. The adoption of common guidelines for disease management.

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