Young cystic fibrosis sufferer Mandy Vella has said she’s “beyond grateful” for the outpouring of support she’s received since going public with her struggles.
“Sometimes it’s harder to raise awareness about an invisible condition, but when you speak to people about it, they understand you, and I’m glad they realise the urgency of my situation,” she told Lovin Malta.
Mandy, 26, was born with cystic fibrosis, a rare, hereditary disease that attacks the lungs and affects around 25 people in Malta.
Cystic fibrosis impacts the cells which produce mucus, causing them to become thick and sticky and resulting in them blocking up tubes, ducts and passageways, particularly in the lungs and pancreas.
It’s a progressive illness that requires daily treatment to constantly remove mucus from the body.
“The way people react to COVID-19 is how people with cystic fibrosis live on a daily basis,” Mandy said. “Using a tissue to open a door is normal for me and I wear a mask when going to hospital to avoid viruses.”
She described the condition as “self-contradictory” as it requires regular physiotherapy but also exhausts her to the point where she cannot exercise.
“Walking is important but right now I barely feel as though I can walk to my car,” she said.
Her lifestyle has only degraded since the COVID-19 pandemic broke out last year.
Vulnerable to the virus, she’s remained indoors for practically the last 12 months, only going out briefly to meet her friends from afar while remaining in her car and eating out with her father once last summer, when the number of COVID-19 cases dropped to minimal levels.
She also lost her job as a real estate administrator, with her company unable to meet her request for reduced hours.
“It’s mentally draining,” she said. “Some people say I can just relax at home, but relaxing gets boring too.”
However, hope for Mandy and other cystic fibrosis sufferers recently emerged through the drug Kaftrio (also known as Trikafta), which was approved for use by the European Medicines Agency last August.
Richard Hunter, a 37-year-old British cystic fibrosis sufferer who is married to a Maltese woman, started taking the drug last September and describes it as a complete life-changer.
“I look at my life as pre-Kaftrio and post-Kaftrio,” he told Lovin Malta. “I used to have to spend hours everyday doing chest physio exercises to clean the mucus out of my lungs but it was a hopeless cause because you can never clear it all out, and it would have returned by the time I’d wake up. It was like trying to save a sinking ship.”
“Within the first 24 hours after taking Kaftrio, I coughed up more mucus than I ever did in my life and within four days, my chest was cleared of mucus. I haven’t coughed up mucus since September.”
“I used to cough up quite a lot of blood but don’t anymore, I used to have to sleep on three pillows and now I can sleep on one, I can now laugh without coughing. The first thing I do when I wake up isn’t cough, like a slap in the face and a reminder you have cystic fibrosis.”
Former Opposition leader Adrian Delia yesterday wrote an impassioned letter to Prime Minister Robert Abela, urging him to do his utmost to provide this potentially life-saving medication to Mandy Vella and other people suffering from cystic fibrosis.
“Malta is passing through a difficult time, the people are getting tired and stumbling, our front liners are broken, but the country cannot lose its moral fibre or its soul,” Delia wrote. “Malta can never let its children die by themselves while it stares from afar without doing anything.”
“The people did their utmost to help families thousands of miles away who were victims of a tsunami and do their utmost to support missions around the world. Istrina provides redemption and hope to people in need. Malta convened entire nations during the Libyan civil war; we were always the nurse of the Mediterranean.”
“We cannot leave Mandy Vella and other people like her alone. We cannot deny her father hope. She’s one of us, she’s our Maltese sister. Prime Minister, we can save her and we have a moral obligation to do our utmost to help. You have the ability, and I urge you to have the kindness.”
Nationalist MP David Thake made a similar call in Parliament yesterday, a sign that the pressure being applied by cystic fibrosis sufferers could be starting to work.
The Health Ministry had said it’s considering the medication for inclusion in the government formulary, but time is of the essence to ensure sufferers’ lungs don’t get scarred to the point they won’t even be able to live a normal life with the drug.
Vertex Pharmaceuticals, the company behind Kaftrio, is allowing people with serious cystic fibrosis to request the drug on compassionate grounds and has pledged to process such requests as quickly as possible.
And while the new drug is expensive, Hunter pointed out that costs to treat cystic fibrosis patients without it build up over time.
“I used to have to take several tablets and use nebulisers, and had to go to hospital twice a year, which was an extra cost for the National Health Service.”
“In the UK, it’s also resulted in people being removed from the lung transplant list, meaning it doesn’t only save thousands of pounds for the NHS but gives people a higher chance of living without a risk that their lung transplant will be rejected.”
Mandy all urged the government to act as quickly as possible before time runs out for her.
“My lungs used to operate at 35% capacity but now it’s down to 23%. It’s an issue of time; the longer I wait the worse it will get.”
Do you think the Maltese government should step in to help cystic fibrosis sufferers?