A FRUSTRATED Isabella Sammarco stamped her little feet, adamant she be heard.
Even though she was just four-years-old, Isabella was sharp enough to realize her mom, Serafina, was protecting her innocence by refusing to give an honest answer to a simple question.
For parents around the world, who will find familiarity in that very adult-child “exchange,” this was nothing out of the ordinary.
Nothing, except the grave nature of the question posed by Isabella that day would be followed by an answer equally as grim.
“She kept asking me, ‘when will I die, mom?’” Sammarco said.
“At first, I skipped around the question and said that we all die sometime, like most parents tell their kids, I guess. But she demanded to know and kept asking, ‘what about me?’
“After I told her the truth, she seemed to accept it and, because of that, she seemed to enjoy every day of her life.”
ISABELLA enjoyed a very happy life until dying aged 14 in 1986.
She had lived those years with the rare genetic blood disorder thalassemia major, a hereditary disease contracted from her parents, who only knew about it when she was eight-months-old.
For the duration of her short life, she had to pump medication through her system overnight almost daily and had to receive blood transfusions every few weeks.
But it was 14 years that her family — including dad, Pasquale, and older brother, Peter — made sure was lived to the fullest, with no regrets and little self-pity.
She attended what was then called London Secondary and boasted a large circle of close friends, many of whom looked up to her and the manner in which she embraced life, despite knowing her sand timer of life was slipping away.
“We tried to make her life as normal as possible and we made sure we took as many family vacations as possible, even though we often couldn’t afford it,” said Sammarco.
“She enjoyed her life and we enjoyed her. When we were on vacation, she would always say, ‘This is the life.’
“She was a gift to us all as she lived in the moment every day.”
What is thalassemia?
Thalassemia is a genetic blood disease. People born with this disease cannot make normal hemoglobin, which is needed to produce healthy red blood cells.
Thalassemia is most often found in families of Chinese, South Asian, Middle Eastern, Mediterranean or African origin.
The only way to know if you carry the thalassemia trait is to have a special blood test. The combination of one gene from the mother and one from the father leads to three different types of thalassemia.
The treatment involves regular blood transfusions, which leaves patients with an excess of iron in their bodies. This dangerous excess iron has to be removed, or chelated.
GROWING up in the family home in Bouchard Court near No. 2 Road and Westminster Highway, Isabella, from that innocent age of understanding the world, knew her life was going to be short.
Back then, there was no known cure for the disease, and in most cases, such as Isabella’s, those suffering from it rarely made it to adulthood.
Through no fault of her own — or, indeed, her parents — Isabella was born with the thalassemia major gene from parents who both, unbeknown to them at the time of her birth, carried the “minor” version of the disorder.
Throughout her life, excess levels of iron were building up inside Isabella, which had major effects on how she looked — the disorder often produces darker skin, more pronounced facial features and stunts growth — and her ability to function normally at school due to extreme fatigue.
“She never felt sorry for herself and was never spoiled,” said her mom.
“We tried very hard not to spoil her; it wasn’t always easy I can tell you.
“There were a few times, not too many, when she was picked on because of how she looked. But Peter (her brother) was very protective.”
Although Isabella had all the support she needed at home and in her circle of trust, there was, of course, a severe lack of understanding in the broader community.
“There is usually very little physical evidence and the lack of energy prompts comments such as ‘you look fine to me’ and ‘there’s nothing wrong with you,’” explained Sammarco.
“The sheer severity of the illness is underestimated because so many people appear to be normal.”
IN 1987, a year after the family lost Isabella, Sammarco started the Vancouver Thalassemia Society, as a support group for other families and to do some fundraising.
The money raised goes to scholarships, sending kids to camp, sponsoring patients to attend the Thalassemia International Federation, — a medical convention which takes place every other year — and the distribution of society information.
In 2012, Sammarco wrote and published a book, Isabella’s Journey, which charted her daughter’s and her family’s battle with the blood disorder.
“I had to write this book as there is still so much mystery surrounding thalassemia and I wanted people to relate to what was, is and can be,” said Sammarco.
“I guess it was therapeutic in a way. But there are adults still dying young and no one seems to know about it.”
Unfortunately, the lack of awareness, said Sammarco, is still the same today and that’s why she said she’ll keep fighting for everyone who suffers from it.
“Awareness is the major problem,” she added.
Now, despite Isabella’s death some 28 years ago, everywhere Sammarco goes she can feel her daughter’s presence.
“She’s always with me in everything I do with the society,” said Sammarco.
“She’s my invisible force. I communicate with her every day in my own way. I don’t grieve her any more. I still miss her, obviously.”
NEXT week, Saturday, Oct. 4, the Vancouver Thalassemia Society hosts its annual fundraiser at the Riverside Grand Ballroom at 1231 Burdette St. from 6 p.m. to midnight.
A special presentation about the treatment of thalassemia by California’s Dr. Vasili Berdoukas will be woven into an evening of buffet dinner, dancing, live entertainment, door prizes, raffles and a silent auction.
Tickets, costing $45 in advance for adults, $50 at the door and $22 for kids aged 4-12 (three and under are free), can be ordered at www.bcthalassemia.org.
