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Blood disorder: Party animal had heart failure

Now a dad-of-three, Patrick Wong learned some harsh lessons while growing up with deadly blood disease thalassemia: while doc urges partners to get blood tested before starting a family

PATRICK Wong knew he’d be in the doctor’s bad books.

He had been partying for most of his 20s and had, by his own admission, been “non-compliant” with his life-saving medications.

But it was only when he was really struggling to even negotiate a flight of stairs in his home that he realized something wasn’t quite as it should be.

Little did he know, he was actually having heart failure, with his body’s most vital organ slowly, but surely, giving up on him.

“As a teen, growing up, I wasn’t very compliant and just wanted to party all the time,” explained Wong, a mortgage broker and father of three small children.

“Many years of inconsistency with the medicine — it needs time and regularity to be effective — had taken its toll.

“For about a month before the (heart failure) diagnosis, I was lethargic and lazy and I just put it down to being out of shape. So I went to hospital and they realized my heart was running at about 30 per cent.”

Wong’s heart was calling it a day due to a potentially fatal accumulation of iron on the organ — caused by the frequent blood transfusions he has had for most of his 36-year life to deal with the rare blood disorder thalassemia major.

After another chastise from the docs, Wong was put on two years of chelation — a process of receiving medication through a pump to clean the excess iron out of the system — every single day to clear up the dangerously high levels of iron.

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.

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LIVING in the Garden City area, Wong was diagnosed as a four year old with the potentially fatal blood disorder.

His deadly condition was passed down to him unwittingly by his parents; his mom had thalassemia minor and dad had hemoglobin E, two very minor blood disorders that showed no signs and required no treatment.

As a little boy, Wong had to go through blood transfusions every four weeks, as well as the aforementioned iron chelation, eight hours every night and five days a week.

“I spent a lot of time at Children’s Hospital. I thought it was great, I didn’t know anything else really,” recalled Wong, who was also born with just one eye, although he doesn’t think that’s anything to do with his blood disease.

“I played hockey and played sports and my parents were never the type to say ‘don’t do this or don’t do that,’ so I had a fairly normal childhood.”

Wong said he had many fellow patients at BC Children’s Hospital who he grew up with and called friends, such was the length of time he spent there.

Sadly, said Wong, not all of them have managed to live as long and as fulfilling a life as himself.

“Many of them have passed away, it’s so sad,” said Wong.

“One of them never, or rarely, did the iron chelation. He just didn’t think (death) would ever come to him and I don’t think he had much support from his parents either.

“There is a stigma or refusal to accept the disorder among certain Chinese cultures. And I think that plays a big part as well in the treatment.

“I do feel very lucky living in Canada, though. We have the treatment here that allows me to live my life almost as normal. In other parts of the world, people are still dying very young.”

One factor that undoubtedly helped Wong stay on track and live as long as he has was the introduction in 2006 of an iron chelation pill, negating the need for him to be hooked up eight hours a day, five days a week, to a pump.

Not everyone can take the pill, however, and some patients still have to use a pump.

 

AS far as avoiding passing his genetic condition onto his children and getting his now wife, Charmaine, tested prior to them both starting a family, Wong said the any results would not have altered the path they took.

“We said no to testing because it was not going to affect our decision to start a family,” said Wong, a Vancouver Thalassemia Society board member.

“I had experience of the disorder and knew what it took to get through it and knew that the treatment of it was good in Canada.”

As it turned out, Charmaine doesn’t carry the gene and, while their children are all carriers — Jacob, 5, and Jaxon, 1, both have thalassemia minor, Zoey, 3, has the less complex hemoglobin E — none require any treatment.

“The dog’s pretty healthy, too,” joked Wong, who recently moved to Delta with his family of five, plus a dog, Pachinko.

“And I will, fingers crossed, live into my 80s or 90s.”

 

 

Deadly duo: Two minors clash to make major 

WANDERING around blind, playing Russian roulette with your unborn baby’s future is a deadly game of chance easily sidestepped in the battle against the rare — but potentially deadly — blood disorder called thalassemia major.

Millions of people around the planet may be unknowingly carrying the thalassemia minor gene, which, on its own, means nothing to the host or people around them.

But if that same person conceives a child with a partner carrying the same “minor” gene, they have a one in four chance of producing a baby with the incurable “major” disorder.

The answer — according to Dr. Hatoon Ezzat, medical director of Hemoglobinopathy Program of BC at the University of BC — is simple: get blood tested before you start planning a family.

“If I have a patient and I realize they’re a (thalassemia minor) carrier, I advise them to have their partner tested by a family physician,” said Ezzat, who’s also a clinical instructor at St. Paul’s Hospital’s Providence Health Care Division of Hematology.

“It’s inherited; babies are born with the disease as the parents are the carriers. They may or may not know they are carriers.

“In some countries, such as Saudia Arabia, Cyprus and Bahrain, — where the disorder is more prevalent — there is pre-marital testing, which would discover if you’re a carrier of the gene. If just one of you is a carrier of the gene, then there’s no problem.”

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Dr. Hatoon Ezzat - Submitted

ONE of the worst thalassemia affected countries in the world is India. Statistics revealed by the Indian Red Cross indicate that thalassemia major affects more than 1 million people in India, with more than 8,000 reported thalassemia births taking place every year.

However, around 30 million Indians are estimated to carry what’s known as the “beta globin gene” in one form or another.

In B.C., there are only 47 adults and children being treated for thalassemia major at two comprehensive centres: 17 kids at B.C. Children’s Hospital and 30 at St. Pauls.

Unfortunately, there’s no definitive way to gauge the actual numbers of thalassemia gene carriers.

“It really is unknown,” said Ezzat.

“There is also a high prevalence of the gene in Chinese and Vietnamese people and, indeed, people from most Eastern countries.

“So, as there are many people from that origin in B.C., then it’s something I’m concerned about.”

The trend, added Ezzat, is for more and more people to migrate to B.C. from parts of the world where the gene is more prevalent, meaning a higher level of awareness of the disorder is required urgently.

 

THE only known cure for thalassemia major is a stem cell transplant (used to be called bone marrow) from a brother or sister; a treatment which started in the ‘90s.

“We now have gene therapy as well, but it’s still going through clinical trials,” added Ezzat.

“But if you manage your patient well, through an iron chelator (which cleans the excess iron out the system), then they can live just as long as anyone else. The iron chelation can also come in a pill form.

“About 15-20 years ago, we started to reverse the trend of people dying young from the disease, but we still need more people to know about the gene.

“Ninety per cent of patients that are managed well — which is the case in B.C., but not in other parts of the world — live to a normal age.”