With precautions, hemophiliacs live active lives

April 16, 2015 Providence Health Team

April 17 is World Hemophilia DayFor a child with hemophilia, a Saturday afternoon spent climbing a tree or playing a game of tag is a potential ticket to disaster. Even a minor scrape, cut or bruise from rough-housing with a pal can be life-threatening. 

The danger for a hemophiliac is that a bleeding injury won’t clot. Without clotting, the afflicted person could bleed to death.

Most people who bleed from a cut or abrasion benefit from their body’s protective reaction. The platelets and proteins in their blood combine as clotting factors. This sticky mixture reaches the injury site and forms fibrous clots to staunch the bleeding.

A hemophiliac can experience bleeding in the following ways:

  • Bleeding from wounds and cuts
  • Nose bleeds
  • Menstrual bleeding
  • Internal bleeding from bruises and in the joints or the brain 

Bleeding is obvious and not-so-obvious

It’s easy to spot a bleeding skinned knee or a nosebleed soon after it occurs. Internal bleeding is much more difficult to identify, especially in babies and toddlers who can’t verbalize how they feel. Bleeding can occur inside the body from bruises they get on the abdomen, back or buttocks. 

Older children and adults with hemophilia also are at risk of internal bleeding from bruises from accidents. Bleeding can materialize in the joints and the brain. Other signs of internal bleeding include dark or black feces, red urine or blood in vomit. 

Two types of hemophilia 

Hemophilia is classified into two main groups: Type A and Type B. Eighty percent of hemophiliacs have Type A. It’s characterized by the absence of the factor VIII blood-clotting protein in the blood. Type B, which is much less common, signals a lack of the factor IX clotting agent. The disease’s severity varies: some patients have only occasional issues, while others with severe hemophilia need to be on constant alert. 

Typically, hemophilia is diagnosed in children after they start walking. Parents notice their child bruises easily or bleeds excessively and alert their pediatric provider. Their doctor can diagnose the disease with blood tests and after ruling out other causes. 

Hemophilia is rare 

This rare genetic disorder is inherited mostly by boys. About 1 in every 5,000 males is born with it. Girls also can inherit the gene, but instead of having the disorder themselves, they have a 25 percent chance of passing it along to their sons. In about 30 percent of those with it, they don’t have a family history of the disorder. Their condition was caused by a genetic change called spontaneous mutation. 

Modern treatment extends lives 

In the early 20th century, hemophilia was considered a death sentence – the average lifespan for someone with hemophilia was 11 years. Until 1968, hemophiliacs lived to an average age of 20. Advances in medical treatment since then have increased life expectancy. With proper care and maintenance, those afflicted today have the same life expectancy as anyone else. 

While hemophilia can’t be cured, it can be managed by knowing how to properly treat a bleeding episode. It also can be managed through factor replacement therapy in which clotting factors are infused intravenously. Parents of young children with hemophilia can prevent issues by encouraging a regular exercise program and maintaining a healthy weight. And, surprisingly, good dental health will reduce bleeding gums and keep the need for dental surgery at a minimum. 

Older kids with hemophilia most likely need to stay away from contact sports. But that shouldn’t keep them from participating in sports such as swimming, golf and bowling. 

But, ultimately, good communication between parents and child – and boundary setting that emphasizes safety – will keep dangerous bleeding episodes to a minimum. 

Your Providence primary care provider can answer questions about hemophilia, or refer you to a specialist if you suspect your child may be a hemophiliac.

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