Hope & Healing: The UF Health Blog

Steel, the hemophilic superhero

As my wife and I were watching television one night, I heard one of the characters say, “You’re a Hemophiliac! Your blood doesn’t clot, your wounds can’t heal, and if it’s bad, you could bleed to death.”

Of course, my wife and I had a great chuckle at how preposterous it was that this could be deduced in the few minutes the show had been on. These few descriptive words seem to represent what most people think hemophilia is all about. Then again, for someone like me who takes care of patients with hemophilia, to have even heard the disease mentioned meant, of course, that I was going to need to keep watching the show, Legends of Tomorrow, on the CW.

What is Hemophilia?
If you haven’t guessed by now, hemophilia is a disease that stops the people it affects from clotting properly. As a result, when these patients have an injury or start bleeding, they have a hard time stopping. Different people will have varying degrees of severity with their bleeding. Those with the disorder have to learn to be careful and how to manage their bleeding when it happens.

Hemophilia is a disease that has been recognized throughout history. Most people probably know something about hemophilia because it is known as the “Royal Disease.” It affected many members of Europe’s royal families starting when Queen Victoria of England gave birth to her 4th child, Prince Leopold, in 1853. Perhaps the most famous member of the family was Tsarevich Alexis Romanov, heir to the Russian Empire. Many people believe that it was because Alexis had hemophilia that his parents came to rely upon the brutal advice of Rasputin, which ultimately resulted in the fall of the Russian Empire. More recently, Ryan White’s life highlighted the tragedy of HIV in the hemophilia community. Ryan passed away in 1990.

From a medical standpoint, we know that hemophiliac patients lack enough Factor VIII or IX. Clots form in the body when we injure our blood vessels. The contact of the broken blood vessel with the proteins, or clotting factors, and the platelets in our blood is what ultimately forms the clot. Because people with hemophilia do not have enough Factor VIII or IX, their clots form slowly, or not at all, and bleeding becomes a problem. In particular, bleeding may frequently affect the joints, which can lead to early destruction of the joints, sometimes as early as in the teenage years. These patients develop the kind of arthritis and joint damage that would normally be seen in people who are in their 70s or 80s.

How is Hemophilia Treated?
Not all bleeding and injuries are the same. Some are very mild, like a mosquito bite or a scrape from a thorn, and would cause very little bleeding or bruising. Others can be very severe, like a fall onto your head or surgery to a major organ. These require very careful evaluation and close monitoring, usually in a hospital. With all bleeds, treatment starts by addressing the area of bleeding. With minor bleeds, this is often enough to control the bleeding. Local measures are remembered with the acronym RICE:
R. Rest. Stop the activity that you are doing.
I. Ice. Apply ice or something cool to decrease the loss of blood and swelling.
C. Compression. Apply pressure for at least 15 minutes.
E. Elevation. Raise the area of bleeding to the same or higher elevation of the heart.
More severe bleeds often require an increase in the amount of factor in the blood, which can be done with the use of intravenous infusion of replacement factor. Sometimes this is done by a doctor or a nurse, but often times family members or the patients are taught how to put the needle in their veins properly so that they can do it themselves.

Growing up with Hemophilia
Nate Heywood, known by his superhero name Steel on Legends of Tomorrow, is so excited to be on the superhero team once it is re-assembled that he refuses to leave. During the adventures, he even gets a chance to meet his grandfather that he always looked up to growing up. In this meeting with his grandfather and at various times during the show, he is able to reflect on what it was like growing up with hemophilia.

Nate says to his grandfather, “All of my life, people have been trying to shield me from the world, your son included. Meanwhile, all I ever wanted to be was a soldier like you.”

All Nate wanted, like all the boys I’ve ever known who have hemophilia, was to be normal and grow up to be like their superheroes and role models. He gets to live out this dream in a fantastic way in the show, but this is not true for most boys with hemophilia.

Nate recalled, “When I was five, I cut my knee and my mother wouldn’t let me out of the house until I was sixteen.” For him to feel this way and for any child with hemophilia to feel this way horrifies me. We want our children to be active and to play with other children just like any other child would. The goal in the treatment of a child with hemophilia is not to whisk them away to be isolated in a padded room so that they will not hurt themselves, but rather to prescribe the proper level of treatment that allows the child to have a normal life and aspire to achieve his or her dreams. Put more succinctly by another character on the show, “Every day, you were afraid to live.” No child with hemophilia should be afraid to live.

Myths about Hemophilia
MYTH #1: Children with hemophilia are more fragile and less capable than other children.

FACT #1: Children with hemophilia can contribute in many ways both physically and mentally. Physical activity, in fact, should be encouraged as it is strength and coordination that helps to prevent many injuries.

MYTH #2: The blood of a hemophiliac is laden with germs and infections that will be passed on to anyone who helps them when they are in need of help.

FACT #2: It is always wise to be careful and have protective barriers when handling blood, but hemophilia is not a contagious disease like strep throat or the flu. A hemophiliac’s blood lacks clotting factor but has no more reason to harbor an infection than any other person’s blood. While HIV and Hepatitis B and C infected a large number of patients during the 1980s, these infections were acquired by hemophiliacs during that time because of contamination of the factor replacement available, which were all derived from other people’s donated blood. The most widely available products today are made using recombinant technology that have little, if any, human blood products. The risk of infection with these modern products is essentially zero. Because of how devastating the prior epidemics were to this community, everyone remains sensitive to its impact and ever vigilant.

MYTH #3: Hemophilia does not affect women and girls.

FACT #3: Females have always been thought to be carriers of the disorder. This is in fact true, and boys can only inherit hemophilia from their mothers; however, even carriers can have bleeding problems. In particular, when carriers have factor levels that are low, they may have problems with heavy and frequent bleeding with their periods, surgical procedures, nose bleeding and childbirth. It is important to recognize this bleeding as a problem because all the treatments available for boys with hemophilia can help girls as well.

A super-serum in real life
Nate Heywood becomes ‘Steel’ in the show. He gains super strength and a nearly invulnerable skin of steel. He gets his powers after being injected by a “super serum” made by Ray Palmer, also known on the show as ‘Atom,’ a genius inventor. The fact that an injected serum would transform him into a superhero is not a minor detail to be lost in Nate’s story as a hemophiliac.

Hemophiliacs all around the world, in fact, have a “super serum” that they are using to make their lives better. Hemophiliacs everyday inject themselves with factor to treat bleeds and prevent them from occurring. It does not transform them into people with super-human powers, but the fact that it almost transforms them into humans without the disease is indeed super.

An even more “super serum” is on the horizon. It is believed that one day in the very near future an injected therapy will correct a patient’s cells of the genetic abnormality that causes hemophilia. If it is successful, hemophiliacs will be cured of their disorder. The trials using gene therapy to cure hemophilia are in their very early phases, but success has been widely reported. As fantastic as it seems, a super-serum is being developed and will one day cure hemophilia.

In the meantime, it is still important for patients with hemophilia to know about their disorder. They need to learn how to take care of themselves. They need to learn how to treat their bleeds and how to inject themselves with factor replacement. But most importantly, they need to learn to not be “afraid to live.”

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