Hemophilia is a genetic condition that prevents blood from coagulating as normal. The condition is only suffered by males. However, although women do not have the associated bleeding disorder, they can be carriers and transmit hemophilia to their children. In fact, a hemophilic male will transmit the defective gene to his daughters but not to his sons: the girls will be carriers but the boys will not inherit the condition.
What does this mean in the context of a traffic accident? As you can imagine, a crash, no matter how minor it may seem, can have serious consequences for hemophilic children. Anything from simple bruising or a nosebleed, right through to fractures and contusions, could all pose a real threat to a hemophiliac child. First, it is worth understanding a bit more about hemophilia, as a foundation for introducing a series of basic precautions to adopt on car journeys.
Hemophilia and the coagulation factor
Hemophilia is caused by a deficiency of a coagulation factor in the blood, which means any hemorrhage, not matter how minor, will not stop by itself, and the patient will be at increasing risk the more time passes before action is taken. The problem is not limited to external hemorrhages, such as open wounds or nosebleeds. In fact, internal hemorrhaging (which may be seen as a bruise, caused by a contusion) can be more dangerous because it may go unnoticed.
Small children and newborns are not prone to bleeding, so any bruising that appears in unusual places such as the stomach, chest or back may be grounds to suspect hemophilia. Of course, a nosebleed is likely to offer more compelling evidence, as the bleeding will not stop. A pediatrician can diagnose hemophilia after a series of tests, and will then determine what kind of hemophilia the patient suffers from and the missing blood clotting factor.
There are two types of hemophilia: hemophilia A, caused by a factor VIII deficiency (this accounts for close to 80 percent of all hemophilia cases), and hemophilia B, in which factor IX is missing.
What you need in the car and planning your route
There are a series of important aspects to bear in mind when preparing a car journey with a hemophiliac child on board (or any other individual who has the condition). The Madrid Hemophilia Association (Ashemadrid) has a number of recommendations for us.
Firstly, make sure you have all the materials required to prepare an injection of the required factor for each child. It is very important that the factor be transported in an adequate receptacle and following all storage instructions: some need to be stored at cold temperatures, others cannot be refrigerated again after having already been refrigerated once... Great care is required at this stage, and you need to make sure you are familiar with the product in question.
As well as transporting the factor under the right conditions, a small first aid kit will be required, including sterile gauze, alcoholic iodine, hydrogen peroxide, bandages and plasters. It is best to take everything you need to prepare the injection with you, just in case the patient needs it, possibly as a result of a fall or some sort of blow.
It is worth locating the closest hospitals along your route, checking distances and travel times to each in the event of an emergency and the child needing hospital care.
As an additional precaution, take identification that states the condition and the factor deficiency, along with the required quantities. You could even make a little notice to attach to the child seat in case of accident. Such a notice may be critical to saving the child's life if the driver or other passengers are unable to notify emergency services that they suffer from hemophilia.
As a final note, remember that in the event of any emergency, such as a traffic accident and trauma, the first priority is to administer the factor (even before taking the child to hospital). Any other action should be taken subsequent to this.
We would like to express our gratitude to Doctor Luis Madero López, Head of the Onco-Hematology Service at the Hospital Universitario Quirónsalud Madrid and Hospital Quirónsalud San José, for his help in the writing of this article.