March is Hemophilia Awareness Month. The Hemophilia Treatment Center at University of Iowa Children's Hospital, located in University of Iowa Hospitals and Clinics, was established 30 years ago in 1977 as one of the first comprehensive care centers for bleeding disorders in the country.
Jorge Di Paola, MD, a pediatric hematologist/oncologist and the director of the Hemophilia Treatment Center, talks about the disease:
What exactly is hemophilia?
Hemophilia is a bleeding disorder. Our blood clots because we have a lot of clotting factors or proteins that allow that blood to clot properly. Patients with hemophilia lack a specific clotting factor, therefore their blood cannot clot well. They cannot heal their wounds well and they bleed more.
Is hemophilia a genetic disorder?
Yes. Hemophilia is a genetic disorder. The genes for hemophilia are located in the chromosome X, which means that females are carriers of the disease but most of the time do not show the disease, while the males are the ones that exhibit the disease.
How is it diagnosed?
Usually the diagnosis is made by a very astute clinician or a pediatrician that sees that a patient bleeds more than usual. When the patient is referred to us, we can make the diagnosis of hemophilia with one blood sample now. We can measure for a specific clotting factor and see if that factor is lacking or not.
Approximately how many people in Iowa live with hemophilia?
That's interesting because hemophilia, they always say, is an equal opportunity offender. Across the globe, hemophilia has the same incidence. It's usually one in 10,000 male births for hemophilia A, which is factor 8 deficiency, and one in 30,000 male births for hemophilia B, or factor 9 deficiency. Usually, if you make the calculations, we serve approximately 250-300 patients with hemophilia in the state of Iowa.
How is hemophilia treated?
We have come a long way in the last 20 to 30 years. In the past, patients used to receive blood products for this, and some of them still do, but recently, in the last decade, we have the advantage of companies making what we call recombinant products. In the laboratory, they make that factor the patient is lacking. The patient can have the medication at home and he can give the medication to himself intravenously.
What is included in comprehensive hemophilia care?
That's another progress step we've made over the last years, I think. We realized that hemophilia is a complex disease with huge impacts in family lives, lifestyle. Therefore we put together a team of specialists including physicians, nurses, social workers, dentists, physical therapists, and psychologists, and we provide that care in a one-day visit to The University of Iowa. The patient can get this comprehensive care in one day and then go to their community and be cared for by their physicians.
Why is it important to be followed at a center like the Hemophilia Treatment Center for an annual comprehensive care visit?
As I said before, we provide the patient with a huge array of educational opportunities and advice, including a treatment plan, for example. We make a very detailed treatment plan patients take with them and when these patients to go to the emergency room locally, they can take our treatment plan and the people in the ER know how to treat this disease. So I think that's why it is so important. It really gets them tuned up for the full year.
Does the Hemophilia treatment Center also follow children diagnosed through adulthood?
Yes, actually our center has three hematologists; including myself, a pediatric hematologist plus two adult hematologists, and the nice thing about our center is that the nursing component and the social service component of our center are for children and adults, too. So the transition from childhood to adulthood is fairly smooth for our patients, so therefore we provide care for patients from birth to the end of their lives.
Is the University of Iowa Children's Hospital involved in any studies with regard to hemophilia?
We have approximately 15 to 20 clinical trials open with new products. Since the advances in technology in hemophilia are so rapid, we have available clinical trials for our patients so they can try different, new medications or different alternative treatments in order to treat their hemophilia.
If someone wanted to learn more about the Hemophilia Treatment Center, whom would they contact?
There are a couple resources. The National Hemophilia Foundation is probably one of the best resources for people that want to learn more about hemophilia,. To contact our hemophilia treatment center directly, call 800-272-3547. |
