Patients Adhering to Hemophilia Treatments Have Better Outcomes

Patients who better understand their condition and the necessity of treatment are more likely to experience positive outcomes, says Hemophilia News Today. This is because increased awareness and education correlates to higher treatment adherence. Find the researchers’ full study in Patient Preference and Adherence.


Hemophilia is a rare inherited bleeding disorder in which someone’s blood does not clot normally. Generally, when someone is injured, the blood cells clump together. Clots form, stopping the bleeding and closing the wound. However, people with hemophilia don’t have enough blood clotting factors. So the blood may not clot normally, causing prolonged bleeding or other complications.

There are two many forms of hemophilia: A (problem with clotting factor VIII) and B (problem with clotting factor IX). Hemophilia most commonly occurs in males; it is rare for women to have hemophilia, although they can be genetic carriers.

Symptoms include large bruises, excessive and consistent bleeding from injuries or medical procedures, irritability, blood in the stool and urine, and joint pain and swelling.

However, additional symptoms are considered severe and serious. These include vomiting, neck pain, double vision, fatigue, an intense headache, sudden joint swelling or warmth, and excessive bleeding. If someone with hemophilia experiences these symptoms, they should see a medical provider as soon as possible. Find out more about hemophilia. 

Haemo-Adhaesione Questionnaire

Adherence, in medicine, means sticking with and following a treatment plan for one’s condition. Patient adherence assists with heightening quality of life, educating patients on their conditions, and reducing symptoms or adverse reactions. However, patients with hemophilia tend to have less than 50% adherence.

Researchers wanted to understand why adherence was so low in these patients. Normally, doctors use questionnaires to understand the elements of adherence: patient understanding about treatments, patient attitudes, doctor-patient communication, and how much a patient participates in decision-making regarding their treatments. However, researchers determined that these questionnaires were not comprehensive enough.

So, the Haemo-Adhaesione questionnaire was formed. This questionnaire covers the following. Any questions below are just suggestions or ideas of what may be present on the questionnaire. However, these questions are not sourced directly:

  • Compliance: is a patient following medical guidelines? Is a patient complying with treatment? If so, how often?
  • Awareness: how much does the patient know about their disease? Do they recognize symptoms? Do patients understand what type of disease management is necessary? Are patients aware of what consequences and complications occur without treatment?
  • Doctor-patient communication: how are patients and doctors communicate? How often are they communicating? What information is being shared? Do patients feel comfortable with their medical professionals?

The questionnaire also discusses types of treatment, preventative measures, and elements of hemophilia.

Questionnaire Development

To build Haemo-Adhaesione, researchers examined medical literature and data on hemophilia. They found that greater adherence is linked to:

  • Treatment difficulties
  • The relationship between doctor and patient
  • Knowledge of hemophilia-related complications
  • Disease awareness (includes things like medication dosage)
  • Bleeding treatment processes

Hemophilia Adherence: A Study

Researchers provided the questionnaire to 146 patients with mild to severe hemophilia. Patient ages ranged between 15 and 64. 82% of patients (120) had hemophilia type A, while 18% of patients (26) had hemophilia type B. 57.5% of patients (84) were receiving regular prophylaxis treatment (prevention and management), and 42.5% of patients (62) were given on-demand treatment.

They found that patients using regular prophylaxis were more aware of their condition, its symptoms, its complications, and its treatments. Thus, greater disease awareness correlated with higher adherence.

However, patients with on-demand treatment also showed a great deal of adherence. This is because the treatment is designed to work during bleeding events. Thus, patients felt that they needed to protect themselves in case such an event popped up. So, they were also more likely to adhere to treatment.

Patients with a family history of hemophilia, or mild symptoms, were less likely to adhere to treatment. This is because their experience with the disease, or displayed symptoms, made them less concerned. However, a family history of hemophilia and mild symptoms correlated with greater treatment difficulties.

Jessica Lynn

Jessica Lynn

Jessica Lynn has an educational background in writing and marketing. She firmly believes in the power of writing in amplifying voices, and looks forward to doing so for the rare disease community.

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