Hemophilia and Living with It

A total of 41 respondents in diverse age groups participated in the survey: the average age of respondents was 25 years, with the youngest being 5 and the oldest 65. Males significantly predominate among respondents (see Graph 1) - their 97.56% participation confirms the strong predominance of male hemophilia patients, clearly due to the gonosomal recessive inheritance of this disease.

In the questionnaire, we focused on awareness of specialized centers for hemophilia patients, use of services provided by these centers, the extent of friendly relationships among individual patients and their mutual assistance, and various other relationships.

Of the respondents, 90.2% know about specialized centers for hemophilia patients, with more than half of all respondents (54%) using the center's services more than once a month and 83.7% at least once every six months. This led us to the question of whether using these centers helps hemophilia patients cope with their condition. We found that higher center usage highly correlates with coping with the disease.[1] Each increase in usage (using a linear regression model (LRM), see Graph 2) increases coping with the disease by 21% (highly statistically significant). People whom the centers helped cope with their condition use centers extensively, at 90%; in contrast, people whom the centers did not help in this regard use centers only at 57%, which is a statistically significant difference. A possible recommendation for patients who cope poorly with their condition is to suggest they visit these centers more frequently and try to make greater use of the services they offer.

One of the other questions asked was whether hemophilia patients take advantage of the opportunity to socialize with other hemophilia patients through social events organized by these centers. A total of 34% responded that they do so regularly, another nearly 27% have participated several times, and the rest do not take advantage of this opportunity. We found that more than half of hemophilia patients (58%) help each other in everyday life, and 41% of respondents have found new friends through hemophilia centers with whom they meet regularly. Young hemophilia patients under 25 also help each other more than older ones. On average, 37.5% of young patients help each other, compared to only 17.5% of older ones; unfortunately, this result is not very statistically significant, but it should be taken into account that our sample is relatively small and undoubtedly with a larger sample, not only this but also other results would be more statistically significant.

We further examined whether the opportunity to socialize with other hemophilia patients correlates with their opinion that the centers helped them cope with hemophilia. We found that this relationship is strongly correlated, meaning that those who socialize more with other hemophilia patients believe that the centers helped them more in coping with the disease. More precisely, a one-point increase in usage (out of 5 possible, from "does not use" to "uses at least once a week") means a statistically significant 31% increase in coping with the disease (based on LRM, see Graph 3). This implies that mutual understanding among people with hemophilia and their cooperation greatly helps in coming to terms with this disease.

In the survey, we were also interested in the extent to which hemophilia patients perceive their condition as a handicap. More than half of them are limited by hemophilia (63%) - of whom half are very limited and half are limited but have managed to adapt. The rest do not perceive the disease as a handicap when following certain rules, and 10% of respondents experience no limitations (see Graph 4). When asked what hemophilia limits them in the most, the vast majority of answers mentioned sports or impaired mobility. An interesting result is also that only 21% of all respondents are limited by hemophilia in their romantic relationships, meaning 79% do not experience any limitations in this regard. The perception of hemophilia as a handicap was also positively correlated with the clinical form of hemophilia. Using LRM, we confirmed that the more severe the form of hemophilia the respondent suffers from, the more limitations they experience in their life.

Following up on the negative perception of hemophilia in their lives, we also asked whether hemophilia had brought anything positive into patients' lives, and more than half of them (54%) responded positively to this question. In their text responses, they particularly valued their overall outlook on life, mainly that they appreciate their lives more and are perhaps more disciplined. It is interesting that from the group of people who found new friends through the centers, 77% claim that hemophilia brought something positive into their lives (see Graph 5). Thus, new friendships through the centers bring certain positive influences into the lives of hemophilia patients.

We were also interested in whether hemophilia patients had ever been in a life-threatening situation and whether they know first aid methods for hemophilia patients. Of the respondents, 98% know first aid methods, and 39% of all have at some point been in a life-threatening situation. Life-threatening situations correlate with the age of hemophilia patients, meaning the older the patient, the more frequently they encounter life-threatening situations. With each year, the probability increases by 1.1%, which may seem like a small number at first glance, but comparing a 20-year-old and a 60-year-old patient yields a significant difference of 44%.

Through this survey, we tried to gain insight into the life of an ordinary hemophilia patient and learn a bit more about mutual cooperation among those affected. The Czech Hemophilia Society and Hemojunior, who cooperated with us throughout the survey, organize a number of events that hemophilia patients can take advantage of, whether bowling tournaments or children's camps not only for children with hemophilia. We concluded that in such life situations, mutual help plays a significant role, that sharing information either in person or via the internet is very useful, and that even with a condition like hemophilia, one can live a happy and joyful life. We thank all participating hemophilia patients who devoted their time to our questionnaire, Veronika Cepelakova of Hemojunior, whose helpfulness and effort in obtaining the necessary data was very helpful, and last but not least Martin Bohun of the Czech Hemophilia Society, who very willingly helped us promote the online questionnaire through the Czech Hemophilia Society website.

The questionnaire was part of a project for the Medical Biology II course, Department of Biology and Medical Genetics, 2nd Faculty of Medicine, Charles University.

Gabriela Mlcochova, Barbora Ovesna, Simona Frydrychova (second-year students of the 2nd Faculty of Medicine, Charles University), Tomas Mlcoch (fifth-year student, Faculty of Finance, University of Economics) ________________________________________

[1] The correlation coefficient is 0.58.