Квалитет на живот кај возрасни со хемофилија третирани со и без профилакса

Abstract

Background: According the definition of World Federation of Hemophilia, Hemophilia is an X-linked congenital bleeding disorder caused by a deficiency of coagulation factor VIII (FVIII) (in hemophilia A) or factor IX (FIX) (in hemophilia B). The deficiency is the result of mutations of the respective clotting factor genes. Analogy to definition for heath of World Health Organization, quality of life is defined as patient-perceived wellbeing and function in terms of physical, emotional, mental, social and behavioral life domains. Aim: Analysis of Quality of Life in registered adults with Hemophilia, those who are on prophylaxis and those who are not on prophylaxis. Material and methods: This is a clinical study for quality of life, in the same time prospective, observational and analytical cross sectional study. Sample was gathered from 140 adult people with Hemophilia. Inclusive criteria were: diagnosed people with Hemophilia and age above 18 years. Exclusion criteria were: diagnosed persons with other bleeding disorders and age under 18 years. Instrument for this survey was Questionnaire for quality of life - (SF-36). Registered people with Hemophilia were invited for interview and regular check up. In Center for Hemophilia and also in the Regional centers (RC) for transfusion medicine in Stip, RC Bitola, RC Tetovo, as well as in the Transfusion services (TS) in Kumanovo and Struga, clinical investigations were performed: laboratory check up and interview with psychologist. Regular check up with orthopaedic surgeon and physiatrist were scheduled. Data gathered from the research was processed with the software SPSS version 22.0 (Windows, Chicago, IL, USA) and was presented with tables and graphs. Results and discusion: The major part of the participants in the sample were from the Polog region and Skopje region, 41 participants from each region, (29,29%). According to the severity of heamofilia, тhe most common was the group with mild form with 85 (60,7%). Regarding the type of prophylaxis, the participants were divided in two groups, as follows: a) receiving permanent prophylaxis and b) receiving intermittent prophylaxis. The positive response for receiving the permanent prophylaxis did not exclude the possibility for the person with haemofilia to declare about the receiving of intermittent prophylaxis. From total of 50 (100%) of the participants that received permanent prophylaxis, the reason for it was target joint in two thirds or 30 (60%), while in 10 (20%) were frequent bleeds and in 10 (20%) were other reasons. Analysis was made of the participants regarding their grade of quality of life. In the group of persons with Haemophilia who are receiving prophylaxis, most prevalent were the persons that stated that their health is "good", i.e. 35 persons (44,9%) and only 5 (6,4%) of the participants declared that they have "poor" health. In the group of people with Haemophilia who are not receiving prophylaxis, a total of 33 (53,2%) declared that they have "good" health and 2 (3,2%) declared that they have "poor" health. In the study, both groups of people with Haemophilia, (with and without prophylaxis) were also analyzed regarding the limitations during their performance of ten daily activities. For p>0,05 a significant association was found between the group the participants belong to and the limitation in the direction of significantly larger limitation in the group without prophylaxis during the following daily activities: running, lifting heavy objects, participating in demanding sports, moving a table, cleaning with vacuum cleaner, bowling, playing golf, lifting and carrying products, climbing more and less stairs, leaning, kneeling, bending, walking more than one kilometer, walking more than few blocks, walking more than one block, dressing and bathing. An analysis was made of the persons with Haemophilia (with or without prophylaxis) regarding the existence of certain problems at the work place or other daily activities in the last 4 weeks as a result of the physical health. With p<0,05, a significant association was found between the group participants belong to and the limitation in the direction of significantly worse condition in the group without prophylaxis due to: shorter working hours or everyday activities, accomplishing less then expected, limitation in any work or activity and difficulties in doing any work or activity. The participants that were treated with or without prophylaxis, were analyzed regarding the existence of certain problems at the work place and the other daily activities in the last 4 weeks as a result of the emotional health. For p>0,05, there is no significant association between the group the participants belong to and the given answer for shorter work hours or daily activities, performing less than expected and limitation in any work or activity. The participants in the trial were analyzed in the last 4 weeks regarding to which extent the physical health and emotional problems were limiting in the daily social activities with family, friends and neighbors. There is no significant association, p>0,05, between the group the participants belong to (with/without prophylaxis) and the answer to the question. Regarding the pain, the participants with Haemophilia from both groups were asked about the intensity of the bodily pain and whether in the last 4 weeks the pain has interfered with their daily activities. For p>0,05, there wasn't a significant association found between the groups the participants belong to and the intensity of the pain, and also the interference in accomplishing the daily activities because of the pain in the last 4 weeks. The participants were evaluated for emotions with six parts Likert scale of possible answers. For six questions of the block the larger average values in the scoring pointed to better emotional health. There is no significant difference, p>0,05, between the two groups regarding the average score for the questions about the feelings. The analysis according to the negative influence of the physical and emotional health to the social activities in the last four weeks has shown in the participants with i.e. without prophylaxis, for p>0,05, a significant statistical difference was not found between the two groups. There is no statistically significant difference, for p>0,05, between the two groups regarding the obtained average scores from the answer to the question about getting sick easier than other people. Conclusion: The study has identified the needs of adult people with Haemophilia and recommendations were given for corrective actions for improvement of the quality of life and its prolongation. Applicability of this study is in implementation of comprehensive National Registry for People with Haemophilia. The implementation is needed of a system (protocols) for regular periodical check-ups of people with Haemophilia and implementation of IT for registration, follow-up and treatment of people with Haemophilia in the Republic of North Macedonia with creation of a web-oriented and flexible software.