Hemophilia in Austria – The Annual Survey of the Austrian Hemophilia Centers

The Annual Survey of the Austrian Hemophilia Centers

H.K. Hartl, P. Bayer, U. Eidher, P. Arends, J. Falger, N.D. Jones, M. Kronawetter, P. Kurnik, I. Pabinger-Fasching, H. Ramschak, E. Reiter, R. Schwarz, W. Streif, H. Türk, H. Wank, W. Zenz and K. Zwiauer

Patients and Methods

The Institute of Social Medicine of Vienna organizes the annual survey of the Hemophilia Treatment Centers (HTC) in Austria. Anonymous questionnaires are collected by the heads, or staff, of the HTC, on the basis of the data they provided the year before.

The analysis 2003 shows the distribution of the patients within the Austrian counties in respect to the place where they live, the number of patients according to the severity of the disease and the distribution according to age. Further the type of the disease and the number of HIV-infections among Austria’s hemophilia patients are analyzed.

Results

We received evaluable data from 359 patients with a mean age of 29 (+/– 20) in a range between 1 and 91 years (Table 1).

23.4% of the PwH are integrated in the working process, 35.9% are children and 9.5% are retired (Table 2).

265 (73.8%) patients suffer from hemophilia A, 48 (13.4 %) from hemophilia B, and 18 (5.8%) are reported inhibitor-patients (Table 3).

30 have a diagnosis of von-Willebrand-Syndrome (8.4%) and 16 (4.5%) other blee- ding disorders (Table 4). 260 (72.4) are in HTC-care and 92 (25.6%) patients are lost to follow up.

I. Scharrer/W. Schramm (Ed.)

34

Hemophilia Symposium Hamburg 2003

” Springer-Verlag Berlin Heidelberg 2005

Table 1. Age

frequency % valid % cumulated %

valid 0 - 14 yrs 102 28.4 28.7 28.7

15 - 29 yrs 102 28.4 28.7 57.3

30 - 54 yrs 107 29.8 30.1 87.4

55 yrs + 45 12.5 12.6 100.0

total 356 99.2 100.0

missing system 3 0.8

total 359 100.0

The relation of patients with mild hemophilia to patients with the severe form of hemophilia is similar (Table 5), the relation of hemophilia A to hemophilia B is according to the literature.

The distribution of the patients according to age and severity of the disease is also balanced, as expected, but there is a significantly fewer number of patients in the age cohort of older than 55 years (Table 6), which is explained by the HIV-infection.

Table 2. Profession

frequency % valid % cumulated %

valid blue collar worker 9 2.5 3.0 3.0

white collar worker 58 16.2 19.1 22.1

self-employee 8 2.2 2.6 24.8

official 9 2.5 3.0 27.7

pensioner 34 9.5 11.2 38.9

others 56 15.6 18.5 57.4

(school-) children 129 35.9 42.6 100.0

total 303 84.4 100.0

missing system 56 15.6

total 359 100.0

Table 3. Inhibitor

age group

0-14 years 15-29 years 30-54 years 55 years + total

inhibitor yes number 5 5 5 3 18

% 5.7 5.4 5.2 8.3 5.8

no number 82 87 91 33 293

% 94.3 94.6 94.8 91.7 94.2

total number 87 92 96 36 31

% 100.0 100.0 100.0 100.0 100.0

Table 4. Hemophilia Type

frequency % valid % cumulated %

valid hemophilia A 265 73.8 73.8 73.8

hemophilia B 48 13.4 13.4 87.2

von-Willebrand-Syndrome 30 8.4 8.4 95.5

other bleeding orders 16 4.5 4.5 100.0

total 359 100.0 100.0

104 out of 274 patients are diagnosed HCV-antibody positive. The majority is found in the age cohort of 30 to 54 years (Table 7). 31 patients out of 275 are dia- gnosed HIV-antibody positive, 6 out of them developed AIDS (Table 8), the majo- rity again found in the group of 30 to 54 years old.

But the data of the Austrian HIV-/AIDS-Fund (Fig. 1) are very different, in 2003 there were 58 PwH recognized recipients of benefit.

Table 5. Severity

frequency % valid % cumulated %

valid mild 150 41.8 46.9 46.9

moderate 31 8.6 9.7 56.6

severe 139 38.7 43.4 100.0

total 320 89.1 100.0

missing system 39 10.9

total 359 100.0

Table 6. Severity / Age

age group

0-14 years 15-29 years 30-54 years 55 years + total

severity mild number 37 40 44 27 148

% 38.9 43.5 46.3 77.1 46.7

moderate number 23 3 4 1 31

% 24.2 3.3 4.2 2.9 9.8

severe number 35 49 47 7 138

% 36.8 53.3 49.5 20.0 43.5

total number 95 92 95 35 317

% 100.0 100.0 100.0 100.0 100.0

Table 7. Hepatitis C

age group

0-14 years 15-29 years 30-54 years 55 years + total

Hepatitis C yes number 2 22 61 19 104

% 2.2 30.1 73.5 70.4 38.0

no number 89 51 22 8 170

% 97.8 69.9 26.5 29.6 62.0

total number 91 73 83 27 274

% 100.0 100.0 100.0 100.0 100.0

Discussion

The annual survey on Hemophilia in Austria tries to give a representative overview.

There is no significant change within the Austrian hemophilia population over the last years detectable. Over the last years we found a slight increase in average age and a reduction of the percentage of iatrogenic virus-infected patients due to safe clotting factors. HAART reduced the mortality rates of HIV-infected patients and the HCV-antibody positive patients benefit from new antiviral therapies.

Table 8. HIV / AIDS

age group

0-14 years 15-29 years 30-54 years 55 years + total

HIV pos. yes number 9 20 2 31

% 11.5 23.0 7.7 11.3

no number 84 69 67 24 244

% 100.0 88.5 77.0 92.3 88.7

total number 84 78 87 26 275

% 100.0 100.0 100.0 100.0 100.0

age group

0-14 years 15-29 years 30-54 years 55 years + total

AIDS yes number 2 3 1 6

% 2.6 3.4 3.7 2.1

no number 92 74 84 26 276

% 100.0 97.4 96.6 96.3 97.9

total number 92 76 87 27 282

% 100.0 100.0 100.0 100.0 100.0

70 60 50 40 30 20 10 0

17 17 16

4 4 4

63 60 58

hemophiliacs orphans other

2001 2002 2003

Fig. 1. HIV-Fund

For the year 2004 we have created an online questionnaire and included questi- ons on bleeding frequencies, treatment regimen and target joints, as well as other bleeding disorders as von-Willebrand-disease.

We know that the data presented is partly biased, but surveys like these are

depending on the collaboration of HTCs and the engagement of the caregivers. We

hope to encourage our contributors in simplifying the participation by technical

means even more, especially as we have to face the difficulties of financing health

care and need to antagonize and to arrive with valuable conclusions to perpetuate

the state-of-the-art-care for our patients.