Psychiatric disorders in hemophilic patients

Fakhari A.1, Dolatkhah R.2

Razi Hospital,Tabriz University of Medical Science

Abstract:

Hemophilia is a life threatening, life long condition caused by absence of or defective coagulation factors. Hemophiliac patients usually are under chronic stresses and consequently suffer from different psychological problems. Anxiety for the risk of bleeding causes stress and difficulty in coping with the situation. Aim of this study was to evaluate hemophiliac patients regarding possible psychiatric disorders.

This study was carried out in Shahid Ghazi Tabatabai Hematology& Oncology Center in Tabriz. Fourty eight hemophiliac patients were selected randomly and were evaluated possible psychopathology with SCL-90 questionnaire .The control group consist of 40 healthy personnels working in Shahid Ghazi Hospital.

Prevalence of various psychopathologic problems among hemophiliac patients was significant. Sixty seven percent of them were anxious,60% were depressed,60% showed aggressive characteristics,72% had obsessive-compulsive traits and 71%had psychiatric features while 52% showed somatization characteristics. Findings also revealed that occurance of similar disorders among control group were three to four times lower than hemophiliac patients.

The study showed that a high percentage of hemophiliac patients suffer from psychiatric problems and are in need to psychological and social support and proper drug treatments.

 

Keywords: Hemophilia, Stress, Social support

Introduction:

Heamophilia is a hereditary bleeding disorder that is due to defective and/or deficient coagulative factors. The clinical manifestations of haemophilia A and B due to deficiency of factor VIII and IX respectively are clinically indistinguishable and occur in mild, moderate and severe forms (1).

Although intra-articular and intramuscular bleeds are the most common and characteristic clinical symptoms of severe and moderate haemophiliacs, they are rarely observed before the child enters his ambulatory years. In fact, the most common presenting manifestations of haemophilia A and B are soft tissue bleeds (41%), bleeding associated with intramuscular injections and surgery (16%) and/or bleeding from tongue or lip bitting(11%)(2).

The severe forms of haemophilia are characterized by frequent hemarthrosis leading to chronic crippling hemarthropathy,when not treated very early or prophylactically. Highly purified concentrates, prepared from human plasma or manufactured by recombinant technology are available for treatment (1).

Unfortunately, all of the problems of the haemophiliacs have not been solved by the availability of concentrated factor products. Patients still are faced with the crippling effects of arthritis, problems with employment, problems with ignorance(both medical and lay),and increased risk of premature death even in a sophisticated, treatment- oriented community(3).

Suicide is frequent in haemophilia.This is related partly due to the high financial cost of haemophilia, a poor self-image that results from crippling hemarthropathy,life-long chronic pain and abuse of narcotic pain medications and family psychodynamics(4).

Because of chronic joint disease, the haemophiliac is often unable to work (4), go to school, do their daily activities and these make socio-economic problems for them. In addition, in developing countries such as Iran there are only a few heamophilia clinics in the big cities .Patients from small cities have to travel  long to reach  heamophilia centers, which is one of the sever stresses for them.

In relation to the clinical manifestation and the associated conditions, Quality of Life (QoL) in children and adolescents with bleeding disorders and their families is a relatively a new topic. It is important to understand more about QoL in this patient population to evaluate and if necessary, to improve the care patients receive. It can be explained by clinical and psychosocial factors (5).A haemophilia-specific health Quality-of-Life questionnaire (named ÒHemophilia-QoLÓ) was developed to assess QoL in adults with haemophilia(6).

This study has been done with the aim of evaluating psychiatric disorders in the patients, who were referred to haemophilia clinic in Shahid Ghazi Tabatabai Hematology&Oncology,Tabriz,Iran,which can have a direct relationship with various stresses that these patients faced with.

Materials and Methods:

Haemophilic patients are referred to Shahid Ghazi Haemophilic Clinic for supportive special cares. Of these patients, 48 cases were selected randomly and evaluated by SCL-90-R questionnaire. This questionnaire is a list of problems that people have (e.g.headaches,pains in various organs, poor appetite,É)and the individuals were asked to fill the questions in the form of grading 0 to 4 (0=not all;1=a little bit;2=moderately;3=quite a bit;4=extremely) which best describes how much that problem has bothered or distressed them during the past 4 weeks including the day of addressing to the questionnaire (7).

Forty cases including hospital staffs and other healthy people were enrolled in the study as control group and evaluated by the same questionnaire. The cases possessing the enough number for psychological consideration, on the base of DSM-IV criteria, were clinically interviewed by psychiatrist and evaluated for psychological problems. The informations were analyzed using SPSS software, with StudentÕs t-Test and Frequency Test, and the results were evaluated.

Results:

The results showed a virtually difference in depression, anxiety, interpersonality sensitivity, somatization, and obsessive compulsive traits between haemophiliacs and control group

figure1
Fig1.Psychiatric traits in Haemophilic and control groups


Also the rate of haemophilia was found low in women (5 women to 43 men) as composed to men; however there was not any difference between women and men in severity of psychiatric disorders. Furthermore, there was no significant difference in relation of age with the prevalence of psychiatric disorders in haemophiliacs and control group.Nevertheless, an excess significant difference in anxiety and depression traits between two groups (P=0.004). The severe and moderate depression and anxiety signs in each group have been shown by different number in Fig 2 and 3.

Fig 2: Depression trait in haemophiliacs and control group

figure2

Fig 3: Anxiety trait in haemophiliacs and control group

figure3

These differences were completely significant in aggressive, paranoia, somatization, interpersonal sensitivity and obsessive compulsive traits too (P=0.003). Phobia had   no difference in two groups and as compared with the normal people, had low range (Table 1)

 

Table 1 . Severe and Moderate signs of psychiatric traits in haemophilic and control groups.

 

           Traits

      Severe Signs

    Moderate Signs

control

hemophilic

control

hemophilic

Anxiety

   14.6

   43.75

    4.87

   20.8

Somatization

  7.31

    22.9

    14.6

   41.66

Obsessive-compulsive

   4.87

   31.25

   31.75

    45.8

Interpersonal sensitivity

   2.43

      27

    19.5

    47.9

Depression

   12.1

    39.58

    21.9

    35.4

Aggression

      -

       -

    4.87

    47.9

Phobia

      -

      4.1

    2.43

    12.5

Paranoia

      -

      4.1

    14.6

    35.4

 

In the last, after assessment of haemophiliacs with SCL-90-R questionnaire, 33 cases (68.75%) with probable distinction of psychiatric disorder, were clinically evaluated and interviewed on the basis of DSM-IV criteria by psychiatrist. Of them, 8 cases (20%) were found suffering from psychiatric disorders.

Among evaluated patients who had been interviewed ,12 cases (37.5%) were found suffering from adjustment disorders associated  with depressive mood,10 cases(20.8%)from adjustment disorders with anxiety,18 cases (37.5%) from major depressive disorders and 5 cases(10.4%) from various types of anxiety. In the control group who being referred to psychiatrist with the probable distinction of psychiatric disorder, were clinically evaluated and interviewed on the basis of DSM-IV criteria. Of these, those possess the distinctions were as follows:3 cases(7.5%) with major depressive disorders,2 cases (5%) of adjustment disorder with depressive mood ,2 cases (5%) of adjustment disorder with anxiety and 3 cases (7.5%) of to other types of psychiatric disorders.

Discussion:

The main difference in the prevalence of all of the psychiatric disorder traits and their severity frequencies in the haemophilic patients and also the high percentage of psychiatric disorders that had been confirmed with standard interviewing indicated that haemophiliacs exposed to very severe and different stresses.

As the references haemophiliacs suffer from low self-esteem, poverty, drug dependency and depression (8).On the other hand as a case-control study in Italy, the results showed that the haemophiliacs have a good psychological adaptation to their disease with the exception of their greater tendency to have less self-esteem than do the healthy subjects. This information could indicate the enormous importance that the psychological variable Òself-esteemÓ plays in haemophiliacs with respect to whether or not they develop depression disorders and/or anxiety states (9).These findings confirm the results from our study, as the prevalent psychiatric problems that were diagnosed by SCL-90-R questionnaire were depression (37.5%) and the different types of anxiety (10.4%).

Furthermore, in the present study a virtual difference was found in interpersonal sensitivity and social functioning between the haemophiliacs and control group that is consistent with previous studies. In a study from Cincinati performed in haemophiliac children, social,emotional and behavioral functions examined and reported that hemophiliac children present with more difficulties with emotional well-being ,including more depressive symptomatology and lower self-perception(10).

A study from Australia investigated psychosocial issues facing young people living with a chronic medical condition. Young people (n=35) were interviewed and it was found that many young people with chronic illness appear relatively resilient in the face of the adjustment challenges present by their illness (11)

A paper from Department of psychology of London reports cross- sectional findings from a national survey of patients with severe haemophilia type A and type B. Over one-third of participants expressed concerns about their drug use, the most frequent of which were about dependence on prescribed analgesics. Pain frequency was the most important predictor of analgesic use (12) and it was the problem in our haemophilic patients too,as about 23% of severe haemophilic patients were drug users, and the more stressful problem of the patients was coping with the pain, especially negative thoughts about pain, that associated with concern about drug use dependency.

Although this study well showed a virtual high rate of the various types of psychiatric disorders in haemophilic patients than normal people, widespread and exactly etiological studies may need to be accomplished.

Child coping, parent coping, social support, adaptive functioning treatment compliance, and illness severity must be considered to better understand and influence overall psychological functioning (13).

Despite the advances in haemophilic treatment and the future potential on the horizon,80% of the haemophiliacs around the world donÕt receive any treatment at all(14).The World Federation of Haemophilia must attempt to tackle these problems,haemophilia assistance programs must be activated in states and comprehensive haemophilic clinics to achieve these aims must be developed to deal partly with these complicated issues.

Furthermore, it seems that the psychiatric disorders of haemophilic patients are forgotten behind the routine haemophilic patients cares, so that it is proposed that attention should be paid to psychiatric disorders and their care with other routine haemophilic patients cares should be paid more and more.

 

References

 

1. Beutler E , Lichtman MA, Coller BA, Kipps TJ, Seligsohtt U. Williams Hematology Sixth edition 2001 ,Chapter123, 1639-1657

2. Benz EJ, Jr., shattil SJ, Furie B, Cohen HJ, Silberstein LE, Mc Glave P,  Hematology,Basic Principle and Practice ,Ronald Hoffman,3rd Edition 2000, Chapter 109,1883-1904

3. Breckenridge RT, Blood-its derivatives and its problems-factor VIII. Ann NY Acad Sci, 1975 Jan 20; 240:165-71

4. Handin RI, Lux SE, Stossel TP, BLOOD, Principle and Practice of Hematology, Second Edition 2002, chapter38, 1195-1218

5. Bullinger M, Von Mackensen S;Haemo-QoL Group,Quality of life in children and families with bleeding disorders.J Pediatr Hematol Oncol 2003 Dec;25(1):64-7

6.Arranz P,Remor E,Quintana M,Villar A,Diaz JL, Moreno M,etal ,Development of a new disease-specific quality-of-life questionnaire to adults living with haemophilia. Haemophilia 2004 Jul; 10(4):376-82

7. Derogatis L.R., Lipman R.S, Covi L, Symptom Check List SCL 90 ,Psychiatric University Hospital Zurich ,Research Department,1999

8.Rodak,Hematology Clinical Principle and Applications,second edition,Bernadette F.Rodak,MS,CLSpH(NCA),MT(ASCP)SH,2002,W.B.Saunders company,chapter43,632-642.

9.Canclini M,Saviolo-Negrin N,Zanon E,Bertoletti R,Girolami A,Pagnan A.Psychological aspects and coping in haemophilic patients:a case-control study.Haemophilia 2003 Sep;9(5):619-24

10. Trzepacz AM,Vannata K,Davies WH, Stehbens JA, Noll RB. Social,emotional, and behavioral functioning of children with hemophilia. J Dev Behav Pediatr 2003 Aug; 24(4):225-32

11. Olsson CA, Bond L, Johnson MW, Forer DL, Boyce MF, Sawyer SM. Adolescent chronic illness: a qualitative study of psychosocial adjustment . Ann Acad Med Singapore.2003 Jan; 32(1):43-50

12. Elander J, Barry T. Analgesic use and pain coping among patients with haemophilia.

Haemophilia 2003 Mar; 9(2):202-13

13. Casey RL, Brown RT. Psychological aspects of hematologic disease. Child Adolesc Psychiatr Clin N Am.2003 Jul; 12(3):567-84

14. Ghosh K. Management of haemophilia and its complications in developing countries.

Clin Lab Haematol 2004 Aug; 26(4):243-51


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