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Physical and psychological health in persons with deafblindness that is due to Usher syndrome Type II.

Research on persons with disabilities and their health conditions is challenging, because persons with disabilities differ in their health conditions and other characteristics, with great variability within a specific disorder. Although knowledge of disability and comorbidity is scarce and fragmented, some reports have indicated that people with disabilities have an increased risk of poor health (Iezzoni, 2009; Marge & Pasick, 1988; Verbrugge, Lepkowski, & Imanaka, 1989). In this article, the focus is on deafblindness and Usher syndrome Type II. Only a few studies have discussed the importance of addressing deafblindness and comorbidity (Millan et al., 2011; K. Moller, 2008; Ronnberg & Borg, 2001). Milkan et al. (2011) stressed the importance of a public health perspective on individuals with deafblindness and Usher syndrome. Comorbidity has an impact on an individual's everyday life situations. Some comorbidity, such as a combination of a visual and hearing impairment, affects an individual's management of health care, the promotion of good health, and the performance of best evidence-based practice. Usher syndrome has so far not been associated with additional disorders than hearing impairments, visual impairments, and balance.

Deafblindness is a heterogeneous disorder, and a person with this condition can have different degrees of hearing and vision loss, the combination of which may have an additional or synergistic mode of action (C. Moller, 2003). The Nordic definition of deafblindness was accepted in 2007: "Deafblindness is a distinct disability. Deafblindness is a combined vision and hearing disability. It limits activities of a person and restricts full participation in society to such a degree that society is required to facilitate specific services, environmental alterations and/or technology" (Nordens Valfardscenter, n.d.).

Deafblindness increases a person's vulnerability, since the lack of basic information occurs in the interaction between the person with deafblindness and the environment, which may result in isolation and social exclusion. The result is often constraints on daily activities and environmental risks, which can be difficult to identify for persons with deafblindness (K. Moller, 2008). An important aspect of the lack of basic information between the person with deafblindness and the environment is ontological security (Danermark & Moller, 2008). Furthermore, people with deafblindness face challenges of trust and predictability, since they largely rely on other people to receive information. Schneider (2006) found restrictions in participation in relation to the environment, often caused by the lack of information on the effects of being deafblind.

Few studies have reported psychosocial consequences of deafblindness, such as anxiety and depression (Brennan & Bally, 2007; Capella-McDonnall, 2005; Chia et al., 2006). Harada et al. (2008) found a considerably higher risk of depression and perceived poor health among men and women aged 65 and older with acquired nonsyndromic visual and hearing impairments. Although their target group was different from that of our study, it is interesting to note that their results indicated a considerably higher risk of poor health among persons with deafblindness than among persons without deafblindness. Being complementary, hearing and vision enhance each other (C. Moller, 2003). Early functional and medical diagnosis is crucial, since the lack of it may result in psychosocial implications and difficulties in coping and adapting (Miner, 1995, 1997; Schneider, 2006).

Usher syndrome (USH) is the most common cause of deafblindness in adults of working age worldwide (C. Moller, 2003). It is an autosomal recessive disorder that affects hearing, vision, and vestibular function (balance) (C. Moller, 2003; Pennings, 2004; Sadeghi, 2005). The prevalence of USH in Sweden is estimated at 3.3 per 100,000 people (Sadeghi, Kimberling, Tranebjoerg, & Moller, 2004). USH can be clinically divided into three types (I-III), and to date, 11 different genes have been detected. It has been suggested that these genes create an Usher protein network, an interactome, which means that different mutations may give rise to similar hearing and vision phenotypes (Kremer, van Wijk, Marker, Wolfrum, & Roepman, 2006). The hearing impairment is congenital and ranges from profound deafness to a moderate hearing loss, while the visual impairment is caused by a retinal disorder known as retinitis pigmentosa (RP) (Hartong, Berson, & Dryja, 2006), an umbrella term for different disorders that cause retinal degeneration. Cataracts often accompany RP. Retinal degeneration leads to contrast sensitivity, light sensitivity, night blindness, visual field limitations, and impaired visual acuity. The vestibular func tion is affected in Types I and III. USH Type I is characterized by congenital bilateral vestibular areflexia with severe balance problems and late walking age, while in USH Type III, the vestibular function deteriorates, leading to increasing problems with balance (Kimberling & Moller, 1995).

Usher syndrome Type II (USH2) is characterized by a moderate to severe congenital hearing loss that remains stable or progresses gradually. The visual limitations, which are usually detected in late adolescence, deteriorate progressively over the lifespan (Kimberling & Moller, 1995). USH2 is the most common form of Usher syndrome in most countries (Leijendeckers, Pennings, Snik, & Bosman, 2009).

Sadeghi et al. (2006) investigated the long-term visual prognosis for persons with USH1 and USH2 and found that progression was faster among men than among women. But no study has focused on differences in the health of men and women with USH2, which are addressed in this article. Thus, the identification of health indicators for people with USH2 will have implications in rehabilitation. The aims of the study were to describe the physical and psychological health of persons with USH2 and to explore any differences in terms of gender.

Method

PARTICIPANTS

The individuals with USH2 were recruited through the Swedish Usher database, which comprised 122 adults with USH2 living in Sweden. All 122 individuals with USH2 were asked to participate and received a questionnaire, Health on Equal Terms (Statens Folkhalsoinstitut, n.d.), and additional information by mail. The 96 persons who responded to the questionnaire were included in the study; hence the response rate was 79%. All the individuals signed informed consent forms to participate in clinical and genetic research on Usher syndrome. The Ethics Committee of Linkoping University Hospital, 1990, 1997, and the Institutional Review Board of the Boys Town National Research Hospital Omaha USA, 1990, 1997, approved the research. The second author clinically assessed most of the participants, and most have been examined several times. The register contains data on hearing, vision, balance, and genetics on approximately 70% of the estimated total population of individuals with USH2 in Sweden (Sadeghi et al., 2004).

The mean age of the USH2 group was 55 years (range: 18-84 years), and the female-to-male ratio was 53% and 47%. The participants had a clinical diagnosis of USH2 based on hearing, vision, family tree, and other clinical observations, and 59% had a genetic diagnosis of USH (see Table 1). The hearing and vision assessments were performed at approximately the same time as the Health on Equal Terms questionnaires were distributed. Vision and hearing assessments for the USH2 group were administered at audiological departments in the participants' home towns. Medical reports, including the assessments, were retrieved after authorization. The participants' hearing impairments were assessed by pure-tone audiometry with the calculation of the average pure tone for the frequencies 0.5, 1, 2, and 4 kHz (PTA4). Visual acuity was measured by Snellen chart-based standard tests and visual field tests (Goldman perimetry) categorized into five phenotypes (1-5), from 1 = "normal" or typical vision to 5 = blindness (Grover, Fishman, Anderson, Alexander, & Der lacki, 1997).

There were no differences between the participants with USH2 and the nonrespondents in terms of the results for mean age, genetic diagnosis, pure tone average, visual acuity, or visual field. A difference was found concerning gender, in that more men with USH2 belonged to the nonrespondents than to the participants (65% versus 47%).

The USH2 results from the Health on Equal Terms questionnaire were compared to those for a reference group comprised of 5,738 persons (Bostrom, 2008). The reference material for this group came from an extant database administered by the Swedish Public Health Institute and Statistics Sweden (2007). The reference population was identified by a simple random sample from the total population aged 16-84 years living in Sweden in 2007, and hence included individuals with and without impairments. The criteria for the sample were created from the Register for the Total Population. A simple random sample consisting of 10,000 individuals was drawn, and identification was controlled to receive updated addresses. The questionnaire was sent to the respondents by mail, and the respondents could choose to answer on paper or by a link on the Internet (Statistics Sweden, 2007). The survey has been conducted annually since 2004 by the Swedish National Institute of Public Health (Bostrom & Nyqvist, 2010).

INSTRUMENT

The Swedish Public Health survey (Health on Equal Terms) questionnaire was used. The questionnaire contains 75 questions that are divided into the following domains: health, living habits, tobacco, gambling, alcohol, financial situation, work or occupation, security, social relations or relationships, and demographic data (for example, gender and age). The questions in the questionnaire were compiled from surveys conducted by county councils in Sweden and from the Statistics Sweden Survey of Living Conditions. The questions were chosen and tested by a method group at Statistics Sweden and by a pilot study conducted in November 2003. The items in the questionnaire were validated for construct validity. Empirical validity is challenging to perform in instruments of a psychosocial nature, which includes many of the questions in the questionnaire (Bostrom & Nyqvist, 2010). In our study, we focused on 36 questions concerning physical and psychological health. The question for general health--"How do you rate your general health state?"--was to be answered on a 5-point graded scale from "very good" to "very poor." Three questions on healthy days and activities of daily living were asked, and the participants were to answer how many days during the previous 30-day period had their physical or psychological health been bad. For activities of daily living, the participants were asked how many days during the previous 30-day period their activities of daily living had been lowered. Questions related to physical health were formulated as follows: Vision: "Can you see and read standard text in a daily newspaper without difficulty?" Hearing: "Can you hear what is being said in a conversation among several people without difficulty?" The answers were graded on a 3-point scale: For vision: "Yes, without spectacles"; "Yes, with spectacles"; or "No," and for hearing: "Yes, without a hearing aid"; "Yes, with a hearing aid"; or No." Questions concerning diabetes, asthma, allergy, high blood pressure, shoulder or neck pain, headache, and fatigue, were graded from "No" to "Yes, but no distress" to "Yes, slight distress" to "Yes, great distress."

Psychological health was assessed by 12 questions about abilities during the previous few weeks, such as being able to concentrate, having feelings of worthlessness, and inability to appreciate the day. Questions related to psychological health were formulated as follows: "Have you been able to concentrate on all your activities in the past few weeks?" and "Have you felt worthless in the past few weeks?" Answers were given on a 4-point scale from "Not at all" to "Not more than usual" to "More than usual" to "Much more than usual." Two questions concerning suicidal behavior were also included, formulated as "Have you at any time found yourself in a situation in which you have seriously considered taking your own life?" and "Have you ever tried to take your own life?" Answers were graded as "No," "Yes, one time," and "Yes, several times."

Results

This section reports the results for the significantly different responses to the Health on Equal Terms questionnaire by the two groups--the participants with USH2 and the reference group. Comparisons are made both between the groups and within the groups. Further gender differences are analyzed.

Analyses were conducted using frequencies, cross tables, and logistic regression analysis. Answers to the items on physical health and psychological health were dichotomized as "no problem" and "problem," those on general health were dichotomized as "good health" and "bad health," and those on suicidal thoughts and suicide attempts were dichotomized as "no" and "yes." For the number of bad health days, a cutoff was made according to Bostrom and Nyqvist (2010): 15 days or more was considered bad physical or psychological health days. Frequencies are given, and the odds ratio (OR) and its 95% confidence interval (CI) were calculated, and the OR was adjusted for gender and age. The significant differences that were found are presented in tables, and the level of significance in all the tests was set at p [less than or equal to] .05.

The 18 items in the questionnaire related to physical health and the 18 related to psychological health were analyzed, and significant differences were found between the USH2 group and the reference group in most domains. The 15 items presented in Table 2 show significant differences between the USH2 group and the reference group. The participants with USH2 reported severe problems with tinnitus, headache, and pain in their shoulders and neck and had significantly worse cases of eczema and skin rashes. Psychologically poor health was significantly worse among those with USH2, including fatigue, inability to concentrate, being unable to accomplish things, feelings of worthlessness, and feelings of being constantly under strain. The participants with USH2 reported unhappiness and depression, as well as anxiety, significantly more than did those in the reference group. Suicidal behavior was more evident in those with USH2 than in those in the reference group for both suicidal thoughts and suicide attempts (see Table 2).

Gender differences in physical and psychological health were found in the reference group, in which 30 of 36 indicators were significantly worse in the women; however, the men had significantly more problems with tinnitus, high blood pressure, and hearing loss (see Table 3).

The differences between the men and the women that were observed in the reference group were not found in the USH2 group, in which the men reported a higher occurrence of physical and psychological poor health. In 2 of the 36 health indicators, a significant difference in pain in the shoulders and neck (men 45%, women 73%, [beta] = 1.154, SE [beta] = 0.444, Wald = 6.758, df = 1, p = .009, OR = 3.172, CI 95% = 1.329-7.573) and incontinence (men 10%, women 26%, [beta] = -1.280, SE p = 0.628, Wald = 4.160, df = 1, p = .041, OR = 0.278, CI 95% = 0.081-0.951) was found between the men and women with USH2.

When comparing the women with USH2 and the women in the reference group, five questions pertaining to physical health and two questions concerning psychological health revealed significant differences in which the women with USH2 exhibited poorer health (see Table 4). Significant differences were also found between the men with USH2 and the men in the reference group in 15 out of 36 questions (see Table 5), 6 of which were related to poor physical health and 9 to poor psychological health.

Discussion

The aims of the study were to describe the physical and psychological health of individuals with USH2 and to explore any differences in terms of gender. The study demonstrated significant differences in physical and psychological health when the participants with USH2 were compared to persons in a Swedish reference group.

PHYSICAL AND PSYCHOLOGICAL HEALTH

Major problems with headache, fatigue, depression, suicidal thoughts, and suicide attempts were found among the participants with USH2 when compared to persons in a Swedish reference group. In the case of men with USH2, the psychological differences were even more marked than in the male reference group.

Several of the findings in Table 2 related to psychological items pertaining to the inability to accomplish things and to concentrate and feelings of unhappiness and depression. Miner (1997) stated that the impact of continued vision loss creates a situation in which self-esteem and self-image are attacked and compromised on a daily basis. The participants with USH2 reported a high degree of feelings of worthlessness, unhappiness, and depression (see Table 2), which was most evident in the men (see Table 5).

Progressive deterioration of vision means that persons with USH2 have to adjust constantly to new conditions and situations, which can result in psycholog ical stress (Miner, 1997; Schneider, 2006). A more rapid deterioration of vision in men with USH2 could be one reason for the men's poorer psychological health. The causes of self-reported poor health among men with USH2 need to be addressed not only from a physical (medical) perspective, but from a social perspective and gender differences and changes in identity over the life course because the progression of vision loss means the constant adjustment to new conditions. Hence, the exact nature of the causes of self-reported poor health and gender differences in those with USH2 requires further investigation.

Fatigue is described as a subjective, unpleasant symptom involving both physical sensations and feelings of tiredness and exhaustion, leading to a condition that can interfere with an individual's ability and capacity to perform activities of daily living (Ream & Richardson, 1996). Our study revealed that fatigue is a major problem for people with USH2 (see Tables 2, 4, and 5). It has been demonstrated that long-term fatigue can result in other disorders and diseases (Afari & Buchwald, 2003), and fatigue in combination with deafblindness may be synergistic. In general, women report more problems with fatigue than do men (Lewis & Wes sely, 1992). Both the women with USH2 and the women in the reference group expressed greater problems with fatigue than did the men in the reference group. However, the men with USH2 described more fatigue than did the women and men in the reference group. Since USH2 involves the gradual loss of vision combined with hearing loss, greater effort is required to adjust and to access information in daily life, which demands a lot of energy. In K. Moller's (2003) study, the respondents with deafblindness reported tiredness and headache as a consequence of their struggle to see and hear. Our study also found that headache was a major problem for the participants with USH2 (see Tables 2, 4, and 5).

Eczema and skin rashes were overrepresented in the participants with USH2 (see Table 2). Eczema has various explanations (Langan & Williams, 2006), not only allergy, but neurodermatitis and psychological factors. Chuh, Wong and Zawar (2006) stressed the importance of a holistic view on skin problems, including both physical and psychological factors. We do not know the etiology of eczema in people with USH2, and more research concerning the relationship between USH2 and the reported skin problems is needed.

A large proportion of the participants with USH2 stated that they had problems because of tinnitus (see Table 2), in common with other persons with hearing impairments (Axelsson & Ringdahl, 1989). Tinnitus is not only a physical phenomenon; it can also be triggered by psychological conditions or changes in psychological reactions, which may further aggravate an already existing tinnitus (Henry, Dennis, & Schechter, 2005).

According to ICD-10 (World Health Organization, 2010), depression is a state of sadness and loss of energy. Self--confidence and self-esteem are also affected, and the person may experience feelings of worthlessness. The participants with USH2 expressed feelings of both worthlessness and unhappiness or depression (see Table 2). The men with USH2 reported a higher level of these feelings than did the men in the reference group (see Table 5). This finding is in agreement with Miner (1997), who stated that depression and at times suicidal behavior are apparent in persons with USH2. The results of our study clearly demonstrate that people with USH2 are at risk of suicidal behavior (see Tables 2 and 5). For the men with USH2, the risk of attempted suicide was nearly six times as high as among the men in the reference group (see Table 5). Bremberg (2008) described suicide as the final step in a chain of events. According to Mann et al. (2005), suicide is a public health issue, and although psychiatric illness is a major contributory factor, it should be seen as a phenomenon influenced by many different factors. Having a hearing impairment and progressive visual impairment poses major challenges in adaptation and in finding strategies to deal with the situation.

The findings of this study call for further research. The men and the women with USH2 had a more similar pattern of physical and psychological health than did the men and the women in the reference group. At present, we do not know the role of the biological, psychological, and social mechanisms that are involved.

LIMITATIONS

One limitation of this study could be that health status was self-reported and that no objective health assessment was made in the public health survey or among the participants with USH2. Since this was the case for both the USH2 population and the reference group, however, it may not have influenced our ability to make comparisons between the two groups.

Furthermore, at first glance, the total number of participants with USH2 in our study may be considered low, but the total number of people with USH2 is small, and the participants with USH2 represented about 55% of the total population of known individuals with USH2 in Sweden. To the best of our knowledge, the number of participants in this study was by far the largest of those in studies of Usher syndrome. It should also be emphasized that the reference group was a random sample representative of the general population of Sweden; it included people with long-and short-term diseases and disabilities. It is reasonable to assume that the differences that were found would have been greater if the reference population had been skewed by the exclusion of persons with various health conditions.

Implications for future research This article has described the poor health status of people with USH2. Both the men and the women with USH2 had significantly poorer physical and psychological health than did those in the reference group. The largest differences were found between the men with USH2 and the men in the reference group, especially in the area of poor psychological health. The identification of factors that can improve physical and psychological health is important and will give rise to future rehabilitation efforts. Special attention must be devoted to the psychological well-being of men with USH2. The findings also call for more research on health-promoting factors.

IMPLICATIONS FOR PRACTITIONERS

The participants with USH2 reported that their physical and psychological health was significantly poorer compared to those in a Swedish reference group. The identification of factors associated with physical and psychological health and well-being is important for the design of future rehabilitation strategies for people with USH2. A special focus must be placed on the psychological well-being of men with USH2. The results showed the self-reported increased risk of comorbidity beyond the previously known physical visual and hearing impairments. Clinicians who are ignorant of this situation may focus only on vision or hearing. Since USH2 is a rare syndrome, the probability of clinicians in small clinics having patients with USH could be one or two in their entire professional lives. Therefore, the management of rehabilitation for persons with USH2 calls for centralization, access to information, and truly interdisciplinary teamwork. A team should also be aware of and have resources for handling poor physical and psychological health of people with USH.

The authors thank Camilla Johansson and Hanna Hagsten at the Audiological Research Centre for their help with the data collection. The Swedish Research Council, the Hearing Foundation, and the Swedish Association of Hard of Hearing People provided support for the research reported in this article.

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Moa Wahlqvist, B.S.W., doctoral student, Institute for Health and Medicine, Swedish Institute for Disability Research, Orebro University, S-701 82 Orebro, Sweden, and Audiological Research Centre, University Hospital, Orebro, Sweden; e-mail: <moa. wahlqvist@oru.se>.

Claes Moller, M.D., professor of medicine, Swedish Institute for Disability Research, Orebro University, and Audiological Research Centre, University Hospital Orebro, S-701 85 Orebro, Sweden; e-mail: <claes. moller@orebroll.se>. Kerstin Moller, M.D., doctor of medicine, Swedish Institute for Disability Research, Orebro University, and Audiological Research Centre, University Hospital Orebro, Sweden; e-mail: <kerstin.moller@oru.se>.

Berth Danermark, Ph.D., professor of sociology, Swedish Institute for Disability Research, Orebro University, and Audiological Research Centre, University Hospital Orebro, Sweden; e-mail: <berth. danermark@oru.se>.
Table 1
Background data for the USH2 and reference groups.

Variable                 USH2       Reference

Number                    96          5,738
Mean age (years)          55           58
Age range               18-84         23-91
Women (%)                 53           56
Clinical diagnosis        96
Genetic diagnosis      57 (59%)
PTA4 (best ear)       73 db mean
Visual acuity          0.4 mean
  (1.0-0.0)
Visual field (1-5)   4 mean (< 10
                       degrees)

Table 2
Frequencies and logistic regression analysis of significant
differences in poor physical and psychological health among
the participants with USH2 and the reference group.

Health status
                                  USH2        Reference
                              participants      group
Variable                        (N = 96)     (N = 5,738)

Poor physical health
   Severe hearing loss            42%            11%
   Tinnitus                       44%            19%
   Severe loss of vision          48%            4%
   Headache                       47%            26%
   Pain in the shoulders
     and neck                     60%            49%
   Eczema, skin rashes            29%            20%
Poor psychological health
   Fatigue                        77%            49%
   Inability to concentrate       21%            13%
   Unable to accomplish
     things                       17%            11%
   Feeling of worthlessness       13%            7%
   Constantly under strain        20%            12%
   Unhappy and depressed          20%            12%
   Anxiety, worry                 43%            31%
   Suicidal thoughts              21%            12%
   Suicide attempts                9%            4%

Health status                 Logistic regression analysis

Variable                      [beta]   SE [beta]     Wald     df

Poor physical health
   Severe hearing loss        1.747      0.218      64.216    1
   Tinnitus                    1.18      0.217      29.681    1
   Severe loss of vision       3.05      0.220     192.549    1
   Headache                   1.017      0.219      21.496    1
   Pain in the shoulders
     and neck                 0.439      0.218       4.07     1
   Eczema, skin rashes        0.474      0.235      4.065     1
Poor psychological health
   Fatigue                    1.278      0.252      25.772    1
   Inability to concentrate   0.644      0.264      5.959     1
   Unable to accomplish
     things                   0.565      0.279      4.108     1
   Feeling of worthlessness   0.737      0.316      5.426     1
   Constantly under strain    0.697      0.271      6.597     1
   Unhappy and depressed      0.684      0.269      6.436     1
   Anxiety, worry             0.539      0.214      6.331     1
   Suicidal thoughts          0.774      0.260      8.888     1
   Suicide attempts           0.980      0.379      6.678     1

Health status                 Logistic regression analysis
                                P
Variable                      value   OR (a)    CI 95% (b)

Poor physical health
   Severe hearing loss        .000    5.738     3.742-8.796
   Tinnitus                   .000    3.255     2.129-4.976
   Severe loss of vision      .000    21.111   13.723-32.479
   Headache                   .000    2.764     1.799-4.249
   Pain in the shoulders
     and neck                 .044    1.551     1.013-2.377
   Eczema, skin rashes        .044    1.606     1.013-2.544
Poor psychological health
   Fatigue                    .000    3.590     2.192-5.881
   Inability to concentrate   .015    1.904     1.135-3.194
   Unable to accomplish
     things                   .043    1.759     1.019-3.036
   Feeling of worthlessness   .020    2.090     1.124-3.885
   Constantly under strain    .010    2.008     1.180-3.419
   Unhappy and depressed      .011    1.981     1.168-3.360
   Anxiety, worry             .012    1.714      1.126-607
   Suicidal thoughts          .003    2.169     1.304-.607
   Suicide attempts           .010    2.664     1.267-5.600

p [less than or equal to] .05; (a) OR adjusted for gender and age;
(b) CI 95% for the OR.

Table 3
Frequencies and logistic regression analysis for significant
differences in poor physical and psychological health among the
men and women in the reference group.

                              Reference group
                                    Men            Women
Health status                   (n = 2,536)     (n = 3,202)

Poor general health                 7%              8%
   Bad physical health
     days [greater than             18%             21%
     or equal to] 15
   Poor psychological
     health days [greater           12%             19%
     than or equal to] 15
   Poor physical and
   psychological health
   prevented ADL [greater           11%             14%
   than or equal to] 15
Poor physical health
   Severe hearing loss              12%             10%
   Tinnitus                         23%             15%
   Headache                         17%             32%
   Pain in shoulders and
     neck                           39%             57%
   Back pain                        42%             50%
   Pain in extremities              38%             46%
   Eczema, skin rashes              17%             22%
   Incontinence                     8%              17%
   Bowel trouble                    19%             30%
   Obesity                          24%             31%
   Asthma                           9%              11%
   Allergy                          24%             32%
   High blood pressure              22%             19%
Poor psychological health
   Sleeping problems                26%             39%
   Lost sleep over worry            7%              13%
   Fatigue                          41%             55%
   Inability to concentrate         10%             15%
   Incapable of making
     decisions                      4%              6%
   Unable to manage
     problems                       8%              11%
   Unable to appreciate
     the day                        11%             13%
   Unable to face problems          7%              10%
   Feeling of worthlessness         5%              9%
   Feeling of lost
     confidence                     4%              8%
   Constantly under strain          8%              15%
   Reasonably happy                 9%              12%
   Unhappy and depressed            9%              14%
   Anxiety, worry                   22%             38%
   Suicidal thoughts                9%              14%
   Suicide attempts                 2%              5%

                              Logistic regression analysis

Health status                 [beta]    SE [beta]    Wald     df

Poor general health           -0.230      0.105      4.782     1
   Bad physical health
     days [greater than       -0.257      0.070     13.631     1
     or equal to] 15
   Poor psychological
     health days [greater     -0.476      0.077     38.686     1
     than or equal to] 15
   Poor physical and
   psychological health
   prevented ADL [greater     -0.316      0.083     14.672     1
   than or equal to] 15
Poor physical health
   Severe hearing loss         0.253      0.085      8.797     1
   Tinnitus                    0.514      0.069     55.522     1
   Headache                   -0.812      0.065     155.289    1
   Pain in shoulders and
     neck                     -0.749      0.055     186.982    1
   Back pain                  -0.354      0.055     41.143     1
   Pain in extremities        -0.406      0.057     51.494     1
   Eczema, skin rashes        -0.292      0.068     18.542     1
   Incontinence               -1.051      0.092     130.680    1
   Bowel trouble              -0.612      0.064     91.650     1
   Obesity                    -0.395      0.061     42.064     1
   Asthma                     -0.347      0.109     10.157     1
   Allergy                    -0.395      0.069     32.485     1
   High blood pressure        -0.247      0.104      5.605     1
Poor psychological health
   Sleeping problems          -0.650      0.059     120.590    1
   Lost sleep over worry      -0.659      0.096     46.857     1
   Fatigue                    -0.567      0.054     110.017    1
   Inability to concentrate   -0.433      0.083     27.415     1
   Incapable of making
     decisions                -0.370      0.122      9.191     1
   Unable to manage
     problems                 -0.301      0.093     10.359     1
   Unable to appreciate
     the day                  -0.168      0.083      4.068     1
   Unable to face problems    -0.430      0.100     18.660     1
   Feeling of worthlessness   -0.585      0.110     28.185     1
   Feeling of lost
     confidence               -0.672      0.119     31.798     1
   Constantly under strain    -0.614      0.088     48.259     1
   Reasonably happy           -0.387      0.090     18.471     1
   Unhappy and depressed      -0.499      0.087     32.986     1
   Anxiety, worry             -0.749      0.060     154.609    1
   Suicidal thoughts          -0.523      0.087     36.305     1
   Suicide attempts           -0.660      0.156     17.909     1

                              Logistic regression analysis
                                P
Health status                 value   OR (a)   CI 95% (b)

Poor general health           .029    0.795    0.647-0.976
   Bad physical health
     days [greater than       .000    0.773    0.675-0.886
     or equal to] 15
   Poor psychological
     health days [greater     .000    0.621    0.535-0.722
     than or equal to] 15
   Poor physical and
   psychological health
   prevented ADL [greater     .000    0.729    0.620-0.857
   than or equal to] 15
Poor physical health
   Severe hearing loss        .003    1.288    1.090-1.522
   Tinnitus                   .000    1.673    1.461-1.915
   Headache                   .000    0.444    0.391-0.504
   Pain in shoulders and
     neck                     .000    0.473    0.425-0.526
   Back pain                  .000    0.702    0.630-0.781
   Pain in extremities        .000    0.666    0.596-0.744
   Eczema, skin rashes        .000    0.747    0.654-0.853
   Incontinence               .000    0.350    0.292-0.419
   Bowel trouble              .000    0.542    0.478-0.614
   Obesity                    .000    0.674    0.598-0.759
   Asthma                     .001    0.707    0.571-0.875
   Allergy                    .000    0.674    0.588-0.772
   High blood pressure        .018    0.781    0.637-0.958
Poor psychological health
   Sleeping problems          .000    0.522    0.465-0.586
   Lost sleep over worry      .000    0.517    0.428-0.625
   Fatigue                    .000    0.567    0.510-0.631
   Inability to concentrate   .000    0.648    0.551-0.763
   Incapable of making
     decisions                .002    0.691    0.544-0.877
   Unable to manage
     problems                 .001     0.74    0.616-0.889
   Unable to appreciate
     the day                  .044    0.846    0.718-0.995
   Unable to face problems    .000    0.650    0.535-0.791
   Feeling of worthlessness   .000    0.557    0.449-0.691
   Feeling of lost
     confidence               .000    0.511    0.404-0.645
   Constantly under strain    .000    0.541    0.455-0.644
   Reasonably happy           .000    0.679    0.569-.810
   Unhappy and depressed      .000    0.607    0.512-.720
   Anxiety, worry             .000    0.473    0.420-.532
   Suicidal thoughts          .000    0.592    0.500-.702
   Suicide attempts           .000    0.517    0.381-.702

ADL = activities of daily living; p [less than or equal to] .05;
(a) OR adjusted for age; (b) CI 95% for the OR.

Table 4
Frequencies and logistic regression analysis for significant
differences in poor physical and psychological health, comparison
between women with USH2 and women in the reference group.

                              Women
                                        Women in the
                              Women      reference
                               USH2        group
Health status                (n = 51)   (n = 3,202)

Poor physical health
   Severe hearing loss         49%          10%
   Tinnitus                    43%          15%
   Severe loss of vision       39%           4%
   Headache                    54%          32%
   Pain shoulders, neck        73%          57%
Poor psychological health
   Fatigue                     84%          55%
   Constantly under strain     25%          15%

                             Logistic regression analysis

Health status                [beta]   SE [beta]    Wald    df

Poor physical health
   Severe hearing loss       2.145      0.288     55.487    1
   Tinnitus                  1.392      0.290     23.075    1
   Severe loss of vision     2.705      0.301     80.775    1
   Headache                  0.976      0.288     11.472    1
   Pain shoulders, neck      0.644      0.316     4.137     1
Poor psychological health
   Fatigue                   1.442      0.388     13.842    1
   Constantly under strain   0.774      0.340     5.199     1

                             Logistic regression analysis

                               P
Health status                value   OR (a)    CI 95% (b)

Poor physical health
   Severe hearing loss       .000    8.544    4.859-15.024
   Tinnitus                  .000    4.022    2.279-7.097
   Severe loss of vision     .000    14.958   8.292-26.983
   Headache                  .001    2.655    1.509-4.671
   Pain shoulders, neck      .042    1.903    1.023-3.539
Poor psychological health
   Fatigue                   .000    4.228    1.978-9.036
   Constantly under strain   .023    2.169    1.115-4.221

p [less than or equal to] .05; (a) OR adjusted for age;
(b) CI 95% for the OR.

Table 5
Frequencies and logistic regression analysis of significant
differences in poor physical and psychological health among
men with USH2 compared to men in the reference group.

                              Men
                                          Reference
                                USH2        group
Health status                 (n = 45)   (n = 2,536)

Poor physical health
   Bad physical health days
     [greater than or            9%          6%
     equal to] 15
   Tinnitus                     44%          23%
   Severe loss of vision        59%          4%
   Headache                     38%          17%
   Asthma                       20%          9%
Poor psychological health
   Fatigue                      69%          41%
   Incapable of making
     decisions                  12%          4%
   Unable to accomplish
     things                     21%          10%
   Feeling of worthlessness     14%          5%
   Reasonably happy             19%          9%
   Unhappy and depressed        21%          9%
   Anxiety, worry               37%          22%
   Suicidal thoughts            21%          9%
   Suicide attempts             12%          2%

                              Logistic regression analysis

Health status                 [beta]  SE [beta]    Wald     df

Poor physical health
   Bad physical health days
     [greater than or         0.768     0.344      4.987     1
     equal to] 15
   Tinnitus                   0.956     0.316      9.173     1
   Severe loss of vision      3.481     0.335     107.894    1
   Headache                   1.067     0.331     10.368     1
   Asthma                     0.970     0.451      4.613     1
Poor psychological health
   Fatigue                    1.150     0.337     11.676     1
   Incapable of making
     decisions                1.066     0.486      4.814     1
   Unable to accomplish
     things                   0.840     0.381      4.872     1
   Feeling of worthlessness   1.113     0.450      6.133     1
   Reasonably happy           0.934     0.399      5.465     1
   Unhappy and depressed      1.027     0.383      7.197     1
   Anxiety, worry             0.727     0.319      5.187     1
   Suicidal thoughts          1.041     0.383      7.374     1
   Suicide attempts           1.729     0.495     12.207     1

                              Logistic regression analysis
                                P
Health status                 value   OR (a)    CI 95% (b)

Poor physical health
   Bad physical health days
     [greater than or         .026    2.155     1.098-4.229
     equal to] 15
   Tinnitus                   .002    2.602     1.401-4.833
   Severe loss of vision      .000    32.477   16.841-62.633
   Headache                   .001    2.907     1.518-5.566
   Asthma                     .032    2.637     1.088-6.389
Poor psychological health
   Fatigue                    .001    3.158     1.633-6.107
   Incapable of making
     decisions                .024    2.904     1.120-7.527
   Unable to accomplish
     things                   .027    2.317     1.099-4.885
   Feeling of worthlessness   .013    3.045     1.261-7.349
   Reasonably happy           .019    2.544     1.163-5.564
   Unhappy and depressed      .007    2.792     1.319-5.913
   Anxiety, worry             .023    2.069     1.107-3.866
   Suicidal thoughts          .007    2.831     1.336-5.999
   Suicide attempts           .000    5.636     2.137-14.869

p [less than or equal to] . 05; (a) OR adjusted for age;
(b) CI 95% for the OR.
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Article Details
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Author:Wahlqvist, Moa; Moller, Claes; Moller, Kerstin; Danermark, Berth
Publication:Journal of Visual Impairment & Blindness
Article Type:Report
Geographic Code:4EUSW
Date:May 1, 2013
Words:7103
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