Chronic pain and fatigue in adults with congenital unilateral upper limb deficiency in Norway. A cross-sectional study
Chronic pain and fatigue in adults with congenital unilateral upper limb deficiency in Norway. A cross-sectional study
Heidi Johansen 1 2
Trine Bathen 1 2
Liv Øinñs Andersen 1 2
Svend Rand-Hendriksen 0 1 2
Kristin Østlie 2
0 Institute of Clinical Medicine, Faculty of Medicine, University of Oslo , Oslo , Norway , 3 Department of Physical Medicine and Rehabilitation, Innlandet Hospital Trust , Ottestad , Norway
1 TRS, National Resource Centre for Rare Disorders, Sunnaas Rehabilitation Hospital , Nesodden , Norway
2 Editor: Benjamin A. Philip, Washington University in Saint Louis School of Medicine , UNITED STATES
To describe Norwegian adults with congenital unilateral upper limb deficiency (CUULD) regarding self-reported chronic pain (intensity, locations, impact on daily life) and fatigue. Analyze associations between chronic pain and demographic/clinical factors and associations between fatigue and demographic/ clinical factors.
Data Availability Statement: The data underlying
this study is restricted by the Regional Ethics
Committee for Medical and Health Research Ethics
in Eastern Norway and by the Data Protection
Officer at Oslo University Hospital. Due to these
ethical restrictions regarding potentially identifying
patient information, data is available upon request
from: Department manager for the Research
Department, Sunnaas Rehabilitation Hospital
Annette M. Juelsen ().
The data are from the Limb deficiency study whose
authors may be contacted at TRS, National
Materials and methods
Cross-sectional study. In 2012, a postal questionnaire was sent to 186 persons with congen
ital limb deficiency, age 20 years. Seventy seven persons with CUULD responded and
are included in this paper. The questionnaire included questions on demographic and
clinical factors, chronic pain (Brief Pain Inventory, Standardized Nordic Questionnaire) and
fatigue (Fatigue severity scale (FSS)).
Mean age was 42.7 (SD 16.0), 71% were women. Sixty tree % reported chronic pain, many
had bilateral pain, most common pain locations were neck (78%) and shoulder/upper arm
(78%). However, reported mean pain intensity (3.3 (SD 2.8)) and mean number of pain
locations (3.0 (SD 2.5)) were moderate to low. Thirty seven persons reported that pain started in
adult age ( 19 years). One third reported severe fatigue (FSS 5). Persons reporting cold
sensitivity and severe fatigue were most likely to have chronic pain.
Congenital upper limb deficiency increases the risk of self-reported pain in neck, shoulder/ upper arm, cold sensitivity and severe fatigue. Pain, fatigue and cold sensitivity may individually affect function, and may together reinforce functional problems. This should be to
Funding: The authors received no specific funding
for this work.
Competing interests: The authors have declared
that no competing interests exist.
taken into account when rehabilitation programs are developed. Further studies of more
representative samples should be conducted to confirm our findings.
This paper presents data on chronic pain and fatigue in adults with congenital unilateral upper
limb deficiency (CUULD) in Norway. The study sample is part of a congenital limb deficiency
(CLD) population described in earlier papers [
CUULD is the most frequent CLD, and is twice as common as lower limb deficiency [
The incidence of CUULD has been reported as 5.25 per 10.000 live births . Different
nosologies and classification systems have been presented [
]. In Norway, the ISO/ISPO
classification of congenital limb deficiency is most frequently used . In this classification, CLD`s are
divided into transverse and longitudinal defects. In transverse CLD`s, all structures distal to a
specific point of the limb are lacking. In longitudinal CLD`s, a bone or several bones are
lacking partially or completely parallel to the long axis of the limb. In addition to the lacking parts
of the limb, blood vessels, nerves and other structures in the remaining part of the limb may
also be affected [
Missing the whole or parts of arm, hand and/ or fingers can cause problems in daily life as
arms and hands are used for most activities. Deficiencies of fingers and hands may affect grip,
the ability to lift and the range of reach. Persons with transverse upper limb deficiencies often
totally lack grip, those with longitudinal deficiencies often have some kind of grip with reduced
power and reach. Depending on the level of the deficiency, some persons with CUULD may
benefit from the use of prostheses. Some prostheses are made for improving function, while
others are used mainly for cosmetics [
]. Use of prostheses may also improve the
participation in the society [
]. However, use of prostheses interferes with sensibility and the person`s
natural movement patterns [
It has been proposed that persons with acquired- and CUULD may be prone to overuse
]. In adults with acquired UULD an increased risk of self-reported
musculoskeletal pain in the neck, upper back, shoulder and in the remaining arm was reported .
Prosthesis-use did not prevent pain [
]. Studies on mixed aquired- and CUULD samples
have shown similar results [
]. The available data on prevalence, localisation, intensity
and duration of chronic pain in adults with CUULD however, is limited, and it is uncertain
whether they have the same prevalence and expression of chronic pain as persons with
A longitudinal study of persons with unilateral below- elbow CLD [
examination at mean age 13 years and follow-up at mean age 37 years, found no increase in physical
complaints, no scoliosis and no differences between prostheses-users and non- users in
relation to physical complaints or scoliosis. Two studies on young adults with CUULD found no
limitations in activity and participation, compared with the general population [
between mild and severe type of longitudinal radial deficiency [
However, in two earlier papers on the CUULD population, also described in the present
study, we presented a significantly higher prevalence of chronic pain (63%) [
] and lower
health related quality of life [
] than in the Norwegian general population (NGP).
Healthrelated quality of life was most reduced in the physical health domain, and the greatest
difference was found in the bodily pain scale [
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Chronic pain is a complex phenomenon covering both physiological and psychosocial
]. Chronic pain is the main cause of long term sick-leave and disability in Norway
[18±20]. In the NGP chronic pain is often widespread, generalized and associated with female
gender, high age, low education level, receiving pension, psychological distress, chronic illness
and low physical activity [18±20].
In other patient groups like Marfan syndrome, rheumatoid arthritis and poliomyelitis [21±
23], fatigue has been found to be the factor most significantly associated with chronic pain.
Fatigue is often defined as an ªoverwhelming sense of tiredness, lack of energy and feeling of
exhaustion, mental, physical or bothº [
]. Fatigue is prevalent in the general population [
it is a common symptom in various chronic diseases, and has been found to be a major
determinant of disability, which significantly influences quality of life and impairs people's work
21, 26, 27
Based on our clinical experience, persons with CUULD often report tiredness and/or pain.
To our knowledge there has not been focus on fatigue as a phenomenon in this population.
We therefore wanted to explore the aspects of chronic pain and fatigue in adults with CUULD.
The aims of this paper were to: 1) Provide a further description of self-reported chronic
pain (intensity, locations, and impact on daily life), 2) Study the prevalence of severe fatigue, 3)
Explore the associations between chronic pain and demographic and clinical factors and 4)
Explore the associations between fatigue and demographic and clinical factors among adults
with CUULD in Norway.
Materials and methods
Design and subjects
In this paper we used data on persons with CUULD from a cross-sectional,
questionnairebased study of adults with CLD. Potential participants for the CLD study were identified using
the electronic database of the TRS National Resource Centre for Rare Disorders (TRS).
Persons 20 years with a CLD were asked to participate. The exclusion criteria were lack of
mastery of written Norwegian, syndactyly, polydactyly, and Poland syndrome without CLD.
The inclusion process is described in detail in a previous paper [
TRS acts as a supplement to Norway's public health services [
]. Persons in one of eight
rare diagnostic groups (CLD, artrogryphosis multiplex congenita, Ehler-Danlos syndrome,
Marfans syndrome, osteogenesis imperfecta, short stature and spina bifida /
myelomeningocele, multiple ostochondromas) may contact TRS, without a referral. Upon contact, they agree
to be registered in an electronic medical database.
A questionnaire was designed in cooperation with representatives from the Norwegian Limb
Deficiency Association (NLDA). Variables included: Demographic factors; given
syndromediagnoses; comorbidity (additional diagnoses); use of technical aids and/or prostheses; pain
and fatigue (detailed below). Description of the CLD (side, type and level for the affected limb,
including marking the deficiency on a figure) was used to classify the deficiencies as left/right,
level and longitudinal/transverse. The classification was done in accordance to Day 1991[
The description of the deficiency was used to create a dichotomized variable ªgrip abilityº: no
grip = no, reduced grip/near to normal grip = yes. Cold sensitivity was investigated with the
question: Do you experience to freeze easily on the affected limb? (yes/no).
Pain was measured in several ways, using questions adapted from the Brief Pain Inventory
] and the Standardized Nordic Questionnaire [
]. These instruments are shown to
have satisfactory psychometric properties [
28, 29, 30
], are validated for the NGP [
3 / 16
other patient groups [
], but not for limb deficiencies. The following variables were used: 1)
Experience of persisting chronic musculoskeletal pain more than three months during the last
year (yes/no). 2) Side of pain (right/left/both sides). 3) Self-reported pain intensity (PI) over
the last seven days, assessed with an 11-point Numeric Pain Rating Scale (NPRS) with 0 = ªno
painº and 10 = ªpain as bad as it can beº. A pain score of 1±3 indicates ªmild painº, a score of
4±6 indicates ªmoderate painº and a score of 7±10 indicates ªsevere painº [
]. 4) Pain
drawings: The participants were asked to mark their pain experience during the past two weeks on
silhouettes of the human body (front and back). The locations of marked areas were scored
using a modified procedure of the Brief Pain Inventory [
]: Each drawing was divided into 11
areas using a template (head, neck, shoulder/upper arm, elbow/forearm, wrist/hand, upper
back, lower back, front chest, hip/thigh, knee/leg, ankle/foot), and each area scored as yes/no.
Summing up the number of marked areas, we made a ªnumber of pain locationº (NPL) score.
The NPL was then categorized into three groups: ªFew pain locationsº (0±3), ªmoderate pain
locationsº (4±6) and ªmany pain locationsº (7±11) [
]. 5) Debut of present pain: ªIn
childhoodº (0±12 years), ªduring adolescenceº (13±18 years), ªin adulthoodº ( 19 years). 6) Pain
impact on participation (yes/no) in: ªhousekeepingº, ªworkingº, ªleisure activitiesº. 7) A free
text description of self-reported factors reducing and increasing pain.
Fatigue was measured using the Fatigue Severity Scale (FSS), a nine-item questionnaire
developed to measure fatigue intensity and the impact on daily functioning [
]. Each statement
(e.g. ªI am easily fatiguedº) is rated on a 7- point response scale, ranging from 1 (completely
disagree) to 7 (completely agree). A mean score is calculated for each person, range 1 to 7 [
Higher scores indicate higher levels of fatigue. FSS has been used in the NGP [
] and in
different chronic diseases [
], and has been found valid and reliable [
]. To assess the prevalence of
ªsevere fatigueº versus ªno fatigueº, the following cut-off values were used: ªNo fatigueº = FSS
mean score 4; ªsevere fatigueº = 5; and ªborderline fatigueº mean score >4 and < 5 [
To measure health related quality of life (HRQOL) the MOS SF-36 version 2 was used [
The SF-36 consists of eight subscales, four mental scales: mental health, role functioning
emotional, social functioning, vitality and four physical scales: general health, bodily pain, physical
functioning and role functioning physical. Mean scores may be reported for each individual
subscale and for two sum scores: mental component summary (MCS) and physical component
summary (PCS). All subscales in SF-36 have 0±100 scales, 100 is best health status score [
The HRQOL in our CUULD sample is reported in detail in an earlier paper [
]. In the present
paper, we used the physical functioningÐand mental health scales to explore associations
between pain and fatigue and physical functioning and mental health. The SF-36 sum scores
were not appropriate to use as independent variables in the regression analyses (with pain and
fatigue as dependent variables) because bodily pain and vitality scales are included in the sum
The study has been approved by the Regional Ethics Committee for Medical and Health
Research Ethics in Eastern Norway and by the Data Protection Officer at Oslo University
In October 2012, letters were sent to 186 adults with CLD, including a letter for written
informed consent, the questionnaire and a prepaid, return-addressed envelope. A written
reminder was sent after three weeks to non-responders. To motivate participation, a pamphlet
describing the study was distributed beforehand to the five limb deficiency centers in Norway.
In addition, it was sent to the two Norwegian companies making upper-limb prostheses and
assistive devices. The pamphlet was also published on the website of the NLDA.
4 / 16
Data were entered into a customized database and processed by using the Statistical Package
for the Social Sciences (SPSS) version 19.0 (SPSS1, IBM, Armonk, NY). For hypothesis testing,
the significance level was set at α = 0.05.
To assess the representativeness of our original CLD sample, we used independent samples
t-test (for continuous variables) and Fisher's exact test (for frequencies) to compare the
questionnaire responders (n = 97) and non-responders (n = 89). The variables compared were
gender, age and place of residence (region). Based on knowledge on general prevalence of CUULD
among CLD (two out of three) [
], we calculated response rate for CUULD in our sample.
Demographic data were analyzed using descriptive statistics. To compare the extent of pain
and fatigue between younger and older adults with CUULD, a cut-off age was set to 40 years,
because our clinical experience indicates increased pain and fatigue from that age. Distribution
of pain locations in the study sample was compared with reported values from the NGP [
by using the chi-square test for goodness of fit.
Univariate logistic regression analyses were used to explore the association between the
dependent variable chronic pain (yes/no) and several factors: age ( 40 years (yes/no), gender,
parenthood, living with a partner, comorbidity (additional diagnoses), prosthesis-use (active
or passive prosthesis), grip ability, cold sensitivity, education ( 13 years, yes/no) and studying
or working (without any disability pension), SF-36 physical functioning, SF-36 mental health
and FSS. Age, gender and the variables that were significantly associated with chronic pain in
the univariate analyses were entered simultaneously into a multivariate logistic regression
analysis. The strength of each association was expressed as an odds ratio (OR) with a 95%
confidence interval (95% CI).
We used Q-Q plots to assess whether the FSS-scores for the analyzed subgroups were near to
a Normal distribution. Linear regression models were used to examine the influence of different
factors on fatigue measured with FSS. We considered the following factors: age ( 40 years, yes/
no), gender, parenthood, living with a partner, comorbidity (additional diagnoses),
prosthesisuse (active or passive prosthesis), grip ability, cold sensitivity, education ( 13 years, yes/no)
and studying or working (without any disability pension), SF-36 physical functioning, SF-36
mental health and chronic pain (yes/no). First, univariate analyses were performed
consecutively with FSS as dependent variable, analyzing one independent variable at a time. Second,
multiple regression models were applied using the independent variables age, gender, and the
variables that were significantly associated with the FSS in the univariate analyses.
Response rate and analyses of representativeness
Of 186 available persons with CLD, 97 persons filled in the questionnaires, yielding a 52.2%
response rate in the original CLD study [
]. There were no significant differences between the
responders and the non-responders regarding place of residence. There were, however,
differences regarding mean age (among the responders, 7.6 years higher, p<0.001) and gender
(more women among the responders, p = 0.003). Seventy seven of the 97 responders had
CUULD. The response rate for CUULD was calculated to approximately 62%.
Demographic and clinical factors
The distribution of CUULD types is shown in Fig 1. Most reported left-sided (47/77), below
elbow (49/77) deficiency and 53/77 reported transverse deficiency. Demographic and clinical
factors are shown in Table 1.
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Fig 1. Type, body side and level of the deficiency. Figure shows number of persons with different deficiencies.
Chronic musculoskeletal pain was reported by 63%. Among those reporting pain, neck pain
(78%) and pain in shoulder /upper arm (78%) was most common, followed by pain in elbow/
forearm (57%) and upper back pain (51%). Bilateral pain was common in all pain locations,
regardless of body side affected by the deficiency. Persons with unilateral pain tended to have
most pain in the same body-side as the deficiency, Table 2.
Compared to the NGP [
], more persons with CUULD reported pain in the neck (78% vs
41%, p 0.001), shoulder/upper arm (78% vs 46%, p 0.001), elbow/forearm (57% vs 21%, p
0.001), wrist/hand/fingers (43% vs 21%, p 0.001) and upper back (51% vs 26%, p 0.001).
Pain in the head (14% vs 23%, p = 0.15) lower back (45% vs 52%, p = 0.48) knee/leg (25% vs
38%,p = 0.004) and ancles/feet (10% vs 22%, p = 0.05) were reported less frequently in persons
On the NPRS (range 0±10), mean pain intensity (PI) for the whole sample was reported to
be 3.3 (SD 2.8). Mean PI score in persons reporting PI >1(n = 55), was 4.7 (SD 2.2). Of 11
possible pain locations (range 0±11), mean number of pain locations (NPL) was 3.0 (SD 2.5) for
the whole study sample, and in persons reporting mean NPL >1(n = 58), mean score was 4.0
(SD 2.1), Table 3.
In persons reporting chronic pain (n = 49); pain limited participation in housekeeping for
23, in leisure activities for 18 and in work-life for 15 persons. Five persons reported that pain
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aPain locations (0±11) created from the pain drawings.
bPercentages only given when n>10.
aPI = Pain intensity, assessed with the Numeric Pain Rating Scale (NPRS). PI.
bNPL = Number of pain locations: head, neck, upper back, shoulder/upper arm, elbow/fore arm, wrist/hand/
fingers, lower. back, chest, hips/thigh, knee/leg, ancles/feet.
cFSS = Fatigue Severity Scale.
started in childhood, 13 in adolescence and 37 in adulthood. Self-reported factors reducing
pain were physiotherapy/physical activity and exercise (32), painkillers (16) and adjusted
activity and rest (7). Self-reported factors increasing pain were heavy physical work, fine
motorand/or static work (42), stress (6) and rest (3).
In the multiple logistic regression model, chronic pain was associated with female gender
(aOR = 5.49, p = 0.025), cold sensitivity (aOR = 4.35, p = 0.035) and fatigue (FSS score)
(aOR = 1.88, p = 0.029). These findings indicate that women are about 5.5 times more likely to
have chronic pain than men, persons who are sensitive to cold are about 4.4 times more likely
to have chronic pain than those without cold sensitivity, and persons with high FSS scores are
more likely to have chronic pain than those with low FSS scores, when controlling for each of
the other factors in the model. The regression model explained 35.1 to 48. 2% of the variance
in chronic pain, Table 4.
Mean FSS score was 4.0 (SD 1.5) for the total study group. Severe fatigue was reported by 25/
77, no fatigue by 39/77 and borderline fatigue by 11/77, Table 3.
Table 5 shows the univariate and multivariate linear associations between demographic and
clinical factors and the FSS score. In the multivariate analyses, chronic pain (p = 0.016) and
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Fatigue measured by Fatigue Severety Scale. A low FSS score indicates low fatigue.
Abbreviations: B unstandardized regression coefficient; cB, crude B; aB, adjusted B- adjusted for age and gender and the variables that were significant in
the univariate linear regression; n, number of persons with CUULD available for crude analysis (univariate linear regression). NI, not included in adjusted
Age, 40 years: yes = 1, no = 0, Gender, women: yes = 1, no = 0. Parenthood: yes = 1, no = 0, Living with partner: yes = 1,no = 0, Education level 13
years: yes = 1, no = 0. Student or working: yes = 1, no = 0. Comorbidity: yes = 1, no = 0. Grip ability: yes = 1, no = 0. Sensitive to cold: yes = 1, no = 0.
Prosthesis user: yes = 1, no = 0.
SF-36 physical functioning (0±100). SF-36 mental health (0±100).
R2 = 22.8%.
mental health (p = 0.046) was found to be significantly associated with fatigue. The regression
model explained 22.8% of the variance in the fatigue scores.
In this study, almost two thirds of persons with CUULD reported chronic pain, many had
bilateral pain, and the most common pain locations were neck and shoulder/upper arm.
However, pain intensity and the number of pain locations were moderate to low. Many reported
that pain started in adult age, and limited participation in everyday life. The study population
reported that heavy physical work, static work and stress increased pain, and that
physiotherapy, exercise, painkillers, adjusting activity and rest reduced pain. Persons being cold sensitive
and persons with high fatigue scores were most likely to have chronic pain. One third reported
severe fatigue, the association between pain and fatigue was strong.
We have not found previous studies describing pain in adult ( 40 years) CUULD
populations. In our sample nearly two thirds reported chronic pain. In a Norwegian study on NGP,
between 19 and 31% reported chronic pain, varying between age-groups, most pain was
reported among the elderly [
]. In persons with CUULD, the most frequent pain locations
were neck, shoulder/upper arm, elbow/forearm and upper back, significantly higher than
reported in the NGP [
]. In the NGP lower back pain was most frequent [
]. In accordance
10 / 16
with our findings, Østlie et al. found a high frequency of neck/ upper back and shoulder pain
among acquired UULD [
]. In two mixed study groups of acquired UULD and CUULD,
many reported neck/ shoulder and upper back pain and more than one third has been found
to have Carpal tunnel syndrome (CTS) [
]. In our study sample we found the same
fraction reporting pain in hand/fingers. CTS have not been investigated in groups of CUULD.
In our sample, more than 50% reported few pain locations, and 7% reported many pain
locations. This is in accordance with findings among acquired UULD [
], but in contrast to
Norwegians with chronic pain, were only 20% reported few pain locations and approximately
50% reported many pain locations [
We have not found previous reports on pain intensity (PI) among adults with CUULD. In
accordance with findings in persons with acquired UULD [
], the majority of our CUULD
sample reported mild/moderate PI, and only a small fraction reported severe pain. The mean
PI reported by the CUULD group with chronic pain (4.7) was higher than in Norwegians with
chronic pain (3.9) [
], and in persons with rheumatoid arthritis (4.3) [
], but slightly lower
than in persons with post- polio (5.3) [
This might be because persons with a distinct visible disability such as limb loss, do not need
to underline their pain, they become believable anyway. This attitude we know from the clinic,
and have earlier been described in Johansen et al. [
]. Also, these findings suggest that
acquiredand CUULD increases the risk of localized pain, most likely due to overuse and compensative
behavior, but perhaps does not increase the risk of generalized pain [10±13]. Results however,
have to be interpreted with caution as differences may be due to methodological issues.
The level of PI and NPL are found to have major impact on how the individual lives with
pain on a daily basis [
]. Many can live well with some pain, but the more NPL and the higher
PI, the more the pain will influence function, activity and participation [
]. In the clinic we
meet persons with CUULD reporting pain. Some convey that they do not perceive pain as
bothersome in a way that interferes with their life. Others may talk about severe bothersome
pain that threaten both everyday life and work-life. Maybe pain has particular importance for
some persons with marginal function, like some individuals with CUULD. And maybe, those
who struggle to pass as non-disabled [
] are especially vulnerable.
Chronic musculoskeletal pain is often associated with fear [
] and hence it is possible that
presence of pain increases fear of losing independence in everyday life. It is conceivable that
the individual pain may be experienced as a notification of loss of function and coping, and
that pain reporting can be an expression of this. This topic was also underlined in a Norwegian
interview study with women with CUULD [
]. The importance of being like everybody else,
have also been discussed in relation to other study populations with moderate disability; late
onset polio [
] and CP [
]. Most of our CUULD participants reported pain debut in
adulthood. This may indicate that pain comes gradually after years with compensatory coping
strategies that often are physically demanding.
Factors reported by persons with CUULD to influence pain are well known from our
clinical experience and are supported in the literature [
]. Heavy physical work, static work,
fine motor activities, and stress will provide overuse and pain over time in all persons ; and
persons with marginal function as persons with CUULD may be even more vulnerable.
Exercise, physical therapy, physical activity, painkillers, and rest are known factors to alleviate pain
]. Approximately half of the study sample reported physical therapy and physical activity
reduced pain. In spite of sparse knowledge about the effect of training and physical exercise on
adults with CUULD, it is likely that exercise and necessity of finding new ways of coping may
be helpful, as described by Stoelb et al. [
] and Johansen et al. [
]. Adjusted and individual
rehabilitation program for persons with CUULD should be developed and the effect on pain
should be investigated.
11 / 16
We have not found previous studies describing fatigue in either acquired UULD or CUULD
populations. Mean fatigue (4.0) in the study group was equivalent to that reported in NGP
]. However, severe fatigue was reported by 33%, considerably higher than in the NGP
], reflecting that although the mean fatigue score was low, a significant proportion of
adults with CUULD report severe fatigue.
Pain and fatigueÐassociations to demographic and clinical factors
In the multivariate analyses age and education level was not associated with pain or fatigue.
However, in our study group the mean age was relatively low, and the education level was
relatively high, therefore we might have a selection bias. Therefore, these findings have to be
interpreted with caution. Female gender was associated with pain but not with fatigue. Some
studies show that women are more prone to develop chronic pain [
] and fatigue [
] in the
NGP. However, in our study group the proportion of men was low; the result therefore has to
be interpreted with caution.
Being cold sensitive was associated with pain when controlling for other factors in the
model. To our knowledge, examination of cold sensitivity in persons with CUULD has not
been done previously. In persons with hand pathology, cold sensitivity is a common problem
] and those with severe cold sensitivity report cold-induced pain . In persons with
CUULD it is known that blood vessels and nerves may be affected in the side of the deficiency
]. Reduced blood flow may perhaps cause cold sensitivity and ischemic pain. In the clinic we
experience that several persons report bothersome cold sensitivity and that some respond well
to warming assistive devices. This is in line with Vaksvik et al.`s findings on persons with hand
injury and cold hypersensitivity [
]. It is likely that cold sensitivity, chronic pain and sever
fatigue may individually affect function, and may together reinforce functional problems. In
persons with CUULD this needs to be investigated further.
Low mental health was significantly associated with high fatigue scores. A strong
association between fatigue and mental health is also known from other patient populations [
In persons with CUULD, reduced ability to perform daily activities and/or work tasks due to
the limb loss, may lead to both mental health challenges and reported fatigue. In accordance
with this, it has been shown that reduced ability to work is associated with a tendency to poorer
mental health in persons with acquired UULD .
In the present study, chronic pain and fatigue were significantly associated, when
controlling for other variables. This close relation between fatigue and chronic pain has also been
found both in general populations [
] and in other diagnoses with musculoskeletal
problems [21±23]. Pain and fatigue may be seen as part of the same phenomenon; as discussed
above it is physically demanding over time to manage with one functioning arm; this may
challenge the patient's capacity and cause both pain and fatigue .
One might have expected an association between chronic pain/fatigue and limb deficiency
variables like prosthesis use and grip ability. We found no such associations, and neither did
Østlie et al. in acquired UULD [
]. The multiple regression models explained 35±48% of the
variation in chronic pain and 23% of the variation of fatigue implying that other factors are
associated with chronic pain and fatigue. These findings need to be explored further.
Strength and weaknesses
A low response rate is a weakness of our CLD-study. This may have led to selection bias in our
sample. The respondents were older, with a lower percentage of men than the non-responder
group. This may have affected our findings regarding variables that are known to be
gender12 / 16
dependent, such as pain and fatigue [
]. Including persons with CUULD only from the
TRS register, may have resulted in selection bias. Those who are registered at TRS usually have
done so because of some need for services, thus potentially affecting some of the variables
studied. Persons choosing to register at TRS may also differ from those who do not in relation to
socioeconomic factors such as educational level .
Self-reported data may be influenced by recall bias. The relatively small sample size leads to
reduced statistical power for several analyses, especially regarding differences between
subgroups. Using a non-validated cold sensitivity measure may have caused information bias. The
survey was conducted late in the fall, a rather cold season in Norway. Pain and cold sensitivity
can be affected by cold weather; therefore, we may have an information bias resulting in higher
estimates of pain and cold sensitivity than if the study had been conducted in the summer. In
Norway, however, it is cold weather during all seasons, and fall is far warmer than the winter
time. We therefore estimate this bias as minor. One strength of our study is that our material is
recruited from a defined geographical area (Norway) with a defined population (5 million
inhabitants), and the response rate among CUULD was considered to be satisfactory and is
higher than in our original CLD study [
]. Another strength is that we have studied adults,
who have answered questions for themselves about issues that have not been investigated
before. Furthermore, pain and fatigue were explored using standardized and validated
Many adults with CUULD report chronic pain, often bilateral, primarily located to the neck
and shoulder. However, they report moderate to low pain intensity and a low number of pain
locations compared to other chronic pain study populations, indicating that a significant
portion do not experience the pain as severely bothersome. A significant fraction also report cold
sensitivity and severe fatigue. A strong association between pain and fatigue may indicate that
everyday life with only one functioning arm is physically challenging, increasing the risk of
developing pain and fatigue. These factors may influence functioning and should be taken into
account when rehabilitation programs for persons with CUULD are developed.
We wish to thank the participants who answered the questionnaire and made this study
possible. We also wish to thank the Norwegian Limb Deficiency Association and professionals in
the Norwegian limb deficiency centers for their valuable help and participation.
Conceptualization: Heidi Johansen, Liv Øinñs Andersen, Svend Rand-Hendriksen, Kristin
Data curation: Heidi Johansen, Liv Øinñs Andersen, Kristin Østlie.
Formal analysis: Heidi Johansen, Trine Bathen, Kristin Østlie.
Investigation: Heidi Johansen, Liv Øinñs Andersen.
Methodology: Heidi Johansen, Trine Bathen, Liv Øinñs Andersen, Svend Rand-Hendriksen,
Project administration: Heidi Johansen.
Supervision: Trine Bathen, Liv Øinñs Andersen, Svend Rand-Hendriksen, Kristin Østlie.
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Validation: Heidi Johansen, Trine Bathen, Svend Rand-Hendriksen, Kristin Østlie.
Visualization: Heidi Johansen, Trine Bathen, Svend Rand-Hendriksen, Kristin Østlie.
Writing ± original draft: Heidi Johansen, Trine Bathen, Liv Øinñs Andersen, Svend
Hendriksen, Kristin Østlie.
Writing ± review & editing: Heidi Johansen, Trine Bathen, Svend Rand-Hendriksen, Kristin
14 / 16
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