Frequency and Self-Management of Pain, Dyspnea, and Cough in Cystic Fibrosis

Article Outline

• Abstract
• Introduction
• Methods
  • Recruitment and Participant Selection
  • Data Collection Measures and Methods
• Results
  • Demographic Data
  • Pain
  • Dyspnea
  • Cough
• Discussion
• Acknowledgments
• References
• Copyright

Abstract 

Cystic fibrosis (CF) has been transformed from a fatal diagnosis in infancy to a chronic disease of children and young adults. Symptom patterns and disease burden in CF may be shifting to reflect the relatively healthier, older population with the disease. Self-management of symptoms is a hallmark of chronic illness, and yet we do not have a good understanding of how CF patients monitor or manage their symptoms. Children and adults were recruited through clinics in three Canadian provinces. Questionnaires with open-ended and close-ended questions in English and French, designed to assess the frequency, severity, and self-management of pain, breathlessness, and cough, were mailed to all the eligible participants. One hundred twenty-three respondents completed the survey, for a response rate of 64%. Eighty-four percent (103 of 123) of participants reported having pain. They reported an average of 2.1 locations of pain, with headache and abdominal pain most frequently described. Sixty-four percent (76 of 123) of participants reported having breathlessness, and 83% (99 of 123) of participants reported experiencing cough. Sixty-three percent (62 of 99) of participants with cough reported that cough always or sometimes interfered with their sleep. A variety of pharmacological and nonpharmacological treatments were used to manage symptoms. Pain and dyspnea are more common than suspected and a wide variety of pharmacological and nonpharmacological measures are used to treat symptoms. Cough is difficult to assess, but disturbed sleep may be an indicator of cough severity and an important symptom to consider when evaluating the overall burden of illness in those with CF.

Key Words: Pain, dyspnea, cough, cystic fibrosis, fibrosis, adult, child, pain measurement, chronic disease

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Introduction 

Since its initial description in 1938, cystic fibrosis (CF) has been transformed from a diagnosis fatal in infancy to a chronic disease of children and young adults. It is estimated that one in every 2,500 Caucasian children worldwide has CF.¹ Approximately 3,400 children, adolescents, and adults with CF attend specialized CF clinics in Canada, and one in every 3,600 children born in the country has CF. The median age of survival for Canadians with CF is 37.0 years.² Although life expectancy has increased dramatically, management of the disease requires a complicated daily regimen of preventive and therapeutic interventions. Monitoring of daily symptoms is an important aspect of this regimen, as a change in symptoms can signal patients and clinicians to intervene.

Symptom patterns and disease burden in CF may be shifting to reflect the relatively healthier, older population with the disease. Knowledge of these changing patterns is important not only to develop appropriate therapeutic responses, but also to create patient-oriented outcomes for clinical therapeutics. In particular, systematic information on the frequency and severity of cough, dyspnea, and pain is lacking in the new, relatively healthier CF population. Moreover, there is no information in the literature on the steps taken by patients to address these symptoms. Self-management of symptoms is a hallmark of chronic illness, and yet we do not have a good understanding of how patients monitor or manage their symptoms in CF. To begin to address this issue, we surveyed the population of CF patients followed in the Canadian Maritimes to gather self-reported assessment and self-management of pain, dyspnea, and cough information.

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Methods 

Recruitment and Participant Selection 

Participants were recruited through the CF clinics in three provinces of Canada. These clinics provide care under a universal health system that does not charge user fees. They follow approximately 250 patients in three eastern provinces in Canada: Nova Scotia, New Brunswick, and Prince Edward Island. Ethical approval for the study was obtained from a total of four ethical review boards within the three participating provinces. Informed consent/assent was gained from study participants and, from their parent/caregiver, if the participant was below the age of 18 years. The data collection period occurred from June 2005 to July 2006.

Questionnaires were mailed to all the eligible participants, with some receiving questionnaires at their clinic appointments or while inpatients. Inclusion criteria were a diagnosis of CF; current residence in one of the three Maritime Provinces; seven years of age or older; ability to read and write in English or French; and no diagnosis of developmental delay.

Data Collection Measures and Methods 

Study participants were asked to complete a demographic form and the study questionnaire. The study questionnaire used both open-ended and close-ended questions to assess for symptom frequency and severity, for example: “In the past 30 days, approximately how many days were you breathless?,” and “How breathless are you?” For dyspnea severity, participants were asked to complete a numerical rating scale (NRS) (rating from 0 to 10) and the Dalhousie Dyspnea Scale (DDS), a pictorial scale validated in subjects aged eight years or older³, ⁴, ⁵ (Fig. 1). Further questions about the impact of symptoms on the participant were included in this questionnaire by asking the location, duration, and treatments used to manage symptoms. The project investigators, who included experts in CF, pain, and symptom management, reviewed these questions. The survey was designed to elicit information on actions or health activities that may not prompt a visit to a health care professional. Therefore, it was ideal for assessing both symptom frequency and self-management. The participants' most recent FEV₁ (the percent of expected was used for analysis) was obtained from their medical chart. Most participants had an FEV₁ result within the year prior to completing the questionnaire.

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• Fig. 1 

  Dalhousie Dyspnea Scale—Effort, Chest Tightness, and Throat Closing scales.

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Results 

Demographic Data 

Two hundred and one patients met the study criteria and were invited to participate. Of those eligible for study participation, 10 were unable to be located (no surveys were sent). Of the remaining 191, 68 did not return their surveys. Of the 68 who did not respond, 10 were unable to be reached for the follow-up phone calls, one declined participation with no reason provided, and one completed survey was lost in transit, never reaching the research team.

One hundred twenty-three respondents completed the survey, for a response rate of 64% (123 of 191). Respondents ranged in age from 7 to 60 years (mean age 19.9 years, standard deviation [SD] 11.7). Table 1 provides detailed demographic data on the respondents. More than half of the respondents were children aged 7–17 years (68 of 123, 55%). The respondents were reasonably healthy individuals, with 64% (79 of 123) reporting no hospital admissions in the previous year, and only 6% stating that they had more than four admissions in the previous year (7 of 123).

Table 1. Demographic Information

Demographic	Number (% of Total)
Gender
Female	71 (58%)
Male	52 (42%)
Age (years)
7–12	32 (26%)
13–17	32 (26%)
18–24	24 (20%)
≥25	35 (28%)
Age at diagnosis (years)
<1	67 (54%)
1–7	32 (26%)
8–14	11 (9%)
15–21	3 (2%)
22–28	2 (2%)
≥29	1 (1%)
Data unavailable	7 (6%)
Province
Nova Scotia	72 (59%)
New Brunswick	43 (35%)
Prince Edward Island	8 (7%)
Other family member with CF
Yes	57 (46%)
Immediate family	43 (36%)
No	65 (53%)
Unknown (adopted)	1 (1%)
Cigarette smoke exposure
Smoker	6 (5%)
Nonsmoker	117 (95%)
Exposed to smoke in the home	11 (9%)
Most recent FEV1 (%)
>81	49 (40%)
61–80	36 (29%)
41–60	27 (22%)
≤40	5 (4%)
Data unavailable	6 (5%)
Hospitalizations in the past year
0	79 (64%)
1	21 (17%)
2	11 (9%)
3	5 (4%)
≥4	7 (6%)
Pancreatic function
Pancreatic sufficient	13 (11%)
Pancreatic insufficient	110 (89%)
Oxygen dependence
Yes	4 (3%)
Day only	2 (2%)
Night only	2 (2%)
No	117 (95%)
Data unavailable	2 (2%)

Pain 

Eighty-four percent (103 of 123) of participants reported having pain in the 30 days preceding the survey. One participant did not respond to this question. The reported locations of pain are noted in Table 2. The category “Other pains” includes pains that did not fit into one of the major categories (e.g., heartburn, menstrual pain, undefined pain). Participants reported an average of 2.1 locations of pain, with 14 participants reporting four or more pain locations.

Table 2. Types of Pain

Headache and abdominal pain were further analyzed, as they were the pains most frequently described. Half of the participants reported headache (62 of 123, 50%). Participants reported greater inhibition of their daily activities with increased headache severity (r=0.45, P<0.001). Table 3 describes the various methods used to treat headache.

Table 3. Pharmacological and Nonpharmacological Treatments for Headache and Abdominal Pain

Pharmacological Treatments, n (%)		Nonpharmacological Treatments, n (%)
Headache
Paracetamol/ibuprofen	57 (92)	Lay down	7 (11)
		Avoid bright lights	3 (5)
		Normal saline mask	3 (5)
Caffeine+codeine+ASA	3 (5)	Cold cloth/cold pack	3 (5)
		Massage	1 (2)
		Do nothinga	1 (2)
Paracetamol+ASA+caffeine	2 (3)
		Surgery for nasal polyps	1 (2)
Antibiotics	1 (2)
Abdominal pain
Paracetamol/ibuprofen	15 (25)	Rest/comfortable position	14 (23)
Antacid	6 (10)
		Do nothinga	12 (20)
Omeprazole/ranitidine	6 (10)
Increase pancreatic enzymes	4 (7)	Heat	11 (18)
Stool softener/laxative	4 (7)	Have a bowel movement	9 (15)
Morphine/pethidine	2 (3)	Massage	2 (3)
Ursodeoxycholic acid	1 (2)	Eat	1 (2)

Of those who reported headache, the length of time they reported headache was less than one year for 16% (10 of 62), 18% (11 of 62) for one to two years, and 19% (12 of 62) for three to five years, with 16% (10 of 62) for more than five years. Eight percent (5 of 62) did not specify the length of time they had a headache, and 23% (14 of 62) provided no response to this question. Significantly more females reported headache (χ²=8.98, P=0.003), with no differences in headache evident between children and adults.

Fifty percent (61 of 123) of participants reported abdominal pain. Of those participants who were pancreatic sufficient (13 of 123, 11%), only one reported abdominal pain. There is a moderate positive relationship between abdominal pain severity and inhibition of activity (r=0.38, P=0.004).

Of the participants who reported abdominal pain, the length of time that they had pain was less than one year for 10% (6 of 61), one to two years for 16% (10 of 61), and three to five years for 7% (4 of 61), with 31% (19 of 61) having pain for more than five years. Five percent (3 of 61) did not specify any length of time, and 31% (19 of 61) provided no response to this question. Table 3 describes the wide variety of measures used by those who experienced abdominal pain. Of those reporting abdominal pain, 44% (27 of 61) stated that they used one or more pharmacological treatments to manage this type of pain.

Dyspnea 

Sixty-two percent (76 of 123) of participants reported having breathlessness in the 30 days prior to answering the survey. Thirty-five percent (43 of 123) reported no breathlessness, whereas 3% (4 of 123) of participants did not respond to this question. The questionnaire prompted those who reported breathlessness to then rate their worst breathlessness in the last month on the NRS (mean NRS 4.1; SD 2.2—see Fig. 2). Seventy-five participants completed the NRS rating dyspnea.

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• Fig. 2 

  Dyspnea rating.

All the participants were requested to complete the DDS, with 92% (113 of 123) completing the scales. However, there is one missing value for the Throat Closing and Chest Tightness scales. The responses to the three scales are included in Fig. 3, Fig. 4, Fig. 5. There is a moderate inverse correlation between severity of breathlessness on the NRS and pulmonary function (FEV₁) (r=−0.30, P=0.009). For the three scales in the DDS, there were moderate inverse associations with pulmonary function (Effort scale r=−0.37, P=0.009; Throat Closing r=−0.44, P<0.001; Chest Tightness r=−0.42, P<0.001). The DSS scales themselves were highly correlated to one another. Using the Spearman rank correlation coefficient (as the scales are not normally distributed), the highest correlation was between the Chest Tightness and Throat Closing scales (r=0.812, P<0.001), with similar correlations between the Throat Closing and Effort scales (r=0.758, P<0.001) and the Chest Tightness and Effort scales (r=0.751, P<0.001). The NRS for dyspnea and the DDS are stratified by pulmonary function (FEV₁). The resulting mean and P-values are presented in Table 4.

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• Fig. 3 

  Pictorial scale (Effort).

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• Fig. 4 

  Pictorial scale (Chest Tightness).

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• Fig. 5 

  Pictorial scale (Throat Closing).

Table 4. Dyspnea Stratified by Lung Function (FEV₁)

	NRS	DDS—Effort	DDS—Throat Closing	DDS—Chest Tightness
FEV1>81%	3.45	2.72	2.02	1.91
FEV1 61–80%	4.04	3.46	2.65	2.91
FEV1 41–60%	5.06	4.48	3.56	3.24
FEV1≤40%	5.00	3.00	3.50	3.50
P-value	0.109	0.001	<0.001	0.001

A subset of participants provided responses to both the NRS and DDS. When evaluating these responses using the Spearman rank correlation coefficient, the NRS was positively correlated with the three scales of the DDS. The NRS correlates best with the Chest Tightness scale (r=0.636, P<0.001), then with the Effort scale (r=0.625, P<0.001), and slightly lower for the Throat Closing scale (r=0.545, P<0.001).

The treatments and actions taken by participants when breathless are included in Table 5. Participants reported greater inhibition of their daily activities with increased dyspnea severity (on the NRS) (r=0.74, P=0.000). There was no association between the severity of breathlessness and the length of time the participant described being breathless.

Table 5. Treatments/Actions for Breathlessness

Cough 

Eighty percent (99 of 123) of respondents reported experiencing cough in the 30 days prior to answering the survey. Three percent (4 of 123) did not provide a response to this question. Of those who reported cough duration, 22% (22 of 99) had cough for less than one year, 9% (9 of 99) for one to two years, and 6% (6 of 99) for three to five years, with 19% (19 of 99) for more than five years. Eight percent (8 of 99) did not specify a specific length of time and 35% (35 of 99) provided no response to this question. The mean number of days with cough in the previous month was 16.1 days (SD 11.5). Participants experiencing cough also reported on the frequency with which cough interfered with their sleep, with 58% (57 of 99) stating always, 34% (34 of 99) reporting never, and 5% (5 of 99) noting cough sometimes affected their sleep. Three percent (3 of 99) did not respond. A significant difference was evident between the amount of cough (length of time) reported and age group (children vs. adults; χ²=6.24, P=0.01), with adults reporting greater amounts of time coughing. The treatments and actions taken by participants with cough are included in Table 6.

Table 6. Treatments for Cough

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Discussion 

Our survey elicited self-reported data on three important symptoms for those with CF: pain, dyspnea, and cough, and the self-management of chronic symptoms across the age spectrum. Overall, this study demonstrates that there is significant symptom burden even in those relatively healthy CF patients with mild pulmonary impairment, and suggests that these three symptoms have important impacts on quality of life. Consistent assessment of these symptoms would elicit reports of potentially treatable symptoms, with the possibility for a subsequent decrease in the burden of disease for CF patients of all ages.

The high percentage of patients reporting pain in the month prior to the survey is concerning. Pain was reported to limit daily activities and, therefore, is likely to limit the ability to adhere to the preventive regimens, which are an essential component of the improvement of life expectancy. Furthermore, pain-related limitations of daily activities may contribute to the symptom burden, including compounding dyspnea through the resultant deconditioning. Systematic assessment of the pain experienced by patients at all levels of severity would allow clinicians and patients to partner together to address pain and tailor responses to the individual's situation. Pain unassessed is pain unaddressed.

The locations of pain described in this study are similar to previous studies of individuals with CF⁶, ⁷, ⁸, ⁹, ¹⁰, ¹¹, ¹², ¹³, ¹⁴ (Table 7). Pain is quite a prevalent symptom in other studies and even in our relatively healthy CF study population. Our study was unique in that we surveyed a large population of relatively healthy children and adults with CF and received similar response rates about pain across age groups.

Table 7. Studies Evaluating Pain in CF

Author	Study	Participants	Main Results
Palermo et al.6	Retrospective pain interview. Completion of the Cystic Fibrosis Questionnaire-Revised (CFQ–R) to assess their HRQOL and FEV1 from chart. Study completed in the United States	n=46 52% female Aged 8–17 years Mean age=12.9 years Mean FEV1: 80.2	•46% reported experiencing pain at least once per week •After controlling for FEV1%, pain frequency remained a significant predictor of CFQ–R scores. Large effects were detected for physical functioning, role limitations, vitality, weight disturbance, and overall health perceptions •More frequent pain associated with decreased vitality, more respiratory and digestive symptoms, and poorer perceptions of overall health •Higher pain intensity was related to more respiratory and digestive symptoms •More perceived bother from pain was associated with reduced physical functioning and more respiratory and digestive symptoms •An increased number of body regions affected by pain was related to reduced physical functioning and increased respiratory and digestive symptoms
de Dreuzy et al.7 (abstract only)	Retrospective questionnaire (one month). Study completed in France	n=62 60% male Aged 4–20 years Mean age=11 years	•74% had pain in past month •19% had pain the day of study •Pain locations: abdominal, 54%; headache, 37%; musculoskeletal, 29%; thorax-lungs, 14%; stomach, 14% (separate from abdomen); muscles, 8% •Mean intensity 4.5/10 •63% report pain with treatment for CF •50% decreased quality of life
De Villartay et al.8 (abstract only)	Retrospective survey	n=104 Children=45 Adults=59	•71% of the children reported pain in the previous month, with 29% having pain the day questionnaire completed •Locations of pain for children: abdomen (53%), headache (13%), chest (10%), limb (10%) •81% of the children reported pain in the previous month, with 42% having pain the day questionnaire completed •Locations of pain for adults: back (32%), chest (28%), abdomen (20%), headache (12%)
Hubbard et al.9	Cross-sectional web-based survey Pain Disability Index and Pain Coping Questionnaire (adapted from the Pain Response Inventory) Study completed in the United States	n=18 67% female 8% gender not declared Primarily adults Age: 33%<22 years Details of ages not included in publication	•Most commonly reported pain locations: chest 72%, joints 61% •Average intensity of a pain episode was 4.17 (scale of 0–10) •56% of the sample reported experiencing daily pain •Participants reported pain disability highest in areas of recreation, occupation, social activities, family/home responsibilities, and sexual behavior •Pain disability was positively related to the intensity and duration of a pain episode
Koh et al.10	Retrospective pain interview (last four weeks) using self-report questionnaire assessing chronic disease-related pain and FEV1 from chart Study completed in the United States	n=46 52% female Aged 8–17 years Mean age=12.9 years Mean FEV1: 80.2	•Pain locations: 50% abdominal/pelvic region, 37% chest, 33% head/neck, 15% leg, and 13% arm •46% described pain occurring at least once per week •Median pain intensity reported to be mild, with 11% reporting moderately intense pain •70% stated pain caused at least a little emotional upset •Majority of patients reported mild procedural pain •Relief of pain: rest (63%), medications (acetaminophen, NSAIDs, or combination of the two) (41%), relaxation (41%), heat/cold (39%), family/friends (39%), distracting activities (36%) •24% used no medications, 0% used opioids •Chest pain or more frequent pain increased limitations •Pulmonary function was not significantly correlated with pain intensity, duration, or amount of emotional upset. There is a trend for children reporting chest pain to have lower FEV1
Festini et al.11	Retrospective (two months) and six questions, multiple choice+description. Study completed in Italy	n=239 54% female 15 centers in Italy Aged 18–43 years Mean age=26.1 years Mean FEV1: 56.71	•94.1% had pain episodes during previous two months •38.4% described four or more locations over a two-month period •16.9% described three locations simultaneously •Pain locations: headache, 63%; stomach pain/heartburn, 51%; backache/lumbar pain, 48%; bone or muscle pain, 44%; joint pain, 41%; abdominal pain, 33%; chest pain, 31%; cervical pain, 28%; and other types of pain, 6% •Max intensity: 32.6% reported pain 8–10/10 (back/lumbar most severe 8.7/10), 49.8% reported six or more episodes in two months (joint pain most often occurred >10 times, 18.1%) •63.5% stated pain caused unfavorable effects on day-to-day life •Treatment: 26.2% did nothing, 42.6% asked CF physician for help, 6.6% decided on their own what measures to take, 3.5% asked general practitioner for help •If tx: 91.5% medications, 22.2% nonpharmacological (massages, acupuncture, herbal remedies, physical activity, and rest), 1.8% took homeopathic products
Broome and Subramaniam12 (abstract only)	Cross-sectional descriptive survey design using a 35-item questionnaire. Study completed in the United States	n=31 CF patients Aged 10–35 years 29% response rate (includes parent surveys) 26 parents responded (parent information not included in results section of this table)	•88% of patients reported pain, 48% reported moderate pain, 13% severe pain •62% of patients reported one to five episodes of pain every month •Most common site of pain: abdomen (41%) •60% reported pain that hinders day-to-day activities •88% missing one to five days of school every month as a result of pain •91% discussed pain issues with their HCPs •Most reported that they were never referred for pain management (no specific numerical values presented in the abstract) •Acetaminophen (67%) and relaxation (42%) are most commonly used modalities for pain relief
Broome and Subramaniam13 (abstract only—available online only)	Survey of Pediatric Pain Practice (Broome 1998)	HCPs 27 of 79 surveys returned 34% response rate 59% MD 31% RN	•Purpose of study was to explore pain perception and treatment practices of HCPs for children and adolescents with CF •48% of HCPs reported disease-related pain episodes in patients in late adolescence (16–20 years) •81% of HCPs reported disease-related pain episodes in patients ≥21 years •89% rated their patients' pain to be moderate, with 56% reporting one to five episodes of pain every month in their patients •Most common site of pain: chest (96%) •Most common treatments prescribed: nonopioids, heat, and positioning •Obstacles to adequate pain management: lack of resources and knowledge
Ravilly et al.14	Retrospective chart review Study completed in the United States	n=78 Two groups Group 1=55 patients who died between 1984 and 1993, with a mean age of 26 years±8 (9–46 years) Group 2=23 patients referred to pain service	•Group 1: 84% reported serious pain •Group 2: 100% had pain •Locations of chronic pain reported by both groups: chest (64%), headache (55%), back (19%), limb (17%), abdomen (15%), and other (8%) •Group 1 had an increase in chest pain and headache in the three years prior to death •Treatment: >50% tried nonpharmacological approaches (acupuncture, TENS, relaxation techniques, and biofeedback); medications most often used were NSAIDs •10 patients used opioids for more than three months

HRQOL=Health-related quality of life; NSAIDs=nonsteroidal anti-inflammatory drugs; TENS=transcutaneous electrical nerve stimulation; HCPs=health care providers.

With regard to dyspnea, the survey results demonstrate a substantial number of patients who are not in the midst of a pulmonary exacerbation and whose pulmonary function is within the mildly affected or normal range. Despite these parameters of normal health, they still report both the experience of breathlessness and the limitation of activities of daily life by this symptom. With correlations between 0.3 (NRS) and 0.44 (with the FEV₁), the NRS and the DDS used in this study appear to be useful and valid tools for eliciting reports of dyspnea, and may prove helpful in evaluating therapeutic responses to this persistent symptom. The fact that the Chest Tightening and Throat Closing scales were more correlated to the FEV₁ than the Effort scale is not surprising, considering the obstructive nature of CF.⁴

A certain number of participants answered negatively when asked about experiencing dyspnea in the previous 30 days, but did indicate experiencing dyspnea when completing responses on the DDS. One possible explanation for this discrepancy is that dyspnea might not be perceived as being more than what the participant considers to be his or her basal status (i.e., his/her “0”), but that basal level still corresponds to a certain level of dyspnea, as shown on the DDS. This perspective views the participants as essentially having their “basal” level reset from years of chronic, background dyspnea. Also, some participants might have prevented dyspnea by minimizing their physical efforts; therefore, they reported no dyspnea, but when confronted with the Effort scale of the DDS, answered differently.

The correlation of the dyspnea NRS to the DDS scales was helpful in further validating the DDS. It is quite logical that the NRS and the Chest Tightness scale and the Effort scale are more closely correlated than the Throat Closing scale, as CF does not cause upper airway constriction in the same way as other respiratory illnesses.

Chronic daily cough in these individuals with a pulmonary disease is not surprising. Cough is known to be a complex symptom that is common in those with a diagnosis of CF¹⁵, ¹⁶ and an indicator of pulmonary exacerbations in adult CF patients.¹⁶, ¹⁷ Cough frequency is a key variable associated with the use of antibiotic treatment for a pulmonary exacerbation.¹⁸ Cough is a difficult symptom to measure, as subjective ratings have proven to be unreliable when compared with objective measures in patients with CF.¹⁷, ¹⁹, ²⁰ However, new automated cough monitors are being developed, which will assist in measurement accuracy for further research in this area.¹⁶, ²¹ Recently updated clinical practice guidelines for cough have also impacted the diagnosis and management of cough.²² As is the case with pain, without a thorough understanding of the frequency and severity of cough at all levels of disease severity, therapeutic responses will remain ad hoc for the individual patient.

The report of sleep disruption is a concern. There may be a level of cough, which does not amount to an exacerbation, yet still disrupts sleep. Amin et al.²³ have found that sleep quality in stable pediatric CF patients has been characterized by lower sleep efficiency and more frequent nocturnal awakenings than a control group of healthy children. Although these researchers did not find a causal relationship between cough and sleep disturbance, sleep duration and efficiency were associated with FEV₁. Sleep disordered breathing is of particular concern with CF patients who have moderate to severe lung disease. Further research is required to determine the various factors that impact sleep.²⁴ Perhaps sleep quality and efficiency would be an additional measurement to consider when evaluating the effects of symptoms on the quality of life of patients with CF.

This study was unique in its design, as the questionnaire included numerous open-ended questions and allowed for participants to enter responses about the types of pain they experienced and all the treatments they used to manage their symptoms. This produced diverse responses that may not have been obtained through a more prescriptive survey. Our study showed that patients are pursuing a variety of self-management strategies to address symptoms. In the absence of a systematic survey in CF clinics, the clinician may remain largely unaware of both the patient's symptoms and self-management strategies. Overall, this is an example of individuals managing their chronic illness, even in a health care system that provides few barriers to access care. Strategies to manage pain, dyspnea, and cough should be incorporated into clinical education for those with CF, so as to weave a clear program for self-management of these symptoms into the rest of the self-management strategies used by those with CF.

There are several limitations related to this study that require mention. The first is the recall bias that may exist with the self-report of symptoms in the month previous to the survey. To overcome this limitation, a prospective or longitudinal study would be required, using a daily diary for providing information about symptoms. The open-ended format that we used may have served to underestimate symptoms. The response rate of 64% is reasonable for a mail-based survey format, but may not be representative of the entire CF population. The results may not be generalizable to more diverse populations of CF patients, as this study took place in a defined geographical area. Use of a control group of healthy individuals without CF and their rating of symptoms experienced during a 30-day period would have been helpful to determine the prevalence in the general population.

This study contributes to the understanding of symptoms experienced by individuals living with CF. Future symptom-oriented research should be directed toward longitudinal research, prospective assessment research, and trials evaluating pharmacological and nonpharmacological strategies for relief. Intervention studies aimed at reducing symptom burden with the potential for improving function and quality of life in patients diagnosed with CF would be beneficial. Qualitative research focusing on understanding the symptoms of individuals with CF would provide greater detail of their lived experience.

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Acknowledgments 

Heartfelt thanks to the CF clinics in Nova Scotia, New Brunswick, and Prince Edward Island for their assistance with this project. The authors extend sincere thanks to Beth Bruce for her support with statistical analysis.

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