Perceived Sleep Quality in Individuals with Inborn Errors of Immunity

Perceived Sleep Quality in Individuals with Inborn Errors of Immunity | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Research Article Perceived Sleep Quality in Individuals with Inborn Errors of Immunity Kerri Sowers, Adel Sawaged, Brian Bowen This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-2440959/v1 This work is licensed under a CC BY 4.0 License Status: Published Journal Publication published 24 Mar, 2023 Read the published version in Journal of Clinical Immunology → Version 1 posted 5 You are reading this latest preprint version Abstract Purpose : Chronic sleep issues can lead to poor quality of life and increased mortality and patients with chronic health conditions often report impaired sleep quality. Higher levels of fatigue have been identified in patients diagnosed with Inborn Errors of Immunity (or Primary Immunodeficiency Diseases). This research sought to better understand perceived sleep quality in individuals diagnosed with IEI. Methods : A survey, which included the validated Sleep Quality Scale, was shared across multiple social media groups for individuals with a diagnosis of IEI. Results : Most of the participants were White/Caucasian females, between the ages of 30 to 74 years. The results of the Sleep Quality Scale suggest that this sample of individuals has moderate impairment of their sleep quality (71.8%), with a mean score of 43.0 (SD = 13.1). When comparing the results of the SQS to other patient populations and healthy control groups, the participants in this study had a poorer sleep quality score. Associations were identified between sleep quality and age, hours of sleep per night, time awake at night, times awake to urinate, attempted daytime naps, chronic pain, and mental health diagnoses. Conclusion : This survey suggests that individuals with Inborn Errors of Immunity have a moderate degree of perceived impairment in sleep quality. Healthcare providers are strongly encouraged to incorporate sleep quality screening in their routine assessments of patients with a diagnosis of Inborn Error of Immunity. Patients who are identified as having impaired sleep quality should be referred for further testing and interventions. Sleep quality impaired sleep fatigue Sleep Quality Scale Inborn Errors of Immunity Primary Immunodeficiency Disease Common Variable Immune Deficiency Introduction Inborn errors of immunity (IEI), which are also referred to in the literature as Primary Immunodeficiency Diseases (PID), are rare genetic disorders that affect innate and adaptive immunity. Clinically, IEI will present as an increased susceptibility to infections, autoimmunity, inflammation, allergies, bone marrow failure, and/or malignancy. As of 2022, the International Union of Immunological Societies (IUIS) Expert Committee has identified 485 known genetic defects, with 55 of them discovered in just the past year.[ 1 ] Inheritance may be recessive, autosomal dominant, or X-linked and there may be complete and incomplete penetrance of the clinical phenotype.[ 2 ] Based on a 2014 report published by Kobrynski et al.[ 3 ], an estimated 6 million people worldwide live with IEI, affecting 29.1–50.5 per 100,000 individuals. Common variable immunodeficiency (CVID) is a group of genetic disorders under the IEI umbrella which share a similar phenotype and is considered to be the most common symptomatic antibody deficiency diagnosed in adults with an estimated prevalence between 1:100,000 to 1:10,000.[ 4 ] There has been an expanding catalog of research into health-related quality of life for individuals with IEI. Individuals with an IEI diagnosis have been found to have challenges with physical function, anxiety, depression, fatigue, sleep disturbance, social participation, and pain as compared to the general population.[ 5 ] Recently, the association between fatigue and IEI was explored using a patient registry database. In a 2017 study, Hajjar et al.[ 6 ] found a significantly higher incidence of fatigue in patients with IEI (25.9%, CI 23.7–28.3) as compared to non-IEI individuals (6.4%, 95% CI 4.9–8.2). Individuals with a diagnosis of CVID had the highest prevalence of fatigue as compared to other IEI types.[ 6 ] Hajjar et al. also found that female sex, higher BMI, depression, bronchiectasis, and autoimmunity were factors associated with a higher degree of fatigue.[ 6 ] A 2020 study by Zhang et al. found significant differences between the CVID and non-CVID cohorts in the domains of anxiety, fatigue, and social participation as scored with the Patient-Reported Outcomes Measurement Information System (PROMIS-29).[ 5 ] A study of pediatric IEI patients found that fatigue negatively impacts quality of life and daily functioning, but was not associated with disease activity or comorbidity.[ 7 ] Given these more recent findings that substantiate fatigue as an issue in the IEI patient population, further investigation into sleep and sleep quality are necessary. Sleep is behavior that is characterized by changes in body posture and body state [ 8 ]; it is also a necessary physiological function to maintain health and well-being.[ 9 ] Nelson et al. (2022) defines sleep quality as an individual’s self-satisfaction with all aspects of the sleep experience.[ 10 ] Sleep quality can be impacted by diet, physical activity, and genetic and environmental factors.[ 9 ] Issues related to sleep and sleep quality are a growing global public health concern due to the association between impaired sleep and poor health outcomes.[ 10 ] There is a recognized association between poor sleep and decreased quality of life and increased mortality rates.[ 11 ] Sleep quality has been linked to health outcomes; poor sleep quality has been associated with higher incidence of cardiovascular disease, diabetes, depression, anxiety, obesity, stroke, and cancer.[ 10 , 12 ] Poor sleep quality may lead to increased fatigue, altered mood, decreased motivation, daytime dysfunction, cognitive impairment, irritability, poor concentration, poor dietary choices, increased alcohol or sleep medication use, and increased inflammation.[ 10 , 12 ] It is critically important that healthcare professionals who treat patients with a chronic disease, such as an IEI, consider the issue of sleep quality as part of a routine health assessment. Sleep disorders have been found to increase the risk of infectious diseases, are linked to the onset and progression of cardiovascular disease and cancer, and increase the incidence of depression.[ 8 ] In addition to influencing many body systems and functions, sleep also helps to regulate the adaptive and innate immune response.[ 9 ] Chronic sleep disturbance also leads to activation of the hypothalamus-pituitary-adrenal (HPA) axis and the sympathetic nervous system, which will impact the adaptive and innate immune responses.[ 8 ] Poor sleep quality is also a risk factor for the development of chronic widespread pain in otherwise healthy populations.[ 13 ] This makes the assessment and improvement of sleep quality in patients who are diagnosed with IEI even more important. In theory, improving sleep quality in patients diagnosed with IEI may help reduce inflammation, chronic pain and infections, and improve their overall immune response. Sleep can be measured objectively and subjectively. There are a wide variety of sleep surveys and questionnaires that allow for valid and reliable measurement of self-reported sleep quality. Technology has also improved the objective measurement of sleep through a variety of wearable devices and apps that measure sleep. This research study sought to better understand perceived sleep quality in individuals diagnosed with IEI. Methods This study was Institutional Review Board approved by Stockton University. The first survey question contained the informed consent information. Qualtrics was used as the survey platform to distribute the survey and collect the responses; the survey was available from April to June of 2022. The anonymous survey link was distributed to individuals with a diagnosis of IEI through multiple public and private social media patient support groups. Participants were eligible to participate if they were 18 years of age or older and had a diagnosis of an IEI. Participants were asked to complete the survey only one time. It was estimated that it would take 20 to 30 minutes to complete the survey. Survey questions were not randomized. Some questions allowed only one response, while others permitted multiple responses. The survey was compatible with any mobile device (such as a tablet or smartphone) or computer. Participants were able to change prior responses and could return to the survey if they were unable to complete it in one sitting. Research students completed the survey to test the survey flow. To improve face validity, the survey was completed by an individual with a diagnosis of IEI prior to sharing the survey with the broader IEI community. The Sleep Quality Scale (SQS) was developed to be an all-inclusive self-report to assess sleep quality during the previous month.[ 14 ] Yi et al. developed this 28-item, 6 factor scale using item and factor analysis; the 6 factors include daytime dysfunction, restoration after sleep, difficulty falling asleep, difficulty getting up, satisfaction with sleep, and difficulty maintaining sleep.[ 14 ] The SQS uses a 4-point Likert scale (0 = few, 1 = sometimes, 2 = often, and 3 = almost always); factors 2 and 5 are reverse-scored to obtain the global total score.[ 14 ] Scores on the SQS can range from 0 to 84, with higher scores representative of poorer sleep quality. The scale was determined to demonstrate reliability and validity. The concurrent validity was established through a significant correlation with the Pittsburgh Sleep Quality Index; the Cronbach’s alpha coefficient was 0.92 for internal consistency and the correlation coefficient was 0.81 for test-retest reliability at a 2-week interval.[ 14 ] Results A total of 555 responses were collected; 472 surveys were completed without any missing data. Partial responses were incorporated into the data analysis. Only responses that were complete were utilized to calculate the total SQS score. Participant demographics are presented in Tables 1 and 2. The beginning of the survey focused on the general demographic and health demographic questions presented in Tables 1 and 2. The survey then focused on questions related to sleep-related diagnoses, symptoms, medications, sleep hygiene and a variety of other sleep-related issues. First, the participants were asked if they had ever been formally diagnosed with a sleep disorder; they were permitted to check off as many responses as applied. Of the 548 who responded: 284 (51.8%) no sleep disorder; 156 (28.5%) sleep apnea; 113 (20.6%) insomnia; 12 (2.2%) narcolepsy; 68 (12.4%) restless leg syndrome; 8 (1.5%) sleep hypoventilation; 33 (6.0%) hypersomnia; 13 (2.4%) REM sleep behavior disorder; 7 (1.3%) circadian sleep rhythm disorder. Next, the participants were asked if they regularly (more than three times a week) experienced symptoms related to poor or disruptive sleep; they were permitted to check off multiple symptoms. Of the 548 who responded: 237 (43.2%) snoring; 227 (41.4%) bruxism; 89 (16.2%) sleep talking; 16 (2.9%) sleep walking; 93 (17.0%) nightmares; 29 (5.3%) night terrors; 192 (35.0%) vivid dreams; 97 (17.7%) none of the listed symptoms. The survey then sought to quantify prescription and non-prescription medication usage. Participants reported taking a prescription medication with a known side effect of fatigue or drowsiness: 246 (45.4%) yes, 240 (44.3%) no, and 56 (10.3%) unsure. Participants reported taking an over-the-counter medication with a known side effect of fatigue or drowsiness: 134 (24.7%) yes, 378 (69.7%) no, and 30 (5.5%) unsure. Participants reported taking a prescription medication with a known side effect of insomnia or alertness: 68 (12.5%) yes, 398 (73.4%) no, and 76 (14.0%) unsure. Participants reported taking an over-the-counter medication with a known side effect of insomnia or alertness: 21 (3.9%) yes, 477 (88.0%) no, and 44 (8.1%) unsure. Participants were asked if they were taking any prescription medication specifically for the purpose of achieving a better quality of sleep: 167 (30.8%) yes, 373 (68.8%) no, and 2 (0.4%) unsure. Participants were asked if they were taking any over-the-counter medication specifically for the purpose of achieving a better quality of sleep: 166 (30.6%) yes, 373 (68.8%) no, and 3 (0.6%) unsure. Given caffeine can have an impact similar to many medications, participants were asked how many cups of caffeinated drinks (coffee, tea, soda) they typically drank in a day. Of the 532 who responded: 91 (17.1%) none; 140 (26.3%) one cup; 176 (33.1%) two cups; 100 (18.8%) three to five cups; 25 (4.7%) six or more cups. Participants were asked how many cups of caffeinated drinks (coffee, tea, soda) they drank within two hours of going to sleep. Of the 532 who responded: 464 (87.2%) none; 52 (9.8%) one cup; 15 (2.8%) two cups; 1 (0.2%) three to five cups; 0 (0%) six or more cups. Sleep hygiene may have a significant impact on sleep quality. Participants were asked about their sleep hygiene practices; they were permitted to check off all that applied. Of the 548 who responded: 288 (52.6%) consistent sleep schedule; 233 (42.5%) relaxing bedtime/pre-bedtime routine; 368 (67.2%) dark and quiet bedroom; 431 (78.6%) cool/comfortable bedroom temperature; 169 (30.8%) electronics turned off in the bedroom; 56 (10.2%) avoid electronics one hour prior to bedtime; 335 (61.1%) avoid large meals and caffeine immediately prior to bedtime; 170 (31.0%) engage in regular physical activity; 211 (38.5%) avoid daytime naps; 22 (4.0%) no sleep hygiene practices used. The survey also sought to quantify the amount of sleep and awake time. First, participants were asked how many hours of sleep they typically get each night, including time spent lying awake in bed. Of the 536 who responded: 20 (3.7%) less than four hours, 78 (14.6%) four to five hours, 255 (47.6%) six to seven hours, 183 (34.1%) eight or more hours. Next, the participants were asked how long they were typically awake if they woke up during the night. Of the 533 who responded: 108 (20.3%) less than 5 minutes, 141 (26.5%) 6-19 minutes, 121 (22.7%) 20-59 minutes, 109 (20.5%) 1-2 hours, 54 (10.1%) more than 2 hours. Participants were then asked how many times they would wake up during the night to urinate. Of the 536 respondents: 118 (22.0%) none, 219 (40.9%) once, 118 (22.0%) twice, 53 (9.9%) three times, 28 (5.2) four or more times. Next, the participants were asked how many daytime naps (lasting five minutes or longer) they took on an average working day. Of the 530 respondents: 368 (69.4%) none, 154 (29.1%) one or two, 6 (1.1%) three or four, 2 (0.4%) five or more. Participants were asked how many daytime naps (lasting five minutes or longer) they attempted to take but had trouble falling asleep; of the 530 respondents: 428 (80.8%) reported none, 94 (17.7%) one or two, 6 (1.1%) three or four, and 2 (0.4%) five or more. The survey also explored the issues of pain, mental health, smoking and shift work, given that all of these could have a potential impact on sleep quality. First, the participants were asked about whether they experience chronic pain that interferes with their sleep. Of the 531 who responded: 310 (58.4%) yes, 204 (38.4%) no, 17 (3.2%) unsure. Next, the participants were asked about their mental health: 149 (28.1%) reported anxiety, 151 (28.5%) depression, 37 (7.0%) other mental health diagnosis, 193 (36.4%) no mental health diagnosis. Participants were then asked about their smoking history. Of the 529 who responded: 379 (71.6%) never smoked, 25 (4.7%) smoked for 1-2 years, 16 (3.0%) smoked for 3-5 years, 21 (4.0%) smoked for 6-10 years, 20 (3.8%) smoked for 11-15 years, 68 (12.9%) smoked for more than 15 years. Finally, the participants were asked about their work shifts. Of the 530 who responded: 321 (60.6%) are not currently working or are working but do not follow specific hours that would interfere with sleep; 13 (2.5%) work very early morning shifts, 173 (32.6%) work traditional day shifts, 10 (1.9%) work late afternoon/early evening shifts, 4 (0.8%) work night shifts, 9 (1.7%) work rotating shifts. There were 472 participants who completed the SQS; the mean score was 43.0 (SD = 13.1) with a range from 5.0 to 81.0. After converting the SQS to mild, moderate, and severe categories (developed by John et al. in 2022 [15]), 67 (14.2%) had mild impairment, 339 (71.8%) had moderate impairment, and 66 (14.0%) had severe impairment. A one sample t test was used to compare the results of the SQS for this sample of participants with normative data and with data collected from research done on populations with other diagnoses. In 2006, Yi et al. validated the SQS using individuals with a diagnosis of insomnia and a control group. [14] Yi et al. found the mean total SQS score for those with insomnia was 31.1 (SD = 13.61, n = 191) and for the control group the mean SQS was 15.8 (SD = 9.06, n = 332).[14] A one sample t test was used to compare the mean SQS score (43.0) from this sample of individuals with a IEI diagnosis to the two groups in the validation study. The mean SQS for individuals with IEI (43.0) was compared to the mean SQS for those with insomnia (31.1); there was a significant difference, t (471) = 19.773, p < 0.001. The mean SQS for individuals with IEI (43.0) was then compared to the mean SQS for the control group (15.8); again there was a significant difference, t (471) = 45.150, p < 0.001. In 2009, Yi et al. studied sleep quality in individuals with a diagnosis of obstructive sleep apnea syndrome (OSAS).[16] In this study, the OSAS group had a mean SQS score of 27.3 (SD = 10.95, n = 40) and the control group had a mean SQS of 9.7 (SD = 6.16, n = 37). The mean SQS for individuals with IEI (43.0) was compared to the mean SQS for those with OSAS (27.3); there was a significant difference, t (471) = 26.076, p < 0.001. The mean SQS for individuals with IEI (43.0) was then compared to the mean SQS for the control group (9.7); again there was a significant difference, t (471) = 55.268, p < 0.001. More recently, a 2022 study by Çakan and Öztürk examined sleep quality in patients with a diagnosis of allergic rhinitis using the SQS.[17] In this study, the allergic rhinitis group had a mean SQS score of 68.68 (SD = 13.15, n = 65) and the control group had a mean SQS of 47.72 (SD = 9.3, n = 65). The mean SQS for individuals with IEI (43.0) was compared to the mean SQS for those with allergic rhinitis (68.68); there was a significant difference, t (471) = -42.559, p < 0.001. The mean SQS for individuals with IEI (43.0) was then compared to the mean SQS for the control group (47.72); again there was a significant difference, t (471) = -7.794, p < 0.001. In another recent study, John et al. [15] examined sleep quality in patients who had been diagnosed with Covid-19. In this study, the participants had a mean SQS score of 28.89 (SD = 12.22, n = 782). The mean SQS for individuals with IEI (43.0) was compared to the mean SQS for those with Covid-19 (28.89); there was a significant difference, t (471) = 23.439, p < 0.001. A Chi Square Test of Independence was used to determine if there was any association between demographic or lifestyle factors that were included in the survey and sleep quality (using the SQS ranked as mild, moderate, or severe). Results are provided in Table 3. Discussion This survey sought to better understand the issue of perceived sleep quality in individuals who have a diagnosis of IEI. The majority of the participants were White/Caucasian females, between the ages of 30 to 74 years, with a diagnosis of CVID. Despite more than half of the participants (51.8%) reporting having no diagnosed sleep disorder, the results of the SQS suggest that this sample of individuals has moderate impairment of their sleep quality (71.8%), with a mean SQS score of 43.0 (SD = 13.1). When comparing the data collected in this survey to the data from Yi et al. [ 14 ] and Yi et al. [ 16 ], there is poorer sleep quality (based on the SQS) in those diagnosed with IEI as compared to both healthy control groups, those diagnosed with OSAS, and those diagnosed with insomnia. The participants in this study also had poorer sleep quality (based on the SQS) as compared to those diagnosed with Covid-19 in the study by John et al.[ 15 ] Interestingly, the participants in this study had better sleep quality (based on the SQS) as compared to both the control group and allergic rhinitis group in a 2022 study by Çakan and Öztürk.[ 17 ] However, the study by Çakan and Öztürk was exclusively male participants and this study was nearly all female participants, which may contribute to variations in the SQS scores.[ 17 ] Regardless of the comparisons made to other studies, it is evident that most of the participants in this study did have moderate impairment in their perceived sleep quality. A Chi Square Test of Independence was used to determine if there was any association between demographic or lifestyle factors that were included in the survey and sleep quality (using the SQS ranked as mild, moderate, or severe). Associations were identified between sleep quality and age, hours of sleep per night, time awake at night, times awake to urinate, attempted daytime naps, chronic pain, and mental health diagnoses. Given that a bidirectional relationship between sleep and the immune system has been established [ 18 ], the investigation into sleep quality in patients with an IEI diagnosis is even more critical. Healthcare providers should perform a detailed health assessment which incorporates assessment of sleep quality. Medications, pain, and mental health should be evaluated as part of a comprehensive sleep assessment. Validated tools, such as the SQS, Pittsburgh Sleep Quality Index, Sleep Disorders Questionnaire, or Functional Outcomes of Sleep Questionnaire can be used to track sleep quality over time and determine when to implement interventions to improve sleep quality. Sleep loss, reduced sleep duration, and sleep disturbance are associated with increases in inflammation.[ 13 ] Given that patients who are diagnosed with IEI are often at an already increased risk of elevated inflammatory biomarkers and autoinflammatory conditions, it is crucial to identify interventions to address issues that introduce additional inflammation. While pain had not been studied in IEI patient populations, 58.4% of the participants in this survey indicated that chronic pain was interfering with their sleep quality. Long-term poor sleep quality has been associated with increased risk of any chronic musculoskeletal pain and chronic widespread pain.[ 19 ] Healthcare providers should assess both acute and chronic pain, if present, and implement appropriate interventions for pain management. Changes in sleep quality may happen with aging, with many older adults reporting unsatisfactory sleep quality and quantity.[ 20 ] Structured moderate to vigorous physical activity is associated with improved sleep quality and lower self-reported fatigue levels in older adults.[ 11 ] In this study, only 31.0% reported that they engaged in regular physical activity. Individuals with an IEI diagnosis should be encouraged to participate in exercise and physical activity, as this may help their sleep quality, general health, and overall quality of life. In this survey, 28.1% participants reported anxiety, 28.5% reported depression, and 7.0% reported another mental health diagnosis. Given that more than half the participants reported a formally diagnosed mental health diagnosis, this is another important aspect that healthcare providers need to explore with their patient. A meta-analysis by Scott et al. [ 21 ] found a dose response relationship that greater improvements in sleep quality led to greater improvements in mental health and that improved sleep quality could contribute to reduced depression, anxiety, and stress. There may also be potential for the reverse, that improvements in mental health could lead to improved sleep quality. Addressing mental health issues may have a secondary effect of improving sleep quality for an individual IEI diagnosed patient. Managing a chronic health condition can significantly contribute to mental health challenges, making mental health assessment, referrals, and interventions especially important for patients with a diagnosis of IEI. Sleep hygiene is a group of behavioral and environmental recommendations to encourage quality sleep.[ 12 ] In this survey, the participants did engage in several sleep hygiene practices, but widespread adoption of multiple sleep hygiene practices was not reported. A dark and quiet bedroom was most often used (78.6%), followed by avoiding large meals and caffeine prior to bedtime (61.1%). Only 52.6% reported the use of a consistent sleep schedule and only 10.2% avoided electronic use one hour before bedtime. Increased use of smartphones and other electronic devices has worsened the epidemic of poor sleep.[ 22 ] While there has been general support for the implementation of sleep hygiene practices to improve sleep quality, studies have shown better outcomes for individual practices and less conclusive results for global implementation of sleep hygiene education.[ 12 ] Healthcare providers should explore the sleep hygiene practices their patient is utilizing and encourage them to expand their use of these methods. Those patients who are utilizing few or no sleep hygiene interventions may find an improvement in sleep quality after implementing these routines. While there are a variety of medications that can be used for those who have poor sleep quality, cognitive behavioral therapy (CBT) has been shown to be more effective than medication for treatment of insomnia.[ 20 ] While this has not been explored as an intervention for individuals with a diagnosis of IEI, there is minimal risk posed with the use of CBT. More research is needed on the use of CBT to improve sleep quality in patients with an IEI diagnosis. This study is limited in its generalizability to all IEI patients, given that 91.8% of participants were female, 83.8% had a diagnosis of CVID, and 95.5% were White/Caucasian. The demographics of the participants in this survey are not representative of the broader IEI patient population. However, given the number of respondents, the survey results are useful if evaluating this subgroup of patients. Future sleep quality assessments should be conducted across a broader patient demographic. This survey was also a sample of convenience, distributed through an online social media platform. Though social media surveys have advantages related to speed, cost, and accessibility, they do have a tendency to overrepresent young, white females, as was seen in this survey.[ 23 ] Surveys distributed through social media platforms are limited in accessibility to those who are members of the social media group. While this survey was distributed to multiple public and private IEI patient groups, the survey participants are limited to those who are actively engaging in those social media groups. Online social media surveys do not capture adequate representation of those who lack internet service, the elderly, low income, or those living in remote locations.[ 24 ] It would be beneficial to have a broader distribution of the survey that allows participation by those who do not have online access; distribution of the survey could be expanded through trusted healthcare providers to reach a wider demographic of IEI patients. All individuals with a diagnosis of IEI were encouraged to participate in the survey, regardless of their sleep or sleep quality. However, online surveys are at risk for sample bias and overrepresentation of the specific issue being explored.[ 24 ] This survey, though limited in generalizability, suggests that individuals with IEI (specifically White/Caucasian females with a diagnosis of CVID) have a moderate degree of perceived impairment in sleep quality. Healthcare providers are strongly encouraged to incorporate sleep quality screening in their routine assessments of patients with an IEI diagnosis. Valid and reliable tools should be used to track sleep quality over time and can assist in the determination of when to implement interventions to improve sleep quality. For patients with significant impairments in sleep quality, referrals should be made for diagnostic assessments, which may include polysomnogram, multiple sleep latency testing, or a maintenance of wakefulness testing. Referrals can then be made to specialists based on the aspect of sleep that is identified as impaired. Additionally, it is important to assess mental health and chronic pain in this patient population. Further interventions or referrals related to those areas should be made. Sleep and sleep quality is a critical aspect of health that can impact quality of life, daily functioning, and long-term health outcomes. Assessing and providing appropriate interventions to improve sleep quality can make a lasting impact on IEI patient health and wellness. Statements & Disclosures This research study did not have any internal or external funding. The authors report they have no conflicts of interest to disclose. Funding: The authors declare that no grants or funds were received to support this research or manuscript preparation. Conflicts of interest/Competing interests: The authors have no relevant financial or non-financial interests to disclose. Availability of data and material: The authors are willing to share the original survey data collected in this study upon request. Authors' contributions: All authors were involved in the development of the study design. Data analysis and the first draft of the manuscript was completed by Dr. Sowers. All authors were involved in the editing of the manuscript and review of the final manuscript. Ethics approval: This research study was IRB approved through Stockton University. Consent to participate: Informed consent was obtained from all participants in this research. Consent for publication: Consent for publication was obtained from all participants in this research. References Tangye SG, Al-Herz W, Bousfiha A, Cunningham-Rundles C, Franco JL, Holland S, et al. Human Inborn Errors of Immunity: 2022 Update on the Classification from the International Union of Immunological Societies Expert Committee. J Clin Immunol. 2022;42(7):1473–507. https://doi.org/10.1007/s10875-022-01289-3 . Bousfiha A, Moundir A, Tangye SG, Picard C, Jeddane L, Al-Herz W, et al. The 2022 Update of IUIS Phenotypical Classification for Human Inborn Errors of Immunity. J Clin Immunol. 2022;42(7):1508–20. https://doi.org/10.1007/s10875-022-01352-z . Kobrynski L, Powell RW, Bowen S. Prevalence and morbidity of primary immunodeficiency diseases, United States 2001–2007. J Clin Immunol. 2014;34(8):954–61. https://doi.org/10.1007/s10875-014-0102-8 . Bonilla FA. Personalized therapy for common variable immunodeficiency. Allergy and asthma proceedings . 2020;41(1),19–25. https://doi.org/10.2500/aap.2020.41.190012 Zhang S, Kline M, Fuleihan RL, Consortium U, Scalchunes C, Sullivan KE, et al. PROMIS-29 survey confirms major impact of fatigue on health-related quality of life in common variable immunodeficiency. Immunol Res. 2020;68(6):379–88. https://doi.org/10.1007/s12026-020-09162-4 . Hajjar J, Guffey D, Minard CG, Orange JS. Increased Incidence of Fatigue in Patients with Primary Immunodeficiency Disorders: Prevalence and Associations Within the US Immunodeficiency Network Registry. J Clin Immunol. 2017;37(2):153–65. https://doi.org/10.1007/s10875-016-0367-1 . Nijhof LN, van Brussel M, Pots EM, van Litsenburg RRL, van de Putte EM, van Montfrans JM, et al. Severe Fatigue Is Common Among Pediatric Patients with Primary Immunodeficiency and Is Not Related to Disease Activity. J Clin Immunol. 2021;41(6):1198–207. https://doi.org/10.1007/s10875-021-01013-7 . Irwin MR. Why sleep is important for health: a psychoneuroimmunology perspective. Ann Rev Psychol. 2015;66:143–72. https://doi.org/10.1146/annurev-psych-010213-115205 . Sejbuk M, Mirończuk-Chodakowska I, Witkowska AM. Sleep Quality: A Narrative Review on Nutrition, Stimulants, and Physical Activity as Important Factors. Nutrients. 2022;14(9):1912. https://doi.org/10.3390/nu14091912 . Nelson KL, Davis JE, Corbett CF. Sleep quality: An evolutionary concept analysis. Nurs fórum. 2022;57(1):144–51. https://doi.org/10.1111/nuf.12659 . Christie AD, Seery E, Kent JA. Physical activity, sleep quality, and self-reported fatigue across the adult lifespan. Exp Gerontol. 2016;77:7–11. https://doi.org/10.1016/j.exger.2016.02.001 . Irish LA, Kline CE, Gunn HE, Buysse DJ, Hall MH. The role of sleep hygiene in promoting public health: A review of empirical evidence.Sleep medicine reviews. 3015;22,23–36. https://doi.org/10.1016/j.smrv.2014.10.001 Choy EH. The role of sleep in pain and fibromyalgia. Nat Rev Rheumatol. 2015;11(9):513–20. https://doi.org/10.1038/nrrheum.2015.56 . Yi H, Shin K, Shin C. Development of the sleep quality scale. J Sleep Res. 2006;15(3):309–16. https://doi.org/10.1111/j.1365-2869.2006.00544.x . John B, Marath U, Valappil SP, Mathew D, Renjitha M. Sleep Pattern Changes and the Level of Fatigue Reported in a Community Sample of Adults During COVID-19 Pandemic. Sleep and vigilance. 2022;1–16. https://doi.org/10.1007/s41782-022-00210-7 . Advance online publication. Yi H, Shin K, Kim J, Kim J, Lee J, Shin C. Validity and reliability of Sleep Quality Scale in subjects with obstructive sleep apnea syndrome. J Psychosom Res. 2009;66(1):85–8. https://doi.org/10.1016/j.jpsychores.2008.07.008 . Çakan D, Öztürk E. The Effects of Allergic Rhinitis on Sleep Quality. J Acad Res Med. 2022;12(1). https://doi.org/10.4274/jarem.galenos.2021.28199 . Besedovsky L, Lange T, Haack M. The Sleep-Immune Crosstalk in Health and Disease. Physiol Rev. 2019;99(3):1325–80. https://doi.org/10.1152/physrev.00010.2018 . Skarpsno ES, Nilsen T, Hagen K, Mork PJ. Long-term changes in self-reported sleep quality and risk of chronic musculoskeletal pain: The HUNT Study. J Sleep Res. 2021;30(6):e13354. https://doi.org/10.1111/jsr.13354 . Brewster GS, Riegel B, Gehrman PR. Insomnia in the Older Adult. Sleep Med Clin. 2018;13(1):13–9. https://doi.org/10.1016/j.jsmc.2017.09.002 . Scott AJ, Webb TL, Martyn-St James M, Rowse G, Weich S. Improving sleep quality leads to better mental health: A meta-analysis of randomised controlled trials. Sleep Med Rev. 2021;60:101556. https://doi.org/10.1016/j.smrv.2021.101556 . Chattu VK, Manzar MD, Kumary S, Burman D, Spence DW, Pandi-Perumal SR. The Global Problem of Insufficient Sleep and Its Serious Public Health Implications. Healthcare. 2018;7(1):1. https://doi.org/10.3390/healthcare7010001 . Whitaker C, Stevelink S, Fear N. The use of Facebook in recruiting participants for health research purposes: a systematic review. J Med Internet Res. 2017;19(8):e290. https://doi.org/10.2196/jmir.7071 . Ball HL. Conducting Online Surveys. J Hum lactation. 2019;35(3):413–7. https://doi.org/10.1177/0890334419848734 . Tables Tables 1 to 3 are available in the Supplementary Files section Supplementary Files Tables.docx Cite Share Download PDF Status: Published Journal Publication published 24 Mar, 2023 Read the published version in Journal of Clinical Immunology → Version 1 posted Reviewers agreed at journal 18 Jan, 2023 Reviewers invited by journal 09 Jan, 2023 Editor invited by journal 08 Jan, 2023 Editor assigned by journal 06 Jan, 2023 First submitted to journal 04 Jan, 2023 You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. We do this by developing innovative software and high quality services for the global research community. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-2440959","acceptedTermsAndConditions":true,"allowDirectSubmit":false,"archivedVersions":[],"articleType":"Research Article","associatedPublications":[],"authors":[{"id":166252764,"identity":"98ab754a-6a0a-4a3d-a0e5-51f7500cf041","order_by":0,"name":"Kerri Sowers","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA/ElEQVRIie3Qu2rDMBSA4WMOaFLJKiOivoLgQOkQ2leJKHgLdMxkAoFkiTu7b5Eps4shXly6dmuLwbMhSwZDKxHSKQJ366B/EJLgQxeAUOgfJtwwPa86N/DTPFoMIVEO2hIcQM4hH0LiddZ2n5AqXWXNYdL3arR5e2keYTLeFpeJ5BXlUyhJ19WNnK00idclUg4J+YgSCdq3FGb3njCcLbTZjpBJDqXxkuvWkdTsPlo83Pa/5NtLpGCOoD2FgQRmydXSkcJL4o27mC7pvk5YnK2IRF0Scf1Azx4iqj1Gx3mq4vUeu2Ov7I+Zr4bP78ZPHnJKD9gJhUKh0B/6AYEZUfSfmMqWAAAAAElFTkSuQmCC","orcid":"https://orcid.org/0000-0001-5456-5144","institution":"Stockton University","correspondingAuthor":true,"submittingAuthor":false,"prefix":"","firstName":"Kerri","middleName":"","lastName":"Sowers","suffix":""},{"id":166252765,"identity":"aef8f240-162a-4b87-b603-22c206a2db6d","order_by":1,"name":"Adel Sawaged","email":"","orcid":"","institution":"","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Adel","middleName":"","lastName":"Sawaged","suffix":""},{"id":166252766,"identity":"37186121-e8c8-4262-b0c3-0cb8e5751d11","order_by":2,"name":"Brian Bowen","email":"","orcid":"","institution":"","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Brian","middleName":"","lastName":"Bowen","suffix":""}],"badges":[],"createdAt":"2023-01-04 03:22:52","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-2440959/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-2440959/v1","draftVersion":[],"editorialEvents":[{"content":"https://doi.org/10.1007/s10875-023-01474-y","type":"published","date":"2023-03-24T20:05:46+00:00"}],"editorialNote":"","failedWorkflow":false,"files":[{"id":44723299,"identity":"6018c075-2a6b-4d8d-8ba5-bc5a7b03e3fe","added_by":"auto","created_at":"2023-10-16 20:15:29","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":220231,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-2440959/v1/6c5f83a2-4d8a-40b0-85b7-68a2d63f64a0.pdf"},{"id":31413350,"identity":"5ee2fd1d-0bac-4399-aaf8-05242d6dd615","added_by":"auto","created_at":"2023-01-11 09:48:35","extension":"docx","order_by":1,"title":"","display":"","copyAsset":false,"role":"supplement","size":15524,"visible":true,"origin":"","legend":"","description":"","filename":"Tables.docx","url":"https://assets-eu.researchsquare.com/files/rs-2440959/v1/035295193a257578e2b59c60.docx"}],"financialInterests":"","formattedTitle":"Perceived Sleep Quality in Individuals with Inborn Errors of Immunity","fulltext":[{"header":"Introduction","content":"\u003cp\u003eInborn errors of immunity (IEI), which are also referred to in the literature as Primary Immunodeficiency Diseases (PID), are rare genetic disorders that affect innate and adaptive immunity. Clinically, IEI will present as an increased susceptibility to infections, autoimmunity, inflammation, allergies, bone marrow failure, and/or malignancy. As of 2022, the International Union of Immunological Societies (IUIS) Expert Committee has identified 485 known genetic defects, with 55 of them discovered in just the past year.[\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e] Inheritance may be recessive, autosomal dominant, or X-linked and there may be complete and incomplete penetrance of the clinical phenotype.[\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e] Based on a 2014 report published by Kobrynski et al.[\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e], an estimated 6\u0026nbsp;million people worldwide live with IEI, affecting 29.1\u0026ndash;50.5 per 100,000 individuals. Common variable immunodeficiency (CVID) is a group of genetic disorders under the IEI umbrella which share a similar phenotype and is considered to be the most common symptomatic antibody deficiency diagnosed in adults with an estimated prevalence between 1:100,000 to 1:10,000.[\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e] There has been an expanding catalog of research into health-related quality of life for individuals with IEI. Individuals with an IEI diagnosis have been found to have challenges with physical function, anxiety, depression, fatigue, sleep disturbance, social participation, and pain as compared to the general population.[\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e] Recently, the association between fatigue and IEI was explored using a patient registry database. In a 2017 study, Hajjar et al.[\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e] found a significantly higher incidence of fatigue in patients with IEI (25.9%, CI 23.7\u0026ndash;28.3) as compared to non-IEI individuals (6.4%, 95% CI 4.9\u0026ndash;8.2). Individuals with a diagnosis of CVID had the highest prevalence of fatigue as compared to other IEI types.[\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e] Hajjar et al. also found that female sex, higher BMI, depression, bronchiectasis, and autoimmunity were factors associated with a higher degree of fatigue.[\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e] A 2020 study by Zhang et al. found significant differences between the CVID and non-CVID cohorts in the domains of anxiety, fatigue, and social participation as scored with the Patient-Reported Outcomes Measurement Information System (PROMIS-29).[\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e] A study of pediatric IEI patients found that fatigue negatively impacts quality of life and daily functioning, but was not associated with disease activity or comorbidity.[\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e] Given these more recent findings that substantiate fatigue as an issue in the IEI patient population, further investigation into sleep and sleep quality are necessary.\u003c/p\u003e \u003cp\u003eSleep is behavior that is characterized by changes in body posture and body state [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e]; it is also a necessary physiological function to maintain health and well-being.[\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e] Nelson et al. (2022) defines sleep quality as an individual\u0026rsquo;s self-satisfaction with all aspects of the sleep experience.[\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e] Sleep quality can be impacted by diet, physical activity, and genetic and environmental factors.[\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e] Issues related to sleep and sleep quality are a growing global public health concern due to the association between impaired sleep and poor health outcomes.[\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e] There is a recognized association between poor sleep and decreased quality of life and increased mortality rates.[\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e] Sleep quality has been linked to health outcomes; poor sleep quality has been associated with higher incidence of cardiovascular disease, diabetes, depression, anxiety, obesity, stroke, and cancer.[\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e, \u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e] Poor sleep quality may lead to increased fatigue, altered mood, decreased motivation, daytime dysfunction, cognitive impairment, irritability, poor concentration, poor dietary choices, increased alcohol or sleep medication use, and increased inflammation.[\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e, \u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e] It is critically important that healthcare professionals who treat patients with a chronic disease, such as an IEI, consider the issue of sleep quality as part of a routine health assessment. Sleep disorders have been found to increase the risk of infectious diseases, are linked to the onset and progression of cardiovascular disease and cancer, and increase the incidence of depression.[\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e] In addition to influencing many body systems and functions, sleep also helps to regulate the adaptive and innate immune response.[\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e] Chronic sleep disturbance also leads to activation of the hypothalamus-pituitary-adrenal (HPA) axis and the sympathetic nervous system, which will impact the adaptive and innate immune responses.[\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e] Poor sleep quality is also a risk factor for the development of chronic widespread pain in otherwise healthy populations.[\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e] This makes the assessment and improvement of sleep quality in patients who are diagnosed with IEI even more important. In theory, improving sleep quality in patients diagnosed with IEI may help reduce inflammation, chronic pain and infections, and improve their overall immune response. Sleep can be measured objectively and subjectively. There are a wide variety of sleep surveys and questionnaires that allow for valid and reliable measurement of self-reported sleep quality. Technology has also improved the objective measurement of sleep through a variety of wearable devices and apps that measure sleep. This research study sought to better understand perceived sleep quality in individuals diagnosed with IEI.\u003c/p\u003e"},{"header":"Methods","content":"\u003cp\u003e This study was Institutional Review Board approved by Stockton University. The first survey question contained the informed consent information. Qualtrics was used as the survey platform to distribute the survey and collect the responses; the survey was available from April to June of 2022. The anonymous survey link was distributed to individuals with a diagnosis of IEI through multiple public and private social media patient support groups. Participants were eligible to participate if they were 18 years of age or older and had a diagnosis of an IEI. Participants were asked to complete the survey only one time. It was estimated that it would take 20 to 30 minutes to complete the survey. Survey questions were not randomized. Some questions allowed only one response, while others permitted multiple responses. The survey was compatible with any mobile device (such as a tablet or smartphone) or computer. Participants were able to change prior responses and could return to the survey if they were unable to complete it in one sitting. Research students completed the survey to test the survey flow. To improve face validity, the survey was completed by an individual with a diagnosis of IEI prior to sharing the survey with the broader IEI community.\u003c/p\u003e \u003cp\u003eThe Sleep Quality Scale (SQS) was developed to be an all-inclusive self-report to assess sleep quality during the previous month.[\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e] Yi et al. developed this 28-item, 6 factor scale using item and factor analysis; the 6 factors include daytime dysfunction, restoration after sleep, difficulty falling asleep, difficulty getting up, satisfaction with sleep, and difficulty maintaining sleep.[\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e] The SQS uses a 4-point Likert scale (0\u0026thinsp;=\u0026thinsp;few, 1\u0026thinsp;=\u0026thinsp;sometimes, 2\u0026thinsp;=\u0026thinsp;often, and 3\u0026thinsp;=\u0026thinsp;almost always); factors 2 and 5 are reverse-scored to obtain the global total score.[\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e] Scores on the SQS can range from 0 to 84, with higher scores representative of poorer sleep quality. The scale was determined to demonstrate reliability and validity. The concurrent validity was established through a significant correlation with the Pittsburgh Sleep Quality Index; the Cronbach\u0026rsquo;s alpha coefficient was 0.92 for internal consistency and the correlation coefficient was 0.81 for test-retest reliability at a 2-week interval.[\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e]\u003c/p\u003e"},{"header":"Results","content":"\u003cp\u003eA total of 555 responses were collected; 472 surveys were completed without any missing data. Partial responses were incorporated into the data analysis. Only responses that were complete were utilized to calculate the total SQS score. Participant demographics are presented in Tables 1 and 2. \u0026nbsp;\u003c/p\u003e\n\u003cp\u003eThe beginning of the survey focused on the general demographic and health demographic questions presented in Tables 1 and 2. \u0026nbsp;The survey then focused on questions related to sleep-related diagnoses, symptoms, medications, sleep hygiene and a variety of other sleep-related issues. First, the participants were asked if they had ever been formally diagnosed with a sleep disorder; they were permitted to check off as many responses as applied. Of the 548 who responded: 284 (51.8%) no sleep disorder; 156 (28.5%) sleep apnea; 113 (20.6%) insomnia; 12 (2.2%) narcolepsy; 68 (12.4%) restless leg syndrome; 8 (1.5%) sleep hypoventilation; 33 (6.0%) hypersomnia; 13 (2.4%) REM sleep behavior disorder; 7 (1.3%) circadian sleep rhythm disorder. Next, the participants were asked if they regularly (more than three times a week) experienced symptoms related to poor or disruptive sleep; they were permitted to check off multiple symptoms. Of the 548 who responded: 237 (43.2%) snoring; 227 (41.4%) bruxism; 89 (16.2%) sleep talking; 16 (2.9%) sleep walking; 93 (17.0%) nightmares; 29 (5.3%) night terrors; 192 (35.0%) vivid dreams; 97 (17.7%) none of the listed symptoms.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eThe survey then sought to quantify prescription and non-prescription medication usage. Participants reported taking a prescription medication with a known side effect of fatigue or drowsiness: 246 (45.4%) yes, 240 (44.3%) no, and 56 (10.3%) unsure. Participants reported taking an over-the-counter medication with a known side effect of fatigue or drowsiness: 134 (24.7%) yes, 378 (69.7%) no, and 30 (5.5%) unsure. Participants reported taking a prescription medication with a known side effect of insomnia or alertness: 68 (12.5%) yes, 398 (73.4%) no, and 76 (14.0%) unsure. Participants reported taking an over-the-counter medication with a known side effect of insomnia or alertness: 21 (3.9%) yes, 477 (88.0%) no, and 44 (8.1%) unsure. \u0026nbsp; Participants were asked if they were taking any prescription medication specifically for the purpose of achieving a better quality of sleep: 167 (30.8%) yes, 373 (68.8%) no, and 2 (0.4%) unsure. Participants were asked if they were taking any over-the-counter medication specifically for the purpose of achieving a better quality of sleep: 166 (30.6%) yes, 373 (68.8%) no, and 3 (0.6%) unsure. Given caffeine can have an impact similar to many medications, participants were asked how many cups of caffeinated drinks (coffee, tea, soda) they typically drank in a day. Of the 532 who responded: 91 (17.1%) none; 140 (26.3%) one cup; 176 (33.1%) two cups; 100 (18.8%) three to five cups; 25 (4.7%) six or more cups. Participants were asked how many cups of caffeinated drinks (coffee, tea, soda) they drank within two hours of going to sleep. Of the 532 who responded: 464 (87.2%) none; 52 (9.8%) one cup; 15 (2.8%) two cups; 1 (0.2%) three to five cups; 0 (0%) \u0026nbsp;six or more cups.\u003c/p\u003e\n\u003cp\u003eSleep hygiene may have a significant impact on sleep quality. Participants were asked about their sleep hygiene practices; they were permitted to check off all that applied. Of the 548 who responded: 288 (52.6%) consistent sleep schedule; 233 (42.5%) relaxing bedtime/pre-bedtime routine; 368 (67.2%) dark and quiet bedroom; 431 (78.6%) cool/comfortable bedroom temperature; 169 (30.8%) electronics turned off in the bedroom; 56 (10.2%) avoid electronics one hour prior to bedtime; 335 (61.1%) avoid large meals and caffeine immediately prior to bedtime; 170 (31.0%) engage in regular physical activity; 211 (38.5%) avoid daytime naps; 22 (4.0%) no sleep hygiene practices used.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eThe survey also sought to quantify the amount of sleep and awake time. First, participants were asked how many hours of sleep they typically get each night, including time spent lying awake in bed. Of the 536 who responded: 20 (3.7%) less than four hours, 78 (14.6%) four to five hours, 255 (47.6%) six to seven hours, 183 (34.1%) eight or more hours. Next, the participants were asked how long they were typically awake if they woke up during the night. Of the 533 who responded: 108 (20.3%) less than 5 minutes, 141 (26.5%) 6-19 minutes, 121 (22.7%) 20-59 minutes, 109 (20.5%) 1-2 hours, 54 (10.1%) more than 2 hours. Participants were then asked how many times they would wake up during the night to urinate. Of the 536 respondents: 118 (22.0%) none, 219 (40.9%) once, 118 (22.0%) twice, 53 (9.9%) three times, 28 (5.2) four or more times. Next, the participants were asked how many daytime naps (lasting five minutes or longer) they took on an average working day. Of the 530 respondents: 368 (69.4%) none, 154 (29.1%) one or two, 6 (1.1%) three or four, 2 (0.4%) five or more. Participants were asked how many daytime naps (lasting five minutes or longer) they attempted to take but had trouble falling asleep; of the 530 respondents: 428 (80.8%) reported none, 94 (17.7%) one or two, 6 (1.1%) three or four, and 2 (0.4%) five or more.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eThe survey also explored the issues of pain, mental health, smoking and shift work, given that all of these could have a potential impact on sleep quality. First, the participants were asked about whether they experience chronic pain that interferes with their sleep. Of the 531 who responded: 310 (58.4%) yes, 204 (38.4%) no, 17 (3.2%) unsure. Next, the participants were asked about their mental health: 149 (28.1%) reported anxiety, 151 (28.5%) depression, 37 (7.0%) other mental health diagnosis, 193 (36.4%) no mental health diagnosis. Participants were then asked about their smoking history. Of the 529 who responded: 379 (71.6%) never smoked, 25 (4.7%) smoked for 1-2 years, 16 (3.0%) smoked for 3-5 years, 21 (4.0%) smoked for 6-10 years, 20 (3.8%) smoked for 11-15 years, 68 (12.9%) smoked for more than 15 years. Finally, the participants were asked about their work shifts. Of the 530 who responded: 321 (60.6%) are not currently working or are working but do not follow specific hours that would interfere with sleep; 13 (2.5%) work very early morning shifts, 173 (32.6%) work traditional day shifts, 10 (1.9%) work late afternoon/early evening shifts, 4 (0.8%) work night shifts, 9 (1.7%) work rotating shifts.\u003c/p\u003e\n\u003cp\u003eThere were 472 participants who completed the SQS; the mean score was 43.0 (SD = 13.1) with a range from 5.0 to 81.0. After converting the SQS to mild, moderate, and severe categories (developed by John et al. in 2022 [15]), 67 (14.2%) had mild impairment, 339 (71.8%) had moderate impairment, and 66 (14.0%) had severe impairment.\u003c/p\u003e\n\u003cp\u003eA one sample \u003cem\u003et\u0026nbsp;\u003c/em\u003etest was used to compare the results of the SQS for this sample of participants with normative data and with data collected from research done on populations with other diagnoses. \u0026nbsp;In 2006, Yi et al. validated the SQS using individuals with a diagnosis of insomnia and a control group. [14] Yi et al. found the mean total SQS score for those with insomnia was 31.1 (SD = 13.61, n = 191) and for the control group the mean SQS was 15.8 (SD = 9.06, n = 332).[14] A one sample t test was used to compare the mean SQS score (43.0) from this sample of individuals with a IEI diagnosis to the two groups in the validation study. The mean SQS for individuals with IEI (43.0) was compared to the mean SQS for those with insomnia (31.1); there was a significant difference,\u003cem\u003e\u0026nbsp;t\u003c/em\u003e(471) = 19.773, p \u0026lt; 0.001. The mean SQS for individuals with IEI (43.0) was then compared to the mean SQS for the control group (15.8); again there was a significant difference, \u003cem\u003et\u003c/em\u003e(471) = 45.150, p \u0026lt; 0.001. In 2009, Yi et al. studied sleep quality in individuals with a diagnosis of obstructive sleep apnea syndrome (OSAS).[16] In this study, the OSAS group had a mean SQS score of 27.3 (SD = 10.95, n = 40) and the control group had a mean SQS of 9.7 (SD = 6.16, n = 37). The mean SQS for individuals with IEI (43.0) was compared to the mean SQS for those with OSAS (27.3); there was a significant difference,\u003cem\u003e\u0026nbsp;t\u003c/em\u003e(471) = 26.076, p \u0026lt; 0.001. The mean SQS for individuals with IEI (43.0) was then compared to the mean SQS for the control group (9.7); again there was a significant difference, \u003cem\u003et\u003c/em\u003e(471) = 55.268, p \u0026lt; 0.001.\u003c/p\u003e\n\u003cp\u003eMore recently, a 2022 study by Çakan and Öztürk examined sleep quality in patients with a diagnosis of allergic rhinitis using the SQS.[17] In this study, the allergic rhinitis group had a mean SQS score of 68.68 (SD = 13.15, n = 65) and the control group had a mean SQS of 47.72 (SD = 9.3, n = 65). The mean SQS for individuals with IEI (43.0) was compared to the mean SQS for those with allergic rhinitis (68.68); there was a significant difference,\u003cem\u003e\u0026nbsp;t\u003c/em\u003e(471) = -42.559, p \u0026lt; 0.001. The mean SQS for individuals with IEI (43.0) was then compared to the mean SQS for the control group (47.72); again there was a significant difference, \u003cem\u003et\u003c/em\u003e(471) = -7.794, p \u0026lt; 0.001. In another recent study, John et al. [15] examined sleep quality in patients who had been diagnosed with Covid-19. In this study, the participants had a mean SQS score of 28.89 (SD = 12.22, n = 782). The mean SQS for individuals with IEI (43.0) was compared to the mean SQS for those with Covid-19 (28.89); there was a significant difference,\u003cem\u003e\u0026nbsp;t\u003c/em\u003e(471) = 23.439, p \u0026lt; 0.001.\u003c/p\u003e\n\u003cp\u003eA Chi Square Test of Independence was used to determine if there was any association between demographic or lifestyle factors that were included in the survey and sleep quality (using the SQS ranked as mild, moderate, or severe). Results are provided in Table 3.\u0026nbsp;\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eThis survey sought to better understand the issue of perceived sleep quality in individuals who have a diagnosis of IEI. The majority of the participants were White/Caucasian females, between the ages of 30 to 74 years, with a diagnosis of CVID. Despite more than half of the participants (51.8%) reporting having no diagnosed sleep disorder, the results of the SQS suggest that this sample of individuals has moderate impairment of their sleep quality (71.8%), with a mean SQS score of 43.0 (SD\u0026thinsp;=\u0026thinsp;13.1). When comparing the data collected in this survey to the data from Yi et al. [\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e] and Yi et al. [\u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e], there is poorer sleep quality (based on the SQS) in those diagnosed with IEI as compared to both healthy control groups, those diagnosed with OSAS, and those diagnosed with insomnia. The participants in this study also had poorer sleep quality (based on the SQS) as compared to those diagnosed with Covid-19 in the study by John et al.[\u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e] Interestingly, the participants in this study had better sleep quality (based on the SQS) as compared to both the control group and allergic rhinitis group in a 2022 study by \u0026Ccedil;akan and \u0026Ouml;zt\u0026uuml;rk.[\u003cspan citationid=\"CR17\" class=\"CitationRef\"\u003e17\u003c/span\u003e] However, the study by \u0026Ccedil;akan and \u0026Ouml;zt\u0026uuml;rk was exclusively male participants and this study was nearly all female participants, which may contribute to variations in the SQS scores.[\u003cspan citationid=\"CR17\" class=\"CitationRef\"\u003e17\u003c/span\u003e] Regardless of the comparisons made to other studies, it is evident that most of the participants in this study did have moderate impairment in their perceived sleep quality. A Chi Square Test of Independence was used to determine if there was any association between demographic or lifestyle factors that were included in the survey and sleep quality (using the SQS ranked as mild, moderate, or severe). Associations were identified between sleep quality and age, hours of sleep per night, time awake at night, times awake to urinate, attempted daytime naps, chronic pain, and mental health diagnoses.\u003c/p\u003e \u003cp\u003eGiven that a bidirectional relationship between sleep and the immune system has been established [\u003cspan citationid=\"CR18\" class=\"CitationRef\"\u003e18\u003c/span\u003e], the investigation into sleep quality in patients with an IEI diagnosis is even more critical. Healthcare providers should perform a detailed health assessment which incorporates assessment of sleep quality. Medications, pain, and mental health should be evaluated as part of a comprehensive sleep assessment. Validated tools, such as the SQS, Pittsburgh Sleep Quality Index, Sleep Disorders Questionnaire, or Functional Outcomes of Sleep Questionnaire can be used to track sleep quality over time and determine when to implement interventions to improve sleep quality.\u003c/p\u003e \u003cp\u003eSleep loss, reduced sleep duration, and sleep disturbance are associated with increases in inflammation.[\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e] Given that patients who are diagnosed with IEI are often at an already increased risk of elevated inflammatory biomarkers and autoinflammatory conditions, it is crucial to identify interventions to address issues that introduce additional inflammation. While pain had not been studied in IEI patient populations, 58.4% of the participants in this survey indicated that chronic pain was interfering with their sleep quality. Long-term poor sleep quality has been associated with increased risk of any chronic musculoskeletal pain and chronic widespread pain.[\u003cspan citationid=\"CR19\" class=\"CitationRef\"\u003e19\u003c/span\u003e] Healthcare providers should assess both acute and chronic pain, if present, and implement appropriate interventions for pain management.\u003c/p\u003e \u003cp\u003eChanges in sleep quality may happen with aging, with many older adults reporting unsatisfactory sleep quality and quantity.[\u003cspan citationid=\"CR20\" class=\"CitationRef\"\u003e20\u003c/span\u003e] Structured moderate to vigorous physical activity is associated with improved sleep quality and lower self-reported fatigue levels in older adults.[\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e] In this study, only 31.0% reported that they engaged in regular physical activity. Individuals with an IEI diagnosis should be encouraged to participate in exercise and physical activity, as this may help their sleep quality, general health, and overall quality of life. In this survey, 28.1% participants reported anxiety, 28.5% reported depression, and 7.0% reported another mental health diagnosis. Given that more than half the participants reported a formally diagnosed mental health diagnosis, this is another important aspect that healthcare providers need to explore with their patient. A meta-analysis by Scott et al. [\u003cspan citationid=\"CR21\" class=\"CitationRef\"\u003e21\u003c/span\u003e] found a dose response relationship that greater improvements in sleep quality led to greater improvements in mental health and that improved sleep quality could contribute to reduced depression, anxiety, and stress. There may also be potential for the reverse, that improvements in mental health could lead to improved sleep quality. Addressing mental health issues may have a secondary effect of improving sleep quality for an individual IEI diagnosed patient. Managing a chronic health condition can significantly contribute to mental health challenges, making mental health assessment, referrals, and interventions especially important for patients with a diagnosis of IEI.\u003c/p\u003e \u003cp\u003eSleep hygiene is a group of behavioral and environmental recommendations to encourage quality sleep.[\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e] In this survey, the participants did engage in several sleep hygiene practices, but widespread adoption of multiple sleep hygiene practices was not reported. A dark and quiet bedroom was most often used (78.6%), followed by avoiding large meals and caffeine prior to bedtime (61.1%). Only 52.6% reported the use of a consistent sleep schedule and only 10.2% avoided electronic use one hour before bedtime. Increased use of smartphones and other electronic devices has worsened the epidemic of poor sleep.[\u003cspan citationid=\"CR22\" class=\"CitationRef\"\u003e22\u003c/span\u003e] While there has been general support for the implementation of sleep hygiene practices to improve sleep quality, studies have shown better outcomes for individual practices and less conclusive results for global implementation of sleep hygiene education.[\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e] Healthcare providers should explore the sleep hygiene practices their patient is utilizing and encourage them to expand their use of these methods. Those patients who are utilizing few or no sleep hygiene interventions may find an improvement in sleep quality after implementing these routines. While there are a variety of medications that can be used for those who have poor sleep quality, cognitive behavioral therapy (CBT) has been shown to be more effective than medication for treatment of insomnia.[\u003cspan citationid=\"CR20\" class=\"CitationRef\"\u003e20\u003c/span\u003e] While this has not been explored as an intervention for individuals with a diagnosis of IEI, there is minimal risk posed with the use of CBT. More research is needed on the use of CBT to improve sleep quality in patients with an IEI diagnosis.\u003c/p\u003e \u003cp\u003eThis study is limited in its generalizability to all IEI patients, given that 91.8% of participants were female, 83.8% had a diagnosis of CVID, and 95.5% were White/Caucasian. The demographics of the participants in this survey are not representative of the broader IEI patient population. However, given the number of respondents, the survey results are useful if evaluating this subgroup of patients. Future sleep quality assessments should be conducted across a broader patient demographic. This survey was also a sample of convenience, distributed through an online social media platform. Though social media surveys have advantages related to speed, cost, and accessibility, they do have a tendency to overrepresent young, white females, as was seen in this survey.[\u003cspan citationid=\"CR23\" class=\"CitationRef\"\u003e23\u003c/span\u003e] Surveys distributed through social media platforms are limited in accessibility to those who are members of the social media group. While this survey was distributed to multiple public and private IEI patient groups, the survey participants are limited to those who are actively engaging in those social media groups. Online social media surveys do not capture adequate representation of those who lack internet service, the elderly, low income, or those living in remote locations.[\u003cspan citationid=\"CR24\" class=\"CitationRef\"\u003e24\u003c/span\u003e] It would be beneficial to have a broader distribution of the survey that allows participation by those who do not have online access; distribution of the survey could be expanded through trusted healthcare providers to reach a wider demographic of IEI patients. All individuals with a diagnosis of IEI were encouraged to participate in the survey, regardless of their sleep or sleep quality. However, online surveys are at risk for sample bias and overrepresentation of the specific issue being explored.[\u003cspan citationid=\"CR24\" class=\"CitationRef\"\u003e24\u003c/span\u003e]\u003c/p\u003e \u003cp\u003eThis survey, though limited in generalizability, suggests that individuals with IEI (specifically White/Caucasian females with a diagnosis of CVID) have a moderate degree of perceived impairment in sleep quality. Healthcare providers are strongly encouraged to incorporate sleep quality screening in their routine assessments of patients with an IEI diagnosis. Valid and reliable tools should be used to track sleep quality over time and can assist in the determination of when to implement interventions to improve sleep quality. For patients with significant impairments in sleep quality, referrals should be made for diagnostic assessments, which may include polysomnogram, multiple sleep latency testing, or a maintenance of wakefulness testing. Referrals can then be made to specialists based on the aspect of sleep that is identified as impaired. Additionally, it is important to assess mental health and chronic pain in this patient population. Further interventions or referrals related to those areas should be made. Sleep and sleep quality is a critical aspect of health that can impact quality of life, daily functioning, and long-term health outcomes. Assessing and providing appropriate interventions to improve sleep quality can make a lasting impact on IEI patient health and wellness.\u003c/p\u003e"},{"header":"Statements \u0026 Disclosures","content":"\u003cp\u003eThis research study did not have any internal or external funding.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eThe authors report they have no conflicts of interest to disclose.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFunding:\u003c/strong\u003e The authors declare that no grants or funds were received to support this research or manuscript preparation.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConflicts of interest/Competing interests:\u003c/strong\u003e The authors have no relevant financial or non-financial interests to disclose.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAvailability of data and material:\u003c/strong\u003e The authors are willing to share the original survey data collected in this study upon request.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthors' contributions:\u003c/strong\u003e All authors were involved in the development of the study design. Data analysis and the first draft of the manuscript was completed by Dr. Sowers. All authors were involved in the editing of the manuscript and review of the final manuscript.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eEthics approval:\u003c/strong\u003e This research study was IRB approved through Stockton University.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent to participate:\u003c/strong\u003e Informed consent was obtained from all participants in this research.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent for publication:\u003c/strong\u003e Consent for publication was obtained from all participants in this research.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n \u003cli\u003e\u003cspan\u003eTangye SG, Al-Herz W, Bousfiha A, Cunningham-Rundles C, Franco JL, Holland S, et al. Human Inborn Errors of Immunity: 2022 Update on the Classification from the International Union of Immunological Societies Expert Committee. J Clin Immunol. 2022;42(7):1473\u0026ndash;507. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1007/s10875-022-01289-3\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eBousfiha A, Moundir A, Tangye SG, Picard C, Jeddane L, Al-Herz W, et al. The 2022 Update of IUIS Phenotypical Classification for Human Inborn Errors of Immunity. J Clin Immunol. 2022;42(7):1508\u0026ndash;20. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1007/s10875-022-01352-z\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eKobrynski L, Powell RW, Bowen S. Prevalence and morbidity of primary immunodeficiency diseases, United States 2001\u0026ndash;2007. J Clin Immunol. 2014;34(8):954\u0026ndash;61. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1007/s10875-014-0102-8\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eBonilla FA. Personalized therapy for common variable immunodeficiency. \u003cem\u003eAllergy and asthma proceedings\u003c/em\u003e. 2020;41(1),19\u0026ndash;25. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.2500/aap.2020.41.190012\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eZhang S, Kline M, Fuleihan RL, Consortium U, Scalchunes C, Sullivan KE, et al. PROMIS-29 survey confirms major impact of fatigue on health-related quality of life in common variable immunodeficiency. Immunol Res. 2020;68(6):379\u0026ndash;88. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1007/s12026-020-09162-4\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eHajjar J, Guffey D, Minard CG, Orange JS. Increased Incidence of Fatigue in Patients with Primary Immunodeficiency Disorders: Prevalence and Associations Within the US Immunodeficiency Network Registry. J Clin Immunol. 2017;37(2):153\u0026ndash;65. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1007/s10875-016-0367-1\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eNijhof LN, van Brussel M, Pots EM, van Litsenburg RRL, van de Putte EM, van Montfrans JM, et al. Severe Fatigue Is Common Among Pediatric Patients with Primary Immunodeficiency and Is Not Related to Disease Activity. J Clin Immunol. 2021;41(6):1198\u0026ndash;207. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1007/s10875-021-01013-7\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eIrwin MR. Why sleep is important for health: a psychoneuroimmunology perspective. Ann Rev Psychol. 2015;66:143\u0026ndash;72. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1146/annurev-psych-010213-115205\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eSejbuk M, Mirończuk-Chodakowska I, Witkowska AM. Sleep Quality: A Narrative Review on Nutrition, Stimulants, and Physical Activity as Important Factors. Nutrients. 2022;14(9):1912. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.3390/nu14091912\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eNelson KL, Davis JE, Corbett CF. Sleep quality: An evolutionary concept analysis. Nurs f\u0026oacute;rum. 2022;57(1):144\u0026ndash;51. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1111/nuf.12659\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eChristie AD, Seery E, Kent JA. Physical activity, sleep quality, and self-reported fatigue across the adult lifespan. Exp Gerontol. 2016;77:7\u0026ndash;11. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1016/j.exger.2016.02.001\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eIrish LA, Kline CE, Gunn HE, Buysse DJ, Hall MH. The role of sleep hygiene in promoting public health: A review of empirical evidence.Sleep medicine reviews. 3015;22,23\u0026ndash;36. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1016/j.smrv.2014.10.001\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eChoy EH. The role of sleep in pain and fibromyalgia. Nat Rev Rheumatol. 2015;11(9):513\u0026ndash;20. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1038/nrrheum.2015.56\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eYi H, Shin K, Shin C. Development of the sleep quality scale. J Sleep Res. 2006;15(3):309\u0026ndash;16. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1111/j.1365-2869.2006.00544.x\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eJohn B, Marath U, Valappil SP, Mathew D, Renjitha M. Sleep Pattern Changes and the Level of Fatigue Reported in a Community Sample of Adults During COVID-19 Pandemic. Sleep and vigilance. 2022;1\u0026ndash;16. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1007/s41782-022-00210-7\u003c/span\u003e\u003c/span\u003e. Advance online publication.\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eYi H, Shin K, Kim J, Kim J, Lee J, Shin C. Validity and reliability of Sleep Quality Scale in subjects with obstructive sleep apnea syndrome. J Psychosom Res. 2009;66(1):85\u0026ndash;8. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1016/j.jpsychores.2008.07.008\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003e\u0026Ccedil;akan D, \u0026Ouml;zt\u0026uuml;rk E. The Effects of Allergic Rhinitis on Sleep Quality. J Acad Res Med. 2022;12(1). \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.4274/jarem.galenos.2021.28199\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eBesedovsky L, Lange T, Haack M. The Sleep-Immune Crosstalk in Health and Disease. Physiol Rev. 2019;99(3):1325\u0026ndash;80. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1152/physrev.00010.2018\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eSkarpsno ES, Nilsen T, Hagen K, Mork PJ. Long-term changes in self-reported sleep quality and risk of chronic musculoskeletal pain: The HUNT Study. J Sleep Res. 2021;30(6):e13354. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1111/jsr.13354\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eBrewster GS, Riegel B, Gehrman PR. Insomnia in the Older Adult. Sleep Med Clin. 2018;13(1):13\u0026ndash;9. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1016/j.jsmc.2017.09.002\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eScott AJ, Webb TL, Martyn-St James M, Rowse G, Weich S. Improving sleep quality leads to better mental health: A meta-analysis of randomised controlled trials. Sleep Med Rev. 2021;60:101556. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1016/j.smrv.2021.101556\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eChattu VK, Manzar MD, Kumary S, Burman D, Spence DW, Pandi-Perumal SR. The Global Problem of Insufficient Sleep and Its Serious Public Health Implications. Healthcare. 2018;7(1):1. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.3390/healthcare7010001\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eWhitaker C, Stevelink S, Fear N. The use of Facebook in recruiting participants for health research purposes: a systematic review. J Med Internet Res. 2017;19(8):e290. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.2196/jmir.7071\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eBall HL. Conducting Online Surveys. J Hum lactation. 2019;35(3):413\u0026ndash;7. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1177/0890334419848734\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\n\u003c/ol\u003e"},{"header":"Tables","content":"\u003cp\u003eTables 1 to 3 are available in the Supplementary Files section\u003c/p\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":true,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":true,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"journal-of-clinical-immunology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"joci","sideBox":"Learn more about [Journal of Clinical Immunology](https://www.springer.com/journal/10875)","snPcode":"10875","submissionUrl":"https://submission.nature.com/new-submission/10875/3","title":"Journal of Clinical Immunology","twitterHandle":"","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"stoa","reportingPortfolio":"Springer Hybrid","inReviewEnabled":true,"inReviewRevisionsEnabled":false},"keywords":"Sleep quality, impaired sleep, fatigue, Sleep Quality Scale, Inborn Errors of Immunity, Primary Immunodeficiency Disease, Common Variable Immune Deficiency","lastPublishedDoi":"10.21203/rs.3.rs-2440959/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-2440959/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e\u003cstrong\u003ePurpose\u003c/strong\u003e: Chronic sleep issues can lead to poor quality of life and increased mortality and patients with chronic health conditions often report impaired sleep quality. Higher levels of fatigue have been identified in patients diagnosed with Inborn Errors of Immunity (or Primary Immunodeficiency Diseases). This research sought to better understand perceived sleep quality in individuals diagnosed with IEI.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eMethods\u003c/strong\u003e: A survey, which included the validated Sleep Quality Scale, was shared across multiple social media groups for individuals with a diagnosis of IEI.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eResults\u003c/strong\u003e: Most of the participants were White/Caucasian females, between the ages of 30 to 74 years. The results of the Sleep Quality Scale suggest that this sample of individuals has moderate impairment of their sleep quality (71.8%), with a mean score of 43.0 (SD = 13.1). When comparing the results of the SQS to other patient populations and healthy control groups, the participants in this study had a poorer sleep quality score. Associations were identified between sleep quality and age, hours of sleep per night, time awake at night, times awake to urinate, attempted daytime naps, chronic pain, and mental health diagnoses.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConclusion\u003c/strong\u003e: This survey suggests that individuals with Inborn Errors of Immunity have a moderate degree of perceived impairment in sleep quality. Healthcare providers are strongly encouraged to incorporate sleep quality screening in their routine assessments of patients with a diagnosis of Inborn Error of Immunity. Patients who are identified as having impaired sleep quality should be referred for further testing and interventions.\u003c/p\u003e","manuscriptTitle":"Perceived Sleep Quality in Individuals with Inborn Errors of Immunity","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2023-01-11 09:48:30","doi":"10.21203/rs.3.rs-2440959/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"reviewerAgreed","content":"","date":"2023-01-18T21:01:53+00:00","index":0,"fulltext":""},{"type":"reviewersInvited","content":"","date":"2023-01-09T15:01:21+00:00","index":"","fulltext":""},{"type":"editorInvited","content":"Journal of Clinical Immunology","date":"2023-01-08T20:32:14+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2023-01-06T16:35:08+00:00","index":"","fulltext":""},{"type":"submitted","content":"Journal of Clinical Immunology","date":"2023-01-04T14:11:46+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"journal-of-clinical-immunology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"joci","sideBox":"Learn more about [Journal of Clinical Immunology](https://www.springer.com/journal/10875)","snPcode":"10875","submissionUrl":"https://submission.nature.com/new-submission/10875/3","title":"Journal of Clinical Immunology","twitterHandle":"","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"stoa","reportingPortfolio":"Springer Hybrid","inReviewEnabled":true,"inReviewRevisionsEnabled":false}}],"origin":"","ownerIdentity":"4d917ef9-564f-4ee4-9937-5b54ca41f61d","owner":[],"postedDate":"January 11th, 2023","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"published-in-journal","subjectAreas":[],"tags":[],"updatedAt":"2023-10-16T20:12:12+00:00","versionOfRecord":{"articleIdentity":"rs-2440959","link":"https://doi.org/10.1007/s10875-023-01474-y","journal":{"identity":"journal-of-clinical-immunology","isVorOnly":false,"title":"Journal of Clinical Immunology"},"publishedOn":"2023-03-24 20:05:46","publishedOnDateReadable":"March 24th, 2023"},"versionCreatedAt":"2023-01-11 09:48:30","video":"","vorDoi":"10.1007/s10875-023-01474-y","vorDoiUrl":"https://doi.org/10.1007/s10875-023-01474-y","workflowStages":[]},"version":"v1","identity":"rs-2440959","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-2440959","identity":"rs-2440959","version":["v1"]},"buildId":"ApUGefWb6u5IBVtyqm6d5","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}