Psychosocial impact of mast cell disorders: Pilot investigation of a rare and understudied disease

Abstract

Mast cell disorders are rare, chronic diseases involving unpredictable physical symptoms ranging in severity, duration, and frequency. Almost two-thirds of participants with these disorders (n = 180) experienced clinically meaningful depressive symptoms. Depressed mood was associated with somatic symptomatology, poorer quality of life, lower resilience, and indirectly with ways of coping. Newly developed measures for this population performed psychometrically well. There was no gender difference in depression but women reported greater use of several ways of coping, greater disease-related distress, poorer quality of life, and more symptoms. Results underscore the need for further research and development of effective psychosocial interventions for this population.

Keywords

chronic illness coping depression quality of life stress symptoms

Introduction

Mast cell disorders are rare, chronic illnesses that include mastocytosis and mast cell activation syndrome. Mastocytosis is characterized by abnormally large numbers of mast cells in skin, lymph nodes, internal organs or linings of the lung, stomach, or intestine (National Institute of Allergy and Infectious Disease, 2012). When mast cells de-granulate, they release pro-inflammatory substances such as histamine, leading to cutaneous, gastrointestinal, skeletal, and cardiovascular manifestations. Reported symptoms are varied and include fatigue, headache, nausea, vomiting, diarrhea with abdominal pain, uterine cramps or bleeding, shortness of breath, atrial fibrillation, peptic ulcers, severe bone pain, impaired consciousness, and flushing (Castells, 2004; Hermine et al., 2008; Theoharides, 1996). The World Health Organization classifies mastocytosis into seven disorders: cutaneous mastocytosis, indolent systemic mastocytosis, systemic mastocytosis with an associated clonal hematologic non-mast cell lineage disease, aggressive systemic mastocytosis, mast cell leukemia, mast cell sarcoma, and extracutaneous mastocytoma (Akin and Valent, 2014). An eighth variant, smoldering systemic mastocytosis, was recently added to this classification (Akin and Valent, 2014). A diagnosis of mast cell activation syndrome is appropriate when mast cell activation symptoms recur in conjunction with increased mast cell–derived mediators in biological fluid and when symptoms are responsive to mast cell–stabilizing drugs (Valent, 2013).

The rarity of this disease, poorly specified diagnostic criteria, and limited physician expertise have contributed to difficulty in determining its prevalence (Metcalfe, 1991; Valent et al., 2012). Two studies estimate the prevalence of mastocytosis to be between 3 and 13 per 100,000 individuals worldwide (Rosbotham et al., 1999; Van Doormaal et al., 2013). In the United States, mastocytosis is considered an “orphan disease” because it affects less than 200,000 Americans (National Institute of Allergy and Infectious Disease, 2012).

Mast cell disorders have received little research attention. A search of the U.S. National Institutes of Health repository of funded research projects (Research Portfolio Online Reporting Tool; RePORT) as of March 2015 revealed nine studies of these disorders, all of which were studies that did not address psychosocial factors. A search of PubMed, PsycINFO, and Scopus databases conducted the same month using the search terms “mast cell disorders,” “mast cell activation disorder,” or “mastocytosis” with “psychological” for work published from 1965 to 2015 identified eight relevant articles (Georgin-Lavialle et al., 2014; Hermine et al., 2008; Jennings et al., 2014; Moura et al., 2011, 2012, 2014; Paul et al., 2010; Rogers et al., 1986). This research is mostly descriptive, reporting the prevalence of depression and some psychosocial aspects of mastocytosis. For the most part, studies are not theoretically driven, and are rife with methodological weaknesses. Three studies, for example, are based on a sample size of 25 or fewer. Additionally, the few studies that use a comparison group do not control for variables confounded with group membership such as age, income, education, or employment.

Monitoring symptoms is a constant, overwhelming task for sufferers of mast cell disorders (O’Neill and Morrow, 2001). Symptoms range in severity, location, duration, frequency, predictability, and controllability both within affected individuals over time and across individuals (Butterfield, 2006), complicating the diagnosis. Hence, patients often suffer for many years with an unidentified disorder, may be misdiagnosed, and may see several physicians prior to receiving an accurate diagnosis. Treatment is similarly complicated. According to Melnikova (2012), less than 10 percent of patients diagnosed with a rare disorder receive a disease-specific treatment.

Although depression and negative mood in patients with mastocytosis have been noted in clinical observations, there is a demonstrated need for systematic, scientifically rigorous research on psychosocial factors associated with mast cell disorders (Moura et al., 2014). A primary goal of this study was to examine the likely but untested possibility that psychosocial conditions contribute to patients’ depression. Psychosocial research on individuals affected by mast cell disorders is particularly needed given the debilitating profile of these disorders as described in the existing medical literature. Hermine et al. (2008) found in a study of 363 patients with mastocytosis that 70 percent felt “disabled” by the disease. Psychosocial factors may exacerbate symptoms of mast cell disorders, as the mast cell is considered a sensor of environmental and emotional stress (Theoharides, 2002).

The purpose of this study was to assess the prevalence of depression in a sample of patients with mast cell disorders and to examine associations of disease impact, coping, and social support with depression using psychometrically robust measures and methods, including some measurement tools developed specifically for this population and pilot-tested here. In addition, based on evidence that women and men tend to experience chronic illness differently (Tamres et al., 2002), we investigated whether gender differences exist in the psychosocial variables examined among disease sufferers.

Rationale for study hypotheses

The expected rate of depression among individuals with a mast cell disorder is difficult to determine. We predicted that it would be comparable to the rate found among sufferers of similarly debilitating chronic diseases. Estimates of the prevalence of depression in individuals with rheumatoid arthritis and lupus range from 13 to 20 percent (Dickens and Creed, 2001; Dickens et al., 2002; Frank et al., 1988), and from 20 to 55 percent (Palagini et al., 2013), respectively. By comparison, the rate of depression in the general population of the United States is 9.1 percent (Substance Abuse and Mental Health Services Administration, 2014).

Chronic illness has been shown to arouse distress (Cohen and Biesecker, 2010), because it influences numerous aspects of a person’s life including emotions, relationships, and employment (Stewart et al., 2004). The unpredictable nature of chronic illness tends to diminish a person’s sense of control over life. Treatment compliance or a healthy lifestyle may not ensure control of the disease (Affleck et al., 1987). However, confidence in one’s ability to control symptoms and to secure a desired outcome has been shown to promote adjustment (Felton and Revenson, 1984; Jim et al., 2006). We hypothesized, therefore, that among individuals with a mast cell disorder, depression would be predicted by poorer physical health and by greater disease-related distress, and quality of life and resilience would be associated with lower depression. To examine these hypotheses, we developed several measures to be used with sufferers of mast cell disorders with the aim that our pilot test of these measures in the current study would prove useful for future research on this population.

We also examined social support because it is an integral contributor to well-being (Baumeister and Leary, 1995), and receiving social support is considered a task of chronic disorders (Stanton et al., 2007). People with spouses, friends, and family members who provide emotional support and tangible aid report better health (Leavy, 1983; Mitchell et al., 1982). We hypothesized that social support would be associated with lower depression in individuals with mast cell disorders.

In addition, we examined coping, as it enables people to manage disease-related distress (Hagger, 2009). Jennings et al. (2014) reported in a study of 420 patients with mastocytosis that more than 60 percent expressed a need to find ways to cope with the unpredictability of symptoms. Coping can be used to regulate negative emotional responses to stressful situations or to alter the situations themselves (Folkman et al., 1986; Lazarus and Folkman, 1984). For example, an individual with a mast cell disorder may use problem-focused coping behaviors by seeking information from a physician and also employ emotion-focused coping behaviors in an effort to manage anxiety (Dunkel-Schetter et al., 1992). Studies examining coping in patients with rheumatoid arthritis have found that problem-focused methods of coping are associated with better well-being, whereas emotion-focused methods are associated with poorer well-being (Felton and Revenson, 1984; Manne and Zautra, 1989; Parker et al., 1988). We hypothesized that ways of coping shown to be maladaptive in prior research, namely distancing, escape-avoidance, and accepting responsibility, would predict greater depression, and that adaptive ways of coping, including planful problem solving, seeking social support, self-control, confrontive coping, and positive reappraisal, would be associated with lower depression.

Finally, based on studies of other chronic disorders, we predicted that women would be more depressed than men, and we explored whether there are gender differences in other study variables as this has not been investigated previously.

Methods

Overview

This study was advertised in a newsletter published by The Mastocytosis Society, Inc., a non-profit organization for patients and families affected by mast cell disorders. Solicitations were also emailed to individuals listed in the Mastocytosis Patient Registry at Brigham and Women’s Hospital, Boston, Massachusetts. Adults 18 years or older with a mast cell disorder were invited to complete an anonymous, 30-minute Internet-based survey about “the psychological impact of having a mast cell disorder.” The study was approved by the Mastocytosis Society Research Committee and the Partners Healthcare Institutional Review Board at Brigham and Women’s Hospital. A total of 180 completed surveys were returned. The extent of missing values in these was minimal, with less than 10 percent missing from all items in the data set. Pairwise deletion was used to maximize the number of participants in each analysis given the novelty and importance of the present investigation.

Depression

The Center for Epidemiologic Studies Depression Scale (CES-D) is a reliable, well-validated 20-item self-report measure that assesses frequency of depressive symptoms within the past week from 0 (none of the time) to 3 (almost all the time). A score of 16 or greater indicates a clinically defined level of depression (Radloff, 1977). The CES-D has excellent internal consistency as confirmed in this study (Cronbach’s α = .94). Analyses were conducted with both continuous depression scores and the dichotomous variable, indicating whether a participant was above or below the cut-off for clinically defined depression.

Disease impact

Items comprising the Mast Cell Disorder Questionnaire (MCDQ) were developed based on the limited existing research about the psychosocial impact of these disorders and interviews with affected individuals. Items were piloted in a separate small sample of patients. Respondents indicated their extent of agreement (1 = strongly disagree to 5 = strongly agree) with each item. The MCDQ is comprised of three subscales: quality of life (11 items, e.g. “This disease has affected my career,” reverse scored), disease-related distress (17 items, e.g. “I am scared because so few doctors know about mastocytosis/mast cell disorders”), and resilience (10 items, e.g. “I feel like I am in control of my life”) (Cronbach’s α = .80, .80, and .70, respectively). A mean item score was calculated for each subscale.

Coping

Coping was assessed with the well-validated 66-item Revised Ways of Coping Questionnaire (Folkman and Lazarus, 1985, 1988). Participants responded on a scale ranging from 0 (not used) to 3 (used a great deal) to indicate the frequency of coping behaviors they employ to manage having a mast cell disorder. Internal consistency for the coping subscales was generally good: Cronbach’s α was .59 for the self-control subscale but ranged from .67 to .76 for all remaining coping subscales.

Social support

Participants responded to 13 items assessing the helpfulness of emotional support providers (including family, friends, spouse/partner) and 13 items assessing the helpfulness of tangible support providers (including employer and medical professionals). Responses were from 1 (not very helpful) to 5 (very helpful). A mean score for all 26 items was calculated. Cronbach’s α was .82.

Symptoms

Physical symptoms were assessed using a 41-item self-report questionnaire specific to mast cell disorders that was developed based on pertinent literature. Participants indicate the frequency with which they experienced a symptom from 0 (never) to 4 (daily). Responses were summed yielding a score that reflects both frequency and total number of symptoms.

Results

Sample description

As shown in Table 1, a majority of participants were White, female, married, college-educated, employed, between the ages of 40 and 60 years, and had a diagnosis of indolent systemic mastocytosis. Participants reported high levels of support, quality of life, resilience, and disease-related distress and also had a high symptom score on average (see bottom of Table 2). Coping by seeking social support, planful problem solving, positive reappraisal, and self-control were most common; distancing, escape-avoidance, confrontive coping, and accepting responsibility were less commonly reported ways of coping.

Table 1.

Sample characteristics.

	n	%
Age (years)
21–39	35	19.44
40–60	108	60.00
61–80	25	13.90
Missing	12	6.70
	180	100.00
Gender
Female	148	82.20
Male	26	14.40
Missing	6	3.30
	180	100.00
Marital status
Married	121	67.20
Not married	52	28.90
Missing	7	3.90
	180	100.00
Diagnosis^a
Indolent systemic mastocytosis	74	41.11
Cutaneous mastocytosis	36	20.00
Mast cell activation disorder	35	19.44
Systemic mastocytosis with an associated clonal hematologic non-mast cell lineage disease (SM-AHMD)	17	9.44
Aggressive systemic mastocytosis	12	6.67
Extra-cutaneous mastocytoma	1	0.56
Non-mast cell lineage	1	0.56
Mast cell leukemia	0	0.00
Mast cell sarcoma	0	0.00
Missing	4	2.22
	180	100.00
Education level
Attended high school	5	2.78
High school graduate	50	27.78
College graduate	67	37.22
Postgraduate degree	49	27.22
Missing	9	5.00
	180	100.00
Racial/ethnic background
White	162	90.00
Hispanic	3	1.67
Pacific Islander	3	1.67
African American	2	1.11
Native American	2	1.11
Asian American	1	0.56
Missing	7	3.88
	180	100.00
Employment
Employed full-time	69	38.33
Unemployed and on disability	42	23.33
Unemployed	32	17.78
Employed part-time	30	16.67
Missing	7	3.89
	180	100.00
Annual household income (US$)
Under 20,000	18	10.00
20,000–40,000	25	13.89
41,000–60,000	15	8.33
61,000–80,000	26	14.44
81,000–100,000	30	16.67
Over 100,000	47	26.11
Missing	19	10.56
	180	100.00

Reflects the diagnostic classification system used prior to new WHO classifications (see Akin and Valent, 2014).

Table 2.

Correlations, means, standard deviations, and internal consistency of study variables.

Variables	1	2	3	4	5	6	7	8	9	10	11	12	13	14
1. Confront coping	–	.389**	.408**	.470**	.479**	.420**	.511**	.409**	.293**	.073	−.161**	.236**	−.048	.221**
2. Distancing		–	.504**	.030	.276**	.339**	.236**	.270**	.011	−.040	−.036	.029	.207*	.024
3. Self-control			–	.215**	.455**	.468**	.414**	.459**	.345**	.025	.321**	.369**	.038	.337**
4. Seek soc support				–	.124	.147	.443**	.418**	.222**	.315**	−.139	.168*	.117	.051
5. Accept responsib					–	.574**	.293**	.251**	.287**	−.079	−.387**	.503**	−.142**	.477**
6. Escape-avoidance						–	.126	.209*	.415*	−.244**	−.537**	.585*	−.330**	.621**
7. Planful prob solv							–	.531**	.214*	.239**	−.100	.192*	.230**	.002
8. Pos reappraisal								–	.229**	.290**	−.154*	.192*	.291**	.021
9. Symptoms									–	.004	−.523**	.415**	−.173*	.550**
10. Support										–	.160	−.210*	.206*	−.291**
11. Quality of life											–	−.772**	.362**	−.683**
12. Disease-rel dist												–	−.339**	.673**
13. Resilience													–	−.414**
14. Depression														–
M	0.84	1.11	1.31	1.39	0.66	1.09	1.36	1.34	2.93	3.42	2.22	3.67	3.22	22.37
SD	0.55	0.62	0.55	0.64	0.67	0.63	0.68	0.66	0.60	0.51	0.77	0.61	0.70	13.44
α	.67	.69	.59	.68	.72	.74	.76	.74	.91	.82	.80	.80	.70	.94

p < .05; **p < .01.

Prediction of clinical depression

Almost two-thirds of the sample (64%) was clinically depressed (CES-D score ⩾ 16), but there was sufficient inter-individual variability in depression scores (M = 22.37; SD = 13.44) to examine predictors of this outcome. The prevalence of clinical depression differed by income (X²(5) = 21.32, p < .001), employment status (X²(3) = 16.03, p < .001), and education level (X²(3) = 9.50, p < .05), and some sub-groups were distinctive. For example, 93.3 percent of participants earning US$41,000–US$60,000 (n = 14) were clinically depressed, in comparison to only 43.2 percent of participants earning over US$100,000 (n = 19). Furthermore, 93.3 percent of participants employed part-time (n = 28) versus 52.2 percent of those employed full-time were depressed (n = 35). Similarly, 77.6 percent of participants with a high school degree were clinically depressed (n = 38), compared to 47.8 percent of those with a post-graduate degree (n = 22).

As shown in Table 3, clinically depressed participants (n = 110) reported significantly lower quality of life and resilience and higher disease-related distress and more symptoms than non-depressed participants (n = 62). Depressed participants also reported more use of confrontive coping, self-control, accepting responsibility, and escape-avoidance than non-depressed participants.

Table 3.

Comparison of clinically depressed and non-depressed participants.

	Depressed		Non-depressed
	(CES-D > 16)		(CES-D < 16)
	(n = 110)		(n = 62)
	M	SD	M	SD	n	t
MCDQ
Quality of life^a	1.86	0.57	2.84	0.71	167	9.15*
Disease-related distress	3.89	0.54	3.22	0.52	163	−7.67***
Resilience	3.00	0.61	3.52	0.72	163	4.99***
WCQ
Confrontive coping	0.91	0.53	0.72	0.58	172	−2.18*
Distancing	1.12	0.59	1.12	0.67	172	0.00
Self-control	1.42	0.50	1.11	0.53	171	−3.77***
Seeking social support	1.42	0.64	1.23	0.59	172	−1.28
Accepting responsibility^b	0.79	0.69	0.37	0.48	171	−4.20***
Escape-avoidance	1.30	0.60	0.72	0.49	171	−6.44***
Planful problem solving	1.37	0.65	1.27	0.69	172	−0.92
Positive reappraisal	1.37	0.60	1.32	0.73	172	−0.51
Symptoms^c	3.17	0.43	2.49	0.61	117	−6.52***
Support	3.36	0.55	3.51	0.38	125	1.63

MCDQ: Mast Cell Disorder Questionnaire; CES-D: Center for Epidemiologic Studies Depression Scale; WCQ: Ways of Coping Questionnaire.

Levene’s test for equality of variance indicated unequal variances between groups (F = 4.43, p < .05), so adjusted degrees of freedom were used.

Levene’s test for equality of variance indicated unequal variances between groups (F = 7.76, p < .05), so adjusted degrees of freedom were used.

Levene’s test for equality of variance indicated unequal variances between groups (F = 6.94, p < .01), so adjusted degrees of freedom were used.

p < .05; **p < .01; ***p < .001.

Binary logistic regression was used to determine the independent association of study variables with clinical depression. This analysis indicated that depression was predicted by physical symptoms (B = 2.03, SE = .84, Wald = 5.89, Exp(B) = 7.62, p < .05), and inversely by quality of life (B = −1.73, SE = .84, Wald = 4.25, Exp(B) = 0.18, p < .05) and resilience (B = −1.66, SE = .83, Wald = 4.01, Exp(B) = 0.19, p < .05).

Associations with level of depressed mood

Given the paucity of research on the psychological impact of mast cell disorders, we explored associations of sociodemographic variables with increments in depressed mood using continuous depression scores. These scores were not associated with marital status, gender, or diagnosis, but were inversely correlated with age, education, and income (r = −.283, −.210, and −.370, respectively and p < .05).

With regard to the hypothesized psychosocial predictors of depression, as shown in Table 2, there were large, positive correlations of continuous depression scores with disease-related distress and symptoms, and with coping by escape-avoidance, accepting responsibility, self-control, and confrontation. Quality of life, resilience, and social support were inversely correlated with continuous depression scores.

Gender differences

Although no gender difference was found for depression, as shown in Table 4, women had higher symptom scores, greater disease-related distress, and poorer quality of life than men. Female participants also reported greater use of confrontive coping, self-control, seeking social support, planful problem solving, and positive reappraisal than did men (all ps < .05).

Table 4.

Comparison of female and male participants.

	Female		Male
	M	SD	M	SD	n	t
WCQ
Confrontive coping	0.88	0.54	0.62	0.63	172	2.30*
Distancing	1.49	0.62	0.94	0.64	172	1.55
Self-control	1.36	0.52	0.98	0.55	171	3.33***
Seeking social support	1.42	0.62	1.13	0.65	172	2.17*
Accepting responsibility	0.65	0.64	0.60	0.72	171	0.37
Escape-avoidance	1.12	0.62	0.97	0.66	172	1.55
Planful problem solving	1.42	0.66	0.97	0.64	173	3.22**
Positive reappraisal	1.41	0.65	0.98	0.61	172	3.28**
MCDQ
Disease-related distress^a	3.72	0.56	3.26	0.78	164	2.76**
Quality of life	2.16	0.74	2.56	0.91	169	−2.48*
Resilience	3.23	0.70	3.16	0.65	165	0.46
Symptoms	2.99	0.58	2.49	0.63	118	3.23**
Support	3.45	0.52	3.34	0.37	128	1.02
CES-D	22.94	13.36	18.92	13.83	170	1.38

MCDQ: Mast Cell Disorder Questionnaire; CES-D: Center for Epidemiologic Studies Depression Scale; WCQ: Ways of Coping Questionnaire.

Levene’s test for equality of variance indicated unequal variances between groups (F = 6.30, p < .01), so adjusted degrees of freedom were used.

p < .05; **p < .01; ***p < .001.

Discussion

Depression was common in this sample. Almost two-thirds experienced clinically meaningful depressive symptoms. Depression was most strongly predicted by poorer physical health; in addition, individuals who reported experiencing poorer quality of life, or who characterized themselves as less resilient, were most likely to be depressed. Consistent with research documenting patterns of coping in rheumatoid arthritis patients using the same coping measure (Felton and Revenson, 1984; Manne and Zautra, 1989; Parker et al., 1988), several ways of coping were associated with clinical depression but these did not predict depression when other predictors were examined simultaneously. This indicates that the association of coping methods with depression was explained by their correlation with variables such as quality of life and resilience. In addition, although there was no gender difference in depression, women reported more use of several ways of coping, and they experienced greater disease-related distress, poorer quality of life, and more symptoms than did men. Tamres et al. (2002) found that women tend to appraise a personal health stressor as more severe than men. Women may use more coping strategies compared to men because they are more distressed by a health crisis, as our results suggest. Nevertheless, it appears that their distress does not result in greater depressed affect than men experience.

Overall, participants in this study had high symptom and disease-related distress scores on average, and depression appears to be more common in them than in individuals with rheumatoid arthritis and lupus, chronic diseases characterized by a similar myriad of symptoms (Baker and Pope, 2009; Dickens and Creed, 2001; Dickens et al., 2002) but which are more common and familiar than mast cell disorders and therefore likely to result in greater acceptance both by health care professionals and by the lay public. In addition, the prevalence of depression among study participants is far above the rate in the American population (Substance Abuse and Mental Health Services Administration, 2014). This is not surprising given that individuals with a mast cell disorder typically endure diverse, uncontrollable, and unpredictable physical symptomatology for an extended period of time before a diagnosis is confirmed, with limited treatment success even after diagnosis and considerable interference with daily tasks despite treatments. In one of the largest studies ever conducted on patients with a mast cell disorder, Jennings et al. (2014) found that 41 percent of participants felt extremely affected emotionally by their disorder, with the unpredictable nature of symptoms being the greatest cause of their distress. This portrait of the physical and emotional condition of individuals with a mast cell disorder underscores an urgent need for further psychological research and development of effective psychosocial treatments for this population. Although there are no studies examining the benefits of psychotherapeutic interventions in mast cell disorder patients, there is some evidence for cognitive behavioral therapy in conjunction with a stress management intervention in other chronic disorders with similar symptom profiles. This treatment combination has been shown to reduce pain, depressed mood, and difficulty sleeping in patients with fibromyalgia (Glombiewski et al., 2010) and to improve coping skills and self-efficacy in patients with rheumatoid arthritis (Astin et al., 2002). This may be a similarly promising intervention for individuals with mast cell disorders, but it remains to be demonstrated.

Social support has been shown to buffer effects of stress on well-being (Cohen and Wills, 1985; Uchino and Birmingham, 2010); as hypothesized, greater social support in this study was associated with lower, continuously scored depression although support did not predict clinical depression. The helpfulness of support from specific providers, as measured in the current study, may not provide a sufficient buffer from the impact of mast cell disorders to prevent full-blown depression. Moreover, not all forms of social support may be advantageous to individuals with mast cell disorders. Some types of support, particularly received support (also labeled enacted support), have been shown to be less consistently beneficial in other populations (Dunkel-Schetter and Brooks, 2009). Nevertheless, findings associated with social support in this study should be interpreted with caution as the instrument we used has not previously been validated.

In addition to its use of novel measures with unreplicated psychometric properties, this study has some other limitations, including its correlational design: We cannot confirm that psychosocial factors such as poorer quality of life contributed to depression as hypothesized rather than resulted from it. It is quite likely that such associations are bidirectional. It would be valuable in future studies to observe affected individuals over time to examine whether variables such as resilience and quality of life forestall depression, as we predict. It is also conceivable that stress and related emotional factors may contribute to the frequency and severity of patients’ physical symptoms over time. There is now a large literature documenting the impact of stress and emotions on physical illness (Cohen et al., 2007; Keller et al., 2012), and it is biologically plausible that mast cell de-granulation is provoked or exacerbated by emotional distress. Investigating affected individuals over time is especially important for a disorder like this which is chronic and characterized by unpredictable symptom flare-ups.

An additional limitation is the unknown generalizability of study findings, as participants were drawn from two sources of self-selected individuals with mast cell disorders. Representativeness of this sample cannot be determined because of the rarity of mast cell disorders and the lack of information about typical patient characteristics. The current sample was predominantly White, well-educated, and socioeconomically advantaged, with access to the Internet. We have no reason to suspect that mast cell disorders are more common in this group than in a more diverse population. Several sociodemographic characteristics were associated with depression even in this fairly homogeneous, moderately sized sample. An important task of future research will be to determine more definitively whether mast cell disorders are more prevalent in particular groups of individuals and whether these groups experience the disease differently.

Despite these limitations, this study is one of the first systematic investigations of mast cell disorder sufferers to examine associations of disease impact, coping, and social support with depression. Measures developed for use with this population performed well psychometrically and may be valuable tools for further research. The findings provide a foundation for more elaborate research on quality of life and emotional conditions in patients with mast cell disorders to elucidate which factors promote adjustment and which may portend poorer physical and psychosocial outcomes. This knowledge can be used to identify individuals at risk, to develop effective interventions, to inform the care of people with mast cell disorders, and to reduce their suffering.

Footnotes

Declaration of conflicting interests

The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Funding

The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

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