Sturge‒Weber syndrome (SWS), also known as cerebral trigeminal hemangioma syndrome, is a neurocutaneous syndrome involving the facial skin, central nervous system and eyes and is often accompanied by glaucoma and facial features. Capillary malformations are distributed by the trigeminal nerve [
1] with an incidence of 1/50000–1/20000 [
2]. Patients with SWS secondary glaucoma may have other retinal complications [
3]. In addition, focal seizures of the contralateral body may also be noted in these patients [
4]. The onset of the disease is sporadic and does not have a genetic tendency. At present, it is believed that somatic mutations occur in early embryonic development [
5]. SWS secondary glaucoma can cause continuous damage to the optic ganglion cells of patients and eventually lead to blindness. Similar to other types of glaucoma, SWS secondary glaucoma has the characteristics of a more hidden onset [
6]. SWS secondary glaucoma patients are often in the late stage of the disease when experience eye abnormalities, and it is difficult to effectively intervene and reverse this condition. The treatment of SWS secondary glaucoma has always been difficult, and there is uncertainty about the long-term efficacy and safety of various treatment methods for such patients given the characteristics of the disease. SWS is a rare disease with multisystem involvement. The treatment cycle is long, the cost of treatment is expensive, and the treatment effect is relatively poor. As the main caregivers in the child's daily life, recovery and treatment, the parents of children with SWS are faced with many pressures. These pressures affect the parents’ quality of life and psychology and make them prone to a sense of disease uncertainty, which is associated with the child's mental health and wellbeing [
7]. Disease uncertainty refers to the individual's lack of ability to judge disease-related stress events [
8]. Illness uncertainty can damage family members' quality of life and physical and mental health, affect family members' role adaptation, interfere with their decision-making functions, and affect the patient's disease recovery [
9,
10]. In recent years, disease uncertainty among parents has received increasing attention, but research has mainly focused on the parents of children receiving palliative care [
11] or children with congenital heart disease [
12] or epilepsy [
13]. Parents of children with SWS have a high level of illness uncertainty, and their psychological state is not good. The illness uncertainty experienced by parents is proportional to their psychological state [
14]. However, there are few reports on the disease uncertainty of parents of children with SWS because the disease is rare. However, our research center currently has the largest sample of patients with SWS reported in the world. This paper aims to initially explore the current situation of disease uncertainty in parents of children with SWS and explore its influencing factors with a view toward developing interventions for medical staff to implement. The paper aims to provide evidence for interventions that are effective in reducing parental illness uncertainty.
Research methods
Based on the literature research and preinterview, this questionnaire was designed by the researchers and included 14 items on topics, such as the relationship between the interviewee and the patient, age, place of residence, marital status, and level of education.
Generalized anxiety questionnaire (7-item Generalized Anxiety Disorder Scale,GAD-7)
The GAD-7 is a screening tool for generalized anxiety disorder and consists of seven items that ask subjects how distressed they have been according to the corresponding symptoms in the past two weeks. The responses are scored on a four-point scale ranging from 0 (not at all) to 3 (almost every day), and the total score ranges from 0 to 21. For the Chinese version of the GAD-7, a total score greater than or equal to 10 points indicates generalized anxiety [
15]. The sensitivity and specificity of the GAD-7 for screening generalized anxiety disorder in general hospitals among Chinese outpatients were 86.2% and 95.5%, respectively. The Cronbach's α coefficient was 0.898, and the test–retest reliability coefficient was 0.856 [
16].
Patient health questionnaire (PHQ-2)
The PHQ-2, which was extracted from the 9-item Patient Health Questionnaire (PHQ-9), was used to study depressive symptoms. The PHQ-2 measures the frequency of depressive mood and anhedonia over the past 2 weeks with scores ranging from 0 (not at all) to 3 (almost every day) [
17]. The sensitivity and specificity of the PHQ-2 for diagnosing major depression were 86% and 78%, respectively, with a score of 2 or higher and 61% and 92% with a score 3 or higher [
18]. The PHQ-2 has been verified and used in China [
19].
Perceived stress scale (PSS-4)
The PSS-4 compiled by Cohen [
20] was used to assess subjects’ stress levels and comprises four items rated on a 5-point Likert scale with scores ranging from 0 (never) to 4 (very common). Compared with the Stress Perception Scale, the PSS-4 is simpler and more intuitive. A number of confirmatory factor analysis studies on the PSS-4 scale have found that it contains two factor structures: positively described items and negatively described items. Among them, items 2 and 3 represent items with positive descriptions, and reverse scoring is used for these items. The higher the score, the greater the perception of stress. The items with negative descriptions include items 1 and 4, and reverse scoring is not used for these items. The total score of the four items represents the total score of perceived pressure. The higher the score, the greater the perceived pressure. The PSS-4 was confirmed to have good reliability in the Chinese population with a Cronbach's a coefficient of 0.833 [
21].
Simplified coping style questionnaire (SCSQ)
The questionnaire is a self-assessment scale compiled by Xie Yaning and Zhang Yukun based on a foreign coping style scale and adapted according to the actual needs and the characteristics of the Chinese population [
22]. The SCSQ consists of 2 subscales of positive coping and negative coping, including 20 items. The questionnaire is assessed using a 4-point scoring method as follows: not coping = 0, occasionally coping = 1, sometimes coping = 2, and often coping = 3. The positive coping subscale includes questions 1–12, which mainly reflect the characteristics of individuals adopting positive coping styles when encountering stress. The overall Cronbach's α of the scale was 0.90. The Cronbach's α coefficient of the positive coping subscale was 0.89, and the Cronbach's α coefficient of the negative coping subscale was 0.78.
Illness uncertainty scale (parent perceptions of uncertainty scale, PPUS)
The PPUS was revised by the domestic scholar Mai Jiaxuan [
23] to assess the level of illness uncertainty of parents of hospitalized children. The scale contains 28 items and 4 dimensions, namely, ambiguity, lack of clarity, lack of information, and unpredictability. The scale uses a 5-point Likert scoring method, where strongly agree = 5 points, agree = 4 points, uncertain = 3 points, disagree = 2 points, and strongly disagree = 1 point. Items 6, 9, 11, 19, 23, and 25–28 are reverse scored, and a higher total score indicates a higher level of disease uncertainty. The scale has good construct validity. The Cronbach's α coefficient of the total scale is 0.91, and the Cronbach's α coefficients of each dimension range between 0.72 and 0.87.
Data collection methods
The study obtained the consent of the department leaders before analyzing the existing patient data in the department. The members of the research group selected the parents of the patients who met the inclusion and exclusion criteria to conduct the survey. When distributing the questionnaires, a unified guide was adopted to explain the content, purpose, significance, and confidentiality of the data to the subjects. For those who could not complete the questionnaires by themselves for various reasons, the investigator explained the above points to them one by one so that parents could decide whether to participate after understanding, and the investigator was responsible for completing the form. The questionnaires were collected on the spot. When the questionnaires were collected, the investigators checked the contents of the investigation. If there were any doubts, the parents were consulted, and the issue was resolved. If any problems, such as missing answers, were identified, the problems were corrected in a timely manner. A total of 108 questionnaires were distributed this time, 105 of which were valid. The effective recovery rate was 97.22%.
Statistical methods
The data were entered by two individuals into Excel, and the data were analyzed using SPSS 18.0 software. Enumeration data are described as the frequency and percentage; measurement data are expressed as the mean ± standard deviation. Influencing factors were assessed using t-test, analysis of variance, Spearman correlation analysis and multiple linear regression analysis, and P < 0.05 was considered statistically significant.