Stigma experienced by ALS/PMA patients and their caregivers: a mixed-methods study

Figures & data

Table 1 Demographic and disease-related characteristics of the participants.

	Patients (N = 193)	Caregivers (N = 87)
Age, years
Mean (SD)	64.6 (10.0)	63.0 (11.3)
Median	65.0	65.0
Range	28–89	28–83
Gender, n (%)^a
Male	140 (72.5)	–
Female	53 (27.5)	–
Relationship to patient, n (%)
Partner	–	78 (89.7)
Son/daughter	–	2 (2.3)
Parent	–	3 (3.4)
Friend	–	2 (2.3)
Other	–	2 (2.3)
Nationality, n (%)^b
Dutch	190 (98.4)	84 (96.6)
Other	3 (1.6)	3 (3.4)
Living situation, n (%)
With partner	119 (61.7)	64 (73.6)
With partner and children	44 (22.8)	17 (19.5)
Alone with children	5 (2.6)	1 (1.1)
Alone	22 (11.4)	3 (3.4)
With others	3 (1.6)	2(2.3)
Educational level, n (%)^c
Low (primary school, lower vocational education)	43 (22.3)	12 (13.8)
Intermediate (secondary school, vocational education)	78 (40.4)	46 (52.9)
High (higher vocational education, university)	69 (35.8)	28 (32.2)
Work situation, n (%)
Paid employment	29 (15.0)	38 (43.7)
No paid employment	77 (39.9)	10 (11.5)
Retired	87 (45.1)	39 (44.8)
Diagnosis, n (%)
ALS	150 (77.7)	74 (85.1)
PMA	43 (22.3)	13 (14.9)
Time since diagnosis, n (%)
<1 year	47 (24.4)	21 (24.1)
1–3 years	76 (39.4)	42 (48.3)
3–5 years	31 (16.1)	9 (10.3)
≥5 years	39 (20.2)	15 (17.2)
Months as a caregiver, n (%)
<6 months	–	16 (18.4)
6 months to 1 year	–	12 (13.8)
12 months	–	12 (13.8)
>12 months	–	47 (54.0)
Type of onset, n (%)
Spinal-onset	149 (77.2)	68 (78.2)
Bulbar-onset	35 (18.1)	15 (17.2)
Respiratory-onset	9 (4.7)	4 (4.6)
Functional impairment (ALS-FRS-R)
Mean (SD)	31.5 (9.9)	–
Median	33	–
P25%, P75%	25, 39
Range	1 – 48	–
Clinical stage, n (%)
Stage 1^d	31 (16.1)	–
Stage 2^e	47 (24.4)	–
Stage 3^f	61 (31.6)	–
Stage 4^g	54 (28.0)	–
Perceived severity of symptoms, n (%)
Not severe	25 (13.0)	–
Quite severe	58 (30.1)	–
Severe	63 (32.6)	–
More than severe	20 (10.4)	–
Very severe	27 (14.0)	–

Note. ALS: amyotrophic lateral sclerosis; ALS-FRS-R: Amyotrophic Lateral Sclerosis Functional Rating Scale – Revised; PMA: progressive spinal muscular atrophy.

^aDue to a human mistake, no information is available on caregivers’ gender.

^bOther nationalities include Belgian (n = 1), German (n = 1) and Italian (n = 1).

^cEducational level refers to the highest level of education completed.

^dFunctional involvement of one central nervous system (CNS) region. This group also includes patients who did not report any impairments (n = 4).

^eFunctional involvement of two CNS regions.

^fFunctional involvement of three CNS regions.

^gNeed for gastrostomy or noninvasive ventilation.

Figure 1 Per-item frequencies for enacted stigma among patients (N = 193).

Figure 2 Per-item frequencies for enacted stigma among caregivers (N = 87).

Table 2 Manifestations of enacted stigma identified from the interviews.

Themes	Subthemes	Definitions	Example quotes
Social exclusion	Workplace discrimination	Experienced discrimination or intolerance in the workplace (e.g. being forced to quit one’s job).	“And when I came back from holiday and talked to the company doctor, he literally said ‘How can I report you better? You no longer have a future.’ I found that so harsh. I have worked very hard year after year, I enjoy my work. It’s the only thing you have to hold onto in your life, actually. And, simply because you have a diagnosis, that is stopped and someone else decides about that”. (patient)
	Relationship distancing	Friends, relatives, neighbors or colleagues avoid/move away from the person with ALS.	“My acquaintances find it difficult. So they don’t come by very often. They sometimes phone, but not like they used to”. (patient) “Those kinds of conversations don’t take place and at a certain point, it just fades”. (patient)
	Exclusion in conversations	Not being included in conversations (e.g. people talking to caregiver instead of patient).	“In conversations with more than 1 person, I am easily forgotten. People mean well, but are impatient. They easily forget to involve you”. (patient) “At first, I was constantly being asked how he [partner with ALS] was doing while he was right there. And I think people do that anyway if someone is in a wheelchair. I said: ‘Well, you can ask him yourself. Just ask’”. (caregiver)
	Exclusion in social events	Being excluded in social events/gatherings (e.g. not being invited to birthday parties). This also includes being invited to events wherein you cannot participate.	“Then you have the team outing, playing volleyball. That is great, but I can’t do that”. (patient) “If there are birthdays, that they say ‘Oh she can’t come, can she?’” (patient)
Stigmatizing attitudes and responses	Being avoided	People looking away or walking the other way.	“When we walk together, she in the wheelchair and I behind it, you notice the effect of limited greetings and contact”. (caregiver) “No verbal responses, but people do react. Looking away, looking the other way”. (caregiver)
	Staring	People staring at the person with ALS.	“The first couple of times when I was in the wheelchair…Then you actually get to go somewhere now and then. People stare at you from head to toe”. (patient) “When you go into the shop with that [dropped head syndrome], people will look. And if your walking gets worse and worse, people will look at you”. (caregiver)
	Over-concern or over-attention	Receiving excessive attention or sympathy from others.	“We are sitting there having dinner with friends and then a woman leans over me and says: ‘You must be enjoying yourself, aren’t you?’, but she doesn’t say it to the four of us. No, she really leaned over me while saying that”. (patient) “People who know us superficially, like the neighbors, they are always a bit careful and “Are you okay?” and that sort of thing”. (caregiver)
	Patronizing	People acting over-protective or like they know what you need (e.g. giving unsolicited advice on how to handle the ALS situation, offering unwanted help).	“I'm getting worse and people see that, obviously. And then they kind of want to protect you. And I think: I'm still good enough to take care of myself”. (patient) “Now that I reported ill, someone said: ‘Good that you’re here today and if you manage to do something, that’s fantastic. And then I thought: no, you can actually count on me’”. (caregiver)
	Others feeling uncomfortable	The experience that other people feel uncomfortable being around or talking to you (e.g. being afraid to ask questions).	“I notice that people are wary about asking [about my condition]”. (patient) “They may be a bit cautious at times or they don’t dare to ask a certain question. While you sense that they are curious”. (caregiver) “You don’t see a lot of colleagues anymore. With ALS, they hardly dare to come around”. (caregiver)
	Incomprehension	Extent to which people living with ALS receive reactions of incomprehension from their surroundings, including friends, relatives and acquaintances, but also professionals and authorities.	“I do have that with certain people. That they thought I could do more than I can”. (patient) “But also, for example, if you need aids, they sometimes say “Yes, it will come in 6 weeks”. Then I think “No, I need it this week”. I sometimes find that there is a lack of understanding in such things. Sometimes you have to explain very clearly what ALS is and why you need it so badly”. (patient) “People see what they see and don’t realize that speech and walking are the tip of the iceberg for me”. (patient)

Figure 3 Per-item frequencies for felt stigma among patients (N = 193).

Figure 4 Per-item frequencies for felt stigma among caregivers (N = 87).

Table 3 Manifestations of felt stigma identified from the interviews.

Themes	Subthemes	Definition	Example quotes (English)
Alienation	Feeling a burden	Feeling a burden to the people surrounding you.	“I often feel very guilty. I know I can’t help it, but I burden everyone with my problem”. (patient) “I get the feeling from them [family] that I should feel guilty”. (patient)
	Feeling ashamed/ embarrassed	Experienced feelings of shame/embarrassment associated with the health condition of oneself or one’s partner (e.g. feeling ashamed of physical appearance or speech, having to feed one’s partner).	“It’s still uncomfortable being washed by someone else”. (patient) “At first, I was embarrassed about my speech, but not anymore. I am now embarrassed to eat in front of others, I spill and slurp quite a bit”. (patient) “At first, you sometimes feel embarrassed about situations when you go out to dinner and he spills things or I have to feed him or something”. (caregiver) “At a certain point, she [partner with PMA] found it a bit embarrassing that she was walking badly and always needed support”. (caregiver)
	Feeling different	Feeling different or “other” compared to the people (without ALS) surrounding you.	“I do feel different, yes. Of course, I can do less”. (patient) “You can’t really participate, because you’re different, because you’re limited. Everyone walks and talks and you can’t walk, you can’t talk. I don’t want to say it’s isolating, but it’s a different position than before”. (caregiver)
	Feeling inferior	Experienced feelings of inferiority associated with the health condition of oneself or one’s partner.	“It is very difficult for me to accept that I can do less and less. It makes me feel inferior”. (patient) “I would have loved to do nice things with my adult children, but I hardly ever do that anymore. So, as a mother of grown-up children, I am worth less and I feel a little less worthy”. (caregiver)
	Feeling isolated	Feeling alone/lonely/isolated due to the health condition of oneself or one’s partner.	“You are just isolated in your own body”. (patient) “I think there is a kind of loneliness and I think that is manifesting itself more and more and more, the longer the process lasts”. (caregiver)
	Feeling left out	Feeling left out/excluded due to the health condition of oneself or one’s partner.	“My family are getting on with their own things, I feel like I'm out of it”. (patient) “Of course you still hear stories about certain people. On the one hand, that’s nice. On the other hand, it’s also annoying, because you are no longer part of it”. (patient) “I don’t count anymore, in real life”. (caregiver)
	Feeling that others are incapable of understanding you	Feeling that the people surrounding you are incapable of understanding your life with ALS.	“Then you also notice that people cannot imagine what it is like for me”. (patient) “There is really no one who actually knows what I experience and feel”. (patient) “I can hardly explain it, what it is like, how difficult it is and what it takes. You have to go through that”. (caregiver)
Perceived discrimination	Feeling not accepted	Feeling not accepted by the people surrounding you.	“I think I do accept myself, but I feel that others find that more difficult”. (patient)
	Feeling ignored	Feeling ignored/not seen by others (e.g. caregivers feeling they are solely treated as an extension of the patient with ALS).	“I would expect society, but also my immediate environment, to be more involved. Asking more about it, maybe offering help, one way or another. That’s not happening”. (caregiver) “This is obviously a disease that you have together, because as a partner you take over more and more. […] But, you notice that as far as the outside world is concerned, it is only my husband who is ill, and the illness affects him. And that’s where all the attention goes”. (caregiver) “I'm not just an extension of [partner’s name]. Yet, that is how I am approached, certainly by the medical world”. (caregiver)
	Feeling judged	Feeling judged or negatively viewed by others (e.g. feeling that people no longer view you as the person you was, for medical decisions, looking old, people thinking that you’re drunk/dumb).	“And then you also see people looking like: ‘Okay, why can’t she do that herself?’” (patient) “I've often noticed that people are startled by the way I talk, think I'm deaf or stupid, and even run away”. (patient) “My husband has been very clear from the start about how far he wants to take it and that quality of life is most important to him and that he doesn’t want to go on to the very end. […] He talked about euthanasia from the start. Then you also notice that that puts people off or that they have a certain judgment about it”. (caregiver)
	Feeling watched	Sense of being watched by other people.	“When I get up from the chair, I have to think for 2 seconds: ‘okay, what position are my feet in? Can I get right above my legs?’ If that’s the case, then I slide and then at a certain moment 1, 2, 3 and then a kind of swing and then I'm standing. I don’t notice it anymore, but you know, everyone is watching: is he going to make it or not”. (patient) “My wife can’t eat independently. Well, she can, but with a lot of tools. And of course (a) you can’t do that in a restaurant and (b) if you can, it is very noticeable. And I don’t want to say that this has an isolating effect for us, but it is nevertheless a special position that you’re in, as if you are always on a stage. It’s not like that, but it feels a bit like that”. (caregiver)
Anticipated stigma	Anticipated shame	Expectation that feelings of shame/ embarrassment will arise under certain circumstances (e.g. when the disease progresses).	“Eating is still going well. But I do notice movement all the time. At some point, I start to tremble. I know that at a certain point I will find that annoying, if it happens in front of others, even if they are my friends so to speak. Suppose you go to a restaurant or somewhere, people will look at you”. (patient) “Yesterday afternoon, I was just sitting working and then he [partner with ALS] suddenly said, ‘I know that I can now, in my wheelchair, for example, pick up the boys from school with you, but I dread seeing the faces of people, that they pity me or that they see how ill I am’”. (caregiver)
	Fear of exclusion	Fear of being excluded or treated differently due to the health condition of oneself or one’s partner.	“You know, I really don’t want to be written off. […] Like someone who just sits at home. You always want to carry on being someone”. (caregiver)

Table 4 Responses to stigma identified from the interviews.

Themes	Subthemes	Definition	Example quotes
Concealing responses	Social withdrawal	Withdrawal from or avoidance of social interactions/situations (e.g. visiting friends, birthday parties, going to a restaurant or theater), due to (anticipated) experiences of stigma (e.g. feeling a burden/embarrassment).	“I find it embarrassing that I can’t get up out of a normal chair. And if I fall, then I need help, to get up. I find that frustrating. Of course, this means you’re going to avoid doing things. I no longer go to the theater, I no longer go to the opera, I no longer go out by car to visit things”. (patient) “That was also a bit of her embarrassment, to not have too much social interactions anymore as she thought she looked really bad at the end”. (caregiver)
	Secrecy	Maintaining secrecy about the health condition of oneself or one’s partner.	“My wife, who did have a positive attitude, never actually told her friends, even her closest friends, how bad her condition really was”. (caregiver)
Resisting responses	Deflecting	Rebuffing other people’s (negative) attitudes or responses as irrelevant.	“That other people look at me, or whatever, I couldn’t care less”. (patient) “I don’t feel uncomfortable because I'm with him. I sort of think, you have every right to be there and I'm not going to hide or anything”. (caregiver)
	Positive outlook	Focusing on the positives rather than on the negatives.	“Of course, there are days when I'm angry, and all I can do is cry all day. But that’s once in a while. I wake up happy every day and try to look at the positives”. (patient) “I still see the positive sides of [partner’s name] more than the ill sides”. (caregiver)
	Disclosure	Opening up about ALS and its limitations to other people (e.g. family, friends, relatives, neighbors, colleagues).	“Whether they ask what I still can do with my hands or ask if, so to speak, I can still poop. I don’t care what. They are allowed to know everything about me. I don’t just tell them everything myself, but I answer every question openly”. (patient) “I think you can only expect understanding from people if you are open about what you are confronted with”. (caregiver)

Table 5 Outcomes of multivariable regression models assessing associated factors of enacted and felt stigma among patients (N = 193).

	B	SE B	β	t	p-Value	R^2
SSCI – enacted stigma
Constant	3.91	0.66		5.98	.000*	0.22
ALS-FRS-R – Bulbar score	0.07	0.03	0.17	2.35	.020*
Clinical stage 2^a	0.09	0.26	0.03	0.34	.731
Clinical stage 3^b	0.58	0.27	0.22	2.18	.031*
Clinical stage 4^c	0.34	0.28	0.12	1.20	.230
Gender	0.31	0.19	0.11	1.68	.095
Age	−0.03	0.01	−0.28	−4.00	.000*
Partner	−0.48	0.22	−0.14	−2.15	.033*
Work	−0.35	0.25	−0.10	−1.39	.165
SSCI – felt stigma
Constant	4.13	0.82		5.07	.000*	0.19
ALS-FRS-R – Bulbar score	0.08	0.03	0.18	2.42	.016*
Clinical stage 2^a	0.75	0.33	0.21	2.29	.023*
Clinical stage 3^b	1.04	0.33	0.32	3.14	.002*
Clinical stage 4^c	0.67	0.35	0.20	1.92	.057
Gender	0.28	0.23	0.08	1.22	.224
Age	−0.04	0.01	−0.24	−3.39	.001*
Partner	−0.07	0.28	−0.02	−.26	.797
Work	−0.35	0.31	−0.08	−1.14	.256

Note. SSCI scores were transformed using square-root transformation.

ALS-FRS-R: Amyotrophic Lateral Sclerosis Functional Rating Scale – Revised; SSCI: Stigma Scale for Chronic Illness.

*p<.05.

^aFunctional involvement of two CNS regions.

^bFunctional involvement of three CNS regions.

^cNeed for gastrostomy or noninvasive ventilation.

Data availability statement

The data that support the findings of this study are available from the corresponding author, MSS, upon reasonable request.