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. 2019 Oct 16;178(12):1883–1892. doi: 10.1007/s00431-019-03469-7

Table 3.

Overview of themes on the impact of MFS on functioning, disability and contextual factors acting as a barrier or facilitator supported by the data: quotes from adolescents with MFS aged 12–18 years

Themes Quotes
Difficulties in keeping up with peers School “Yes, Marfan’s does take up a part of my life, because I have to go to the hospital very often, for appointments and other things. I try to schedule my visits to the hospital so they do not conflict with school, and I do not have to miss any classes, but that is difficult. It is usually very busy at the hospital, and the appointments have to take place during school hours. This means I miss many classes. And I cannot participate in a lot of activities during Phys-ed.” A6
Sports “I really notice that I am not always able to participate. In sports, in particular, I often have to give up earlier. My knees ache a lot, so many times I stop before we are done.” A8
Leisure “I had problems last year; we went to Disneyland in Paris, and I was exhausted after only an hour and a half. At the time, my back, ankles and knees were really bothering me.” A13
Friendships/relationships “Well most of the time I am too tired to meet with friends after school and then, at home, I sit down on the couch to relax. So I do not really have time to meet with someone, that’s hard, but I usually play a game on my phone or I play online games together with friends. “A8
Work “At first, I had a part-time job at the local drugstore, stocking shelves, but I had to quit because my back was causing a lot of pain. So, I had to make a decision. I mean, it is a shame, because it was nice to earn some money, but my health is my main priority.” A5
Being and feeling different from peers Appearance “I am taller and thinner than the other kids in my class, and I have spider hands.” A10
Fatigue “I went to a concert with some of my friends, and I was really very tired. I was thinking “this is not right,” but on the other hand, I realized that I had had a really good day, so...” A2
Pain “Yes, the pain makes me unable to bend my hand fully. I can now, but I was not able to do it yesterday, and usually, it stays that way for a couple of days. I do not know if I strained it, or if it is caused by too little connective tissue, but when it happens, I really have a lot of pain in my wrist. I often drop my phone, I just lose my grip on it.” A1
Activities

“I have an elevator pass that allows me to use the elevator, because taking the stairs is too hard, and I have a second set of books, so I do not have to carry them from home to school. The second set is kept at school, in my locker, so my bag is lighter. It prevents me from having to lift things, and keeps me from getting tired so quickly.” A3

“I am able to clean my room or do things like that, but when we go grocery shopping, my mother will tell me I do not have to carry the heavy items because I cannot.” A12

Feeling different “It bothers me sometimes, being an exception. I am insecure because you can see that we are very tall or very thin, and people will notice the dent in my chest, and ask me “what’s wrong with you?”; It’s not always nice, having to tell them you have Marfan’s, and I have to keep telling them over and over.” A4
Contextual factors reportedly acting as a barrier or facilitator
Coping with MFS Acceptation “In the beginning, when I was younger, I had a hard time dealing with the fact that I have Marfan’s. But then I accepted the fact that I have an illness, and this is here to stay for the rest of my life, it is not going to change or anything. So accepting it really helped, and nowadays it’s no longer a problem for me.” A6
Humorous and relaxed outlook on life “Yes, I was disappointed when I had to give up basketball (highest junior league) because of Marfan’s because I really liked playing. But it’s in my character to try and make the best of the situation. So yes, now I am going to try to become as good as I can in my music.” A10
Pro-active/planning “I plan a lot in advance. For instance, if I know there are four tests (at school), I start preparing early, so I have less to do each day, and not two whole paragraphs on one day. That is too exhausting, and I get a headache if I do too much.” A18
Avoidance and denial “I really do not want to have anything to do with Marfan’s. I quickly go to the hospital, and that’s it for me.” A7
Self-esteem/image “When I went swimming, people would react to my chest, saying things like “look at that” and other things. Yes, it bothered me a lot, and it prevented me from going sometimes. I would think “I really cannot deal with this now” and not go.” A5
MFS knowledge I know you get thinner when you have Marfan’s, you can break bones easier, and your eyes can be more sensitive. And yes, your aorta also grows, or something. Other than that, I do not really know all that much about it.” A19
Ability to express needs “If something happens to me, I think I would like to be able to talk to someone who also has Marfan’s and who has experienced the same thing.” A15
Support and peer group acceptance Friends “Yes, I have explained what Marfan’s is to my friends, so they understand it completely, and they are considerate. One of them actually just sent me a message, wishing me good luck today.” A14
Parents “Yes, when I am worried, I can talk to my sister, and to my parents.” A18
Teachers/school “I was unable to take the stairs or to keep up with the rest during the Phys-ed classes. I also had trouble studying, because there were too many stimuli at school, and after school, I was so tired, so basically, I just slept a lot. And I always had too little time to study or to do my homework. Now, at my new school, they understand that I have Marfan’s, and I get a lot of guidance and support. I am doing a lot better now.” A13

MFS, Marfan syndrome