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• To recognise the profound physical, social and psychological effects of lymphoedema on the lives of children and young people
• To identify the importance of early diagnosis and referral for assessment and treatment
• To gain a greater understanding of the challenges experienced by children and young people with lymphoedema, and their families
Background Lymphoedema is a chronic condition that requires lifelong, time-consuming and laborious management. It can have significant effects on physical, psychological and social well-being. Children and young people with lymphoedema require access to expert services to aid early diagnosis and referral for assessment and treatment.
Aim To explore the perspectives of children and young people living with lymphoedema and those of their families, as well as their experiences of the national paediatric lymphoedema service in Wales.
Method A qualitative approach was adopted using semi-structured interviews with participants who had been referred to the national paediatric lymphoedema service in Wales.
Findings A total of 15 families were interviewed. Five themes were identified: lack of professional awareness of paediatric lymphoedema; a journey to diagnosis as lost in the system; ‘being me’ – what it feels like to have lymphoedema; managing lymphoedema and feeling supported; and the benefits of a national paediatric lymphoedema service. Two sub-themes were identified within the ‘being me’ theme – body image and self-esteem, and loss of control.
Conclusion Lymphoedema has a profound effect on daily life, body image and self-esteem. Participants tended to be resilient and determined to continue with their lives. Importantly, they valued being under the care of a service that understood their condition.
Nursing Children and Young People. doi: 10.7748/ncyp.2021.e1363
Peer reviewThis article has been subject to open peer review and checked for plagiarism using automated software
CorrespondenceElizabeth.Coveney@wales.nhs.uk eccoveney@gmail.com
Conflict of interestThis work was developed by members of the Lymphoedema Network Wales National Team
Thomas M, Gabe-Walters M, Coveney E (2021) Living with lymphoedema: children’s and young people’s perspectives. Nursing Children and Young People. doi: 10.7748/ncyp.2021.e1363
AcknowledgementsWe would like to thank the children and young people and their families for contributing to improve and inform future provision. Our personal thanks the Swansea University researchers for completing this study. We hope that this research demonstrates the importance and value of lymphoedema management for children and young people
Published online: 09 August 2021
Lymphoedema is an incurable long-term condition caused by failure of the lymphatic system and is thought to affect more than 200,000 people in the UK (NHS 2019). There are two main types of lymphoedema (Woods 2007):
• Primary lymphoedema caused by faulty genes that affect the development of the lymphatic system. It usually starts during infancy but can present later in childhood or adolescence.
• Secondary lymphoedema caused by damage to the lymphatic system, for example because of cancer treatment, infection, trauma and immobility, among other causes.
Symptoms of lymphoedema include swelling, pain, reduced mobility, skin conditions and increased occurrence of cellulitis (Morgan et al 2005). Lymphoedema requires daily management but it is time-consuming and laborious, relying on massage, compression therapy, exercise and attentive skin care.
In a UK study that explored the experience of 20 children and young people aged 6-18 years with lymphoedema and that of their families, Moffatt and Murray (2010) identified delays in diagnosis and in access to knowledgeable and expert clinicians. Delays in diagnosis mean that parents often become advocates for their child’s healthcare. Body image issues were also identified in the study, with young people experiencing challenges in wearing fashionable clothes and shoes. Lymphoedema was also found to have significant effects on psychological and social well-being, for example children did not want to meet, socialise or play with other children due to fear that their lymphoedema would be revealed. This indicated that children and young people with lymphoedema need access to services to aid early diagnosis and referral for assessment and treatment.
Lymphoedema services for adults in Wales were initiated in 2011 following a review and publication of the Strategy for Lymphoedema in Wales, which identified variation in care due to the lack of dedicated services (Welsh Assembly Government 2009). However, lymphoedema services for children were uncoordinated, resulting in a lack of seamless care. In 2015, the Welsh Government acknowledged that care was inequitable and supported the establishment of a national paediatric lymphoedema service to provide quality and consistent lymphoedema care for children and young people aged 0-25 years. The service started in November 2015 and has received 402 referrals from healthcare professionals including doctors, nurses and allied health professionals.
Lymphoedema Network Wales is the body that was set up to maintain a strategic oversight of lymphoedema services in the country. Researchers from Swansea University were commissioned by this body to undertake a study to explore the health and well-being of children and young people with lymphoedema in Wales (Maunder et al 2017). The purpose was to understand their experiences of the national paediatric lymphoedema service to inform and improve future care provision. This article was developed from the unpublished full study report completed by the Swansea University researchers.
• Healthcare professionals should be aware that children and young people may not want to educate them on what lymphoedema is but may be happy to explain how it affects their lives
• Managing lymphoedema can be challenging and healthcare professionals should be aware of how this can affect a family’s daily life
To explore the perspectives of children and young people living with lymphoedema and those of their families, as well as their experiences of the national paediatric lymphoedema service in Wales.
A qualitative approach was adopted using semi-structured interviews to promote open dialogue with children and young people and their families. Interview questions were derived from the literature, for example: ‘I’d like to know more about what it’s like to have lymphoedema. Can you tell me what it’s like for you?’ (Braun and Clarke 2013).
All 130 children and young people with primary and secondary lymphoedema who had been referred to the national paediatric lymphoedema service and their parents were sent an age-appropriate information pack containing details about the study between September 2016 and January 2017 (the study period). There were no exclusion criteria. Interested potential participants contacted the national paediatric lymphoedema service, who then informed the researchers at Swansea University. Consent and assent forms were included in the information pack and completed accordingly. Parents were able to attend the interview and were active participants. For children under seven years of age, parents were able to participate without their child.
The interviews were conducted during the study period in the family home by two researchers from Swansea University and each lasted approximately 60 minutes. They were audio recorded digitally.
The interviews were transcribed verbatim. Transcripts were managed and coded by hand. All transcribed text was analysed by the research team using Braun and Clarke’s (2006) framework for thematic analysis until a consensus was reached on the main themes.
Fifteen families were included in the study. A total of 13 children and young people (ten females and three males) aged between seven and 22 years were interviewed. Of these 13 children, 11 were interviewed with their parents (n=13) and two over 16 years old were interviewed without their parents. In addition, two parents were interviewed without their children who were between two and five years old.
Five themes were identified from the interviews, with two sub-themes:
• Lack of professional awareness of paediatric lymphoedema.
• A journey to diagnosis as lost in the system.
• ‘Being me’ – what it feels like to have lymphoedema. This included two sub-themes:
• Managing lymphoedema and feeling supported.
• Benefits of a national paediatric lymphoedema service.
Participants described repeatedly the need for healthcare professionals to show more awareness and understanding of lymphoedema. They described experiences of healthcare professionals who were unable to identify lymphoedema promptly, resulting in unnecessary tests, investigations and treatments:
‘I was born with it… When I was younger, I had an MRI [magnetic resonance imaging] and they said they couldn’t do anything… I didn’t see anyone for six years… Then I went back to the doctor because it was painful.’ (Family 1)
‘When I say I have epilepsy people say “Oh” like they know, but when I say I have lymphoedema they are like “Sorry what is that?” and they don’t understand and when I describe it, even then they don’t get it.’ (Family 2)
After diagnosis, some parents described having to access NHS services out of area, and so had to travel from Wales into England, requiring long journeys and overnight stays that resulted in extra financial costs and disruptions to family life. Families often felt misunderstood, isolated and were tired of explaining lymphoedema to healthcare professionals:
‘We had seen doctor after doctor and specialist after specialist and no one mentioned any of these things… I don’t know what to call it, I just knew her leg was swollen and wasn’t right but I didn’t know that that is what it was.’ (Family 3)
Young people thought that an increase in public knowledge would avoid misinterpretation of lymphoedema:
‘There are people out there who have got a swollen foot, and they don’t realise they have lymphoedema… I am not sure if doctors know about it sometimes.’ (Family 4)
Most participants reported challenges in obtaining a lymphoedema diagnosis leading to hardships:
‘They were slow to identify the condition because we were back and forth [to] the chiropodist for the best part of a year and nothing was happening… No one seemed to know.’ (Family 2)
‘In the beginning it was a bit of a nightmare… Yes, those two weeks are a long time and are hell – because you are thinking the worst and hoping for the best… A year down the line and still not knowing… We just wanted to know what it was and get her the best treatment she needed and move forward.’ (Family 5)
The time between first noticing something was wrong and receiving a diagnosis varied from eight months to eight years. Participants reported numerous visits to various healthcare professionals, which indicated inadequate professional knowledge and expertise of paediatric lymphoedema. This led to delays in diagnosis and caused considerable distress to parents. Parents were advocates – ‘knowing something was wrong’ – but they were dismissed and told that their child was overweight or would grow out of it:
‘The GP prescribed antibiotics and said that the child was overweight, I queried why he was in pain if he was just overweight and he brushed me off and we were left again with nothing.’ (Family 6)
Children and young people described what it felt like to have lymphoedema:
‘My foot feels jelly-like… Like my right foot is weird when I’m walking on it –sponge-like and slightly wobbling.’ (Family 2)
‘Sometimes my hand turns blue… I will just wake up and it is sore.’ (Family 12)
Pain was a regular occurrence for many:
‘I do get pain… High heels make my ankles swell a lot… When I start walking my knees start to feel horrible, like I can’t properly walk, and almost feel pudgy, almost like a bit inflamed.’ (Family 2)
‘When I wake up it is sore all day… Like lots of different points where it can hurt from, and they are all different… It is achy, it is a sharp pain and then it is everything.’ (Family 13)
Parents described feeling helpless when their child experienced pain:
‘It was hard watching such a young child go through pain and not knowing what to do… You could see he was crippled in pain and… It is like a banging sensation when the pressure plays up… He curls up in a ball… We end up going to accident & emergency or the doctors and they say give him paracetamol but that is not strong enough so it is like knocking on a brick wall.’ (Family 7)
One young person reported that she tried to continue with usual activities but this was sometimes challenging:
‘I do play the piano and guitar… When I was little I used to play the violin but we stopped that because it was the arm – where you hold it up… But I don’t play the piano as much as I would like to.’ (Family 8)
Young people were able to articulate how their lives were affected by lymphoedema. Some reported being acutely aware of the effect on their body image:
‘I remember going on Duke of Edinburgh [award] and it was hot and we were going through this brambly bush and I was the only one wearing trousers because I didn’t want to get scratches over my legs. Everyone else was wearing shorts.’ (Family 2)
‘I was dancing all night… Afterwards I had to keep my legs up, and the swelling wouldn’t go down for a few days.’ (Family 4)
A few described feeling insecure when forming new relationships, being self-conscious of their physical appearance and having to wear compression garments. Frustration and anxiety were also evident related to being unable to wear the latest fashion in shoes and clothing or concern about the attitude of others:
‘I was worried about how it was going to look like to my boyfriend. Is he going to want to be with someone who wears these socks?’ (Family 4)
‘If I was going out on a girly day in a dress, I wouldn’t wear them [compression garments].’ (Family 4)
Many of the children and young people reported being unhappy about the compression garments, which were functional rather than stylish, feeling conscious of being different to their peers. Some parents also noted the effect on body image:
‘You don’t want her to appear different… Embarrassing for her when she went on holiday on the beach with friends and she didn’t want to say, “I need to take my socks off” – she didn’t want people to see her feet…’ (Family 9)
Children and young people reported a lack of control in managing their lymphoedema in school as they could not stretch or elevate their limbs, which differed from being at home. Sports lessons were an issue as the children were conscious that their compression garments were visible underneath their sports kit. This forced disclosure to fellow pupils resulted in loss of control and privacy:
‘When I have swimming or PE or games the stockings have rubber marks and I have to tell people what it is.’ (Family 10)
‘He can’t go out in shorts if he has got this stocking up to his knees.’ (Family 11)
Parents worried that their child would be bullied or not accepted in school. They were anxious about the potential effect on their education:
‘When he is in trousers the socks come up to his knees and even if he is in shorts they look more like tights… People have picked on him… “Why are you wearing girls’ socks?”… He doesn’t like standing out… It is embarrassing.’ (Family 11)
Having experienced long waits for a diagnosis, parents were pleased that treatment and management strategies were available. However, they also reported that completing the daily treatments was overwhelming and establishing a routine had to fit with family life:
‘We try to get him to bounce up and down in the day and do as much exercise as possible and… A little rub on his legs with cream at the end of the day and just try and manage it.’ (Family 7)
‘We massage them in the night and soak them in Vaseline… She has the socks on all day.’ (Family 5)
Participants were pleased to understand some of their symptoms and to know that support was now available:
‘It is nice to know there is a reason why it is puffy and it is nice to have someone to go to – to tame the puffiness and make it look a bit more like this leg. I know they will never look identical but it is nice to look a bit more normal, not one thinner and one puffy.’ (Family 3)
Most participants reported that their friends or partners were supportive of their condition:
‘It would be different if his own friends were horrible about it, but they are not.’ (Family 6)
‘He [boyfriend] actually puts them on for me [compression garments] sometimes, and pulls them off sometimes, and makes a joke.’ (Family 4)
Participants stated repeatedly the need to meet others in similar situations to gain support and exchange tips and information:
‘It would be nice… To have somebody my age roughly that I could talk to would be quite nice as they have been in your shoes and know what you are doing, you are not just left by yourself.’ (Family 12)
‘It is their normal isn’t it but it is not normal to everyone else… Particularly at her age now, if she had other people that she could link up with who also had a leg that didn’t look the same as the other one.’ (Family 3)
Parents stated repeatedly that they valued being able to receive supportive care from the national paediatric lymphoedema service.
Most participants were positive and complimentary about the national paediatric lymphoedema service. They expressed feelings of relief that they were under the care of professionals who were experts at treating and managing lymphoedema and understood their concerns and needs:
‘They [the service] were helpful… Getting me to try out some tapes to use on my feet as she [the therapist] was worried [about the increasing foot swelling].’ (Family 2)
‘I just want to say that the help I have received from the service… They have gone out of their way to help me… They understand.’ (Family 4)
Parents also felt less vulnerable and were relieved because they did not have to actively seek out the right medical attention. They valued having one point of contact and coordinated care:
‘She arranged for us to see the vascular anomalies man… She arranged to be down there at the same time. I have never seen that joined up thinking anywhere.’ (Family 3)
Families living in rural areas particularly appreciated having an outreach service nearer to home, which meant they avoided having to make long journeys, reducing time away from school and, for parents, time away from work. Flexibility to work around the school day was appreciated by parents, children and young people:
‘Would book the appointments for the end of the day so that it meant he didn’t miss much school then.’ (Family 6)
Participants valued having access to a service where they thought professionals understood their condition and what it felt like to live with lymphoedema:
‘We were a bit worried about flying and what would happen on the flight and what if she got an infection but they [the service] were helpful.’ (Family 9)
Parents thought that the national paediatric lymphoedema service addressed the specific needs of their child. They valued receiving care near their homes and being taught techniques to manage the condition from experts.
The findings from this study have provided greater understanding of the challenges experienced by families and have informed service delivery and opportunities for development of the national paediatric lymphoedema service in Wales, for example, seeing the patient jointly with the consultant to reduce time away from school.
This study found that lymphoedema was a constant throughout the lives of children and young people and their families, with profound physical, social and psychological effects.
Delays in diagnosis and treatment have been reported previously (Moffatt and Murray 2010, Hanson et al 2018, Moffatt et al 2019a, 2019b). This study found delays of up to eight years to establish a lymphoedema diagnosis, which was distressing for children and their families. Parents also reported that, before the establishment of the national paediatric lymphoedema service, care was not coordinated, resulting in unnecessary examinations and investigations. For some, although travel to a specialist centre across the Welsh border yielded a diagnosis, it was costly.
Previous studies have described the experiences of some parents, who knew something was wrong but received no information from healthcare professionals, which left them feeling anxious about the diagnosis or let down by the system (Todd et al 2002, Moffatt and Murray 2010). Harding (2012) described a lack of ‘professional awareness’ of lymphoedema as a potential cause of these delays, which was echoed by the participants in the current study.
Moffatt et al (2019a, 2019b) found that the process leading to diagnosis was expedited when there was access to a specialist lymphoedema service. Families participating in this study agreed that it was positive to have a local service to support them in managing their condition, and that there were numerous benefits of having a national paediatric lymphoedema service. These included the coordination of appointments, thereby reducing high travel costs and supporting school and work by arranging appointments at a mutually convenient time.
The current study demonstrated the significant effects on children and their families of living with lymphoedema every day. Hanson et al (2018), interviewing families in Australia, reported similar findings, with their participants reporting low self-esteem and restrictions on their lifestyle. Harding (2012) also found that those with lymphoedema reported issues with self-esteem and self-image. Moffatt and Murray (2010) found that family life was described in terms of integrating daily management with family routines. This sense of making lymphoedema ‘fit’ was echoed in the current study with concerns about self-esteem, body image, school and forming relationships. The pain often described by the families indicated concerns about ongoing management, educating healthcare professionals about the effect of lymphoedema and ensuring that families found the appropriate support.
The families also discussed the anxiety they experienced when having to take part in activities, such as sports at school or going to the beach, as a result of the child needing to wear compression garments for management or a desire to keep the affected limb hidden; similar experiences were also described in Hanson et al (2018) and Harding (2012).
Current management strategies for lymphoedema involve daily rituals of skin care and wearing compression garments. Exercise is also an important component of management. In this study participants reported feeling overwhelmed at the prospect of establishing a routine, which reflects previous research. According to Moffatt et al (2019a, 2019b), the control of lymphoedema requires persistence with treatment and a personal belief in ability to manage lymphoedema so that it remains under control. These findings emphasise the need for healthcare professionals to understand the potential challenges of lymphoedema management.
The study represented only 15 families in Wales living with lymphoedema and therefore it is not possible to generalise to the wider population. Furthermore, this article was developed from an unpublished full study report completed by Swansea University researchers and personal interpretations from the authors may have inadvertently caused bias through their selection of aspects of the full report.
This study was conducted to explore the health and well-being of children and young people with lymphoedema in Wales and to understand their experiences of the national paediatric lymphoedema service to inform and improve future provision. The findings provided greater understanding of the challenges experienced by families and have informed service delivery and opportunities for development of the national paediatric lymphoedema service. Further research is recommended in several areas, including raising awareness of lymphoedema and its effect on daily life, pain management, and strategies to alleviate the effect of lymphoedema on daily activities.
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