Childhood lymphoedema is a complex condition that can present in many ways and may lead to significant physical and psychosocial complications. The initial diagnostic journey often encounters misdiagnoses, referral to numerous inappropriate services, and delays in a referral to lymphoedema specialists. And there is often poor understanding and communication among teachers, leading to negative encounters at school. All of this can leave children and their parents frustrated and confused.
Dale (1985) examined the incidence of primary lymphoedema and estimated a frequency of 1 in 6000, with a sex ratio of approximately one male to three females. Childhood lymphoedema, however, is very rare. Smeltzer and colleagues (1985) calculated the annual incidence rate in children and young people under 20 years of age to be 1.15/100 000 population, but this is likely to be an underestimate.
Management
Management of lymphoedema is lifelong and is focused around reducing and re-routing the swelling by applying compression and carrying out lymphatic massage, maintaining healthy skin and preventing cellulitis, encouraging the natural lymphatic flow through exercise, and maintaining a healthy weight. Compression is the linchpin of management in lymphoedema and is delivered using a variety of methods including hosiery, bandaging, and adjustable wraps.
Difficulties with compression
Wearing compression on a lifelong, daily basis poses difficulties for some in terms of body image, ability to wear some styles of footwear or clothing, and time commitment needed to apply and remove the garments. These difficulties can be magnified in children and young people living with lymphoedema because of the additional normal issues with adolescence, such as peer pressure, body image and self-esteem.
Studies by Harding (2012) and Hanson et al (2018) examined the experiences of children and adolescents living with primary lymphoedema including having to cope with compression garments.
Younger children often feel they are treated differently because of their garments or targeted by bullies, which makes them reluctant to make new friends. Adolescents express a need to take a break from their garments because of the social isolation they cause (Harding, 2012; Hanson et al, 2018). Most children are unhappy with their hosiery, describing them as being inappropriate for their age and difficult to apply, and many wear clothing that conceals their garments. Conflict often arises between children and their parents regarding compliance with hosiery or shopping for clothes and shoes (Harding, 2012; Hanson et al, 2018).
Conflict can arise with teachers because of their lack of awareness and compassion because uniform policy cannot be adhered to or they assume that children cannot participate in certain activities because of their compression garments (Harding, 2012). This lack of awareness is not exclusive to teachers, however. Children feel that the medical community and wider public are also poorly informed about lymphoedema, and this affects their ability to ignore their garments and feel normal (Harding, 2012).
Compliance
Adolescents embrace the transition to young adulthood and increased responsibility for their treatment but, when given the choice, they tend to wear their hosiery less often. However, the transition from children's to adult services is a difficult one, especially if this means seeing different health professionals and possibly having to wear different garments. These difficulties are eliminated in services where both adults and children are treated.
Often there is discord between the child and the parent around issues of compliance with hosiery and the inability to wear normal clothing and footwear. Encouraging compliance with hosiery is a difficult balance to achieve but routine is helpful. Young adults who lack the stable routine of getting ready for school every morning often forget to wear their garments. Compliance can also be improved for newly diagnosed children if there is a gradual introduction to wearing hosiery on a daily basis to help get used to them.
Adapting treatment to meet the individual needs of the young person will help with compliance. Garments can be made to look similar to school socks or, by using really vivid colours and motifs, they can be fun and individual. Hosiery manufacturers have been keen to work with lymphoedema specialists to support the needs of children in terms of their compression garments.
Raising awareness
The British Lymphology Society (BLS) and the Lymphoedema Support Network (LSN) support a ‘Sock It to Lymphoedema’ campaign during Lymphoedema Awareness Week, encouraging those involved in or affected by lymphoedema to wear odd socks to raise awareness of the condition. Children with lymphoedema have been supported in this campaign by friends and siblings, and some have attended an activity day organised for children with lymphoedema and their families (Lymphaletics) wearing odd socks. Events such as this can help with compliance with hosiery by encouraging peer support and helping children with lymphoedema feel more normal. Having the opportunity to meet children with similar needs can help reduce the feelings of isolation and being ‘the only one with lymphoedema’. Children and their parents have also found being able to visit the hosiery company representatives' stands at the Lymphaletics event useful, where they have been able to see companies' entire range of garments and other products.
Difficulties facing health professionals
Specialist health professionals also face difficulty treating childhood lymphoedema. Lack of funding, training, and treatment guidelines means children are being managed by a small number of specialists in the UK, most of whom treat both adults and children. Although there are many studies looking at best practice in paediatric lymphoedema, none of these is of suitable quality to guide practice. A systematic review of conservative management of lymphoedema in children examined a total of 738 possible studies to find that only eight were suitable for inclusion (Phillips and Gordon, 2014). These eight studies looked at a range of treatment practices, including use of a pneumatic compression pump, low-level laser therapy, compression, and manual lymphatic drainage, but the reviewers concluded that only poor quality, low-level evidence is provided by these studies. There is a need for more robust research on treatment practices for children with lymphoedema and more up-to-date prevalence figures are required to help plan service provision. However, specialists have acknowledged the need to address these issues and, through groups such as the Children's Lymphoedema Special Interest Group (CLSIG), are working together to help improve service provision and management for children with lymphoedema. Unfortunately increased workloads and lack of support for research and service planning have a negative impact on this. Despite these difficulties, the author and colleagues working in this area have found that children and their parents feel greatly supported and develop a more positive outlook following assessment at specialist lymphoedema services.