Growing up with juvenile scleroderma
What is it really like to grow up with scleroderma? We look at the various ways that this condition can affect our children, both physically and emotionally, as they navigate the path to adulthood.
Localised scleroderma is the most common form of scleroderma in children, accounting for over 90% of all juvenile cases. It is more prevalent in girls and although symptoms can present at any age, the disease often becomes apparent by the age of nine.
Localised scleroderma mainly affects the skin and connective tissue. It typically causes patches or lines of hardened skin to develop, usually upon the trunk of the body, on the limbs or on the face. It is not thought to overlap with systemic sclerosis, which can also attack other systems of the body and occurs far less frequently in children.
About juvenile scleroderma
The cause of juvenile scleroderma remains unknown, and although no clear inheritance pattern has been identified, multiple cases do sometimes arise within a family. There may be a link to family history of other autoimmune conditions, and it is only through research that we may fully understand the true extent of the relationship between genetics and scleroderma.
Juvenile systemic sclerosis is extremely rare, accounting for less than 10% of all cases in children. This will be a serious, long-term condition that may progress over the years; however the prognosis is generally far better for affected children than adults. Systemic sclerosis can affect any part of the body and is usually characterised by Raynaud's and thickened skin. It may also impact upon the internal organs, including the heart, the lungs or the kidneys; therefore continued monitoring and treatment is likely to be required.
Early diagnosis and prompt treatment will be crucial, in order to reduce the symptoms and limit any lasting damage. Since localised scleroderma involves the skin, it may also affect the underlying fat, muscle and bone if it remains untreated, leading to longer-term complications.
Treatment options will vary depending on how the child is affected by their condition: for example, medication to dilate the blood vessels may help to alleviate Raynaud's attacks. Some children are treated with anti-inflammatory medication, with drugs such as Methotrexate shown to improve skin symptoms in many cases. Occupational therapy may be recommended, as well as physiotherapy to help in maintaining movement and flexibility within the joints and the skin. In cases of systemic sclerosis, the treatment plan may be more complex, depending on how the condition is progressing.
Growing up with a chronic illness
For most children with localised scleroderma the prognosis is generally good, at least from a medical perspective. Because scleroderma literally means 'hard skin,' it is characterised by skin involvement for many young people. This can affect a child's appearance and impact significantly upon their self-confidence. A great deal of growing up naturally occurs during the teenage years, as children face academic pressures, exam stress and fluctuating social dynamics. Living with scleroderma may represent an additional challenge, as children may have to miss school and social activities as their condition dictates, as well as dealing with the side effects of certain medications.
Everyday stress and anxiety are known to trigger flare-ups of various autoimmune conditions, and scleroderma seems to be no exception. It appears that there may be a connection between emotional stress and disease activity; as young people living with this condition have reported that during particularly stressful times, their symptoms become worse.
As ever, finding the right support is key, and it is important that this is available for children of all ages in negotiating the various aspects of growing up with scleroderma. Since every child is different, it is important that everyone is able to access the level of support that they need. If you are concerned about your child, talk to the school in the first instance. Your GP can also make a referral to psychological support services if you feel that your child would benefit, and your nurse specialist may also have some practical suggestions to help. Scleroderma can impact upon the whole family in a number of ways, so if you would like some additional support or to connect with other affected families, please contact our Helpline or visit our online community and social media platforms.