Lupus presents in different ways in young people and requires evolving treatment strategies coupled with ongoing emotional support.
The last 10 years have been a roller coaster ride for Katrina and her mother Lilian. The search for a diagnosis and then Katrina’s diagnosis with lupus in 2009 had a dramatic effect on their lives, they say, as hospital appointment after hospital appointment became the new normal, and pediatric then adult doctors tried to figure out what was wrong.“They knew it was something rheumatological, but they didn’t know what,” says Lilian, remembering the worry that she went through over seeing her daughter so ill at the time.
But it wasn’t only Katrina’s physical health that was deteriorating; her mental health also suffered, with anxiety and depression rearing their ugly heads, which at age 9 was not something her mother had expected.
“I really wish that someone had said to me at that point [of her diagnosis] you need to consider the emotional impact of the disease, particularly as it has no cure; it’s not going to be over and done within the next year at all,” Lilian says
“I wish someone had had that discussion with me so that I could have supported Katrina better,” she adds. Low moments are “part and parcel of managing the dramatic life changes that happened because of this illness,” says Lilian. “Lupus is a multisystem disease that will affect you socially, emotionally, and financially.”
Childhood-onset SLE is very different from adult-onset SLE
Systemic lupus erythematous (SLE) can present in a myriad of ways in young people, which is very different than in adults, Clarissa Pilkington, MBBS, MRCP, explained recently at the British Society for Rheumatology annual conference in Liverpool.
In children, particularly those under the age of 6, lupus symptoms may be atypical and differ from child to child: These usually have a single underlying genetic component, such as C1q deficiency, which you need to look out for, Dr. Pilkington says.
It wasn’t until well after her diagnosis that Katrina’s genetic link was found. Several of her aunts on her father’s side had signs of lupus, although it wasn’t something the family ever really talked about or associated with having the disease.
As a consultant adolescent and pediatric rheumatologist working at Great Ormond Street Hospital in London, Dr. Pilkington is well aware of the emotional toll that lupus can exert on young people. This is why a psychologist is an essential part of the multidisciplinary team (MDT), she says.
“Family support by the MDT is also extremely important, and our clinical nurse specialists are very good at doing this, but there are many patients who do need a psychologist” to help get the best out of school and their development.
Doing less well in school or psychiatric symptoms may the first signals of there being something untoward happening in children, but “it’s the beginning of their lupus that is causing the problems,” Dr. Pilkington observes. So, when treating young people with lupus, it has to be done within the context of the family, of growing up with lupus, and with recognition of the effect on schooling.
Long experience of lupus
“The consultations in this group of patients may be slightly different from other lupus consultations,” Dr. Rahman notes. They may bring a parent with them because that’s how it has always been, he says, and they may have definite views about therapy, such as an unwillingness to use steroids based on their long experience of having lupus.
Importantly, “the whole multidisciplinary approach that they grew up with may not be available to them now, and that might be a challenge,” Dr. Rahman says. “It’s a big change sometimes, and they may be concerned that you have a very different approach from their previous clinician.”
Not only that, but the patient and carer may have very different agendas. This is a time when young adults are perhaps finding their voice and having to take on the responsibility for their own care as parents start to step back as being the main carers. Young people just want to live as normal a life as possible, and the continuity of treatment may waver as this transition occurs, but don’t underestimate us, says Katrina.
“You may think I don’t know anything, but I do; I’ve learned a lot,” she says. “People can underestimate me: They say ‘Oh you’re only 18; you don’t know much about anything,’ but I know a lot, and my opinion matters.”
While there is still a long journey ahead with lupus for Katrina, she’s in control. She is driven to try to make things different for herself and for other young people and has already participated in several studies, including the UK JSLE Study. Being part of this study has given her opportunities she had not thought of before, enabling her to become a patient advocate and help improve awareness and understanding of the disease.
“I want to advocate to raise awareness for young people, particularly those with long-term health conditions such as lupus and anxiety, conditions that I suffer from,” she says on her website Katty’s Territory.
“I want to show how I can conquer different struggles that come with being a young person and managing an illness. Achieving your goals and dreams is possible despite any problems you face; I want to show and empower other young people to follow their dreams.”
Sharing her knowledge and experiences, both positive and negative, of living with lupus via her website and blog and writing articles for the LUPUS UK magazine is helping her come to terms with having a lifelong illness. It’s also validation that her voice matters and hopefully, she says, will lead to better services and care, and one day, perhaps even a cure.
Dr. Pilkington disclosed educational and departmental support from AbbVie and departmental support from medac Pharma and Dr. Rahman holds a patent on a rheumatology drug. No other relevant conflicts of interest were disclosed.
Sara Freeman is a freelance reporter for MDedge News.