Growing up with lupus carries huge emotional, physical toll

By Sara Freeman

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.

SLE affects schooling and social relationships

“The problem with schooling is that these patients often look quite well, and the schoolteachers really don’t understand that the fatigue is a major issue. So, they feel that their pupils are just ‘swinging the lead,’ ” says Dr. Pilkington.

Looking well has been one of the most frustrating things about lupus for Katrina. “That’s probably one of the hardest things: People look at me when I tell them, and they are like, ‘Are you sure?’ ” Katrina adds: “That was the hardest thing at school – people wouldn’t believe me. I could be dying inside and people would go ‘You’re fine; you look fine,’ ” she says.

But it’s not just fatigue that means young people can’t get their homework done, Dr. Pilkington notes, it’s also the time they miss from being at school and being with their peer group. This affects their ability to make friends of a similar age and participate in out-of-school activities.

Before her diagnosis, Katrina was quite academic and confident, says Lilian. Although she’s very determined and has been well educated through homeschooling, she maybe didn’t quite get the grades that she would have done had she been able to attend school normally and have that additional tuition and support.

“We have to fight to try to get them more help at school from the teachers, and to encourage better understanding within schools so that the children don’t lose out,” Dr. Pilkington suggests.

 

Coping with accumulating disease

Now that she is older, Katrina is very aware of how lupus has changed her life and feels its effects every day. She knows that the disease has already caused significant damage to her body.

“I have more choices to do things, but there’s so much damage from before that there are still things that I can’t do. Even if I am physically able, I am not mentally in a place to do certain things anymore,” she says.

Accumulating organ damage, even when well controlled, is a big problem in young people with SLE, as data from the UK Juvenile-Onset Systemic Lupus Erythematosus (JSLE) Study Group show.

“One of the things we’re enormously concerned about is organ damage, particularly in terms of lifelong effects that that may have,” Michael Beresford, MBChB, MRCP, MRCPCH, observed at the BSR annual conference. Evidence shows that patients with JSLE have much higher disease activity than adults, and can already have significant organ damage by the time they are diagnosed.

What is most concerning, Dr. Beresford says, is that more than one organ system may be affected – almost one in five (17.8%) young people with SLE have one or more organ systems involved at presentation, increasing to just over one in three (37.3%) by the time they reach adult care, which is about 11 years in the UK JSLE cohort.

Then there are the accumulating effects of the necessary medications – notably steroids and immunosuppressants – which can cause distressing side effects, an impact that can be more keenly felt during childhood and adolescence, and into young adulthood.

“We’ve come a long way in improving the care of children with lupus,” says Dr. Beresford, a pediatric rheumatologist working at Alder Hey Children’s Hospital in Liverpool, England. However, “we need to do much more, and we need to work collaboratively with those working in the adolescent transition phase, and particularly with those who are looking after children who’ve started with lupus and are now being looked after in the adult sphere.”

Dr. Beresford and Dr. Pilkington have both been involved in the SHARE (Single Hub and Access Point for Pediatric Rheumatology in Europe) initiative, a European program that was launched 5 years ago to try to optimize care for children and young adults with pediatric rheumatic diseases throughout Europe, including childhood-onset SLE. This includes helping develop guidelines for the diagnosis and management of childhood SLE (Ann Rheum Dis. 2017;76:1788-1796) as well as ensuring that young people have the help they need as they move from pediatric into adult care.’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.

Supporting the transition into adult care

“By the time children reach adult services, over 50% of them will have had lupus for over 5 years, sometimes even up to 10 years, so that’s significant when you think of the morbidity associated with that,” says Dr. Beresford.

Adult rheumatologist Anisur Rahman, MBChB, PhD goes further: “By the time patients attend my clinic, they’re very experienced patients with lupus and quite different to the adult patients who have come via their GP and referred as a new case of lupus.”

Dr. Rahman, an adult rheumatologist at University College London Hospital, adds: “These patients have a lot of lived experience of lupus, and they know a lot more about their lupus than I do; I would be a fool not to take advantage of what they know about their own lupus.”

While some hospitals in England have a dedicated unit to help with the transition from pediatric to adult care, many do not, Dr. Rahman commented at the BSR annual conference, and adult services are very different, which is something Katrina and Lilian know all about.

When she was diagnosed, Katrina was initially seen in an adult rheumatology clinic, until she was finally referred to a specialist pediatric rheumatology unit.

“Adult clinics have a very different atmosphere,” says her mother. Lilian adds that not only are there are different kinds of treatments and approaches, but also they are just not geared up to deal with the emotional support needs of young people.

“That was very difficult and at a time when neither of us understood very much about lupus, and I think initially, in that first year after diagnosis, she was quite depressed,” says Lilian.

Young people just want to live as normal a life as possible, and the continuity of treatment may waver as this transition occurs

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.

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