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Mediterr J Rheumatol 2016;27(3):94-7
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Transition of the patient with Childhood-onset SLE
Polyxeni Pratsidou-Gertsi
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Pediatric Immunology and Rheumatology Referral Center, First Department of Pediatrics, Aristotle University of Thessaloniki,  Hippokration General Hospital, Thessaloniki, Greece

Abstract
This review aims to depict the profile of the childhood onset-SLE (c-SLE) and the related challenges that need to be managed in order to achieve an uneventful programmed transition from the family-oriented Pediatric Rheumatology Outpatient Clinic to the adult patient-focused one. Transition is a dynamic, continuous and interactive process where a triad is involved: the medical staff, the pediatric patient and the family. Their mutual collaboration and interaction start at the announcement of the diagnosis and is continuously supported by the ongoing multi-dimensional patient’s and family’s education. A prerequisite of a successful transition is the stepwise consciousness of c-SLE per se as well as the development of essential skills for the management of medical, psychosocial and educational/professional consequences derived during the course of the disease. The ideal time point of the final phase of transition (moving to adults’ care) requires disease remission and the patient’s confirmed dexterity for life self-management and coping with his/her difficult and unpredictable chronic disease.
Keywords: Childhood-onset Systemic Lupus Erythematosus, transition, process, Juvenile Systemic Lupus Erythematosus, young adults with lupus
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1. The portrait of pediatric SLE

Pediatric systemic lupus erythematosus (SLE) or Childhood onset SLE (pSLE) - or as previously termed, juvenile SLE (juvenile SLE) - is SLE with an onset prior to the patient’s 18th birthday.1

The disease commencement and course have a greater load as compared to adult SLE. More specifically, pSLE has a more aggressive onset, and predominant initial involvement of systems and organs differ; with a greater likelihood of any organ involvement, and not only the kidneys or the CNS. Thus, a prompt diagnosis aiming to the early and aggressive disease interception is crucial, in order to prevent the damage development in a still growing organism.2-6

2. Challenges for the adolescent from the time of pSLE diagnosis until the transition to the adult Clinic

The disease impact on an individual and family base is summarized in Table 1.7 These consequences derive from both the disease per se and the effects of the therapeutic options. The adolescent is asked to give his/her consent, following  his/her information about treatment choices, suggested therapeutic changes and the impact of potential drug-induced adverse events; as for example, from administration of steroids,  immune-suppressants or even agents, still “off-label’ for pediatric patients. As pSLE is typically diagnosed in adolescence, the period for acceptance, self-education and self-management preparation in respect to the disease, is shorter than in Juvenile Idiopathic Arthritis.7 Thus, the stepwise transition process needs to be more intense.

Table 1: Impact of pediatric SLE on the patient and the family

 

3. Characteristics of the adolescents with pSLE that the health care providers involved in the transition need to know about

Patients with pSLE can remain under the care of a pediatric Rheumatologist until chronological adulthood, according to his/her will.10 However, during the follow-up period, he/she will be prepared for the gradual "weaning" from the "physician-family" umbrella, to become independent and develop trustful relations with the health professionals of both Clinics.

The psychosocial maturity, though, may not progress in parallel to chronological age in several patients with chronic diseases, as in pSLE.10-13 Additionally, unpredictable changes of the residence due to educational or working conditions may require an unscheduled switch of the adolescent with pSLE to another medical team or family physician. Lastly, at this critical period, when the pSLE patient is challenged to get acquainted with another physician of his/her confidence, several factors may interfere with this effort: the creation of sexual relationships, the restrictions regarding appropriate methods of contraception for pSLE patients, as well as possible pregnancies. All these factors make the young patient’s effort to balance between life and the requirements of his/her disease very difficult (Table 2).

Table 2: Issues for discussion and solution during the transition process

 

4. The process of transition from the pediatric to the adult Rheumatologist

Regardless of the transition model chosen by the relevant medical team, there are certain predefined targets, which, if achieved, predict a successful integration into the new adult-oriented medical setting.

Education on the self-management of pSLE

The gradual, planned multi-dimensional patient and family education on the disease characteristics and management is individualized: it progresses according to the pace imposed by the family. It aims to develop the patient’s skills in respect to coping with the medical, psychosocial, educational or professional consequences emerging during the disease course. In the meantime, the patient is getting training on the successful cooperation with the care providers’ team, including the pediatric rheumatologist, pediatric nephrologist and other physicians or health professionals, such as psychologists. This teamwork will lead the family and the patient to give their consent and co-decide on any medical decision.10-12 During the course of the disease, pSLE can affect not only the patient’s emotional state, but also higher cognitive functions, such as the synthesis of ideas, and the attitude towards the challenges of life in respect to professional and social rehabilitation. Therefore, the patient deserves to be supported by specialists experienced on the characteristics of pSLE.4, 7,9,15,16

Most adolescents seek additional health-related information online.17 For Greek patients, there are reliable web sources written in the Greek language that originate from well-known Rheumatology Organizations/Societies such as www.printo.it and www.tosomasoumilaei.gr. These trustworthy sites contribute to the development of ranking the reliability of websites containing disease related information.

Transfer to the adult Rheumatology Clinic

The optimal timing of the final transfer to an adult Rheumatology Clinic is when the patient is in clinical remission and has a proven ability to self-manage his/her life and cope with his/her difficult and unpredictable chronic diseases.14,18,19

The final steps towards adult Rheumatologists are escorted by a medical report delineating the patient’s disease phenotype that is addressed to the adult physician in case that they had not previously evaluated the patient in a joint Clinic (Table 3). 4,11,18,19

Finally, if the patient wishes so, his/her parents may be present at his/her entrance to the novel healthcare setting, in order to be both ensured for the well-scheduled ongoing of the disease monitoring.10,19

Table 3: Accompanying medical report of the pSLE patient

 

CONFLICT OF INTEREST

The author declares no conflict of interest.
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