Diapositiva 1 - USL 2 Umbria

Università degli Studi di Firenze
Reumatologia
Ambulatorio di transizione
TRANSITION CARE: IL LINK TRA REUMATOLOGIA
PEDIATRICA E REUMATOLOGIA DELL’ADULTO
Fernanda Falcini
… con la disponibilità di cure mediche
più efficaci
molti bambini con malattie croniche, a rischio
per la vita, fibrosi cistica, diabete, malattie
metaboliche etc. raggiungono l’età adulta
in condizioni soddisfacenti ma bisognosi di
cure assidue da parte del sistema sanitario.
… negli ultimi anni abbiamo assistito ad
un progressivo aumento dei bambini con
malattie reumatiche croniche.
… ad un aumento dei bambini reumatici che
raggiungono l’adolescenza e l’età adulta con malattia
attiva o esiti psico-fisici più o meno invalidanti.
J Pediatr 2006; 148; 824-7
Volume 12, Issue 2 May 1998
Paediatric Rheumatology
OUTCOME JIA FOLLOW UP AT LEAST 10 YEARS
Il bambino con AIG, LES, malattie reumatiche
esordite nei primi anni di vita come arriva
all’adolescenza ?
all’età di giovane adulto?
L’adolescenza
La malattia
L'adolescenza è una fase della crescita che segna il
passaggio dall'infanzia alla vita adulta.
L’adolescente deve conquistare la propria
indipendenza e costruire una sua identità
al di fuori della famiglia.
Deve distaccarsi psicologicamente dalla propria
famiglia per costruire una nuova immagine di
sé che rifletta un'identità adulta e matura.
ADOLESCENZA
Si instaura il desiderio di ribellione nei confronti
della famiglia e dell’autorità dei genitori.
Si hanno atteggiamenti intolleranti, di sfida e
una grande instabilità emotiva.
Infanzia
Adolescenza
Giovane adulto
Il passaggio dall’età infantile all’adolescenza e all’età
di giovane-adulto
per un individuo che ha una malattia reumatica
cronica, con esordio nei primi anni di vita, è molto
complesso e diverso nei vari paesi in relazione alle
diverse culture e ai differenti sistemi di assistenza
sanitaria.
TRANSFER
Transfer is described as an event that happens on one occasion when information
or people move from one place to another. In the context of moving from paediatric
to adult care, the moment of transfer can be considered to have happened when the
paediatrician discharges a young person from their care and sends a referral to an
adult physician or when the adult health-care team sees a patient for the first time. It
is very important that clinical responsibilities at this time are clearly defined by the
paediatric and adult teams involved, so that it is clear to the patients and their
families and to the health professionals who have the responsibility for the young
person's health care at any one time.
This is particularly important for situations when urgent advice is sought during this
period.
Robertson L. Best Practice &Research Clin Rheumatology 2006; 20:387-97
TRANSFER
Il momento in cui il pediatra trasferisce il ragazzo al
“servizio dei giovani- adulti”
oppure
quando lo Specialista degli adulti incontra
per la prima volta il ragazzo.
TRANSFER
Il trasferimento di solito avviene durante una
fase molto delicata nella vita dell’adolescente
indicata con il termine di
“Transizione”
TRANSITION
Transition is defined as a multifaceted active process that attends to the
medical, psychosocial, and educational/vocational needs of adolescents
as they move from child-orientated to adult-orientated life styles and
systems. The aims of transition are:
 To provide coordinated, uninterrupted health care that is ageappropriate, developmentally appropriate and comprehensive.
 Promotion of skills training in communication, decision-making,
assertiveness and self care are integral to transition, so that on moving
to adult care a young person has control and independence with
respect to their health care.
Robertson L. Best Practice &Research Clin Rheumatology 2006; 20:387-97
TRANSIZIONE
“… processo attivo che riguarda le necessità mediche,
psico-sociali ed educative degli adolescenti durante il
passaggio dalla fase pediatrica-adolescenziale alla fase
adulta …”
TRANSIZIONE
Riguarda il soggetto nella sua interezza e
prevede 4 tappe fondamentali:
 Consolidamento della propria identità
 Costituzione di relazioni al di fuori della famiglia
 Raggiungimento di una propria indipendenza
 Scoperta della propria vocazione
BACKGROUND
Many young people with childhood-onset diseases, including
rheumatic diseases, continue to require medical care into adult
life.
There are many differences between paediatric and adult health
care which can make this change a dramatic and difficult one for
young people and their families.
Transitional care services aim to equip young people with the
appropriate knowledge and skills to cope with this change.
Robertson L Best Practice & Research 2006.
Transition from paediatric to adult care.
Bridging the gaps or passing the buck?
Viner R. Arch Dis Child 1999; 81:271-5.
… the process of transition from pediatric to adult health
care providers is one part of a wider transition,
or developmental process, in which the child moves
from being
a dependent child
to an independent adult.
Rosen DS 2003;33:309-11.
BACKGROUND
Il passaggio da un sistema assistenziale
pediatrico, molto presente, ad uno
specialistico per adulti crea notevoli difficoltà
ai ragazzi che devono affrontare un nuovo
tipo di gestione della loro malattia e
alle loro famiglie.
DIFFERENZE FRA L’ASSISTENZA PEDIATRICA E DELL’ADULTO
Il passaggio dei giovani con malattie reumatiche
esordite in età pediatrica alle cure degli adulti è
impegnativo perché molti di loro continuano ad
avere malattia attiva o significative sequele in età
adulta.
Clinical Investigator, CFRI
Clinical Associate Professor, Division of
Rheumatology, Department of Pediatrics, University
of British Columbia
Tucker Lori B, Cabral DA
Rheum Dis N Am 2007; 661-72
Transition from child-centered to adult health-care
system for adolescents with chronic conditions.
Position paper of the Society for Adolescent Medicine
Blum RW et al. 1993;14:570-6.
Transition is an active medical process
… the purposeful , planned movement of adolescents and young
adults with chronic physical and medical conditions
from child-centered to adult-oriented health care system.
Transition to adult health care for adolescents and
young adults with chronic conditions.Position paper of
the Society for Adolescent Medicine
Rosen DS 2003;33:309-11.
The goals of an organized, coordinated transition to adult
health care for young people with chronic conditions are:
• To optimize health
• To facilitate each young person’s attaining
his or her maximum potential
AIG, LES, DMG, VASCULITI, M KAWASAKI,
BEHÇET, WEGENER, PAN,TAKAYASU …
… continuano in età adulta con tutte
le loro problematiche
Il momento della “transition” varia
da un paese all’altro,
in particolare su base culturale
TRANSIZIONE
 Età cronologica
 Maturità
 Stato di malattia
 Famiglia
 Compliance del paziente
 Indipendenza
TRANSIZIONE
Scegliere il momento giusto per trasferire
un giovane
ad un servizio per giovani-adulti è
la chiave
per la buona riuscita del passaggio.
TRANSIZIONE
Decidere solo in base all’età anagrafica può indurre a
commettere errori, ad effettuare il passaggio in un
momento nel quale il ragazzo non è ancora pronto o
è troppo tardi
perché da tempo non è più a suo agio
in un ambiente che lo fa sentire
troppo bambino.
Malattia
attiva/riattivata
Uveite
Complicanze
Ridotta
BMD
AIG
Transizione
ATM
complicanze
Problemi
psicologici
Rifiuto ed effetti
collaterali
della terapia
Contraccezione
gravidanza
Studio
Lavoro
Ridotta
crescita
Invalidità
Chirurgia
Juvenile chronic arthritis into adulthood:
a long‐term follow‐up study
Zak M. Pedersen FK
Rheumatology 2000; 39: 198-204
Objective. To evaluate a group of 65 adults with a history of or persistent
juvenile chronic arthritis (JCA), on average, 26.4 yr after disease onset.
Results. Active disease was present in 37% of the study participants, of which 80% had
either extended pauciarticular or polyarticular JCA. 11% of the study subjects were in
Steinbrocker functional classes III and IV and 22% had undergone JCA‐related major surgery. The
pain visual analogue scale, health assessment questionnaire, erythrocyte sedimentation rate and
C‐reactive protein (CRP) were significantly increased in those participants who had active JCA at
the time of the study. Disease duration proved to be the parameter most strongly
associated with an unfavourable disease outcome.
Conclusions. Although the study group was biased towards the more severe cases, the data
suggest that the long‐term functional outcome in JCA is, in more than one‐third,
associated with active disease persisting into adulthood, increasing residua and the need for
surgery.
JUVENILE IDIOPATHIC ARTHRITIS IN ADULTHOOD
AND ORTHOPAEDIC INTERVENTION.
Malviya A, Johnson-Lynn S, Avery P, Deehan P, Foster H.
Clin Rheumatol. 2009;28:1411-7
The study included 144 patients with median disease duration of 19 years. Survival analysis
showed that joint surgery was observed in the majority (75%) of patients with disease duration
over 40 years with a trend for less joint surgery in patients with oligoarticular JIA. In total, 41
patients (28.5%) had received joint surgery, and 17/41 (41%) have required multiple procedures.
Of those who have required joint surgery, 20/41 (48%) had started MTX in their adult years, with
only 5/41 (12%), starting MTX prior to first joint replacement and none within 5 years of disease
onset. Of the patients who have not had joint surgery to date, most (46/103, 45%) were receiving
MTX or another immunosuppressive agent; in the majority of cases, MTX was started within 2
years of disease onset. Many adults with JIA require joint replacement surgery and ongoing
immunosuppressive treatments, emphasising that JIA is not a benign disease. Many patients who
have had joint replacement surgery have had exposure to MTX albeit after many years after
disease onset; it remains to be seen whether patients who have received MTX therapy early in
their disease course will ultimately have less requirement for joint surgery.
ASSESSMENT OF BONE MINERAL DENSITY IN ADULTS WITH A HISTORY OF
JUVENILE CHRONIC ARTHRITIS. A CROSS-SECTIONAL LONG-TERM
FOLLOWUP STUDY
MAREK ZAK, CHRISTIAN HASSAGER, DANIEL J. LOVELL, SUSAN NIELSEN,
CAROL J. HENDERSON, and FREDDY K. PEDERSEN
In the JCA study group, we found not only reduced BMD, but also evidence of
increased bone turnover, a possible mechanism of accelerated bone loss
in our study population. These findings suggest that JCA patients may, later in life,
also be at risk of developing premature osteoporosis and associated fractures.
Evidence of increased bone turnover in the JCA group suggests that inhibition of
bone resorption (inhibition of osteoclast activity) may represent a rational
therapeutic approach to prevention of osteoporotic fractures in selected adult
subjects with either active JCA or a history of the disease.
Arthritis & Rheumatism 1999; 42:
790–798, April 1999
Children and the Risk of Fractures Caused by
Oral Corticosteroids.
Van Staa et al, JBMR 2003;18:913
2010; 37:1935–43
Patients with JIA have a low bone mass and, after a first increase due to therapy,
do not reach a healthy condition over time despite our current more effective
drugs. These patients have a high risk of osteoporosis in early adulthood.
Lupus 2014:23:57-68