Mariane de Montalembert has been a Paediatrician at the Necker-Enfants Malades Hospital in Paris, France since1985. She has since gained certificates in infectiology and transfusion. In 1994 she became a Doctor of Ethics. She has been responsible for the centre for haemoglobinopathies in Necker-Enfants Malades Hospital since 1994. She has developed several researches in clinical paediatrics about sickle cell disease and thalassaemia, and is responsible for the French register of sickle cell disease children treated with hydroxyurea.
Dr Montalembert has authored or co-authored several book chapters, and many papers in peer-reviewed journals.
Abstract: Transition – a vulnerable time
Almost all children with sickle cell disease (SCD) now reach adolescence in Europe and the United States (1,2). However, most of them develop manifestations of previously silent complications such as pulmonary hypertension, renal insufficiency, or neurosensory impairments (3), which require closer follow-up, more frequent hospital visits, more treatments, and more self-management of the SCD to limit the morbidities and to avoid mortality, which nevertheless remains extremely high in young adults with SCD (4). Unfortunately, this crucial period during which disease control should be optimised occurs in the midst of the psychological changes characteristic of adolescence, when the patients may feel overwhelmed by the burden of daily care and may want to escape from what is perceived as unfair limits imposed by the family and doctors. Furthermore, at adolescence, patients transition from child-centred care to adult-oriented care. SCD adolescents may be particularly vulnerable, as they may experience pubertal delay and/or severe jaundice, which may lead to teasing by peers. Adolescents with SCD may experience academic difficulties, related in part to frequent absences from school and in part, in some patients, to cerebral vasculopathy of variable severity. Many of these adolescents have been forbidden by their doctors to engage in sports. The adolescent may be concerned about being infertile as a result of hydroxyurea therapy. Parental overprotection may cause additional suffering. All these factors in combination may contribute to cause depression, low self-esteem, and even post-traumatic stress disorder (5). Furthermore, major differences exist between most paediatric and adult wards, with most paediatric units offering games, school, other activities, and even holiday camps. Importantly, urgent treatment of pain and accelerated admission pathways are now available in most paediatric units receiving SCD children but are rarely provided by adult care systems.
The first study on the transition period in adolescents with SCD was performed in 1994 by Telfair et al. (6) and showed that adolescents and young adults were primarily concerned about how they would pay for medical care, how they would be treated in adult settings, and whether the new medical staff would believe them when they reported pain. Gradually, several multidisciplinary teams developed programs to improve transitioning in SCD patients. For example, Treadwell and co-workers in California developed a ‘passport to adult life’ underlining the main features of a successful transition: ‘be educated, be empowered, be prepared’. We have written our own checklist of the main items that the adolescent must know, such as the name of the disease; main pathophysiological characteristics; mode of transmission; baseline haemoglobin level; treatment; role of each drug; course of action in the event of pain, fever, or priapism; method for escalating analgesics; location of the blood group card; and how to get to the emergency department. The checklist must be reviewed every year starting at the age of 13 years to assess transition readiness. We have created games and brochures (www.rofsed.fr). Education is provided during regular visits but also mostly during peer group sessions, which are held outside the hospital with a doctor, a teaching nurse, and a psychologist, all belonging to a teaching structure funded by the French Agence Régionale pour la Santé (ROFSED).
Empowerment means creating a distance with the parents. Our experience is that the youth may feel more ready than the family for the transition, and that the parents may experience more anxiety about the transition than the youth. Involving the parents in the process in some way or another may contribute to decrease parental anxiety. Some centres hold a ‘transition open day’, when the young people and their parents can visit the adult facilities.
The transition must be discussed early, as soon as 13 years. We suggest seeing the child without the parents. Some centres propose that the young people meet the adult staff at 15 years of age. Alternate consultations in adult and paediatric units performed routinely between 15 and 17 years of age have been used, with subsequent transition to adult care (E Aimiuwu, clinical nurse specialist at the Whittington hospital, personal communication). A transition nurse and a psychologist may play key roles (7) and may continue to support the young adult in the first year following the transition to adult care. The NIH summarised the guidelines for transition in 2010 as follows: there should be a hospital transition policy in place, and preparation and planning should start at an early age (13-14 years) (grade C); a detailed review should be carried out at 15-16 yrs, to include knowledge and understanding about SCD, management, concerns about healthcare in an adult setting, and readiness to transfer (grade C); a transition or adolescent clinic should be available to allow the adolescent to meet the adult sickle cell team, and a formal review and handover should take place (grade C); and adult and paediatric protocols for managing complications, in particular pain episodes, should correspond as much as possible (grade C) (8).
Despite these publications and recommendations, real-life transitioning for SCD patients falls far short of the ideal. A survey of US paediatric providers published in 2011 showed that 97% of the 30 clinics having accepted to answer the questionnaire had an identified accepting adult provider but that only 60% routinely transferred their patients to an adult haematologist specialised in SCD; 50% of clinics had a written list of desirable self-management skills; 70% of clinics reported assessing patient readiness to transition; and transition was first discussed at a mean age of 15.7 yrs (range, 13-18), and occurred at a mean age of 19.6 yrs (18-25) (9).
References
1) Quinn CT, Rogers ZR, McCavit TL, Buchanan GR. Improved survival of children with sickle cell disease. Blood 2010;115:3347-52
2) Telfer P, Coenh P, Chakravorty S, et al. Clinical outcomes in children with sickle cell disease living in England: a neonatal cohort in East London. Haematologica 2007;92:905-12
3) van Beers EJ, Van Tuijn CF, Mac Gillavry MR, et al. Sickle cell disease-related organ damage occurs irrespectivelyof pain rate: implications for clinical practice. Haematologica. 2008;93:757-60.
4) Lanzkron S, Haywood C. Trends in mortality rate and age of death in sickle cell disease (SCD): 1979-2005. Blood. 2010;116:[abstract 736].
5) Hofman Hofmann M, de Montalembert M, Beauquier-Maccotta B, de Villartay P, Golse B. Posttraumatic stress disorder in children affected by sickle cell disease and their parents. Am J Hematol 2007; 82: 171-2.
6) Telfair J, Myers J, Drezner SJ. J Transfer as a component of the transition of adolescents with sickle cell disease to adult care: adolescent, adult, and parent perspective. Adolesc Health 1994; 15: 558-75.
7) Howard J, Woodhead T, Musumadi L, Martell A, Inusa B. Moving young people with sickle cell disease from paediatric to adult services. Br J Hosp Med 2010; 71: 310-4
8) NIH standards and Guidelines for clinical care ‘2nd edition October 2010)
9) Sobota A, Neufeld EJ, Sprinz P, Heeneey MM. Transition from pediatric to adult are for sickle cell disease: results from a survey of pediatric providers. Am J Hematol 2011;86:512-5