Sickle cell disease: Moving from child to adult services

Delegates at the Haematology Association of Ireland 2024 Annual Meeting received a highly engaging talk from Prof Mariane de Montalembert, Necker Hospital for Sick Children, Paris, France, on the complex nature of sickle cell disease (SCD), and how she has and continues to work towards optimal transition of paediatric patients to adult services. Prof de Montalembert is a leading international figure in SCD treatment and is globally recognised for her work in paediatric haematology. She said that clinicians in Ireland will meet more patients with SCD in coming years as there are “so many patients now in Europe”.

Prof de Montalembert began with an overview of the most debilitating complications and burdens of the disease such as stroke, pain, organ damage, and bacteraemia. “Thanks to screening, prednisolone use, hydroxyurea, and correct use of transfusion, minimum survival in high income countries is above 95 per cent nowadays, which is not the case in Sub-Saharan Africa,” she noted. Prof de Montalembert said that life expectancy in SCD is 50 years, and that the disease is generally classified as benign. She added that “dying at 50 is not very benign”.

I strongly advise having a transition nurse

Prof de Montalembert warned that adolescence is “a major risk factor” in SCD and explained that children also experience more symptoms as they get older. Those over 14 have multiple complications, with pain being particularly difficult to manage, attendees heard.

“They need closer follow-up, more frequent hospital visits, exactly at a time when they would like to get rid of the disease,” Prof de Montalembert continued, and detailed the problematic symptoms such as delayed puberty and jaundice that are “not easy to live with”. She also discussed the risk of infertility associated with hydroxyurea, highlighting misinformation on social media, and evidence that the side-effect is reversible on stopping the drug in most cases. She also advised that many patients have depression, post-traumatic stress disorder, and other psychological issues, adding that refraining from sporting and other activities is “a bad decision”.

The complex relationship between the clinician, child, and parent was also discussed.

“Each case is different,” she said, adding that the young patients “just want to be normal”, that they often exhibit a lack of trust, and that parents often try to restrict their behaviours.

Prof de Montalembert proceeded to present myriad data demonstrating a peak of complications and death during the post-transition period, and the importance of appropriate care during this phase.

“I explain the disease as soon as I see the children and mother…. At six they receive education sessions,” she said, outlining the “money and resources” required to support therapeutic education for parents and children. The conference also heard that Prof de Montalembert speaks to and reviews the adolescent without the presence of the parent. She described the positive effects this has on her relationship with them.  Prof de Montalembert strongly recommended early input from the adult multidisciplinary team, as well as visits to adult facilities.

“I strongly advise having a transition nurse,” she said, and described the role as “the most effective solution” to ease the transition. “The transition nurse is a familiar face, she provides education, she coordinates clinic visits and appointments, she can be contacted by phone,” Prof de Montalembert described a range of roles the transition nurse can perform.

The final aspect of the talk focused on the ‘Best Success’ summer camps Prof de Montalembert and her team established to create a social network for these children and adolescents, and to reinforce education about medications, motivation, and knowledge of SCD itself.