Transition to an adult model of healthcare for FH

The transition from paediatric to adult healthcare involves changes in care teams and system design. As a result, adolescents and young adults are often overlooked, leading to a lack of follow-up and loss of compliance. Young people often consider healthcare of low importance compared with other aspects of their transition to adulthood, such as education, employment and relationships. Therefore, it is essential to begin educating them about FH and its management as early as possible, and by 10 years at the latest. Better education of young people with FH should improve long-term adherence to clinical management.

Although cardiac events are rare in children with FH, they can occur in those with HoFH and in young adults with severe HeFH. It is vital that young people recognise symptoms and know to seek urgent medical help, particularly as they transition to adult care and independent living.

Key proposals for transition from childhood to adulthood are summarized in Box 3:

Box 3 Proposals to facilitate a smooth transition to an adult model of healthcare for familial hypercholesterolaemia (FH)

1. Children with FH should be taught about their condition and its management as early as possible, and definitely by the age of 10 years.

2. The importance of diet, healthy lifestyle measures and avoidance of smoking/vaping should be explained and stressed before puberty.

3. Adherence to lipid-lowering therapy (LLT) should be monitored, especially during puberty.

4. Girls should be given advice on contraception.

5. If adolescents wish, they can be seen (partly) without their parents.

6. Before transfer to the adult service, adolescents should be made aware that from age 18:
(i) LLTs other than those used in childhood are approved and prescribed; and
(ii) low-density lipoprotein-cholesterol (LDL-C) treatment goals will be lowered to 1.8 mmol/L (70 mg/dL).

7. Upon transfer to the adult service, the hospital physician taking over care from the paediatrician should actively review and, if necessary, adjust the lipid-lowering regimen. This should be clearly emphasized during the handover of care.

8. If other major risk enhancers are present, one could strive from age 16 for a lower LDL-C goal to facilitate transition to adulthood when the treatment goal will be lowered to LDL-C <1.4 mmol/L (55 mg/dL) according to the 2025 update of the 2019 ESC/EAS Guidelines for the management of dyslipidaemias.

9. An adolescent with FH, and especially those with HoFH, should preferably attend a transition clinic or a joint clinic with the paediatrician and internist/cardiologist at least once before transfer to the adult service.