Enhancing self-care in adolescents and young adults with sickle cell disease

The complexity of sickle-cell disease (SCD), one of the most common severe monogenic disorders in the world that affect red blood cells, necessitates a carefully integrated care regimen.

Unlike normal red cells that pass smoothly through the blood vessels, sickle cells are stiff and sticky and tend to form clumps that get stuck in the blood vessels and obstruct blood flow. The result is episodes of extreme pain crises, as well as chronic damage to vital organs as the lungs, kidneys, spleen, and brain due to oxygen deficiency.
When red blood cells sickle, they also break down prematurely, which can lead to anemia. Anemia will cause shortness of breath, fatigue, and delayed growth and development in children.

As recently as three decades ago, children with SCD usually did not survive into adulthood. Today, because of advances in screening and treatment, more than ninety percent of individuals with SCD reach adulthood, and life expectancy is good.
With increased life span, individuals with SCD often encounter transitions of care. This includes changes from pediatric to adult care in the setting of home- and hospital-based care.
These care transitions have been shown to be particularly challenging for patients with SCD and are associated with increases in morbidity and mortality.

In sickle-cell disease (SCD) care transition from pediatric to adult care is associated with increases in morbidity and mortality. Successful transfer from pediatric to adult care has its foundation in a strong collaboration among the adolescent and young adult, his family and friends, and the health care system to support building skills and positive disease management.

In Antwerp, we did a small survey amongst our adolescents during transition to adult care. This survey showed that there is a strong need for more accurate information on their disease and its life-long psychosocial impact not only for the patient himself but also for friends, school directory, etc. Additionally, all patients felt the need for a medical card they can carry with them with crucial information to help them in expressing their medical, psychological and social needs.

Our intent is to develop new educational material for patients with accurate and illustrated information on SCD to enhance self-care and foster medical independence: introducing a new booklet with accurate and illustrated information and an USB-Card patients can carry with them.

First step is a booklet with important basic information about SCD and transition from pediatric to adult care based on recently published guidelines. The six goals are:
1.What is sickle cell disease; 2. Learn about general measures; 3. Learn about preventive measures for SCD complications; 4.Learn about general pain plan; 5. Emergencies: what to do and when to visit the emergency department; 6.Learn about reproductive medicine: anticonception, partner testing etc. and where to find additional useful information.

To make sure the information is clear we collaborate with a few of our dedicated young patients (Dutch, French and English speaking), to move forward with this Graphic Medicine project.

Second, we use all the information in the new booklet to create a USB-card which patients can carry with them. We write medical history, chronic medication and contact information on the card so it improves accurate communication with healthcare professionals during family visits abroad and international student exchange projects. We plan to expand it with a personal pain plan, advice on sports, and information for specific situations at school and in their professional life.

These two major goals lead to a second phase where we will look together with patients into new media options like Apps and Interaction through Social Media.