Tailored care for a unique disease: Managing sickle cell disease

When it comes to caring for patients with sickle cell disease – there is no one-size-fits-all approach. William Osler Health System’s (Osler) paediatric sickle cell clinic provides specialized care and education to patients living with the disease and their families, from newborn through to adulthood.

We spoke with Amanda Wagner, Nurse Practitioner, to learn how this unique disease is treated and managed for patients, close to home.

What is sickle cell disease?

Sickle cell disease is an inherited blood disorder that comes from a genetic mutation in both parents. The disease impacts individuals of many diverse backgrounds, presents particular challenges and impacts everyone differently.

Think of it as this: anywhere the blood flows in the body, problems can occur. Symptoms can include chronic pain, enlarged spleens, and a higher risk of infections, which can lead to other conditions.

How is it diagnosed?

Early diagnosis is so important, and we are lucky to have newborn screening in Ontario – this allows us to begin education and early prevention of complications related to the disease as soon as possible.

Because of the disease’s unpredictability, tailoring care to each patient’s needs and experiences is so important. This is where Osler’s clinic comes in!

Tell me about the paediatric sickle cell clinic.

The paediatric sickle cell clinic at Peel Memorial Centre for Integrated Health and Wellness is a satellite clinic and is run in affiliation with the Hospital for Sick Children (SickKids).

Because the community we serve has a very high prevalence of sickle cell disease, the clinic allows us to extend specialized care to patients within their own community, saving them long trips downtown and time away from work and school.

Our team also works with and educates other departments, including Osler’s Emergency Departments (ED) and inpatient units, to ensure care for sickle cell patients is seamless across our health care system.

How do you support patients through the program?

Because sickle cell disease is unpredictable, the care we provide is tailored to each patient. Based on their lived experience, we work to understand what complications or health challenges may come up in the future that may impact their care. We prescribe specific medications that help counteract the symptoms of sickle cell disease to improve their quality of life – this means less pain and ED visits, and more time for being present in class at school and for extracurricular activities.

We also provide something that is very important and that is education. When patients enter adulthood, they transition out of paediatric care. Many sickle cell patients may have had their care and symptoms managed by the adult(s) in their lives, so it’s important to ensure they understand the disease fully as they become independent. We work with patients to plan and help them feel confident about managing their symptoms as they get older.

What is the most fulfilling part of your role?

Seeing patients that I diagnosed through newborn screening who are now graduating high school and becoming young adults is incredibly rewarding. It’s also bittersweet, you get to know them so well throughout their lives and work through some really challenging times with their families, and suddenly they’re grown up.

However, knowing you had a part in helping them get to their next stage of life with the tools they need to succeed is very fulfilling.