Supporting patients and healthcare professionals to improve outcomes
People living with SCD need coordinated disease management, not just pain management
Expanding standard of care to include monitoring and management of chronic complications, in addition to acute symptoms and complications, may be an essential step in improving outcomes in patients with sickle cell disease (SCD)
It has been published that treatment of SCD is more than just pain management; a long-term plan and comprehensive approach may help improve outcomes and life expectancy. Since the clinical manifestations of SCD are unpredictable, it is essential to monitor and treat known complications to minimize progressive and cumulative damage.
The following table is adapted from “Understanding the Complications of Sickle Cell Disease” from the Centers for Disease Control and Prevention for the patient care team.1
Select Complications of SCD
- Conduct a comprehensive pain assessment
- Advocate for appropriate pain management
- Help patients understand potential triggers and avoidance strategies
- Ensure patients know how to take pain medication to manage acute pain
- Encourage fluid intake (unless contraindicated, as in the presence of heart failure or kidney disease), ambulation, and incentive spirometry
- Conduct a comprehensive respiratory assessment, noting even subtle changes in respiratory status
- Report any changes in respiratory status to the patient's care team
- Teach and encourage the use of incentive spirometry
- Monitor vital signs and record elevations in temperature
- Teach patients or their parents the importance of monitoring fever and receiving age-appropriate immunizations
- Answer patient questions with the understanding that priapism can be an uncomfortable and anxiety-inducing topic
- Be sensitive to the potential psychological effects the condition may have on patients
- Teach patients that priapism is a potential complication of SCD
- Emphasize the importance of reporting events to prevent adverse sequelae and of seeking medical attention for prolonged episodes within 4 hours of onset
- Remind patients who experience priapism to pay attention to precipitating factors
- Conduct neurologic assessments routinely in children and adults and maintain a high index of suspicion for silent cerebral infarction in patients who demonstrate neurologic deficits
- Report any acute changes in neurologic status to the patient's care team
- Teach parents about the importance of routine transcranial Doppler ultrasound screening in children with sickle cell anemia
- Remind parents that a child’s poor academic performance may signal neurocognitive deficits resulting from silent cerebral infractions
- Encourage parents to discuss poor academic performance with the healthcare team
- Assess parents’ understanding of the need to seek care for any emerging neurologic symptoms
- Conduct a thorough assessment of abdominal pain, closely monitor temperature, and anticipate the need for a sepsis evaluation
- Teach parents the importance of immunizations, how to measure their child’s spleen, and the importance of recognizing fever early and notifying the child’s healthcare team immediately
- Understand the SCD complications that can contribute to chronic pain
- Perform a comprehensive patient assessment and history
- Obtain a thorough medication history
- Teach patients which types of chronic pain are more or less amenable to different interventions and how to use both pain management medications and nonpharmacologic interventions effectively
- Conduct a thorough chronic pain assessment (type of pain and underlying mechanism) and maintain a high index of suspicion for avascular necrosis (AVN)
- Refer patients experiencing hip pain for orthopedic consultation
- Assess patients, especially those with frequent ED visits and hospitalizations, for the presence of psychosocial health complications to identify any who may benefit from social work, psychiatric, or case management referral
- Include mental health and psychosocial challenges in conversations with patients
- Monitor renal function and intravenous (IV) fluid administration, especially in patients with a history of renal failure
- Assess kidney disease risk factors throughout hospitalization and maintain a high index of suspicion for proteinuria or reduced urine output
- Monitor patients for any changes in respiratory status and report even minor changes, such as elevated respiratory rate or decreased oxygen saturation, to the patient's care team, as they could be early signs of acute chest syndrome
- Work with other members of the healthcare team to minimize disruptions to patients’ dialysis schedule
- For patients at high risk for kidney disease, discuss nonsteroidal anti-inflammatory drug (NSAID) administration with the patient's care team prior to administration, and monitor fluid intake and urinary output
ASH Guidelines
Improving SCD care starts with building trust and meaningful partnerships
Patients want somebody they can trust. We have to hold our hands out. We have to come honestly. We have to come patiently. It's why we need partnerships based on trust, so that we can get to a place where we can move forward together.”
Wally R. Smith, MD
It is important to identify and address challenges that include the whole person and their circumstances, not just their clinical symptoms
Start a conversation with your patients and open up a dialogue to help:
- Understand the patient’s perspective about their disease
- Understand the patient’s emotional mindset and social situation
- Identify available mental health or community resources
- Understand if they have experienced health-related racial bias and/or discrimination that can impact HCP-patient trust and communication
Help patients live beyond their SCD and discuss not only long-term treatment goals but also life goals
Help patients develop a long-term plan that includes personalized life goals. Patient long-term goals may be health related or something patients would like to achieve.2
You can provide structure to these plans by planning out short-term goals that build up to their long-term goal. Clearly define goals, set realistic expectations, and check in with patients to motivate them and measure progress.2
Take a closer look at SCD
start with a genetic defect See how the unpredictable
complications of SCD start
with a genetic defect
References
1. Centers for Disease Control and Prevention (CDC). Major complications of sickle cell disease and nursing implications. Accessed October 12, 2020. https://www.cdc.gov/ncbddd/sicklecell/nursing-implications.html 2. Centers for Disease Control and Prevention (CDC). Living well with sickle cell disease self-care toolkit. Accessed July 27, 2020. https://www.cdc.gov/ncbddd/sicklecell/documents/LivingWell-With-Sickle-Cell-Disease_Self-CareToolkit.pdf