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Table 4 Examples of strong SCD NHLBI recommendation and evidence for individuals with SCD according to the NHLBI guidelines and recent randomized controlled trials. Boxes denote action items an individual can take based on the NHLBI guidelines.

From: Adapting medical guidelines to be patient-centered using a patient-driven process for individuals with sickle cell disease and their caregivers

Provider Guidelines First version of Patient-Centered Guidelines Patient-Centered Guidelines after iterative process described in Fig. 1
Use an individualized prescribing and monitoring protocol (written by the patient’s SCD clinician) or an SCD-specific protocol whenever possible to promote rapid, effective, and safe analgesic management and resolution of the vaso-occlusive crisis in children and adults (Pain action plan) Sometimes blocked blood vessels can cause a sickle crisis, which involves severe pain. In order to help you quickly and safely during these crises, talk to your SCD doctor about creating a set of rules specific to your needs. Include rules about getting medication, and how often your doctor will check in with you. What can be done at home to manage the pain?
• A pain action plan describes how to manage sickle cell pain at home. Action plans should be used as soon as the pain starts. An example of a pain action plan can be found in Forms.
□ Ask your health care provider about creating a written pain action plan that works for you.
• Call your health care provider if the pain does not get better, or gets worse even though you are using your pain action plan.
Treat avascular necrosis with analgesics and consult physical therapy and orthopedic departments for assessment and follow-up If you experience discomfort caused by your bones not getting enough blood supply, sometimes called avascular necrosis, talk to your doctor about taking pain medications. Your doctor may recommend you see physical therapy and orthopedic doctors. What is the treatment for avascular necrosis?
• Treatment options depend on how much the joint is affected.
□ Talk with your health care provider about sending you for physical therapy to make the muscles around the joint stronger and more flexible.
□ Talk with your health care provider about ways other than medication to manage your pain:
• Using heat, such as a warm compress, warm bath, or a heating pad
• Gently massaging the area that hurts
• Doing something to distract you from the pain like listening to music, drawing, watching TV, or writing in a journal
• Doing deep breathing and relaxation exercises
□ If approaches without medications do not help, talk with your health care provider about medications to control the pain.
□ A special health care provider (orthopedic surgeon) may see you for additional evaluation and treatment.
◦ Sometimes surgery is needed if other treatments do not work.
In infants 9 months of age or older, in children, in adolescents, and in adults with SCA, offer treatment with hydroxyurea regardless of clinical severity to reduce complications (e.g., pain, dactylitis, ACS, anemia) related to SCD If your child with SCD is between 9 months old and 18 years old, check with his or her doctor about using the drug hydroxyurea to try to lessen complications of SCD. Examples of complications include pain, finger/toe swelling and redness, and low red blood cell count. What is Hydroxyurea?
• Hydroxyurea is a medication that increases the amount of fetal hemoglobin in red blood cells. Fetal hemoglobin helps to keep the red blood cells from sickling.
• Hydroxyurea is not a cure for sickle cell disease, but it may help decrease many of the complications of the disease, including:
◦ Anemia
◦ Pain episodes
◦ Episodes of acute chest syndrome
◦ The need for blood transfusions
◦ Long hospital stays
• In adults, some studies have found that hydroxyurea helps you live longer.
Who should take hydroxyurea?
• Talk with your health care provider about hydroxyurea if:
□ You have sickle cell anemia (type SS or sickle beta thalassemia zero)
□ You have sickle cell disease (type SC or sickle beta thalassemia plus) and have pain or other sickle cell complications that affect your ability to do your daily activities or that affects your quality of life.
No definition in guidelines Not included in the first version What is pulmonary hypertension?
• Pulmonary hypertension is more common in people with sickle cell disease than in people without sickle cell disease.
• Your heart is made up of two pump systems. The right side of your heart pumps blood to your lungs to pick up oxygen. The left side of your heart pumps blood to the rest of your body.
• Blood pressure measured with a cuff on your arm is measuring the pressure it takes to pump blood to your body. High blood pressure is called hypertension.
• The pressure it takes to pump blood to your lungs can also be measured. High blood pressure here is called pulmonary hypertension.