Pharmacist's Role in Supporting Patients with Sickle Cell Disease

"Pharmacists are essential in bridging the gap in transitions of care within the sickle cell community. Does your sickle cell center utilize a pharmacist expert?”

Sickle cell anemia is a genetic disease characterized by the deformation of the red blood cells into a sickle shape compared to the normal oval shape of the red blood cells. It is an autosomal recessive genetic disease; passed from both parents to offspring if parents carry the mutated gene.?

Living with sickle cell disease can be very challenging because it is a chronic disease that affected all organ systems starting with abnormality in red blood cell shape to spanning complications such as pain episodes (Vaso-occlusive crisis) and progressive organ damage spanning from joints such as hip and knees to organs such as liver and kidneys.

Sickle cell anemia is?a disease characterized by acute-on-chronic pain, and an immunocompromised or weakened immune system. Patients with the disease usually are more tired than typical, are more easily susceptible to common infections than others, and most live life interrupted and? punctuated by numerous hospital visits to help manage complications from the disease

Patients with sickle cell anemia also periodically experience a complication known as acute chest syndrome, which mostly presents itself as bilateral lobe pneumonia and can easily be misdiagnosed if providers are not experienced in caring for patients with sickle cell disease. Acute chest syndrome is so serious that if not diagnosed and treated properly and promptly can lead to death within a few days. Acute chest syndrome is a medical emergency in the Sickle cell population.

Sickle cell disease is one of the many orphan diseases with a complicated presentation since it affects every organ system in the body. Patients are subjected to oxygenation interruptions too many times during their life spans, hence if not properly controlled it affects growth spurts, energy levels due to extremely low levels of hemoglobin, and progressive consequences such as acute kidney disease secondary to transfusions and even medication use through life.?

Pharmacists practice at the forefront of our healthcare system. We are most likely to encounter many patients before they make it to the hospitals. It is very important to be prepared to help your SCD patients by empowering yourself with knowledge of the disease. Understanding pain manifestation and treatment options are very important in support of SCD patients, especially with the opioid crisis plaguing our nation.?

Pharmacists' skills are needed more than ever to bridge the gap between pain management and holistic chronic disease management, starting with one sickle cell patient at a time. Let's explore how you can make a difference in the life of a sickle cell disease patient starting today;

When you encounter a patient in pain, ask the patient the stage of their pain treatment; are they newly discharged from the hospital and still experiencing high to moderate pain? Is their pain medication a maintenance dose to help prevent future episodes of pain? Do the patients understand the difference between as-needed pain (PRN) pain medication and scheduled doses? Are they aware of other non-medication methods to relieve their pain? Do they employ additional pain management methods? What is their baseline of pain functionality? What barriers do they have in affording and taking their pain medications?? Are they experiencing any allergies that need to be addressed? What is their current lifestyle (worker, student, disabled, or transitioning from pediatric to adult care)? The list is endless and I shall examine the pharmacist opportunities below?

Thinking of pain as the fifth vital sign (American Pain Society, 1999), it is very subjective, and treatment continues to fall short, especially in the SCD community. A serious barrier to appropriate treatment results from the opioid epidemic. As pharmacists, we are called to duty to put others' interests before ours hence it’s important to utilize empathy and treat every patient with respect despite your own pain experiences and perceptions. Research indicates that SCD patients have a very high threshold of pain, most patients go about their lives normally while experiencing low to moderate pain, and for some patients daily pain is a normal state of living, for this reason, it is important to listen effectively and believe the patient when assessing their pain levels.

Ask patients the right questions. What is your pain level? Do you understand which pain medication to take for breakthrough pain and which to take for chronic pain??

Educate the patient on how the medications work, the possibility of addiction and or pseudo-addiction with opioids, the possibility of adding Non-steroidal anti-inflammatory drugs ( NSAIDs) to their regimen if need be, and how to gradually transition from acute management to periodic chronic control at lower doses.?

Talk to your patient about the pain ladder to help understand their disease manifestation and be a resource even after your initial encounter. Follow up, check in on them and support them back to their regular stable health. Be honest with patients about the progressive nature of their SCD and encourage them to be self-advocates through educating themselves.

Pharmacists can also help patients prevent hospitalization by handling the pain early, and by educating the patient on the possible use of over-the-counter pain medication options while adding non-pharmacological options such as hot baths, mild exercise, heating pads, heating blankets, and other temperature regulation mechanisms. Controlled ventilation to minimize anxiety secondary to pain episode anticipation. These simple interventions if used timely and appropriately can sometimes help with mild to moderate episodes of pain crisis without hospitalization. Educate patients about oxygen demand and supply and the importance of minimizing their oxygen needs by balancing stressful situations,? reducing anxiety sources, and maintaining proper sleep hygiene.

Ask patients about the barriers they face in getting their treatment. Educate them about pharmacy shopping, the importance of managing medications in the same place, and establishing specialty care with their hematologist and primary care ahead of time in case of a move for college or job changes or just relocating in general. Treatment with established providers provides the benefit of a better understanding of their disease state and expert guidance from their drug experts. Is their medication needing prior authorization? Pharmacy teams should handle it with urgency since every missed dose can mean the patient needs to be readmitted to the hospital for pain and possible organ damage.

Talk to your sickle cell disease patient about their mental health. Look for key signs of depression and anxiety. Mental health plays a very important role in the prognosis of patients with sickle cell disease. Most patients can be depressed and anxious without even realizing they are, it is our place to identify and discuss our observations with both the patients and their primary care teams. SCD patients can thrive with a holistic approach to care and the pharmacist is the center point of maintaining this success. We are equipped to educate patients on lifestyle modifications such as their dietary needs, exercise benefits, and proper medication use. All these areas are needed for patients to live a complete lifestyle while gracefully handling their disease emergencies and progressive complications.

In summary, pharmacists, especially transitions of care pharmacists, community pharmacist, and pain management pharmacist needs to make it a duty to educate themselves completely about sickle cell disease and advocate for patients with the healthcare team. It starts with empathy, followed by passion, and guided by our duty. When we are prepared and ready for lifelong learning through service and care,? we keep our promise to “ consider the welfare of humanity and relief of suffering our primary concern”?

Ojong Bate? PharmD. MBA.