The Code Switch: Music Therapy Implications for Individuals with Sickle-Cell Anemia
By Keivanna Thomas, MusicWorx Intern
Music is a language that everyone can understand; it is a breakthrough medium for those who are surrounded by walls of self-pity, helplessness and negative self-image. Because music plays such a role, music therapists are one of the most equipped groups of professionals to address the emotional, social, and societal challenges faced by individuals who are frequently isolated and misunderstood. One such group is people living with sickle-cell anemia. Sickle-cell anemia is the most common form of sickle-cell disease, a group of blood disorders that affect up to 100,000 individuals of African descent in the U.S. alone (Hassell, K.L., 2010). Other groups affected include Hispanic-Americans and individuals of Middle-Eastern, Asian and Mediterranean descent (American Society of Hematology).
Sickle-cell is a chronic, genetically-inherited illness that often culminates in acute periods of hospitalization. The disease is caused by the mutation of one hemoglobin-Beta gene: a single switch in the genetic code that carries vast implications (National Human Genome Research Institute). Given the ethnic populations most affected by the disease, there are many socioeconomic as well as psychosocial implications to consider in the therapeutic treatment of sickle-cell patients.
One of the primary purposes of red blood cells is to distribute oxygen throughout the body via hemoglobin. However, this function is largely disrupted when cell shape is atypical. Sickle-cell anemia is characterized by the abnormal shape/function of one’s red blood cells, which I will periodically refer to as RBCs. “Sickle” cells, as they are called, look more like crescent moons as opposed to the normal full-moon shape of RBCs. As a result of their shape, symptoms of chronic pain and fatigue occur. Often, the pain suddenly escalates when sickle RBCs collect around the openings of blood vessels, blocking the passage of blood throughout the body (Hanson-Abromeit, D., & Colwell, C., 2010). The healthy red blood cells are then unable to do their job, and oxygen is cut off to any tissues and/or organs that are on the receiving end of the blocked vessels.
These acute episodes of extreme pain are known as crises, and they occur on an average of 0.8 pain episodes per patient-year (Platt, O.S., et al., 1991). Sickle-cell crises often result in hospitalization, and are known to cause long-term bone deterioration, organ damage and, in extreme cases, organ failure.
Currently, no direct cure for sickle-cell anemia exists; the most recently-approved treatment option is bone marrow/stem cell transplantation. This option, however, is expensive and still not covered by most insurance companies. Furthermore, the sickle-cell patient has to have a matched sibling in order for the procedure to become an option. Even then, a bone marrow transplant comes with significant risks. Thus, the most common treatments for sickle-cell anemia include IV fluids, opioids for pain management, and blood transfusions.
The psychosocial implications for patients with sickle-cell are a direct result of both frequent hospitalizations and family dynamics. Acute periods of hospitalization in adolescents with sickle-cell anemia result in the disruption of normal life activities: school, work, family gatherings, time with friends, enjoyable hobbies, etc. These limitations can lead to delevation of mood, anxiety, and a lowering of . Patients are often left feeling as though they are “defective,” or as if there’s something wrong with them. Many times, this feeling is inadvertently exacerbated by parents and other family members who may “bubble wrap” their loved one by treating them differently from other members of the family. For example, parents of children with sickle-cell may discourage or even prohibit them from participating in certain life activities for fear of triggering a pain crisis. The “coddling,” as one individual with SCD described it, can continue well into adulthood. On the other hand, patients may not have family support, resulting in isolation and loneliness.
Many individuals with sickle-cell also exhibit self-blame, as each period of hospitalization takes a toll on family members. Many families of color in the U.S. are uninsured or underinsured, and are less likely to be approved for quality insurance when chronic diseases such as SCD run in the family. Parents commonly take time off of work to be with their children during hospitalizations. Thus, individuals with SCD sense the economic stress put on family members with each pain crisis, and may feel as though they are a constant burden to their caretakers. While statistics vary from country to country, some individuals living with sickle-cell struggle with depression and suicidal ideation (Cole, 2007), believing that life would be better for everyone without them.
Many sickle-cell patients develop a fear of being seen as drug-dependent, especially as they get older. Pain is an abstract construct that only the affected individual can express. However, because routine prescription of opioids for pain management can lead to drug dependence, individuals face the risk of not being believed when asked about their pain levels. Thus, the use of drugs such as morphine and oxycodone to provide pain relief can not only cause physical symptoms such as fatigue and loss of appetite; it increases the likelihood of isolation and self-blame in patients (K. Thomas, personal communication, May 6, 2021).
Music therapy is known to reduce pain perception in chronically ill patients (Bailey, 1986). Because music therapy is not a drug and doesn’t come with negative side effects, music therapists can and should be an integral part of inpatient care for individuals with sickle-cell.
In addition to addressing pain management during hospitalizations (Rodgers-Melnick et al., 2018), music therapy can help to address psychosocial symptoms by providing a patient with opportunities for autonomy, promoting positive self-talk/self-image, and providing room for self-discovery and expression. Music can open the door to healthy communication, and can thus be useful in helping parents, siblings and other members validate their loved one’s independence. Parents of children with SCD can learn how to create safe yet flexible boundaries with their child.
Medical research is currently underway for a cure to sickle-cell anemia. In the meantime, individuals with sickle-cell need to know that they are seen and that they are not alone. Music therapists are in a unique position to fill in the gaps in the healthcare system for this population. We are uniquely positioned to validate patient experiences, use empathetic speech, and provide a safe space for emotional expression. Before a patient with sickle-cell disease can get access to a licensed counselor as an outpatient, they will likely encounter a music therapist in an inpatient hospital setting. Music therapists could serve as the first line of defense in helping sickle-cell patients address their psychosocial needs. In order to best fill this gap, music therapists should educate themselves and others about the realities of the disease, as well as the everyday lives of those most affected by it.
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