APS BULLETIN
JULY/AUGUST 1999 - VOLUME 9, NUMBER 4
Myra Glajchen, DSW, Department Editor
 
Living with Sickle Cell Disease: From Suffering to Empowerment
Deborah G. Oster Pannell, Founder, Sickle Cell Advocates for Research and Empowerment, Inc. (S.C.A.R.E.)
 
When people think of sickle cell disease, they usually think of pain. Although the physiological effects of this genetic blood disorder stem from a defect in the hemoglobin amino acid chain, which affects the red blood cells' ability to circulate freely and transport oxygen efficiently throughout the body, the hallmark symptom of sickle cell disease is an excruciatingly painful episode known as a sickle cell crisis. As a result, people with sickle cell disease are often perceived as long-time sufferers, victims of uncontrollable pain crises that can strike without warning. They are also often regarded as people who have little or no control over their own behavior or their lives in general. All too often, unfortunately, people with sickle cell disease tend to internalize this perception.
 
An additional challenge is the fact that individuals with more severe forms of the disease can become physically dependent on the opioids prescribed to treat their chronic pain. This physical dependency is frequently confused with drug addiction, which is a psychological condition. The individual with a high need for narcotic pain medication must also function within the complex interaction between racial, ethnic, and class stereotyping that generally exists in our culture. The fact that the majority of people with sickle cell disease in this country are of African or Latino descent and many, particularly in urban centers, are members of the economic underclass represents additional burdens for these patients. Indeed, a 1994 study showed that both families and medical staff perceived that Whites receive better medical care than Blacks (Chestnut, 1994). Adults, in particular, complain of long waiting times for treatment at hospital facilities and of being undermedicated during severe pain crises. Moreover, patients with sickle cell disease may also have to withstand accusations of faking the pain or engaging in drug-seeking behavior. Such problems have undermined this population's faith and trust in the healthcare system.
 
Fact and fiction about sickle cell disease
The need to unravel the myths and truths about sickle cell pain and its clinical treatment is great. In 1994, my husband, Ivor Balin Pannell, who has sickle cell disease, and I founded Sickle Cell Advocates for Research and Empowerment, Inc. (SCARE) to address these concerns from the perspective of people with sickle cell disease and their families. To further our goal of empowering people with sickle cell disease, we have created our own terminology and moved into the area of advocacy for families affected by sickle cell disease so that we can retain greater control over our own lives. For example, Ivor is not a sickle cell victim, a sickle cell sufferer, or a "sickler" He is a sickle cell defier, a term we developed several years ago as part of our own lexicon and that is slowly making its way into the common medical vocabulary. Our focus is on language that more accurately reflects a proactive relationship with the disease. We attempt to live not in denial of or in combat against sickle cell disease but rather with a healthy defiance of the limitations imposed by it. For us, this means not only holding medical professionals accountable to higher standards with regard to the treatment of our community, but also taking responsibility for our own behaviors
 
A 1992 study showed that children with sickle cell disease who had a history of more frequent hospitalizations were often given less medication than other children with similar complaints, but had a less-extensive history of documented pain episodes (Armstrong, Pegelow, Gonzalez, & Martinez, 1992). In these situations, the very behaviors that children developed to help them cope with their pain and obtain medication from nurses seemed to work against them and instead aroused suspicion and antagonism from their care providers. It seems paradoxical that patients whose disease affects them most severely are often the ones subjected to less than optimal treatment.
 
Pain management issues
A severe sickle cell crisis is a traumatic event. Individuals seeking appropriate care for symptoms related to their disease should not be forced to defend their medical needs, particularly at a time when they are at their most vulnerable. McCaffery (1968) provides an excellent practical definition of pain, suggesting that pain is "whatever the experiencing person says it is, existing whenever he says it does" (p. 95). Even the exaggerated fear of inducing a dependent or addictive state in a patient is not sufficient reason for a physician to withhold necessary medication from a patient who is in the middle of a sickle cell crisis. Pain of such magnitude should be dealt with effectively, aggressively, and decisively. Later, the necessary steps can be taken to develop an individually tailored taper protocol to lessen the potential for withdrawal symptoms as the crisis begins to resolve.
 
It is essential that sickle cell disease be regarded as the high-maintenance illness that it is. Every sickle cell crisis is different, and each requires an approach that is attentive to the stated needs of the individual. Often, the patient and family are well informed about the sickle cell defier's specific disease pattern. Practitioners should consider making use of the patient and family's experience of the disease. Similarly, members of the sickle cell community need to become well versed about the disease and how it manifests in their particular case. To that end, we encourage adult patients and parents of children with sickle cell disease to keep a log on their condition and become familiar with their blood values and other indicators and, in general, to learn as much as possible about the disease and its treatment.
 
Healthcare providers should bear in mind that most patients who have symptoms of a sickle cell crisis are anxious about their pain. Many individuals with sickle cell disease were raised with the notion that they would not live past their 20s, and although such information is largely outdated, a sense of paralyzing fear and fatalism remains throughout much of the community. In addition, repeated episodes of false accusations and mistrust on the part of doctors and nurses have created an underlying sense of anger and frustration in patients with sickle cell disease. These accumulated feelings do little to foster a sense of trust, and it is often the healthcare providers who must take the first step toward building a healing partnership.
 
Positive partnerships
One such partnership has been developed at the Comprehensive Sickle Cell Center at Montefiore Medical Center in the Bronx, New York. Through its ongoing program of regular clinic visits as well as the ancillary services of a day hospital, this outpatient treatment facility offers a multilevel continuum of care to the sickle cell community. The day hospital, which provides short-term care during acute sickle cell crises, offers a welcome alternative to the emergency room, which is so often fraught with friction and tension. The advantages of the day hospital are many: easy access, suspension of stereotyping by the staff, belief of the patients' reports of pain, aggressive symptom management, empathy, and compassion. Over the last 10 years, clinical data have proven that the center's program of regular clinic visits supplemented by treatment in the day hospital has reduced the number of acute sickle cell pain crises in patients and prevented unnecessary in-patient hospitalizations. It is a model well worth considering.
 
When dealing with the most frustrating cases of sickle cell disease, patients, family members, and healthcare professionals must remember that being in pain tends to bring out the worst in people. Compassion and patience are essential when dealing with individuals who have spent a lifetime coping with unpredictable, repeated painful episodes. Conversely, it is up to those of us in the sickle cell community to strive for a sense of independence, accountability, and empowerment in our own lives. As we become more confident in our own abilities as empowered patients and advocates, we will be better equipped to collaborate with healthcare professionals in the interest of our own health care and that of our loved ones.
 
Deborah G. Oster Pannell founded Sickle Cell Advocates for Research and Empowerment, Inc. (SCARE) in 1994 with her husband, Ivor Balin Pannell. She is also an independent documentary filmmaker.
 
References
 
Armstrong, F.D., Pegelow, C.H., Gonzalez, J.C., & Martinez, A. (1992). Impact of children's sickle cell history on nurse and physician ratings of pain and medication decisions. Journal of Pediatric Psychology, 17(5), 651-664.
 
Chestnut, D. (1994). Perceptions of ethnic and cultural factors in the delivery of services in the treatment of sickle cell disease. Journal of Health and Social Policy, 5(3/4), 236.
 
McCaffery, M. (1968). Nursing practice theories related to cognition, bodily pain, and man-environment interactions. Los Angeles: University of California at Los Angeles Students' Store.
 
To read this article on the American Pain Society site click here:
http://www.ampainsoc.org/bulletin/jul99/advocacy.htm
 
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