INTRODUCTION — This topic was written by an individual patient diagnosed with sickle cell disease (SCD). It is intended to offer clinicians insight into the experience of a single individual from that individual's point of view. This description of a particular patient's experience is not intended to be comprehensive or to provide recommendations regarding diagnosis, treatment, and/or medication information. It is not intended to be medical advice or to be a substitute for the medical advice, diagnosis, or treatment of a health care provider based on the health care provider's examination and assessment of a patient's specific and unique circumstances.
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CHILDHOOD AND ADOLESCENCE
Diagnosis — I have SCD, but it took several years to reach the correct diagnosis. There have been many changes in the care of people with SCD over the years, and I feel like I have experienced many of them.
I was initially diagnosed with sickle cell trait during my newborn screening, which was federally mandated in the 1970s for African American babies born in the United States (and is now routinely done in every state).
Sickle cell trait is an asymptomatic carrier condition, but around age 8 to 10 years, I began to have pain in my arms and legs. That triggered my parents and medical team to realize that I had something more significant. My father had sickle cell trait, so they knew SCD was a possibility and quickly referred me to a hematologist for more testing.
Over the decades that followed, my type of SCD was categorized as sickle cell anemia and subsequently as sickle cell beta+ thalassemia. The beta thalassemia mutation (which must have come from my mom) means that my level of normal adult hemoglobin (hemoglobin A) is low, and my blood cells have a high level of sickle hemoglobin.
Learning about the disease — Growing up, I was blessed to have a mother who was a nurse. She remained abreast of treatments and remedies related to my disease, and she taught me to be an advocate for myself and my treatment. My parents also instilled a Christian faith in me that helped me learn my purpose and develop a spiritual confidence in dealing with the many uncertainties life throws my way, including SCD. This faith is woven into all of my life experiences.
Many of my parents' inner-circle friends were members of the medical community, as well as a close aunt who was a pharmacist. My aunt explained the importance of taking folic acid at an early age. She remains a confidant and one of my health consultants.
Medical care — Interactions with my primary care physician and hematologist were positive experiences. I felt comfortable with this medical team and learned how to articulate my pain episodes, to distinguish chronic pain from temporary pain like migraines or a twisted ankle, and to communicate which type of pain I was having. The sharpness of a broken wrist or broken ankle is distinctly different from the dull achy pain of SCD.
During my elementary and middle school years, I was regularly admitted to the hospital in the fall; the temperature changes precipitated severe pain crises. I vividly remember being in the hospital every Thanksgiving for about five years straight. I fell in love with the Macy's Day parade that I watched on the hospital television, and my dad and I watched NFL football, which I also grew to love.
During my teenage years, my medical team and I communicated frequently. We had extensive conversations to help me understand the sickling process of my blood cells, how the stickiness of the cells made it difficult for them to move through my blood vessels, and how the cells got "stuck" in certain areas of my body like the joints.
Painful needles — I specifically remember the metal needles back in the 1970s being borderline torturous, and I hated when an inexperienced hematologist technician would have to try multiple times to successfully get my drip going for my fluids and pain medicines during a pain crisis.
Needless to say, I am ecstatic on the improvements in needle manufacturing. Unfortunately, even as an adult, I still occasionally encounter an inexperienced hematologist technician.
Sports — This medical team was often consulted on my desire to engage in physical activities. I remember consulting with them about wanting to play high school basketball and the intense conversations that decision entailed. Concerns were raised about exertion or an injury causing a pain episode. The medical team suggested that this could be detrimental to my health, and my parents said no to basketball.
I eventually joined the dance team, which was low impact. This provided much needed exercise and social activity, improved my health, and lessened my pain episodes.
College — I went to college equipped with a care treatment plan for pain episodes, which I shared with the on-campus clinic and nurse, the resident assistants in my dormitory, and my roommates. My care treatment plan included instructions from my hematologist and primary care physician that detailed:
●Ways to prevent pain episodes.
●Ways to immediately address pain episodes – Start pain medications, rest, inform resident assistants and professors as needed.
●Instructions and pain management plan to be provided if a visit to a hospital emergency department was required.
It was always imperative for me to be able to provide these details because SCD is a rare disease, and many in the medical community are not familiar with the pain management process for sickle cell patients. I was taught early on what to do during a pain episode, when to seek medical help, and the triggers that could cause my pain episodes.
I visited the school clinic a few times during my undergraduate years, and the nurse practitioners were receptive to providing me the assistance needed.
A change for the worse — Care at area hospital emergency departments was drastically different from what I had experienced as a child/adolescent. They often considered me a drug-seeking addict. I was always baffled as to why. I attributed it to many in the medical community not being familiar with what to do with a SCD patient, since many rarely encountered one. I also wonder if they may have thought I could not know more about treating my disease than them, since they were the medical professionals. However, my lived experience had made me an expert on what works best for my body.
Often, I would avoid the hospital during a pain episode. During a pain episode, it is often difficult to breathe, let alone to talk in depth and explain why I needed acetaminophen with codeine, or to have an extended conversation about how I even know what to ask for to help with my situation. This experience of having to educate the emergency department personnel about my condition during a severe pain episode occurred too many times to count.
One thing I hadn't mentioned yet was that most individuals with SCD typically have small or thin body frames, which sometimes led emergency department personnel in the 1990s to assume that sickle cell patients were drug addicts. I fit that body structure description, and during a pain episode I appeared frail. The lack of medical record sharing during this period meant that the emergency department personnel had no other information about me than what they saw in front of them. I felt at a loss to communicate the severity of the pain.
I specifically remember an encounter in the early 2000s when I consumed more alcoholic beverages than my body could handle while attending one of my college homecoming weekends. A pain episode resulted, and I was taken to an area hospital emergency department where my friends, all well versed in my disease, explained my condition and situation to the intake nurse practitioner. There was no immediacy shown to me for my condition by any of the emergency department support staff, including the attending physician. It took over three hours for me to be seen and provided any pain relief. The limited support ended after about two hours of intravenous fluids and pain medications, and I was discharged. The treatment was not adequate to relieve or even ease the pain, and I ended up at another hospital medical emergency department within hours of being discharged from the first one. The experience was similar, but I refused to let them discharge me until I felt better, so I resorted to lying when they asked me my pain level, because I was (and still am) an expert in my body.
This experience was the final straw that told me I could not sit silently in the face of inadequate treatment for severe pain. I consulted my father, a lawyer, and he advised me to send a letter to the executive leadership and operating management team of the facility. I sent a scathing letter, copying everyone at the facility, and I received a letter of apology from the executive leadership. This experience was life changing for me; it showed me that if I didn't speak up, no one else would do so on my behalf, but when I did, I would be acknowledged. I retain a copy of that letter to this day due to the impact it had on my life.
Learning more about my body — Much of what I experienced during my college years taught me tremendous lessons on pain management and triggers, many of which I effectively incorporated in my daily life. They included:
●Weather awareness – Cold temperatures can precipitate pain episodes.
●Altitude changes – Flying was fine, but I couldn't travel to areas with high altitudes and low oxygen like Denver, Colorado.
●Plan ahead for potential stressful events – Final exams were a good example.
●Modified exercise regimen with revised breathing technique/pattern.
●Alcohol intake must be limited – Alcohol can be dehydrating and can precipitate pain episodes.
Additionally, I pursued a graduate degree, which provided an opportunity for me to learn how stress can affect my pain episodes. I also took up running as an exercise/stress reliever and quickly learned that my blood cells don't carry as much oxygen as normal blood cells. I learned that lack of oxygen and overexertion can sometimes cause pain episodes and require an emergency department visit.
FAMILY, CAREER, ADVOCACY
Having children — Fast-forward to marriage and childbearing. By this point I had adopted the process of interviewing physicians, and I did so with obstetricians (OBs) as well. Following the self-advocacy skills I'd been taught and enhanced over the years, I spoke with my OB about my SCD.
The person I found was not highly familiar with SCD, but we had several conversations of what my pain episodes were like and how they should be treated. She was willing to learn and listen; she was receptive to hearing what worked for me. She consulted with my hematologist, and together we developed a pregnancy plan to manage my pain episodes when they occurred.
Over the years, I was blessed to give birth to not one, but two, miracle babies, whom I call Heartbeat#1 and Heartbeat#2. Giving birth was something I never thought I could do, but I continued to learn my body and my capabilities that God had given me. Together, my OB and I learned how to manage the triggers for pain. We also recognized how SCD expanded my pain threshold to the point where birthing pains were mild to moderate, and my deliveries were complication free. That partnership was a pleasant surprise, and she navigated me through both pregnancies.
My OB was open, honest, and frank. She said that she would not jeopardize me or my baby while I was in her care; however, she was direct in saying that I was her primary focus and that she would make the best decision for me and my well-being based on my health history. She explained to me that when I had an episode that I should come to her hospital's emergency department, where there would be a care plan in place for my treatment and she would be notified.
The plan worked flawlessly during both of my pregnancies. As expected, I had two to three pain episodes during each pregnancy. I called her office to let her know I was headed to the emergency department, and they were prepared for me when I arrived. I don't think I completed the paperwork before I was moved to a room, had an IV in my arm, and a monitor placed on my fetus. The treatment was excellent.
More self-advocacy — I have just lived through my 40s. As an adult and living in different states, my medical treatment continued to vary, but it was always better than what I experienced in the 1990s and 2000s. No one experience stands out, as I learned to manage my pain episodes as best as possible from home. Also, knowledgeable physicians became more comfortable with prescribing stronger medications that I could take at home and rest until the episode ended. The process of interviewing and sometimes educating potential members of my medical team continues.
As one example, my joints have been damaged from the numerous pain crises, resulting in avascular necrosis (AVN). It is a common occurrence in SCD patients and often requires surgical treatment. I had to have one shoulder surgery in 2014 and am awaiting other joint-repair recommendations.
I have embraced my life experiences and continue to manage my triggers, reduce stress, stay weather aware, eat with awareness, exercise regularly, take preventive medications, maintain medical appointments, and have regular contact with my SCD clinician team.
As an adult, I have been blessed to establish a career while managing my SCD. However, I am well aware that some people living with SCD are unable to maintain regular employment due to chronic pain, frequent hospitalization, and other SCD complications.
Involvement in the SCD community — In hopes of providing my perspective and my life being an example, my labor of love is to assist others with managing and living with SCD.
When I talk to people (other people with SCD, pharmaceutical meetings, medical students, and physicians), I generally present my story as one of blessings and miracles. It is important to me to focus on the positive aspects of my experience. None of my siblings has SCD. I feel God chose me for this role because I could bear it. I ask people to see each patient as a new person whom they have not met before, and to try to help them as much as possible.
I was introduced to the Sickle Cell Foundation of Tennessee (SCFT) in 2012 by the late founder and my friend, Dr. Trevor K Thompson of Memphis, Tennessee. The Foundation's mission aligned with my strategic goal of helping fellow SCD patients manage triggers, learn their bodies, and advocate for themselves. I serve in the role of State Director for the SCFT. In this role, I organize workshops, meetings, and information-sharing sessions for SCD patients and caregivers that cover a variety of topics including stress reduction, parenting and caregiving, and wellness and medical interventions.
I have spoken professionally about many of these issues, including:
●Weather and elevation challenges
●Vitamin supplements and topical options
●Nontraditional gig employment options
●Financial management and budgeting
●Back to school preparedness – Individual education plan (IEP) processes
●Dating with SCD
●Parenting with SCD
●Caregivers of patients with SCD
●Coronavirus disease 2019 (COVID-19) and SCD
I have also participated in and represented the SCFT on several research projects, focusing on research dissemination practices, guide to SCD 101, and how to navigate research articles. My participation in these and other SCD projects allows me to continue to learn and share information with the patient stakeholder families to improve lived experiences.
WHAT I'VE LEARNED — Lifelong habits instilled by my parents included faith, ongoing education, and self-advocacy, which I have carried through adulthood.
I have learned that it is critical to living as best a life as possible as a SCD patient. I remain acutely aware of my body, pain-episode triggers, environmental settings, and the latest medical treatments and advances in the field of SCD. My lived experience makes me an expert in my health, and self-advocacy is key to living productively. I always try to give physicians the benefit of the doubt when they seem unfamiliar with my disease.
Finally, everyone's body is different, and my experience is only one example of what I've learned and some areas of success.
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