Perspectives from the People: Life with Sickle Cell Disease

Adults with sickle cell disease (SCD) shared their insights in a panel discussion at the 2018 Management of Sickle Cell Disease Conference held by the Sickle Cell Disease Association of Illinois on November 9, 2018. Their comments, summarized here, echo the sentiments of many other Americans who live with the daily effects of this disorder. Panelists discussed their daily challenges, the internal and external factors that most influenced their quality of life and ended with hopeful advice for younger patients.

Adaptability is Essential to Success

Mr. Dennis McCullum, a successful businessman, opened the session with introductions and asked his fellow panelists to summarize the scope of SCD’s effect on their lives. Mr. Marqus Valentine responded that he found it hard to find employment, having had one, short-term job to date. He is grateful for his supportive family, who at times have had to “choose between paying bills or paying for his medication.” His mother, a nurse, must balance his transportation needs for emergency and routine medical care with his siblings’ school schedules and other needs. The second panelist to speak, Ms. Ifeanyi Beverly Chukwudozie, added that chronic fatigue is a major hurdle that she must overcome to reach her educational goals. Emergency hospital visits to seek care for acute SCD complications interfered with her exams and course schedules, ultimately diverting her from her dream of becoming a military doctor. The US military has a policy against the acceptance of individuals with this condition for enlisted roles, and she was denied based on this fact. Ms. Chukwudozie stated that “sickle cell changed [her] story.” This was echoed by Ms. Dorinda Lawrence-Sims, another panelist who aspired to serve in the military but instead built a small business of her own. She often feels like she must slow down for her body to “catch up” with her planned activities. Two other women on the panel also shared that SCD complications or related discrimination also caused them to modify their career plans.

When asked of the impacts to her life, Ms. Annette Delgado described the upheaval experienced after her family, following their doctor’s recommendation, relocated to Puerto Rico for a warmer climate, only to find that a different climate-related factor, hurricanes, negatively impacted her health as well. After relocating for a second time, she feels that she has finally found an acceptable balance of social support, medical resources, and environmental conditions. Ms. Delgado attributed starting hydroxyurea therapy for her ability to begin working as an airline gate agent. However, her employment had to end when she developed avascular necrosis (AVN), a painful complication of SCD resulting progressive blood vessel damage causes deterioration of one or more joints. These patient accounts show the impact of this genetic disease on all aspects of life including relationships, education, and career.

Finding a Sense of Purpose

Next, Mr. McCullum asked panelists to describe the adjustments they have made to create fulfilling lives for themselves, stating that “Sickle cell disease doesn’t stop us!” Mr. Valentine told a story that demonstrated his mental resolve at an early age. As a young child, he was unable to play outside much of the time and would get easily fatigued while walking home from the school bus stop. Often, neighbors saw him from their cars and stopped to offer a ride, but he confidently responded, “I’ll be alright, I just need to rest here, and I’ll make it in my own time.” This determination led him to start the Sick Cells nonprofit organization. They collect the stories of diverse patients and distribute the distilled wisdom for all. He stated proudly, “Sickle cell molded who I am.” Ms. Chukwudozie reiterated the need to limit her participation in some of the normal rites of childhood, yet she did not internalize a message of defeat. Instead she sought “ways to turn ‘You can’t…’ into ‘How can I…’” She focuses on what she can do to keep a positive mindset. She is currently in a STEM (Science, Technology, Engineering and Math) graduate program and plans to use her voice to advocate for others. Ms. Delgado learned to advocate for herself at a young age while translating for her Spanish-speaking grandmother during her clinic or hospital visits. She spent her time reading when she couldn’t participate in other activities. After discovering only one book in her local library about sickle cell disease, she made it her personal mission to learn all that she could about the condition. Today, she uses that knowledge to take a proactive approach to managing symptoms early. It was clear that each panelist relied on determination and creativity to achieve meaningful milestones in their lives and ultimately leave a broader impact in their communities.

What Makes a Great Sickle Cell Doctor?

The topic of discussion then changed to give special recognition to compassionate medical care providers. Ms. Johnson felt that her primary care provider understood her needs and medical history well. She told a story of her doctor thwarting someone’s attempt to use her name falsely to gain admission to the hospital, simply because she knew it was an unusual time for Ms. Johnson to seek care. Ms. Delgado passes several community hospitals to reach her regular medical team because of the personalized care that she receives. Both Ms. Chukwudozie and Mr. McCullum stated that their hematologists maintained a close physical presence during invasive medical procedures to provide detailed medical guidance and reassurance. Mr. Valentine shared an especially moving story: as a young child, he received pediatric care at one institution but left when his family relocated. As an adult, he was admitted to a different institution where coincidentally a nurse and doctor recognized him from the previous clinic, after several years of no contact. They shared a hug and excitedly exchanged updates on the events of their lives, which Mr. Valentine likened to a college reunion. Later, a doctor from the same team delayed his vacation plans to help Mr. Valentine recover from an acute SCD-related illness in time to see his sister graduate college in Scotland. Across the panel, it seemed that the factors that made these healthcare providers stand out as exceptional were:

  • A shared vision of what success looks like. Sometimes this benchmark was linked with the patient’s ability to engage in meaningful life moments
  • High emotional intelligence which helped them to see the patient as an equal, yet with a unique identity
  • Service-oriented practice style. In extenuating circumstances, these physicians dedicated additional time to ensure a positive outcome for their patients.

 

Next, the group discussed the current crisis of opioid pain medication misuse in the US and how their medical care has been impacted. Panelists described their challenges and solutions to the difficulties imposed by institutional policies originally intended to curb misuse of these medications.  Patients also expressed concern that physicians already seemed hesitant to prescribe opioid pain medications at the elevated dosages required by adults faced with chronic complications. They wondered if adding additional administrative processes to their care plan might further increase suspicion and stigma in medical settings. It’s clear that this topic is an important area for patients, families, and their healthcare providers to collaborate effectively.

Words of Wisdom

Mr. McCullum closed the session by asking the panelists to give advice for parents of newly diagnosed infants or to young adults transitioning to adult care. Their inspiring messages collectively reassured young people and families that they could live purpose-driven and fulfilling lives even as they face challenges posed by an overall lack of treatment options. Their words are paraphrased below:

  • You are not alone. If you feel alone find local people to connect with or reach out through social media to find support.
  • Don’t internalize the limitations that others place on your life. Instead, take responsibility to learn about yourself and explore new ways to expand your capabilities.
  • Seek out local organizations that serve the needs of people affected by SCD. These groups can connect you to helpful programs and resources.
  • Take your medications as prescribed and work with your doctor to develop a health plan that suits you well. Prescription folic acid and hydroxyurea are only effective if taken regularly, this is also true for some other medications used in treating sickle cell disease.
  • Keep a positive mindset. Find habits and lifestyle choices that help you stay motivated.
  • Educate yourself to prepare for increased complications as you or your loved one age. Most of the panelists seemed to agree that their symptoms increased beginning at 20 to 30 years of age. Remember that this is a vulnerable time for all young adults, especially those managing a life-long medical condition.
  • Build up your mental strength. Sometimes your body will be weak, but a strong mind will help you through the healing process.
  • Know your medications and report changes in their effectiveness or side effects to your doctor right away.

In summary, patients expressed hope that they could overcome the challenges present in their lives due to this disease. They also highlighted that attentive treatment from compassionate, knowledgeable medical professionals was vital to their progression toward their personal and professional goals. Creativity, critical thinking, and determination were common themes in the stories shared by panelists and meeting attendees. Keep the conversation going by finding ways to incorporate some of the wisdom held by sickle cell patients into your own life.

 

This blog article was sponsored by Global Blood Therapeutics Inc. GBT Logo Small, the views expressed by the author are her own.

 

 

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