General

Actually…Things Have Gotten Better : Improvements In Sickle Disease

If you are a sickle cell patient in 2018, hardships constitute a significant aspect of living with sickle cell disease. The issues go far beyond having a painful illness, and for many include obscure medical and social concerns sickle warriors privately navigate alone. The list of dread often includes real suspicion of going to the emergency room, particularly a new one, for fear of mistreatment; or the subtle assumptions of some staff that you are a drug-seeking addict, there to get high-not receive treatment for a painful sickle cell crisis. Even hatred of pain medication and the way opioids can make you feel, force warriors in a painful sickle cell crisis into a lose-lose situation. Choose physical agony, or compromise your beliefs for pain relief.

Sometimes the concerns are less complicated; nevertheless, they are just as annoying. You may hate being cooped up in a hospital bed surrounded by other smelly, coughing, sad and sick people. Or the constant disruption a sickle cell crisis does to personal and professional life will erode the patience of a saint. The list of distasteful experiences that sickle disease force you to confront is long, still despite that truth, actually things have gotten better.

When tallying all that confronts the SCA community, counting improvements can seem foolish. It is like discussing improved race relations in the age of Black Lives Matter, and Donald Trump in the White House. True, this is not 1930’s America, but if you spend five minutes on the internet noting the routine racially offensive thoughts posted in anonymity, it is hard to feel like you live in a post-racial world. Still, change exits whether it is evident or not, and race relations have vastly improved just as the quality of sickle disease treatment is much better, just within my lifetime.

Do Little…No, On Second Thought Do Nothing

As a recently diagnosed six-year-old with sickle cell anemia, doctors entered my life and rocked my world. Restrictions flooded my life and became the new norm. All through elementary school, I sat on the gym floor watching my classmates run and play with all the freedom and joy children could muster. I lived a life full of restriction, and while everyone meant well, I paid a heavy social price for all that coddling.

It was another fifteen years before doctors began to instruct me to participate in all the physical activity I could tolerate. To embrace physical activity, not avoid it -but not overdo it. This new way of thinking allowed me to understand my body, decipher what triggers caused crisis, and what things were not harmful to me.

Things were just different in the 70’s. My hematologist, head of his department, would advise my mother to wait three days before coming to the hospital for a sickle cell crisis! Three days of agony to determine whether the crisis was severe enough for a hospital stay. The days of pain at home seemed to be unimportant. As a result, I spent countless hours in severe pain, waiting for that third day to go to the hospital.

The nights were the worst! Alone, in agony, all night long – hour after hour, with no one coming to help me. These were the worst sickle cell moments of my life. As a child, the solitude of a pitch black, dead-silent house, as slow hours passed away; while pain overwhelmed me, and I wished for my mom’s presence, or the pain to go away so I could at least sleep, consumed every second of the night. The loneliness of it all was breathtaking, and to this day, it remains the most significant scar on my psyche courtesy of sickle cell anemia. So knowing that in 2018, doctors encourage sickle patients to head to the ER as soon as crisis pain presents itself is a source of comfort, especially knowing children will not waste days needlessly suffering when relief is readily available.

I’m Sorry You Will Have To Wait A Little Longer

Growing up, I spent countless hours in ER waiting rooms. Sitting for three hours plus was a regular occurrence. A sickle crisis was not considered high on the priority list, so we waited. I can not say why wait times were so long; I am not sure how the medical community approached sickle disease management. I just know in the seventies, I encountered hospitals with obscene ER wait times.

Currently, the sickle cell anemia CDC website states sickle cell patients may encounter long wait times in the ER. So the issue remains, however, those wait times are nothing like they were in the seventies; the CDC, researchers, and advocates are aware of the ER wait issue and attempting to address the problem. Improved triage assessment means 2018 sickle patients will not routinely wait three-plus hours to see a doctor. While it still occurs, it is the exception, not the rule. This was not the case in the seventies and the ER wait times of today are better than they were 40 years ago.

Keep on Pushin’

I state this points to bring hope to the community, not minimize the real problems currently existing. There is serious work to do. The opioid crisis and its issues are unfairly impacting sickle disease treatment. Cancer treatment has been lucky to be exempted, sickle cell and other diseases have not. That is a clear and present danger to the needs of some sickle disease patients. But we must remember, we have hydroxyurea today, we did not 50 years ago. We have bone marrow and stem cell transplants as a cure option, we did not have such options when I was a teenager. Scientists have mapped the human genome, and gene editing technology like Crispr may provide breakthroughs for sickle cell down the line. For a 2018 newborn diagnosed with sickle cell disease, a cure within their lifetime looks possible, if not likely.

There is a long way to go, on what has traditionally been a very bumpy road, but to keep us motivated along our journey, I suggest we look back from time to time to see how far we have come. Improvements in sickle disease treatment are occurring. So let us continue to fight the good fight and keep on pushin’ for the day a cure comes a knocking.

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