From time to time, I like to start the column defining what sickle cell is. Sickle cell anaemia is a complex inherited blood disorder where the red blood cells, which carry oxygen around the body, develop abnormally. The disorder mainly affects people of African, Caribbean, Middle Eastern, Eastern Mediterranean, and Asian origin.
Normal red blood cells are flexible and disc-shaped, but in sickle cell anaemia, they can become rigid and shaped like a crescent (or sickle). The sickle-shaped cells contain defective haemoglobin, the iron-rich protein that enables red blood cells to carry oxygen from your lungs to the rest of the body. The abnormal cells are also unable to move around as easily as normal-shaped cells and can block blood vessels, resulting in tissue and organ damage and episodes of severe pain. Such episodes are known as a sickle cell crisis or a vaso-occlusive crisis. They can last from a few minutes to several months, although on average, most last five to seven days.
The abnormal blood cells also have a shorter lifespan and aren’t replaced as quickly as normal blood cells. This leads to a shortage of red blood cells, known as anaemia. Normal red blood cells have an average lifespan of about 120 days in circulation, but sickle cells only live for about 10 to 20 days. As these red blood cells get destroyed too soon, it causes one to have fewer red blood cells in one’s system.
Those living with sickle cell usually experience all through their lifetime hemolysis, which is the destruction of red blood cells and vaso-occlusion in multiple organs, that leads to complications such as cardio-pulmonary disease, strokes, and avascular necrosis. Sickle cell, as one age, brings about complications that limit mobility. These various complications will lead to frequent hospitalisations, and this could additionally increase the risk of functional decline in old age.
Adults with sickle cell may have more frequent hospitalisations than those living without sickle cell. Ageing is defined as deterioration in physiological function that occurs over time. Adults with sickle cell demonstrate the substantial and early deterioration of multiple organ systems that lead to complications seen frequently in geriatric populations, such as cardiopulmonary disease, sensory deficits, and a decline in physical and cognitive function. Adults with sickle cell are at increased risk of extreme functional decline due to complications such as avascular necrosis of the hips, often requiring surgical interventions. The capabilities and vulnerabilities of older individuals with sickle cell are measured by physical function, cognitive function, psychological state, nutritional status, social support, comorbidities and medications.
With increasing survival, complications of sickle cell, which develop without one’s knowledge over time, are becoming more apparent and common in older patients, particularly those in their fifth decade and beyond. Other age-related non-sickle cell conditions could add to the morbidity of someone living with sickle cell. A regular and comprehensive review and monitoring for early signs of organ damage is necessary, as not all symptoms are attributable to sickle cell. This is to help limit the delay in diagnosis and in starting appropriate interventions. It seems over the years, the primary goal in the management of the older adult with sickle cell has been improving anaemia and minimising organ damage; the time has come for medical teams around the world to be more proactive in considering curative therapies previously offered to the younger patients, as people are living longer now. Curative or experimental interventions should also be discussed early with older people living with sickle cell before complications render such patients ineligible for these treatments.
As someone who falls into this category, in terms of living beyond the life expectancy for people living with sickle cell, I can say that firstly, I never expected to be alive at 50 and as a result, I did not plan for beyond 50s. Here I am alive and well (bar sickle cell) and achieving milestones. I look back and if I knew I would be alive now, I would not have stayed in employment that did not care that I live with sickle cell; I would have been outspoken about the condition well before now; I would have challenged medical teams in the hospital. Basically, I would have handled the challenges of sickle cell differently. The bottom line is, I learn, and I move.
Secondly, I have better knowledge looking after myself. When I was younger, I hardly took any tablets. I mean when I moved out of my parents’ home. But with age, tablets, supplements, and a healthier eating habit have helped me so far. Writing this column weekly means as I research on what I write about, I also learn. I am now making much better healthy lifestyle choices.
Lastly, I see growing old as a privilege that I do not take for granted. I have gone back to learn at a higher institution and it was a challenging experience because of sickle cell, but getting the certificate meant a lot to me. Sometimes, when one is constantly sick and one’s life is regularly disrupted by the thing called crisis, it is easy for one’s mental health to be affected and have low self-esteem. But now, I think to myself and say, ‘Who is he who saith a thing, when the Lord has not said it? No one has the final say over my life, except God who created me.’ I ask myself, when sick, ‘Do you want to live or die? Do you want to achieve your purpose or not because of sickle cell? Why should you give sickle cell so much power to control your aspirations in life?’ The answer is: I no longer do and whenever I have a crisis, like recently during my graduation ceremony, I think, ‘This too shall pass,’ and it did.
If you would like to get in touch with me about sickle cell, do so, via email: [email protected] And do check out my blog: https://www.dailylivingwithsicklecell.com/ My book on sickle cell – HOW TO LIVE WITH SICKLE CELL and my other books are available for purchase on www.amazon.com
