Understanding what jaundice is and how it affects sickle cell patients

Jaundice is one of the most prevalent manifestations of sickle cell disease. Caused by an excess production of the compound bilirubin, jaundice results in a yellowish discoloration of the skin, mucus membranes, and the white part of the eyes, which is called the sclera.

In this column, I’ll focus on the eyes, where the appearance of jaundice is most prominent. Depending on the severity, the condition can turn the eyes light, deep, or greenish yellow.

Pathophysiology

In people living with sickle cell disease, exposure of red blood cells to low oxygen tension causes them to adopt a sickle shape instead of the standard biconcave shape found in healthy people. These sickle-shaped cells not only have an altered structure but also become sticky and fragile, leading to a shorter life span. While normal red blood cells live for approximately 120 days, sickled red blood cells survive only 10 to 20 days.

Due to their abnormal shape, sickled red blood cells are more prone to hemolysis, which releases the yellowish-colored bilirubin into the system. In sickle cell patients, the breakdown of red blood cells occurs faster than the liver can filter, accumulating excessive bilirubin in the body, which causes jaundice.

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Additionally, the presence of gallstones can contribute to jaundice in sickle cell patients. When bilirubin builds up in the gallbladder, it can lead to the formation of gallstones. These gallstones have the potential to block the bile ducts, impairing the flow of bilirubin from the liver and exacerbating jaundice.

Certain genetic mutations can also cause severe jaundice in sickle cell patients. Those who express these genetic variations often experience more severe jaundice that may not respond to conventional management approaches.

Furthermore, other conditions such as sickle cell crises, infections, reactions to blood transfusion, iron overload, sickle cell hepatopathy, and certain medications can also contribute to jaundice in these patients. It’s important to note that jaundice in sickle cell patients can occur solely due to the normal sickling process and does not necessarily require any additional causes.

Personal experiences

During my childhood, I was unaware of my sickle cell status until the age of 11. However, my mother would occasionally notice the yellow appearance of my eyes and prepare a vegetable smoothie that she believed would help. I trusted my mother’s judgment and happily drank the smoothie, even though I didn’t fully understand the reason behind it.

During my junior high school years, before I knew about my sickle cell status, something peculiar happened. Teachers noticed my yellow eyes and ignorantly concluded that I had yellow fever. I quickly relayed this information to my mum, who rejected the idea and reassured me with her strong Christian faith. These were my childhood experiences with jaundice.

Once I became aware of my diagnosis, I gained a deeper understanding of jaundice as one of the manifestations of the disease. As I grew older, I closely observed my jaundice. I recognized that it becomes pronounced with an impending crisis or infection. Consequently, it became an important warning sign for a sickle cell crisis.

Most of the time, jaundice in sickle cell patients is not a cause for concern since it can occur due to the normal processes of the disease. However, if the jaundice unusually deepens, it could indicate an infection or an impending crisis. This deepening jaundice may also serve as an indicator for preventive management, indicating the need to take medication, stay hydrated, rest, or seek medical attention. Suppose the jaundice is accompanied by symptoms such as fever, bone pain, abdominal pain, unusual paleness, or weakness. It could indicate an ongoing crisis, infection, or the presence of gallstones, which requires immediate management.

Furthermore, if jaundice occurs during or after a blood transfusion, it might be due to transfusion reactions or iron overload. If it occurs after using a new medication, it may be that the drug is toxic to the liver.

Stigmatization and management

Unfortunately, some people stigmatize sickle cell patients due to their jaundice, assuming it’s a contagious disease. If you are a sickle cell patient, it is important not to let this behavior affect your self-esteem. Educating yourself about the condition and seeing your hematologist can help you manage your symptoms effectively.

Adequate hydration, medications like hydroxyurea, and procedures like exchange blood transfusions can help manage jaundice. Wearing glasses can help alleviate concerns if you feel bothered by jaundice. Remember, never hesitate to educate others and stand up against stigmatization.

In conclusion, understanding the manifestation and management of jaundice in sickle cell disease is crucial for patients and their caregivers. Patients can effectively manage jaundice and overcome stigmatization by gaining knowledge, seeking medical advice, and advocating for themselves. Remember to always seek professional help when needed.

Note: Sickle Cell Disease News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Sickle Cell Disease News or its parent company, BioNews, and are intended to spark discussion about issues pertaining to sickle cell disease.