Understanding jaundice in sickle cell disease, why it happens, when it is normal, and when medical attention is needed.
Written By: Dr. Victory UwagwuAffiliation: SSHAI Editorial VolunteerPublished: 24 September, 2026 / Learn with SSHAI
Yellowish discoloration of the white part of the eyes (jaundice, also known as scleral icterus) is common in sickle cell disease (SCD) largely because SCD causes chronic hemolysis, which is the premature destruction of red blood cells.
To put this in perspective: while normal red blood cells circulate for about 120 days, in SCD, the damage shortens a red blood cell's lifespan to just 10–20 days. When hemoglobin is broken down, it produces unconjugated bilirubin, a yellow-orange pigment. Under normal conditions, this pigment is transported to the liver to be conjugated and excreted. However, persistent destruction of those red blood cells overwhelm the liver’s capacity to process it. This causes a buildup in the bloodstream known as hyperbilirubinemia, which ultimately makes the sclera appear yellow.
Jaundice usually appears in the eyes first well before skin discoloration occurs. This early visibility is driven by four key physiological factors:
High Elastin Affinity: Bilirubin binds strongly to elastin, a structural protein prevalent in the transparent tissues covering the eye (specifically the bulbar conjunctiva and episclera).
Blood Supply: An extensive network of small blood vessels quickly delivers circulating bilirubin directly to these ocular tissues.
Stark Contrast: The naturally bright, white sclera acts as a high-contrast backdrop, making subtle yellowing immediately obvious.
Zero Pigment Masking: Unlike skin, the eye's thin, transparent surface lacks melanin and other pigments that might obscure slight color changes.
Not necessarily.
Mild, stable jaundice can be part of a person's usual SCD pattern, particularly when chronic haemolysis is present. However, it should not automatically be dismissed as 'normal.' A new, rapidly worsening, or unusually deep yellow discoloration deserves assessment, especially when accompanied by other symptoms.
Yellow eyes become more concerning when the change is sudden, markedly worse than usual, or occurs with symptoms suggesting haemolysis, gallbladder disease, biliary obstruction, or liver involvement.
Severe or persistent abdominal pain, particularly in the right upper abdomen.
Fever or chills.
Marked weakness, dizziness, fainting, breathlessness, or confusion.
Dark coloured urine.
Pale or clay-coloured stools.
Persistent vomiting
Bleeding.
If you are experiencing any of these symptoms, kindly consult your doctor.
The evaluation depends on your complaints. Your doctor may request a complete blood count (CBC) and reticolocyte count, urinalysis, liver function test (LFT), haemolytic markers, and abdominal imaging—often ultrasound when gallbladder disease is suspected.
Chronic haemolysis increases bilirubin delivery to the liver and biliary system and predisposes people with SCD to pigment gallstones (cholelithiasis). Contemporary cohort data continue to show that gallbladder disease is one of the most common hepatobiliary complications of SCD. A 2025 30-year review of 1,009 people with SCD reported cholelithiasis or gallbladder sludge in 42.7% of patients. A 2026 multicentre Brazilian cohort of 2,778 people likewise found cholelithiasis in 35.9%, with indirect bilirubin consistently associated with gallbladder-related outcomes.
No.
It is common to explain SCD jaundice by saying that red cells are destroyed so rapidly that the liver cannot keep up. This is useful as a simple teaching analogy, but it is incomplete. Elevated bilirubin in SCD reflects increased destruction of red blood cells and can be influenced by bilirubin handling, inherited variation in bilirubin metabolism, gallstone disease, hepatic sickling, transfusion-related iron overload, viral hepatitis, and other liver or biliary conditions. Therefore, significant or changing jaundice should be interpreted in a clinical context.
Know your usual baseline and report a significant change in jaundice to your doctor.
Keep routine SCD appointments and follow the disease-modifying treatment plan prescribed by your clinician.
Seek prompt care for severe abdominal pain, fever, confusion, marked weakness or breathlessness, dark urine, pale stools, or rapidly worsening jaundice.
If you receive regular blood transfusions, remain engaged with monitoring for transfusion-related complications, including iron overload.
Do not assume every episode of yellow eyes is 'just sickle cell'; other causes of jaundice can occur in people with SCD.
Key Takeaway
Yellow eyes are a visible clue, not a diagnosis.
Do not assume every episode of yellow eyes is 'just sickle cell'; other causes of jaundice can occur in people with SCD.
Do not disregard warning signs - severe abdominal pain, fever, confusion, marked weakness or breathlessness, dark urine, pale stools, or rapidly worsening jaundice.
Seek early medical intervention for Red-Flag symtoms.
Know your baseline, but don't ignore a change.
1. Deeb M, Leung KK, Ward R, et al. Hepatobiliary complications in patients with sickle cell disease: A 30-year review of 1009 patients. Hepatology Communications. 2025;9(5):e0712. doi:10.1097/HC9.0000000000000712.
2. Centers for Disease Control and Prevention (CDC). Complications of SCD: Liver Problems. Updated May 15, 2024. https://www.cdc.gov/sickle-cell/complications/liver-problems.html
3. Adigwe OP, Onavbavba G, Onoja SO. Impact of Sickle Cell Disease on Affected Individuals in Nigeria: A Critical Review. International Journal of General Medicine. 2023;16:3503–3515. doi:10.2147/IJGM.S410015.
4. Al-Samkari H, et al. Management of liver complications in sickle cell disease. World Journal of Gastroenterology. 2019;25(39):4621–4633.
5. Akinbami A, et al. Correlates of gallbladder stones among patients with sickle cell disease: A meta-analysis. 2021. PMID:34584966.
6. Elendu C, Amaechi DC, Alakwe-Ojimba CA, et al. Understanding sickle cell disease: Causes, symptoms, and treatment options. Medicine. 2023;102(38):e35237.
7. National Academies of Sciences, Engineering, and Medicine. Addressing Sickle Cell Disease: A Strategic Plan and Blueprint for Action. National Academies Press; 2020.
8. Kato GJ, Steinberg MH, Gladwin MT. Intravascular hemolysis and the pathophysiology of sickle cell disease. Blood. Background literature summarized in contemporary reviews.
9. REDS-III Brazilian SCD cohort study. Laboratory and genetic characteristic associated with gallbladder-related outcomes in sickle cell disease in Brazil. PubMed PMID:41692868. Published 2026.
