Most people in Ghana know someone living with sickle cell disease. We talk about the pain crises, the hospital visits and the need to stay hydrated. What we talk about much less is the eyes. Sickle cell disease can quietly damage the retina, the light-sensitive layer at the back of the eye, often without any symptoms until the damage is advanced. The good news is that a regular dilated eye examination can catch many of these changes early, while there is still time to act.
This guide explains how sickle cell disease affects the eyes, who is most at risk, the warning signs to watch for, and how often people living with sickle cell should have their eyes checked.
Why this matters so much in Ghana
Sickle cell disease is the most common genetic disorder in Ghana. Ghana's Ministry of Health has estimated that about 15,000 to 20,000 babies are born with the condition every year, around 2% of all live births, and that roughly one in four Ghanaians carries the haemoglobin S or C gene (Ghana News Agency). The World Health Organization has likewise reported that 15% to 30% of people in West African countries such as Ghana and Nigeria carry the sickle cell trait (WHO).
With better care, more children with sickle cell disease are growing into adulthood. That is wonderful news, but it also means more people living long enough for eye complications to develop. Eye care needs to be part of routine sickle cell care.
How sickle cell disease affects the eyes
In sickle cell disease, red blood cells can change into a rigid, curved "sickle" shape. These cells do not pass easily through tiny blood vessels, and they can block them. The retina depends on a network of very small blood vessels, which makes it especially vulnerable.
According to the American Academy of Ophthalmology's EyeWiki, eye damage from sickle cell disease, known as sickle cell retinopathy, is usually described in two forms (AAO EyeWiki):
- Non-proliferative sickle cell retinopathy. Small vessels in the outer (peripheral) retina become blocked, which can cause small bleeds and scarring. Many people have no symptoms at this stage.
- Proliferative sickle cell retinopathy. When parts of the retina are starved of oxygen for a long time, the eye can grow fragile new blood vessels. These abnormal vessels can bleed into the jelly that fills the eye (vitreous haemorrhage) or pull on the retina and cause a retinal detachment. These are the main ways sickle cell disease causes vision loss.
Because these changes usually begin at the edges of the retina, central vision can stay clear for a long time. You can read, drive and work normally while damage is building up. This is why waiting for symptoms is not a safe plan.
Who is most at risk?
It may surprise many families that the people at highest risk of serious eye complications are not always those with the most severe general symptoms. EyeWiki notes that proliferative retinopathy is seen more often in people with haemoglobin SC disease (HbSC) and sickle beta-thalassaemia than in people with HbSS (sickle cell anaemia) (AAO EyeWiki). In the long-running Jamaican cohort study cited there, retinopathy was found in 43% of people with HbSC and 14% of people with HbSS by about age 20.
Other points to know:
- The risk tends to increase with age, and changes can begin in the teenage years, particularly in HbSC disease (Oxford sickle cell eye guideline).
- Some people do not know their exact haemoglobin type (genotype). If you or your child has sickle cell disease, ask your doctor which type it is, and share that with your eye doctor.
- Eye injuries need extra care in anyone with sickle cell disease or sickle cell trait. Bleeding inside the front of the eye after an injury can be more complicated when sickle cells are involved (AAO EyeWiki).
Warning signs that need urgent attention
See an eye specialist as soon as possible, the same day if you can, if you have sickle cell disease and notice:
- a sudden shower of new floaters (dots, threads or cobwebs drifting across your vision)
- flashes of light
- a dark shadow, veil or curtain across part of your vision
- sudden blurred vision or loss of vision in one or both eyes
- any injury to the eye, even one that seems minor
These signs can point to bleeding inside the eye or a retinal detachment, and fast treatment can make a real difference (Oxford sickle cell eye guideline). You can read more about floaters and flashes in our article on understanding floaters and flashes.
How often should people with sickle cell disease have their eyes checked?
Because early retinopathy often causes no symptoms, regular screening is the most important step. International expert recommendations include:
- An expert panel report summarised by the American Academy of Family Physicians recommends referral to an ophthalmologist for an annual dilated retinal examination starting at age 10, with re-examination every one to two years if the results are normal (AAFP).
- EyeWiki recommends a complete eye examination at least once a year for people with all types of sickle cell disease, including SC, SS and S-thalassaemia (AAO EyeWiki).
- People who already have retinopathy usually need yearly or more frequent review, as advised by their eye specialist.
Your eye doctor will advise the right schedule for you, based on your age, genotype and what they find.
What happens at a sickle cell eye check?
A sickle cell eye check is painless. It usually includes:
- A vision test and a general examination of the front of the eye.
- Dilating eye drops, which widen the pupils so the eye doctor can examine the whole retina, including the edges where sickle cell changes usually start. Your vision may be blurry and sensitive to light for a few hours afterwards, so bring sunglasses and avoid driving yourself home.
- Where needed, retinal imaging, such as photographs of the retina or an optical coherence tomography (OCT) scan, to record and monitor any changes. See our diagnostic services.
If treatment is needed, options described by the AAO include laser treatment to help shrink abnormal new blood vessels, injections into the eye in some cases, and surgery for bleeding that does not clear or for retinal detachment (AAO EyeWiki). Your eye specialist will explain which, if any, is right for you. Learn more about our laser eye treatments.
Practical tips for families
- Put the eye check on the calendar. Pair it with your regular haematology or sickle cell clinic visit so it is not forgotten.
- Start in the pre-teen years. For children with sickle cell disease, plan the first retinal examination by around age 10, or earlier if your doctor advises. Our children's eye care team can help.
- Tell every doctor about your sickle cell status. Some eye medicines and procedures need extra care in people with sickle cell disease or trait.
- Protect your eyes from injury. Wear protective eyewear for sports, DIY and work with tools or chemicals.
- Keep up with your overall sickle cell care. Good general management, as advised by your haematologist, supports your whole body, including your eyes.
- Know your genotype. Ghana has been expanding newborn screening, but many adults have never been tested. Knowing your status helps you and your doctors plan.
Book a sickle cell eye check in Accra
If you or a family member lives with sickle cell disease and has not had a dilated eye examination in the past year, now is a good time. At Accra Specialist Eye Hospital, our eye specialists can examine the retina and advise on the right follow-up schedule.
Book an appointment online or call us on 0500 060 545. If you notice sudden floaters, flashes, a shadow over your vision or an eye injury, please seek care immediately.
This article is for general information and does not replace a personal consultation with a qualified eye care professional.
Sources
- American Academy of Ophthalmology, EyeWiki: Sickle Cell Retinopathy
- American Academy of Family Physicians: Management of Sickle Cell Disease: Recommendations from the 2014 Expert Panel Report
- Oxford University Hospitals / NSSG: Eye complications in sickle cell disease, clinical guideline
- World Health Organization: Sickle-cell anaemia, report by the Secretariat (A59/9)
- Ghana News Agency: National strategy launched to improve sickle cell disease care
