ACANTHAMOEBA KERATITIS

Acanthamoeba Keratitis (AK)

Is a serious and severe eye infection that can cause excruciating pain and damage the cornea, potentially leading to vision loss, blindness or loss of the eye.

When diagnosed early, AK can be treated effectively, often resulting in favorable outcomes. The harm done to the cornea may have a minor effect on vision, with scarring potentially being minimal. People with AK need to cultivate patience and resilience as they face the challenges of recovery.

This infection occurs when a tiny organism called Acanthamoeba attaches to a small injury on the surface of the cornea, which is the clear front part of the eye. Once attached, it starts to damage corneal cells as it multiplies rapidly and penetrates deeper into the corneal layers.

This free-living amoeba is often present in various bodies of water, tap water, and soil worldwide. While anyone can contract AK, contact lens wearers, farmers and people with eye injuries are at significantly higher risk.

Source Image: Navaratnam, Jesintha & Utheim, Tor & Rajasekhar, Vinagolu & Shahdadfar, Aboulghassem. (2015). Substrates for Expansion of Corneal Endothelial Cells towards Bioengineering of Human Corneal Endothelium. Journal of functional biomaterials. 6. 917-45. 10.3390/jfb6030917.
Source Image: Navaratnam, Jesintha & Utheim, Tor & Rajasekhar, Vinagolu & Shahdadfar, Aboulghassem. (2015). Substrates for Expansion of Corneal Endothelial Cells towards Bioengineering of Human Corneal Endothelium. Journal of functional biomaterials. 6. 917-45. 10.3390/jfb6030917.

Although AK is classified as a rare disease, it is becoming increasingly common among contact lens wearers and information is hard to come by.

The importance of protection is often unknown to contact lens wearers. Research is limited, AK is frequently misdiagnosed and there is no standardized treatment protocol. This adds to the complexity of managing AK for both patients and healthcare providers.

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SEVERE VISION LOSS

One of the most concerning aspects of AK is the potential for severe vision loss.

Acanthamoeba causes 5% of corneal infections worldwide. Yet is disproportionately responsible for over 50% of cases resulting in vision loss. One quarter of these patients lose 75% of their vision after the condition has resolved.

In fact, approximately 41% of AK cases eventually require a corneal surgery or even become blind. Most cases are unilateral (affecting one eye) while 4% to 11% are bilateral (affecting both eyes). Tragically, AK has a high enucleation rate of 6%. Some AK patients choose to have the affected eye removed.

Because contact lens wearers are at increased risk of developing AK, it is essential to educate them clearly about the serious consequences they may face through improper handling or care of their lenses.

HOW DOES IT HAPPEN?

The Acanthamoeba has a strong affinity for attachment to the corneal epithelium and contact lenses. Contact lenses material can absorb water and provide a hospitable environment for the parasite to grow.

Acanthamoebas use bacteria naturally found on the surface of the eye as a food source while being trapped under contact lenses. The amoeba enters the eye through small scrapes that can be caused by contact lenses, minor eye injuries or trauma to the eye. Once inside the cornea, this parasite is extremely difficult to eradicate. This makes it challenging to treat without damaging the eye through the toxicity of the anti-amoebic eye drops currently in use.

Image Source: Scanning electron micrograph (SEM) of two Acanthamoeba trophozoites approaching an indent in a contact lens, © Fiona L Henriquez, Adam Hammerschmid. University of the West of Scotland (UWS), U.K

Different layers of the cornea with acanthamoeba

Image 5: source © Ronnie Mooney, Roderick Williams and Fiona L Henriquez. University of the West of Scotland, U.K. New solutions in the prevention and treatment of Acanthamoeba keratitis By Ronnie Mooney, Roderick Williams and Fiona L Henriquez.
  1. Acanthamoeba can enter the eye by attaching to contact lenses.
  2. The depleted immune activity allows binding to the epithelial layer.
  3. The amoeba can begin to feed on the epithelial cells.
  4. Micro-abrasions on the epithelial layer provide an opportunity for the amoeba to access the Bowman’s membrane and stroma of the patient.
  5. Drug pressure can instigate encystation of the parasite.
  6. Which can then begin reinfection upon removal of this pressure.

CYCLE OF ACANTHAMOEBA

The life cycle of Acanthamoeba is typically divided into two stages:

TROPHOZOITE STAGE

During the trophozoite stage, the amoebas are active and mobile, multiplying very rapidly, feeding on bacteria or other small organisms in their environment and damaging corneal cells, including the nerves, through their metabolism. This is when a patient might experience a lot of pain, light sensitivity and sometimes a deterioration in their vision.

Images of a Trophozoite –  supplied and used with permission from Dr. Christopher A. Rice (Purdue University). Preparation of cells and Scanning Electron Microscopy (SEM) imaging of amoeba was performed by A. Cassiopeia “Cassie” Russell (University of Georgia) on Dr. Peter Bush’s Field Emission Scanning Electron Microscope (FESEM) with Oxford Energy-dispersive X-ray Spectrometer (EDS) – Hitachi SU70 (University at Buffalo)
Image 3: Throphozoite
Images of a Trophozoite –  supplied and used with permission from Dr. Christopher A. Rice (Purdue University). Preparation of cells and Scanning Electron Microscopy (SEM) imaging of amoeba was performed by A. Cassiopeia “Cassie” Russell (University of Georgia) on Dr. Peter Bush’s Field Emission Scanning Electron Microscope (FESEM) with Oxford Energy-dispersive X-ray Spectrometer (EDS) – Hitachi SU70 (University at Buffalo)

CYST STAGE

In the cyst stage, the amoebas are passive, having formed protective double-walled cells that allow them to survive in harsh environments with low nutrients or high toxicity. This can be due to changes in pH levels or exposure to therapeutic agents.

Images of a Cyst –  supplied and used with permission from Dr. Christopher A. Rice (Purdue University). Preparation of cells and Scanning Electron Microscopy (SEM) imaging of amoeba was performed by A. Cassiopeia “Cassie” Russell (University of Georgia) on Dr. Peter Bush’s Field Emission Scanning Electron Microscope (FESEM) with Oxford Energy-dispersive X-ray Spectrometer (EDS) – Hitachi SU70 (University at Buffalo)
Images of a Cyst –  supplied and used with permission from Dr. Christopher A. Rice (Purdue University). Preparation of cells and Scanning Electron Microscopy (SEM) imaging of amoeba was performed by A. Cassiopeia “Cassie” Russell (University of Georgia) on Dr. Peter Bush’s Field Emission Scanning Electron Microscope (FESEM) with Oxford Energy-dispersive X-ray Spectrometer (EDS) – Hitachi SU70 (University at Buffalo)

This means that the Acanthamoeba enters the eye (cornea) in the form of trophozoites, where they begin to multiply until they are deprived of nutrients or are subjected to toxic medications. At this point, they transform into highly resilient cysts, allowing them to withstand even the most hostile conditions for extended periods of time, ranging from months to years. If treatment is halted prematurely or lacks effectiveness, the surviving cysts can revert to trophozoites, resuming their multiplication and destructive behavior. Estimating the likelihood of such relapses is quite difficult.

Image source: Global Alliance Against AK White Paper

Treating AK requires a careful balance; the treatment must be sufficiently toxic to eliminate the trophozoites and the cysts while preserving the strength of the corneal cells.

HOW CAN AK BE PREVENTED?

Contact lens wearers are particularly susceptible to AK because contact lenses can cause small defects on the cornea, absorb water, or trap the parasite, which is found in any country in the world. Therefore, it is crucial to never let water touch your contact lenses. In addition, it is important to follow proper contact lens hygiene and handling to further reduce the risk of AK infection.

WHAT ARE THE SYMPTOMS?

AK patients might encounter the following signs

  • Inability to wear contact lenses
  • Foreign body sensation
  • Irritation or grittiness
  • Light sensitivity from mild to extreme
  • Redness
  • Excessive tearing
  • Change in vision (blurred to complete loss of vision)
  • Pain from mild to excruciating
  • Headache
  • Severe eye pain and trigeminal neuralgia (pain which occurs on the side of the face)

These symptoms can have a substantial impact on a person’s quality of life.

If you suspect that you may have AK, it is crucial to take out your contact lenses and seek medical attention without delay.

DIAGNOSIS

80%

Misdiagnosed

48%

Herpes Simplex Virus

25%

Fungal Infection

4%

Bacterial Infection

Unfortunately, when it comes to correctly diagnosing AK, statistics show that around 75% to 90 % of AK patients are being misdiagnosed with herpes, bacterial, viral or fungal pathogens, amongst others. More worryingly,  48% of the misdiagnosed AK cases are herpes simplex, which can lead to the administration of corticosteroids and steroids, making the infection worse and leading to a poorer outcome. Another 25,2 % as fungal infection and 3,9% are mistaken as bacterial infection.

The symptoms may be similar to other infections. However, the treatment is very different. It is vital that the correct diagnosis of AK is made quickly. Due to misdiagnosis, it can take days, weeks, months or even over a year to detect AK, during which time the amoeba can continue to multiply or hide deeper in the cornea.

TREATMENT

Despite the disturbing symptoms the first priority in treating AK is to eradicate the parasite. The cornea specialist will prescribe anti amoebic eye drops to attack the amoeba along with other helpful eye drops and oral medication.

The initial phase of treatment involves using the drops every hour 24/7.

In the second phase these eye drops are used during waking hours. The duration of this whole process depends on the severity of the infection, the availability of the drugs and the experience of the specialist. This can take from six months up to one year or even longer. However, in some cases, the medication may be stopped to see how the eye responds. If the infection flares up again, the treatment can be restarted.

Once the amoeba has been eradicated, the specialist’s next priority is to help restore vision. This may include cataract surgery, corneal transplants, and other procedures. From clear sight to loss of vision or removal of the eye, the outcome of treatment varies widely.

The journey is characterized by many ups and downs, an intense drop regime, excruciating pain and many eye appointments, leaving little space for daily life. AK affects not only the eye, but also the mental and emotional health of the patient.

AFTERMATH

Patients need to take extra care of their eyes to prevent further damage or re-infection after treatment for AK.

It is likely that the patient will need to continue to use eye drops or other medications for several months or even years after the initial infection. The doctor may also recommend wearing special contact lenses or glasses to protect the eyes.

Unfortunately, AK can still have serious effects even after the infection has been treated. Some people may experience long-term or permanent damage, which could include vision loss, blindness or, in rare cases, the need for removal of the eye.

It is also important to note that the disease itself, as well as the oral medications used in treatment, can lead to additional issues, such as trigeminal nerve pain, dental problems, stomach issues, or elevated liver and kidney levels.

Regular blood tests are essential when taking oral medications for AK treatment. These tests help to monitor liver and kidney function to detect any potential side effects early. You can reduce the risk of serious complications and ensure your overall safety during treatment by being proactive about your blood counts, allowing your healthcare provider to make any necessary adjustments to keep you safe.

Many AK patients also experience psychological trauma (medical PTSD) and may need professional support to help them cope with these challenges.

INCIDENCE OF AK

Worldwide Acanthamoeba is widely prevalent in water sources like oceans, lakes, rivers, swimming pools, tap water and even bottled water. The increased use of contact lenses for convenience, sports, costumes, and social media trends is contributing to a higher risk of developing AK.

Contact lens wearers are often unaware of the risk associated with wearing contact lenses while sleeping, showering, swimming or participating in water sports.

Two recent studies reveal an estimated incidence of 2.342.9 AK cases per million globally, resulting in around 19.000 – 24.000 new infections each year based on the UN’s 2024 world population estimate of 8.1 billion. However, infection rates vary from country to country, with the UK, Portugal, Egypt, India and New Zealand having the highest per capita numbers, followed by Canada, the Netherlands, Italy, Singapore and Australia.

Image source: The estimated annual incidence of Acanthamoeba keratitis by regions (as defined by the UN). The figure used with permission from co-author Xu. Source: Yuheng Zhang, Xizhan Xu, Zhenyu Wei, Kai Cao, Zijun Zhang, Qingfeng Liang, The global epidemiology and clinical diagnosis of Acanthamoeba keratitis