Ophthalmology

Standard of Care & Ongoing Monitoring

At a Glance

The standard of care for Choroideremia involves rigorous longitudinal monitoring through annual exams using advanced imaging like FAF and OCT. Care priorities shift from establishing baselines in your 20s to closely monitoring accelerated decline in your 30s and preserving central vision after 40.

Because there is currently no approved cure for Choroideremia (CHM), the “Standard of Care” focuses on rigorous longitudinal monitoring—tracking the disease carefully over time to protect remaining vision and prepare for emerging therapies ^[1]^[2].

A diagnosis of CHM is not a signal to wait for blindness; it is a signal to begin a lifelong partnership with a specialized care team.

The Monitoring Schedule

While every patient is unique, standard clinical practice generally involves an annual comprehensive eye exam ^[3]^[4]. These visits are not just to check your prescription for glasses; they are meant to capture detailed data on the “islands” of your remaining vision ^[5].

Your Essential “Toolbox” of Tests

At every major follow-up, your doctor should use multimodal imaging. These tests are vital because your central vision (the eye chart) often stays stable even while the disease is moving ^[6]^[5].

Fundus Autofluorescence (FAF): Measures the residual RPE area. This is the most critical measurement for tracking the “island” of surviving cells ^[7]^[8].
Optical Coherence Tomography (OCT): Measures the ellipsoid zone (EZ), which shows the health of your light-sensing cells ^[3]^[8].
Microperimetry: A functional test that measures the sensitivity of specific points on your retina. This is often more sensitive to change than a standard visual field test ^[4]^[6].

Monitoring by Life Stage

The priorities of your care will shift as you move through different decades of life.

Age 20 to 40: The Baseline and Transition

During your 20s, the disease typically progresses slowly ^[9]. The goal here is to establish a strong “baseline” so you know your individual rate of change.

The Transition Window: Between ages 30 and 40, many patients enter a phase of accelerated decline in their best-corrected visual acuity ^[10]. During this decade, your doctor may recommend more frequent visits (every 6 months) to monitor the center of your vision closely.

After Age 40: Protecting the Center

After age 40, the focus shifts toward preserving foveal function (reading vision) ^[11]^[12].

Advanced Tracking: Even if the peripheral vision is very limited, maintaining the central “island” is the priority.
Rehabilitation: This is the time when low-vision rehabilitation becomes most critical, focusing on tools like high-powered magnifiers and adaptive technology to maintain independence ^[13]^[14].

Supportive Care: Beyond the Clinic

While research into gene therapy continues, you can take active steps to support your eye health today:

UV Protection and Transition Safety: Wearing high-quality, wrap-around sunglasses that block 100% of UVA and UVB rays is recommended outdoors to reduce environmental stress ^[1]. However, be extremely cautious during lighting transitions. Because your eyes already struggle to adapt to the dark, walking indoors or into a heavily shaded area with dark glasses on can create a severe trip and fall hazard. Remove your sunglasses before entering dimly lit spaces ^[15].
Nutrition: There is currently no evidence-based dietary intervention (like high-dose Vitamin A) proven to slow CHM ^[1]^[16]. A balanced, healthy diet is recommended for general well-being.
Genetic Counseling: Ongoing counseling can help you understand inheritance patterns for your children and keep you informed about new clinical trials that require specific genetic markers ^[17].

Common questions in this guide

How often should I see my eye doctor for Choroideremia?

Standard clinical practice generally involves an annual comprehensive eye exam. However, if you enter a phase of accelerated visual decline—typically between ages 30 and 40—your doctor may recommend visits every six months to monitor your central vision more closely.

What imaging tests are used to track Choroideremia progression?

Doctors use multimodal imaging to track the disease, even when your central vision seems stable. Key tests include Fundus Autofluorescence (FAF) to measure surviving cells, Optical Coherence Tomography (OCT) to check light-sensing cells, and Microperimetry to test specific spots on your retina.

At what age does Choroideremia progression typically speed up?

Many patients experience a phase of accelerated decline in their best-corrected visual acuity between the ages of 30 and 40. Before this, during your 20s, the disease typically progresses more slowly.

Should I wear dark sunglasses indoors if I have Choroideremia?

No, you should remove sunglasses before entering dimly lit spaces. Because your eyes already struggle to adapt to the dark, wearing dark glasses indoors or in heavy shade creates a severe trip and fall hazard.

Are there any diets or vitamins proven to slow down Choroideremia?

There is currently no evidence-based dietary intervention, such as high-dose Vitamin A, proven to slow down Choroideremia. Doctors recommend maintaining a balanced, healthy diet for your general well-being.

Curated prompts to bring to your next appointment.

1.Based on my current rate of progression, do you recommend annual or bi-annual monitoring?
2.At my last visit, what were the exact mm² measurements for my residual RPE area and ellipsoid zone?
3.Should I be referred to a low-vision specialist now for a baseline assessment, even though my central vision is still good?
4.Are there any specific lifestyle modifications, like UV protection or dietary changes, that you recommend for my situation?
5.How will our monitoring plan change once I reach the 'accelerated decline' window in my 30s or 40s?

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References (17)

1
Choroideremia research: Report and perspectives on the second international scientific symposium for choroideremia.
Chan SC, Bubela T, Dimopoulos IS, et al.
Ophthalmic genetics 2016; (37(3)):267-75 doi:10.3109/13816810.2015.1088958.
PMID: 26855058
2
Molecular Therapy for Choroideremia: Pre-clinical and Clinical Progress to Date.
Kalatzis V, Roux AF, Meunier I
Molecular diagnosis & therapy 2021; (25(6)):661-675 doi:10.1007/s40291-021-00558-y.
PMID: 34661884
3
Prospective deep phenotyping of choroideremia patients using multimodal structure-function approaches.
Hagag AM, Mitsios A, Narayan A, et al.
Eye (London, England) 2021; (35(3)):838-852 doi:10.1038/s41433-020-0974-1.
PMID: 32467628
4
Exploring Scotopic Microperimetry as an Outcome Measure in Choroideremia.
Taylor LJ, Josan AS, Adeyoju D, et al.
Translational vision science & technology 2024; (13(9)):29 doi:10.1167/tvst.13.9.29.
PMID: 39348136
5
A Prospective, Observational, Non-interventional Clinical Study of Participants With Choroideremia: The NIGHT Study.
Maclaren RE, Lam BL, Fischer MD, et al.
American journal of ophthalmology 2024; (263()):35-49 doi:10.1016/j.ajo.2024.01.022.
PMID: 38311152
6
Ranked Importance of Visual Function Outcome Measures in Choroideremia Clinical Trials.
Josan AS, Taylor LJ, Xue K, et al.
Investigative ophthalmology & visual science 2024; (65(13)):58 doi:10.1167/iovs.65.13.58.
PMID: 39601638
7
Long-term Natural History of Atrophy in Eyes with Choroideremia-A Systematic Review and Meta-analysis of Individual-Level Data.
Shen LL, Ahluwalia A, Sun M, et al.
Ophthalmology. Retina 2020; (4(8)):840-852 doi:10.1016/j.oret.2020.03.003.
PMID: 32362554
8
Measurement and Reproducibility of Preserved Ellipsoid Zone Area and Preserved Retinal Pigment Epithelium Area in Eyes With Choroideremia.
Hariri AH, Velaga SB, Girach A, et al.
American journal of ophthalmology 2017; (179()):110-117 doi:10.1016/j.ajo.2017.05.002.
PMID: 28499705
9
CHM/REP1 Transcript Expression and Loss of Visual Function in Patients Affected by Choroideremia.
Di Iorio V, Esposito G, De Falco F, et al.
Investigative ophthalmology & visual science 2019; (60(5)):1547-1555 doi:10.1167/iovs.18-25501.
PMID: 30995293
10
Bilateral visual acuity decline in males with choroideremia: a pooled, cross-sectional meta-analysis.
Bozkaya D, Zou H, Lu C, et al.
BMC ophthalmology 2022; (22(1)):29 doi:10.1186/s12886-022-02250-z.
PMID: 35034620
11
Natural History of the Central Structural Abnormalities in Choroideremia: A Prospective Cross-Sectional Study.
Aleman TS, Han G, Serrano LW, et al.
Ophthalmology 2017; (124(3)):359-373 doi:10.1016/j.ophtha.2016.10.022.
PMID: 27986385
12
Choroideremia Gene Therapy Phase 2 Clinical Trial: 24-Month Results.
Lam BL, Davis JL, Gregori NZ, et al.
American journal of ophthalmology 2019; (197()):65-73 doi:10.1016/j.ajo.2018.09.012.
PMID: 30240725
13
Mapping the Outcomes of Low-Vision Rehabilitation: A Scoping Review of Interventions, Challenges, and Research Gaps.
Ekemiri K, Adebo O, Ekemiri C, et al.
Vision (Basel, Switzerland) 2026; (10(1)) doi:10.3390/vision10010003.
PMID: 41562954
14
Low vision rehabilitation in improving the quality of life for patients with impaired vision: A systematic review and meta-analysis of 52 randomized clinical trials: Retraction.
Medicine 2021; (100(28)):e26669 doi:10.1097/MD.0000000000026669.
PMID: 34260576
15
Clinical Manifestations and Genetic Analysis of 5 Korean Choroideremia Patients Initially Diagnosed With Retinitis Pigmentosa.
Kim JH, Han JW, Choi EW, et al.
Journal of Korean medical science 2022; (37(3)):e5 doi:10.3346/jkms.2022.37.e5.
PMID: 35040292
16
Choroideremia Gene Therapy.
Lam BL, Davis JL, Gregori NZ
International ophthalmology clinics 2021; (61(4)):185-193 doi:10.1097/IIO.0000000000000385.
PMID: 34584056
17
A hypomorphic variant of choroideremia is associated with a novel intronic mutation that leads to exon skipping.
Waldock WJ, Taylor LJ, Sperring S, et al.
Ophthalmic genetics 2024; (45(2)):210-217 doi:10.1080/13816810.2023.2270554.
PMID: 38273808

This page provides educational information on the monitoring and standard of care for Choroideremia. Always consult your ophthalmologist or specialized care team to determine the best monitoring schedule and supportive care for your specific vision needs.

Get notified when new evidence is published on Choroideremia.

We monitor PubMed for new peer-reviewed studies on this topic and email a short summary when something meaningful changes.

Guide Contents