Achromatopsia

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ORPHA:49382OMIM:216900H53.5
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4Active trials25Specialists8Treatment centers1Financial resources

Where are you in your journey?

UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
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Overview

Achromatopsia (also called total color blindness or rod monochromatism) is a rare inherited eye condition where the cone cells in the retina — the part of the eye responsible for seeing color and fine detail in bright light — do not work properly or are absent. Because cones are needed to see color and to see clearly in daylight, people with achromatopsia cannot distinguish colors and have very poor central vision. The retina also contains rod cells, which handle vision in dim light, and these usually work normally in achromatopsia, meaning many people see better in low-light conditions than in bright ones. The main symptoms appear from birth or very early infancy and include extreme sensitivity to light (called photophobia), involuntary eye movements (nystagmus), very reduced sharpness of vision, and a complete or near-complete inability to see colors. Most people with achromatopsia see the world in shades of gray, black, and white. There is currently no cure for achromatopsia, but several supportive treatments can help manage symptoms. Tinted lenses, dark glasses, and special contact lenses can reduce light sensitivity and improve comfort. Low vision aids can help with daily tasks. Exciting gene therapy research is underway in clinical trials, offering hope for future treatments that may restore cone cell function.

Also known as:

Key symptoms:

Complete or near-complete inability to see colors (seeing only in shades of gray)Extreme sensitivity to bright light (photophobia)Involuntary, repetitive eye movements (nystagmus)Very poor sharpness of vision (low visual acuity, typically 20/200 or worse)Better vision in dim or low-light conditions than in bright lightDifficulty reading standard print without aidsSquinting or closing eyes in bright environmentsCentral vision loss or blurringDifficulty recognizing faces in bright lightPreference for shaded or indoor environments

Clinical phenotype terms (20)— hover any for plain English
Abnormality of refractionHP:0000539MonochromacyHP:0007803Pendular nystagmusHP:0012043Undetectable light-adapted electroretinogramHP:0030465Color vision test abnormalityHP:0030584Inner retinal layer loss on macular OCTHP:0030620Central scotomaHP:0000603Hypoplasia of the foveaHP:0007750Absent foveal reflexHP:0030825Abnormal macular morphologyHP:0001103Abnormal pupillary light reflexHP:0007695Retinal pigment epithelial mottlingHP:0007814Attenuation of retinal blood vesselsHP:0007843Eccentric visual fixationHP:0025549Retinal pigment epithelial atrophyHP:0007722
Inheritance

Autosomal recessive

Passed on when both parents carry the same gene change; often skips generations

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗OMIM ↗NORD ↗

FDA & Trial Timeline

5 events
Mar 2026RINGERS: New indication approved
FDAcompleted
Jul 2025Natural History Study of Inherited Retinal Diseases

Zhongmou Therapeutics

TrialRECRUITING
Aug 2017Safety and Efficacy Trial of AAV Gene Therapy in Patients With CNGA3 Achromatopsia (A Clarity Clinical Trial)

Beacon Therapeutics — PHASE1, PHASE2

TrialACTIVE NOT RECRUITING
Apr 2016Safety and Efficacy Trial of AAV Gene Therapy in Patients With CNGB3 Achromatopsia (A Clarity Clinical Trial)

Beacon Therapeutics — PHASE1, PHASE2

TrialACTIVE NOT RECRUITING
Nov 2015Safety and Efficacy of rAAV.hCNGA3 Gene Therapy in Patients With CNGA3-linked Achromatopsia

STZ eyetrial — PHASE1, PHASE2

TrialACTIVE NOT RECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

No FDA-approved treatments are currently listed for Achromatopsia.

4 clinical trialsare actively recruiting — trials can provide access to cutting-edge therapies.

View clinical trials →

Clinical Trials

4 recruitingView all trials with filters →
Other1 trial
Natural History Study of Inherited Retinal Diseases
Actively Recruiting
PI: Yin Shen (Renmin Hospital of Wuhan University) · Sites: Wuhan

Specialists

25 foundView all specialists →
DM
David Jacobs, MD, MBA
Specialist
PI on 2 active trials
JB
James Bainbridge
GREENWOOD VILLAGE, CO
Specialist
PI on 1 active trial29 Achromatopsia publications
YS
Yin Shen
Specialist
PI on 1 active trial331 Achromatopsia publications
MM
Matt Feinsod, MD
LONG ISLAND CITY, NY
Specialist
PI on 2 active trials
PP
Paul A Sieving, MD, PhD
SACRAMENTO, CA
Specialist
PI on 1 active trial
JC
Joseph Carroll
Specialist
4 Achromatopsia publications
LK
Line Kessel
Specialist
3 Achromatopsia publications
MH
Michael B Hoffmann
Specialist
3 Achromatopsia publications
MG
Michalis Georgiou
BOSTON, MA
Specialist
3 Achromatopsia publications
MP
Mark E Pennesi
PORTLAND, OR
Specialist
3 Achromatopsia publications
GR
Günther Rudolph
Specialist
2 Achromatopsia publications
ST
Stephen H Tsang
Specialist
2 Achromatopsia publications
AK
Angelos Kalitzeos
Specialist
3 Achromatopsia publications
IG
Irene Gottlob
CAMDEN, NJ
Specialist
5 Achromatopsia publications
MM
Michel Michaelides
Specialist
5 Achromatopsia publications
EB
Eyal Banin
Specialist
4 Achromatopsia publications
NL
Netta Levin
Specialist
4 Achromatopsia publications
KS
Katarina Stingl
BALTIMORE, MD
Specialist
3 Achromatopsia publications
SK
Susanne Kohl
ROCHESTER, NY
Specialist
7 Achromatopsia publications
BM
Brian P Brooks, M.D.
Bethesda, Maryland
Specialist

Rare Disease Specialist

PI on 8 active trials
JP
James Bainbridge, Prof
GREENWOOD VILLAGE, CO
Specialist
PI on 2 active trials
DP
Dominik Fischer, Prof.
Specialist
PI on 2 active trials
BK
Barbara Käsmann-Kellner
Specialist
4 Achromatopsia publications
ST
Stephen Tsang
Specialist
PI on 2 active trials

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Financial Resources

1 resources

NAGLAZYME

BioMarin Pharmaceutical Inc.

NAGLAZYME Patient Support (BioMarin RareConnections)

Patient Assistance
Manufacturer Program
Accepting applications

Travel Grants

No travel grants are currently matched to Achromatopsia.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Achromatopsia

Disease timeline:

New recruiting trial: Natural History Study of Inherited Retinal Diseases

A new clinical trial is recruiting patients for Achromatopsia

New recruiting trial: National Ophthalmic Genotyping and Phenotyping Network (eyeGENE (Registered Trademark)), Stage 3 - Expansion of DNA and Data Repositories for Rare Inherited Ophthalmic Diseases

A new clinical trial is recruiting patients for Achromatopsia

Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.Which gene mutation is causing achromatopsia in my child or myself, and what does that mean for other family members?,What type of tinted lenses or contact lenses would work best for managing light sensitivity?,Are there any gene therapy clinical trials that my child or I might be eligible for?,What low vision services and educational supports should we be accessing right now?,How often should we have eye exams, and what should we be monitoring over time?,What accommodations should I request at school or work?,Are there any support groups or patient communities you would recommend for people with achromatopsia?

Common questions about Achromatopsia

What is Achromatopsia?

Achromatopsia (also called total color blindness or rod monochromatism) is a rare inherited eye condition where the cone cells in the retina — the part of the eye responsible for seeing color and fine detail in bright light — do not work properly or are absent. Because cones are needed to see color and to see clearly in daylight, people with achromatopsia cannot distinguish colors and have very poor central vision. The retina also contains rod cells, which handle vision in dim light, and these usually work normally in achromatopsia, meaning many people see better in low-light conditions than i

How is Achromatopsia inherited?

Achromatopsia follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Achromatopsia typically begin?

Typical onset of Achromatopsia is neonatal. Age of onset can vary across affected individuals.

Are there clinical trials for Achromatopsia?

Yes — 4 recruiting clinical trials are currently listed for Achromatopsia on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.

Which specialists treat Achromatopsia?

25 specialists and care centers treating Achromatopsia are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.

What treatment and support options exist for Achromatopsia?

1 patient support program are currently tracked on UniteRare for Achromatopsia. See the treatments and support programs sections for copay assistance, eligibility, and contact details.