Hereditary sodium channelopathy-related small fibers neuropathy

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ORPHA:306577OMIM:133020G99.1
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Overview

Hereditary sodium channelopathy-related small fiber neuropathy is a rare genetic condition that affects the small nerve fibers throughout your body. These small nerve fibers are responsible for sensing pain, temperature, and controlling automatic body functions like heart rate, sweating, and digestion. In this disease, mutations (changes) in genes that control sodium channels — tiny gateways on nerve cells that help send electrical signals — cause these small nerve fibers to malfunction or become damaged over time. The most common symptoms include burning pain, tingling, or prickling sensations, usually starting in the feet and hands. Many people also experience episodes of intense pain that can come and go. Because the small fibers also control involuntary body functions, patients may have problems with sweating (too much or too little), dizziness when standing up, dry eyes or mouth, and digestive issues. The severity of symptoms can vary widely, even among family members who carry the same genetic change. There is currently no cure for this condition. Treatment focuses on managing pain and other symptoms. Medications used for nerve pain, such as certain antidepressants, anti-seizure drugs, and topical treatments like lidocaine patches, are commonly prescribed. Some patients may benefit from sodium channel-blocking medications. Research is ongoing to develop more targeted therapies based on the specific genetic cause.

Key symptoms:

Burning pain in the feet and handsTingling or prickling sensationsStabbing or shooting pain episodesIncreased sensitivity to painReduced ability to feel temperature changesAbnormal sweating (too much or too little)Dizziness or lightheadedness when standing upDry eyes or dry mouthDigestive problems such as constipation or diarrheaSkin color changes in the hands or feetRapid heart rate or irregular heartbeatFatigueDifficulty sleeping due to painFeeling of walking on pebbles or sand

Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Hereditary sodium channelopathy-related small fibers neuropathy.

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No actively recruiting trials found for Hereditary sodium channelopathy-related small fibers neuropathy at this time.

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No specialists are currently listed for Hereditary sodium channelopathy-related small fibers neuropathy.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

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

Travel Grants

No travel grants are currently matched to Hereditary sodium channelopathy-related small fibers neuropathy.

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Community

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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 specific gene mutation do I have, and how does it affect my sodium channels?,Are there any targeted treatments available for my specific genetic variant?,What pain management options would you recommend starting with, and what are the side effects?,Should my family members be tested for this genetic change?,Are there any clinical trials I might be eligible for?,How often should I have follow-up appointments and repeat skin biopsies to monitor progression?,What lifestyle changes can help manage my symptoms day to day?

Common questions about Hereditary sodium channelopathy-related small fibers neuropathy

What is Hereditary sodium channelopathy-related small fibers neuropathy?

Hereditary sodium channelopathy-related small fiber neuropathy is a rare genetic condition that affects the small nerve fibers throughout your body. These small nerve fibers are responsible for sensing pain, temperature, and controlling automatic body functions like heart rate, sweating, and digestion. In this disease, mutations (changes) in genes that control sodium channels — tiny gateways on nerve cells that help send electrical signals — cause these small nerve fibers to malfunction or become damaged over time. The most common symptoms include burning pain, tingling, or prickling sensatio

How is Hereditary sodium channelopathy-related small fibers neuropathy inherited?

Hereditary sodium channelopathy-related small fibers neuropathy follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.