About the disease
Understanding the disease Recklinghausen
Recklinghausen disease, also known as:
Type 1 neurofibromatosis (NF1) is a type of disease that affects the nerves. Nerves help send information from the brain to every part of the body and back. They are involved in everything from tasting food to watching movies and playing sports.
In order to repair the damage and keep it healthy, cells in the body need to grow and make copies of themselves (divide). Most cells in the body send and receive messages telling them to do this. However, too much growth and division can be a problem.
The gene inside the cells helps control these messages and make sure that cells don’t grow and divide too much. When this gene is malfunctioning (mutated), cells around the nerves continue to send messages. This causes clusters of cells (tumors) to form on the nerves
Some people with NF1 develop non-cancerous (benign) tumors around large groups of nerves. They are called plexus neurofibromas (PN). PN that are difficult to remove during surgery are called non-surgical. NF1 affects people in different ways, but it often affects different parts of the body. The effects of PN usually depend on where they are in the body.
People with NF1 sometimes experience:
- Sight problems
- Problems with memory, concentration, attention and coordination
- Weaker bones
- Skin growths and brown spots, called “cafe au lait spots”
- Mobility problems
How selumetinib works
Selumetinib blocks the messages that tell tumors to grow. Without these messages, cells do not grow as fast.
In people with NF1 and PN, it can help control the growth of tumors around the nerves and reduce pain.