Osler-Weber-Rendu syndrome
There's no cure for HHT, but there are effective treatments and life expectancy is usually very good.
Some patients can be managed by their GP, and some will need to be under the care of a specialist.
Because there are increased risks during pregnancy for women with HHT (such as a slight increased risk of a major bleed or a stroke), it is recommended that if HHT is in the family, this is brought to the attention of the specialist in charge of the woman's care.
Patients who have regular nosebleeds will probably lose a lot of iron through this loss of blood, especially if they also bleed from telangiectasia in the gut.
It is important to replace the lost iron with iron supplements dietary changes alone may not be enough.
Read about the importance of iron and the treatment of anaemia with iron supplements and iron-rich food .
Those with severe nosebleeds may need emergency nasal packing, where the nose is packed with ribbon gauze or a special nasal sponge.
Some people may need to see an ear, nose and throat specialist for treatment. Laser therapy may help (see below).
More severe cases may respond to skin grafting or other surgery.
If a lot of blood has been lost from bleeding inside the body or after nosebleeds, a blood transfusion may be needed.
Read about blood transfusions .
Telangiectasia on the skin or in the lining of the nose can sometimes be improved with vascular laser or intense pulsed light (IPL) treatment.
For the skin, a referral to a dermatologist is usually needed. Around two to four treatments a yearmay be needed, so the overall cost may be significant if the treatment is not available on the NHS.
For the nose, a referral to an ear, nose and throat (ENT) specialist is needed, and these treatments are usually available on the NHS.
Laser and IPL machines produce narrow beams of light aimed at the visible blood vessels in the skin. The heat from the lasers damages the dilated veins and causes them to shrink so they are no longer visible, with minimal scarring or damage to the surrounding area.
Laser treatment can cause pain, but most people do not need an anaesthetic.
AVMs in the body may require specialist treatment. Your HHT specialist will explain any procedures to you in more detail.
For example, AVMs in the lung are usually treated, even if they are not causing any apparent problems. They are treated by embolisation, a procedure that blocks the blood supply to the AVM. A tiny plug is inserted inside the artery supplying the abnormal blood vessel.
Embolisation has virtually replaced open surgery, and is normally carried out under sedation (which will help relax you during the procedure).
For brain AVMs, embolisation, surgery and stereotactic radiotherapy (precisely delivering radiation to the blood vessel) are possible treatment options. Liver AVMs may require different types of specialist treatments.
However, these treatments aren't always appropriate. Your specialist will explain why many AVMs, such as those in the liver and in the brain, may be better left alone, as the risks of invasive treatments may outweigh the potential benefits.
NHS Choices information on hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu syndrome), with links to other useful resources.
In HHT, some of the blood vessels do not develop properly. Normally, the arteries branch off into smaller and smaller blood vessels until they reach a network of capillaries (tiny blood vessels) that
Someone withHHT will have a faulty gene (genetic mutation) and, usually, this will have been inherited from one of their parents. Thisgene normally provides instructions for making certain proteins f
Symptoms usually start in childhood or in the teenage years. Nosebleeds Often, the first sign is developing Nosebleed . They can start at any age, often in childhood. Nosebleeds may be frequent and
There's no cure for HHT, but there are effective treatments and life expectancy is usually very good. Some patients can be managed by their GP, and some will need to be under the care of a specialist
If you have HHT, your clinical team may pass information about you on to the National Congenital Anomaly and Rare Diseases Registration Service (NCARDRS). This helps scientists look for better ways t