Most major manifestations of HHT, including AVMs and ruptured telangiectases, are very treatable. They cannot yet be prevented and HHT cannot yet be cured, but the options for treatment allow HHT patients to live normal lives. It can take some time to find the right treatment for you, but don’t give up! HHT is indeed very treatable and there are experts who want to help you develop a treatment plan specific to your HHT.
90% of individuals affected by HHT will develop chronic nosebleeds. In some people nosebleeds may be an uncommon occurrence and one that is easily managed, while others may have multiple nosebleeds every day. The Epistaxis Severity Score (ESS) is a useful tool for tracking nosebleeds to help determine whether treatment is necessary. If you do receive treatment you can use the ESS to track the effectiveness of the procedure.
If your HHT physician determines that you need treatment for nosebleeds, there are several options available to you:
Coagulation therapy changes liquid blood to a congealed form that is less likely to bleed. This is a quick and effective way to stop nosebleeds, although the procedure usually needs to be repeated periodically.
Medical treatments can also include oral medication such as tranexamic acid, estrogen, and tamoxifen.
Several medicated sprays and ointments have been used in small numbers of patients and are currently being studied in clinical trials. If you are interested in participating in studies for nosebleed treatments, talk to your HHT physician to determine which treatments you may be qualified to try.
Surgery is typically only considered when nosebleeds are so severe that a patient’s quality of life is affected, and when coagulation therapy has been repeatedly unsuccessful. There are two options recommended by experts for surgical treatment of nosebleeds.
Many experts prefer laser coagulation to electric or chemical as it reduces the damage to the inside of the nose, but others have reported success with bipolar electric cautery.
In Young’s Nasal Closure Procedure, the nostrils are sewn closed to prevent the extreme blood loss that accompanies severe epistaxis. This surgery is very effective, though patients can no longer breathe through the nose, and senses of taste and smell can be affected.
This procedure blocks an artery and typically stops nosebleeds that have been unresponsive to other treatments. Embolization is only effective for 6-8 weeks and as a result is used primarily for emergencies until another therapy can be started.
Not all AVMs require treatment. Screening by an HHT expert is the best way to determine what course is best for you. If your physician decides that you need to treat your AVMs, there are several treatment options available.
The recommended treatment for AVMs depends almost entirely on the size of the malformation and its location in the body. AVMs can develop and change over time, so periodic screenings are important to ensure your safety and help.
In this procedure, a catheter is used to place a small medical device or quantity of glue (in the case of a BAVM) inside an artery. This device blocks the AVM and reduces or stops the blood flow to an AVM to relieve the pressure on the walls of the blood vessel.
- May be used to treat Brain AVMs that present a risk of hemorrhagic stroke.
- May be used to treat Lung AVMs to prevent stroke and brain abscess.
The success rates of embolization are so high that surgery is rarely required as a treatment for PAVMs.
- The surgical procedure for Lung AVMs removes the part of the lung containing the AVM.
- The surgical procedure for Brain AVMs removes the AVM or places a clip on the site of the malformation.
This procedure uses focused radiation to destroy the AVM tissue, and is only used for Brain AVMs.
Brain and Lung AVMs are not the most common type of AVM, but they are the most likely to require treatment. AVMs can also be present in the Liver, GI Tract and Spine.
Liver AVMs are common but typically do not require treatment. If treatment is required, it is essential that an HHT Center physician is consulted regarding the best option. Procedures to treat Liver AVMs can be very invasive and have the potential to worsen the condition of the patient if an HHT specialist is not involved.
AVMs are also common in the GI Tract, usually in the stomach and upper portion of the small intestine. When necessary, intestinal AVMs can be treated by laser, tranexamic acid, IV Avastin and other angiogenesis inhibitors. The most significant problem with intestinal AVMs is anemia caused by chronic bleeding. Anemia can be treated separately from the AVM with iron supplements and blood and iron transfusions.
People with HHT who suffer from bleeding should be routinely screened for iron deficiency. It is important that iron deficiency be treated aggressively, by replacing the iron stores in the body. There are multiple methods of replacing iron stores.
Heme iron (from meat) is more easily absorbed by the body, while non-heme iron (from meat and plant sources) is harder to absorb. Because vegetarians only consume non-heme iron, their bodies cannot absorb as much of it, so they are at a higher risk for iron deficiency. If you are deficient in iron, your body will absorb more of it from food than it would if you had good iron stores.
Here are some easy ways to increase your intake and absorption of iron:
- Consume iron with a source of vitamin C to increase absorption. Many fruits, vegetables, and juices are good sources of vitamin C, including citrus fruits and juices, cantaloupe, strawberries, broccoli, peppers, and tomatoes. E.g. drink a glass of orange juice with your morning oatmeal
- Meat enhances absorption of non-heme iron, e.g. beef in chili will help you absorb the iron in kidney beans
- Cooking meals in cast iron pans allows iron from the pan to be absorbed by the food
Read more on Pumping Iron in HHT
Iron stores can be replace by taking iron by mouth in the form of iron supplements (tablets, liquid, suspension or elixir). Consult your HHT Center physician for recommended products and dosage. Oral iron can be associated with gastrointestinal side effects such as nausea, bloating, and constipation that frequently limit their use.
In individuals with severe iron deficiency anemia or frequent/severe bleeding, oral iron may not be enough to restore iron stores. In these cases, it is important that treatment with IV iron be considered.
A hematologist may consider intravenous (IV) iron if a patient is intolerant of oral iron or has ongoing blood loss. There are several IV iron products that can be recommended, consult your HHT physician for dosage amounts and time required to administer the dose.
Advantages of IV iron:
- Larger amounts of iron can be replaced at a time
- Reliability and not impacted by dietary issues
- Little to no GI side effects (which are common with oral iron)
Disadvantages of IV iron:
- Time and cost
- Side effects, such as weakness, body aches and headaches
- Risk for serious reaction (anaphylaxis)