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Hereditary Hemorrhagic Telangiectasia (HHT) Treatment

December 18, 2017 By Dr. Jeremy Feldman

 treatment laboratory

Nose bleeding

The first step in treating a nose bleed is applying pressure.  You should lean forward slightly and pinch your nose with firm pressure.  Don’t let go for ten minutes.  When you let go, don’t try and blow your nose and avoid packing your nose with tissue paper.  When you blow your nose, you will displace the clot that has stopped the bleeding. Similarly, when you pack your nose it may help stop the bleeding but when you pull the tissue paper out you will pull off the clot that has formed and your nose will start bleeding again.

If your nose bleed does not stop or if it is very rapid then go to the local emergency department.  They may have to pack it with a “RhinoRocket”. This is not a comfortable experience but it is very effective.

Prevention of recurrent nose bleeding in HHT is a challenge.  The easiest intervention is regular use of nasal saline spray.  This prevents drying of the nose tissue.  You will need to have a good relationship with an ear nose and throat doctor (ENT).  They will periodically cauterize bleeding vessels.  There are some advanced treatments that can be helpful for very severe cases.  Laser resurfacing of the lining tissue of the nose is effective and preferred over routine cautery.  In the most severe cases, topical application of Avastin (a chemotherapy drug that prevents blood vessel growth) may be considered.

In 2009, a study was done that showed Tamoxifen (an anti-estrogen medication) had a beneficial effect in reducing the frequency of nose bleed episodes in both men and women.  This treatment was well tolerated.  Tranexamic acid is an oral medication that improves the stability of blood clots.  This medication may be used to reduce nose bleed frequency but reports of increased blood clot and stroke risk have limited enthusiasm for routine use.

Skin Telangiectasias

Generally, no treatment is needed for these.  However, dermatologists may sometimes remove telangiectasias that bleed frequently.  A good foundation or coverup is very effective in hiding these vessels on your face.

Pulmonary Arterio-venous Malformations (AVMs)

Pulmonary AVMs come in two types—large ones that are easily seen on CT scanning of the lungs and small ones that are not able to be easily seen.  Larger AVMs should be treated with embolization (blocking off the blood vessel).  This is accomplished by placing coils, specialized glue or specialized plugs into the abnormal blood vessel.  The smaller AVMs are not able to be embolized and are periodically monitored with CT scanning.  Unfortunately, these abnormal blood vessels often return after treatment.

Care should be taken to determine if pulmonary hypertension is present prior to embolizing larger AVMs.  Our practice is to measure pressures in the pulmonary arteries and then balloon occlude the abnormal blood vessel and then remeasure pressures and flows.

Patients with pulmonary AVMs should receive antibiotics prior to invasive procedures including dental procedures.  This helps prevent brain infections.

Bowel Arterio-venous Malformations (AVMs)

There is general consensus that larger abnormal vessels of the bowels should not be treated unless there is active bleeding from the abnormal vessel.  The indiscriminate embolization of AVMs in the bowel can lead to damage to the bowel.  If there is an acute significant bleeding episode from a bowel AVM, then endoscopic treatments (therapies using a cameral in the bowel) or embolization may be used.

Brain Arterio-venous Malformations (AVMs)

Abnormal blood vessels in the brain are a particularly complicated problem.  These are best managed with a neurosurgeon who is expert in brain blood vessel problems.  Often times these are just monitored over time.  Other times they may be embolized and then surgically removed or treated with radiation therapy.

Brain Aneurysms

When identified these are generally treated either with a catheter-based embolization procedure or brain surgery to remove the aneurysm.

Liver Arterio-venous Malformations (AVMs)

There is clear data in the literature that these abnormal blood vessels should not be treated with embolization.  In the past when this was done, patients had severe liver failure that resulted.  If the abnormal blood vessels progress and lead to liver dysfunction, liver transplantation should be considered.  The medication Avastin has also been used in cases of advanced liver failure due to liver AVMs.

Pulmonary Hypertension

PAH due to HHT is treated with the same medications as other causes of PAH.  An important difference is that we generally avoid blood thinners due to the increased risk of bleeding.

Iron Deficiency Anemia

This is a very common problem in HHT and can lead to fatigue and may increase the stroke risk.  Oral iron pills are a first line but many patients require periodic intravenous iron infusions.  We monitor iron levels in patients a few times a year.

Pregnancy and HHT

Women with HHT who become pregnant are at increased risk of complications and should be managed by a multidisciplinary team with experience in HHT and the complications of HHT.  Spinal and epidural anesthesia should be avoided unless the spinal AVMs have been excluded.

Airline Travel

Flying on an airplane is generally safe but you should check with your physician.

Filed Under: Pulmonary Hypertension Treatments

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