Posted by: gljcm2 | April 10, 2004

HHT: They Aren’t Just Nosebleeds

“They Aren’t ‘JUST NOSEBLEEDS’

MY STORY

I was diagnosed with HHT in my early 20’s after numerous daily nosebleeds that led to anemia. This was not the first time I had seen a Dr. regarding my bleeding. But finally I was not told, “It’s JUST a nosebleed.”

As the name suggests, I had inHerited this condition (from my dad); I had nosebleeds (Hemmoragic); and I had the tiny red spots (Telangiectasias) on my fingers and my lips that blanched when pressed.

For both my Dad and myself, the major symptom was the nosebleeds. While still teaching full time, I experienced up to 8 nosebleeds per day. They might last only a minute, but often could occur for 20 to 30 minutes. My nosebleeds occured at school int he middle of classes; during the night when I was sleeping; in the morning in the shower (change of temperature from cool room to warm water) ; as I stepped out of bed (change of elevation) ; when I became frustrated or rushed; when I bent down toward the floor and even as I sat reading. Sometimes they would occur for no apparent reason.

After 4 laser surgeries in Vancouver, where the final 2 created more problems than they helped, we finally discovered iron infusions in Red Deer, AB. When my blood dropped too low, I would enter hospital for a day, be hooked up to an IV of black iron, and be infused for about 51/2 to 6 hours. The results were fantastic as for the first time in many years, I was able to attain reasonable levels of iron in my blood. As iron carries the oxygen through out bodies, this is very essential.

In 2007, I experienced my first nosebleed which required blood transfusions after passing out from the loss of blood from a single nosebleed. It was my first ambulance trip and was very frightening as I actually felt like I could die after so much blood loss. This was also very frightening as now I was experiencing the more severe problems as my dad had, and he had passed away at 64.

In my dad’s later years, he also experienced internal bleeding from spots in his intestine and bowel and as a result had part of the intestine removed to stop the internal bleeds.

Today I am doing much better having discovered that the humidy in Mexico is truly a preventative for this condition. In the past two years I have created a situation where I can be down there for six months and during that time my blood actually improved. As well, for the first time I can breathe through my nose which is not constantly dried with blood. What a wonderful feeling.

The symptoms of hht vary from one person to the next. As you will see in the following information taken from the hht.org site, hht may affect the nose, lips, mouth, lungs, GI tract, brain, liver or spine. Unfortunately many doctors have never heard of it, or have read only a few lines in a medical text years ago. However, thanks to the HHT Foundation more research, information, and clinics are being made available all the time. There are, in fact, a number of clinics around the world specifically for the diagnosis, treatment and prevention of hht and its symptoms.

The following paragraphs are borrowed from the hht site. If you wish further information, please click on the link at the bottom of the page, and you can learn much more about this condition.

“The following Diagnostic Criteria for HHT were recently published by an international group of HHT experts. The diagnosis of HHT is considered definite if three or more of the following four criteria are present suspected if two of the following four criteria are present 1) Nosebleeds- spontaneous and recurrent 2) Telangiectasia- multiple, at characteristic sites, including lips, oral cavity, fingers and nose. 3) Internal telangiectasia or AVM- lung, brain, GI, liver or spinal 4) Family history-parent, sibling or child with HHT according to these criteria.

No one with HHT has all of the signs and symptoms listed below. One of the very characteristics of HHT is its extreme variability even with a family. A parent may have horrible nosebleeds, but no AVM in an internal organ. Yet, their child may have a nosebleed only rarely but AVMs in one or more internal organ. We also can not predict how likely someone is to have one of the hidden, internal AVMs based on how many nosebleeds or skin telangiectasia they have. In other words, the person with HHT who has infrequent nosebleed is as likely to have an AVM in their lung as the person with HHT who gets severe daily nosebleeds.

Telangiectasia in the nose, along with the nosebleeds they cause, are the most common signs of HHT. About 95% of people with HHT have recurring nosebleeds by the time they reach middle age. The average age at which nosebleeds begin is 12, but they can begin as early as infancy, or as late as adulthood. The nosebleeds can be as infrequent as once or twice a year, or can occur daily. When a nosebleed occurs it can last only seconds, or occasionally hours. The amount of blood lost may be one or two drops, or enough to require a blood transfusion. As with most things that are variable in human beings (i.e. height and shoe size), the majority of people with HHT are in between the two extremes.

Telangictasia in the skin of the hands, face and mouth are also found in about 95% of all people with HHT. These often do not become apparent until the 30s or 40s, however. They appear as small red to purplish spots or distinct areas of delicate, lacy red vessels. In some individuals with HHT they become quite prominent by late adulthood, in others they are subtle. These telangiectasia on the skin and in the mouth can bleed also, but they are less likely to than those in the nose. Both telangiectasia of the skin and nosebleeds have a tendency to become more numerous with increasing age. But with this too, there are many exceptions!

About 25% of those with HHT will develop GI bleeding. Again, it can range from mild to severe. Telangiectasia can be found anywhere in the gastrointestinal system, including the esophagus (swallowing tube), the stomach, the small intestines, and the colon (large intestines). Most commonly, the stomach and the beginning of the small intestines are involved. They look similar to telangiectasia on the skin. Telangiectasia in the GI tract do not cause pain or discomfort. Symptoms of GI bleeding are black or bloody stools and/or anemia. Anemia (low blood count) in turn can cause fatigue, shortness of breath, chest pain or lightheaded feelings.

Approximately 30% of people with HHT have one or more AVM in the lungs (pulmonary AVM or PAVM). AVMs in the lung have a risk to rupture, particularly during pregnancy in women when blood pressure and blood volume tends to increase. This can be life-threatening. However, there are additional concerns about untreated lung AVMs. The capillaries between an artery and vein in the lung have functions in addition to slowing down the blood in an artery before it enters the vein. These capillaries act as a filter for impurities (clots, bacteria, air bubbles) in the blood, before the blood circulates to the brain. Someone with a lung AVM above a certain size is thus at significant risk for stroke (what happens when a clot goes to the brain) or brain abscess (a brain infection that can result from a bacteria getting to the brain). Stoke and brain abscess can also be life-threatening or disabling. Fortunately, lung AVMs are usually easily and completely treatable. The recommended screening and treatment for them is described below.

Brain AVMs are found in about 15% of people with HHT and can also be successfully treated in most cases. They can be life threatening or disabling if they bleed. Since they often do not cause symptoms of warning prior to bleeding, we strongly recommend screening for them in all people with HHT. Spinal AVMs are more rare and can also be removed. They can cause pain in the back over the spine, or loss of feeling or function in an arm or leg.

Liver AVMs can also occur, but their frequency in HHT is not well known. They are unlikely to rupture and most are not currently treated. Large AVMs in the liver sometimes cause heart and liver failure, usually later in life. Heart failure can occur if the heart has been overworked for years, pumping extra blood through the low resistance pathway of an AVM (in this context an AVM is sometimes called a Shunt), as well as through all the normal vessels of the body.” *1
*1.http://www.hht.org/whatis.asp

Take me to HHT.org for more information

J O I N HHT Support — HHT Support through sharing experiences.

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Responses

  1. Glad to see what you wrote. Our family has been greatly impacted by HHT, but thanks to the work being done by the HHT Foundation and the Centers of Excellence worldwide, we have seen remarkable improvement through new treatments. For information, go to http://www.hht.org

    • Thank you Gwen for your comments. I’m glad to see that someone found this little article. Yes, the HHT Foundation has done wonderfuil work. I just returned from Mexico 2 weeks ago where the weather is very humid. Here in Alberta it is extremely dry. The result is that once I come home I am bleeding many times a day. I went to the HHT site last week and was surprised to see Thalidomide is being use or tested for treatment. It looks like there has been some great progress in the past five years. I should probably be going to one of the conferences and learning more. I hope you have a Dr. who works well with your family in the area of HHT. Where I am there was so little known about it, my Dr. is very open to any information I can bring to her. If you’d like to chat sometime, please feel free to contact me through webpress. Have a great evening.
      Linda

  2. Hi there. Nice to read more information on HHT. I am a 52 year female. I started having nosebleeds at 25 years of age. My sister and I attended an HHT Conference in Connecticut and were positively diagnosed with HHT. We have a high incidence on our maternal side, with our mother and her siblings having been greatly affected. Four of five of our siblings are affected. We have attended HHT Centers and I have had laser treatment which lasted for one year. Having been hospitalised several times, one specialist recommended hormone therapy. It stood to reason because as I approached middle age, the bleeds became unmanageable. I am now taking premarin which has proven to be extremely successful. I no longer have daily heavy bleeds, only the occasional one maybe twice a week lasting a minute or two. Hormone replacement therapy was my saviour!

    • Thanks so much for sharing. Interesting they are using hormone therapy. Thats never been suggested to me. My solution has been to spend my winters in a humid Mexico rather than dry Alberta and that has helped immensely. Two years ago I had my first complication when I developed a 9 cm mass in my abdomen which ended up being an issue with my blood vessels. 2 procedures later I was good to go again, but it was a scary time for sure. i noted that many of your family are affected. Our case appears different. my dad had it but not his sister and I had 4 siblings and none of them are affected. so far my kids are fine too. One of the greatest blessings is talking to others who understand….those also affected and the wonderful HHT centers. Thanks again for writing.

    • Hi Janie 3 years ago you replied to an hht post I wrote on my blog. You mentioned hormone therapy was working for you. I am wondering how you are doing now. Is it still working? Has your hht changed? I turned 60 this year and have experienced much more serious bleeds. We are now using a gel in my nose that seems to be helping. I’d love to hear from you. Maybe we have info we can share.
      Linda


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