What is HHT and AVMs?

I have done some research for everyone. I hope this answers some questions everyone might have.





What is hereditary hemorrhagic telangiectasia (HHT)?

HHT is a genetic disorder that causes abnormalities of blood vessels. Most blood vessels in the body of someone with HHT are normal. However, a small percentage of the blood vessels in a person with HHT have a specific type of abnormality.
Blood vessels are the tubes that carry blood around our bodies. There are two types of blood vessels: arteries and veins. Arteries carry blood under high pressure out to all areas of the body after being pumped by the heart. Veins carry blood that should be under low pressure, back to the heart. An artery does not usually connect directly to a vein. Usually there are very small blood vessels called capillaries that connect an artery to a vein.
A person with HHT has a tendency to form blood vessels that lack the capillaries between an artery and vein. This means that arterial blood under high pressure flows directly into a vein without first having to squeeze through the very small capillaries. This place where an artery is connected directly to a vein tends to be a fragile site that can rupture and result in bleeding. We usually call a blood vessel that is abnormal in this way a telangiectasia (tel-AN-jee-eck-TAZE-ee-ya), if it involves small blood vessels. We tend to call it an arteriovenous malformation (AVM) if involves larger blood vessels. So, an AVM might be thought of as a big telangiectasia. The basic abnormality in the blood vessel is the same.
Telangiectases tend to occur at the surface of the body such as the skin and the mucous membrane that lines the nose. AVMs tend to occur in the internal organs of the body. The telangiectases (plural for telangiectasia) and AVMs of HHT occur primarily in the nose, skin of the face, hands, and mouth and the lining of the stomach and intestines (GI tract), lungs, liver and brain. It is not currently known why these abnormal blood vessels tend to occur in certain parts of the body and not others.

What is an arteriovenous malformation (AVM)?
Normally blood flows from the heart through large arteries to all areas of the body. The arteries branch and get smaller until they become a capillary, which is just a single cell thick. The capillary bed is where the blood exchanges oxygen and nutrients with the body tissues and picks up waste. The blood travels from the capillary bed back to the heart through veins. In an AVM, arteries connect directly to veins without a capillary bed in between. This creates a problem called a high-pressure shunt or fistula. Veins are not able to handle the pressure of the blood coming directly from the arteries. The veins stretch and enlarge as they try to accept the extra blood. The weakened blood vessels can rupture and bleed and are also more likely to develop aneurysms. The surrounding normal tissues may be damaged as the AVM “steals” blood from those areas.
There are four types of AVMs:
1. Arteriovenous malformation – abnormal tangle of blood vessels where arteries shunt directly into veins with no intervening capillary bed; high pressure.
2. Cavernoma – abnormal cluster of enlarged capillaries with no significant feeding arteries or veins; low pressure.
3. Venous malformation – abnormal cluster of enlarged veins resembling the spokes of a wheel with no feeding arteries; low pressure, rarely bleed and usually not treated.
4. Capillary telangiectasia – abnormal capillaries with enlarged areas (similar to cavernoma); very low pressure.
AVMs can form anywhere in the body and cause symptoms based on their location.

Lung AVM
Approximately 30-50% of people with HHT have one or more AVM in the lungs (pulmonary AVM or PAVM). AVMs in the lung are at risk of rupture, particularly during pregnancy when blood pressure and blood volume tend to increase. This can lead to life threatening bleeding. In addition people with lung AVMs are at risk for stroke (a clot in the brain blocking off blood flow) or brain abscess (a bacterial brain infection). Stroke and brain abscess can be life threatening or disabling. In the normal lung, the capillaries between an artery and vein act as a filter for impurities (clots, bacteria, air bubbles) in the blood, before the blood circulates to other parts of the body, including the brain. When lung AVMs are present, these particles can pass through the AVM, go to the left side of the heart and then on to the brain or other organs. Fortunately, lung AVMs are usually easily treatable.

Brain AVM
Brain AVMs are found in about 5-20% 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 warning symptoms prior to causing a brain hemorrhage, we 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 AVM
Liver AVMs can also occur, are relatively common, but rarely cause sudden, severe medical complications like lung and brain AVMs can. Liver AVMs almost never bleed and most are not currently treated. Large AVMs in the liver occasionally 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”.

It's been a lil crazy....

Sorry I haven't updated in a long while. It has been crazy here at work since I have been out. I was out 2 days last week and I've been playing catch up since last Thur.

Well all my doc appointments went great last week. We don't have to go back and see the heart doc or my blood doc unless I need them. The heart doc said everything looked great from what an ultra sound could tell us, and that I won't have to see him again unless we find something wrong after he gets here. The blood doc was happy w/ my progress and my counts so I won't have to see him unless I need blood or iron which will hopefully won't be anytime soon.

I got my glucose results back that weren't so good, it was 170. But Dr. Garrison thinks they gave me the wrong test. So I went today to have the test again. I should get those results back w/ in a day or two. I will see Dr. Garrison next week for my 2 wk check.
I go back to Nashville this Wed. to see a lung doc. Hopefully we will pass all her test too. This actually one of the most important exams I will have. They will be looking for AVM's in my lungs. They already know that I have one in my liver (which isn't a big deal) but we are praying I don't have any in my lungs. I can also have them in my spine and I will have a MRI done to see if I have any there. So please remember me in your prayers on Wed.

On a better note.
There are so many other things I need to tell.....were to begin.....

Mackenzie is walking!!! But still wants to craw b/c she knows she can get there faster that way.

Emily is in football homecoming.

Mom and Becca are planning my baby shower for this Sunday at Provo Community Center at 2pm all are invited.

Can't remember if I told you or not but I do have the baby's room cleaned out and is ready to paint. His bedding is almost done (Christon's mom is making it). I didn't go with a "theme" just certain colors, navy blue, tan, burgundy, and hunter green, all plad.

One last thing we still don't have a name......

Well I better get back to work only an hour left to go. Hope everyone has a great week.

12 wks and counting....

Believe it or not I only have 12 wks to go. I haven't gotten nervous yet but I am sure that part will show up soon. I am quite a bit larger than I was a few weeks ago and we (baby and I) are continuing to grow.


I go to the doctor or I should say doctors next week. I was to go back this week but was able to push it back one more week so that I can see all my doctors with in 2 days of each other. I see Dr. Peera (baby's heart doc) 8:30 Tue morning, then from there we will visit w/ Dr. Garrison (OB) at 11 then hopefully find a hotel room and rest some before we go out on the town that night....lol. We will most likely eat and go to bed, b/c we have to be back at the hospital to visit w/ Dr. Stein (my hematologist) at 8:30 Wed morning. After this appointment I will start going every 2 wks and Christon isn't very happy about it at all and I don't really understand why. He knew that it would eventually have to happen but I think it has snuck up on him also w/ gas prices up and having a gas hog to drive he is a lil worried about the money, which I am too. Not sure yet what I am going to do when I have to start going every week. With his schedule he won't be able to go with me and I hate asking anyone else to go, but I will cross that bridge when it gets here.

We have started on his room. Christon's mom and dad came over on Sunday and we got it cleaned out. Before we started you couldn't even see the floor let alone walk through it. His mom has started on making the bedding and she is picking up the paint sometime this week. I hope to get it painted in the next week or two.

Mom and Becca are planning my baby shower for October 5th at 2pm at the Provo Community Center. We are registered at Target and Wal-Mart. I hope everyone can attend. This weekend is Friend and Family Day at Belmont and Leigh is coming in. I am excited to compare bellies w/ her once more. Hopefully this time we can get some pics made so I can share. Heaven's Gaits and Hell's flames has been rescheduled for Oct 12th, 13th, and 14th just wanted to let everyone know.

Also I want to think everyone for praying for my grandmother. She is still in the hospital but doing good. She had a cardiac cath done Monday. She had one 100% blockage and one 99% blockage. They wanted to do surgery but with her other health problems they were afraid healing would be a problem. So they put 2 stints in and we are praying for the best. So if you will please continue to pray for her and our family.

I want to give you a name up date.....WE STILL DON'T HAVE ONE. When he came up with Riley I said no then I started to like well then he changes his mind. Now he is stuck on Memphis, and that isn't going to happen at all. So we are still looking and taking suggestions.

Hope everyone has a great rest of the week.

Way COOL new web site.....

I have found a way cool web sit. You have to try this it is SO much fun.

http://www.yearbookyourself.com/

You make your year book pics. Here are some that I have made:

Just thought I would share that with everyone.

Also an update on mamaw. She was to have her heart cath the morning but they weren't able to do it b/c something was wrong w/ her kidney's. They are going to wait and make sure they are working correctly before doing the cath. Please remember her and papaw in your prayers.

Thank yous, prayer needed and sorry its been long...

I just had a few minutes to stop in and update. There are only a few things I have time to share with everyone.

I want say thank you for all the names that have been sent to me. I think some of you all (I won't say any names LAURIE...lol) have had a lot of fun with this. We are still trying to find one that we agree on so keep them coming.

My grandmother Anna Rose (mom's mom) is in the hospital. They took her yesterday morning after she had fallen. They think she has had a heart attack. They weren't 100% sure as of last night when I last talked to them. They were going to run more test. They have yet to tell Mamaw or Papaw what is going on. I think they are waiting until they are 100% sure. So please everyone remember her and papaw in your prayers.

I need to get back to work. Hope everyone has great rest of the day. I will update on Mamaw when I learn more.

Name update....

Here are some that we have liked:

1. Kenton Levi
2. Kenton David
3. Ethan - we need another name with that
4. Riley - this is a name that Christon just loves but I'm just not sure, we also need another name with this one.
5. Winston - we both like

When we first found out that we were having a boy Christon came up with a few names him self:

1. Earson
2. Eldabrock
3. Jegs
4. Summit
5. Jeep

After laughing my butt off at him for even thinking I would go for any of these I had to ask were he got the first one from. It is a brand of a camshaft. I told him he was crazy if he thought that my child was not going to be named after a motor part. The second one is a name brand of a motor, third and forth is are places he orders motor parts from, and the fifth well I hope everyone knows what Jeep is also there is a guy named Jeep Vanworner that races in the Lucas Oil Late model Series (why would you do that to a kid). To explain his thinking for the ones that don't know my husband, he is what they call a gear head. He loves anything to do with motors, cars, you name it.

Thanks for all your help. I hope I have given you all some idea of what we are looking and NOT looking for...LOL.

Needing some help....

Ok I truely believe this kid is goin to come home with out a name. We can't decide on anything. We have choosen a few names but now Christon has changed his mind on some of them. I am about ready to kill him.

I was hopeing you all could help. I need some ideas. Laurie has been emailing me lists of names and I was hopeing you all could do the same. Just send a comment if you don't mind.

Thank you and remember it is a boy.

Sorry...

Sorry it has been so long updating. With the short week it has been crazy here at the office.

I hope everyone had a great labor day weekend. We didn't do much b/c it was so hot out.

Saturday Christon's dad put his car in the car show at Greenville. They left about 7:30 and Bubby, lil mac and I met them over there later that morning. It was SOOOO hot. We did stay for a while. We looked at all the cars, I think there were about 180 there. Me, lil mac, Christon's mom and aunt went into a store so they could look around and cool off. After that I told Bubby I was ready to go, I knew if I was miserabley hot lil mac was too. So we went on home. I hung out there for a while and watched tv w/ Bubby while lil mac played. Let me tell ya watching tv w/ Bubby is not fun. He puts it on a channel long enough for you to get interested in the show and then turns it. If you have ever rode in a car with Jonna Lee she is the same way with the radio, he is as bad (if not worse) as she is. Laurie you know what I am talking about. Well back to my story. He got ready to go to the river and helped me get her bags together for her to stay the night with me. On the way home I called Christon and they were still at the car show so I stopped at Jason and Tiff's to visit w/ them. On the way there I noticed lil mac wasn't happy at all and when I got her out of her car seat I knew why. She had messed her diaper and it was all the way up her back. It was on the car seat, her clothes, and now me. I get her in the house and Tiff helps me get her cleaned up. We let the girls play while we caught up with each other. She said that they had had a rough day. Earlier while she was cleaning Emma was pushing one of the kitchen chairs around following her and fell, as she fell the chair went with her and landed on top of her giving her a pretty nice brus on her fore head.

Then while Emma was in the laudary room w/ Tiff she cut her leg on something. Tiff said she's afraid someone is going to think she beats her kids. I told her no one will think that they know that accidents happen and kids will be kids. Then Jerry (Tiff's dad) came in. He had walked over just to see what was going on. Hannah ended up walking back home w/ him. Tiff wanted to get Emma asleep so I went on to Christon's mom and dad's. We ate dinner and then went outside for a bit. Lil mac LOVES it outside. We went and swung in the new gazeebo his mom had gotten.

Isn't she just adorable.

We left there about 9pm and I knew she was goin to go to sleep on the way home and she had to have a bath. She had sun screen on and where we had been outside most of the day she had to have one. So when getting her out of the car seat I woke her up. We got in and I give her a bath (which she was not happy about) and I figured she would go back to sleep. So I jump in the shower hopeing Christon will have her asleep when I get out. NO they are both wide awake playing. I was ready to go to bed but she wasn't. So I just let her play, there was no since in fighting w/ her. When she started to get fussy I fixed her a bottle and she went to sleep before finishing it and we both went to bed.

I have to get some stuff done today before I leave and I have to leave early today. I am having my glucose test done today and doing some shopping for Hannah's birthday on Saturday. So I will update so. Everyone have a great day.