Update 16/6

Feeling very tired today.

Got my last few blood tests back. The thyroid function is back to normal (for now).

My blood count is good - but the iron levels are 1/3rd below minimum.

Have to get back onto the iron supplements to try to build up from the blood I lost last week. It's interesting how the body works. My doctor said I did into the iron stores first, then into the red cells second ... which then causes anemia. So I'm just a fraction away from that.

My BP is down from last weeks record (now 150/90) but that's still too high. The bleeding has been less - not so long now, which is good. Much happier with that, but still annoying when it happens.

I go into the city on 9/7 to see another ENT specialist.

HHT On House

I finally found the links - but missed the episode when it aired, when HHT featured on HOUSE (tv show, on TEN in Australia).

Season 3, ep 16 http://www.housemd-guide.com/season3/316secret.php
&

http://www.televisionwithoutpity.com/show/house/top_secret.php?page=13

The patient of the week

• Ex-marine who thinks he has gulf war syndrome because he has unexplained fatigue, rashes and joint pain. This patient turns up in a dream House has before the patient is given him even though House has no memory of ever having seen him before.

The mis-diagnoses

• Some of the things they suspect, test for or try to treat include, autoimmune diseases, lymphoma, tumors in the brain, infections,

The final diagnosis

• Hereditary Hemorrhagic Telangiectasia, a genetic disease that destroys the patients capillaries. The problems caused by this can be fixed with a few surgeries

UK web Site - HHT

I found the following web site, out of interest...

http://www.hht.me.uk/

click on the DISCUSSION section for interesting chit chat

Web Site - HHT

I became a member of the HHT Foundation in the states.

They have a number of really useful pages (for me).

I've exchange a few emails with them too when things got tough & they came back with some really good advice. So I really appreciate their input too!

http://www.hht.org/living-with-hht/

This was a short extract about what it is...

Hereditary Hemorrhagic Telangiectasia (HHT) is a genetic disorder of the blood vessels, which affects approximately 1 in 5,000 people. It affects males and females from all racial and ethnic groups. The disorder is also sometimes referred to as Osler-Weber-Rendu (OWR) after several doctors who studied HHT about 100 years ago. In 1896 Dr. Rendu first described HHT as a hereditary disorder involving nosebleeds and characteristic red spots that was distinctly different from hemophilia. Before Dr. Rendu’s work, doctors did not understand that individuals with what we now call HHT have abnormalities of their blood vessels, not a clotting problem in the blood itself. Drs. Weber and Osler reported on additional features of HHT in the early 1900s. More than a hundred years later, HHT is still often misdiagnosed in affected individuals and many doctors do not understand all of its manifestations.

http://www.hht.org/about-hht/

What is 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.

some links: laser therapy

I found a couple of links about the argon & c02 lasers. They are used it seems in dermatology & cancer treatment which is good.

http://dermnetnz.org/procedures/lasers.html

The above article says ...

Vascular lesions

Lasers have been used successfully to treat a variety of vascular lesions including superficial vascular malformations (port-wine stains), facial telangiectases, haemangiomas, pyogenic granulomas, Kaposi sarcoma and poikiloderma of Civatte. Lasers that have been used to treat these conditions include argon, APTD, KTP, krypton, copper vapour, copper bromide, pulsed dye lasers and Nd:YAG. Argon (CW) causes a high degree of non-specific thermal injury and scarring and is now largely replaced by yellow-light quasi-CW and pulsed laser therapies.

so that seems to be appropriate for the telangiectases that I have...

I had been prep'd once before when I had treatment in Westmead Private Hospital & thought it was quite funny when they prepared my body with wet towels & the like to minimise the risk of flamability when the laser was in use.... Maybe I imagined it - but this article reminded me of that! (refer "laser safety section"!!!)

The other articles are also interesting, btw.

http://www.massey.vcu.edu/cancer/?pid=1532

http://cancer.stanford.edu/information/cancerTreatment/methods/laser/

Friday the 13th

Ahh. there must have been something in the date!

I woke up & was not feeling very well at all. Actually, Karen had to wake me up as I had another bad night with bleeding.

I had a visit today from a client who strongly encouraged me to see the doctor.

I couldnt get into my normal doctor or the specialist (who is away for 3 or 4 weeks at the moment) so we chose a new doctor in Penrith.

She seemed quite nice.

I've also had a little bit of bleeding from 2 new external spots which is interesting - one on my arm (but that seems to have gone now) and one from a spot under a small mole on my side. That one will need to be followed up urgently.

After reviewing my medical history (shortened condensed version..) she took my blood pressure & wanted to know what was really going on.

It was sky high

I've never been to a doctor (never been able to get in to see a doctor) so close to a big bleed & the blood pressure was way up. I've never had a reading that high before! (170/120).

The doctor contacted another ENT who fit me in straight away.

That was really good. I'm always reluctant to go to the doctors & specialists as you are always seeming to spend so much money & get back so little from medicare and the health funds....

That was a good appointment, as I had visited him some 12 years earlier also with a nose bleeding problem. Except we didnt know that it was a problem back then - just a one-off.

So it is interesting to start to get a timeline of events - I hadn't realised it was a problem for me back in 1996. I think from memory it would have just been a couple of really bad bleeds at the time; nothing to worry about anyway.

It was good though as the specialist wanted to know what he'd missed back then as compared with the condition now, which of course was much more severe. So I really appreciated that.

Come to think of it, the above happened on the Thursday (not the Friday). my memory must be going too!

Friday I felt very off - very low on the energy quota & quite pale.

The ENT gave me a couple of different ways of managing the severe bleeds which was really a good thing.

He also arranged for a referral to an ENT who works out of St Vincents Private Hospital in Sydney. I;m not too keen about the travel; but if it is a solution, it is worth looking into.

I think its called argon laser therapy - apparently much better than C02 and the general electrocautery that I have done under a general anesthetic.

I think the appointment is in about 3 weeks; so that's not such a long wait.

We had a discussion about emergency treatment. One thing the specialist had said (or scolded me?) is that instead of waiting 1+ hours to seek treatment, I have to reduce the window down to 20 minutes. Up to now I've been "managing" the condition between an upper & lower parameter - so I dont bother anyone unless I go outside the parameters... Recently I've felt that I have not been coping with the blood loss too well, so I guess this is a good thing to narow the time down significantly before seeking treatment.

I guess I am paranoid about calling an ambulance - but the last few times they have been absolutely wonderful. It's when you get to hospital that things seem to be left wanting....

My local hospital is Nepean. But after discussion with the ENT he suggested (strongly) that if I need to make it to a hospital Westmead would be the better option as they have ENT surgeons after hours; where I dont think Nepean does. Based on my past experience there, I think that is correct.

What I'm getting from the discussion is that I have to seek treatment earlier so I can bounce back quicker - by leaving it until I'm really sick, that's not a good thing.

So it's good to keep things (or get things) back into perspective.

This weekend I went to the shops for some groceries. That was a mistake as I was absolutely exhausted for the rest of the day. Maybe a bit to optimistic.

I slept most of today & also took it very easy (Sunday).

Tomorrow I go off to my normal doctor & hope to have a good discussion on where I am at.

I had a blood test a couple of weeks ago as the thyroid function was low (?) - probably as a result of the bleeding.

The ENT recommended a further series of tests on Friday to see how my body was coping with the bleeding, so the results should also be back tomorrow.

I get so use to the blood tests, they dont really worry me any more. However, Friday's test I went sweaty & feint. I guess that was telling me too that I have lost a lot of blood in the last week. Sometimes I need something like that to poke me (literally) to know I need to slow down.

Queens Birthday Weekend

I've had a good 6 week period now without much bleeding.

About 2 weeks ago I felt like I had a slight cold or flu. Nothing much to worry me.

Then on the long weekend I was "struck down" with a sever bout of chronic fatigue & tiredness. I slept most of the weekend away.

On Queens birthday Monday - about 3am in the morning, I was woken up with the severe bleeding again. Not nice. I think I detest that the most - especially when I was having a sound sleep.

Unfortunately the bleeding carried on into the following days - Tuesday, Wed & Thurs as well - all early morning & big (> 40 mins - 1.5 hours).

You get used to it after a while...