More is Not always Better

I guess the reason I am writing all this is because being symptomatic does not always mean an increase in meds is needed. More is not always better. In most centers across the country clinicians are no longer doing routine RHC's annually since in most cases they yield no significant data unless you as a patient are showing signs for concern. They are still the golden rule for diagnosis but echo measures seem to be quite capable as well as 6 minute walk distances and other PFT’s as needed for updates to know where the patient is – is the progression progressing, is it stable. Titrating a patient’s medicine is based on how they feel which is a reasonable thing to do as many clinicians adjust medicines based on symptoms of the patient. This is the art of clinical practice where knowing a patient and the limitations of tests and finding the balance that makes patients feel better is the measure of a good clinician.

PHers and especially “newbies” need to know that sometimes all symptoms are not directly related to pulmonary arterial hypertension; and that being on too much Flolan or other PAH therapy, may look or feel exactly like being on too little. The effect of too much drug causes a high output cardiac failure. This is sometimes only detectable on right heart catheterization and I tend to have a history of sometimes not knowing what my body is telling me. That’s why I trust my doctor and ask him lots of questions.

I want to back track a bit. I hadn’t been feeling well – having trouble breathing, got a little sob when I would move about – and that of course is short of breath, so I called my doctor. He scheduled an appointment to see a cardiologist on Wednesday and then an appointment for a RHC on Thursday – unless I could get in earlier. A RHC was pretty much standard operating procedure for me as I’ve had a number of them. Probably 15 or more over the past five plus years. I even remember my first one. I was wheeled into the OR on a gurney and then wheeled into the ICU when they were done. I do remember feeling out of sorts as the unknown is always scary and when they mention an invasive procedure and the heart in one breath... It is just out right scary. After that first one, I know what it is about, I now walk into the OR, have the procedure and walk out and go home. Well, that’s the plan.

The weekend arrived and departed, Monday came and went but come Tuesday morning I got a call from my nurse who said there is a bed available and to come to Cleveland as soon as I could but don’t rush – I had time to shower… I called Tom and within less than an hour we were on our way. I am a “good scout”; I have learned to be prepared. All my “just in case stuff” was loaded into an overnight – actually is was my old computer case – and my son-in-law recently gave me his old lap top when he bought a new one – and that is now on my “just in case” list, it came with us and we were good to go.

The car was loaded up – all that just in case stuff. My back up medicine (Flolan) mixed and ready to change at a moments notice, extra ice packs (plus the large one to keep those frozen), the usual stuff in my “six pack” cooler – the Hickman line change, back up batteries, the spare pump, back up supplies if I needed to clean or change my site… quite the array of goodies. And that was just the cooler. I also need extra oxygen tanks Just In Case something happens to the one I am using, like running out of air. When I walk out the door the items I carry would be similar to what a “normal” person uses for a week long trip… Whether I’m gone for a day or a week, I take the same amount – well except for clothing, THEN we would need another case to lug around. I always carry and extra set of undies when I go anywhere and I am so glad I do. I have had to use that JIC (just in case) set several times when my stay ends up being more than the trip over, you know, and added day or two. *;* Oh, must not forget the Revatio/Viagra another JIC.

We got into the car and Tom noticed the gas was low… well go figure… he also mentioned that we should take a quick trip through the car wash… since spring has sprung I had those little furry things falling from the tree onto my car (along with some bird poop droppings—they’re baaack) and there was some left over snowy/salt splattering on the side of the car. Tom started the car and there was a POP sound and after several tries, the car finally started. This never happened before – is this an omen… We went to the car wash and he chose the one with the extra under carriage wash setting. We drive in ~ and stop. The wash starts but only on one side… we both looked at each other with big grins. Luckily the owner was right there. So we drove around again – the owner gave us the money, pushed the button and off we went again. Same thing happened, this time Tom and I really laughed and said how the undercarriage would be really clean. The owner came, pushed a few buttons and mentally I’m sure kicked a few things and told us to go around again. This time we went into a different stall and low and behold, it happened again… the owner came, checked out this and did that and finally it worked and the car had a nice warm bath/shower. The owner stopped us as we left and said we should have the cleanest undercarriage going…. *;*

We got on the road, had a stop sign and the car shut off…. Next stop light, it shut off again. A message came on the dash to service engine soon. Tom said that’s it we’re going to the car dealer. Luckily it was only a mile up the road. I saw guys I hadn’t seen in years (remember, I don’t get out much), it was like a reunion. The owner came through the service department, the manager of the service department was the same from years back, one of the office “girls” came out for a question to the manager and I knew her too. It was great seeing people I hadn’t seen in 5 years or more. Even under the circumstances, I was made happy. They took us in right away knowing I had to be in Cleveland, put the car on “that” machine that reads it’s insides (reminds me of that gizmo on Star Trek that “doc” always used); did a little of this and a little of that; reset the whatever they are; took it for a run and said we were good to go. Guess what, they think it was the “shower” got some wires wet and the car acted up. Ask me if I’m surprised….

We made it to the Cleveland Clinic, went to registration, got right in instead of the usual delay, my room was ready, I was hungry so we stopped at their food court for a “to go”, and off we went. I was to go to H22. Now I know H means hospital and I am usually on the ninth floor as they specialize in heart care and that is usually where PH patients are sent… BUT no, when we followed the signs it said Intensive Care Unit – Heart Failure. Say What!!! Why was I going to ICU… then I remembered, when you have a swan in your neck and you don’t go through SOP (standard operating procedure) you have to be in ICU – their policy. I was told, we would get it done and I would be on my way home again - by early evening. May not need that lap top to communicate with my phriends after all…

There was a staff meeting, someone else needed a procedure and about 5 ish the doctor came in did the normal stuff, painted my neck, put drapes all over me and the fun began. A poke here, you will feel pressure there, can you feel that (well dah), another prick, now that’s better. I mentioned I could feel it in my arm – wrong turn down the tunnel… He had trouble getting past the Hickman line, I finally felt a flutter in my heart and told him he was there… felt another flutter, my legs started to tremble, I was getting nervous and I try so hard to be a good trooper but the trembling wouldn’t stop… They then brought in an x-ray machine to make sure it was in line and not hung up on the Hickman. I’m thinking hmmmmm…. They start doing their readings and the next thing I know is the PH Fellow is called…. I’m thinking again hmmmmm. My legs are really shaking now but I was able to keep my upper body from shaking and I guess when you have something in your heart (besides the Hickman) and there is a person on the other end of this catheter moving it, you tend to do that – you don’t want to move and upset the apple cart. Although my “mean” pressure was in the low 50’s, apparently my cardiac index and output was waaaay off. I was at a toxic level of Flolan – this is not good. Actually this is very bad… definition of toxic – poisonous, deadly, lethal, noxious – just to name a few. I was told I had to come down on Flolan and come down now. They would do the titration now and continue through the evening if necessary so I could go home by morning. Didn’t really like that thought but it sounded like a plan. They would titrate me down half a nannie every hour on the hour along with a blood draw (this would give them the cardiac index and output) and also my BP reading. I hate those automatic blood pressure readings – they are too tight and really hurt (I still have the bruise). It was 169/95 and my normal is 110/60. After the first one, they put it on my lower arm and that felt a little better. I then got a child’s BP cuff and that was much better. I went down to the 150’s and once I made it to 110 and another time to 115 but for some reason it went back up to the 150’s or 160’s…

I hooked up the lap top and started chatting off and on with several of my phriends – they certainly help me stay sane. I didn’t like what I knew was going to happen and was happening. We chatted between “sets”.

Did I ever mention that my doctor is going to change Murphy’s Law to Merlie’s Law – because of what I have been through over the years. It really is appropriate… If something is going to go wrong, it will and it will happen to me. There was a debate or discussion about the titration. ICU’s policy is no titration after 7 p.m., they had to call my doctor and he mentioned that since we were coming down it was different. ICU’s thought is, most of the doctors have gone home for the evening and couldn’t be reached in a timely manner if needed. It was decided since the Fellows were close by and on call, the procedure would begin. About 7 o’clock the first blood draw and the first ½ nannie down…. An hour later, another ½ nannie down, a blood draw and BP reading. The results of the first blood draw showed that I had come down to 2.9 from 3.8. The goal was to reach 2.5 to 2.8. That would be a safe level. The next set showed I went up, the next set showed I went up again 3.86 then to 4.38. I was going the wrong way and of course getting a little anxious, this I am sure did not help my situation. Eleven o’clock came and it was time to take my Revatio – another BP reading (going up anyone), a blood draw (all they had to do was turn the spicket from my Swan) and ½ nannie down on the Flolan. That reading showed I went way up and the Fellows were called (5.05 to 7.29). No more Revatio. Apparently, the Revatio and the Flolan were creating a major problem and my index was so high they were very concerned. I didn’t know any better so I was just being me. The doctor asked me some questions and said she would be close by “Just In Case”. Oh how I love that phrase. My index went up, the index went down and then to be different, it went up again. At 3 o’clock, the Fellows were called again and this time they played with the Swan, they also did a wedge. The x-ray machine was brought in and “pictures” were ‘took’. They again wanted to make sure the catheter had not moved or may have slipped out of place… like where would it go. They stopped going down on the nannies but they still took the blood draw. By 6 a.m. I was at 6.57 – not good.

I’m sure I was the buzz of the floor by Wednesday. Those nurses who came back on duty asked what I was still doing there… We joked about it often. It was almost a betting game – would my index go up or down. We tried having me lay still for at least a half hour before each set – we tried me sitting up to see if that would be different – it was but then I would yo yo again. My PH nurse came for a visit, my doctor also came – and the Fellows also stopped in off and on during the day. It was decided that my body needed a rest and also gave us all time to go over all those figures, but we started titrating down again. The regimen was to be started again but only the draws would be every 2 hours instead of every hour. BP was still every hour and I was so exhausted, I slept through it all, I didn’t even wake up for the blood draws or the BP reading until 6 a.m. on Thursday. The first thing I asked was what were the readings… One was 6.8 and the next was 4.3. Well at least now I am heading in the right direction. I was starting to get symptomatic, having trouble breathing, was I going down too low… I even had trouble talking… I really didn’t want to come down any more on the Flolan. My doctor came and said the last reading was 4.3 and I still needed to come down… they had just finished a reading that he hadn’t seen and it was 2.8 so I told him – he said if that’s the case, I could go home. It was and I did…. Yippy ~ Tom was called and came and got me.

So now, I am off Viagra/Revatio which I had been on for about 5 years and my understanding this may be a temporary thing. I am down on my Flolan and I’m not sure if this will be a temporary thing or not. I am still symptomatic and at this point we’re not sure if it could be a “panic” attack or if some other little disease may be acting up. Panic attack – stressed out – who me – why would we think that!! Last fall I was tested for asthma and it seems I have that, never did before – don’t like those meds but hey, who am I – just the patient. Could also be allergies, it’s that time of year and since PH I have always had difficulty with the seasonal changes in nature. Winter to Spring, Spring to Summer to Summer to Summer (very difficult) and Summer to Fall. I don’t seem to have too much trouble from Fall to Winter. I have always had trouble with humidity and I guess that’s not too common with us PHers but it does happen. I also wonder if it could be the barometric pressure – Mother Nature is such a wonderful gal and we must not forget Merlie’s Law.

It has been a week now since this happened. I am more tired than I was before but I am sleeping well through the night and even have an afternoon nap. My fingers don’t feel like stuffed sausage anymore, my eczema seems to be clearing up – so I’m not going to complain. If my heart is in good working order, I’m good to go.

April 25, 2008

I guess I should have mentioned in my "little" epistle that one of the reasons I asked for an increase in my Flolan over the years is that I was not having side effects -- no jaw pain, I did have some flushing, feet didn't hurt... I was told, or thought that if I didn't have jaw pain and the other side effects then I didn't have enough Flolan besides, I was symptomatic. Just made sense to me; no jaw pain, having trouble breathing, getting sob when you walk from one room to the next -- need more meds. You know that old saying, no pain, no gain. Well, we are all unique in this phamily and as I stated, we all react differently to our PH therapy. We are still trying to figure me out. Is it asthma, could it be allergies or is it something else altogether different. Stay tuned for any updates *;*

April Fool's Day -- I think not

Today started off pretty typical for a Tuesday. Groceries were delivered around 8 a.m., I can't believe I was just getting up when they came. I put what needed put away and checked my "mail". Not too much this morning but I am so far behind in getting it either sent on or deleted, that I should be shackled to this machine to get it done. It's almost like a chore and I so love this little box that brings so much joy into my life. I chat with phriends, I e-mail so many, I hunt and search on the world wide web. I would be lost with out it.

I decided to change my site -- that usually takes some time and I want to start putting on notes about the meetings. Well, naturally, I ran into some complications so I called tech support, they helped and I was on my way again to changing things when the doorbell rang. It was the cable guy. *;* I had ordered a phone line through my cable company. It's only $2 more than the "regular" phone company -- well for 6 months anyway -- and I would have long distance, caller ID and so much more. Yesterday, I only had local calling. (See below - Incoming Call) I was excited because now I can call anyone, anywhere (in the continental USofA) and any time I so choose. What more could I ask for. I do have to give up my cell phone but I think in the long run it will be worth it.

The line was installed or reconnected or whatever it is they do and low and behold, the phone rang. My phone showed it was Tom calling. Wow, I could actually see who was calling me. I was like a kid at Christmas time -- a new toy. We chatted a bit, he said he would be a little late getting here today but that's fine...

The cable guy left and I went back to working on my site. Frustration, frustration, frustration. I decided I needed a break. Tom came, we had lunch and started watching TV -- pretty much our daily routine. But I am on the PHA Help Line this week so I had a few calls and of course, I usually end up at my computer helping someone find a doctor or maybe a support group; answering questions about PH or just listening to their fear and concern. This really is a dastardly disease and extremely scary when you first find out about it.

Tom left for the day and I thought I would try out my new long distance service... I called one friend in another town and she wasn't home, I called another friend and she also wasn't home. Bummer, my new toy worked but I couldn't share it with anyone right now. Then the phone rang, I figured it was a return call but no, it was a contractors service calling because I had signed up for a "prize" and although I didn't win, I got a courtesy call. She was very polite so I listened to her spiel. When she realized that I wouldn't need their services in the near future, she said "I noticed you answered the phone with something about a help line". Wow, I was surprised and a little amazed that she noticed that. A caring person. She asked me what it was about. I explained what Pulmonary Hypertension is and what the Help Line is about and a little about PHA. It almost seemed like she may know someone with PH symptoms. She asked could she have someone call me to inquire. I said sure, that's what the line is about. Patients talking with those who are either patients or someone who knows someone, a caregiver or family member or maybe a friend. She said God Bless you for doing this, people need people to care. Wow, kinda made my day. Come to think of it, she didn't even use the 800 number...

I think this is the first April Fool's Day that no one played a prank or said a joke or something. It was a very positive day and I don't get many of those. Maybe spring is coming.

Spring Has Sprung

Spring has sprung, the grass has riz, I wonder where the daisies is.

This is something I remember from way back when -- there is more to it but for right now, that will do. As far as the grass has riz, well. At least I can see it now. The snow is mostly gone just patches of it left here and there and what we were suppose to get today DIDN'T HAPPEN. Yippy, Skippy... I am tired of being cold, I am tired of seeing cold -- it has been an exceptionally long winter and I am SICK AND TIRED OF IT!!!!!

The beginning of this month, the 8th to be exact, I was to have my first Support Group Meeting of the year. We intentionally skipped January because it is January and who knows what snow might befall us. Well, guess what, January was pretty decent but come the 7th of March there was this MAJOR snow warning. Suppose to be the biggest snow fall of the winter season. I had to call all those who responded they would come to the meeting and tell them it was postponed. I sent an e-mail to those I have e-mail addy's for and posted on the site. I had to call the Library where we meet to see what would be the next available date we could have a meeting, that ended up being the 22nd of March. Sounded good, the only problem with that date is, it was the day before Easter and some folks had plans.

Well, back to that major snowstorm. It was and I am soooo grateful that I postponed the meeting. The Cleveland area got about 20" of snow. It was a record breaker and the further west you went in Ohio, the more snow they got. Some of the members wrote and told me they had 4' snow drifts in their yard. I had mentioned to some that if I had to go by snowshoe, I was going to the meeting but when Friday came and I saw how dangerous it could be for others to travel, it was a no brainer -- we could have the meeting another day.

Then comes time for the new date. You guessed it, another snow storm only 6" this time. I had people call and ask if it would be postponed again but I had checked with the Cleveland area weather and they said by 10 a.m. the roads would be dry as the sun would melt most of the snow.

We had our meeting and it was good to see everyone again. I started off by saying, Happy New Year, Happy Ground Hog Day, Happy Valentine's Day and Happy St. Patrick's Day. So much had happened since we last met in December, I had 4 pages of notes. I expected 42 but had some cancellations at the last minute. Those that had colds didn't come as they didn't want to spread any germs around. I am thankful for that. We PHers have to be careful, we tend to "catch" things very easily.

This year we have a clean up crew and they did a great job. We have to wipe down the tables, vacuum the carpet and put out the trash. Does that sound like a sound.... :o)

Now I'll have to get ready for our May meeting *;*

Have a great day.

Another Super Tuesday coming right up

I'm not sure if I want to say it's our right and privilege to vote or it's our privilege and right to vote. But either way, we need to VOTE. Some of our votes will wipe out someone else's vote but at least we are voting and have that privilege to do so. This is a great country many don't realize how fortunate we are.

Some will moan and groan about this is the way it should be, some will moan and groan that it should be a different way. It really doesn't matter how you moan and groan unless you VOTE and let your respective politicians know what you want and how you feel about a "situation" for lack of a better word; they will never know unless you tell them. And you need to tell them when they are elected. How do you feel about this legislation, how do you feel about a different one. They need to know, and we need to tell them.

Tomorrow is a way to let them know. Ohio, Texas and Vermont have their primaries and that will be a start to let them know WHO you want to represent you. The main election will be November 4th. Now that's going to be a day of infamy. I'm not sure if I'm excited about that or totally scared... there is a lot going on in this country right now. Moaners and groaners are in action and are being heard but are they listening?

We of course all want someone who cares, someone who will listen is also a key element; someone who will do what we want or need them to do. Someone who will guide us in the right direction; and when I say us, I also mean this country. Years ago I read a book and I think it was titled: P. S. You're Not Listening. It was about a special needs teacher who taught mentally challenged students. The main jist of the story is/was, sometimes we hear but we are not listening.... Good point. I try very hard to remember that.

Well, get out there and vote. Vote for your choice. Vote for what you believe is right. There are many countries that do not have this choice. That's what is so great about this country we don't have to agree and we can disagree with each other but we do need to VOTE. Let your voice be heard, chose some who you feel will listen.... You are a registered voter, right.

Sometime in the near future, I will write with what I want them, the politicians, to hear -- oops, listen to.

Why do we need leap year?

Just happened to think about what today is... It's leap year, we should all be jumping for joy.

The Gregorian calendar, which now serves as the standard calendar for civil use throughout the world, has both common years and leap years. A common year has 365 days and a leap year 366 days, with the extra, or intercalary day ~ designated as February 29. A leap year occurs every four years to help synchronize the calendar year with the solar year, or the length of time it takes the earth to complete its orbit about the sun, which is about 365¼ days.

The length of the solar year, however, is slightly less than 365¼ days—by about 11 minutes and 14 seconds. To compensate for this discrepancy, the leap year is omitted three times every four hundred years.

In other words, a century year cannot be a leap year unless it is divisible by 400. Thus 1700, 1800, and 1900 were not leap years, but 1600, 2000, and 2400 are leap years.

In Coming Call

This just tickles my funny bone every time I think of it. In Coming Call - gimme a break....

Quite a while back the battery on my portable phone bit the dust. So on my monthly toilet paper run to Wal-Mart, I added a battery to my list. Upon checking those out, I realized it was just as cheap or cheaper to buy a whole new phone. Simple math... a battery cost as much or more than a new phone. I love the concept of supply and demand, it really works. So to the new phone section I went. I'm thinking well, if I have to get a new phone I might as well get one that lets me know who is calling. My friends who have those always rant and rave about that feature. If they so choose, they don't have to answer the phone and on the other plus side, they know who is calling or at least the phone number being called from -- did that make sense. The prices were very reasonable and I bought one in the "middle" price range. It had buttons that do this and buttons that do that, I was quite impressed.

When I got home, I read the directions and it says you have to contact your local phone company to start the "service". That should have been a dead give-a-way so I called the phone company. I am told in order to have that service of knowing who will be calling, it will be an additional $8 + per month on my phone bill. I say "I don't think so." I already have my long distance disconnected to save on that expense, why would I add something that I really don't need. So now when my super duper phone rings with all those added buttons on it, I have a message that says 'IN COMING CALL' well dah, what else would it be. :o)

What is Pulmonary Hypertension

This is a little something I found on the web a while back, sorry I can't give the credit where it is due but I didn't copy as to where I got it from... must have had a PH moment. A more updated version of what we are use to seeing.

What is pulmonary hypertension?

Pulmonary hypertension is a rare lung disorder in which the arteries that carry blood from the heart to the lungs become narrowed, making it difficult for blood to flow through the vessels. As a result, the blood pressure in these arteries -- called pulmonary arteries -- rises far above normal levels. This abnormally high pressure strains the right ventricle of the heart, causing it to expand in size. Overworked and enlarged, the right ventricle gradually becomes weaker and loses its ability to pump enough blood to the lungs. This could lead to the development of right heart failure.

Pulmonary hypertension occurs in individuals of all ages, races, and ethnic backgrounds although it is much more common in young adults and is approximately twice as common in women as in men.

Why do the pulmonary arteries narrow?

Scientists believe that the process starts with injury to the layer of cells that line the small blood vessels of the lungs. This injury, which occurs for unknown reasons, may cause changes in the way these cells interact with the smooth muscle cells in the vessel wall. As a result, the smooth muscle contracts more than normal and narrows the vessel.

What are the symptoms of pulmonary hypertension?

Symptoms of pulmonary hypertension do not usually occur until the condition has progressed. The first symptom of pulmonary hypertension is usually shortness of breath with everyday activities, such as climbing stairs. Fatigue, dizziness, and fainting spells also can be symptoms. Swelling in the ankles, abdomen or legs; bluish lips and skin, and chest pain may occur as strain on the heart increases. Symptoms range in severity and a given patient may not have all of the symptoms.

In more advanced stages of the disease, even minimal activity will produce some of the symptoms. Additional symptoms include irregular heart beat (palpitations or strong, throbbing sensation), racing pulse, passing out or dizziness, progressive shortness of breath during exercise or activity, and difficulty breathing at rest. Eventually, it may become difficult to carry out any activities as the disease worsens.