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 *;*