Two years and doing great!

Ok so it's been a really long time since I've updated this blog! Lots has happened in the last six months! I figure since I just passed my two year "anniversary" of diagnosis I should write an update! I am still seeing Dr Bajwa at Shands and I love him! So glad I found him - Chicago is VERY far!! I saw him at the end of January after having an echo and everything looked great. My heart is still enlarged but functioning well. All was going great until about two weeks ago when I caught a bug from Ashleigh (damn daycare!). It was some sort of respiratory infection and made me feel like crap! One afternoon I decided to run to Walmart to do some errands while Ash was at school (so much easier by myself!) On the way I really wasn't feeling good when all of a sudden it was like my ears popped and I heard kind of a rushing sound - I thought I was going to pass out. I pulled over as fast as I could and called my dad to pick me up. My heart was pounding a mile a minute and I was shaking so bad! I think all from nerves. Anyway, I called Dr Bajwa and he ordered a seven day course of levaquin to try to kick the infection. On day seven I had to go to Dr Jamidar's office to get my INR checked. On the way there I again thought I was going to pass out. This time my whole body got really hot. I took alot of deep breaths and kept driving - I just wanted to get there! I know probably not the safest move but anyway when I got there I still didn't feel great. I asked them to take my BP and it was 86/64 (my normal is low 100's/50's-60's) so it wasn't super low but I wonder what it actually was when I felt like I was going to pass out. I got to see Dr Jamidar while I was there. He thought it was probably just my BP and I was dehydrated from being sick and not my PH. He put a 24 hr holter on which was normal. He also did an echo. He said the pressures were 55 - I freaked! The last echo they were in the 30's. I called Dr Bajwa first thing the next morning. After describing everything he said he really didn't think it was the PH. It may have been the levaquin but we made an appointment to go see him. I went this past Thursday and got all great news! I have been feeling great - infection is gone, no shortness of breath, no feeling of passing out. He wasn't concerned about the echo - most doctors don't pay attention the pressure reading on the echo - they look at the whole picture. I have it ingrained in my brain from Mayo that the echo is the end all be all of tests. Anyway, he was able to measure my cardiac index noninvasively (the cardiac index basically shows if the heart is functioning normal or in failure). Normal cardiac index is about 2.6-4.2. Mine was 3.3! Yay that made me feel much better! Then I did a six minute walk and did even better than last time! I walked 423 meters and my heart rate never went above 107 and O2 level never dropped below 95! Woohoo! I was excited :) He doesn't think I need a cath anytime soon - he only does them when really needed. As a side note I had called Chicago about two months ago to make an appoitment for a cath up there but Dr Rich said there wasn't any reason to do a cath so that means no trip to Chicago this year! So anyway I will follow up with Dr Bajwa in June (my normal scheduled appointment). The other big news was the 2nd Annual Rolling PHor A Cure Bunco Tournament! I hosted it on May 5th and it was a great success. We raised almost $2300 for PH research!! I'm hoping next year will be even better!! With that my total raised to date for PHA is just over $8500!! Thank you SO much to everyone who has made a donation! At the end of June I will be attending the PHA conference in Orlando. I am very much looking forward to meeting other PH patients and learning everything I can about PH. I was asked to sit on a panel for the special events session - I was honored to be asked! I am looking forward to that as well! I will post an update when I come back! I think that is about it - all and all life is great :) I am a lucky girl!

Another PH doc!

OK, it's been FOREVER since I've updated this! I guess that's good because it means I really haven't had anything to report! Things have been going really well - I've been feeling good and keeping busy with work and Ashleigh! I hosted a Bunco tournament in June and raised over $3000 for PHA! Thanks so much to everyone who contributed - it was a great success! I am planning the next one for either April or May of next year which will be here before I know it so I better get moving! Also, November is PH awareness month so I did a one hour class at work for the nurses and RT's and basically anyone who wanted to come. I had a total of 25 people and quite a few more asking if I will do another one because they weren't able to make it. I thought that was a pretty good turnout and I am planning on doing one more at the end of the month! The more people who know something about PH the better!

Today I went to see yet another PH doctor. I really wanted to establish with someone down here in case anything should happen. Chicago is pretty far to go in an emergency! After going to Chicago we decided that we would follow up with Dr Mubarak down here. Well he had the nerve to up and move to Michigan!! I didn't want to go back to Mayo so I found Dr Bajwa at Shands in Jacksonville. He was great! He was very nice, positive and really knows his stuff! He didn't pressure me to do any research trials which was nice (not that I am against research - obviously we need it to find better treatments and a cure but I just don't want it to direct my care if it isn't really in my best interest). Anyway, he said I am doing great. As far as the calcium channel blocker thing he said not to be upset about it. He said the fact that I was initially a responder (only about 12% of PH pts are) is a good indicator that I will do well (I feel like I should be constantly knocking on wood! I really don't want to jinx myself!!) He also said not to get worked up about numbers and what the echo shows. As long as I am feeling good and my cardiac output is ok then I am doing fine. My cardiac output on my last cath was normal. He had me do a six minute walk. I walked 420 meters and my sats never dropped below 98%. I honestly think I could have done better but the way they do it is to have me walk up and down a short hallway - the constant turning around slowed me down! I am going to have an echo and then follow up with him again at the end of January. It was a very good visit and we definitely felt comfortable with him. Now let's just hope he doesn't decide to move away anytime soon!! I guess that's all for now. I will update again after my next visit!

Back to Chicago

Well we are back from our road trip! I don't know that I would ever drive again if given the choice! Although I guess I can't complain because Ashleigh did really well. We left last Monday (May 2nd) with a sick child. She had a 102.8 fever on Sunday and a horrible sounding cough. She woke up a little better Monday morning. I couldn't get her into see a doctor until 2:30 so since she seemed better (and her temp was down to 99.7) we decided to leave. We skipped going to the zoo and just headed to Georgia. We spent the night in Macon and drove to Atlanta in the morning. We went to the Georgia Aquarium which was cool. Ashleigh had fun - she loves fish! She was doing better too - fever was being controlled with tylenol and motrin and she was acting totally normal. After having lunch at the Hard Rock we headed to Bowling Green, KY where we spent the night. The next day was a long hike to Chicago. We stopped to walk around a little and have lunch but then just kept going. We finally got to our hotel around 5:30. We hung out for the night because we had to be up bright and early the next day for the cath.

I was supposed to be at the hospital at 7:30. We were a little late because traffic was horrendous and we had to stop for gas, otherwise we would have been stranded on the side of the road calling AAA!! No one seemed to mind we were late - I knew we'd be sitting around for a while anyway! I think they brought me into the cath lab a little after 9:00 which really wasn't bad - I figured I'd be waiting longer than that! Dr Rich did the cath through my neck instead of my groin. It really wasn't a big deal - the area was numbed up so all I felt was a little tugging (plus I was doped up with some valium!!) Overall the results were good. My mean pressure was 39. I can't remember what the systolic was or if he said how my heart size looked. The bad part was I really didn't react to the calcium channel blockers. First he gave me 50 of adenosine and I dropped to about 36 then he went up to 100 and I didn't feel so good with that - I got hot and my heart was racing! It was a little nerve racking but he said it was a normal reaction to the medication and he turned down the dose. Within a minute or two I felt fine. After the procedure was done he came and talked to us. He said that the echo and the EKG looked better from the previous ones I've had done and my pressures are much better. He said I am definitely not a candidate for IV meds at this time. He said it's good that my systemic BP is low - it was 85/54 when I was lying on the table. He also said that although I am not a CCB responder my prognosis looks good. He wants me to go back on the Revatio - he likes it better and the dose can be increased if needed where as Adcirca can not be increased. He doesn't want to add anything else right now. I will call him in about a month and let him know how I feel. He wants me to go back to redo the stress test - he wasn't impressed with my 11 minutes. I don't think I will go back just for the stress test - it costs way too much money to go up there. We will have to see how I am feeling and find out if one of the docs here will do the test.

I am disappointed that I didn't react to the CCB's. I feel like we wasted ALOT of time and money. If Dr Mubarak at Shands had just done the cath and the CCB challenge and not the whole put me in ICU thing we would have saved alot. I guess I can't change what's already done. I am very glad to hear that things look good and I do feel good. But now the question is which doctor do I follow??? They all seem to have different ideas on what is the best treatment. Dr Burger at Mayo likes Adcirca (and would push me to go into his clinical trial where he is trying to figure out if more than one PH drug is really beneficial), Dr Mubarak thinks PH patients should definitely be on two types of meds and Dr Rich thinks PH patients shouldn't be on two meds. He says there is research showing that Bosentan with Revatio actually makes things worse (I have not heard this one before - I need to look more into that) plus he likes Revatio and not Adcirca. So who the heck knows?!? I do know that although it was a pain to take the Revatio three times a day I felt better on that. Since being on the Adcirca I have had chest pain on and off (nothing serious but I just notice some discomfort sometimes that I never felt with the Revatio). I think I will go back to the Revatio and see how I am feeling in a month and go from there. Luckily I have an awesome cardiologist who I love and can just ask to do an echo :) Since no one seems to think the echo is any good for the pressures at least we can see the size and function of my heart. We got to leave the hospital right after he talked to us - that was a great part of having it done through the neck - no bed rest!

So that night Ashleigh started complaining her ear hurt and she was really fussy. We called our pediatician and got some antibiotics and ear drops. The next day was our only full day of vacation (no driving a million miles and no doctor appointments). Luckily Ash woke up feeling ok (not 100% but better). We went to the Field Museum and the Children's Museum at the Navy Pier. It was a fun day :) Saturday morning it was time to get back in the car and head to KY. We got there pretty early in the afternoon so we decided to go do a few things. We stayed in Horse Cave (a very small town about a half hour north of Bowling Green). We went to a wax museuem and got some homemade fudge (yummy!!) Then we took Ashleigh mini golfing for the first time - that was interesting!! She had fun! It was really pretty there and the weather was beautiful. The next day we took her to Dinosaur World (I didn't really want to go but she was excited - it turned out to be pretty cool!) Then we headed towards Georgia. We stopped and had lunch/dinner at Olive Garden which was crazy busy for Mother's Day. Totally worth the wait though - I love that place! We stayed over in Macon again then drove the rest of the way home today. So glad to be back! Ash is doing much better - the cough is just about gone and she has not once complained about her ears. Just in time to go back to germy daycare tomorrow!

Our trip to Chicago

Eric and I went up to Chicago this past Monday to meet with Dr Rich. It was the first time we had both left Ashleigh. That was a little nerve racking and sad! I knew she was in good hands though because she was staying with my parents. After checking into the hotel we went down to the Navy Pier which was about a 15 minute walk from the hotel. It was a cool place but would be better to visit when it's warmer - not when it is 32 degrees and windy! We had dinner at Harry Carey's which was very good then went back and hung out at the hotel.

Tuesday morning we got up super early to start our day at the University of Chicago Hospital. The first test I was having done was an MRI - boy was that a disaster! They brought me back around 8:15 (my appointment was scheduled at 9:00) and told Eric I would be gone for a couple hours. They said the prep would take a little while and the scan about an hour. I was not looking forward to lying in a tube for an hour! Anyway they brought me back and started an IV. That was the extent of the "prep" then I waited around until 9:30 when they finally brought me into the MRI. I felt like I was in that thing forever and come to find out I was - 2 hours!! I don't know what the deal was but I was not pleased. A few times I was ready to tell them to shove it and get me the hell out of that machine! I was starving because they said I couldn't eat before the test and I hadn't had any coffee so between the lack of caffiene and the super loud machine my head was pounding. Of course Eric was freaking out in the waiting room because I had been gone for three hours and no one was telling him what was going on. I was glad when that was over!

After that ordeal I went and had my echo which went smoothly. I finally got to eat some lunch and get some caffiene once that was done :) My appointment with the doctor was next on the list. We really liked him - he was very nice and down to earth. He said he hates PH and will keep fighting to find more treatments and a cure. He was the person who discovered that calcium channel blockers work for PH - 30 years ago! He said that he wants to do another cath and a calcium channel blocker challenge during that - he said he likes to use adenosine instead of nitric oxide. Based on that he would decide whether or not I should start CCB's. He asked if we could extend our stay and do it Thursday but we didn't think that would be possible so we decided we are going to go back. Now looking at how much it's going to cost to go back we should have stayed! He said that it's not an emergent thing. He said I look good - my physical exam is essentially normal. My echo hasn't changed since the previous one which is good and bad because that means my heart is still enlarged but at least it's not worse. He didn't tell me what my pressures were because he said they are too unreliable on the echo so he never looks at them! He encouraged me to do the pulmonary stress test because he said it gives them a lot of good information. I was very scared to do it because it is on a treadmill and I really didn't want to pass out! I agreed to do it in the end because I knew I could stop at any point. He said twenty minutes is the most they make you do. I stopped at 11 minutes. My heart rate was almost 160, I was sweaty and getting short of breath. My oxygen level never dropped below 98%. I probably could have gone a little longer but I was too nervous. The inclines kill me! I didn't get to see him again after that so I'm not sure what he makes of the results. They said I could call or email any questions and they will get back to me so I guess at some point I can do that!

After that very long day at the hospital we went to the Hard Rock Cafe for dinner and then walked around Michigan Ave. I didn't last too long because it was freezing and I just wanted to go back to the hotel and warm up! Wednesday morning we were up bright and early again and got everything ready to head home. The weather in Chicago was clear but it was stormy in Orlando. That flight was probably the scariest one I have ever been on! Turbulence usually doesn't bother me but it was really bad. At one point there was a really loud bang and the plane shook and there was burst of white light - the plane was hit by lightening!! It was crazy!! Once we finally landed I was never so happy to have my feet on the ground!

Ash did great with Grandma, Pa and Boomer! She wasn't as excited to see us as I thought she would be - I guess she was just too spoiled while we were gone!

I was supposed to have a follow up at the Mayo on Monday but I am postponing that until I get the cath done in Chicago. There is no point since I don't know what to do about the medications. We will just have to wait and see. I just hope we are doing the right thing - I don't want to burn any bridges! If only Dr Rich would move to Florida :)

Thanks again to everyone for all your positive thoughts and prayers!! They are really appreciated so please keep them coming!

Second Opinion

Today we went to see Dr Muburak at Shands in Gainesville for a second opinion. Prior to my last visit I was hesitant of going because I thought things were going great so why bother. I'm glad I went!

He is very nice. He is laid back but not afraid to voice his opinion! He doesn't believe in echos. He doesn't think they are accurate and shouldn't be used for making treatment decisions. In his words "they are bullshit"! When he reviewed the cath that I had done at Mayo he was surprised to see that I reacted to the nitric oxide and haven't been started on calcium channel blockers (CCB's). Only a small percent of PH patients are CCB responders. When the CCB works you often don't have to go on any other medications. According to the cath report my cardiac output was low therefore they didn't start me on any CCB. The normal cardiac output is about 5 L/min and the cath showed mine to be about 3 L/min which would mean my heart wasn't functioning well. When there is poor heart function CCB's are not an option because they can cause further damage. He said that he doesn't know how they calculated my cardiac output so he doesn't know how accurate it was. He thinks I should try a CCB challenge which can be done in one of two ways. One would be outpatient where they would give me the meds and see what happens. The other is to be admitted to the ICU for a few days, have a catheter placed to continuously measure my pulmonary pressures and cardiac output and slowly give me CCB's and monitor my reaction. This isn't something that is done very frequently. I asked him how often he has done this test and he said once! Not very reassuring! However if I decide to give CCB's a try this is the route I will take. I figure I will know what my cardiac output is prior to starting and if it's too low then we don't proceed and if it's ok then we try it and I am in a controlled environment where people can react right away if something went wrong. He told me about a doctor in Chicago - Stuart Rich - who is an expert with CCB's and other PH treatments. He suggested I see him for his opinion. I looked him up online and what I found was impressive. He has been treating PH for 30 years and helped lead the way to start using IV meds for treatment. It looks like Eric and I will be taking a trip to Chicago! One more opinion can't hurt and it will be like a tie breaker between the two docs I have already seen! If the CCB's end up not being an option Dr Muburak said he would put me on a second PH medication such as Tracleer or Letaris. He agreed that there aren't any studies to prove two types of meds work better than one but from what he has seen it does. The other good thing that he said is that he classifies me as Stage I not stage II - basically he thinks I am doing well which is nice to hear :)

I have a renewed sense of hope that things are going to be okay. The last month has been a little rough with getting the bad news from my last visit that my heart wasn't as strong and the PH community lost two young women to this stupid disease. One was only 32 and the other 26 - life just isn't fair. It only makes me more determined to fight and to find a way to help find a cure. I have spoken with PHA and with their help I am going to organize a bunco tournament where all proceeds will go to PHA for research and PHA programs. I have a wonderful group of friends who are going to help make it a huge success! I'll post more about it once the details have been ironed out.

Thanks again to everyone for all the good wishes and prayers. They really work so keep them coming :)

Not such great news

I had my check up with Dr Burger today. I have been feeling great and went to the appointment thinking I was going to get a great report. Well it wasn't horrible but it wasn't what I wanted either. First I did my six minute walk. I didn't get short of breath at all and walked the farthest I have to date. Then I had my echo and blood work. My pressures were about the same as last time - the systolic was 59 and the mean was 44. The bad part is my heart size and function are worse. The right ventricle is moderately enlarged and the function is mildly hypokinetic (doesn't pump as well). Dr Burger is changing my medication from Revatio to Adcirca. They are in the same class but he feels the FDA approved dose of Adcirca is higher and more effective. He was very confident that this medication will work to improve my heart. He said this disease is like a roller coaster and sometimes there are going to be dips but we just need to do what we can to get back up the incline. I know that I'm not always going to get a great report, it's just the nature of this horrible disease but it's hard to take because I feel so good! I blame myself because I have been lifting Ashleigh alot - even though Eric always tells me not to and the doctor did tell me not to lift more than 10 lbs. I figured I felt ok so what harm was I doing - now I know :( The good thing is it can be reversed and I am going to continue to think positive and do all that I can to get better (even if that means I can't pick Ashleigh up so much - or any more than absolutely necessary). I think if she was able to choose she would want her mom to be around for many years instead of being held all the time at two years old!

The other positive thing was when I asked about working. He told me I can't go back to the bedside which is what I expected but he is ok with me working in an office or doing some kind of desk job. This is an improvement because before he really didn't want me to do anything. Now I just have to actually find a job!!

I will start the new meds probably next week - I have to wait for the approval from the insurance company and the pharmacy to get the meds. I am a little nervous and hope that I don't have side effects. That's one good thing about Revatio - I tolerated it very well. But the awesome thing about Adcirca is that I only have to take it once a day instead of three times a day! That will be so much easier!

Then if all this wasn't enough, after picking up Ashleigh from Chris and Charlie's I hit a deer in their neighborhood! Luckily I wasn't going very fast and no one was hurt (I don't think the deer was either because it ran off). My car does have some damage - the front grill is cracked and the bumper is loose. Hopefully that doesn't cost an arm and a leg to fix!

I am not going to let all this get me down though because Christmas is only a few days away :) And then my baby girl turns 2!! I have no idea where the time has gone!

One last thing - I go back to see Dr Burger and get all the same tests again in three months. Please keep the prayers coming :)

PH Blogging Day

Today is PH blogging day so I am updating my blog! For anyone new to my blog the first posting gives my whole story from the beginning. I've been feeling really good overall. I get short of breath once in a while if I do too much but I just slow down and I feel ok. I feel very blessed to be feeling as good as I do. I am grateful for every minute of it because I know that it could change so quickly. That's one of the many sucky things about PH - no one knows how long the meds will work - everyone is different. I pray all the time that they continue to work well for me. I try to always be positive - some days it's not easy but it truly makes a difference.

I go back to see Dr Burger on December 20th. I will hopefully be able to join the clinical trial at that time that we talked about the last visit. He is studying the effects of more than one medication on PH. Almost all PH patients are on different types of PH meds because they work in different ways. My doctor however says there haven't been alot of actual studies done to show this is really effective. Hey if I can get some meds for free and get paid to go to Jacksonville count me in, especially for a med that I would have wanted to start anyway! I have been on coumadin for the last month or so. We are still trying to figure the right dose for my target INR range (clotting time) of around 2. I had my blood drawn today and it was 1.8 so we're getting there. I'll have it checked again next week.

I am very much looking forward to the holidays. We are having Thanksgiving here - I love having a house full of family :) Then Eric, Ashleigh and I are taking a trip to Disney for a week then it's Christmas!! I can't wait to see Ash this year - she is going to be so cute!! I hope and pray for a cure because I want to be around for all Ashleigh's Christmases. I love that little girl more than I thought it was possible to love anyone :)

Anyway, this blog is supposed to be about PH! For all the up to date info, check out www.phassociation.org. That site is where I get all my information - it was the only one I found early on that had positive things to say - the first thing I saw when I opened the webpage was a quote "PH is not a death sentence" After everything else I had found online that really made me smile. It also led me to Generation Hope, an online support group for people my age with PH. It has been extremely helpful and comforting to know that I am not in this alone.

Also for anyone who doesn't have a Hope for A Cure bracelet and would like to get one please visit my fundraising page www.firstgiving.com/cureph. Every little donation helps towards finding a cure. Sometime in the near future I am going to start planning a bunco fundraiser to raise awareness of PH and of course money for research. More info to come on that later!

Well, thanks for checking out my blog! I will update again after my next trip to Jacksonville.