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.

Another great visit!!

I went back to see Dr Burger today and again got great news! My pressures are down again! The mean was 52 the last time and now it's 41, the systolic was 77 and now it's 60! I'm now classified as having moderate PH - not severe!! I had tears in my eyes when he told me my numbers I was so happy :) Of course I still have to get them lower - I'm still not near normal but it's so encouraging to know that the medication is working. All my lab work looked good too. I didn't do as well on the six minute walk (I went 60 feet less than last time) but he wasn't concerned about that. The good thing about the walk was my oxygen level stayed at 98% (the last time it dropped to 92%). I am going to double the dose of the medication I am taking now and go back to see him in three months. We will look at the meds again when I go back to decide if we are going to add anything else or not. He did tell me he's ok with me starting coumadin (it's a blood thinner) but it has to be managed by my cardiologist. I will be calling him in the am to make an appt! I am also going to call in the morning to schedule an appt for a second opinion. I just want to confirm that we are doing everything we should be doing for me to feel as good as possible! I am feeling good now and hope to stay that way. I am able to climb the stairs in my house at a normal pace and not get short of breath - that is a big step for me!! Thanks to everyone for all the well wishes and prayers - they really are working :)

Pressures are improving!!

So I had my follow up today and got good news!! My echo showed that my pressures are a little bit lower (the systolic was 91 and now it's 77, the mean was 58 and now it's 52). Severe PH is classified as a mean greater than 55 so I am just below severe. Still a long way to go but the numbers are going in the right direction! Also my right heart function has improved and I was able to walk farther on the six minute walk. He said that they look for people to walk about 45 feet more than they did before meds and I walked 60 so he was very happy with that! Overall he said "I give you two thumbs up!" Coming from someone who was so grim and dire when I first met him, this means alot :) He wants me to continue as is for the next two months and then I will go back and see him again. He said he thinks the Revatio will keep improving my numbers. If not he will increase the dose, change the med or add another med - there are lots of options which is great! I am hoping this continues to work because I am tolerating it very well - no side effects! The only bad news of the day was he doesn't want me lifting Ashleigh or going back to work :( I asked if he thought I would ever get to go back or should I look for something else. He told me not to jump on anything yet, give it some more time and we will reevaluate in September. We'll see - it kinda sucks not having a paycheck. Thank God for my mom - don't know what we would do without her. And for my dad for letting her stay here for so long! I know it's hard and they miss each other :(

The fundraising is going well - $1,120 so far!! Thanks so much for all the contributions - every little bit helps to find a cure!

Quick update

Just wanted to let everyone know I have my next appointment scheduled with Dr Burger on July 8th. I will also be getting blood work, an echo, a nitric oxide exhale test (not sure what this is - it's something new) and I'll do another 6 minute walk. I can't wait to go and see how things are going! I am feeling good but since I have never really felt terrible it's hard for me to judge how well the meds are working. I do feel like I can climb the stairs a little easier so that is a positive sign.

I also wanted to thank everyone again for their donations to my website. I have raised $745 so far!!

Fundraising Website

I started a fundraising website to help raise money to fund research on treatment and hopefully someday a cure for PH. The money goes directly to PH Association. For each donation I will send a "Hope for a PH Cure" bracelet. This was the easiest way for me to "sell" my bracelets. Any and all donations will be greatly appreciated. I also want to raise awareness of this rare disease. Each bracelet will have an information card with facts about PH. The website is www.firstgiving.com/cureph. Checks and money can also be given to me directly and I can send them into PH Association.

I started my meds on Thursday morning and so far so good. My blood pressure has been tolerating it very well and is actually a little better at times. Hopefully it's bringing down my pulmonary pressures! We will see when I go back to see Dr Burger in a month or so. Thanks again for all the support and prayers - it means the world to me and has been very helpful :)

Good News!!

I had my cath today and got some good news! During the cath they gave me inhaled nitric oxide while measuring my pressures. Within a minute or two my pressures started coming down - I heard the cardiologist say "wow!" (in a good way!) He said normally it takes about 15-20 minutes for people's pressures to start coming down but I reacted very quickly. After the cath Dr Burger came and met with us. This time he was very positive and happy. He said it was great that I responded so well to the nitric oxide so he is going to start me on oral meds (thank you God for answering my prayers!!) I am starting tomorrow morning on Revatio. I have to take it three times a day. The down side is it is a vasodilator which means it will dilate all the vessels in my body, not just the ones in my lungs so I have to carefully monitor my blood pressure. Hopefully it won't affect my BP too much but if it does he said there are other things we can try. I am going to go back and see him in about a month or so and we will evaluate how well the medication is working. He said if it is working well and my pressure is tolerating it then he will discuss the possibility of joining a clinical trial to see if two drugs working together is better than just one. Right now I am just so grateful that I can take PO meds. I am going to continue to be positive and pray that it works!

Also while I was there today Dr Burger asked me to participate in a research study. I would have to walk on a treadmill then have an echo done to see how my pressures are affected by exercise. All other tests are done at rest so they are doing this trial to see the effects of exercise. It can only be done on newly diagnosed patients before starting medications. I was nervous to do it but I figured my participation is important in helping with PH. They told me only to walk until I felt uncomfortable - they weren't going to push me to go any further. Really it wasn't too bad. I did just what they asked and walked on an incline for a few minutes until my heart started pounding and I stopped. They did the echo right away. Unfortunately they didn't tell me much but I hope I helped give them information they need to improve outcomes for PH patients.

On that note I wanted to let everyone know that I am going to be making bracelets to sell in support of PH. I want to raise awareness of this rare disease as well as raise money for research. They are going to be purple beads (the color for PH awareness) with a HOPE bead in the middle. All the profits are going to be donated to PH Association for research in finding better treatment and hopefully a cure for PH. I will be starting a website and once that is up and running I will let everyone know.

Primary or Seconday??

So it was a very long day in Jacksonville today but all the testing is done. I had everything done to determine if my PH is primary or secondary. First was the blood draw - 13 tubes in all!! Then I went for all the pulmonary stuff. I had PFT's - which I hated. I had to do an exercise test where I stepped up and down on a set of stairs. After 1 minute and 41 seconds I had to stop because my heart was pounding and I was starting to get short of breath. I was scared I would pass out but after resting for a minute I felt fine. Then they drew an ABG - the girl was good. It hurt less than the lab draw! Then I had my six minute walk where I walked around the halls for six minutes! Every minute they recorded how far I went, my oxygen level and my heart rate. Everything was stable. I then went to cardiology for my echo, chest xray and EKG. Last but not least I had the VQ scan to determine whether or not there are any blood clots in my lungs. That was probably the worst test only because for the first part I had to hold a mask over my face and breathe in and out - I felt like I was being suffocated - it was awful. The rest was a piece of cake - just lay on the table while the camera takes pictures.

After everything was done I waited to see the doctor. He seemed much more friendly this time (probably because he was ready to start a 3 day weekend!) He said everything so far looked fine so he pretty much determined I have primary PH. All along I knew this was going to be the outcome. I am ok with it and ready to fight :) Next Wednesday I have my cath scheduled. During the cath they will give me meds to see what will help bring down the pressures. I am hoping and praying I will be on oral medications and not IV. I guess I should be happy to be on whatever works but continuous IV is just another hurdle I don't want to have to leap. I guess I will cross that bridge when I get there.

Eric and I stopped for dinner on our way home. There was a cute 5 month old baby sitting near us and it made me a little sad. I still haven't completely come to terms that I can never get pregnant again. I basically try not to think about it. When we got home Ashleigh was so happy to see us and that made me feel sooo much better. We were all playing upstairs and she was cracking up laughing - probably the biggest and cutest belly laughs I have ever heard :) It made me realize how very lucky I am and how much I have to fight for!

I want to say thank you to everyone for all their positive thoughts and prayers. It helps more than I can say. This has been tough to deal with but is made a little easier knowing we have so many people who love and care about us and will do anything to help. I have to especially thank my mom - I definitely would not be able to get through this without her. She has been here since I had the first cath. Since I can't pick up or carry Ashleigh she does it all for me. And thanks to my Dad for letting her stay here - it can't be easy :) I can't wait for Dad and Katie to come down in a couple weeks!

How it all started

So I figured the easiest way to keep everyone up to date on what's going on with my situation was to start a blog. Plus I thought it will be helpful to me to get everything off my chest.

About six months ago I noticed I was feeling short of breath walking up the stairs in our house. I just figured I needed to start exercising more - I must be out of shape. On February 12th I passed out for the first time in almost four years. A couple weeks later I passed out again. I chalked both up to the neurocardiogenic syncope I was diagnosed with four years ago when the passing out started. At the beginning of March I decided to go see my cardiologist because the shortness of breath was still happening and I thought he should know I started passing out again. He basically thought I was out of shape but since I am young, don't smoke, am not overweight, blah blah blah he would do a thorough workup. At the end of the month I was scheduled for an echo and stress test. During the stress test I passed out. I walked for less than four minutes. I completely freaked out everyone in the office. They said they have seen very few people pass out during a stress test and none as young as me. Even Dr J was nervous which scared us. At this point I think he knew something else was going on. He ordered a stat CT of my coronary arteries which was done two days later. For that test I had to take two different meds which both lowered my blood pressure. While walking out to the waiting room I passed out again. This time I think it was just because my BP was so low. I ended up going to the ER, got some fluids and was sent home. I went back to see Dr J and he decided he wanted to send my to see a cardiac specialist at the Mayo Clinic in Jacksonville. My appointment was scheduled for May 21st. He did breifly mention pulmonary hypertension but didn't make it seem like a big deal so we didn't really think too much about it. Oh and the cardiac CT was fine.

In mid May we started getting copies of all my records from Dr J's office to bring to the Mayo. In his dictation of the echo and the office visit he stated I have mild pulmonary hypertension and my pressures were elevated on the echo. We started freaking out because we had been researching PH on the internet and it's all pretty grim to say the least. We decided we wanted to have a cardiac cath done because that is the only way to definitevly diagnose PH. I had the cath done on May 12th and sure enough my pulmonary pressures were high and I was officially diagnosed. The next day I had a TEE done to determine if I had a hole in my heart that could be causing the PH. I never thought I would hope and pray to have a hole in my heart! Unfortunately it was negative (if there had been one it was possible to be repaired and the PH could have been resolved).

On May 21st I had my appointment with Dr Burger at the Mayo. He is a leading specialist in treating PH. Eric had been freaking out the week before the appointment and I was pretty calm. I didn't think it was going to be so bad. Unfortunately I was wrong. My pressures were worse on the cath than I had thought. My PA pressure was 90/40 (normal is 25/10) and the right side of my heart is enlarged. He told us we need to be aggressive in treating this. I said whatever we need to do let's do it as soon as possible. First thing is to determine if it is primary or secondary. Primary just happens with no cause (only occurs in 5-6 per 1 million people!) Secondary is caused by something else (connective tissue disorders like rheumatoid arthritis, sarcoidosis, lupus, or a pulmonary embolism or other lung conditions). So this week - Wed, Thurs and Fri I have a bunch of tests scheduled to determine if something is causing all this. Next week I will be scheduled for another cath. During that cath they will give me meds to see what will work to bring the pressures down. Eric asked what the life expectancy is because everything we read online is very discouraging - 2-3 years for primary PH. He wouldn't give us any specifics but said if we can get the pressures down towards normal and not cause any more damage to the right side of my heart I will live. I have found a great website PHassociation.org that is very encouraging. There is no cure for this but there are different meds to treat it. Ten to fifteen years ago there were only 1-2 meds to choose from and now there are 8-9 with more in clinical trials. Hopefully someday there will be a cure. There are many people living normal, productive lives with their PH being controlled on meds. I will be one of those people :) I do admit that I am very scared and don't think this is fair. I keep hoping I will wake up from a bad dream but I know that isn't going to happen. I have to stay positive because I have a whole lot to fight for - especially my beautiful baby girl who is probably a miracle. My pressures were elevated four years ago on the first echo I had done. Because PH is so rare it was never mentioned but had I learned back then that I have PH I never would have had Ashleigh. Everything happens for a reason. Once diagnosed with PH pregnancy is out of the question because there is a huge risk of death. So my dreams of having a big family have been put on hold. For now I am going to treasure what I have. I will write soon once I know what the next steps are going to be.