Introduce Yourself

[kaye-norlin]

Hi,
I am Kaye, 66 years old, a recently sort-of retired social worker, current yoga instructor for people with health issues or disabilities, and an adjust psychology instructor at the local junior college. I currently work part-time for my old employer writing reports- yuck but it is something to do and $ and I can do it from home. I live in north central Illinois in a small town about 100 miles southwest of Chicago. Before transplant, I think I have tried most of the PH drugs; my liver didn’t like many of them. The last drugs I took included Flolan, Revatio, and Opsumit; I was also on oxygen. The Flolan and oxygen were added in September 2016; I was sick at the time and a month later “crashed” and was diagnosed as end-stage PH. No one wants to hear that!!! I was put on the transplant list in April 2017 and was transplanted January 3, 2018, so I have 2 birthdays to celebrate now!
I love to sew and have costumed plays for local theater productions and want to get back to that very soon! I also do Renaissance festivals (not this year because of dust) and we go in full costume; I even made one to accommodate my Flolan pump and O2 tank. I want to do more sewing for myself now that I have lost equipment and pounds. I also have had an organic garden for 37 years but this year I will just give directions; I like canning food. I love to exercise, cook and eat good food, travel, ride horses (next year), and learn new things.

[judie]

Hi Everyone,
I am not sure if this is the correct forum. I had a routine echo for a genetic condition I have (Ehlers Danlos) which is a connective tissue disorder. The first echo had to be repeated as it was erroneous. It is saying all 4 of my valves had major issues. It turns out there is no change compared to an echo I had 11 years ago. But it did pick up this pulmonary hypertension I believe the reading was just under 60. I will have a right cath done in 2 weeks.
My first impression was being petrified of the cath due to other issues. I have systemic mast cell disease and rheumatoid arthritis which may be the cause of it all.
I am mildly symptomatic, I have been in denial up to now but proof is proof and as a retired RN I am not going to be foolish. I need to learn as much as I can to prevent getting worse.
I would appreciate any insight one can give.
Also how quickly did this progress for you. I exercise most days and although I feel older (60) in July I think I’m doing fairly well.

[kathleen-sheffer]

Hi Judie,

Thanks for sharing your story with us. A right heart cath is the only way to diagnose pulmonary hypertension. My echo’s were never accurate, nor could they be consistently correlated with the pressures recorded in my caths. I’m glad you are going forward with the procedure in two weeks. I encourage you to stay positive and focused on the present while you wait. They may find nothing. I hope the cath goes smoothly. You should be able to go home the same day. No need to address anything now without those results. Take care!

[azebraman]

Hi all I’m Vik. 33/M and new to this forum. Living with thoracic insufficiency (TIS), restrictive lung disease, and Ehlers-Danlos syndrome since birth. Diagnosed with Class III PHTN in 2008 due to the thoracic insufficiency catching up with me. Pulmonary function between 40-55% depending on the test and exercise saps me so bad I can’t clean my own house, but hey, “I look perfectly fine!”.

Looking forward to researchers paying more attention being paid to Class III PHTN/TIS. It’s so rare I’ve yet to find even one other person online in a similar situation. Looking for online penpals to share stories with. You’ll see me rant elsewhere on these forums. 🙂 Thanks in advance for letting me be here!

[kathleen-sheffer]

Welcome, Vik, and thanks for introducing yourself. You’ve definitely nailed the rare disease category. Can’t imagine operating at 40-55% lung function. Is lung transplant (or heart-lung since you’ve had a VSD) an option for you?

[azebraman]

Kathleen,
Despite being in the < 55% range on most of my PFTs, I’m still at 85%+ in DLCO — So not into transplants for now but would be interested in hearing survival stories because I’d thought that 5-10 years was the best one could hope for and if it’s longer, who knows I might have a normal life expectancy. Fortunately I won’t need HLT because my heart is repaired fully (at the expense of ribcage damage).

Thanks for everybody’s support!

[kathleen-sheffer]

Vik, it’s definitely something to keep in mind for the future (hopefully a long way off). Some of the first people to receive transplants 30 years ago are still alive today, and I have several friends who are well past the 10-year mark. Nearly 70% of recipients age 35-49 survive past the 5-year mark. They’re always learning new strategies, though, and I was so glad I was able to wait 16 years with the PH therapies available to me. Are you taking any medications to treat your PH?

[azebraman]

Kathleen — I am only doing supportive treatment right now. Spiriva (COPD/asthma inhaler) to alleviate shortness of breath during exercise (not just typical exercise but also walking up too many stairs!). My pulmonologist didn’t want to start with endothelin drugs because of the side effects.

[kathy-mack]

Hi im kathy 72yrs.young from cape cod ma. I was just diagnosed with ph. I had a pacemaker put in last yr. and thought that would take care of my breathing problem. But no. I got much worse and cannot breath too well walking a short distance. And i cough a lot. Who do i see first a pulmonary dr or a heart dr. I know i need meds. Im on a nebulizer and a hand held one. And lasix. Whats next for me im mee to this awful ph.

[kathleen-sheffer]

Hi Kathy, I’m glad you found our site.

You’ll want to see a pulmonary hypertension specialist – generally a pulmonologist.The Pulmonary Hypertension Association offers a search engine to find accredited PH Care Centers and physicians.

Though Massachusetts General has a pulmonary hypertension program, they are not officially accredited by PAH (for what that’s worth). It looks like the best choice for you may be Boston’s Brigham and Women’s Hospital. They have a multidisciplinary program that combines pulmonary and cardiovascular teams. A friend of mine has been treated there for years and just received a heart-lung transplant – she loves their program.

I know traveling is difficult, but when I was diagnosed in 2000 I traveled from California to New York City to see the leading physicians at Columbia. In my opinion it was definitely worth it! I wish you luck on your journey to get the treatment you need.

[brittany-foster]

Kathy,
I am a Boston patient too! I would highly recommend Brigham and Womens. Their team of specialist work very closely with the cardiologists there and they are actually right next to each other at the clinic. They are very thorough. Many of the doctors that treat PAH there are also specialized in heart conditions so it is a win win. I see Dr. Cockrill at Brighams. I’m not sure if she is accepting new patients. But she is very great at what she does and sees patients with heart failure too. If you have questions about this program please feel free to message or just comment back.

[kathleen-sheffer]

Britt, I didn’t know this – so cool! Do you know my friend, Camille Frede? Thanks for chiming in about B&W 🙂

[brittany-foster]

Hey! No I don’t know her ! I wonder who she has for doctors. Didn’t she just recently get a transplant? I saw something on one of the groups about her I think! Hope she is doing well! 🙂

[kathleen-sheffer]

Yeah, she had a heart-lung transplant in March and she’s doing great!

[brittany-foster]

That’s amazing ! I love to hear about the successes ! I know she is in great hands there 🙂

[robin-frankel]

Hi guys, I’m new to this. Got diagnosed last week with pressure of 94/37, this is cardiac cath, EF of 40%% so I it seems heart failure too. I have struggled with rare diseases my whole life like behcets, sjogrens, recurrent guillian Barre, spinal stenosis but the Drs just said this pulmonary hypertension is very severe, both primary and secondary, hard to treat. I will go to Mayo clinic in a month. Dr says maybe I can get 6to9 years given heart issues. Wow I’m a pain in the butt itpatient, I don’t take no for an answer but this is tough. What do you tell your friends, they just look it up anyway and get upset! My husband is crying. I don’t like that. My plan is to start walking. Am I stupid? My mom was given six months and she started walking with that oxygen. Didn’t know I had heart issues too. Help me out here, guys, I don’t like being the problem child!

[rick]

Hello everyone, I’m Rick I’ll be 38 next week, I’m from Boston. I was just diagnosed with this a week and a half ago. Wasn’t getting enough oxygen to my brain and fainted. I was really lethargic and tired for 3 days. It takes my legs to be falling off before I go to the Dr’s for anything. Luckily my Fiance was therw and called the ambulance. My right side of my heart is a lot bigger than the left and I guess I have lung disease. I’m on my 11th day without smoking and you know what? It’s not a problem cause I’m gonna do everything I can to stick around as long as I can for my daughter who is 5 months old and my Fiance. Before them, I just didn’t care about myself. I really did a number on my body and it finally caught up to me so I can’t say I’m that surprised about this. Well, any help or advice/suggestions would be so appreciated. And Thanks for listening!

[brittany-foster]

Hi Rick.
It’s great to have you here. Although a diagnosis is always so mentally and physicslly challenging, it is good to have support from the people who understand. It is hard to find support groups and many prefer the world of online and that is why this is a great start when looking for support. Like you, I also receive my care in Boston at Brigham and women’s. I stayed over there a few weeks and I am so happy with the care I received inpatient and outpatient. Did they do a heart cath on you or have they mentioned this to you? If you ever have any questions please feel free to reach out since i am a fellow Boston girl!

[rick]

Awesome! Thanks Brittany! I’m at Tufts Med Cen getting my care. My Fiance’ and I don’t have much at all family so it’s good to get support wherever I can find it amd PHN looks like a great place to find some! I actually have my first appointment with my Pulmonologist on the 3rd of July. I don’t know why they wait so long but it’s nerve wracking. I did have an appointment with my PCP the other day but she didn’t seem to know much about the disease and even made me feel like I was making too much a deal out of it? She also said she hadn’t had the time to look over my hospital notes yet? I’m like wtf kind of dr does that knowing a patient of theirs was in the hospital for 6 days? Anyway, I’ve read a lot about the heart cath and I’m expecting to hear more when I go to my Pulmonologist. I’m not thrilled about that. Also, while I was in the hospital, they couldn’t get an IV anywhere so they did a Central IV that went from my neck to the tip of my heart. That was not fun at all. Well, thanks for saying “hello” and welcoming me. I really appreciate it!

[brittany-foster]

Rick,
I am wishing you the best of luck at your appointment. I know that sometimes the waiting around is the hardest part. Take this as a time to keep track of symptoms, write down any questions, etc. Also make sure to add in some distractions to your day and try to stay as busy as possible to keep your mind off of things. Do some things that you enjoy ! I am glad you are seeing a specialist. It’s unfortunate that there are many doctors who don’t know much about this disease. Have trust that your specialist will be great! Keep us posted.

[dori-herrick]

Hello, my name is Dori. I live in the suburbs of Milwaukee, WI. My symptoms were mistaken for asthma for over 20 years. I was finally diagnosed at 42 with Class 1 (PAH), Stage 4 with Heart Failure. They initially didn’t expect me to survive, but I am one stubborn fighter.
I also have systemic Rheumatoid Disease, which attacks my lungs, and Fibromyalgia. Because the Rheumatoid Disease would attack a transplant and greatly lessen life expectancy afterward, I have opted not to be placed on the list.
I am currently on Remodulin, Revatio, and Opsumit to try to slow the progression of the disease. I am also on constant oxygen to function.
I recently spent a week in the hospital with severe pneumonia, which blocked about 70% of my lungs. At one point, I was on 12L oxygen just to walk to the bathroom. Despite nearly dying again, I have been recovering. With the help of a visiting Physical Therapist over the past 3 weeks, I have strengthened my lungs enough to function at 4-5L oxygen. This is a better-than-expected recovery, but I did mention that I am stubborn.
Since my diagnosis, I have spent a lot of time reading medical journals to get educated on my disease and how I can best help my body continue to live. I try to get plenty of rest despite insomnia and pain, I eat mostly fresh produce to keep my sodium-intake as low as possible, and I limit my fluid intake to reduce the stress on the kidneys. But, the most important thing I do is to keep a positive outlook and share what I learn with others.

[brittany-foster]

Hi Dori,
Thank you for introducing yourself. I find it so interesting to hear about other’s and their stories. Your situation is similar to mine in that they diagnosed me as having exercise asthma for many years of my life. It wasn’t until they actually checked my pulse ox while walking around and on exertion that prompted them to think it was much more than that. I am lucky that they decided to test that.

I am also on oxygen therapy and can’t even imagine having a bad bout of an infection like you did that dropped your oxygen level so much that you were on 12l. That’s truly amazing that your body was able to fight so hard for you during that time and is continuing to fight and push forward now.

Please continue to keep us updated on your progress with the physical therapist. I know PT and pulmonary rehab has been so beneficial to many of our members here.

All the best to you always!

[donnah]

Hello! I’m Donna, a 59yo mom of 5 and grandma of 11! I led a busy life until April of this year, when I landed in the hospital with a mild pneumonia but not-so-mild hypoxic encephalopathy. After a slow 2-mo recovery of sorts and a million non-invasive tests, my fantastic pulmonologist referred me to a PH doc, whom I just visited yesterday. He said based on everything done so far, he’s certain it’s PH, but scheduled me for the invasive, gold-standard testing that I know most of you have had already, which will (hopefully) determine the cause, severity, and treatment plan. Next Monday I’ll have a TEE to check for shunting, a R heart cath, with exercise if needed, an angiogram and left heart cath also, if needed. I’ve not been able to come off of oxygen since I became ill in April; I can occasionally leave it off for a couple of hours if I’m just sitting at my desk in my office in the middle of the morning. By afternoon I’m too fatigued, and find my sats dropping and/or heart rate rising.

As many of you have experienced, this was a very abrupt lifestyle change; my doc says some patients can go along with a low level of PH, and then when a pneumonia or other lung insult happens, their threshold is overcome, and they cannot recover. He is planning to do some med trials during the cath, and I am hopeful he will find some treatment to help me get a bit of my life back! I live in Colorado, already a mile high, and am really missing getting into the mountains–tried one trip to about 8500 feet, which didn’t go well.

I’m thankful to have found this group, and I have some questions for you! I would love to hear if you have any suggestions for the cath(s); I’m not looking forward to them! The R heart cath is definitely happening; the left heart only if it’s needed. He’s doing a neck insertion for right, and will do wrist if he does the left. Any advice on recovery time? I am working part-time as an administrator, so will try to work ahead so I can take most of next week off. Is it reasonable to expect that I might be able to get back to work by Thursday or Friday?

Secondly, for any of you on oxygen, do you still find your HR increasing with even mild activity (walking to the kitchen for a drink of water) although your 02 sats are staying in the 90s? I’ve been practically living with a pulse oximeter on my hand to see what I can tolerate and what I can’t. If I take my oxygen off to walk to the kitchen, I’ll drop into the 80s with a HR of 100+, especially if it’s later in the day and I’m tired. I can usually shower and get dressed in the morning without it, but after about 15-20 minutes I’m in the same situation. But there are times, even with my oxygen on, that my HR will easily hit 100, which, of course, means it’s able to compensate and keep my sats up, but it makes me feel miserable…dizzy and exhausted, mostly. I notice if I’m doing something for an extended period of time, like laundry, housework, etc., and my HR is in upper 90s/low 100s (with oxygen on), I may be out for days afterwards. I’ve given up shopping and most outside activities (other than going to work a few mornings a week) because it’s just too exhausting. I love my job and don’t want to leave it, but I find it challenging to have the presence of mind to keep up with the complex mental gymnastics it takes to do the job well. I would also love to hear how treatments have improved your daily life!

My family has been wonderful through all of this, although they are very concerned. One of my daughters and her family are moving in with us to help myself and my husband (who has COPD, but is still working and is doing fairly well); we have two children who have autoimmune disorders and are disabled. They also live with us, as well as my son’s wife 🙂 With such a large, multi-generational household, we are getting ready to partition off part of the house into an apartment for myself and my husband so if I need to rest, I’m able to do so with separate living quarters. The other day, I was able to have my 4yo granddaughter for a “sleepover” for the first time since I’ve been ill; as I came around the corner after dressing, and did not have my oxygen on, she said, “Grandma, you don’t have your tube on!” They’re keeping a pretty close eye on things 🙂

This is far more than I’m sure you’d like to know, but any suggestions or advice you’ve got to offer, I’d love to hear! I was an RN in a previous life (before kids!), so have a medical background and have done a lot of scary reading, which, I know, is not individualized. I thought at first that if I had PH, it was mild, until my last pulmonology visit–when it dawned on me that I am certainly in at least functional class III, if not IV. Since I don’t know yet the type of PH (although they’ve ruled out lung disease and there’s apparently not much CAD), I don’t know what to expect or how much my functional status will improve. I do know my pulmonary artery is “clearly larger than the aorta,” according to the cardiologist…not a great finding. I also have poorly controlled diabetes, although I’m trying improve on that!

Thanks to all who have made it through my long post, and I appreciate all your discussions; I’ve read through most of them the last few days. I’m looking forward to “meeting” you!

Donna

[brittany-foster]

Hi Donna,
Great to meet you on here. Although it is scary to have this possible diagnosis and all of these symptoms you are experiencing, it is nice to read that you have such great support from your family. From what I am reading it seems like you are also confident in the abilities of your medical team to help figure out what is going on for you and the best course of treatment. I know that they will do all the right things in order to get you feeling as best you can. That is always the goal of treatment. Although, as you know, there is no “cure” there are still ways we can get the most out of our life and continue on with daily living the best we can. Having a quality of life is the most important thing for me !

I am only 27, but am also on oxygen therapy and like you was diagnosed in April but 3 years ago! I had to learn my limits on what I can and can’t do on oxygen and without it and most of these lessons were learned the hard way as I began to think I could do a lot more than I could and really pushed myself. My biggest advice to anyone is to really pay attention to your body and the warning signs that it gives you during the day. It seems like you are already doing this with noticing different levels of fatigue through the day. For me, one of the biggest signs that my oxygen needs to be on or turned up is a brain fog feeling and not responding as quickly as I usually would. Make a list of some of these signs that you notice in yourself and patterns that you see. This will also be helpful for doctors when determining when to give certain medications to optimize your symptom management through the day.

Make sure to ask for copies of all testing and results that you have been getting as well as reports after the appointment. Keep this all in a folder so in case of any emergency, or upon seeing a new doctor, you can show them this folder so they aren’t scrambling for information. This is a rare condition and the more info you actually have in your hands the better and a better way to advocate for yourself and keep some sense of control over your body!

As far as the heart cath goes, since having congenital heart disease I have had many. Some through the neck, groin, wrist. The one through the neck I have found is the most uncomfortable and have needed serious pain meds combo during it to keep me comfortable. I can assure you that after the medication I was in a “great” state of mind ! lol! I’m sure you can only imagine the conversation as I told the surgeon I wanted to “drunk dial” my cardiologist hahaha! But on a serious note, ask them if they plan on doing this with sedation and if not if they have a plan for pain management if you are uncomfortable during it. Sometimes they allow music to be played during the procedure. I know that hearing some background noise really helps me relax. Also, having someone in there that is explaining everything as it is happening is important to me. Try to think of ways that you know would help you to stay present and as relaxed as possible and write this down and talk about it with the doctor. In childrens hospitals they always explain everything to the patients and sometimes even have books and videos with pictures so they know what to expect. Many adult hospitals don’t do this to this extent unless they are asked. The MORE I know the better and I’m sure it might be the same for you !

Welcome to the forum. I’m really glad you are here. I know you are in good hands. Please keep us all updated and focus on your support system right now. They will get you through this!

[donnah]

Thank you, Brittany! I was looking at another thread discussing cath pain…I have a condition called CRPS (RSD is the old name for it), which is an abnormal pain response. Right now I have it in my left arm, as the result of nerve damage thanks to a drunk driver…but it can spread to other limbs. I am a bit concerned about that. If this is going to be an extremely painful procedure I’ll have to do some research on this.

Did they do an exercise cath when they did your neck insertion site? How did they medicate you into a “great” state of mind and still have you exercise, if so? 🙂 Do they actually get you off the table onto a bicycle, or do it at the foot of the bed?

Did you have a med trial during any of your caths? Did you have to stay overnight? They told me to be prepared to stay over. From your description on the other thread, it doesn’t sound like I’ll make it back to work next week…is that common for everyone who’s had a neck cath?

Sorry for all the silly questions…just trying to get a better idea of what to expect. I didn’t think of these questions while still at the dr’s office, of course!

I’d love to ask you a couple of more questions about oxygen, but I see there’s a separate forum for that, so I’ll start a thread there. Thank you so much for your kind response, and so much information! I’ve read many of your articles, and you’re an excellent writer 🙂

Donna

[brittany-foster]

Hi Donna,
when I had my exercise cath done, at first they didn’t give any type of pain meds to put me into a better state of mind with the pain. They just really numbed up the area and I honestly didn’t feel the cath until it was in because of the way my vasculature narrows at certain points. That is why it was so painful for me. But they usually have you exercise sitting up and biking (it isn’t as scary as it sounds) and you technically shouldnt feel the cath at this point. The only pain or pressure you would feel is supposed to be where they initially insert it. They gave me high doses of narcotics through IV and a combo with another medicine to calm me down (I was seeing the galaxy after that lol!) But was still awake and able to move my body. So they actually did a supine (laying down) bike test and put the pedals at the end of the bed. This isn’t super accurate because I wasn’t up but it was still able to give them an indication as to what is going on.

[brittany-foster]

Also,
even with the extreme pain, they gave me the option to stay over but I didn’t want to, I wanted to just leave. I wish I did though to help pain manage after ! But it ended up being okay that I went home. Most people can go home right after. Unless they have to monitor you with medications and put you on new meds or a higher dosage, they may want to keep you for further monitoring to see how you are responding to things.

[robin-frankel]

Hi, Donna, my name is Robin and I am also a Grandma, working person, and newly diagnosed with CTEPH. I am hoping to qualify for a surgery that will help my type of PH. But I recognize many of the problems you are having. I love my job (psychotherapy) but it is getting more difficult to fight the brain fog and exhaustion. Parking lots are my nemesis in the Florida heat. I also can maintain an O2 sat of 96 while up and around but my heart beats wildly and tests show it is failing to withstand my lung pressures that are in the nineties and eighties. Does anyone else’s O2 fall when you lie down? The surprise of exhaustion that results from mild activity is daunting, is it not? My grandbaby came with her mom for a week and she wanted to go out to the park etc. So disappointing to tell her I can’t 😟. Good luck with your diagnosis! Robin

[brittany-foster]

Robin,
Yes! The brain fog and exhaustion is so hard to manage. I feel the same way with my heart racing. Do you see a cardiologist as well? Mine put me on a medicine to slow the heart rate and the workload of the heart. It helps a lot with significant changes in heart rate with small amounts of activity.

[kristine]

Donna,
I am also in Colorado, it’s good to know someone here in my backyard so to speak. Everyone else seems far away, and we face the high elevation which only makes this more complicated or exacerbated.

[rick]

Hey everyone. I just wanted to check in with you. I don’t have anyone else to talk to. Right now I’m writing while in bed at a shelter. I really screwed up guys 🙁 after 2 1/2 years sober, I relapsed. I feel so ashamed especially with a 6 month old child. It happened 3 times. I can tell you why and how it happened. There is no excuse for it. It’s all my fault but stress really had a big part. My Fiance (now ex I guess?) Got into some arguments and I just couldn’t cope and my heart skips and feels like its coming out of my chest sometimes and it scares the sh*t out of me. So I relapsed on xanax to try to relax but I ended up taking too many and acting really scarey. So my Fiance’ kicked me out. I’ve had to sleep outside a few times and she just doesn’t care? All she wants from me is to come over during the day to take care of my daughter so she can rest. I feel like shes using me to a point. But I really need to focus on myself right now. This is my 6th night at the shelter and they kick you out after 6 nights and after 4 days out, you can come back for another 6. I feel like pneaumonia is trying to settle in my chest and that’s when my PH is the worst. I’m so scared guys and girls. I missed a PH appointment because i was in detox. Now the next available isn’t til end of August. I’m so disappointed at myself. I feel mentally unstable and I don’t know where I’m gonna sleep tomorrow night and the nights after that? This girl is so hard on me. Wants me out to prove to her i can stay sober on my own for a year! I’m still sober now but I really do feel older than my age inside and have a bad feeling about this whole thing. I’m sorry for the rant. I got no one else. Thanks

[brittany-foster]

Hi Rick,
as someone who as battled through different types of mental illness and an eating disorder, I can relate to what it is like to relapse into something that you logically know is wrong. There are so many different reasons why people relapse and for me personally, it has to do with a feeling of being out of control physically and mentally at the same time. The added mental stress just sends my feelings into overdrive especially because there is already constant physical stress on my body on a day to day basis. I know that right now, with relapsing, you may feel like a failure and I get that. I can understand what it’s like to feel like you lost it or lost control and that’s a scary feeling. I know that it helps me to have others for support who can be there for me. Do you have close friends or family members that can be with you during this time and help direct you on what to do next for yourself? I want you to get the best care possible because although you may feel differently, you DO DESERVE CARE! I would suggest going to a hospital or an ER, especially if you don’t feel ready to leave the shelter. It’s best to keep yourself safe and get yourself into a better mental state. It sounds cliche, but with recovery and with our physical health, we HAVE TO take ONE DAY AT A TIME and for me that means even one minute at a time. You lost control but need to look at the minutes as a new minute and today as a fresh start. You were on track before and you CAN get there again. I would seek help either from family members, friends, and definietly the hospital if you feel like you need it for your mental health. Put yourself first. You need this.

[rick]

Brittany,

Thanks so much for sharing your wonderful insight and recommendations. Unfortunately, I don’t have the support contacts most people have. No family, not many friends, and at this point I feel like the only one true support I’ve had out there all these years (my Fiance’) is done with me too. I feel so alone. I don’t wanna sound weak or for pitty but it is what it is, I am pretty much alone right now. I’m actually waiting to get into a shelter right now but they may have too mamy people as it is 🙁 so I’m trying to think of my next step and it isn’t looking good. To tell you the truth… I don’t know if I’m gonna make it thru this thing…

[kathleen-sheffer]

Rick, I’m so sorry to hear you’re in this situation. Brittany made some good recommendations, and I agree you should consider checking into an ER if you’re feeling ill and don’t have a place to stay the night. You can also try to drop in to see your specialist. The receptionist might say they have no available appointments, but if it’s an emergency (and it sounds like it is), your doctor will want to see you. I know you’ll make it through this even stronger than before.

[jen]

I might not belong here because I haven’t been diagnosed. Long story short, after taking Vyvanse (a prescription stimulant) for 3 years, I rather suddenly started having problems with shortness of breath, dizziness, and heart palpitations when I took the medication. I’ve had several EKGs, an echocardiogram, a stress echocardiogram, and wore a ZIO patch monitor for 10 days. My heart, rhythms, and valves seem to be in decent shape and my cardiologist is not concerned. Propanolol (a beta blocker) was prescribed to help me deal with these suspected medication side effects. Propanolol does help but I don’t like the idea of taking a medication to cope with the side effects of another medication, especially if the side effect is very rare and of unknown cause. Also, I feel like my stamina is slowly decreasing even when I don’t take the Vyvanse. I used to feel fine engaging in aerobic activity but now it seems to require more effort and I have mild discomfort that is difficult to pinpoint or describe. I remember asking a medical person if pulmonary hypertension would show up on my echo test and was told that it would (and there was no indication of it). However, I’ve since read that PH can easily be missed. I guess I’m just wondering if my story resonates with anyone and if you have suggestions.

[kathleen-sheffer]

Hi Jen,
Everyone is welcome here! I’m very curious about where you first heard of pulmonary hypertension, given how rare the disease is. Your symptoms do sound all too familiar, and an echocardiogram is not able to formally diagnose pulmonary hypertension, though it often shows signs of elevated pressures. Trust your gut and keep asking. It’s not normal to feel the way you do! Often patients are diagnosed only after seeking a second or third opinion. You might ask for a referral to a pulmonologist if your cardiologist isn’t concerned about your heart. Even when I was listed for a heart-lung transplant because my pulmonary hypertension had progressed to end stage disease, cardiologists said my heart still looked “good!” I’m sorry you are feeling this way. @jimannt44 and @terry-d-blissett seem to have expressed similar frustrations and might be able to give some input in how to handle this discussion with your team.

[jen]

Thanks Kathleen. I will keep asking and I’m also documenting what I’m feeling and when. Re how I first heard of pulmonary hypertension: I’ve been online a lot trying to match my symptoms with a possible ailment and PH seems to match the best since my heart is in good shape.

[kimberly-van-soest]

Hi Everyone,
Honestly I am not much of a talker around my family and friends, but I thought it would be nice to share my story with all of you.

My name is Kimberly van Soest, and I am 20 years old. Unlike most of you, I am living in the Netherlands. I feel like there is not that much information on iPAH in Dutch, So I decided to find more information in English after I was diagnosed 1.5 months ago. That is how I found this website. The one thing that caught my attention is that this forums seems to have more younger adults. In the Netherlands, most of the people diagnosed are somewhat around the age of 40, which is a completely different life stage.

Well this is my story. I have always had breathing issues, ever since the age of 12. I only experienced these breathing issues during playing soccer. At the time, my GP assumed this was asthma, and gave me medication. These meds did not help me. This was the first time I started to think that I did not have asthma. Also, I did not really match with the symptoms. Asthma seems to be predominant during the summer, whereas my symptoms were during the cold winter months. This story continued for over six years, with me getting almost every type of medication available for astham, with zero result. At 18, I decided to quit soccer, because it became more of a struggle every time. My symptoms stayed the same and I thought quitting soccer was the end of it.

That was until about one year ago. When I experienced a new kind of fatigue. I moved to a new apartment, and had to climb two pair of stairs. This was tiring, but I was able to do so. However, the last six months I was exhausted after climbing those same stairs. I never experienced something like this ever, not even during soccer. That is when I decided, to once again, consult the GP. As you might understand, I did not really feel like going, because nothing ever helped me in the past.

Again I got the same types of tests, and given my history with Asthma, they adviced me to start again with ventolin. (I had stopped my asthma meds from the moment I realized they were not helping me)
The GD adviced me to put my prejudices about the meds aside, and that I would come back for another test after one month to see if it had helped and made a difference.

Fortunately, (when I look at it now) I did not make it through that one month. On the 23rd of June, my boyfriend called 911, because I was not able to breath. This was due to a cold. However, it was also my first(!) time in the hospital in 20 years time. After doing some test and making an echocardiogram, they noticed that something was off.

Although I was able to breath like I was used to, they told my I had to stay the night, because my echocardiogram ‘showed something disturbing’

This was a huge suprise for me, because I had the work the following morning at 8. I have to admit, I tried to get out of this hospital stay, but deep down I realized that they might finally find something.

The hospital stay was very impressive to me, because I had never been in a hospital other than being a visitor. Nevertheless, each day I noticed that something was very wrong, because the doctors in the hospital would not even let me walk the hallway without guidance.
The first day already, the doctors told me that I did not have asthma. I stayed in this hospital for 3 days, but when they assumed PH was the problem to all of my problems, I got taken to an expertise centre, in Rotterdam.

Once I was in Rotterdam, the doctors told me about the right heart Cath. This was, as they explained me, the only way to be sure that I did have PH.
Although they were very certain before this right heart cath, I officially got diagnosed (i)PAH WHO class iv on the 28th of June, on my sister’s birthday. Everything then went very rapidly, I got a broviac hickman catheter placed in my right breast, so that I could get started with the veletri therapy. Once I was able to change my medication by myself, I could leave the hospital. I can’t help but wonder, I got diagnosed within 5(!) days, once I was in the hospital. But the funny thing is, in all those years, I was not once send to a professional.

Unfortunately, this is not ‘the end’ of the story. See, we were already familiar with PH. My mother’s sister, my aunt, had a daughter about 15 years ago. She was diagnosed with PAH, just like me, but died at the age of 3. Given the fact that PAH is a very rare disease, and two people in the same family are diagnosed with it, raises the question if this kind is one that runs in the family. The result to that test will be around november, my birthday month. I hope this will not be the case, since our family has gone through so much already.

Although I have learned many things, the one thing that stood out is that of my gut feeling. I always knew that something was off, but I was scared and raised in a way that you do not complain unless something is really bad. Therfore I never asked the GP to send me to a hospital. If anyone reads my story, diagnosed or not, I hope you would have the courage to listen to your body and seek for help.

I got diagnosed on time and with luck, because that cold, in a strange way, might have saved my life.

[brittany-foster]

Hi Kimberly,
I’m so amazed after reading your story. I can so strongly relate to every single thing that you said. I am also in my 20s (27) and was officially diagnosed over a year ago but have been on oxygen for almost 3 years now. Your story is so honest and is a lot like mine and many of those with PH.

When I was younger I also played many sports including soccer and hockey. I had a burning pain in my chest with playing and it never seemed like I was getting in enough air even when I was breathing fast. It made me dizzy, feel tired, but I was able to somehow push through it. Doctors said I had exercise induced asthma for YEARS. inhaler after inhaler never helped me at ALL.

I was then diagnosed as having anxiety, being depressed, and other mental illness because they simply couldn’t find an explanation to my shortness of breath that progressed when I got older. It wasn’t until a nurse walked me around with a pulse ox monitor on that they noticed something was seriously up and my oxygen levels were dropping to 78 with minimum exertion (which is why I have the need for oxygen with this).

PH is such a rare condition and a serious one so after reading your story I’m glad you were placed in the right care from the doctors and that they knew how serious it was. They also did the right heart cath which is great that they got it done so quickly but also must have been so much information for you to take in at once and I can only imagine how stressful that must have been. I also get that even though it’s stressful, it’s somewhat of a relief to have answers and an explanation for your gut feeling all this time.

Thank you so much for sharing with us and you can know that this is a great group for support and to post any topic of discussion that you want! There is also a group for young adults with PH on the forums . Feel free to reach out any time !

[kimberly-van-soest]

Hi Brittany,

It is indeed a lot to take in at once. But I have always been someone who likes to know everything at once, rather than one step at the time. For example, my doctors gave me a lot of papers to read, but failed to tell me in person that the possibility of getting pregnant is almost zero. I have read this in one of the brochures they gave me. To be honest, that was the most painful news. I have always had a dream of becoming a mom myself.

To be honest the best description of how I feel is about the diagnose is numb. At first, like you mentioned, I even felt a sigh of relief because I finally got the right diagnose.

The thing that hurts me the most is my family seeing me differently and me noticing that my family is in pain because of me. They treat me different as well, more fragile. And although I completely understand their response, it does make me feel uncomfortable.

[brittany-foster]

Kimberly,
I completely get it. I don’t like being treated differently and don’t like all the stress that I am putting my family under when I am in the hospital, when they have to take time out of work, when I am sick etc. There is so much worry that goes on for us and for those who care about us. I always remember that this is something that would only be normal for others to worry about, especially the ones that truly love and care about us. If roles were reversed and one of my family members was sick, I would also be equally as worried and concerned about them. It’s so different when it’s us that is the one who has this though. I’m treated like I’m fragile even by strangers when they see me with the oxygen, but when I build up my strength I surprise people with what I’m able to do. I have confidence you will build up your strength and the medications will start to make you feel better than you are right now. It takes a big adjustment and once the “numb” stage has passed you will work on discovering the things you still CAN do and will start to build off of that. Allow yourself to feel sad and upset, worried, confused, mad about the possibility of not having children. I learned that it’s best to feel all of that and express it to people you feel comfortable talking about it to. Bottling those feelings up just creates more stress on your body. I’m here to talk when ever! I know this is a lot and extremely overwhelming.

[kimberly-van-soest]

Thank you for your supporting words. I really appreciate them. If you need anyone to talk to, I am here too. I wonder how you are doing at the moment? I wonder, are you able to travel by plane?

[brittany-foster]

Hi Kimberly,
I apologize for the delay. I am not as active on the site on the weekends as I am during the week so I am just able to respond now!As far as how I’m doing now, I have my good days and bad and sometimes bad weeks unfortunately. I am on oxygen pretty much 24/7 because my oxygen is just very low which makes me feel like I have to push through most days just to get through it. I am working part time as a nanny which is great and an awesome distraction. But at just 25 years old I had to leave my job as a teacher because I just couldn’t keep up. I am able to travel, but planes are very difficult for me because of the pressure on the planes and I end up feeling terrible for weeks after taking a plane ride so I try to avoid them as much as possible. Do you do any travelling? Are you using oxygen therapy at all? Are you able to work?

[kimberly-van-soest]

Hi Britanny,

No need to apologize, just answer whenever is convenient for you. I am asking you because I used to travel quite often by plane. However ever since the diagnosis, my doctor has told me that they strongly discourage me to travel by plane due to the high pressure. They do mention that there is a possibility, if I were to take oxygen during the flight. This does make it harder to spontaneously book a flight, as I normally did. I do not have any oxygen at the moment, as I try to keep off the oxygen as long as possible.

As for working, I used to work until all this happened. It was just a side job, since I am still studying. The doctors adviced me to stop working until my body is somewhat used to the medication. I hope to start working again, around September.

[brittany-foster]

Kimberly,
I know how frustrating it is when you are told not to work or to take a break from work. It’s hard to keep busy and stick with some sort of routine while adjusting to medications. I know that your doctors are telling you what is best for you and your body right now so the best thing any of us can do is just trust that. If the doctor is thinking about you going on oxygen for a flight, I would suggest looking into an oxygen company if your doctor sends them a script. That way you can get a portable oxygen concentrator that is approved by an airline and at least can have that for if you want to fly. It’s always worth it to travel when you are physically able to, even if it means wearing the oxygen for the flight. You should ask about that and getting a script for that from the doctors!

[seagypzy]

Hi, I am Joni. I am a 62 year old nurse from Colorado. I moved here 2 years ago and thought the altitude was the problem. I felt like my asthma kicked into high gear. I had a sleep study last year and ended up in nocturnal oxygen. I went to the ER today with SOB and what I thought was maybe a pulmonary embolism. My resting pulse ox was 86 percent and my lips were blue. I has a cat scan and they measured my pulmonary artery at 3.8cm, I guess normal is 3.3cm. Anyway, they told me I have pulmonary htn. I live an hour from Denver and I am going to make an appt with Dr Bastich. Hopefully he can reccomend a good Cardiologist. This is unexpected and scary. I do feel much better with oxygen. Happy to meet all of you!

[brittany-foster]

Joni,
I know what you are going through is so scary right now. My pulmonary artery is also enlarged due to congenital defects that I was born with of the heart. It is worrisome to have oxygen levels that are lower than normal and notice things like blue of the lips. Unfortunately those are some of the warning signs that oxygen is needed so I am glad that they are giving it to you and that is helping you feel more comfortable. Do they think you are going to leave the hospital with oxygen? Are they running more testing when you are in the hospital or as an outpatient? I would suggest making sure to see as PH (pulmonary hypertension specialist) or a pulmonoligist that has seen patients with PH. Also, a cardiologist is a good doctor to have as part of your care team as you are followed for this. The standard for diagnosis of PH is a right heart cath where they measure the pressures in your heart and pulmonary artery to make the final diagnosis. PH may be expected as a diagnosis from scans and from echos (ultrasound) of the heart, but the right heart cath is what confirms it. Please keep us updated on your progress and what is going on ! Thinking of you and wishing you well!

[donnah]

Hi, Joni! I’m also in Colorado, although north of Denver rather than south, like you are 🙂 I’m also new to PH; was in Estes Park in April hosting a women’s retreat when I apparently had a small stroke, became extremely hypoxic, and have never been the same since. A bit of pneumonia, mild atelectasis, and it took me off a cliff, as my doctors say. Now I’m up to 3L continuous 02, and drop into the low to mid-80s very easily without it.

So, as far as altitude…I know you’re about 1k feet higher in the Springs than I am in Weld County, and I also know I have not tolerated an increase in altitude well at all this summer. One trip to the National Park was very difficult, and I’ve been to about 7200 feet twice since I got sick, and needed to up my oxygen every time. It’s so hard to live in Colorado and not be able to go to the mountains like I used to!

I hope you can find a good cardiologist/PH specialist; my PH doc is actually a cardiologist, although I’ve been bouncing back and forth between pulmonology and cardiology. I have though, like Kathleen suggested, thought of traveling to sea level for a while to see if I felt better, but I have a large family here in Colorado, and it would be so difficult to move away from all my children and grandchildren. I am hoping for some improvement with medication; I’ve been told by several people in this medical community that they’ve seen huge improvements in many of the patients that come in to see my doctor. Thankfully, our doctors are used to treating people at this elevation, so if anyone knows how to do it, they will!

Hang in there…I was an RN in another lifetime, (now working part-time as a church administrator), and I know it would be difficult to be on oxygen while you’re working. Can you find a position that would be physically less demanding, such as teaching?

Best wishes to you! There are several others here from Colorado…hopefully you’ll hear from them, as well!

Donna

[brittany-foster]

Hi Donna,
Thank you so much for your comments and your words of advise. I know they are greatly appreciated ! I can only imagine how hard it must be and the extra strain that is put on the body when being in higher eleveation. I took a trip and stayed at the top of a mountain at a resort in Canada this past May and felt the difference x10 when I came home, even though it was a short period of time. My chest felt congested and tighter and my heart rate was faster than how it usually was. I ended up having to turn my oxygen up quite a bit and had JUST ENOUGH tanks with me for the trip (thankfully!!!) It just made me think of my fellow PH friends like all of you who live in the higher elevation. It makes me happy to hear that you really trust your care team. I’m sure they acknowledge the elevation as being a factor in all of this too! Much love to you and thank you again for your responses.

[seagypzy]

Thank you, Brittany! I have a large condenser at home that I use when I sleep at night. They sent me home, but I am going to buy a pulse ox and monitor my saturation levels throughout the day when i am off oxygen. Do you have any suggestions for a good brand? I wonder how accurate these are.
I have been feeling so sluggish and out of it this last year. Now the beast has a name. I am questioning if the altitude in Colo Springs is making things worse for me.
I called and tried to make an appt with pulmonology but they want me to see Cardiology first. I have an appt in two weeks. I might have to be on oxygen at work,too, and i am not sure that is possible. This has put my whole life in perspective and it is amazing how focused you can become on your health with a diagnosis this serious. I keep denying it but nobody healthy walks around with a sat look level of 86 percent.
Thank you for all of the great suggestions. I am grateful to be part of this forum! Joni

[kathleen-sheffer]

@joni all the monitors I’ve used have been slightly off, but not by too much that it really matters. I’d recommend bringing the monitor you buy to appointments and simultaneously comparing it to the hospital’s machines. I have this superstition that the hospital’s monitors are always lower than the ones I use at home. by comparing the two you will know for the future that yours is off by 2 or 3 percentage points, or whatever it comes out to be. I’d be concerned if it is off by 5% or more and try a different model.

My doctor didn’t want me to travel above 4,000ft elevations. Obviously there are other PHers living at higher altitudes, but in my anecdotal experience, those patients tend to suffer. Of the three PH patients I knew who lived at altitude, all three have had lung transplants.

Having spent a lot more time at altitude since my heart-lung transplant, I know it can be hard even for healthy people. I was at 5,800ft earlier this week and had a headache most of the days and felt more winded going up and down stairs. Maybe you can take a vacation to sea level and see if you feel significantly better?

Love your attitude about naming the best – you will get help now that you know something really is wrong.

[brittany-foster]

Hi Joni,
I am glad that you found us here on the forums. Like Kathleen said in the above comment, I would ask the doctors what brand they prefer. Usually you could even ask a pharmacist and they would know what is best. I use the CVS pharmacy brand and it is always the same as the doctors one or give or take a couple points, but if my sats are low it captures it. It also monitors how strong of a reading it is, the better the reading is, the more accurate it is. (There’s a line that goes up or down depending on how good the reading is). Things like circulation problems or cold fingers can throw it off and give a poor and inaccurate read. Another way to tell about good readings is if you compare the heart rate that it is giving you to the heart rate on a monitor or a heart rate that another device measures. If they match up than chances are it’s accurate. That’s how they did mine for accuracy at the doctors when they were shocked it dropped to 78% in a matter of a minute.

I know all of this is so scary and overwhelming. We have all been there before and we all want to support you ! You are doing all you can! It is a process.

[kathleen-sheffer]

@brittany-foster that’s super cool that it gauges how accurate the reading is!

[seagypzy]

Thanks, Kathleen and Brittany, I am going to buy a pulse ox tonight. I am focusing on losing some weight. There is a lady that cooks delicious low calorie meals in bbulk. I started getting them today. I am also thinking about early retirement and moving back east. I have family support there, too. Thanks to you both for sharing your knowledge. You have been living this. I am curious what type of PAH is causing all this havoc. :(. It looks like there is different treatment for all of the fdifferent types of PAH. Knowledge is power 🙂

[kathleen-sheffer]

Sounds like you are on the right track with your goals and considerations for the future. It doesn’t all have to happen at once so make sure to give yourself time, and credit for what you are doing.

There are different types/groups of pulmonary hypertension (PH), but pulmonary arterial hypertension (PAH) is one of the five groups. Most likely you have PAH/IPAH – idiopathic (I) in front of the PAH means they’re not sure what caused it. The other groups are caused by underlying diseases that remain the focus of treatment instead of the PH. We’re still learning and defining the disease so it can get confusing. Glad you are connecting with other patients!

P.S. This is a great infographic on the types of PH.

[brittany-foster]

Hi Joni,
yes it certainly depends on each individual case. For me, mine is secondary and caused by a condition I have where my brain doesn’t receive the proper signals to breathe. This has caused my lungs to be pretty restrictive over the years and as a result I have significant respiratory muscle weakness. For me, the only treatments I am on are medications for my heart, medications like steroids to reduce inflammation, 24/7 oxygen therapy, and bipap therapy with oxygen at night to help when my body doesn’t breathe as it should which causes the higher pressures in my artery and more pressure on the heart. Everyone’s story is so fascinating to me because we are all so unique!

I’m glad you are taking he right steps to live your best life with the cards you have been dealt. It is always a good idea to optimize nutrition and get on a healthy eating track! I’m sure this will help you feel as better as you can 🙂 Keep us updated on your progress.

[seagypzy]

Thank you! I am a women’s health nurse, so i had to dig deep to remember my cardiopulmonary lessons.
It is good to see that Donna is a nurse, too. I am sorry about your stroke.Hopefully my Dr will be able to help me like yours has helped you! Hypoxia seems to just creep up on me. In the ER my lips were blue and I started to vomit. It is strange that I have a medical background and sometimes I am not aware of what is going on in my own body. I hope I will be able to realize I have a problem a lot sooner next time.
Thanks for the link, Kathleen!

[brittany-foster]

Joni,
recognizing our bodies warning signs comes with lots of practice and doesn’t happen overnight ! It’s amazing to me what I have been able to push through and “ignore” over the years. Turns out, I shouldn’t have ignored it at all! I always wish I could have talked about my health concerns earlier to a doctor but went about my whole life thinking it was “normal” until someone told me it wasn’t and actually monitored how low my levels were getting upon exertion. It’s funny that I always complained of shortness of breath and feeling overly tired after exercise but it was just chalked up to “asthma” without anyone ever putting a pulse ox on my finger. The doctors that I am seeing now have said “that is the first thing we do, even for young children complaining of this”. I now use this information to help others and spread awareness about the 6 minute walk test !

[andrew-michael-rossi]

Hi Everyone!
My name is Andrew Michael Rossi, I’m 67 and another retired nurse! I was diagnosed with a R&L card cath on my birthday ( 7/25) and my regular pulmo believes from the report he sees I have pulmo arteriel and venous hypertension with some mild left heart failure. Now I see Dr Neal Chaisson at the PH Clinic at Cleveland Clinic on Tuesday 9/4. Not sure what to expect but getting that appointment so quickly has me assuming this pulmo hypertension thing is something to be seriously taken care of with speed. Just curious to know did anyone go for their initial dx appointment and then get admitted to the hospital? I have been having terrible SOB on exertion esp bending over or showering. I can still do my treadmill for 1 hr each day at 2.5 settings ( well most days). I’ve been experiencing some nausea lately, edema, wheezing, a wicked dry cough and at times dizziness. The diueretics have pulled about 3 liters of fluid out of me in the past several days and I have dropped from 168.6 lbs to 161.0 this am. Now I am feeling like the fluid is building up. I have been having bizarre chest discomfort. Its Like a burning pain or then a dull pain like under my left axilla. Pain in my back around my thoracic region. Am I experiencing the joys of PH? During my evaluation for transplant, it was discovered that I have abnormal kidney functions and rated CKD Stage 3. I never knew I had this but it bumped me off being considered for listing at Temple in Philly. Cleveland Clinic kicked me to the curb as well.
So back to my story. In 2008 I was exposed to a toxic chemical which put me into respiratory 3 times. The third time the docs ( back in Boston at BIDMC) did a tracheoplasty which helped my bronchus and trachea from being collapsed. It was extensive and the transplant surgeons are nervous about doing a transplant. Oh so after the trachea surgery I went home and developed a high fever and breathing so bad that I went into respiratory arrest. My VNA nurse had already called 911 and the medics bagged me and got me to Yale Medical Center. (I was working at the VA Med Cntr and going to Central Conn State University for my Masters) Turns out I somehow got mrsa in my lungs which literally cavetated my lungs and put me in end state lung disease. They pretty much said go and get your affairs in order and I had other plans. I slowly improved, did pulmo rehab) weaned myself off oxygen and my meds so that when I walked into my pulmonologists office (we’re in Ohio now) he was flabbergasted, I did well for about 5 yrs then I needed the O2 for bedtime then upon exertion and then 24/7 Going from 3L to 6L now. In addition, the prednisone has induced diabetes ( for joy!) I have a weakened immune system, plus I have an autoimmune disease called Sjogrens Syndrome. I’ve had cataract surgery, and now developing glaucoma,hypothyroid, low T, etc In total since 1990 I have had 24 surgeries. Oh yeah, I have a Baha cochlear device to hear on my left side since I had Ramsey Hunt Syndrome which kills your acoustic nerve and been living with that since I was 30 yrs old. My spouse, Jack and I have been together for almost 40 years. We have a cantankerous little poodle dog named Sammy from Miami. I’m, pretty positive in my outlook on life. This too I shall beat into submission I still have a good 20 yrs or so to do some serious living finally!

[brittany-foster]

Hi Andrew,
Wow! You certainly have quite the story! I’m glad that doctors were able to act quickly when you were going through all of that. I am also glad that you will be seeing a PH specialist and they got you into seeing someone quick. If you have worsening symptoms, never hesitate to call the office. Even if you haven’t had an appointment with that particular doctor. Sometimes they are able to see you the same day or at least get you properly evaluated. Like you, i experienced many of those symptoms especially the pain near the rib cage. I had a lot of shortness of breath and am currently on oxygen able to adjust it as needed. When i get any type of infection it immediately attacks my lungs and really makes it harder and harder to get back to my baseline. I understand the worry and wondering what these symptoms are from and if they’re all related to PH. I know your doctor will be able to give all information about this when you see them! Keep us posted and best of luck at your appointment!

[andrew-michael-rossi]

Hi Brittany, Thanks for the warm welcome! Its odd, I was reading an email from my regular pulmo doc and he said he wanted me to see the PH clinic docs to see if my mild pulmonary hypertension is worth treating. I thought to myself “what?” do we wait until it jumps from mild to moderate? From experience can PH emergent issues happen lickity split? I hate to be crying wolf everytime I think something is going wrong. Also I was speaking to my nephew who is an ER doc and he said PH is not a rare disease. So who and what do I believe? Guess I’ll have more answers tomorrow. I’ve been feeling pretty crummy the last couple days, so I’ll be glad to see the docs. Oh by the way, do you have a cardiologist as well? Mine happens to be an interventional cardiologist who does cardiac caths. I’m due for my annual checkup and was curious to know if there was some subspecialist who worked with PH docs. Hope I get to meet all the other people in this group. Ciao for now!

[brittany-foster]

Hi Andrew,
PH is considered a rare disease. From my own experiences, I have had flare ups of my PH symptoms due to illness, weather, lower oxygen levels, extra bodily stress or more physical acticity etc. I’m sure once you see a specialist you will feel more comfortable or at least less worried about what you are experiencing. The symptoms definitely can get worse some days and thats just part of PH for me and many others. There are good days and bad and flare ups. I do see other specialities along with a PH doctor because of my underlying conditions that contribute to PH. I see an electrophysiology doctor for my arrythmias and pacemaker, a GI doctor for issues related to PH, neurology, cardiology, sleep medicine doctor for my sleep apnea etc. They are all in the same hospital as my PH team so it makes it easier to share info and communicate. I would do your best to coordinate care and make sure the doctors are talking with one another! If possible, have as many specialists as you can at the same hospital.

[melainie-garcia]

I am Melainie Garcia. I am 54 years old and was diagnosed with PH when I was 43.

I gave birth to my one and only child in 2007, when I was 42. I went into this pregnancy with some concern because I have a congenital heart disease. I got the blessing of my cardiologist and found a high-risk pregnancy OB-Gyn and after a miscarriage, became pregnant with my daughter in about 15 months of trying.

The pregnancy was difficult from about the 5th month on. My daughter was fine, but I was not. Swelling, SOB, PAIN PAIN PAIN! I used motorized carts from month 5 to keep my independence when I went shopping. By the end of my pregnancy, I was falling asleep almost any time I sat down and I would cough up pink-tinged froth at night. It was rough. But then my daughter was born. She was perfect. Three days later I was admitted through the ER with heart failure. A week later I was home, having lost 26 lbs in water weight and feeling really good. Thought it was all behind me. Nope.

In a year’s time, I was diagnosed with asthma, pulmonary sarcoidosis, severe sleep apnea, and finally pulmonary hypertension. It was a VERY difficult year. I am on a low dose medication for anxiety-depression. It helped me get out of the strongest depression period I have every experienced. I don’t feel as deeply depressed any more, but this/these chronic illnesses can really do a number on your head.

It has taken 9 years to get my medication/self-care/oxygen needs to get me where I am. I do well most days, but I bet if I traded bodies with someone for a day, they would be thinking WTF! Our “normal” is far from normal “normal”. Just today, I was watching a man about my age walking effortlessly in JCPenney. It amazes me that people don’t feel the way I do. I barely remember what feeling the way they do, feels like anymore.

Over the years I have picked up a Bi-Pap machine, two concentrators (one for home and one for work) a defibrillator, a Melody valve and my constant companion AirReal, my oxygen tank.

[brittany-foster]

Hi Melainie,
Thank you for introducing yourself to us. It sounds like you went through such a scary time in your life around a time where it is supposed to be all about happiness and having a baby. I’m so glad that you were blessed with a child, but I’m sorry that you had to go through so much discomfort and suffering to get there. I can’t even begin to imagine the worry that must have caused you not only worrying about your own life, but the life of a baby! It truly is a testament to your strength and perseverance during that time. I have congenital heart disease too so I know how hard it is to hear the risks that are involved with a pregnancy. I’m sure there are others who found out about their PH after giving birth or had more symptoms during their pregnancy if PH was undetected up until that point.

I’m thankful that you have the medical devices that you need to help make your daily life a little more manageable. I know the feeling of seeing people who are your age doing things that you wish you could be doing. It’s hard for me too to see my friends who are in their 20s living their life without the same worries and concerns that I carry with me.

It’s good that you are open with talking about your mental health because mental health is an important piece of managing a chronic illness too and medications can really be a game changer for some ! This is A LOT to deal with and that’s okay to get help!

Thanks for the introduction and I’m sure we will be chatting more on here!

[amy-peeples]

My name is Amy Peeples . I was diagnosed 3/8/17. I had been very very sick for a really long time . My pressure was 168 at dx and I remember my doctor saying my heart was grossly enlarged during my first RHC. I went into icu and went on sub q remodulin , Letaris and addcirca . As well as the diuretics .
I hated sub q and was very persistent with each site change that that would be my last .
Six months after diagnosis I had a second RHC done and my pressure had dropped to 38. With in 24 hours I took off the sub q and was on full dose uptravi and have been fortunate enough that I live my life with out any symptoms of PAH as long as I take my medication .
It has been a long journey and there was a time before dx that I thought I would be lucky if I lived a few more weeks . I’m very grateful to be alive and well and just want to make the most out of what ever life has left for me .

[brittany-foster]

Hi Amy!
I know we have connected on other forms of social media but I’m so glad to connect with you here too. It’s amazing that your pressures went from being so high to now in the upper 30s. It’s remarkable what medication and a strong will can do ! I’m sure your body was NOT ready to give in to this condition that easily and even though there was so much pain, you did what you had to do in order to survive. I really admire you for that. I hope that the medications keep allowing you to get the most out of life ! 🙂