What's the typical life expectancy for someone diagnosed with pulmonary hypertension?
My husband was diagnosed with pulmonary hypertension last year. He hasn't quit smoking or lost any weight. He is still active when he takes his meds regularly. He is 47 and I am angry that he may not see 50 because he won't do what his doctor has said to do. What's the typical pulmonary hypertension life expectancy?
The life expectancy for someone with (secondary) pulmonary hypertension is favorable if they get the proper treatment. Pulmonary hypertension develops because a person has some underlying medical problem that somehow effected the blood vessels in the lungs, causing the blood pressure in those vessels to rise. Many lung and heart conditions can lead to this, as can smoking. Since your husband is still smoking and not following the advice of his doctor, you realize he is not helping his condition. I understand that you are angry and frustrated that he doesn't want to take care of himself. My advice:
- Don't nag him. It will not make him want to listen to you. It will shut him down more.
- Really listen to him. Has he ever said why he cannot stop smoking? Has he said the meds make him feel bad or have side effects he doesn't like? Has he discussed any of these problems with his doctor?
- Get help for yourself. You are angry because you think he doesn't care about you enough to take care of himself. Perhaps it would help you to talk with a counselor or join a support group. You need to deal with your feelings, or you may end up getting ill yourself!
In all my experience as a nurse, one thing I do know is that you cannot make anyone do something that they do not want to do themselves. I know that it is hard, and understand that I know how frustrating this must be for you. I don' think your husband even realizes that his is hurting you. I believe he is trying to deal with his illness in his own way, and really doesn't know what to do. So, his defense mechanism is to not do anything at all.
I would also recommend contacting his physician's office to see if they have any further advice on how to deal with this. They may even be able to direct you to a support group in your area that might help you. I wish you luck!
I was hoping to find out the answer to the above question regarding the lifespan of a PPH patient so have posed it again; although a little different circumstances. What is the estimated lifespan of a 29 year old female with primary pulmonary hypertension stage 4 taking flowlan? Thank-you
Life expectancy is a basic concern, so I would like to have seen that part of the question addressed, rather than tossed off. If there is no data on life expectancy, write that. If SPH doesn't necessarily shorten a life--unlike PPH--that would be at least a partial answer. The rest of the answer helps the wife to cope. I came here searching for the same answers about my SPH, so I'll go back to the google indexes.
For Primary Pulmonary Hypertension, The average is three years. We must remember that there is a lot bringing that average down due to not ever knowing they had the disease. My friend died after just six months of diagnoses. so you never know.
I was diagnosed with IPAH as a stage4 case in my early 20's. I lived for a little over 5 years with the disease. Then I needed a lung transplant. I know people who've lived with it for 13 years before they need one, but some people need it sooner.
This was very frustrating. I am so tired of needing real answers and getting the run around. This is too important of a question to be handled this way. The question was "What is the life expectancy of someone with pulmonary hypertension?" I know there are many variables, but those could be discussed in the article. Again, it feels like someone has played a cruel joke on me. No the article was not in any way helpful, and actually it was hurtful. First of all, do no harm!
here it states untreated the life expectancy is about 3 yrs, treated is 10+ years. there is no exact time frame. as with many sicknesses there are to many variables to give an exact time frame. so the best answer is about 3 years with no treatment.
check out the link i posted, its a short article but i found it helpful.
I see a nurse practisioner, she told me that she did not feel comfortable enough to treat this, so she is referring me to a cardiologist, if she won't treat me; I will see a pulmonologist. Go see the cardiologist first and see what they say. If they can't help, then ask to be referred to a pulmonologist. Okay? Until then, keep your cool.
WITH REGARDS TO HOW LONG YOU HAVE IT CAN RANGE FROM 6 MONTHS TO 3-10 YEARS IT ALL DEPENDS ON WHAT STAGE YOUR IN WHEN YOU FIND OUT AND HOW BAD THE DAMAGE ALREADY IS. IN MY CASE I WAS ALREADY IN STAGE 3 BY THE TIME I FINALLY FOUND OUT WHAT I HAD BY THAT TIME IT HAD ALREADY INVOLVED BOTH LUNGS AND RIGHT SIDE OF MY HEART. AS FOR TREATMENTS, WELL THEY ANYTHING AND EVERYTHING THE DOCTORS COULD THINK OF. ALL IT DID WAS MAKE ME SICK ALL THE TIME, AND REALLY MESS WITH MY QUALITY OF LIVING, SOMETIMES IN LIFE WE JUST HAVE TO MAKE A CHOICE QUALITY OR TIME I TRIED TO BALANCE BOTH WHICH WORKED FOR SOMETIME, BUT IT REALLY COMES DOWN TO HOW YOU WANT SPEND YOUR TIME. IN MY CASE THE DOCTORS SAID I WOULD NEED A HEART LUNG TRANSPLANT AND IN OCT 2011 I WAS FINALLY PUT ON THE LIST, BUT BY THAT TIME ALL MY OTHER ORGANS, MAINLY MY LIVER WAS DYING I SPOKEN WITH SEVERAL DOCTORS AND THEY SAY ABOUT THE SAME THING THAT NOW I'M IN THE LAST STAGE 4 AND I NEED GET MY FAMILY READY. EVEN BEFORE TALKING TO THE DOCS I ALREADY KNEW I WAS GETTING CLOSE THEY TELL ME I WILL BE LUCKY TO MAKE IT 2012 WELL WE WILL SEE I HAVE 2 SONS AND 3 GRANDCHILDREN AND I HAVE FOUND THAT THEY JUST DO NOT WANT TO ACCEPT THE FACT THAT I'M NOT GOING TO BE AROUND MUCH LONGER, MY YOUNGEST GRANDSON WHOM I SPEND THE MOST TIME WITH KNOWS I'M SICK, AND HE SEEMS TO UNDERSTAND MORE AND ACCEPT IT MORE THEN ANYONE ELSE, AND HE'S ONLY 7 BUT HE HAS ALREADY LOST 3 GRANDPARENTS AND HE THE ONE WHO TELLS ME IT'S GOING TO BE OK. I THINK THAT'S THE HARDEST PART WHEN YOU KNOW YOU ONLY HAVE A SHORT TIME LEFT KNOWING THAT YOU WON'T BE THERE FOR THEM. I HOPE THIS HELPS SOMEONE SOMEWHERE
i HAVE an autoimmune disease that I have lived with very similar to Lupus. I now have had a diaognosis of Pulmonary Hypertension(secondary). I am trying to understand what lies ahead for me. I know and feel the quality of life being diminished at this rate slowly. I do believe I am in the earlier stages, but I also have Diabetes, Dermatoimyositis, Cushings, Renaulds and Rhumetoid Arthritis. I am 61 and a grandmother of three. Your letter did help me and I want you to know that. Thank You. I too believe that quality of life i important and I do not want to hinder or hurt my family with rising medical bills and prescriptions. I wish you well and I hope your words are good sources of encouragement for others
I AM 57 YEARS OLD IN 1996 ON MOTHERS DAY WHILE IN ICU FOUND OUT I HAD PPH. WHAT SO WHAT IS PPH I ASKED. I HAD A CENTRAL LINE FRESHLY IN WITH FLOLAN RUNNING THROUGH. WITH LOTS OF QUESTIONS AND ANSWERS I DID NOT UNDERSTAND I GOT LOST IN MY OWN WORLD.IT CAME TIME FOR ME TO FACE THE REALITY THE CENTRAL LINE WAS COMING HOME WITH ME ALONG WITH FLOLAN WHICH NEED TO BE MIXED DAILY,CENTAL LINE CHANGES, CLEANING THE PORT,THIS AND THAT WHICH I UNDERSTOOD NONE OF IT. THANK GOD I RASIED A STRONG DAUGHTER, WHO HAD A ONE YEAR OLD SON AND A JOB. WHEN LEARNING SOMEONE HAD TO LEARN HOW TO MIX MEDICINE AND HOW TO CARE FOR ME BECAUSE I CANNOT LEAVE THE HOSPITAL UNTIL THEN SHE NOW TOOK ON JOB OF KEEPING MOM ALVE. THE LOVE OF A DAUGHTER. NOW 16 YEARS LATER, I WENT FROM FLOLAN 8 YEARS AGO TO TRACLEER. TRACLEER IS A PILL I TAKE TWICE A DAY. IN 1996 WE THOUGHT I WOULD NOT SEE MY GRANDSON START SCHOOL,NEXT YEAR I WILL BE AT HIS HIGH SCHOOL GRADUATION. I AM GOING STRONG.PULMONARY PRESSURES ARE GOOD AND A STRONG HEART. I WANTED MY GRANDSON TO KNOW HIS GRANDMA I WANTED TO LIVE SO I CONVIENCED MYSELF I COULD BEAT THIS AND STILL LIVING WITH PPH I HAVE A GREAT LIFE. SO THERE IS REALLY NO NUMBER THAT CAN BE PUT ON HOW LONG YOU WIL LIVE WHEN HAVING PPH. MY ADVICE IS LOVE YOUR CHILDREN AND GRANDCHILDREN AND FAMILY TO THE FULLEST AND THANK GOD AND HIS ANGELS EVERYDAY. I REALLY HOPE MY STORY WILL HELP EVERYONE LIVING WITH PPH SEE THERE ARE BRIGHTER DAYS AHEAD AND TO EMBRACE THEM.
My daughter was born with congenital aortic stenosis n had her first of 4 open heart surgery at 6 days old. the last was at 8 yrs which reduced her hypertention from around 120 pressure to in the 40's. she had been doing well until early 2009 she stopped breathing on us and was revived by my husband. in feb they told us that it had returned and dont know why? they tried viagra n a newer drug that i cant remember. then in july 29 her pacemaker stopped so back to bris hispital then had to wait 2 wks cuz the only dr that would touch her had to go to japan for a wk, and then they also had to book a peadiatric anethesist so that took anothet wk!! by then she was very unwell with het liver and servere restless feet. i walked in with her to theatre, kissed her told her i loved her, she nodded smiled n went to sleep. that was the last time i saw her beautiful blue eyes n cheeky smile. at 6pm on 7th aug 09 we lost our beautiful ash. She only was 16.
When I was diagnosed in 2003, I was told that I have only 3 years to live. I am still here. Please see how i am living with pulmonary hypertension day to day. http://livingpah2004.hubpages.com/hub/Natural-remedies-for-high-blood-pressure-in-lungs-Pulmonary-Hypertesion
I don't know if this will help anyone one or not but I see a lot of people asking how long u can go on livin with PPH,well as of 11/27/2012 my sister in law lived with it for 14 yrs, this took a hard tole on her body, but never he spirit or will to go on,she got to see her youngest child graduated last yr, then from there it was down hill,RIP Nancy Johnson you will be missed but we know u are no longer in pain and when u fell into ur moms arms and passed we know your dad was there to welcome u into his, we love you,,,,,,so I can say 14 yrs is a good number for PPH .... Thanks
I have had PAH for more years thatn I know, but was diagnosed about 6 years ago. My doctor is totally nonchalant about it all - he has never told me that this is a disease that can kill me. He just keeps testing me every 6 months to see if things are worse. I had no idea that most people only live a few years, but then again, I had lived over 50 years after open heart surgery, and no one else has either, so my husband calls me the exception to ervey rule! But it would be nice if my doctor would be upfront about this. If I only have 5 years to live, I want to retire. I don't want to spend the aslt few years of my life stressed out all the time from my job.
I was DX'd in 2007...they rushed me in, and installed the Flolan pump. I was on that for a year and a half, then they dropped me to Remodulon, for another 1 and a half. 7 years later...I have no line, and am on Tracleer and Revatio. I am doing fine...I really have no problems...I get around on my own, I do everything I want to. I am 38. I think it's totally case by case...and I think it's definitely important, that if you think something is wrong...get to the doctor...do what they want to do...let them treat, I know a cath is very invasive and scary...but it will save your life...Flolan is like rocket fuel. My heart pressures were way up there, around 120...last time they checked I was down around 65. But, they say that that is not their main concern...they look for wall thickness of the right aorta. I expect to be here in another 7 years...and maybe 7 after...
My husband is 46 and was diagnosed in May 2012. He is on oxygen 24/7 now and is in a wheelchair since his last heart attack 3 weeks ago. he has good days and bad days. His is only getting worse. Im sorry your going thru the same thing. I wish we could talk to each other. I will pray for you.
I was diagnosed in February 2012. And now I'm on oxygen 24/7. The Dr put me on Remodulin ,Tracleer, and Adcirca along with other medications due to my congestive heart failure. I'm 46 sometimes I feel so robbed from family unable to participate in activities, my job which I had to give up after 24 years. In my thoughts I wonder if I will be here to see my children finish school, college and beyond that. I pray for everyone that has PAH and focus on whats good in your life.
I am still going through the testing process. I can tell you this in 2010 I had the same symptoms that put me in the hospital in 2013 and both times PH was suspected. These last 3 months have been doctor after doctor an I have decided my granddaughters are worth me being here, so I take my meds and inhalers and we WALK 2 miles every night after dinner! I push both those kids in a stroller I'm losing weight, spending time with my girls!! I am going to live to see my great grandkids!! At first I felt I was given a death sentence, but I have something to live for . . . MY GIRLS and ME!!! God bless all of you!
I suffered symptoms in Sept. 2006, was dx in Nov. 2006, needed Flolan Cath right away, Flolan plus many other treatments worked for just under a year. I wad hospitalized in Sept. 2007, on life support by Nov. and on Dec. 7 was given 5 days at most....on Dec. 12, 2007 I received a double lung and heart tx! I am doing amazing and thank God for my miracle.
Much of what is written here is very outdated. Please go to the PHA (Pulmonary Hypertension Association) headquarters phassociation dot org for the most up-to-date information about all forms of PH... there is just SOOOO much misinformation on the internet. First, PH & PAH refers to a myriad of dx and it is not possible to give even a vague idea of an "average" lifespan... too many variables. There's been a ton of research in the past 8-10 years and new drugs seem to come out every few months (or weeks)... though there are no drugs for PH patients with left heart involvement. Many patients (and family members) do not seem to understand this and many seem to feel their doctors are somehow not treating them, but the fact is, the drugs used for most PH patients are very harmful for those with left heart involvement. Anyhow... not to sound 'preachy', but... please go to <www.phassociation.org> to get lots of reliable information... including names & phone numbers for every PH Specialist in the USA... and many in other countries. There are also chat rooms and discussion boards for asking and answering PH questions. And, for heaven's sake, do not let just any doctor do a RHC (right heart catherization... which is THE ONLY method to accurately diagnose PH) because it is almost 100% true that the PH specialist will want to do their own RHC... they look for somewhat different facts than regular cardiologists and pulmonary doctors. Also, several people made mention of how dangerous and invasive the RHC (rt heart cath) is... however, patients MOSTLY do not feel any pain and the entire procedure is not nearly so scary as many would have you believe.... just be positive you have an experienced PH Specialist either doing the RHC... or on your team. Good luck... PS Most people dx'd with PH (and treated by a PH Specialist) have a much longer life span that some articles on the internet would indicate. The majority of the hundreds and hundreds in the PHA organization have been dx'd at least 6-10 years ago with many living with the diagnosis for 20-25 years. It is NOT the death sentence it once was.
I've had secondary Pulmonary Hypertension since 1998. In 1978 I had open heart surgery for a hole in the heart (Atrial Septal Defect Repair). This was a congenital defect but not discovered until I was 26. The doctors attribute this heart defect to my PH. My pressures were 112 when the PH was first diagnosed. Over the years they've gone as low as 68 and hover in the 80's - 90. I moved from St. Louis, MO to FL in 1999. I feel this move saved my life! Getting away from cold weather, getting to Sea Level and Lowering Stress have helped enormously! In 1998 I was on Oxygen 24/7.... After moving to FL I was able to ween myself down to only using the oxygen at night. In 2012 I started taking Tracleer; 125mg twice a day. I can't honestly say if it is helping or not. I've had a lot of headaches and other side effects. I get a blood test every month for Liver function and Anemia as the drug can cause problems there.
My main recommendation is to get to Sea Level as higher altitudes take a tole on your breathing as does cold weather. Stress also affects PH so do what you can to lower that. Eating healthy and getting some moderate exercise helps also. Yoga and Meditation are good.... I've gotten a lot of personal help through Eckhart Tolle's books, talks & dvd's on being present and in the Now. Check him out!
My sister was diagnosed over 5 years ago. She will be 61 in February 2014. She takes a few medications, and her doctor says prognosis is cautiously good. Ten years or more ago, 80% of people with this condition died within a couple of years. Now, 80% can live if they take the diagnosis seriously and follow their doctors' advice. My sister gets exercise when she can, gets together with friends as often as possible, and is looking to get a new pet. I expect to have her around for many, many years.
"No one can predict how long you will survive with primary pulmonary hypertension. This is especially true for patients who respond to treatment, as this improves the outlook, according to Dr. Nabili." This is from the article someone suggested for an answer to the "How long do I have..." question. I see several people say they're very irritated and think they're getting the 'run around' instead of an answer, but... honestly, I've said it before... there is no way to come up with an accurate 'average' because of the huge variables with this disease. As I stated in an earlier response, MOST of the research quoted here is WAY outdated and scares people to death. The past ten years have brought a plethora of new & effective treatments plus many additional PH Specialists. IF any doctor diagnoses you with PH, the best thing you can possibly do is read the Pulmonary Hypertension Association website, find a PH Specialist on their list, and see ONLY a PH Specialist for PH treatment. <www.phassociation.org> The disease is too rare for even most pulmonologists to treat... and certainly any other doctor is just 'poking in the dark'. Just as you would not choose to have thoracic surgery from an orthopedist, you do not want to choose anyone other than a PH specialist to treat PH... period. No exceptions. The RHC (Right Heart Catheterization) is the ONLY approved diagnostic tool to specify PH. An echo is only an estimate. It is advisable to not have the RHC until you see the PH specialist since they will likely want to do their own RHC (or have a colleague do it).
Good luck to all, but please do not depend on someone's estimate as to how many years you have left to live. Your situation is likely nothing like the other person's. IF anyone can give you an accurate assessment, it will be YOUR physician... and most of them won't touch that question with a ten foot pole. They know all too well the variables. BTW... I was also given the ubiquitous "2-3 years' estimate when I was initially diagnosed... 13 years ago. It isn't anything other than a number on a piece of paper. And every day the chances for a longer life get better... another new drug was announced just last week. :)
Read more: http://www.ehow.com/facts_5925986_life-expectancy-primary-pulmonary-hypertension.html#ixzz2lbPJ5nJj
My mother was diagnosed with Primary pulmonary hypertension in 1991 when i was a year old. After going to a cardiologist, he told her there was no treatment and she should go home and enjoy the the time she had left. Well she was not going to take that for a answer. She found a doctor in chicago named stuart rich who has dedicated his life to this condition. He is the top doctor for this rare disease and also teaches at the university of chicago school of medicine. He has written many articles and continues to be a leader in developing life saving treatment plans for his patients. He saved my mothers life 24 years ago and continues to provide optimism for patients who are often left with a doom and gloom answer from a doctor who has little knowledge of this disease. I encourage all of you to go to doctor rich for optimal results and care. We live in florida but will be making another trip to see doctor rich soon due to her having some recent issues. You can not put a price tag on your life and you should get treatment from a doctor who is used to treating this condition. So i would not take any doctors word you will only live a certain amount of time. If that were the case my mother would have passed 24 years ago. Please seek care from a doctor that is used to treating your condition and never accept a expiration date of your life from a doctor.I have never met doctor Stuart Rich but i can assure you when i do i will thank him for saving her life so i could have my mother growing up and still to this day.
I had went to doctors that just pushed me away then last year in march I found a doctor that had figured out what was wrong with me he put the hickman on my right side and had the veletri right away was in the Tlc for 4 days then 2 weeks after that just in the hospital I am glad i found Dr.Runo he saved my life and if I have any problems he helps me out. Iam back at home now my numbers are in the high 70's and i have to be on oxygen 24/7 and on lots of pills. I get lots of side affects from the pills iam on 120 for water pills and still retaning water in body.Just want to know what is going to happen next I feel stuck and it scares me to find out what is going to happen next my wife is my only support and with out her I don't know what I would do with out her.
Hey all, my husband was diagnosed with PAH in 2008. He sees a pulmonary specialist and a cardiologist. He wears a medicial pump device that gives him his medicine and it has saved his life. I hope this helps someone!
I don't have pulmonary hypertension, but my husband does, he has had it for 9 years, has done very well was on the IV treatment previously, got better went to the inhaler, from the inhaler, back onto the IV treatment and we're hoping he will be off of it again soon good spirits staying positive in my opinion is the best medicine.
I am sitting in a hospital room as I type, my 13yr old son breathing oxygen, wearing a pump on his side (for constant med. flow) and taking multiple other drugs. He has been diagnosed with Idiopathic P.H. (unknown cause). His heart is enlarged, his pressures in his heart are @ 130 when 20-30 is normal. I dont expect a long time together so I hope I can make it the best possible time.
Dear friends, We are not able to say about one's life span or life expectancy. Everyone is unique, so how can we compare? I have been diagnosed with Severe PAH at 12yrs. ( I have VSD-LTGA) I am now 36 yrs old , married and got 2 kids a boy and girl. No wheezing , no synopsis or clubbing. i am having no medication. i am fine here with my office job. one thing i practice is that i am vegetarian. for more details is required feel free to write to me .
My dad was diagnosed with pulmonary hypertension in the beginning of 2013, and it really took atoll on our family especially my mom, he was 24/7 oxygen @ 10liters a minute, there something about how a person can't survive more than 3 years on 24/7 oxygen I was starting to think that he would never make to my wedding or even graduation, but then this last Sunday he got a call to get a lung transplant and I have seen pictures of him and he looks the best I have ever seen him. To cur the whole disease get a lung transplant, I is miracle in the making
in regards to average lifespan of a PAH patient my best friend Tessie just lost uer battle woth this horrible disease. lifespan is not good if you dont get on and ov medicene like flolan where you are onfused. she lived with this disease before she lost her battle with ot. its a horrible disease and a friend of mine who is a nise said PAH is a death sentnence when you get it. so if you are smoking you really need to quit. my best friend had quit smoking for 7 years then one day she started back up. she smoked weed as well. thos is a terrible lung disease. my bestie couldnt quit smoking but i pay those of you on this thread will. i am not a nurse or doctor just a best friend of over 33 years. plaes visit the Pulmonary Hypertension Association website. they have all the newest information on thos dosease. they will even. send you a packet with a book on this disease and monthly newsletters. my friend had pneimonia lots and merca from the iv port and there were always problems woth the port coming put etc. but the flolan will keep you alive. she had also had a stroke a few years ago but survived. the pah association website is where o fpund dr sud in ohio for my friend. it shows all the places they treat this as well and they can tell you whoch nursing home or hospital can treat pah. prayers and best of luck to ypu all!!! missing my bestie, Vicky
Hi all, I'm Tess and I was diagnosed with Pulmonary Hypertension with Eisenmenger's Syndrome due to a hole in the heart (patent ductus arteriosis) in 2008 at the Royal Brompton Hospital. I was told I wouldn't live to my 18th birthday that year, but I'm nearly 25 now and currently doing well and I'm stable. I take sildenafil 100mg three times a day and am living life to the full. I'm currently studying a Masters in Music Therapy to help others with difficulties using music, as music has got me to where I am today. You can read my story and follow my blog http://musichelpsmebreathe.blogspot.co.uk/
Hello. I have PAH. I was diagnosed in 1992. I am also an RN. I have been there ; done that; and have gotten a T-shirt. I Was quite upset to see so many frustrated ppl on here looking for a specific time frame !! There is none. The person still smoking needs to attend a local support group to get a reality check. I put it like it is ! Some ppl might just say --oh well. What the heck--going to die amyhow. But going like that is selfish without trying because there are lots of options. There are as of December 2015--14 drugs to treat. They do have side effects. After a few weeks the symptoms usually dissapate and are tolerable. Those drug side effects beat the PH symptomology if it goes untreated ! Education of the disease process and how it effects your body as a whole--mentally amd physically is a must !! Knowledge alleviates the fear !! So I have had good years. A couple tough ones and am starting/adding on with another drug soon ! So 24 years here ---long term survivor. Google Pulmonary Hypertension Association--another person gave good advice --they are amazing folks !! You get updated nees on therapies and treatments. Patient stories. Events, etc. in areas around the country you can attend. There is a PH Convention in Dallas this year ! Not sure of exact date-- if you have never attended, the PHA will pay for your travel, food, accomadations, and attending the convention !!! It is an amazing and learning experience and the best of the best Doctors/Nurses/Drug Co. Ppl. Supply companies---anything related is there. Make friends and get some hope that a cure is coming. There are also some Facebook support groups you can join. Peace and Hope to all who have it and bless all who care and love us ! AVA
I have secondary PH due to COPD, and I'm not a smoker. It kind of knocked me for a loop, but I began a vitamin regimen that is working good for me. Aside from that, I just use an inhaler. I know I'm sick, but I don't feel sick. Aside from some fatigue, I do very well, and I'm 75. A lot of dealing with this is attitude.
I was diagnosed with PAH in July 2014 at the age of 60. I have been wearing an I.V. 24/7 since then. First, I took Valetri but turned out I was allergic to it. After a couple months of itching, I went into the hospital (only as a precaution) and they switched me to Remodulin. At first, I felt so sorry for myself and since I'm a private person, I hated wearing the I.V. Initially, I went to the E.R. when my heart began racing and wouldn't stop; had some fatigue before that but just figured I was getting out of shape since I no longer worked and had gained about 10 lbs). I went into respiratory distress and luckily, the pulmonologist who treated me 6 years prior for sarcoidosis in one lung, was called in and transferred me to Ochsner Hospital in New Orleans where Dr. Stacy Mandras diagnosed me; she's a cardiologist specializing in PH and has been wonderful. She, too, refused to give me an answer when I asked her how long I would live, though I saw in books that the average time from diagnosis to death was 6 to 8 years. That's NO LONGER TRUE, people! There's tons of differences between all of us and there are lots of IV drugs, oral drugs, surgical procedures for some types of PH, and they come out with new drugs all the time! Research is ongoing and some researchers predict a cure in 10 to 20 years. Some of us will live that long and see a cure. I wanted an answer to that question for a long time but it's an answer no one can give you. Seriously -- it's not a dodge by the medical provider, it's an unknown and they would be irresponsible to attempt a guess. I'm 3-1/2 years since my diagnosis and I feel pretty darn good. My husband helps me mix my meds every other day and the Remodulin is easier to mix and much easier to deal with than the Valetri I was allergic to. If you didn't see the I.V. in my arm, you wouldn't know I had a serious disease. Some days, maybe once or twice a month, some months not at all, I feel very tired for no reason but heck, I just take it easy that day and look forward to the next day. The only side effect from my medication I really suffer from is diarrhea and I take two or three anti-diarrheal pills each day; if I will be away from a bathroom for an extended period and have been bothered by a bout that day, I just take an extra pill. Usually, once I take two anti-diarrheal pills in the morning, I'm okay until the next morning, when I take another two pills. I had headaches and nausea on the Valetri, so just having a bit of diarrhea with the Remodulin is a blessing. If I damage my I.V. line, I know I have 4 hours to go home, get another line out and reconnect; naturally, I connect ASAP but I am not hesitant to be out and about as I was on the Valetri, which you had to reconnect in 5 minutes (I still went out but I carried an emergency pack; in case anything happened, I had a vial of medicine and all my supplies with me so I could mix up another batch right away). I go out by myself as my husband is no longer too nervous to let me out of his sight. I have never had an I.V. line infection and though I miss not swimming or taking a bath, I hadn't been swimming for years before I was diagnosed. Of course, once I was told I couldn't swim, naturally I wanted to swim! In June 2016, I got pneumonia and while hospitalized for that, they noticed the level of oxygen in my blood was low at night, which is true for about 25 percent of PH patients; so now I hook up to an oxygen machine at night. Though I'm not crazy about it, I feel blessed that I don't have to use it 24/7. I don't FEEL out of breath so I have to force myself to connect at night but still consider myself lucky. It's not so bad, just a tad noisy. My husband has no problems sleeping right next to me, as the machine noise doesn't bother him in the slightest.
In late 2014, I began having severe back pain and finally saw an orthopedist in early 2015. It turns out the huge doses of steroids I took for the sarcoidosis in my lung in 2007/08 have caused my bones to start "crumbling" and I now live with 8 broken vertebrae in my back; nothing can be done so I'm in a lot of pain from that, and I very carefully manage the powerful opioid drugs that I take several times a day. I had to laugh, as I realized PH is nothing compared to the back pain I have from the steroid damage. I would KILL to "just" have my PH as at least I had no pain, or no pain to speak of. You would laugh, though, as I am lucky that I do not suffer from chronic constipation that most opioid patients deal with -- that old diarrhea caused by my Remodulin is helping me avoid the even worse problem of constipation! I have now developed some curvature of the spine and am no longer able to wear my high heels I love except on a rare occasion. I always prided myself on my good posture and loved being 5'8" and still proud to wear 3-inch heels. Heck, now I wear 3-inch wedges instead with no problem! I can't stand up perfectly straight anymore but do the best I can. I have to look back at myself when first diagnosed with PH and realize it hasn't been half as bad as I thought it was going to be. I'm lucky to feel pretty good all the time (except for horrible unrelated back pain), have a super I.V. drug keeping me healthy and alive with just a single side effect, and a caring husband to help me mix my meds. When he was in the hospital for 9 days, I mixed my own meds and was even able to connect myself to the I.V. cord all by myself; some days, it took me a minute and other days it took me 15 minutes, but I knew I could do it so I persevered. When it took a long time, I'd be covered in sweat from making the effort but I knew if I kept trying, I could connect myself. I felt great that I could take care of myself if necessary, too. Both my husband and my doctor told me how proud they were of me that I didn't panic and just figured if I kept trying, I could indeed hook myself up I urge everyone to try to let go of that feeling that you HAVE to know how long you're going to live; no one who's healthy knows when they will die so we don't need to worry about it either. My doctor has patients from the age of 8 to the mid-80s. Hopefully, I will be one of her patients that make it to my 80s as well; but I am enjoying my life and so can you. Years ago, PH patients were told to get their affairs in order and enjoy the time you have left. If anyone tells you that today, FIND A NEW DOCTOR who is experienced in treating this disease.
And try to remind yourself: "The Will of God will not take you where the Grace of God will not protect you."
Stay Connected With Caring.com
Get news & tips via e-mail