"BLOGGING FOR PULMONARY HYPERTENSION AWARENESS"

Shaye's Story (written by her mother, Teresa)

2010-11-22T09:08:00.002-06:00

I want to share our story of PH and how we came to this diagnose in hopes that other families can be diagnosed early enough for treatments to help them live a longer life. Looking back now, the signs were there and were overlooked due to another diagnoses. It is not uncommon for PH to be misdiagnosed especially in children because of all the other illnesses that mimic PH. I can tell you to be your childs advocate and if you feel in your gut like the diagnoses that was given isnt right, keep fighting, go to the next dr. Recently another one of our kids Julia who is ( 8 year old ) has been having some symptoms and the Drs want to rule out PH for her, so she will go tomorrow for EKG and chest Xrays. Keep us in your thoughts tomorrow and hoping for good results.

Here is our story of PH and how it all began.

Shaye was diagnosed with extreme anxiety in 2007, but during the 2 years that followed, her symptoms would continue to get worse, and her Dr would say it was due to her anxiety. Heart palpatations, dizziness, sweating, nausea, chest pains. What we would find out later is all of this was caused by something else, something we could never have prepared ourselves for.

Shaye's story begins on February 19th, 2010. She fell at school in PE and hurt her back. The nurse said she was fine and sent her back to class. She came home and we did heating pads, tylenol, etc and a week later it still wasnt feeling better. We decided to take her to the pediatrician. Her dad took her, and they sent them to the hospital next door for xrays thinking she hurt her back somehow. They told them it would be about 2 hours before the results would be back and the Dr would call us. A couple of hours passed and they called my husband and told him that she had fractured her spine, of course are you kidding me???? Was our reaction. They said she needed to be taken to the Texas Childrens hospitals ER. I left work and went by to get the xrays to take with me. Picked up Shaye, while my husband stayed home with the other 3 children. We left for the hospital which was about a 30 minute drive from Katy where we live.

We waited and waited to be seen, seemed like forever. We finally were taken to a room. The ER Dr first listened to her chest and asked, " she has a heart murmur"? I said no. He said, " she does now and it isnt a good one". He said he was going to go look at the xrays and would be back. When he returned about 45 minutes later, he had another Dr with him who he introduced as the cardiologist on call. Of course when I heard cardiologist, my heart sank. He asked if I wanted the good news, or the bad news first. I said the good news, I just wasnt ready to hear anything else. He said the good news is that her spine and back were perfectly fine. The bad news is something is wrong with her heart! My heart sank. The cardiologst took over, and told me that he was ordering an EKG because the xray also showed an enlargement of the ride side of her heart. She failed the EKG so he told me they were ordering an echo cardiograham. About 3:30 in the morning, they were doing the echo on her. It took a very long time it seemed, at least an hour and 1/2. He told us they were admitting her for more tests. We were taken to her room on the cardio floor about 5 am, and I thought we would be able to get some rest before more tests were done, but as soon as we were in there, the Cardio came in and told me they suspected Pulmonary Hypertension. We had no clue what that was, but our lives as we knew it was about to be changed forever! She had a heart cath about a week later which was the final Ph test. It showed her pressures in the high 90's and the Echo had only showed approx 50. Big difference.

The reason for her back pain when she fell the Dr said that her lungs were at their full capacity due to the high pressures so when she fell she felt pain. We have been told without medication she has about a year. With medication, they arent certain, it all depends on the medications and how they work. Her levels are severe, and all we can do is hope and pray the medications keep her levels low to slow down the progression.

Shaye started out with Tracleer and then Revatio was added along with Coumadin, Prevacid and an inhaler. In August she was having more symptoms of PH and so she went back in for another ECHO and we were told it had progressed severely and would need to be admitted to start the IV medication remodulin. She went into ICU and was in there approx 8 days. It was nothing nice and she had alot of reaction to the flolan and was sick alot. We made it through and her central line was placed and she came home on the remodulin with the cadd legacy pump. It has not been an easy ordeal. There have been so many emotions, anger and just overwhelming feelings dealing with the daily ups and downs with a child with PH. Shaye also has ADHD and can not be treated with medication for it so her body is fighting against the PH and the inability to do what she wants to do. She pushes herself to the limit and goes until she cant go anymore.

PH is a progressive terminal illness, so there is no cure, but hopefully we can slow the progression down. We have taken a step back and learned to look at life differently, because you just never know when your child, or someone you love can be taken from you. Life as we knew it has been changed forever. We have hope, we hope for a cure one day. All we can do is take it one day at a time and take the good with the bad in hopes for that better day to come along with a cure!

Tammie S's PH Story

2009-11-19T12:41:00.002-06:00

Before I begin my story I would like to tell you a little about myself. I’m a mom of 5 children, a military wife (family), support group leader, and PEER Mentor. I’m not an OU fan! I’m just married to one. I’ve been married for 12 years. I enjoy football and UFC along with most any sport. I love to read and write poetry. I also love to travel, visiting the zoo, and going camping. I was a nurse before we discovered I had PH. My first plane ride was on the Air Force’s C-130 Hercules on a spouse flight. The C-130 was the plane my husband worked on now he’s an engine mechanic for the KC-135. He has recently switched from Air Guard to the Air Reserves. We’ve also have our own mini zoo. We have 2 dogs, 2 hamsters, 2 tree frogs, a fish, and a lizard. Now that you know a little about me here’s where my story begins.

Many people have asked me how I got diagnosed with primary pulmonary hypertension. Instead of repeating myself numerous times I started writing my story.I need to tell you my family history of PH so you can understand my own story. So our history begins:I am not sure the exact dates or the exact years. I was in middle school/ jr. high when my family first heard of PH. Being the normal young teenager I didn't pay much attention and had a mind set that it wouldn't happen to me.

To begin the PH runs on my mom's side and from generation down. My grandparent’s had 7 children. Aunt Ray, Aunt Leona, my mom, Aunt Sheryl, Aunt Tina, Uncle Robert and Aunt Rhonda~ twins. 4 of the 7 have had or have PH. I’m the only one so far in my generation.

In 1992, my Aunt Rhonda was pregnant and started having problems with weight gain and breathing. In August of 1992 at 5-6 months pregnant she went into the Hospital. The doctor delivered the baby thinking the pregnancy was causing her problems. Within about 72 hours from going to the hospital her body started shutting down. An autopsy was performed and PH was found. She was 24 years old.

Between 1992 and 1994, my Uncle Robert, Rhonda's twin, was diagnosed with PPH. They had said that many twins will have the same illness/disease. I remember that he lost A LOT of weight and his lips were ALWAYS blue. I also know that my uncle didn't follow his doctor's directions. He didn't follow them because he felt that he had to work to provide for his family. He didn't have medical insurance and because of lack of state funding for PH. In August 1994 at 27 years old he passed due to the PPH while waiting for a heart transplant.Sometime between 1992 and 1995, my Aunt Leona was diagnosed with PPH. I remember she had to take A LOT of medicines. Half her dresser was covered in medicine bottles. She had good days and bad ones. I remember she had to get echos and right heart caths done many times. In November of 1995 she had a double lung transplant. The lungs were from a 13 year old girl from New Mexico. Back then we didn't have the technology we have today or the medicines we have today. The "new" lungs developed pneumonia, causing her to pass within the first few weeks after transplant.

My Aunt Tina has been living with PH for about 11 to 13 years. She is my inspiration story. When she was diagnosed with PH she spent 28 days in the hospital and left with a triple lumen used for Flolan, an IV heart medication, and oxygen. The doctors were very surprised as to they thought she wouldn't make it and sure not to leave the hospital. She had to list for a double lung transplant. Now years later she has had many central lines replaced, battled some hard battles, yet keeps on living. She is now OFF Flolan and uses only pills to control her PH. She has been off Flolan for just over a year (as of 2/2009) with no adverse side effects.

Now, knowing that PH runs in our family begins my own personal walk with PH. I have been tested, with echocardiograms; 2 times prior to finding I had PH. I was tested after my first 2 pregnancies. At each time PH was ruled out. I was tested because of not having good pregnancies. When I was on my third pregnancy I had a hard time with breathing and ultrasound proved it was how my son was laying on my lungs. My youngest son had to have oxygen for about 5 hours after birth. During labor we had a hard time getting him to come down and out the birth canal. A year after that and only being 24 years old I was on my fourth pregnancy. During my pregnancy I started noticing I was having problems with not being able to breath and hurting when I did. I couldn't do simple things like picking up my youngest child from his bed. I had blue lips. I lost my breath A LOT. After telling my OB/GYN at the next appointment, I was put on bed rest. The doctor looked into PH and was looking for answers to what might be causing my problems. Two weeks later I went back to the OB/GYN and he wanted me to get on EKG done and to read what my oxygen rate was at. I went in at the hospital to do the EKG, which was normal, but my oxygen level was in the 70's. They didn't let me leave the hospital and I was admitted. That was on Friday. Over the weekend I gained a pulmonary doctor and a cardio doctor. They decided that I couldn't make it to full term with my pregnancy. Monday Jan. 20, 2003, I delivered my twins 2 months early by c-section. Before delivery we tried to do a right heart cath. and didn't have enough time. I spent 2-3 weeks in the hospital during which I had MANY tests done. There were doctors called in from 3 different hospitals. Ruling out PH with an angiogram, we weren't sure what caused the low oxygen rate. I went home on oxygen. 5 months later I went off oxygen and went back to work doing CNA work. October 2004 I begin work at Mercy Hospital in Their Convent. I took care of the Sister's who are unable to care for themselves. I started not being able to breathe when doing simple things like vacuuming and walking. I had to sit and catch my breath. The patients I was used to lifting on my own I couldn't any more. I was tired and weak. During this time I started having abnormal female bleeding. I went into my OB/GYN, the same who delivered the twins. We tried to control the bleeding with 4 birth control pills per day. He said that a hysterectomy would be needed. During 1 month’s time I started having more problems with my breathing and it was that I had to rest more often and even taking a nap after work just to have energy to last thru the evening. My OB/GYN wouldn't do the hysterectomy before doing a pulmonary and a cardio work up. I went back to the same doctors who I saw before. I went to the pulmonary doctor and did a 6 minute walk, a chest x-ray, and a breathing test. The doctor said it was either asthma or pulmonary hypertension. I was treated for asthma while setting up another EKG. A month later I was diagnosed with PPH. I then went to a doctor who treats PH and is with a transplant center. I did a right heart cath and had to stay over night in the ICU. 1 month later I started Flolan. So from Feb. 2005, to May 2005, was the wait for diagnoses and treatment.

Working at the hospital I have benefits that if I used a doctor at the hospital I worked at then I wouldn’t have such high co-pay. I then went to search for a doctor who was knowledgeable and treated PH patients. I called those at the hospital who were on my insurance. I would ask questions like do you know what Pulmonary Hypertension is? How many PH patients do you have? How long have you treated PH? I didn’t find what I was looking for. The good thing is Oklahoma does have a PH doctor. I just have to pay higher co-pays. I noticed a lot of states don’t have PH doctors. My advice would be to call and do research and find a doctor you feel confront able with.

Around July 2005, I started having chest pain and found out that my gall bladder was enlarged and that I had 5 gall stones about the size of a half dollar. Now I needed my hysterectomy and my gall bladder removed. Yet I had to wait to where my dose of Flolan was stable and safe. In October, I went into the OB/GYN to get a pelvic ultrasound to find I had a 6.5mm zest on my right ovary. A normal zest every woman makes when cycling is about 2 to 3.5mm. I also wanted a mole removed that was showing signs of cancer. We found a doctor who was willing to work with my OB/GYN to do the gall bladder surgery. On Oct. 20, 2005, my 27th birthday, I went into have surgery. That was at 5 a.m. and later I was admitted to ICU because my blood pressure was not good. The next thing I knew is I having internal bleeding (the OB/GYN had said that a person with PH would 98-100% chance of not making it if internal bleeding happened) and was off to an emergency surgery. A surgery I wasn't expected to live through. I got into the OR and saw my mom's best friend's husband, a.k.a. Uncle Russ, who is a sub tech. I knew when I saw him that everything would be okay. I stayed 1 week in the hospital because my body wasn't producing platelets. I also have had swelling in my belly and have had to get it drained twice. The first time we took off about 2-2 1/2 liters. The second time we took off 3- 3 1/2 liters. That was just 6 weeks apart. After this time my PH became worse and I found out I was in need of a double lung transplant. While going thru the testing and listing process I was put on Revatio. Revatio was the medicine that could help with PH and help so well that I might not need a transplant. So far it has helped and I'm on inactive status for the transplant!!

Since then I have had a hole in my central line with was fixed and then later in the same spot another hole and it was replaced. When I had the first hole I was telling the line placement team I wanted the line replaced and not just fixed. The second hole happened less then 6 months. So because of this I encourage each and everyone who has medical concerns to always listen to your body!

April of 2008, I switched from Flolan to IV Remodulin. I switched because I believed that Remodulin would fit my lifestyle better. My lifestyle of being a mom to 5 children, ages from 14 to twins who are 6, and also a military family. What had made me think about the switch was when I had a lady come talk about the Remodulin pumps at a support group meeting. I got interested! I then asked my doctor, Accredo, my family, and did some brief research on the internet. I then had a PEER Mentor come speak at another support group meeting and was hooked! I did more research, talked more with my family and doctor. I finally just said I’m ready and can we get things set up for this time. Things worked out and I’ve been on Remodulin since.

I’ve battled a line infection and had another weeks stay in the hospital and a week at home with IV antibiotics. During the weeks stay I did have another central line replaced. This time I went from single lumens to a double. I’m now the support group leader for Oklahoma, have gone to the PH conference in Houston, have many websites I try to take care of and keep updated so I can reach out to those who need support. I’m also working with a lady to start a second support group in Oklahoma. Our support group has done a few fund raisers and we’re working on this year’s fund raiser. I also became a PEER Mentor myself and have begun sharing my story around the states! I’m also working on finding a way to donate my PH lungs to help PHers. I believe that with donating the PH lungs to researchers would give more hands on to help with medications and one day a cure!!!

I have a big support group outside my support group. I have family and friends that are great with supporting me. My in laws have also been a great support. I also have a RTA who more then just another support person, but a friend!

PH has affected my family for many years. As of my husband, children and in-laws, it affects us everyday. There are things we can't do. We have just learned to adjust and found other activities to do. The activities I still do are dance, belly dance, camp, lazy around in the pool, travel, and sing to name a few. When I do extra activities or travel I do rest up and take naps before and afterwards. I try to stay positive and take life one step at a time.

Here's more information.
• When I switched from Flolan to Remodulin I asked my doctor, Accredo, and my family and friends. I wanted all of those who support me and are involved in my care to agree for the switched. I did this many times before making the final decision to change treatment. When I switched from Revatio to Adcirca I just switched. I switched because my side effects, even with trying to control the side effects, were not letting me be compliant so when Adcirca came out I was like okay doc. I want to try that. He asked me if that doesn't work what else do we do. I told him we work TOGETHER and find a solution to make it work! He said ok.
• I'm working on doing research and coming up with a way to donate my PH lungs to do PH research. I just got some new information in Sept. and am coming very close. As for donating it would be either when I pass (which seems to be the only way at this time) or if/when I transplant.
• I have a support group outside my PH support group. My family including my in laws, my friends, my MySpace and face book friends, along with an RTA who is a great help and a wonderful friend.• There are still things I can't do like stairs, vacuuming, doing laundry(at times), swim, play sports, and other things like that. BUT there ARE things I still enjoy like getting in the pool/lake. I just relax on a floatie. Like I said I dance, sing, and I watch sports. I've even come up with a way mom can play hide & seek with her children. So basically I've adjusted what I can't do to make it something I CAN DO!
• My greatest advice for you is to make sure you ARE your own voice and that you SPEAK UP about your treatment and other things pertaining to your health. Ask questions no matter how small you think they are. The only dumb question is the one not asked! Listen and look for signs to known when to call your doctor. Remember YOU are the MAIN person who is PART of your health care TEAM! Doctors don't always communicate with each other so YOU might have to do the communication! Remember there is HOPE! You are NOT alone! Join a support group. These people know what it means to just have a bad day and you don't have to find words to explain, they just know & understand. (Which is the biggest thing my support group members talk about.)
• I have a quote it goes like this; "I have Ph it's not curable and it isn't going away. I won’t wake up one day and it be gone. PH does NOT control my life. I control my PH so I CAN LIVE LIFE!!!"

Diana's PH Story

2009-11-05T20:35:00.001-06:00

I am Diana, 54 years old with PH secondary to interstitial lung damage secondary to scleroderma. I was diagnosed with the scleroderma about 3 years ago but had some CREST symptoms as long ago as 12 years with my esophagus now it is full blown and progressing rapidly. I was diagnosed with PH 1 year ago this month. it has progressed to class 4 and I am on Flolan along with Revatio and lots of diuretics, digoxin and pain meds for the scleroderma. I take 14 pills in the morning..... a few more about 2:00 and then 10 at dinner time. It is very hard to type this with my painful crooked fingers but I fell it is important to share my story with others so they can be encouraged.

I love Jesus and was a church secretary for 15 years at our church. I retired Dec. 08 and went on SSD. That is something I should have done months before I did but I just didn't want to "give in".

I do not drive any more and can only walk around in the house with my o2 on 5 l/m so I cannot walk to the car or from the car to a store or anything like that. I have a wheel chair and a jazzy power chair and someone goes with me everywhere I go outside the house. MY husband is a truck driver and owns his own truck and gets his own loads and is gone quite a bit so Ii am at home on my own in the afternoons.... I have caretakers in the mornings for Flolan stuff and giving me a shower which I cannot do on my own any more. This all has brought my h usband and I closer together than we have ever been. The Lord has been by my side holding me up through all my down times and good times as well.

I am so thankful for everyone who has encouraged me and filled me with helpful info on the PH web site

G. C.'s Story

2008-12-02T21:33:00.001-06:00

This is my first time blogging, so please bear with me. Back in 1993, I had a beautiful baby girl, and was working full-time as an operating room nurse, and taking "call" (that means that if an emergency surgery is needed after usual hours, they "call" you to come in to help do it). The amount of hours that I would weekly log would be as if I had both a full-time and a part-time job.

Then came the opportunity to loose the last of the pregnancy weight gain that I hadn't lost so far. That came in the name of Fen-Phen. My family M.D. prescribed it, and little by little, I was loosing the weight that I wanted to. But in exchange for this weight I noticed the start of swollen ("edematous") lower legs & ankles, and at times, disturbingly shortness of breath. Having access to a cardiac (heart) monitor (I also worked in the Recovery Room), one day I hooked myself up to it, and ran off a strip that printed out a long line of what my heart beat looked like. What I saw, I didn't like, and so I brought it over to an anesthesiologist not helping with surgery at that time.He strongly suggested that I go to the Emergency Room.

After being scoffed at by the Cardiologist in the E.R. (Back then I was a 43 year old female. I'm older now, and still female, and still scoffed at); he had quite a different expression when the labwork came back indicating that I was in the process of having a "small" (non q-wave) heart attack. This was a very small community hospital, and I was shipped to a much more evolved hospital.There I had the first of my angiograms (to date, I've lost count, but I know this is true-- the number of catherizations that I have had now number over 40)... and when I mention that to any of the neccessary people involved, even to this day, they claim I've set a new record of sorts... Whoopee. (I was hoping for a dozen roses on my 12th, but that never happened. How disappointing!)

Now my days are spent nearly totally inactive; I'm on Letairus (which seems to help), but have leg swelling that even amazes me at times, a variety of wheezing sounds that range anywhere from a herd of demented ducks to a hacking cough that wakes me up at night with a range of sounds that are quite interesting to say the least. Any type of physical activity makes me breathless, and my heart race.

Up until my last echo (a week ago) the numbers showed that I had pulmonary hypertension. But now the big dilema, (despite having taken the Fen-Phen) is which came first-- the left or the right-sided heart failure because of my previous heart attacks (I have had a total of 4, with 5 blocked stents). And those pulmonary numbers vary, which I find very confusing.

It seems that because of additional diagnoses, (abnormal cardiac arteries-- one of which is called a "myocardial bridge",& has it's own unique problem-- which doesn't respond to traditional coronary artery disease treatment, and all of the other arteries are fine), pulmonary hypertension, multiple sclerosis, and newly diagnosed hypothyroidism (which wasn't there when I first started seeing the Pulmonlogist).

I'd love to know of anyone (doctor or patient) that has some insight into what it's like to be in my spot-- having newby interns trying to tell me one day it's my heart giving me trouble, the next day then telling me that I don't have any heart problems (despite coronary bypass and many interventions), and then the best answer that makes me see red-- that i"m "just anxious".

I often have to explain what "Myocardial Bridge" means, as well as what a certain procedure I have had called "T.M.R." (Transmyocardial Revascularization). I am the one in the bed as a patient, not the one wearing the white coat and stethoscope around my neck!!! I want answers and I want to feel like somebody knows what's going on, and can come up with a plan for me!!! I feel like I'm back-tracking...not making any real progress, only more confused... help please?
Thanks.

Phyllis' Story

2008-11-24T16:54:00.002-06:00

My health problems all started sometime in 1995. The first thing I noticed was that my index finger on my right hand kept turning white from the last joint to the tip. I went to my Primary Care Physician, and he did some blood work. He said I had Lupus and wanted to put me on Prednisone. I said I wanted a second opinion. I went to a Rheumatologist, and he said I didn’t have Lupus but a Positive ANA and Raynaudes. He said that I probably had an unidentifiable Connective Tissue disease, but that right now he didn’t have enough pieces of the puzzle to diagnose it. He said to go home and forget about it. I had a Doppler of my arm because they couldn’t get a pulse on my right ulnar artery. The doctor said there was no blood flow from mid-arm to the middle of my hand. So, I had an arteriogram of my arm. It revealed that the right ulnar artery was missing mid-arm to middle of hand, but it was OK up the other side. They didn’t know what happened to it, but they didn’t think it was a birth defect. They said it had atrophied, been attacked by my own body or was in a deep spasm and wouldn’t let the blood pass through. The next thing that happened was I developed Irritable Bowel Syndrome, followed by loss of speech in both ears. I had to have a Hysterectomy, and then later my Gallbladder removed. I also started having swallowing problems which was diagnosed as GERD or acid reflux. In 2001, I had pneumonia, and after it was supposedly gone, I continued to have chills, low grade fever and strange food cravings. I went to a Rheumatologist, and he said that my immune system was turned on and couldn’t turn off. He gave me a dose pack of Prednisone, and the symptoms went away. He said at that time that I had Undifferentiated Mixed Connective Tissue Disease and Fibromyalgia. I also had dry eyes. In October of 2006, I started having shortness of breath. I had been troubled with shortness of breath on exertion for a few years before, but my recovery was quick and I thought I was just out of shape. This time, my recovery wasn’t so quick, and I became worse and worse. My chest felt heavy like it was weighted down. My back hurt between my shoulder blades. I was very tired. I went to my Primary Care Physician. He did a lot of tests, and put me on Thyroid medication for low thyroid, but I didn’t get better. My legs were swollen and hurt a lot. I was getting weaker and weaker. I went to a Cardiologist, and she said she couldn’t find anything wrong. I had several tests there, including a Nuclear Stress test. I could hear my heart beating in my ears. I thought something was very wrong. I was sent to a Pulmonologist. I told him my medical history and asked if he thought I could have PAH. I had been doing research on the Internet, and that seemed to be a fit as far as everything I knew to be true of my symptoms. He said it was a likely possibility. He suggested I see a PAH specialist at Vanderbilt University Medical Center in Nashville, Tennessee by the name of Dr. Ivan Robbins. I went to see a Rheumatologist instead. He did a lung scan, and he said I am calling your Pulmonary doctor. You need to get to Vanderbilt ASAP. I think you are in danger and have PH. I was put on a list to see Dr. Robbins on March 13 of 2007. On February 9th, I woke up about 1:17 a.m. (one week to the day prior to this, I had passed out) and told my husband I needed to go to the ER as I thought I was having a heart attack. I told him if I waited until March 13th, I would be dead. He called an ambulance. I went to the local hospital ER. They admitted me, and started me on Lasix. They called my Cardiologist, who in November couldn’t find anything wrong with me except for a slight heart murmur. She was shocked at my current condition. My Pulmonary doctor came as well. He called Vanderbilt, and Vanderbilt said I could come straight to their hospital as soon as they had a bed. I was taken by ambulance the next evening. Once I arrived, they assigned me to a team of doctors. Dr. Robbins also came to see me. They did a lot of tests including a V-Q Lung Scan, Echocardiogram and Right heart catheterization which showed PAH. I was diagnosed as a high stage III. I went home after five days on Revatio, Lasix, Potassium, Warfarin, and Ventavis was ordered for me. Although, Dr. Robbins thought I needed Flolan. While I was in the hospital, they pulled 26 pounds of fluid off my body. No wonder, I hurt everywhere. After a month on Ventavis, I agreed that I was backsliding and needed Flolan. So on April 1, 2007, I went on Flolan. I have progressed so much since then that now I am a candidate for either Inhaled Remodulin when it comes out of trials or Letaris and Revatio combination therapy. Time will tell. Thanks to Vanderbilt, I am alive and showing signs of progress. As far as my Connective Tissue Disease goes, they think I may have a form of Scleroderma or a form of CREST, but they still aren’t certain.

Tera's Story

2008-11-19T11:25:00.003-06:00

Hello, My name is Tera, I am the oldest of three siblings. I entered into this journey of life at the age of 33. I am a wife, mother and a medically retired paraprofessional and coach.

After leading an active, vibrant lifestyle, I suddenly found myself immobilized! As I try to think back to where this all began, it’s like trying to put a puzzle together. I remember the basketball season of 2004 getting short of breath while trying to run plays on the court. I had to stop running with them and just give directions. I went to the doctor , but he said it was bronchitis. I took the medication and it cleared up for a while. It was even harder for me to keep up with the kids in our classroom. I had to use the elevator to ge to the office, cafeteria and library. The stairs was taking everything out of me, turning my hands purple, and leaving me gasping for air.

It was on a cold day in January of 2005, I woke up feeling short of breath but I insisted on going to the grocery store with my cousin Stacey. While in the store, she kept asking me if I was okay. I told her yes because I didn’t want to upset her because she was expecting at the time. I went to my Mother’s house when I got home, Tiffany and Natasha was making pecan candy, they took one look at me and Natasha says something is wrong with you, your lips are purple. I then walked to my house stopping several times to catch my breath. I made it inside and my husband looks at me and says , to me , go sit down and I’ll put the groceries up. I went to the bathroom to look in the mirror but by this time I am completely out of breath , my lips hands and feet are purple. I can’t yell for Tarais so I call Averil from my cell phone to come and take me to the hospital. When she arrives at my house, she looked at me and said are you crazy, you will not die on me in my van. Tarais dialed 911, it took all but three minutes for someone to respond to the call. Gerilyn was an EMT on call that day, when she heard the address she responded to the call without waiting for the ambulance. I’m glad she did, she saved my life. When the ambulance arrived , all I remember hearing is we’ve got to roll we are losing her fast. Unfortunately the doctor in the emergency room said it was a severe asthma attack and that I could go home after several hours of taking breathing treatments.

Another month has passed and there is no change. I live in the lab having blood drawn, I’m having to call in sick on a regular basis and I’m missing most of our softball games because my hand can’t take the cold and I am in constant pain. I make another doctor’s appointment ,making sure that he understands that something else is going on other than asthma and bronchitis. By this time, I have to make an appointment with the dermatologist also because now I have ulcerated blisters on two of my finger tips..

It’s now March and my fingers aren’t healing. The dermatologist performs a biopsy on my fingers, he’s puzzled by the results of the test, he continues to treat me for my fingers but he insisted that I see my doctor because something serious is going wrong with my health. By this time I feel as if I’m on a roller coaster ride that just won’t stop rolling. I go back to the doctor again , this time he makes appointments for me to see a pulmonary specialist and a rheumatologist.

I’m in the office of the rheumatologist with thoughts running through my mind, if I can’t breathe than why am I seeing a doctor for arthritis . After my exam, I feel like I have been questioned by a lawyer and still have no answers. He sends me to lab of course and then to do a CAT Scan of my lungs.

It’s now May and my steps are getting shorter and harder to make and my lungs feel as though they are always going into spasms. I get a call from the rheumatologist to come in for a visit the next day. My Grandmother and I sit patiently in the waiting area, I’m thinking to myself I can’t believe arthritis can cause all of this. The nurse calls my name and takes us to the room, the doctor walks in and says Tera, I have bad news for you, you have a Lung Disease called Scleroderma. He goes on to say it’s uncurable but there is a treatment that can possible slower the deterioration of your lungs, he went on to say that he was sending me to a cardiologist because he was afraid that it maybe something wrong with my heart also. As part of a treatment plan he put in a request for me to take chemotherapy for my lungs. I looked at my Grandmother and I thought to myself, oh my God ! I’m going to die!

I’m driving home and I’m thinking of how will I tell my parents , my sister and my brother along with my husband and the rest of my family and friends that I have a life threatening illness that has no cure. My Grandmother sees the fear in my face and the tears falling from my eyes as she’s whispering a Prayer to God. She said to me , you know how to Pray and I taught ya’ll to depend on God for everything and right now is no time to Doubt Him.

It’s the end of July and I’m at my second visit with the cardiologist, he says that he need to send me for a test ASAP! The test was scheduled for August 3, 2005 , I walked in not knowing what to expect. My Mother (calm as she think she maybe ,until it’s all over then she breaks down) tells me to stop being nervous it’s just a simple test. Well let me tell you, that test landed me in a hospital room with tubes in my neck going to my heart. I had lay flat on my back and could not move for the next 24 hours. When I looked into my Mothers eyes, I could see that she had been crying. Later on I see my Grandmother and Averil walk through the door then my Tee Cheryl and Stacey(of all people, she cries when I cry), not long after they arrive my Godparents show up with this look on their face as if they know something that I don’t know. The next morning my doctor comes into the room and says my love, you have a very rare heart disease called Pulmonary Hypertension which has no cure. I’m thinking two life threatening illnesses with no cure, way to go Tera.

I’m two years into my marriage , just purchased a new home now how do I go home and lay all of this on my family. I felt like someone just ripped me open and had taken every ounce of joy from me and my family. I was empty , lost and in dismay. Never did I think that nothing could happen to me, I’m just used of taking care of everyone else, now someone will be helping me to get through the rough times.

The storm before the storm came on August 23, 2005, it’s my first day of chemo, my Mother and Grandmother was there to support me. I signed in and waited anxiously for the nurse to call me. When my name was called, I walked through the door and I started to tremble and the tears rolled down my face, just as I started to calm down, I heard a call for Code Blue. It was for a patient on the other side of the room. A team of doctors rushed in as if we were in a Trauma Unit. I thought to myself, if this is what chemo does to you , this is my first and last treatment. They put me in a room another patient since I was nervous. She said to me, baby cry if you want to because this is your life, but it’s not as bad as it seems, it gets easier every time you walk through that door.

Three days later we are packing up our home in Boothville, LA , headed for Amite, LA in the wait for Hurricane Katrina to make landfall , praying that she wouldn’t destroy our hometown. On August 29, 2005, our lives would change forever . I woke up to a power outage and I’m on oxygen and have no back up tanks. I had just started using oxygen, so I didn’t know who to contact. Every one in my family is worried about me, so I told them I was fine. As the days passed by , I could feel my breathing getting worse but I didn’t say anything. Finally , I got in touch with the oxygen company and they had an office open ten minutes from where we were. Thank God we got in touch with them we were out of power for three weeks . Some nights Averil had to drive me around town just so that I could cool off in her van with the A.C. running wide open freezing her. I felt sorry for her but my lungs surely appreciated the air.

Now I’m really feeling empty and lost I’m faced with health issues and the lost of my home and vehicle. I go to my rheumatologist for my checkup and he tells me that things are still looking the same and there is no change in my health. Well he lays another one on me, he advised me not to move back home for at least seven years because of the bacteria, it wouldn’t be healthy for my lungs. Now we are faced with having to make Amite, LA, our new home. I know that life takes us in many different directions and we must find the courage and hold on to Faith in God to move on, but at what point do I start to move forward. I continued to take chemo until January 2006.

On April 11, 2006 which was my 34th birthday, I went to visit my lung specialist. As he walked through the door, I didn’t see that smile on his face that he usually greeted me with. I asked him where was that smile that used to greet me with? He said Baby Girl we need to have a serious talk, so I said okay I’m listening. In the middle of our conversation he asked me to go home and get my business in order . I asked him what did that mean, he looked at my Mother and said to her, I’m giving your daughter 4 to 6 moths to live, those are my thoughts from looking at all of her test results, then he turned to me and said I’m only speaking from a doctors point of view I am not God, He has the last say. I took a deep breath to hold back the tears. On the drive home, I was very quiet ,my Mom on the other hand as calm as she seems, I could hear the fear in her voice as she talked with my brother on the phone. She looked at me and said it’s okay just live your life to the fullest, live each day as if it was your last day, enjoy your life. Every one took the news hard as expected. My brother came to visit one evening and I could tell that he had been crying before he came inside, he asked me, Tut how can you walk around with a smile on your face knowing that you might die, my answer was I had made Peace with God and whatever His decision was it had to be. But the truth of it was , I was living to die, I would wake up each morning wonder at what hour of the day I might die. I didn’t tell a soul because I wanted to feel brave, I felt that if I could hold my composure that I could keep every one else together.

On October 31, 2006, six months later I was rushed to doctor’s office because I wasn’t feeling well and my breathing was shallow. I was thinking to myself, Lord I know I’ve had six moths to prepare for this day but just wait, don’t take me yet please, I’m not ready. Dr. Ventura looked in my eyes and told my husband to turn the wheelchair around and head for the emergency room. They started working on me right away. My Mother showed up and my husband was torn apart, I don’t think they were much good for each other. By this time I knew that she was having a hard time excepting the fact that I was sick. Later on that evening, who shows up at the hospital but Stacey. Little did I know that she had called in sick and stayed home because she had felt that something was going wrong. I was of no help to her because I really thought I was going to die, it felt good having her at my side although all she could do was cry, I couldn’t talk to her so I just held her hand and we cried together. I found myself on a thirteen day stay in the Critical Care Unit at Ochsner Hospital .

The doctors were puzzled by what was going on because they couldn’t get a diagnosis from the blood test. They started sending blood work to doctors at universities in many different states. Non of the antibiotics were working and the oxygen was breathing 100% for me. Dr. Ventura came in and told my Mother that he was doing all that he could do but he was losing me. I had a deadly pneumonia and my body was not responding to the medications. I started praying Lord if you can DELIVER Daniel from the Lion’s Den, then Lord I know that you can HEAL me. Five days later Dr. Ventura comes into my room and says this must be a miracle from God because I had made a 360 degree turn with my breathing, they were able to turn down the setting of the oxygen.

I am currently taking a medication named Ventavis for my PH and it is working well, it has put me back on my feet. I Thank God everyday for my family and friends. God is my ROCK but they are my stand. They stand to give me encouragement every time I see that little smile on their faces when I do things for myself with some limitations but I count it all JOY! My Son is my sunshine, of course he is your average teenager, but I can get pass that. He’s always there when I need him, come rain or shine he’ s carrying oxygen tanks to the car, in the house, to the store and to Church. He’s even there to help with household chores, sometimes he complain but find me teenager that will not complain. My Mother and Grandmother to me is like a rose, their tears are like the petals once they’ve fallen and withered up the bush stands firm until it’s time to bloom another set of petals. My Father is quiet, but I can see the pain his heart endures every time I look him in the eyes. My husband won’t talk with me about how he feels, but he shares his emotions with other people when I am not around, sometimes I wish he would just say something. My sister is also quiet and won’t say much to me about it, but on the other hand, she still thinks that I should always do for her and think about me later, of course it’s why I love her, she’s my baby sister and I have her Spoiled. The kids in the neighborhood race to me when they see me outside, running just for a hug and a simple how are you feeling? Do you need help today?

God has blessed me to be able to attend a PHA Conference in Houston Texas in June of 2008. I was enlightened to have learned all of the information I did about living life with PH. It has changed my life completely, now I am inspired to raise awareness to other people about this Disease. I hope that in my endeavor to have a fundraiser for PH that I may change someone’s life forever. The support that I receive from everyone
Gives me a reason to FIGHT FOR A CURE!

In all that I go through, I know that God Will never put more on me than I can bear. Even when my clouds hang low I won’t complain because there is always somebody going through more than me. So I say to all Pher’s , family and friends when it seems as if it’s never going to end just STAND and let God see you through!

Marilyn's Story

2008-11-18T23:00:00.002-06:00

Today is PH Blog Day so here I go. I was diagnosed in 1997 with Pulmonary Hypertension, a rare, incurable disease that cause s high blood pressure in the pulmonary artery between the heart and lungs and can lead to heart failure. I've been on several therapies including IV infusion 24/7 for six years ; now I am on an experimental drug, some pills that I take twice a day. I have come a long way and I feel better now than before I got sick. Twent y years ago, the only treat ment was a heart - lung transplant . Thank God for modern medicine and possibly a cure in the future using stem cells from the patients own bone marrow. Everybody enjoy the holidays. Peace Out.

Your Phriend,
Marilyn
Woodland, California

Allen's Story of being a Caregiver!

2008-11-18T22:57:00.000-06:00

I am the surviving widower of a beautiful, loving, courageous PHer whom I choose to honor via this e-mail. While I have vivid and wonderful memories of our short time together, I want to use this e-mail to offer words of encouragement to the caregivers, spouses, SO’s, children, and other relatives and friends of all PH patients. Your support and love are so verrrrry vital and important to your loved one with PH. While you are taking care of your loved one, remember to take care of yourself. Keep an open dialog with your support group and do let yourself feel!

Allen

Kim's Story

2008-11-18T22:50:00.001-06:00

Hello. I was diagnosed with pulmonary hypertension in June,2008. This cameafter 2 years of shortness of breath, weight gain due to intolerance toexercise, frequent chest pain, fatigue to the point that I took a nap everychance I got and was unable to care for my family let alone my ownactivities of daily living..I kept going to my doctors and they kept sayingI must get the weight off. I was so discouraged because I would try so hardand the scale would not budge. I was a nurse with 32 years of wxperience andhalf believed that I was "just out of shape". I worked 12-13 hour shifts 2days a week till June..on my off days I could barely get up often sleepingin the lazyboy chair..My family was supportive..I don't know what I would'vedone had they not been..I had never seen pulmonary hypertension listed as adiagnosis and hap to look it up when my diagnosis was given..you see mycardiologist told me "you have pulmonary hypertension during the latter partof a cardiac cath...that was all the explanation I got-he left..I wasreferred to a pulmonologist a week later- you can only imagine what I wentthrough with the wait. I looked this up on the web and was thankful that Ifound the pha association website..I was given much info from people whohave gone through oh so much with this menace of a disease. I did see a phspecialist under the encouragement of the ph friends on this website..he wasable to help my symptoms with medication....5 months after diagnosis I havemany more good days...in June I really didn't think I would make it tillJuly..the shortness of breath was severe, leaving the house was dreadedsince I could not breath in the humidity..My son's wedding was in June and Ican barely remember it because I kept thinking I was going to collapse..Ihave sleep apnea and atrial fibrillation(which is controlled with meds) andI use a cpap machine to sleep at night. The doctors think my sleep apnea wascaused by my obesity and sleep apnea..I never smoked or took illlicit durgs,never had asthma ..never did I dream that I would have this . When you seeme park in a handicap parking spot you may not recognize me. You may think Idon't deserve to park there, trust me looks are deceiving. You can't lookat someone and know how sick they are. I have learned to cherish each momentof my day, my family,my friends..each experience...God's word says to Bestill and know that I am God. Yes I give thanks to God because He is withmethrough it all. A lot of people are on oxygen, lung transplant lists andiv meds to give them hope..and yes we do have HOPE..we need for people to bemade aware of this ilness so more research can be done to find a cure. Nowthe only cure is a lung transplant and often a heart is needed as well.Go totje phassociation website and learn more about this disease. I am sure thereare many people who have this that have not been diagnosed, meaniong they donot have access to proper treatment..Please learn more and help us to haveHOPE.

Kim

Jas's Story

2008-11-18T19:46:00.003-06:00

I was diagnosed with PH in August 2001 at the age of 42. I had been short of breath for quite sometime. I was told it was probably that I was taking a long time to get over the pneumonia I had. I was also told by the disability company that I might benefit from some form of physical conditioning to increase my physical tolerance. It was suggested that I might benefit from some goal orientated counseling. I actually had been off work due to lupus for a while and was trying to do a gradual return to work. I was struggling at work just making a bed.

One day I ended up in emergency very short of breath and very low oxygen level. It was in the 50’s.Once again I was told it was pneumonia. I was having a lot of pain, I explained to them it was like pleurisy which I had in the past after a bronchoscope. They did blood gases and X-rays , gave me oxygen and pain medication . They said the X-ray showed an enlarged artery. I was going to be admitted to the ward. I asked if I could go home. The respirologist said only when my oxygen levels came up. After quite a few hours my oxygen levels came up and I was able to go home and was put on antibiotics.

Since being diagnosed with Lupus in 1984, I see a rheumatologist on a regular basis. There was a student there that day and I was asked if he could also come in for my check-up. I told them about my visit to emergency and told them the X-ray showed an enlarged artery. I also told them how hard it was for me to walk up the flight of stairs on the ferry. All my life I had been a very active person and involved in sports, working full time and raising a family, now it was difficult to walk up stairs. The student felt the pulse on my neck and said I needed and echo before I fell through the cracks. He seemed very concerned. So my rheumatologist sent a letter to the respirologist.

My respirologist, which had seen me in emergency, ordered an echo. I went for a follow-up and that is when I was told I had pulmonary hypertension. He seemed quite serious as well .He said the pulmonary artery pressure on echo was 110. I was put on calcium channel blockers . I also had some oxygen testing and was put on oxygen at night. He said I would be seeing a specialist in Vancouver and would get a call.

I went home and looked up pulmonary hypertension and found the PHA website. I showed my husband and said, “I don’t have that kind of pulmonary hypertension.” I told him I probably have secondary pulmonary hypertension, which is probably not serious. That’s about how much I knew about pulmonary hypertension then. I also had a follow-up with my Doctor and asked him if I was going to die. He said yes you could, but we will deal with it the same way as we have with the lupus. My doctors have been very supportive with me since they had been treating me. They have always believed me when I would say something is not right.

I got a call on October 15, 2001 to go to Vancouver General for tests. I went over the next morning. That is where I met my pulmonary hypertension specialist. I had a lot of tests. I was quite happy that there was an oral medication now available for pulmonary hypertension. I had an ultra sound of my liver and was told that I would be having a liver biopsy. I found out I had liver fibrosis, which was probably caused by a medication called methotrexate. I was on this medication for lupus. That meant tracleer wasn’t and option right now.

I was pretty devastated when I left the hospital. My girls were 14 and 7 at that time. I was told to start on coumadin and we would wait and see if my liver enzymes came down after discontinuing methotrexate. So now I had to wait before starting a new treatment for pulmonary hypertension. At the end of April 2002 my pulmonary artery pressure was now 114. Thank goodness my liver enzymes returned to normal. I started on tracleer in June 2002 and I am still taking it. It’s been over two years since viagra was added. I am doing pretty well. My girls are now 21 and 13 and they say they notice that I can walk faster than I used to. My last echo in July 2008 showed a pulmonary artery pressure of 27!

Colleen S's Story

2008-11-18T14:50:00.004-06:00

My PH story begins when I was born in September 1975. I had a collapsed left lung, and I was taken away before my mom could even hold me. In fact, my mom didn't get to hold me for 3 days because she was dealing with an infection of some sort. Eventually, my parents got to take me home, and all seemed fine. I wasn't gaining much weight from feedings, and because of that, I didn't get my first set of shots at the time they were usually given. My mom took me to the free clinic for them, and one of the doctors there thought they heard something with my heart. He suggested that my mom talk to my pediatrician about it. She did, and my pediatrician heard nothing, so nothing was done. When my mom brought me for my second set of shots, at the same clinic, the doctor again said he heard something going on with my heart, and thought something was very wrong. I looked bluish around the lips, and he was very concerned. So, my parents again brought me to the pediatrician, who finally referred them to Children's Hospital in Buffalo, NY.

When my parents brought me in for the appointment with the cardiologist at Children's, the doctor took one look at me, and took me before all the other people in the waiting room. They knew something was very wrong! They did an echo, EKG, eventually an RHC (right heart catheterization, and finally told my parents that I had atrioventricular canal (a congenital heart defect, two holes in my heart), and pulmonary hypertension. They told my parents there wasn't much they could do, to take me home and allow me to live a fairly normal life. They weren't sure if I'd live a year (I was diagnosed at 9 months old), or 50. I think they were really thinking I wouldn't make it past a year! They told my parents to seriously consider having another baby right away. Of course, my parents were scared to death with this news, but they brought me home and did the best they could for me!

I spent my childhood very short of breath most of the time when I played with my sisters, or with the neighborhood kids. I did run around with everyone, trying to keep up, but I had to take a lot of breaks! Naps were a normal, almost every day occurance. I never played sports, or even took gym classes in school. I was limited on what I could do,but my mom still found other ways I could participate in things to feel like I wasn't missing out on too much. When my classmates were off at gym, I would go down to the first grade classroom and help the teacher with things. I was in 7th and 8th grade, and it was during that time I decided I wanted to become a teacher myself!

High school and college were more personal challenges for me to deal with. I wanted to be able to do things my peers were doing, but I knew that I couldn't do most of them. My heart seemed to handle things well so far, not having any major problems growing up. I went to my cardiologist regularly, getting echos every time I went. No issues really. Growing up, the only med I was ever on for my condition was digoxin, for the heart. Pulmonary hypertension seemed to just be in the background, and I never knew exactly what it was, let alone how to say it!

When I got out of college, I started subbing for a couple months before I found a job as a preschool teacher in a daycare. I worked there for a couple months before I got another job as a preschool teacher for Headstart. It was working at Headstart when my health slowly started to decline. My classroom was on the 2nd floor, the cafeteria where the kids had to eat (and we were required to eat with them) was in the basement, so climbing 6 flights of stairs a few times a day started really hurting me. Not to mention that little preschoolers are germ factories! I also was dating at the time, trying to go out partying almost every weekend, and going back to grad school for my masters' degree. During my third year at Headstart, I really started getting sicker. I had double pneumonia, bronchitis, and was on my third respiratory illness when my PCP told me, with tears in her eyes, I needed to quit my job. But honestly, I don't think I'd still be here if she didn't tell me to do that!!

I quit my job, but decided to finish my masters'. I spent a lot of time recovering from being sick while working, and just getting a lot of rest besides going to school. My PCP eventually referred me to the Cleveland Clinic for a lung transplant evaluation. I went for the first time in 2001. I was put on oxygen 24/7, and told I needed to start Coumadin. The next couple times I went back, transplantation was talked about, but I didn't have any testing done for it. In 2003, I was told I was going to start Tracleer. I had no clue what this was, the doctor really didn't tell me! A month later, I realized it was a pill. Whew! I had read about Flolan, and I sure did NOT want to start that!

I began Tracleer on October 12, 2003. People pick on me a lot for remembering dates for things, but remembering a date that significantly changed my life is no laughing matter. I didn't notice any changes with the Tracleer until maybe a month after starting it. It was extremely subtle, but a change, nonetheless. I went to vote for something, and on the walk back from the building to my car in the parking lot, I realized I wasn't short of breath! I couldn't believe it! I got back home, walked from my garage to the house (which sometimes causes me to get SOB), and felt ok! I started crying! My life was all about being SOB most of the time doing anything physical, and here I was, feeling OK. After that, the Tracleer kept improving my condition. I went from never being able to take gym at school, to walking 2 miles on the treadmill!! It was like magic!

Over the next few years, I felt the Tracleer had sort of tapered off. I asked about Revatio, and started it on August 16, 2007. After being on it for awhile, I felt it helped, but I did increase the dosage again about a year later. Between Tracleer and Revatio, I feel good most days. Yes, I still get tired, yes I still get SOB, but it's not as bad as usual when I was growing up. I've learned to make adjustments to whatever I do. I've learned to take my time with things, and not overdo something. If I'm going to exercise one day, I won't also clean. If I'm going to attempt vacuuming, that will be my exercise! If I'm going to have a full day being out somewhere, I make sure the next day is for rest. I usually stick to what I say I'm going to do, although I can be hard-headed about stuff and push my limits every once in awhile. But for the most part, living with PH has been much easier for me with medicines that were not even available 33 years ago when I was diagnosed. I thank God for allowing me to have so many years so far, and I pray it can continue!!

During the past 7 years since I've been going to Cleveland for treatment, I have found a new focus in life, PH awareness. Since I found out about the PH Association, I've been going there daily, sometimes several times a day, to read the message boards and chat with phriends. I host 2 chats there, and I answer questions or respond to posts when I feel I can share something useful. I also run my own PH support group in Buffalo/Niagara Falls. That was definitely a blessing for me to start, not only for my own sense of belonging, but for those who join, too. I keep busy with my life, and I surely rest when I need to do so! I'm managing with my PH, and I can only hope it continues!

Parker's Story (as told by mom, Jennifer)

2008-11-17T23:01:00.004-06:00

“Parker has a condition called pulmonary hypertension. That means the blood pressure in his lungs is too high. His heart has to work really hard to get the blood to his lungs and that’s why his heart is enlarged. Now, there’s all sorts of tests we will need to do to see if we can find a cause for the pulmonary hypertension and he’s going to need to stay on oxygen, so you’ll be here for a few days while we figure everything out. And, stay away from the internet because if you read some of the stuff that’s out there it will scare the H-E- double hockey-sticks out of you…”

On February 29, 2008, my baby boy, Parker, was diagnosed with pulmonary hypertension, a rare lung disease. As the doctor at Brenner Children’s Hospital explained his new diagnosis to me, I actually felt a bit of relief to finally know what was going on with him, but I had no idea how my life would be turned upside down as I began this journey with Parker.

Parker was born on April 30, 2007 after a long day of waiting. He weighed 8 pounds, 9 ounces and measured 21 inches long. He was healthy and beautiful. We were overjoyed to be blessed with this sweet baby boy. As the weeks passed, Parker seemed to be fine. He wasn’t a very good eater and he wasn’t as big as my other two babies had been, but he was happy, easy going and content. By the time Parker was 8 months old, he still couldn’t sit up by himself, he wasn’t gaining weight like he should, he had a random cough and wheeze and I had a nagging feeling deep inside that something just wasn’t right.

On February 26, 2008, after a month of high calorie formula, Parker had only gained 5 ounces. Our pediatrician finally heard Parker cough and wheeze while we were there and I saw a look of concern on his face as we discussed a few common things that could cause Parker’s symptoms. He recommended treating Parker for asthma and reflux and he also agreed to order a chest x-ray at my request, even though he really felt that it would be normal. It wasn’t normal--it showed an enlarged heart, something none of us were expecting. That led to an EKG, which was also abnormal, and that led to a hospital admission and an echocardiogram that diagnosed Parker with PH. Parker had every test you can imagine while he was in the hospital, as his cardiology team tried to find a reason for his PH. All the tests came back normal. He was scheduled several weeks later to have a heart catheterization, the only test available to accurately measure the blood pressure in the lungs.

On April 8, 2008 I took Parker to Brenner Children’s Hospital for the heart catheterization, expecting to only be there overnight, and to come home with some answers. Instead, Parker ended up in the ICU, after having what the doctors called a pulmonary hypertensive crisis. I left my sweet baby boy that morning smiling and playing and saw him at the end of the day lifeless and white with countless tubes and IV’s hooked up to him. He was on a ventilator and he was completely sedated. It was hard for me to comprehend how this had happened. I was completely overwhelmed; all I wanted to do was take my baby in my arms and rock him, and I couldn’t even do that.

Because of complications with the breathing tube, Parker stayed on a ventilator for 18 days. My husband and I stayed with Parker constantly. We prayed for him to pull through this terrible ordeal and come home with us, but we also tried to prepare for the worst. Finally, 4 days before his first birthday, Parker came off the ventilator and he celebrated his birthday in the ICU. 13 days later, we brought him home--this time with oxygen, a permanent IV in his chest and several oral medications.

6 months later, Parker is like a new baby! He finally weighs 20 pounds, he crawls, climbs, and gets into everything like most 18 month olds. But unlike most 18 month olds, he wears a little backpack wherever he goes that holds his IV pump, he is continuously hooked up to oxygen, and he maintains a strict medication schedule. Despite all of this and all that he has been through, Parker is still as happy, easy going and content as he was when he was born. We are so grateful for the progress he has made in the last 6 months and we are so grateful to be part of his life. We may never know why Parker has this terrible disease and we may never find a cure for him, but there’s always hope and we can never give up on that.

Donna's Story

2008-11-17T21:52:00.003-06:00

If you are a regular reader of my blog, by now you've seen the footer that I try to include on all of my November posts, the one that is a "public service announcement" about my condition, Pulmonary Hypertension.

While the entire month is PH awareness month, Tuesday, November 18th, is PH Awareness Day. So why the fuss about a rare condition that only affects about 100,000 people worldwide? Because the symptoms of PH (dizziness, shortness of breath, chest pains) can be attributed to multiple other conditions. Because by the time PH is diagnosed, the condition could have progressed to late stage. Because PH can be a secondary condition to other illnesses such as clotting disorders, Scleroderma, emphysema, bronchitis, and COPD. Because early diagnosis can prolong life. And because anyone, ANYONE can be diagnosed with this life-threatening, incurable condition. It cares not about race, financial condition, gender, or religion.

I try not to let the fact that I have a chronic condition affect how I live my daily life, but, as would be expected, changes have been made and certain precautions are taken on a daily basis.

Some things I bet you didn't know about living with Pulmonary Hypertension:

...that because of an enlarged right ventricle in my heart, I am unable to take decongestants for a cold. I am vulnerable to racing heart episodes

....that it takes me twenty minutes or more of relaxation time to recover from sorting the laundry. Bending over is very difficult for me because of the propensity to dizziness, and the "strain" of bending over repeatedly forces me to use more energy than I can expel easily. Then there's the whole lugging the laundry basket to the laundry room and actually picking it up (or bending over) to load the machine. Whew!

...that after a morning of standing over the copier machine in the school work room, I come home and lie in bed for a couple of hours to "recuperate."

...that I have difficulty walking up even the slightest incline. The front walkway to the school office slopes up ever so gently, but to me it might as well be a mountain

....that I use a shopping cart just to have something on which to lean sometimes. Just leaning on something can be helpful, which is why at times I have been known to use a cane. I'm considering getting a "rolling walker" as well

....that, although I have the legal right to park in a designated handicapped parking spot, it's not always the most convenient because it's not located right by the front door. Many times, the spot is located at the end of the walkway, because that's where the ramp is. The builders don't consider that all handicapped people don't use a mobility aid; sometimes proximity is more important than a ramp

....that many times I am not able to chaperon a field trip with my son because I can't handle all of the walking around. This makes me, and my son, very sad. But I always manage to make award ceremonies and help with homework.

...that the reason I have maid service is not because I'm lazy but because I'm physically unable to handle the housework for my 1800 square foot home.

....that whenever I travel all of my medications (a total of 12 different ones) need to be carried in a gallon Ziploc bag so that all prescriptions are visible through airport security. I also have to carry my CPAP (breathing machine) that treats my sleep apnea. You can see why I hate to fly, right?

...that even day trips away from home are managed so that I won't be too far from a rest room because of the diuretics ("water pills") I take.

...that I'm considered the "least sick" of those with my condition. In spite of these limitations, I still have an enormous amount of freedom. I don't use oxygen, I don't have a catheter for medicine, and the biggest thing of all - I'm NOT bedridden.

All in all, I'm still a very blessed individual.

**If you would like to read Donna's private blog, posted to the right in the blogroll, please email her at azdice@gmail.com to ask permission!**

Colleen B's Story!

2008-11-15T21:14:00.003-06:00

My name is Colleen and I am a 29 year old wife, and mother of a wonderful two year old boy. I am a daughter, a sister, and a granddaughter. I am surrounded by incredibly supportive family and friends. I don’t look or even act sick, but I have Primary Pulmonary Hypertension.

Prior to my pregnancy, I was extremely active. Fast forward to a few months after the birth of my son. I began to exercise to try to loose the baby weight. Throughout the year of 2007 I became increasingly short of breath on exertion, which I chalked up to needing to get in shape. That winter I came down with pleurisy (an infection in the lining of the lungs). I had three bouts of the illness in a little over a month, with the final one landing me in the ER. There, the workup showed an enlarged heart and a slightly abnormal EKG. I was told it was probably nothing, but to go see a cardiologist “just in case”.

That “just in case” turned into a nightmare very quickly. On January 2, 2008 I received my diagnosis. I made the mistake of coming home and looking it up on the internet. Thanks to vast amounts of misinformation, I thought I was going to die, and soon. Words like “progressive and fatal” are common when reading information about PH, and there really are no words to describe what it is like hearing them applied to you. News like that would rock anyone’s world. As the mother of a young child all I could think of was my son, and how he might not have a mommy to grow up with. I decided, in true stubborn Irish fashion, that this was unacceptable.

The crazy thing about all of this is that on most days I feel fine. I won’t be climbing mountains or taking a jog anytime soon. I’m not a big fan of stairs. But, I keep up with a two year old and all my normal activities just fine. In the back of my head I always know I am sick. I know that the nature of this disease is I will probably worsen as time goes on, but often it is hard to believe.

Life expectancy used to be 3 – 5 years from diagnosis. Thankfully, the treatment for PH has come an incredibly long way in recent years. Now I, the girl who barely used to take aspirin, am on three different drugs. I sleep with an oxygen tube in my nose. All of these medications have improved my quality of life, and will probably prolong it to some degree. However, they won’t save me, they won’t cure me. I need a miracle for that. I don’t care if the miracle comes from God or the breakthroughs of medicine (are they one and the same?), but that is my only chance of survival.

Through all of this, I feel blessed by other miracles. It is a miracle that a nasty “bug” led to my early diagnosis. It is a miracle that my cardiologist was on the ball enough to find the proper diagnosis and place me in the hands of a great specialist. This diagnosis can take years to get, and I got it in a few weeks. It is a miracle that I am even alive. There is about a 50/50 chance of surviving a pregnancy when you have PH, and I made it. Now, I am waiting for the miracle of my cure. I have faith that I will get it.

**Colleen has a PH blog set up, please visit it by clicking here!**

Eliana's Story!

2008-11-14T23:05:00.001-06:00

My name is Lyndsi. I am 28 years old and when I was only 23 I learned that my baby had Pulmonary Hypertension. Her name is Eliana. She is our middle child. Jake (my husband) and I have 3 children all together. Eliyah who is 9, Eliana who is 5 and Israel who is 4.

Here is our story: I was induced with Eliana because they knew there was something wrong with her heart, so they wanted to make sure that they had all the staff ready for her. She came into this world on her own time! They told us it usually takes many women a long time to deliver with petocin (sp?) so we sent home our family and told them we would call when we thought it was close. However, in true “Eliana style” she was out in no time, so it was NO EPIDURAL for mommy! Once she was born, she was very blue and having trouble breathing, so they let me get a quick glance and rushed her down to the Newborn ICU. A little later I got wheeled to my room and couldn’t wait to go down and see her. Jake (my husband) had left the hospital to get us some food so I called the nurse to take me down to the NICU. As the nurse and I entered the doors we were approached by 3 or 4 nurses/doctors ( I wasn’t sure at the moment) who told me I needed to go back to my room and I that I couldn’t see her yet. I was so upset, scared and confused, and to this day I still have no idea why I wasn’t able to see her then. I sobbed on my way back to my room and quickly called Jake and told him what had happened. After a few hours of waiting, they told Jake and I that we could go down and see her. I remember seeing her for the first time hooked up to every tube and every machine imaginable, including a ventilator to make her breathe. I was so afraid to touch her, but wanted so desperately to hold her.

In the coming days she was able to come off the breathing tube and only had oxygen, and eventually (after 2 weeks) we were able to take her home. Jake and I were terrified taking her home with oxygen tanks and tubing swirled through our home. I remember falling asleep every night with my hand on her chest so I could feel her breathe and sliding my had over her little nose to make sure I could feel her oxygen tubing was in place. She would breathe so fast (80 times per minute…try it and see how out of breath you feel) and couldn’t drink her bottles because she was working too hard to breathe. We used to add vegetable oil to her milk for extra calories because she wouldn’t gain weight.For five months we went back and forth to the doctors (cardiologist, pulmonologist, and pediatrician) to try and find what was wrong with our baby. The pulmonologist saw her and said, “Her lungs look cloudy on x-ray, but she should grow out of it. Bring her back in 3 months.” I knew we didn’t have 3 months.

Finally our cardiologist said that she thought she might know what she had, but she didn’t feel comfortable enough to treat it. She said we should consider going to Denver Children’s Hospital to get her looked at. I said “Send us NOW!” I remember driving into Denver feeling sick to the very core, so unsure and afraid of what would happen. All I knew was that they thought she had this disease called Pulmonary Hypertension. I didn’t know what it meant, but I knew we could either leave Denver with oral medication or an IV that they called a broviac in her chest that would be connected to a pump. I definitely didn’t want that for my baby, so we prayed for the best.The next day she would be seen for a consultation with the cardiologist and the day after that they would take her into surgery to have a procedure done called a cardiac catheterization.They told us that most kids do really well and were up and around the next day. I still so vividly remember laying her on the table and walking away….that has to be one of the scariest times of my life. I remember waiting for 4 hours in the waiting room wondering, and questioning God, “Why are we here? Why us? Why our baby?”

Finally the doctor came to talk to us and told us that she was very very sick. She needed the IV medication or she would die. She was so sick they weren’t able to take her off of life support and we needed to make a decision. Needless to say, Jake and I were absolutely devastated. I remember walking down the hallway of the hospital and falling to the floor crying. In a matter of two days we found out that our baby was dying from a disease that was fatal and if she could pull through this latest set back, her only hope for survival was an IV in her chest. What were we supposed to do? The next thing Jake and I did was pray, we prayed for God to show us what to do and we decided to go through with having the IV placed. We finally got to see our baby after this long procedure and I have to say, she looked nothing like Eliana. She was so swollen and again hooked up to every monitor, tube, machine imaginable. Day and night for 3 weeks we stayed by her side watching her gag on the breathing tube over and over and after 2 very scary tries, she was able to come off the breathing tube. During this time, we received a crash course on how to care for our child. We learned that this new line would be her life line and we had only 3-5 minutes to get her a new line if this one came out accidentally. Her pump could never be stopped or it would be fatal. I remember sitting in a conference room at the hospital with Jake, my mom, dad and little Eliyah (our oldest daughter, she was only 3 at the time) as a nurse taught us how to mix her medication. There were syringes, needles, masks, gloves, pumps, medication, saline and a book full of instructions. I felt like a zombie as I sat there listening, but all it sounded like to me was like the sound of the phone calls on Charlie Brown, “Wa wa wa wa wa.” That’s all I heard.

Slowly Eliana recovered and we were able to make our journey home with so much more than we got there with. Now we were even more terrified than when we first brought her home on oxygen. We had to begin a rigorous schedule of mixing meds, oral meds, oxygen, and dressing changes, complete with sterile drapes, gloves and masks.As we learned more about this disease from the internet, we felt so alone and so afraid, and we knew we had to kick our faith into full gear! Since then we have held on to God with all that we have and we have not let go!

Five years later we are still doing all the things we did in the beginning for Eliana’s care each and every day, and our arms are a little tired from holding on so tightly to God, but everyday He give us strength to go on and we do just that.We feel so blessed to have Eliana for another year. After 5 years of having PH you are considered a SURVIVOR! 5 years is the average life span of a PH patient after diagnosis, so her birthday this past July was a HUGE deal!

Lyndsi

Barbara H's Story

2008-11-13T20:57:00.002-06:00

I am living with Pulmonary Hypertension.

In Dec 2004, I was admitted to the hospital, On an echo cardiogram it showed that I had mild Ph, I was not told about this, nor did I know.

In August 2005, I started going to the Dr's with SOB, chest pain, and they assumed I did not want to work, so I was put on allergy shots.

In September 2006 I had been to a cardiologist, three primary care dr's and two nurse practitioners, I was desperate to find out my problem, I had a stress test done and told it was negative.

I walked into the dr's office and I told him, in Nov 2006 that I could not breathe, could not walk, I was in really bad shape, he wrote me a prescription for a nerve pill, handed it to me and told me to be sure and take it everyday. I never took a one of them. I made it through the holidays that year and decided that maybe it was my nerves and I should have taken that pill after all.

At the end of Feb 2007, I went back to the dr's ofice because I felt like I was passing out, I was having severe stomach pain, mild chest pain, nausea, so he admitted me to the hospital again.
I had lots of test done and I was tired so I feel asleep, I woke up and there were 3 cardiologist, a pulmonologist, and a medical dr standing all around me, I thought I must have died. Then I was told the news and I was sent to a Pulmonary Dr in Lexington, ky, he was so good to me.

I was put on Revatio after he did a heart cath, and my pressures dropped from 95 to below 30 now.

I just recently started taking Letairis 5 mg once a day along with the Revatio.

I am feeling much better now health wise, although I am not happy with the illness, I do pray for a miracle cure someday.

Barbara

Susan's Story

2008-11-13T10:44:00.004-06:00

My name is Susan. Until I was 48 years old, I was mostly a normal person. I was married, had three nearly grown children, had a teaching job I just loved and was relatively healthy--or so I thought.

I had had trouble breathing when I walked up any incline for about a year and a half. I thought that I must be out of shape and so I started to walk more to get back in shape. Several months before my 49th birthday, I also started having terrible gas pains; not in my abdomen where you would think, but in my back and around my heart. Sometimes I even thought I was having a heart attack. One day I walked across campus and up a long flight of stairs. When I got to the top of the stairs I was completely out of breath. I was desperately gasping for air and my heart was pounding out of my chest. I felt like I had just run a marathon and wasn't going to make it. I had to lean up against the wall at the top of the steps and wait until I got could get control of my breathing before I was able to continue down the hallway. That's when I knew that something was horribly wrong and I was sure it was my heart.

In December 2000, I had a stress test at my local hospital. I did less than two minutes on the treadmill before my heart rate was high enough to get an accurate reading on the EKG. Good news! My heart was in good shape. The cardiologist who read my stress test recommended I get an echocardiogram to rule out pulmonary hypertension, a disease that I had never heard of. The echo confirmed his suspicions but I still had no idea how serious it was. When my PCP got the results of the echo, he immediately made an appointment with a Pulmonologist. The Pulmonologist ordered many tests (I thought at the time that it was every test known to man) and referred me to a PH specialist. When I saw the PH specialist, he had even more tests done to confirm the PH and rule out anything else. He decided that I did have PH and that it was secondary to CREST syndrome. The good news is that it was caught fairly early and that I was a good candidate for Flolan and a transplant. I started Flolan, a continuous IV medication in 2001 and it saved my life. I taught for a semester after that but in early 2002, after a second bout with pneumonia that required 18 days in the hospital, I decided it was time to retire from my beloved profession.

It's been almost 8 years since all this started. When I was diagnosed, Flolan and lung transplant were the only treatments for PH. Fortunately, the Flolan worked so well that there was no need for a transplant. Still, I had a lot of side effects with Flolan and went through a period of depression. Since then there has been an explosion of treatments. In 2005, I was converted from IV Flolan to IV Remodulin in combination with Viagra. This combination reduced many of the side effects that I had been having with Flolan. Although I am still disabled to work, the new therapy mitigated many of the side effects of Flolan and gave me back a large measure of the quality of my life. I am still very functional and lead a full, rewarding life. But I still hope for a cure for PH someday.

Susan

Barbara T's Story!

2008-11-12T21:44:00.000-06:00

Here's my PH story but there are many more just like it:

The main thing that we all have in common is being misdiagnosed for so long and the lack of awareness of the symptoms of PH, Pulmonary Hypertension. Had I known them, maybe I or my husband would have put the two and two together & I would not have been bedridden for so long. Prior to my diagnosis, Chuck took me to 14 different doctors, I had 3 hospital stays & many trips to the ER.

I went to my doctor's office a lot because of the bronchitis that just would not go away. I even went once with my feet and ankles being so swollen that I couldn't put on my shoes. My primary care physician is with the University of TN Medical Group. I thought that if I were with a group of new doctors, they would be more up-to-date on "newer or rare" diseases.
Since I have so many illnesses and am on so many medications, I stomped my foot to see the same doctor on every visit so that he would be familiar with my case. Finally, December 9, 2002, he ordered an Echocardiogram to make sure I did not have a fluid buildup around my heart. Well, he called me on the 12th to let me know I had PH. It went right over my head. Since I had never heard of it, it couldn't be too serious. I had asked him about a lap-band or having a gastro-bypass and he said let's get a handle on the PH then worry about the weight. The shortness of breath, the heaviness of my chest, the swollen ankles, the extreme fatigue, hoarseness and lightheadedness were all attributed to my weight, asthma & ulcerative colitis. I also have Lupus Anticoagulant that caused three strokes in 1991 at the ripe old age of 37.

I went to a PH specialist in Nashville. He said that he did not think that I had PH although the cardio who did the right heart cath said I had mild PH. My PCP was not convinced so he sent me to a local pulmonologist who sent me to another cardiologist for a right & left heart catheterization. Turns out that my pressure level had increased since no treatment was started earlier. It went from 24 in Feb. to 31 by May. (normal is 7-12) I was in Class IV meaning that I had the inability to carry out any physical activity without symptoms & the right side of my heart was enlarged. Dyspnea, (shortness of breath) and/or extreme fatigue were always present even at rest. Discomfort was increased by any physical activity.. I began treatment with Tracleer in June of 2003.

I'm now 54 and really miss working with my husband, Chuck, our daughter and son in our family-owned wedding business. I have been unable to participate in the weddings since 2001 due to this disease. It took over two years for the PH to be diagnosed. I wondered for the longest why God would take the weddings, my girls - the brides - and working with Chuck every day from me since I loved it all so much. I know now that it is to make people aware of pulmonary hypertension & its symptoms.

The sad part is that this disease is easily diagnosed with an echocardiogram if only the physician knew about it! The problem is, many, (in our case 14 different ones), do not know about it. I do not blame anyone. If you don't know about a disease, you just don't know. This has GOT to change. The public AND THE DOCTORS must be educated about this insidious disease or at the very least be aware of it & its symptoms.

PH, high lung pressure, is in no way connected to the type of hypertension that is measured with the cuff on your arm. The only way to confirm PH is with a heart cath. I was originally dx'd with an echo. It estimated my PAP (pulmonary arterial pressure) at 78.
http://wwwphassociation.org/Learn/What-is-PH/

http://wwwphcentral.org/medical/whatisph.html
I've received a lot of knowledge & support from these groups. I've also started a Support Group in Memphis, TN.

Barbara,

A PHer

Diana's Story

2008-11-11T20:21:00.002-06:00

Hi! My name is Diana and I have PH. Although my numbers are low (27 at rest), I knew after attending the PH conference this summer and speaking to other PHers I was a member. Funny thing is I felt relieved as I had been going to different cardiac doctors telling them I was feeling worse not better.

Finally I went back to a family doctor who had "guaranteed" that I wouldn't have any heart troubles for five years... advising I could "do anything" as far as exercise was concerned. Little more than a year later I had a pericardectomy, (removal of the pericardium aka "sack" around your heart. Going back to the doctor turned out to be the smartest thing I could have done as he sent me to another cardiologist who diagnosed my pulmonary hypertension through the echo, which if you are reading this as an informed person is not considered a means for PH diagnosis.

As the cardiologist's nurse gave me the diagnosis over the phone ( I never heard of pulmonary hypertension before then), I got on the internet and having the blank scared out of me is an understatement!

So, I packed a suitcase and traveled to the conference to "see what I was in store" for. No ticket for the conference, not even sure if I'd be allowed in...but I knew I'd find someone who'd speak to me, even if I had to stand outside the hotel and wait for people to leave!

Thank God I didn't have to go to such extremes! I slept in my car the first night before the conference after driving alone to Houston, Texas from Fayetteville, Arkansas for the first time. (I had to conserve my money as I wanted to get into the seminar if allowed! Remember I had just been told earlier in the week!)

I met some wonderful people and got help with a room for the next 2 nights and learned more than I could have for years on the internet! Found a good , no great doctor, and have traveled twice out of state to see him!

Although it is sad I have been diagnosed with PH, I feel lucky to have gotten diagnosed as soon as I have so maybe I can limit the damage to my heart.

People have said my story is so unbelievable, yet its true. My dr. says I have arterial changes and I will be looking at a lung transplant which scares me, but I will do whatever I have to to give the example to my 4 children..."don't ever give up!" It has taken me all summer and into the fall to come to this conclusion, but it is what I want to leave for a legacy!

God bless you all!

Diana

Sue's Story

2008-11-08T19:00:00.002-06:00

Hi,
My name is Sue and I have secondary Pulmonary Hypertension caused by Limited Scleroderma. I was diagnosed the end of April, 2008. But first I will tell you a little about myself. I am 53 yrs. old, married with two grown children, four grandchildren and a new grandson due in January. Before diagnosis, I worked at a factory where rope was made and distributed. Now on to my PH story:

In February of 2008, I began experiencing shortness of breath whenever I went upstairs, did laundry or walked from my car to wherever. It would last for several minutes until I rested. This was unusual for me so I went to my Family Physician who gave my inhalers for asthma. Two months later I was still short of breath on exertion, so he decided to do an echocardiogram and the results were PH. From there I was sent to a PH specialist who did all the expected tests including a right heart catheriztion which showed pulmonary pressures of 110. Normal pressures are 25-30. So I have severe pulmonary hypertension. Tracleer was started in July but was stopped for two weeks because of liver enzyme elevations. I have restarted it again at 1/2 dose for a couple of months and go from there. I have improved to where my heart is back to normal size and function. I no longer have SOB, and generally feel pretty good. With the treatments available now and more forthcoming in the near future, I feel confident I will be treated successfully. Thank you for reading my story. I live in Ontario, Canada.

Sue

Marcia's Story

2008-11-07T18:12:00.004-06:00

I have a rare disease and I think it's important for you to read about the details of this disease and my life.
I used to go to camp in Oklahoma every year with my 5th graders. We hiked and climbed the Arbuckle Mountains and I was very active. I did this for 9 years before transferring to another school and teaching 4th grade. During the spring of my second year at this new school I noticed being short of breath (sob) whenever I walked quickly and then tried to talk (which is quite a bit when you are a teacher!). I began noting that I was sob quite a lot and for things that didn’t usually leave me breathless. I was 52 and not excessively overweight.
After 15 months of many doctor visits, including a cardiologist and pulmonologist and many, many tests showing nothing amiss, and being diagnosed with asthma, I learned that I had quite a serious disease. I was in left heart failure by then, with several side effects and had Pulmonary Hypertension. It is a rare illness with statistics of 2 in a million people. I had never been seriously sick! I couldn’t be in heart failure! I spent a week in the hospital, when the only time I had been to a hospital was to have babies. Denial did cross my mind.
As the reality hit me and the tests continued I sought support and found two websites dedicated to helping people with PH. There is an abundance of caring, knowledgeable people there and I learned all I could. I was a great candidate for clinical trials of new drugs to help others. My left heart failure healed with the right drugs. I now have wonderful doctors nearby who specialize in this rare illness. I am new to the world of Pulmonary Hypertension. I'm lucky because many PHers have to have an invasive medicine that is infused through an IV and pump.
I was lucky I didn’t get it ten years ago when medications were few. I’m blessed to learn I have it at a ripe age when my children are grown. You can’t have children because the medications cause birth defects and the pregnancy endangers the life of the mother. Many learn of their diagnosis in their 20s, when they are just beginning their lives. Many have young children but no energy to care for them. They haven’t been in the work force long enough to qualify for Social Security or retirement. Many are too far from PH specialists and have to deal with local doctors who don’t know anything about their disease. I hear horror stories everyday. And occasionally, about every 3 months, we get the sad news that another person we know has passed away.
I have a large network of internet Phriends, as we call ourselves. There are 6 approved drugs out now with many, many more in clinical trials. I have been able to participate in two clinical trials. I take a new drug, Letairis, in which I was in the clinical trial for 2 years. I am currently taking oral remodulin, which is in clinical trial.

When you have a disease this rare you want to do all you can do to help others who are fighting for their lives. There is hope. Most of the people I know with PH were told they had 3-5 years to live and to get their affairs in order. Fortunately I was not told that and more people are living longer with this disease than ever before. I plan to be around for a good, long time.. There is hope and that's what my life has now become ~ living for and fighting for hope and a cure.

Marcia

Pam's Story

2008-11-07T18:11:00.002-06:00

MY NAME IS PAM, I WAS DIAGNOSED IN 2001 WITH PRIMARY PULMONARY HYPERTESION WHICH IS LOW BLOOD PRESSURES IN THE LUNGS NOT HIGH. I HAVE TO TAKE VENTAVIS ,BEFORE THAT I WAS ON REMODULIN SUB Q.AND I AM STILL ON TRACLEER. I SEE THE LUNG DR. ABOUT EVERY MONTH TO THREE MONTHS. MY HEART DR. UNLESS I HAVE PROBLEMS I SEE HIM EVERY SIX MONTHS. MY HEART NUMBER IS 90 AND I HAVE WENT FROM 140 IN MY PULMONARY WAVES TO THE 70 OR 100. SO THE PRAYERS AND THE MEDICINES I TAKE HAVE HELPED ME TREMENDOUSLY.THERE IS HOPE FOR ALL WHO HAVE DISEASE ESPECIALLY LUNG DISEASE LIKE OURS. I GO TO THE PAH MEETING AT LEXINGTON HOSPITAL IN WEST COLUMBIA WHEN THEY HAVE THEM. I AM TRYING TO PLAN TO GO TO SPARANTBURG ON NOV 8.I WEAR OXYGEN AT NIGHT BECAUSE MY OXYGEN LEVELS GET DOWN TO 75 AND THAT MAKES THE HEART PUMP EVEN HARDER NOT GETTING THE OXYGEN. I WANT TO WRITE MY CONGRESSMEN AND OTHER REPRESENTATIVES BUT I JUST DON’T KNOW WHAT TO SAY. HOW TO SHORTEN THE DISEASE I HAVE WITH ALL THE TEST AND MEDICINE AND DOCTORS I HAVE SEEN OVE R THE YEARS. NOW FOUND OUT I HAVE FIBROMYALYGIA.WHEN I WAS ON REMODULIN IT CAUSED RHUMETHYROID ARTHRITIS THEN I GOT OFF OF THE REMOD. AND NOW I HAVE FIBRO. I DON’T UNDERSTAND WHAT IS GOING ON WITH MY BODY. I HAVE CHF FROM THE PPH WHICH MEANS THE RIGHT SIDE OF MY HEART IS SWOLLEN AND I GET FLUID BUILD UP SO ON MEDICINE FOR THAT TWO DIFFERENT FLUID PILLS. WELL ,THAT’S ALL I FEEL UP TO WRITING.
THANKS,

PAM

Mary's Story!

2008-11-07T18:10:00.000-06:00

My name is Mary, and I am probably the oldest patient with Pulmonary Hypertension. But it doesn’t really matter, 8 months to 80 years, it is the same terrible disease. I began with the usual symptoms- fatigue, shortness of breath, unable to climb stairs, having to “take a break” several times while preparing a meal, doing laundry, etc. I have been to good doctors and bad. I was underdiagnosed, undiagnosed, misdiagnosed, etc. After six years I finally found a doctor who would listen to me—REALLY LISTEN—and also talk to me. After a right heart cath, he diagnosed me with mild pulmonary hypertension secondary to scleroderma. I have been on Tracleer for nearly three years and have been stable for the past year. I am still not able to do much, but at least I am here and watching my grandchildren grow up. My advice to new patients- advocate for yourself- no one else is going to do it for you. I also am a firm believer that we need better education for medical personnel, including doctors, so this disease can be recognized faster and patients sent to the proper specialists. I believe there must be many like me who are treated for asthma, copd, etc. who, if their doctors were only willing to take it a step further, would find they have pulmonary hypertension and obtain treatment. Pulmonary Hypertension is progressive, but it can be slowed and often stopped, and with research we will someday find a cure.

Mary

Susie's Story

2008-11-07T18:05:00.003-06:00

My journey first began when I was 29 years old. I was always active and I began to get short of breath after a little exertion. I let it go, figuring I must be out of shape. I let it go until I was dizzy and my left arm was going numb. I went to my doctor, who did test after test to try to figure out what was going wrong. I was sent to a heart specialist, where I only saw a nurse. I was sent on my way, and the nurse told me that I was just out of shape and that I needed to start an exercise regimen. Embarrassed by this, I didn’t go to the doctor again for several months.
I finally went back again with the same symptoms. I was tested again. Again, nothing. I was put on high blood pressure medication and I was sent home. It would be another six months before I went in again. But now, symptoms were worse, and now my left arm was going numb as well. My doctor did more tests. This time a chest x-ray was done, and an Echo and CT scan were performed.
One month later my life changed. My doctor told me that my right ventricle was enlarged. I did not think that it was as serious as it was until I made the mistake to go on the internet and search. I ended up on some old sites with old news and I was scared to death. I literally pulled some of my hair out and was crying so badly. Word of advice: Do not go to unreliable or old internet sites, because they will scare the heck out of you!
Later, I went to the Mayo Clinic to see the specialist, and I was immediately hospitalized because they thought that I had a small blood clot in my lungs. I underwent two cardiac catheterizations and the pressure in my heart was 110. I probably would not have lasted going undiagnosed much longer. I was counseled on my condition extensively at St. Mary’s Hospital; what a great place, so caring. I returned home to Michigan, scared to be away from the safety zone I felt that around me when I was at Mayo.
I had gone into a depression for quite some time, I was so scared to leave my kids and husband without me. I signed up for the conference in 2006, but I couldn’t bring myself to go. After I got over that depression I decided to make sure I would know all that there is to know about this disease, no matter how painful it may be. I researched the newest medication, the best doctors, nutrition, exercise; anything I could find. I am now in control of my health.
I decided to do research on a doctor at the University of Michigan. Her name is Vallerie McLaughlin. I made the decision to have her monitor my condition. It was the best thing I’ve ever done. She is the best! My mindset has changed since meeting her. She makes me feel as though I have control of my life but she is right beside me. I am not just a number to her. I was on Ventavis when I went to see her, and she increased my high blood pressure medication to 720 mg a day, she started me on Revatio, and, recently, on Tracleer.
Now, five years after my first symptoms, I feel great. I ride a stationary bike four and a half miles, four times a week. A few years back I could not walk through my yard without having to sit down to catch my breath. I do ask questions every time I go to the doctor now, and I am not afraid to ask about anything that can help me. This February will be my two year mark. That was when I was diagnosed, at least. My doctor says I can walk farther than a healthy woman my age, and that makes me feel great! I am still nervous when I go to my doctor, but at least I am informed now, and I am armed with more knowledge.
I believe that there is hope for all of us. We must not forget about everyone in our community and beyond who are dedicating their lives to finding a cure for this terrible disease. I do not know how long I will be able to feel this good, so I thank God for every day I wake up alive. I wrote my first letter to the editor last night and I plan to submit it as soon as I proofread it. To all of us with PH: May God protect us and guide us in our time of need. And a big thank you to all of the people behind the scenes. God bless you all. My mom and dad always said I was one in a million!

Susie