Tammie S's PH Story

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

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