I initially sent this in June to a message board group I belong to when several newly diagnosed people joined the group. Most were similar age to Sean’s and it seemed they needed hope. I share our journey through a mother’s eyes to help with encouragement and give hope to anyone newly diagnosed.
Hello to Everyone, Friends and Family as you are all to me –
Welcome to the group for all of you just hearing and learning of this new diagnosis which has rocked your world. It does have light at the end of the tunnel of fear, doubt and questions that are living quite comfortably in your minds right now. Here is our story of PPH and I hope it helps shed some hope and light to those of you now grappling the news of this diagnosis.
In 2002, Sean, my son who was not yet 19 was having episodes... they sounded like seizures from 1100 miles away but when Sean came home we went to the neurologist and had the EEGS run and no seizures or abnormal brain waves were found. That was in July. He seemed fine, but just a little more tired and willing to sleep than he used to be, but we had just been through the hell of losing my mother, his grandmother, and the light of the family. She was our matriarch and Mom had kept us all well grounded. Okay so there was my thinking, it is grief and these episodes were maybe stress induced, but now he was here with me and it would be fine. And it was, except the sleeping part, and I assumed it was grief.
October of the same year, Sean and I drove back to Colorado where we are from, to visit friends and family. He was asleep for the majority of the trip, from southern California to Colorado Springs. He had complained of a sour stomach and not feeling well, general malaise, and we both thought it was bad food from a fast food joint. When we got to Colorado he almost passed out twice, and again he tells me bad food, he was physically ill from the trip so we slowed down and got to the motel and I figured he would be okay. Well, he wasn't. Two episodes of syncope (fainting) and we were at the hospital emergency room. When the triage nurse took his pulse she had this STRANGE look on her face. Okay no problem I tell myself she is just not used to goth look kids and he was goth- pale, kind of cyanotic around his lips and fingers- but again I am thinking food poisoning....
She takes his pulse then blood pressure then leaves us and comes back with another blood pressure machine, so I am thinking hmmmm what is happening...
The next thing I am realizing is she is whisking my 18 year old child off to the cardiac area of the emergency room, with several bells and whistles going off. The swarm of people around Sean scared me enough, just a year earlier my mother was the person in the emergency room bed and she was dead in six months. So I am freaking out, his heart rate on the machines above say 210- I was thinking 210 pulse????? He was in heart failure and also tachycardia. Okay I am standing back with my daughter and we are watching this drama unfold in front of us. One person says he is dying and I am freaked out and holding my breath. Well the meds which were supposed to work within 5 minutes to reduce the heart rate didn't, it took over 45 minutes before his heart slowed down enough to be thought of as "safe" and still there were swarms of technicians, doctors, nurses and the whole gamut surrounding this young man who was telling me he was fine. I felt like I was the worst parent ever- I should have taken him immediately to an emergency room somewhere in the middle of no where while we were on the road. But I didn't. Then they whisked him off to x-ray when he was finally stabilized only to bring him back to tell me his heart was three times the size it should be and no one knew why.
The cardiologist on call was in the cath lab with people and couldn't come, but he had ordered an echocardiogram so I watched while the tech did it and wondered what the colors were in the pictures. She didn’t say a word though when I asked, and Sean was out of it. I looked at my son with all these tubes and machinery hooked up and he tells me that he is okay. I am not listening any more.
We had to wait and wait and wait some more before we met with the cardiologist. That’s when the bomb was dropped. His words were we don't have any idea of what is wrong with your son, has he taken drugs, done this or that and we are keeping him here to monitor his heart failure and to try to figure out how this has happened. Sean chimes in (he is absolutely under the influence of drugs to keep him still) and tells the doctor he isn't staying unless there is cute nurse in the ICU. Okay he is only 18, so I laugh. Humor had saved me time and time again while dealing with the sudden downfall of my mother's health it had to protect me here. The doctor looks at us both like we are complete lunatics...Dr. Metz though sends Sean up to the ICU and the saga continues. And he had a cute nurse.
The following day Sean has a TEE (transesophageal echocardiogram) and that tells the doctor more than an echocardiogram, and a pulmonologist comes into the room and gives us this diagnosis of possible Pulmonary Hypertension. Okay, then he draws on the little white board in the room and tries to explain what PH is. I am so confused and terrorized, but listen with my mouth as shut as I can keep it. When he leaves the nurse brings in this tome of medical maladies and looks up pulmonary hypertension for us. I read it is a chronic progressive disease of middle aged women usually diagnosed in the early to mid forties... I'm thinking WHAT?!?!?
The cardiologist, Dr. Metz, tells us he hasn't really treated young cardiac patients and he called in a pulmonologist to help, and then another cardiologist, a pediatric cardiologist, and the right heart catheterization is scheduled. I finally leave the hospital to shower, call my husband and stop by a friend's house that has internet to look this disease up. That’s when it got bad ~~ really bad because of what was written about PPH on the Internet. I thought my son was going to die. I was in the hospital with my son day in and day out, even my pseudo daughter gave birth while Sean was in ICU so I was running the stairs between the ICU and Labor and Delivery while we were there (the nurses probably still talk about the crazy lady with two kids in the hospital at the same time running the stairs...)
The right heart cath was supposed to only take an hour, but after two and half hours I still hadn't seen anyone and I assumed the worst. They can have complications. Dr. Metz came out and explained it was PPH, and there were no specialists in Colorado Springs but there were doctors in Denver. He told me Sean's resting pulmonary pressure was 113 HG/mm which is seriously high. Seriously. And then our insurance sent us to specialists in Denver. So after a week in ICU and CCU in Colorado Springs where the doctors saved my son's life we were now off to Denver. I drove right behind the ambulance which took my son to the NEXT hospital.
We saw PH specialists there and since it was a teaching hospital, Sean had computer access to himself (lucky dog of a kid) because he could sweet talk the nurses and beguile the doctors into letting him have a computer in his room. More research on PH. The doctors there told him one day shy of his 19th birthday that he could live 6 months to 2 years if lucky without medication, or go on Flolan, an intravenous medication to try to help slow down his inevitable death. I am so glad Sean chose the medication. The doctors also offered Sean the option of Bostentan, which is Tracleer, but his PH was class IV and advanced so they recommended the Flolan to try to save his life. And it has.
A central line was placed into Sean's heart, and the medication was started. A wonderful nurse spent a week teaching us life with Flolan. We learned how to sterilize our hands and general area to mix medication. We were taught how to clean the site where the line went into Sean's chest and I was taught how to change the dressing. What a birthday gift. What a nightmare. My son was still weak and cyanotic; he couldn't get from the bed to the bathroom in the hospital without resting first. This all happened in a short 3 weeks (15 DAYS!) in Colorado. A patient coordinator from the hospital got us home with all the care help we could need. A plane flight was scheduled. My husband flew out to bring the car back. Sean couldn't be in a car, never for 1100 miles. Blood clots might form and he would die. So we flew home with Sean on oxygen 4 liters 24/7 and once we got here the test of the strength of a person began.
The first thing was a trip to our medical facility a when the pulmonologist said you are going to be recommended for the lung heart transplant list because your PH is so advanced (Class IV) there is no chance of improvement without transplant. This is all going on while he is fighting the side effects of being on Flolan, the jaw pain, the diarrhea, the vomiting, as well as the other medications which were now a part of his daily schedule. I tell you I slept like nothing that first month listening for the pumps to beep, something to sound wrong, it was scary. I was afraid to open his bedroom door for fear he wouldn't be alive.
We went to our fire station and taught the paramedics about what a 911 call would be from our house if it was Sean. Sean and I taught them what we had been taught about Flolan and PH. Sweet and simple, and they've needed it once since Sean was diagnosed. It helps you if you need them. They understand and will remember if you need to call them. We even gave them teaching samples of the Flolan and equipment for the central line so they knew what it was all about. I was scared. Then we go forward.
So we were referred to the Kaiser office at Sunset (in LA, January 2003) to start with the lung transplant stuff. Blood draws, tests, more blood, even more blood, and that is just the beginning. Walk tests, lung tests, the whole experience is mind boggling. You have to talk to psychiatrists, and psychologists, and an extensive medical history and the works, financial stuff too. Once you get all the papers in order they then submit it to the hospital which would do the transplant which in our case was UCSD (University of California, San Diego). Then you are still being monitored closely for your PPH, as well as getting watchdog eyes from the transplant coordinators because you can't jeopardize yourself before the 3 day trip to meet with the actual transplant teams who do the surgery and post op care. Okay we made it through all the beginning paperwork and tests. Sean couldn't even walk 1/2 block in LA to get from the doctors office to the car without a 15 minute rest.
He makes it into the beginning phase of transplant, a three day visit to the hospital in San Diego for another barrage of testing. Now this is July of 2003....
When you meet with these doctors the whole family and support system go. They have special specialists who see you, the patient, and more specialists who see the family. The doctor who saw Sean, Dr. Yung, was a physician who helped to develop Bostentan so I was a rapt audience for whatever he said. He was very informative. I took notes, we had questions. I took them too. Sean impressed the doctor; Sean was dressed in a suit which surprised the doctor. Sean found out after a month wait that he was accepted as a candidate for transplant. But then we looked at the survival rates for people receiving chest block transplants. Not so hot in the five years post-op survival rate.
Meanwhile after living through this day to day drama of having PH, Sean is living. He spent the first year in the house, in a chair, on the computer playing video games and not daring to go out. Talk about depression. He had to increase his Flolan to 23ng/kg/min, but that wasn't bad compared to other people with PH, they could be up to double that amount. He had tried Tracleer and it messed up his liver enzymes, so it was discontinued. He finally took off the oxygen mask about 2 months after we got home, to using it only to sleep. He doesn't even use it to sleep now, and I have nudged him to breathe once in awhile when he is asleep because he is a bit cyanotic. When we went to Colorado a year ago we packed the helio in the car just in case. He used it once. He is on Tracleer again; he has good liver enzyme tests and is awaiting the approval of Sixtasentan, which is a drug like Tracleer which doesn't have the liver damaging side effects. He takes digoxin, lasix, coumadin, potassium and now is on Revatio (sildenafil) in hopes of getting off the pump he has worn for almost 5 years. Wow it has been awhile. What a terrific improvement.
Sean is full time in school now exploring a degree in medicine (he could be a doctor by osmosis with what he has been through), he doesn't use oxygen unless it is really smoky from fires (not since 2005). Sean took the "death sentence" of PH and turned it into his living expression of he has an illness but it doesn't have him. He is pursuing a friendship with a sweet young lady, and life is good. Oh this isn't to say it has been easy, semesters at school have had to be dropped because of week long visits to the hospital. Two hospital trips a year is the norm. That doesn’t include emergency room visits. People freak out when they learn he has an illness they don't understand. I go to the emergency room with him to protect him from medical people who try to stop his pump or have no clue about Pulmonary Hypertension, and we go on.
I would classify Sean in between class I and II in the range of classes provided by WHO in function now. He lost about 100 pounds after his diagnosis, he is getting better in what he can get better at. He doesn't give up. He has not had a right heart cath for awhile now, almost two years, he doesn't want to know if the pressure has been significantly reduced because his life has significantly improved since the first 3 week trip to the hospital finding out he has some weird woman disease... Yes I know it isn't limited to one sex of people, or one type of person, but he still can think like a kid at times....
This past September he was REMOVED from the transplant list because he is doing so well with the medical treatment of this illness. No, he is not cured, no, he is not fixed, but he has improved greatly and significantly enough that they are working with him to try to remove him from Flolan. I have trepidations as some doctor’s think that once you are off a medication which has worked for you then if you need it again it won't work at all, and Flolan is the gold standard for Pulmonary Hypertension.... But it is Sean's life, and he has beaten the odds of life with PH so I stand beside him as he goes through his trials and we just keep on keeping on.
Sean tried the local support groups but as a young man it was weird and uncomfortable to sit with women old as his mom and talk about his illness. He said it wasn't for him. I can respect that as I couldn't go into a room of 20-somethings and talk too freely about life on a day to day basis without discomfort. The first friends he made online have moved on to Higher Ground.
Life with PH is not death; it is just a major change to what you know of as life. You can get better in functionality, you just have to get the right medications and do what you need to so you can improve your life. When one PH doctor wouldn't listen to Sean, he fired her. I laughed but that was many days after him doing that, and now his doctor works WITH him instead of treating him like he cannot make informed decisions about his own life. PH takes a lot of time and energy until you adapt to life with it, so I imagined it as an entity at first when we were dealing with it, but now it is just a part of our house. Everyone is seemingly on medication for one thing or another so asking if you have taken your meds gets a chimed response from everyone in the house when it was initially directed at one person.
Going for a walk or wrestling with his dog is normal now for Sean and it wouldn't have been a few years ago, but in dealing with the illness you learn what you can and cannot do. Normal daily living things are normal again, and I consider our family blessed. Sean cared for his Tata when he was dying and he could pick him up and move him to the bed or chair when he fell. I think the PH softened Sean so he could do these things, or maybe life just gave him the ability to do things most kids his age couldn't fathom. Anyhow we are very blessed, it has been life changing but not all for the bad, it has really improved life as a family and it allows me to see through different eyes than I had before.
If you have recently been diagnosed and are scared and frustrated and confused -- it gets easier, listen to the people here and they'll help as they can. Keep records and copies of EVERYTHING in a notebook for yourself, and write your questions down for your doctors. Your doctors should listen and answer them and if they don't -- sit them down and ask anyhow, and don't budge until you get an answer. Living with PH is difficult and finding a great doctor who will help is even harder. PH is still a rare lung disease so many pulmonologists don't understand the treatment or what is best for PH patients. Finding a specialist is PARAMOUNT, because they will listen to your needs and fears and have options for your health. Support groups if possible are a great help because you will be received with open loving arms. Find a group that you are comfortable with, wherever you find hope and support~
Update- July 2007
Sean now has a MAJOR life! He is up to treatment levels on his Revatio and he is only on 8ng/kg/min of Flolan. Still decreasing too on that medication YAY! NO PUMP SOON! He is working full time, spent a weekend at the River, with his sweet lady friend and her family.
He is rarely home and getting ready for fall semester to start. He plans on a full semester of school, and he is looking forward in life. I rarely see him except on laundry days, I hear him come home at night. He is happy. He is moving like an almost 24 year old man. He has goals and dreams and he is reaching for his stars. He is moving along and taking care of himself. He does it because he can. Maybe you cannot do this right now, but you can always reach for the stars, and get to where you are your best. So don’t give up hope, don’t think life has ended it hasn’t. It has just changed. Everything takes time, and sometimes you want to bite your lip, but you can do it because I have seen it done. If I can get through this journey you can too- so don’t lose faith.
The people here will talk and help in any way they can, so just ask when you have a question. We’re all here to love and help you, we are walking this life of PH, and we laugh, sing and share joy together as well as emotionally support and answer what we can from our experiences.