I’m Aware That I’m Rare: Betty Lou Wojciechowski

Betty Lou “Wojo” is a retired school teacher who lost her husband Jerry and two children (Michael and Matthew) to Pulmonary Arterial Hypertension (PAH), a rare blood vessel disorder that affects people of all ages and causes high blood pressure in the arteries of the lungs that can lead to heart failure. Betty Lou discusses how she turned her sorrow into strength and why the Wojo family has established PH research grants in Jerry, Michael and Matthew’s names.

Transcript:

My name is Betty Lou Wojciechowski and I’m from Mission Viejo, California.

It was 1983, and I thought my son had a funny heart sound. His doctor thought he had some little blip in his heart and sent me to a pediatric cardiologist who said, “Oh yeah, we’ll have to do a valve replacement when he’s older.” That’s what I thought was going to happen, which I thought was a horrible thing, and then they went to do a heart catheterization to see how bad that surgery would be, or when it out to be held, and came out and said, “I’m sorry, your child is not going to live. Your child is not going to live to be a teenager,” and at this point he was four. We said, “Are you sure?” He said, “Yes,” and he explained in a really tiny little picture, “Here’s a picture of the heart, and here’s the way the blood goes, and he doesn’t have this, and this won’t work here, and it’s all pushing back.” I didn’t understand it at all. I had never heard of … at that time they called it primary pulmonary hypertension. I had no idea what he was talking about, and said, “What can we do about it?” He said, “Call Stanford, see if you can get a heart and lung transplant.”

At that time I didn’t even know if I had insurance, I had just been hired as a school teacher. I quickly called to see if I had insurance that would cover lung transplants, which of course I didn’t, but then we called Stanford who said, “Oh no, there’s only been 20 done in the world. The youngest patient we’ve ever had is 18, we’re not touching your four year old.” I wouldn’t give up. I wanted a second opinion, and I wanted a third opinion, and I kept going. I wanted somebody to tell me it was going to be okay. We went to the head of pediatric cardiology at UCLA, and she looked at the tape of his catheterization, and said, “I won’t even put him through the procedure again, because it could kill him just to have that.”

She looked at the tape, and she said, “I’m very, very, sorry.” I said, “Well what can you tell me? How long do these people live?” She said, “Personally I couldn’t live like that. I want you to just live as normal a life as you can, and he might have a year.” Now the guy that did the first cath was in September, the beginning of September of 1983. This lady saw him on November 3rd.  He died on December 6th. There was no time to figure out anything else, or to do anything else. We were just completely blown away. It was like someone cut us off at the knees. We had four little kids, and our baby was all of the sudden going to be gone. We tried to make his life as normal as possible. He went to preschool, he carried around oxygen with him in a little tank. His friends would push it around for him. He had a 50 foot cord so he could go on the swings, and the slides, and that kind of stuff.

Then one day he was in my arms at my school during lunch time, and he had a sudden seizure, passed out, his heart stopped beating. Called the paramedics, they couldn’t get him going again, and took him to the hospital. There was several hours before they finally said, “Sorry, there’s nothing we can do.” We had lost our baby, and we really didn’t know why, or what to expect. We said to the doctor, “What can you tell us about the disease?” He said, “You’re never going to meet another person with pulmonary hypertension, don’t worry about it.”

That didn’t turn out to be the case.

I spent the next 10 years not even being able to say my son Matthew’s name without falling apart, and completely crying, and I couldn’t tell the story, I didn’t really understand the disease. We told his siblings that he had a heart lung problem, and even his brother who was a year older than him use to say, “My little brother had a heart lung problem and he died.” That’s really all he knew. There’s a lot of things I probably missed in my kid’s lives over the next 10 years, although they didn’t put me away somewhere. I was actually functional, but not really as involved in life as I should have been.

Eleven years after Matthew died, suddenly my husband, Jerry, starts feeling short of breath.

Now the year before he had had what we were told was a heart attack, although it was just a blood clot in one coronary artery, but not having any idea about pulmonary hypertension, it never occurred to me it could have something to do with that. They did an angioplasty, and a few months later he had the same symptoms of being out of breath, and just so tired. They put him in the hospital, they did some tests, and his doctor who knew how we had lost Matthew said, “I know what you have, but I can’t tell you. I want you to go to San Diego and have them look at you.”

We thought he was being put in for a battery of tests so that we would come out with some interesting answer of how to fix him. We went to UC San Diego, and the doctor there, who is a famous PH doctor now, with tears in his eyes, looked at his X-ray and said, “I’m really sorry but you have primary pulmonary hypertension.” I didn’t believe it the first time. I really didn’t believe it the second time. All those years there was no medication, there was no treatment, there was no support group, there was nothing for me to hang on to. It was pre-internet. We tried really hard to email, or contact somebody over this new internet thing in the early 90s, because we thought with Matthew that transplant was the only way to fix this problem, we thought that was the cure. We tried to go that direction when the doctor told Jerry he had it. “Can you put him on the list?” He said, “Oh sure I can, but you’re not going to live that long. Go home and get your affairs in order, get on full time disability, I’ll give you oxygen, get a wheelchair, you’re done. I’m really sorry.” He cried as he sent us off.

I kept looking at a transplant, and kept talking to people about that, and through a friend of a relative found out about one other person who had the same disease. He was older than Jerry. Jerry was only 46 when it was diagnosed, this man was in his 70s, and now he was feeling a lot better. I said it couldn’t be the same disease, I just don’t think that’s possible. It turned out it was. He had seen … Gave me a number to call Doctor Brundage… Doctor Bruce Brundage at Harbor UCLA in southern California. We signed up for every clinical trial we could think of, thinking, “I’m going to be dead before the school year is over.” Within a few months he said, “I don’t know if it’s in my head, but I think I feel better. I’m going to try to just go to school for a few days and teach.” It turned out that he was so much better that he returned to teaching school the next year. He taught school for 10 more years after that.

The glitch in the whole thing was that right after he was diagnosed, soon after he was diagnosed, they said, “Bad news, this disease only strikes two people per million, that was the odds for your son when he got it. However, now that we know your son and your husband have it, you’re an even smaller group of about 6 percent of the people for whom it’s in their families.”

This was 1994, 1995, that was before the human genome had been mapped. Nobody really had any idea except that if it’s in families it’s different than if it’s sporadic. The screening tool that they used then was echocardiogram. They gave an echocardiogram to my other three children. My daughter Michelle was fine, my younger son Mark was fine, and my middle son Michael had slightly elevated pressures. Like if they’re suppose to be 25 his were 32. He had no symptoms. I thought, “Well it’s a mistake. It must be wrong.” They said, “Well let’s just verify it. We’ll take him in and do a heart cath just to make sure.” When they did the heart cath, although he still had no symptoms, his pressures were in the 60s. They said, “Oh yeah, he definitely has it.”

We went to Doctor Brundage, and we said, “What do we do about someone who has the disease but no symptoms.” He said, “I don’t know. There is no one who has, who is in that situation.”

He didn’t seem to have any symptoms at all, he worked two jobs, he went to school, he got married, they had a baby. His life seemed to be progressing normally for about two and a half years. Then he suddenly got pneumonia. When he got pneumonia, what I know now is that the pneumonia inspired his pulmonary pressures to spiral out of control, they couldn’t control it, he bled out in his lungs.

One day he had a fever, two days later he was in ICU on a respirator, and 12 days later he died at the age of 24. It was really, really, a hard blow, while still mourning our children Jerry and I went on. He was a teacher, I was a teacher, our other two kids were learning to cope, and being afraid that their turn might be next because we had no idea what the future held for them.

We gave our blood, and our blood went to Vanderbilt, and our blood went to Utah, and our blood went to Columbia Presbyterian. I think our blood maybe went to other countries, I don’t really know. We were willing to give them any part of us we could, to make some kind of progress in this disease. As a result, in the year 2000 when the human genome was mapped, lots of places of higher education already had our blood samples. After they had mapped the human genome, I said, “Do you have any idea if my kids have the gene mutation?” Now they knew there was a gene mutation. Because they were both over 18, they wanted to tell them personally.

They told them one at a time, first Michelle, “Yes, you have the gene mutation even though you don’t have the disease.” “What does that mean?” “That means you have a 15 to 20 percent chance of getting it at some point in your life.” Then they told my son the same exact thing, so my remaining children the gene mutation, and they both go to have echos, and exercise tests every few years. They recommend ever three to five years. So far everything is fine. It’s like living your entire life waiting for the other shoe to drop. You’re always thinking, “Could be this year. It could be next year. Should I do this? Should I go here? Should I try this? Should I go to elevation?”

My daughter, for example, had to decide, “Should I get pregnant? I don’t have pulmonary hypertension,” which when you do you shouldn’t get pregnant, but she has that gene mutation. If she got pregnant would that push her into having it? Her husband didn’t want to do that to her. As a result they wound up adopting rather than having their own biological children.

My son, on the other hand, decided whatever God handed him, that’s the way it was going to go, and he was just going to let nature take its course. He and his wife had a child. After about six months they finally decided, “OK we better have the gene test and see what the story is, he could have inherited it from me.” We sent off the blood, and sure enough, right before Christmas, right before his first birthday, we found he also carries the gene mutation.

He’s fine, he runs around, he gets stitches, he falls off his bicycle, just like every other kid does. Doesn’t seem to have anything that slows him down, but as his grandma watching him, and being terrified that any moment one of my children, or my grandchildren could come up with this disease again, and have to go through all this again, is pretty hard. I watch him run through the sprinklers in the summer, and if his lips even look a little bit like they’re turning blue I want to run to the hospital. Of course there’s nothing wrong with him, he’s just fine. Caleb is perfectly normal, but I said to Mark the other day, “When will you take Caleb back to get another echo?” He said, “Oh, I don’t know. Several years from now.” He doesn’t really want to think about it. I don’t blame him. He goes himself every three to five years, so he thinks that’s about the schedule he should have with his son.

When we looked into Jerry’s family history we found that he had 10 people in his mother’s side of the family. There were six women, all of whom now have lived to their 80s, 90s, and 100s. There were four men, all of whom died before they were 60 years old of possible tuberculosis, possible lung infection, bronchial pneumonia, things that all could have been misinterpreted and been pulmonary hypertension. We assume it came from that side.

I’ve been after the researchers that I know for a number of years, saying, “How about testing my blood?” Because they said, “You know, every child you have there will be a 50 percent chance of passing on the gene.” My children were already born when we found out about the gene, we found out about the disease. I said, “How fair is that, that all four of my children, four out of four of them, got the gene mutation? That couldn’t just be happenstance. Only 50 percent are suppose to get it. I bet I pass on something that combines with that.” The state of research is such that they will look at the most likely place it came from, and test that, and they’re not ready to test me yet. It would prove nothing, first of all, it wouldn’t change anything for my kids. They’ve got the mutation already. I’m not worried about getting it at this point in my life.

Jerry’s doctor eventually told him, in 2003, “You know, there’s only so many breaths you’re going to be able to take. If you want to be able to spend more time with your family, I suggest you stop teaching now.” Now he didn’t feel like, physically, he needed to stop working, but the doctor said, “Stop now, enjoy,” and he did. He officially retired in 2004. In 2006 he had pneumonia that almost took his life. He was in a hospital in San Diego for over a month. His mom and his sister flew out to say goodbye. Miraculously, he came back around, and they sent him home. Because of that, my kids and I wanted to celebrate, and we bought him a replica of his car that he had when he was 17 years old. We bought him a 1963 Impala, he loved driving it, and he loved just looking at it.

When he was in the hospital during that 2006 time, something happened that had nothing to do with pulmonary hypertension, he twisted his knee, and he couldn’t seem to get back the strength in his knee muscles. He wound up in a wheelchair, but not because of his breathing, because of his knee. Then he had some really bad stomach issues, and nobody could tell us why. That kept happening until he was in so much pain, he had these attacks in his stomach, like nine times in one day, where he would throw up bile, and just be screaming in pain, to the point where I finally called 911.

At that point he wound up in our community hospital, and they kind of didn’t know what to do with him. I had to educate them on pulmonary hypertension, and we found a doctor who was interested enough to … so interested to listen that he went to the Los Angeles doctor’s offices, and to the San Diego ones, to learn all about pulmonary hypertension to try to help Jerry in his last few weeks of life. His heart was not strong enough to keep beating. His heart was completely worn out.

We lost him in 2007, 10 years after Michael died.

I attribute my strength to my husband. Jerry taught us all how to just keep smiling no matter what happened to you. He had a rough life growing up on a farm. He just kind of instilled in us, you live every minute to your fullest. We both believe in a God that has a big plan, and we don’t get it at all, but apparently the things that have happened to our family are part of his plan. That doesn’t make me go, “Yay, that’s swell, I’m so happy.” It makes me be able to go, “Well, I’m not going to understand it, but I need to accept it, and to move on.” I feel like if God brings the sun up every morning I have a choice, I can put my head right back under the pillow and not even notice it, or I can get up and make the most of it. I think that’s what’s sustained us, you know? Our love for each other, our love for the world, for life, for the lives that my kids and my husband have already lived. We need to keep moving forward.

The other thing is, it’s very, very, empowering to be able to speak to someone who has just found out they have this horrible diagnosis, and say, “I’ve been there, I understand. Let me hold your hand and walk you through it. You are not alone.” That makes me feel like my little boy didn’t die for nothing. I didn’t lose my husband after only being married for 36 years, when we should have had twice that. I feel like there’s a reason for that, I don’t know what the reason is, but if I can help one other person to feel comfortable, more comfortable, with their new normal that includes pulmonary hypertension, then I feel like I’ve made a difference. I think my kids and my husband, they’d be proud of me for staying involved, and for contributing, and for doing all kinds of things that would call attention giving this disease awareness, and promoting advocacy, and everything else. Just helping other people to have hope, even if they don’t share my religion, they can have hope. They can have a good attitude.

I feel strengthened by that. It helps me … I mean, I could just sit around and cry all the time, I’m sure I could accomplish that with no trouble, but it doesn’t help any body else. It certainly doesn’t help me. When I reach out and help you, then I feel like, “OK, there was a reason for me to have gone through all this pain, so that I could help you get through yours.”

I sometimes speak to people who are in the business of the pharmaceutical industry, and I say, “Would you please turn my pain into your passion? I don’t want you to just go out and sell a drug to a doctor. I want you to remember my story when you’re thinking about telling the doctor why this patient should be on this particular drug. Not because you’re getting paid for it, but because this will change their entire life.” You could look this up in some annals of medicine, and find something from 30 years ago that said, “You’ll die in the next two years.” That’s not the case any more. Now we have drugs to treat the disease. At that time, when we started out, no treatments, no support groups, no pharmaceutical companies, so we’ve come a long, long way, because people continue to stay involved.

We established a research grant in Jerry’s name for adult research, and in Michael and Matthew’s name for pediatric research. It’s so gratifying to us to know that even though they’re not here, I don’t know if they can see what’s going on or not, but because of their demise, because of the things they went through, and the things that we went through, other people are now being given the opportunity to research more ways to treat newly diagnosed people, to treat people who are still alive who made it longer than my guys did. That’s very empowering, and very … It makes me very hopeful.

I don’t want to have another family lose half their family to a disease where there’s no treatment, no cure, no hope. That’s not my vision, my vision is that these people went before us and prepared a way for the people who are coming ahead of us. I know there’s thousands of people out there that will look at my story and either get some inspiration, or get a little bit of hope, a glimmer of, “I might be able to make it.” That will make me very happy.

I feel like God pours into you strength, the things that he has in your life. I think he prepared me one step at a time so that when I lost my little one, and it took me by such surprise, that I was somewhat prepared to lose my older son, and that those loses, and seeing Jerry going through those loses, and sharing that with him, helped me carry on after I lost him. I would say we’re all stronger than we really think we are, and you don’t know that until you’re tested.

I’m Betty Lou Wojciechowski and I’m aware that I’m rare!

Listen to “I’m Aware That I’m Rare: the phaware™ podcast” at www.phaware.global/podcast/. Learn more about pulmonary hypertension at www.phaware.global. #phaware