Heather has two girls, a new liver, and joy

Heather was diagnosed with Primary Sclerosing Cholangitis young, then made it through nursing training, graduate school, dating, getting married, and having her first baby before her PSC symptoms started to really affect her quality of life. She speaks about how she and her husband approached having another child, planning for, then recovering from, a living donor transplant, and how roles and work have shifted in their family.

Resources mentioned in this episode on the PSC Mami resource page.

Transcript

PSC Mami: Welcome to PSC Mami where we share stories at the intersection of PSC and parenthood. PSC, or primary sclerosing cholangitis, is an autoimmune disease where the immune system gets the wrong message to attack its own bile ducts, over time this leaves them scarred and blocked and in turn shuts down the liver, which, surprise! is in charge of over 300 functions in the human body. To be diagnosed with this rare, unpredictable and incurable disease can rewrite anyone’s life journey including their vision and experience of parenthood.

All stories and information shared on PSC Mami are personal experiences and opinions and should not be used as the basis to treat yourself or others.

Today we are talking with Heather. Heather and I met at a PSC conference, my very first one, where I was super scared, and it was the one where I had the question about can PSC patients become moms. And, I can very confidently say that Heather is one of the reasons I have a son today. Because she was one of the people who look me in the eye and told me that it was not only possible but awesome. And I'm really excited to share her story, or have her share her story with everyone today. Heather lives in Dallas with her two children and her husband.

Hi Heather!

Heather: Hi!

PSC Mami: Welcome, thank you so much. Tell us a little bit about where you’ve come from. What's been your journey? What has PSC in your life meant for you?

Heather: It’s definitely been different at different stages since diagnosis. I was diagnosed halfway through college. I was it was between my sophomore and junior year, and I had actually just started the nursing school portion of my four-year program, and so I was kind of immersed in learning about illness and how to take care of people, and then I found myself battling an illness. So that was very interesting. I was having symptoms since I was in my teens, and off-and-on had gone to talk to doctors, but nothing definitive had come from it. Until I went to get some lab work done in the middle of college, when I was home on a summer break feeling tired and gaining weight, which I felt I could also a tribute to college in general, but was actually diagnosed with PSC. At that time, they also discovered the ulcerative colitis. At that point in my life I felt that my life was just starting out. I was just starting college, starting my nursing program, excited about the possibility of my career, and here I have kind of this very unexpected news.  They like to tell everybody 10 to 15 years before you need a transplant. Because, statistically that's what they knew back at that time, and they also like to say that PSC it was mostly diagnosed in middle-aged men. Which, I discovered in my course of illness that everyone looks at you like you have three eyes because you are young woman. I kind of felt like my doctors in general really didn't know what to do with me, much less what to do with any of the issues surrounding young women; hormonal issues or menstruation or fertility or childbearing or anything like that. PSC was sort of a foreign unusual diagnosis and then being a young woman with it was even more perplexing. So my doctor at that point, who is amazing and great, but also a middle-aged man, also did not have any advice to offer at that point in the disease but thankfully I was early on and mild at the beginning. Ultimately, it would be another 16 years before I would even get to the point where we were thinking about getting a transplant.

So yeah, I went about college. I finished nursing school. I, you know, gave PSC and the diagnosis some thought some brain space, but really at that point I wasn't having major symptoms. I wasn't having problems. After I kind of had my crisis of how is my life going to look, can I do the things that I'm going to want to do? Can I travel? Can I have a family? You know those were all questions that came up for me early on you. At that point of my life I wasn't ready to have a family, to get married, you know, any of that stuff, So, I kind of realized at that point in my life, I had to do the next step, which was keep studying, finish school, college, start my career and I really was able to do all those things.

My ulcerative colitis symptoms were very mild. I hardly had any issues and other than having surveillance colonoscopies and occasional MRIs and lab work for monitoring my disease. And my symptoms were really very quiet for many years. So I was completely thankful. It even got to a point where I really didn't have to think about it everyday. And I know that sounds weird because I was taking medications everyday for it. PSC was really not a major player in my life. I gave it a little bit of thought every now and again, and it did ultimately play into some of the decisions that I ended up making. Because I know there were times when I was working, I was single, and I also felt like I always need to have a job that provides health insurance. Because when this goes bad, I need a way to pay for it. I would love to take a year and just travel Europe, but I need to work I need to earn money. So I do you think that it did have to play into some of my decisions, but thankfully my career was such that nursing is flexible- flexible schedule and it was really something that I could continue doing.

PSC Mami:You were diagnosed very young and were single and working. How did having PSC play into how you approach things like dating?

Heather:  It was a couple of years after diagnosis before I dated or really seriously dated anybody. I looked healthy. I was active. I was living an active lifestyle, and I wasn't very limited by PSC or anything at that point. I had guys that I dated and after getting to know them for a little while, I just usually casually mentioned it,  “You know by the way, I have this liver disease called Primary Sclerosing Cholangitis,” and you know, I might have played it off a little like. “Blah, blah, blah, it's not bothering me right now. but eventually I might need a transplant. Ha ha!” So, but really, when I met my husband, actually I was in grad school and studying nursing as well. We met online, and so it was kind of funny how online dating lends itself to asking a lot of questions before you even meet in person. So after that we connected and really hit it off. I actually knew that he was hooked. He really liked me, I could tell. We hadn't met in person yet, so I felt that the stakes were not that high. And, I felt it was kind of easy to drop the news, “Oh you know, if you feel like you like me, before you go all in on this let me tell you this thing that I have.” And he was like, “Oh great, thanks for telling me.” And I told him not to Google it that that was a really bad idea, but I'm sure he did. And he came back with some questions.

PSC Mami: Once someone tells you not to do it...

Heather: Right, exactly I mean I'm sure he Googled it anyways. Who doesn’t Google it? You kind of have to, but the information that you get there is not the most accurate and probably the scariest thing you can find. You know he came back and said “We all have things and that's not a big deal to me. We can get through it. And, he really has just been a solid rock, and I mean I'm so thankful that I kind of presented it early on. That I didn't really have a lot to lose if he had said no at that point. I would have had a lot more to lose if he had left halfway through the time when it had gotten hard. We stuck through it, and I'm so thankful for that. You know ultimately, anyone who is worth ending up with is going to be okay with it and is going to be strong enough to handle it and that's what you need for this to have a successful relationship

PSC Mami: And in your relationship how did talking about things like becoming parents, how did PSC plan to that, or did it?

Heather: It did but not maybe in the way we expected. Actually, shortly after we got married I was having some hormonal issues. I went to my doctor who did some testing and was like, “oh we think you're going through early menopause.” And here I am like a few months into marriage thinking what? And so this was in my late twenties. And they also did some testing that said, oh there's probably only a 10% chance that you can get pregnant. And I was not having any problems with my PSC at this point. That threw me for a loop and felt totally out of left field. And retrospectively, I think the hormone kind of issues were related to PSC, but at that point really there was no way to know that or to prove that. So we very first thing in our marriage, before we were even really ready to have kids or to want to have kids, we found out a we probably can't. So we just kind of put that away in the back of our minds, thinking well this might not even be a possibility for us, and if that's okay and if the time comes then maybe we'll talk about adopting. If we feel like that's something we really want to keep pursuing. Fast forward 3 years or so, that we had kind of decided not to prevent anything and not to try for anything, and we got pregnant. It was kind of a surprise a very pleasant surprise that we weren't necessarily prepared for or expecting but it was definitely a surprise that we wanted since we had wanted to always have kids.

PSC Mami: At that point when you are pregnant was your PSC pretty quiet as far as you knew?

Heather: It was. The major thing for me and actually in both of my pregnancies was my itching got very very bad and I really hadn't had any itching before the pregnancy or before my first pregnancy, I had maybe add a few episodes of cholangitis over the 15 or so years but really during pregnancy or the second half of my pregnancy my itching was just through the roof so that was probably the worst part of it for me but, you know, definitely doable with some medications and things like that.

PSC Mami: How is your relationship with your medical team while you were pregnant, how would you describe how they walk with you in that journey, had they had patients who had had PSC and have been pregnant before?

Heather: So the doctor I was seeing at the time was a woman and a mom herself and she had patients, kind of one of her specialties was pregnancy and PSC, and so she was excited she was actually compiling like a little book of baby pictures of patients with PSC who have had babies. And so she was very encouraged and supportive. And you know, she may be followed me a little more closely during pregnancy. We checked labs a little bit more frequently, maybe every 3 months instead of every 6 months that we've been doing at that point. I also chose a high-risk OB-GYN. Somebody that was familiar, I don't think she actually had a PSc patient before specifically, but she was familiar with liver disease in pregnancy. So I chose doctors that worked at the same hospital so they could easily share information and if I was hospitalized I had all my specialist Under One Roof. That was huge for me. I did that for during my first pregnancy and during my second pregnancy. I switched medical centers and teams, but also again, I chose another high-risk OB-GYN that was at the same location as my at that point it was my transplant team and transplant doctors. So when I was going through that delivery and afterwards and recovery again all of my specialists were under one roof and could all come to contribute and talk to each other, which I think made my care more seamless.

PSC Mami: At what point did PSC start making itself more present or known in your life? And what point of motherhood were you at when that happened?

Heather: Actually four months after my oldest was born, we had, besides the itching in pregnancy, we had a very uneventful pregnancy. And she was born healthy and was doing great. And four months after she was born, I noticed I was jaundiced, and I had never noticed visible jaundice before in myself. So I made an appointment for a follow-up and my labs were kind of all over, all elevated much more than they usually were. And so we did an ercp and went in and I had a blockage. They were unable to pass through that structure and at that point things started going worse. I got cholangitis and infections and just some complications after that procedure and because of that stricture. And so for the next 4 months or so I was in and out of the hospital and on long-term antibiotics. I had to have some biliary drains place just to get through that bile structure just to open things back up. So my daughter was, that was the time between when she was 6 months old and 10 months old.

PSC Mami: What was that like?

Heather: That was very hard. Thankfully my husband was not working at that point because I was working. I had gone back to work and he was home with her, so thankfully, with me being in the hospital he was able to stay home and care for her. But that was a hard hard time being separated from her at that point I had even just, I had been breastfeeding and had to stop breastfeeding because I was so sick and in the intensive care unit at one point. I got discharged, got hom, tried to keep breastfeeding but my body was just so sick and dealing with infection that we just had to stop at that point.

I think I started feeling better and resolving from that acute episode, as they called it when she was about 10 months old. And things kind of stabilized and my liver got back on track. But I called that the beginning of the end of the liver. Because that really was when PSC made itself known. I feel like life has been pretty easy up into that point as far as symptom management, not much that I had had to deal with until then. We were definitely kind of in crisis mode at that point and I wasn't able to work any longer. And so my husband went to work and kind of put his school plans on hold. And then I ended up being able to stay home and take care of her once I got healthy enough. So our family roles kind of all flip-flopped at that point, but I kind of recovered and stayed home with her until just recently when I started back to work. Yeah things kind of stabilized and we felt normal again and got to do our travels and our vacations and our adventures, and I got to kind of live life again.

PSC Mami: How old is she now?

Heather: She is almost 5. And I have another one who is almost two.

PSC Mami: And how was it you said that first attack when she was small, the beginning of the end. Take us a little bit through what happened between then and now with your second. How did you get to subbing a second child what was it like how did your PSC progress?

Heather: After that initial episode where I got really sick for a little bit, I got over that and got back to normal. Normal, except that I still had these biliary drains that in, and when they tried to take them out, everything was closed up again. So I had to have these external biliary drains, and I ended up having those for the entire four and a half between that time until I had my transplant, so that was definitely limiting in a lot of ways. In the fact that, for a while they were very painful, and I think anyone that has had them will will say that they're painful for a little while. But thankfully, the longer I had them the less painful they got and the less bothersome and I had to have those changed out every 6 to 12 weeks.

That was probably one of my biggest challenges of that time. Because I couldn't swim or submerge myself in water, essentially. I couldn't really run or jog because they would move around and be irritating so I kind of felt like it limited me a lot I didn't ever get to go swimming with my daughters. Didn't really get to go jogging. But we still traveled. We still do a lot of things. I still felt like I still had a fairly good quality of life, but at that point it had declined because I was no longer able to work. I had these tubes. I couldn't do the normal things that we really wanted to do. I couldn't go in the water at the beach past my waist. However, my liver had kind of stabilized, and that really was just life for a little while. That was our reality.

In the process of talking to my doctors and not necessarily being sick enough otherwise for a transplant, there was kind of a lot of back and forth about how long can we do this. We're finally feeling good and our life is good. I felt I could do almost everything except those things that I mentioned. I felt that I've already had a few years good years on my life because of these tubes keeping everything in check and keeping everything draining, and so we really just thought we're really just going to have these until we need a transplant. And my doctor, said she had a mom who had kids who had had those tubes for 10 years, and she hadn't needed a transplant. And I thought 10 years! I don't think I can do that for 10 years. So ultimately, we had to switch to the transplant center that did living donor transplants because we really just felt we needed to pursue the next thing, because life with drains and tubes and kids wasn't the best anymore. I thought my kid’s whole childhood is going to be me not able to do anything with them and just getting sicker and sicker. That was kind of the process of how we are feeling like it was getting closer to transplant time even though my health had kind of free stabilized.

In the meantime after having had our first daughter. I mean she really was the light of our lives and made life so full of joy that we couldn't imagine ever not having had her. We talked again to my doctors, my new team at this point, and said we want to have more kids what does that look like. And it was a big challenge, because they said you know that you have these drain tubes, your liver is not doing quite as well as even the first time, and so might you know your liver might not recover if something happens. We thought, well you know, we didn't really completely plan it the first time so we don't even know if we can get pregnant again and all of those good things. However, both of my teams said well if this is something you want to do, we'll make it we can make it happen if you want to have another. At the same point that we thought oh this might get to the point where we need to pursue transplant, we found out we were pregnant again.

And so that sounds funny and that sounds really irresponsible but we thought, we just felt like we either we wanted a baby or we wanted to have a transplant because I want to move on with life and we knew we wanted to have more kids. It really just was a question of can we have more kids sooner or would we have to wait and have a transplant and have more kids later. I will say that my faith played a big part in that just cuz we felt we were kind of leaving it more up to God and really, our faith really helped us at that time as we really didn't know what the next thing was. And when we find out what the next thing was going to be, we were just thrilled, so I had my second daughter in 2016. And kind of already knew at the point when we got pregnant that it was probably going to be tougher because of the state that my health was in was a little bit worse and I had the tubes. And just in a practical level they changed those tubes in radiology, they don't like to do it if you're pregnant. However, I had talked to my doctors before I even got pregnant, and they said we try not to do it during the first trimester and then whenever we do it we just shield you with the lead, and try to do as minimal radiation as possible so it's very low-risk. So that was one of the things that was really great, because the radiologist really worked with me in minimizing any risk during pregnancy and didn't do any unnecessary radiation or anything when I was pregnant. They definitely were okay with the whole scenario, and I got them on board too before we even conceived. You know just to make sure that that was something that would work.

PSC Mami: Those are for folks that you want on your side right.

Heather: Yes, absolutely!

PSC Mami: And your second pregnancy you said the itching was there anything else that came up for you?

Heather: There was the Itching and I had one episode of cholangitis, so I was admitted with a fever to get IV antibiotics and right before I had my daughter they were just concerned about some of the blood flow to the placenta. I don't know that that was specifically PSC related, but they don't know that it wasn't. So I would say was hospitalized for 3 weeks before I had her just so they could keep monitoring. At that point my high risk OB said yeah, you're the patient that I lose sleep over at night.

PSC Mami: How did it feel hearing that from her?

Heather: Well I thought well everyone is saying really nice things but when she said “I'm losing sleep at night.” I thought, well maybe, they're a little bit more worried than I thought they were. Or they were more worried than I was, but I was being closely monitored, so I knew I was getting the best possible care. We checked my lab work one night and incidentally my liver enzymes were crazy high. It looks like my blood was not clotting, so they immediately decided to induce, like the next morning. My daughter was born the next day. I was actually getting albumin transfusions and vitamin K during delivery and I'm pretty sure I kind of had everybody freaked out. However, she was born healthy and doing great and she had a tiny little stay in the NICU because she had a little bit of breathing difficulty but I think that was because she was born slightly early.

PSC Mami: What was that experience like for you, you sound very calm now, but what was that like for you  When you were going through it?

Heather: I feel like I am a fairly calm person because I have to be. I was an ER nurse and a NICU nurse and was used to dealing with crisis situations. So I feel like whenever there is one happening, I am kind of calm and separated from it. So I think at the time, I was thinking oh, ok we’ll deliver her and it will be great, no big deal. And it really was, because literally, I had a natural delivery with no medications and a doula and it was perfect on one hand. And on the other hand I'm getting blood transfusions and trying not to bleed out. I think my doctors kind of thought I was a little crazy, but...

PSC Mami:You can definitely tell that you are an ER nurse by the fact you can even make those two statements and be like “it was great!”.

Heather: And you know, I think after she was born she had to spend a little bit of time at the NICU and that was probably the hardest time because you know you just want to take her home and be normal, and not have to stay and go to the NICU, and all that stuff you know. But at that point I had been a NICU nurse so nothing really surprised me. I just think I had a unique experiences and unique background to kind of be like, okay this is temporary and she'll be okay and we'll be okay. We can do this. As hard as it was, everything was worth it and it still is.

PSC Mami: And you are at that point where you were acutely aware that you are on the road to transplant, so you were coming home with a newborn, knowing that the next step since it was “which one we will will we do first?”. Baby came first, transplant is next. How was that for you and your husband as you were planning and being with your baby?

Heather: After Emery was born, you know I really recovered very quickly. My liver recovered, all my numbers came back down, I was feeling great you know and I thought well we're not going to actively pursue anything until she's at least a little bit older. We wanted her to be one. I think we had this goal of if we could not have to actually think about transplant until she's one, it'll give a chance for her to be a little bit older. We can try to breastfeed and do all of these normal mom things. It was interesting, because I ended up having a living donor transplant, which is different in that we got to kinda choose the timing of it, somewhat. You know I might have even gone a couple more years without actually needing one. If I had just been waiting on the list, specifically, without the option of living donor, I probably wouldn't even be transplanted yet. So thinking about the timing about when to do it, we thought, well she'll be young enough not to remember and it would be easier if she were smaller anyway. My older daughter, she wouldn't remember as much and it might be easier just to get it done. So it was interesting kind of trying to think about timing of it with the ages of our kids, but those are kind of all things that we had to think about. And I'm glad that I did get to breastfeed her until she was 14 months old, like that's huge for anybody, even somebody that isn't facing transplant. So that was really really neat and kind of a gift that we got to give to each other.

PSC Mami: Especially because you had your transplant scheduled, how did you prepare for the care of your children for the time that you needed after? Having young kids and needing to plan for your own recovery, what was that process like for you?

Heather: Timing the transplant, my sister was my donor. We thought, oh let's do it in the summer because both of the grandparents, as in my mom and my husband's mom, work for schools so they have the summer off and we needed the Grandma's to be on board to help take care of the grandkids. So that was a big deal for us that we kind of got to pick the timing of it. We planned for my husband's mom to come down for a month and she stayed here with the kids. My mom was off work and she also took turns helping out, and my mom also planned to take off work after about the first 8 weeks when my husband's mom had to go back to work. And so we really had the benefit that a lot of people don't get to have in knowing the timing of it and figuring out when we could get all our family here and on board. And there's a nonprofit that provided us with a short-term nanny for three months that was here for 40 hours a week taking care of the girls. So we were super blessed in that we had so many resources. Also, my transplant center is 15 minutes away from my house. I mean, really, so many advantages that I know aren't options for many people, and I am so thankful that we had ways to make it easier on all of us.

One of the things that we did to kind of prepare was that we talked to a child life specialist and my transplant center has a child life specialist that is dedicated to working with children of ill adults. So we made an appointment with her and she kind of helped my older daughter talk through the process of why is Mommy going to have a surgery? What's wrong with her liver? And helped her talk that out through play and helped us really know how to talk to her about it. You know she had been out to the hospital a year ago when Emery was born. She was only three at that point, so I don't know how much she understood. It was really helpful to get tips on how to talk to my kids about it, or at least to my older one. Emery was only one at that point, so she just needed someone who would consistently take care of her and love on her.

PSC Mami: What were some of the most helpful tips you found?

Heather: Some of the tips were to talk on her level and use words that she can understand. So probably tips from Doc McStuffins would be good too, but you know mommy's livers not working very well and just really simple words, and I'm going to have surgery and Aunt Christine is going to give Mom a part of her liver. And none of that really makes sense when you say it, but to use really simple words that she can understand and also to make sure that she knew that this was not something she could have catch. That it wasn't contagious is not something she could get by being around me and she kind of also helped us talk through things to prepare, like when Mommy comes home I’m going to be feeling sore, and I'm going to be resting a lot and I'm not going to be able to pick up and do things like that. Mommy always wants to give you hugs but you not might not be able to sit on my lap right away. The more words that we could talk through with Emilia and prepare her with it easier was, and she knew what to expect instead of finding out as it was happening. And one of the things too was if she had gone up to the hospital to visit just to have somebody specifically there talking her through it, telling her what the medical equipment was. She likes to play with making the bed go up and down, and dialing the number on the phone to order the meals for room service, and just kind of play things that made it fun and engaged her in the process.

PSC Mami: How was it for you when you came home for your recovery how was it for you interacting with them, what did it feel like for you and them?

Heather: The hardest part was not being able to pick up Emery, because she was 14 months at the time, and I couldn't pick her up and she actually learned how to walk, of course, in that three weeks that I was in the hospital. So I came home and she's toddling around the house and wants me to pick her up. But I could kind of help her up onto the couch and have her sit next to me and snuggle. Which was really nice. We would get a lot of snuggles. They would come up next to me and Garrett or their grandma would say okay let's give Mommy time to rest. And I would just go in the other room. In one respect, recovery might have been a little slower cause I was trying to do a little more initially that I should have. I wanted to walk around the house and I wanted to do things for the kids that they wanted me to do for them, like help them and get dressed and whatever. But then I think on the other hand it was really good motivation for me to stay present and engaged. Watching them do things brought joy. It’s a physically hard process and, you know, it’s mentally hard as your body is going through so many changes. And you feel that you're never going to feel better. But they definitely kept me up and getting outside and getting fresh air and giving me the motivation to go on walks and get out and do things. I think it was amazing to have my kids part of that process, even though it was hard for them for me not to be normal. I feel that 3 or 4 or so months went really quickly and things went back to normal and it was kinda just a little blip.

PSC Mami: How is it now, how far out from transplant are you?

Heather: I'm 9 months out and things are amazing. I am so blessed that all my complications that I had happened all the beginning, and I have not had any major anything since then. So I have to go to my follow-up appointments and get labs and stuff, but life feels great and recently I started working again after four years. So that is a new challenge in  our lives.

PSC Mami: How is that?

Heather: It’s great. Also, a challenge in our family. You know anytime you change family dynamics, there is a little bit of change. Having the girls have a sitter now while I'm working, I think having the time that they had last year with the nanny and their grandparents and stuff has made them a lot more open to having other caregivers. Just the things that they can learn from other people is invaluable. It has also been great me going back to work and using my knowledge and skills again. I feel I am helping people and giving back a little bit of what I've been given and using my passion in my knowledge again to help people. So it's really been a good change. It's something I really enjoyed. I still get to be here a lot of days with my kiddos and watch them grow up. Just in the last 9 months I've gotten to do, I've gotten to swim and go to the water with them, and go to the pool, and to the beach, and go skiing, and go jogging. Just things that I hadn't done that I couldn't just because of those drain tubes. I feel like I can be normal mom again, which is really nice.

PSC Mami: How would you say PSC, I'll say, allowed you to re-envision your life?

Heather: I would say I try to embrace life, and I think I've always been of the mindset that life is a big adventure. And that's kind of how I live. But I also think that having PSC makes me think I want to do the things now that I want to do now for myself or for my family and not you know necessarily wait around for things either for things to happen or to do things that I want to do. You know I think my husband and I kind of have the mindset of our family values is that we want to spend our time together and traveling as much as we can and doing as many things as we can, making as many memories as we can, putting our time and resources into that maybe more so than accumulating stuff. Who knows how long we have, or how many good healthy years there are, you know we don't want to wait until our kids are old or until we retire to see or do things. We want to do it now, so. You know overall I think it's kind of giving us that perspective, but you know I think the key for me is not living in fear of the future but in being excited for the future. Even though sometimes the future doesn't seem exciting. Those years leading up to transplant felt like you know whatever this is the end. But choosing not to live in fear, I think has let us embrace the here and now and just a little joys of enjoying everyday life with the kids and the little things that they do. They are they just are so full of wonder and excitement about the world, so I kind of feel like I am a kid again living it through their eyes, which is this really me and exciting and they make our Lives full of joy. You know PSC tries to steal joy and excitement about the future. I would say choosing to not live in fear was key for me.

PSC Mami: You mentioned you had those moments of asking a question, is this the end? Like how did you navigate those?

Heather: I decided that it was okay to think about that for a little while and to give that a little space, and then move on and not dwell on it. My husband and I had a couple of conversations of what if this and what if that. I would want you to fall in love and remarry and blah blah blah kind of all those things. But in reality, thinking okay that might be reality, but it probably won't be so. We gave it a little bit of space and talked about a couple of things that we felt maybe we had to talk about. I didn't necessarily make a will or those kinds of things, although I think that could also be a good idea. I had to realize that I was okay with if my kids happened to grow up without a mom. That is probably the hardest part of the process that you have to go through mentally in this whole question of parenthood period and realizing that that might be the case. But I also had the perspective of working with the kids and working with people who have been sick themselves. And there really is no guarantee that anyone has a tomorrow. It feels a little bit more weighty when you feel like you know a reason that could steal your tomorrow, but knowing that no one is guaranteed a future necessarily helped me put it in perspective. It's definitely okay to think about dying. It's okay to think about my kids not having a mom. But not dwelling on that at all, because that was a darker place to sit and dwell. But planning for the future, planning for the next thing, was kind of what kept us going.

PSC Mami: Is there anything that we haven't covered that you want to touch on? Or anything else you want to tell someone who is at the beginning of this journey?

Heather: I would say at the beginning of the journey, just know that it is a journey and you don't have to know everything now at the beginning of the journey. You don't necessarily know how it's going to look, and it's so different and it's so personal for each person and each family. There is no right or wrong way to do it. You are brave. You're brave for being here and listening to this and hearing other people’s stories. And that you can have your own beautiful story. I think that is what I would say.

PSC Mami: Thank you so much it's been so great chatting and I look forward to seeing you at the next conference.

Heather: Great I look forward to seeing you too.


 

Bonus:

PSC Mami: From your perspective as a nurse,  how people should look for that those kind of medical teams, I hear you were very purposeful in how you chose your teams. Are there things that you can talk about that would help people?  Is there advice about how to go about choosing your health care team? 

Heather:  I chose both of my liver specialist based on recommendations from other specialists that I had connected with through the PSC Partners conferences. Shortly after diagnosis, I had been up to the Mayo Clinic in Rochester for a second opinion, and I had gone plugged in with them and ask them for local doctors in Dallas that they really recommended. So they threw out a couple of names. So that's how I actually chose my liver specialist here in Dallas.  My first specialist was at University Hospital, so I kind of looked at all the OBGYNs that practice at that hospital and looked for somebody that was highly recommended by my peers and by her peers and also I called the office before I made an appointment and talked to  one of the nurses that worked at the office about PSC. I did a little bit of research but then interviewed my doctor before I chose them, and I would tell anyone that if they have access to a university or to a teaching hospital or transplant center that's probably the first place I would look. Because those specialists tend to be more up-to-date with the latest research. If it's a teaching hospital they really have to be up-to-date with research because they're teaching the next generation of physicians. Then the university hospitals tend to participate in research so they tend to be on the cutting edge of medicine and used to managing high-risk populations. You can just call and talk to them and ask what their experience is managing a high-risk patients with liver disease.

PSC Mami: One of the things I always loved about having you in the room at the conferences was that you had the personal experience but you also had this really great perspective about "this is what the research shows," how do you manage knowing all the information from a medical professional perspective and also how it affects you personally?

Heather: Yeah, really there is no research out there is very little research on pregnancy and PSC specifically, it is definitely a field that needs to be researched. I think, at least at the point when I was pregnant a few years ago when I was looking into the published research there was a paper published about maybe 10 women who had PSC that were pregnant of at the Mayo Clinic and so really there wasn't research.  Talking to my peers at the PSC conferences, we were getting a lot more personal stories than really had ever been collected anywhere in the literature and so and this is one of the things that I really love about your podcast is that it's a great way to put these stories in one place which really doesn't exist very much in the PSC community in regards to Women's Health and pregnancy. Two of those tidbits just knowing from the medical side of it there are so many conditions in pregnancy that can be high risk and having liver disease I don't know if itself automatically causes any of the high-risk conditions, unless your liver is really end stage and cirrhotic and you're having a lot of problems with that like portal hypertension and like stuff like that. Really early stages of PSC when your liver is still doing very well and functioning very well there really is no real research so far that's published yet, maybe it's being worked on and I hope it is, specifically how it relates to pregnancy. Even any of the research they have doesn't specifically mean that each person would specifically relate to them, you still have to take the information and sift through it with your physicians, your hepatologist, your OB-GYN or high-risk OB-GYN and try to put the pieces together as far as how your body and other conditions you might be dealing with in addition to PSC- how that all might fit together. And how your  body might respond to pregnancy.