Belle talks cystic fibrosis, postpartum psychosis, mast cell activation syndrome and PTSD.
Hi, I’m Brianne Benness and this is No End In Sight, a podcast about life with chronic illness.
Today I’m talking to Belle about cystic fibrosis, pospartum psychosis, and mast cell activation syndrome. Belle’s story touches on so many different elements of life with chronic illness that I’m really excited to share it with you. She talks about insurance issues, the decision to get a port, and how to be your own best advocate.
This episode has more background noise than usual, so if that’s overwhelming to you then I definitely recommend checking out the transcript at noendinsight.co. Because Belle is a parent. you can occasionally hear her daughter MacKenzie in the background. I don’t want to apologize for this, because I think you’ll all understand that chronic illness doesn’t exactly lend itself to ideal recording conditions and I really appreciate it when people make time to talk to me however they can. I do, however, want to apologize for the occasional saw noises in the background. Those are 100% on my end because we were having repairs done to the roof of our garage and the contractors didn’t exactly give us a strict schedule to plan around. Anyway. Noises happen.
I also want to add a quick content note to mention that Belle briefly discusses self-harm early in the episode while talking about her postpartum experience, and she also discusses sexual assault later in the episode when PTSD comes up. These are both super brief and non-descriptive, and if you hop forward a few seconds you should be past each topic.
Before we start, here’s my disclaimer:
This podcast is not intended as a substitute for professional medical advice, diagnosis or treatment. Make sure you talk to your practitioner about any questions or symptoms.
Brianne: No matter what the diagnosis is, I like to start with asking were you healthy as a kid or how was your health as a kid?
Belle: Pretty healthy. I was a cheerleader, played softball, basketball and everything. As far as my health as a kid, I was pretty healthy except for when I was a baby and a toddler, I had a lot of migraines. Okay. And our children’s hospital never figured out why those migraines were happening. And I was very constipated as a child. Like I had to have enemas and suppositories and stuff for not only my headache but also the constipation. So other than that, like once I started school I really didn’t have any sort of issues, didn’t have issues going into my teenage years. In my first pregnancy with my son at 16 there wasn’t really any.
Brianne: You probably had a number of tests done for whatever health-related stuff and everything was looking good at that time?
Belle: Except for what they thought was asthma, which they diagnosed me with when I was 10. Then because of my depression, they said that my small size was due to anorexia. I was never anorexic.
Brianne: But you were in a weight class that medical professionals were suspicious of? I’ve talked to other people who have kind of said that.
Belle: I’ve always been under BMI, so underweight, at like a 16 BMI. So they were always like, “Well you’re underweight, you’re not eating or if you are eating, you’re probably throwing it up because you’re thin.”
Brianne: And you’re like, “That’s not happening. But it doesn’t matter, kind of.” And then I just want to like… So that was at 16 but up until that point, so your migraines had gone away kind of on their own before that?
Belle: I still had headaches and migraines, but they weren’t as frequent and they really happened when I was having like allergy type flare-ups, so now they’ve kind of figured out what they think was triggering them when I was little, which was probably sinus headaches and sinus pressure.
Brianne: And especially as a kid, I’m sure it would be really hard to explain what your symptoms were. Like you’re just a little kid and you’re like, “It hurts!” That’s it. If you’re even at that point. And then digestion had obviously improved, but you were underweight, which may or may not have been related to anything. Okay. So then you had your first pregnancy at 16 and as far as pregnancies go, things went pretty well?
Belle: I gained seven pounds. He was seven pounds 10 ounces, that was the only abnormality.
Brianne: Wow. And then at some point, obviously things started to change. So what happened? Or also, how was it having your first baby? I can’t imagine. And now, as you’re cuddling with your baby, it’s a lot of energy for a healthy person.
Belle: My first baby, I didn’t hemorrhage but I was overdrugged. They had me on demerol, phenergan and an epidural. And with him, I don’t remember holding him for the first time or anything like that. I just have pictures and that’s it. That’s all I have. And after he was born, when he was five months old… I could only breastfeed him for five weeks, and then I started suffering from postpartum psychosis. So I was hearing voices. Postpartum psychosis is not a thing that’s highly talked about either.
Brianne: Right, you hear more about postpartum depression.
Belle: Yeah, or postpartum anxiety. And so when I was dealing with that I didn’t know what to do, so I just kind of tried to fight it the best I could. You know, people say you could get depressed or you could get a lot of anxiety issues leading into the first year after having a baby. But nobody ever told me that psychosis was a thing and you could start hearing voices and having hallucinations.
Brianne: Right. That would be scary!
Belle: And I was having hallucinations of people that weren’t there. People telling me to kill my son, that I’d be better off without him, hearing voices in my head, all of these things. And that was the only real issue. And that was attributed to how much medicine they had given me.
Brianne: At birth?
Belle: Yeah. Because I didn’t get that bonding time with him that I really needed.
Brianne: And had you gone to the doctor pretty quickly? Like did it take you a while to realize something was happening, because I think that would be one of the difficulties.
Belle: Yeah. He was five months old and I checked myself into a psych ward. I woke up one morning and I looked at my mom and I said, “I need help.” And so we went into the counseling associates, one of our local counseling places as an emergency case. And my mom told them that I was homicidal and suicidal and I needed to be admitted. And of course they verified that with me, because I was 17 about to be 18. I was 17 when he was born. So I automatically was like, “I need help.” I went in for a week and they put me on depression meds and anti-psychosis meds because they were like, “Well, you’re just depressed.” Depressed doesn’t equal hallucinations and voices. And they just weren’t understanding what I was saying, I guess. Or they hadn’t dealt with teen moms that had PPA.
Brianne: It didn’t feel like they were hearing you, it sounds like.
Belle: And so we went from dealing with that and me getting out after a week on meds to a week later, me falling asleep in class because I had taken two of the same meds that I wasn’t supposed to take together by accident, to further on in the week cutting. So I immediately stopped all of my meds, like I’m not doing this anymore.
Brianne: Right. It’s not working and it’s not helping with what it’s supposed to help with. It sounds like.
Belle: Then it slowly just kind of dissipated as my son continued to get older. And then when I really got sick was back in 2015. I’d fallen at work and they couldn’t figure out why.
Brianne: Like a fainting kind of fallen or a tripping kind of fallen?
Belle: My knee gave out. It was like I couldn’t put weight on my leg anymore. And I just melted to the floor.
Brianne: Yup. Yeah. Melting is a good word for something that bodies can do.
Belle: After my shift I went to the hospital, my oxygen saturation was fine. They did x rays on my knee. They were like, “We don’t know.”
Brianne: “You seem fine now,” kind of thing.
Belle: Also, “We’re going to wrap your knee and we’re going to give you crutches and you’re going to go see an orthopedic doctor.” I said, “Okay.” I went to the orthopedic doctor. The orthopedic doctor looked at the x rays and he was like, “I don’t see anything wrong with you. You seem fine. You look fine. Your knee is working fine. I don’t know what to tell you.” I developed a petechail rash from my knee down that lasted a day. Nobody could explain why that happened. But no blood clot. So I started to research on the internet because at this point I had lost my job.
Brianne: Right. And was it a job where you’d been on your feet?
Belle: Yeah, I was a cashier.
Brianne: Yeah. Okay. And that was not feasible probably.
Belle: Yeah. And so I lost my job because I had had a couple of other episodes that they thought were cardiac episodes where my blood pressure skyrocketed, but my pulse rate went into the toilet per se, or vice versa. My blood pressure would sink and my pulse rate would skyrocket. Which doesn’t make any sense because typically if your pulse rate goes up, your blood pressure would go up. And so when all of that was happening, I was having to leave work and go to the ER and they couldn’t explain it. They sent me to cardio. They did an EKG, an echocardiogram. They were both fine. There was some minimal leakage around some of my valves. But that was pretty well it. And then I had fallen. They were like, “Well, we don’t know why that happened either.” So I started researching. And I had a friend call me. And my friend called me and she was like, “Your random symptoms that nobody can explain sound like difficulties of undiagnosed cystic fibrosis.”
Brianne: Sorry about my background noise! [re: intermittent saw noises]
Belle: It’s fine. I have it too.
Brianne: Okay. So your friend, somehow either they stumbled upon it or they read something or whatever.
Belle: She had twins that were born double delta F508s. And she didn’t know she was a carrier of the disease. And when her and I had been working together, her and I kept getting sick at the exact same times if we were within the six foot… if we were both sick. Because we were passing each other’s germs to each other without knowing it. So when she called me with that information, I googled it. Of course found Mary Frey and the Frey Live youtube channel. And watched a couple of her videos about what it was and what it was about. And I was like, “That sounds sort of fitting.” For the fact that if I was sick and my oxygen sat, even though at the hospital they were fine, maybe my muscles and stuff weren’t getting enough oxygen and that’s why my knee gave out. Muscles need oxygen to maintain strength and blood flow. If they don’t have it, they weaken and you melt into a puddle on the floor.
Brianne: And then after the incident it could right itself very quickly so as to be not specifically detectable.
Belle: And then with my trouble breathing and inhalers never really helping, and then my low weight gain, I was like, “Hmm. This kind of all adds up and makes sense.” So I called my doctor, because we thought it was rheumatological and all of my rheumatological stuff came back normal. Except for my vitamins. They came back low. And so I called my doctor, I said, “Hey, humor me, do a sweat test.”
Brianne: And did they humor you?
Belle: She humored me. She did a sweat test and it was high.
Brianne: And so what is that testing for?
Belle: Sweat testing is two electrodes that go on your arm, that induce sweat production. They collect the sweat and anything below 30 is normal, between 30 and 59 is borderline, and anything 60 or above is positive for cystic fibrosis typically. So when they do this testing it has to be done a very specific way and very little contamination and I have to make sure I’m not eating certain things or taking certain medications, otherwise it could falsify the test. I got my first results back on my 23rd birthday. They were high. I was on my way to Seattle with my husband and we were just going up there for a turnaround trip and we were in Kansas I think when they called and they said, “When can you get in here for blood work?” I said, “I’m on my way to Seattle, it’ll be like four or five days before I can get in.” They said, “Okay. When you get back, head up to the lab, the order will be waiting.” So I did and got back from Seattle after speaking with a lady who was training and new service pup. And I have interests in service pups. And so her and I got to talking and come to find out her husband was one of the cystic fibrosis doctors in Seattle. And she was like, “I’d like you to come have coffee with my husband and kind of talk and get his opinion on everything.” And his opinion was that I most likely had cystic fibrosis and not to quit until I had the proper medication and diagnosis and all of that.
Brianne: Yeah, like a treatment protocol.
Belle: And so when I got back, she ran the lab. She sent the lab to Mayo, they ran 408 mutations I think. All of them came back negative.
Brianne: Okay, and so that’s genetic testing at this point?
Belle: For CFTR mutation. Where there’s over 1200 at this time, 1200 known mutations.
Brianne: Right, so identified.
Belle: There’s 1700 identified mutations, with 300 still being tested. And so when those mutations came back, she was like, “I’m not going to humor this anymore. I don’t think you have it. I’m moving on from this.”
Brianne: Great, thank you.
Belle: So I called my rheumatologist and I said, “Hey, I need an appointment.” So they gave me an appointment. I went up there and I talked to her and I said, “I need a referral to pulmonary,” She goes, “You’ve never seen a pulmonologist even though you’ve been diagnosed with asthma since you were 10?” I said, “Never seen one. Don’t know what that is. I know it’s a lung doctor, but other than that, I know nothing.” So she goes, “Okay, I’ll send the referral.” So she sent it but because of my insurance, when I went to the pulmonologist, they had to have a referral from my PCP. So the doctor that told me she wasn’t going to humor it anymore is now mad at me because I went above her head. And so she’s yelling on the phone. She finally sends over the referral. I bring that to the pulmonologist. The pulmonary doctor looks at me and says, “I don’t think you have it, but I want you to do another sweat test.” I said, “Okay.” So I did another sweat test. Lo and behold, it was high again. I said, “Okay, now what do we do?” He was like, “I want to send your results to  and see what  says.”
Brianne: And is that like I specialized testing facility? Like a private lab?
Belle: Yeah, they’ve been the longest running lab to do CF testing privately for a very long time.
Belle: So they took my blood, they sent it to .  came back in big bold letters across the top they said “CF carrier.” Because I have one known disease-causing mutation and the other mutation is supposed to be a benign mutation. He was like, “I still think you have asthma. I’m going to put you on all of these breathing treatments. And not anything else. And we’ll go from there.” Well by this time I had fired my primary care doctor.
Brianne: Sounds like that was a good move.
Belle: Even though she’s well known because she’s the daughter of our town vet. And so I fired her and found another doctor, Dr. Roberts. Dr. Roberts is a female doctor from the Caribbean. And I went up to her office in Fort Smith and I said, “Here’s what we know. Here’s what we have. Here’s what I can get you. And here’s the paperwork.” And she looked at it and she goes, “Do they not know sweat testing is the gold standard for diagnosing cystic fibrosis?” And I said, “They should because they’re pulmonologists, but apparently not. So can we see a CF team?” She goes, “I think that’s a good idea.” So she sent me to the cystic fibrosis team in northwest Arkansas. They’re a non-accredited facility.
Brianne: Okay. So what… So who is there or how does that work, and how does that work with insurance?
Belle: Cystic fibrosis teams are specialized care teams that consist of pulmonary, some type of therapist, either physical or respiratory. Typically a nutritionist, a pharmacist, social worker, coordinator… I think that’s it.
Brianne: Okay. But a robust care team, definitely.
Belle: Sometimes GI depending upon symptoms, but you don’t see GI every time. They’re usually an outside team that just work with your care team. But every time you see the doctor, you typically see doctor, coordinator, social worker, physical or respiratory therapists, nutritionist, and a pharmacist. Appointments are typically four hours long.
Brianne: Yeah, that makes sense. It’s a lot of people to see.
Belle: And you do PFTs every time and you get blood work drawn every time you go. And so I went up there and they looked over my paperwork. They said, “We don’t think you have CF, we don’t know what you have.” I was like, “Great. You guys are supposed to be the people to know what is happening and you don’t know what is happening and you’re not helping.” So my doctor is like, “Well, we’ll do more testing.” So we go through more tests. And they tested, they did what’s called a fecal fat test, which is how much fat you have in your stool when you poop.
Brianne: So it’s like you’re not digesting it properly.
Belle: Yup. And so they did that and it came back at an eight, but I was not told the proper direction of how much fat you’re supposed to consume and all of that. So I knew I wasn’t eating 150 grams of fat a day because I didn’t eat that much fat in my normal diet at that time because it made my stomach hurt. Well, he put me on creon. Creon is a pancreatic enzyme to help you digest food. And he put me on that. I was on symbicort, albuterol four times a day, an albuterol inhaler, ranitidine, now the creon, flonase, and that was it at that point. That was in February. In June, I went back and he said, “I’d like you to see Mayo Clinic. I don’t have any answers for you. We don’t know why you’re so short of breath and can only do eight sit to stands in a row. So I started trying to get that ball rolling.
Brianne: Yeah, how was that?
Belle: While trying to get that ball rolling, I had insurance problems first. Well first I had a problem trying to get an appointment because Mayo books out for forever.
Brianne: Yeah right, and it depends why you’re going, but it’s hard to get there is my understanding.
Belle: And so trying to get there with everything, we got an appointment scheduled finally after like three months of me calling.
Brianne: Okay. Just to even get into booking basically.
Belle: Yeah. And so we booked my appointment for December 18. While trying to go through all of that, they ask for your insurance and all of this. Well they ran my insurance and my insurance rejected it because in 2018 as of January 1, you could not take any sort of government assisted or government funded insurance outside the state of Arkansas.
Brianne: So you could only get treatment within your state.
Belle: But there was a way to possibly override that. So we had sent in what’s called a network exception form saying, “Hey, she really needs to go here.” All of my doctors on my team agreed. And that was supposed to be all we needed to get everything going and get me up there to the team. And by this point it was November, it was about a month before I was supposed to go. They rejected it.
Brianne: They rejected the form?
Belle: Insurance did. Insurance said, “Well, you can get the exact same care here in the state of Arkansas. You aren’t seeing an accredited team anyway.” So while working with a case manager, I was attempting to figure out how to get in to see the accredited team. By this point I needed another new PCP.
Brianne: How come this time?
Belle: Because my PCP went to end of life care. She switched specialties. And when she switched specialties, she required everybody to either go to somebody else in her practice or leave her practice completely and go from there.
Brianne: So find a new person.
Belle: Well, I didn’t want anybody else in her practice. So I started trying to find new PCPs, finally I found one. And so I’d gotten another new PCP and he sent the referral for me to the accredited team down in Little Rock.
Brianne: Okay. So still in state, but a new specialty care team?
Belle: Yeah. Trying to get the form approved for me to still go to mayo. Well, they couldn’t see me until December 14.
Brianne: Which was a little late.
Belle: That’s not enough time. So my husband and I made the executive decision to take out his work insurance, still go see the Little Rock team, but fill out his forms and stuff during open enrollment so we would have a fallback. And change my appointment to Mayo, which got changed to January 28. I went and saw Little Rock, my respiratory therapist, she was like, “If it looks like a duck, talks like a duck, walks like a duck, it should be a duck. It shouldn’t be a mouse in duck clothing.” So she was like, “We need to get you on a vest. Your PFTs are normal, but they’re not 100% normal.” And so we started working on getting me on vest therapy and more treatments. We started hypertonic saline and we got a sputum culture. Well, I was culturing H flu and my lungs. And that’s pretty normal for CF patients. And when that happened they weren’t concerned about it. We continued on and right after I got out of the clinic, which was on a Friday, Sunday I was feeling ill. I just wasn’t up to par or anything like that. So I called my team, they tried me on several different antibiotics and then I was admitted for a week and released. And after all of that they were like, “Well you need to be on pulmozyme. So then I was arguing with the insurance company about pulmozyme and why it was needed and everything like that. And I’m getting closer to Mayo, I leave the 28th and this is like the second week of January.
Brianne: Okay. And at that point were you still trying to get a waiver for coverage or would the new insurance have possibly covered it?
Belle: We just let our new insurance cover it. They wouldn’t cover it fully, but they would cover a lot of it. So we went from there and I was trying to get… We still had our regular coverage that covered all my prescriptions and stuff like that and anything that I had in state. So we’re fighting with them for coverage of pulmozyme and coverage for a vest.
Brianne: Okay. And are those both really expensive treatments in terms of what they cost insurance?
Belle: My vest is a mobile vest, so it doesn’t plug into a wall. It’s called a monarch and it’s by hill-rom and I think the estimated cost is like 20 grand.
Brianne: Okay. So certainly not cheap. But I assume it’s a one-off thing as opposed to say an infusion that costs that much every month. Right.
Belle: And then pulmozyme is $4,000 a month.
Brianne: Okay. Right. That kind of thing, which is… They’re both expensive but one off and also recurring.
Belle: The vest got approved before the pulmozyme did because my respiratory therapist is amazing and continued to call insurance and argue with insurance and argue with hill-rom about everything. But when I was released from the hospital, I was given a loaner vest, one that plugs into the wall that I could use for therapy. So I wasn’t going without therapy at home during the interim.
Brianne: And what does it do? How does it work? How often do you use it?
Belle: For the normal vest, which is called a 105 or even an afflovest, they work by blowing up air to a certain pressure, whatever pressure you set it to, and then oscillating the air inside. So it vibrates at a certain hertz frequency. And that didn’t do a whole lot for me.
Brianne: Okay. And from the beginning, like before you got your own vest? So you were trying it out anyway.
Belle: Yeah. And it kind of helped me make a decision between an afflovest or a monarch. Monarch is a one-of-a-kind type of vest made by hill-rom that uses magnets on the inside to basically hit you. They swirl around and they hit each other. And I don’t understand the mechanics of it all, but it’s supposed to mimic chest PT that you would get from a respiratory therapist. Which is where they cup their hands and they basically beat you to death on your back and your sides and your front and everywhere else.
Brianne: Okay, so they’re like stimulating all around your chest cavity.
Belle: Yeah, trying to get the mucus out of your lungs. So I went with the monarch, which is a 14-pound vest in and of itself. I’m a very small person. I weigh 94 pounds and I’m 5’4″ almost 5’5″. I’m like 5’4″ and 3/4. And so that works by hitting or oscillating, trying to get the mucus on the bottom of your lungs up so you can cough it out. That works in succession with a nebulizer.
Brianne: Okay. Yeah. And with drugs in the nebulizer, or just with saline?
Belle: With drugs in the nebulizer. Like for me, I do a nebulizer twice a day. In the morning I do albuterol and hypertonic saline, 7%. it comes in three, five, seven and 10. And then at night I do it with albuterol, hypertonic, wait 30 minutes and do pulmozyme.
Brianne: Okay. Yeah. It’s such a process, right? Just having the medical routine where you have to time everything and fit everything in and stay awake for everything.
Belle: The weird thing about pulmozyme is pulmozyme is actually made from Chinese hamsters ovaries. So the ovaries of a Chinese hamster.
Brianne: Sure. Perfect. Medicine is weird.
Belle: And like… how? But okay. Because pulmozyme works, basically how my routine works is the albuterol is used to open my airways even though we found out later the albuterol really does nothing for me but it helps to keep my airways open with the nebulized hypertonic and the nebulized pulmozyme which can be bronchoconstrictors.
Brianne: Okay. So it’s like opening it up to get everything else in there.
Belle: And the hypertonic clings to the mucus and gives it moisture because you can have really dry mucus in your lungs. CFers have a full range of different kinds of mucus, either it’s dry and crusty, thick and wet, thick and dry… It ranges with everyone.
Brianne: The whole texture.
Belle: They think mine is very dry because they can’t see it on like an x ray or even a chest CT. So they just think mine is very dry and there’s not a whole lot, but stuff that I need to get out. So they put me on the hypertonic that hydrates and loosens it and then the pulmozyme breaks it up. It makes it easier to cough up.
Brianne: Okay. And so that is like a literal enzyme, I’m guessing, it’s some kind of a biologic? Comes from a hamster….
Belle: Yeah. I don’t understand how that works with pulmozyme.
Brianne: But it’s a mucus breaker upper.
Belle: I don’t know what part of the Chinese hamster’s ovaries help break apart mucus, but it works. So I’m not gonna question it. I mean, my creon is made from pancreatic enzymes of a pig.
Brianne: Wow. Medicine is a weird world, I think. Sometimes we forget. So you eventually got that approved, the pulmozyme.
Belle: Yeah. So they tested some plant enzymes to see if they would work as efficiently as pig enzymes for religious purposes.
Brianne: Or like vegans.
Belle: They don’t work as effectively if at all. So they’re still working on that process? So, while we’re fighting and going back and forth, I finally get pulmozyme approved like two days before I’m supposed to leave for Mayo. It’s supposed to be an overnight shipment. But my Walmart had already ordered it and had it sitting. But my insurance typically won’t let you go through Walmart. It has to go through a specialty pharmacy and be overnight mailed. Me being me, I probably didn’t go about it correctly, but I threatened them. And I said, “You need to approve my pharmacy to release it to me, even if it’s a one-time thing.” So I’m at my pharmacy and I’m waiting on them to call me back. And my friend who works there, because I live in a small town, she keeps pressing the button to submit it to insurance. And it finally goes through and she’s like, “I don’t know what I did, but it went through.” And about that time I get a phone call from insurance saying they’ve let it release for a one-time shipment because why make my pharmacy pay for the medicine and then not be able to get the money back from the person that’s supposed to take it from them. And so I get ready to leave for Mayo, which is packing a suitcase, two children, a vest machine, a nebulizer, all my medications and my pulmozyme has to be refrigerated!
Brianne: I was going to ask that. Yeah.
Belle: I’m going to Minnesota during a week that’s supposed to be the polar vortex in a one-ton truck with my grandparents. There is no room. Our floorboards are filled with stuff. The bed of the truck is filled with stuff. We get to just outside of Mason City, Iowa, and we stopped for the night. But of course that means getting my pulmozyme out of the car, making sure my shakes are out of the car because I’m on nutritional supplements. They can’t freeze, otherwise they explode. Trying to make sure I have all of my meds inside the hotel room to take when I leave and when I wake up.
Brianne: Yeah. And using a nebulizer in foreign places is extremely annoying. I’m sure using a vest in other places is also frustrating. Like you get a routine.
Belle: My vest is much quieter than a regular 105 vest or even like the afflovest. It’s just simply quieter. My nebulizer’s not so quiet. It’s just not, it’s a big powerful nebulizer.
Brianne: Well it’s an air compressor! Like…
Belle: For CF treatments. I mean they make smaller ones that are quieter and are more affordable, but with the amount of treatments I do every day, it just wasn’t feasible. I’d burn through tiny compressors in like three months. So when I got done there we went to Rochester the next day, which is about a one-and-a-half-hour drive. Of course we went and got food and I had a waitress spill water all over me.
Brianne: Not today!
Belle: And she was very rude. So just really…
Brianne: Yeah, you’re off to a good start.
Belle: And we got up there, we checked into our airbnb, which was really nice to have just a place where we could relax and everything like that. The airbnb had left us a note and they had like bowls with snacks and stuff like that. Chips and candy and cookies and anything that I could want or need to snack on. And so we got to relax part of the day Sunday and then Monday I had to be at Mayo at 9 am. Of course there’s snow on the ground and it’s freezing and we get to Mayo. My grandmother comes with me with the baby and we see the doctor, a pulmonary doctor with Mayo clinic. He’s also their assistant professor of medicine. He was like, “Well, what is your main goal to accomplish while you’re here?” I said to figure out why I’m so short of breath, but I have no respiratory symptoms as far as PFTs go. So my pulmonary function tests were always within normal range, but I get winded talking or doing things.
Brianne: And was that… did you have any other ongoing chronic symptoms at this point or did that tie in to other things? Like whatever, I don’t know what else that would be for something like CF. But people will have, I dunno, brain fog or different kinds of chronic pain or your energy levels kind of, what fell under that umbrella?
Belle: Fatigue. Fatigue still, stool issues even on creon, not gaining weight, staying pretty stagnant. Not dropping, but not gaining either.
Brianne: Yeah. When that was your intention in terms of diet and supplementation and stuff.
Belle: Shortness of breath, not being able to walk long distances, not being able to take care of my kids, chronic cough that has been around forever, and heart palpitations.
Brianne: Right. Yeah. You’d mentioned some kind of tachycardia and stuff, episodes.
Belle: Yeah. Things like that. And they’re like, “Okay, we’re going to run a bronchodilator test to see if you respond to bronchodilators, we’re gonna do a CPET, which is a cardiopulmonary exercise test. We’re going to see endocrinology, you’re going to see nutrition and you’re going to see a gastroenterologist.” And I said, “Okay, this sounds like fun.”
Brianne: Yeah. Lots of stuff.
Belle: So the next day I had to be there at 6:45 in the morning.
Belle: And I had a sputum culture that I had to do, which is an expressed sputum doing 10% hypertonic, trying to get me to cough up something. And that one I think was good, but there still wasn’t enough for Mayo’s lab to swab, which makes no sense because my lab can swab it and they get the exact same sample. So if my lab here can swab it and get results and they don’t say it’s not enough for adequate retrieval or whatever. Why can’t your $8 billion lab to do it? But my $1 million lab can do it. But I wasn’t going to argue. I just left it and continued on with further testing.
Brianne: Yeah, you had a big day.
Belle: Then I had to do a bronchodilator. And a spirometry. And they were like, “Well you can’t do a bronchodilator and a CPET back to back. That’s not how this works.”
Brianne: Or like, what one of them does will influence the other one.
Belle: Yeah. So they had to be six hours apart. So my schedule got completely changed that day and I had to go from doing a spirometry to doing my CPET to then doing my bronchodilator. My CPET was relatively normal. 75 watts, leg fatigue was the main issue why I couldn’t continue going. And I lasted seven minutes on the bike. And they didn’t see anything as far as cardio went or as far as lungs that was holding me back. They’re like, “Well this doesn’t make sense.” So they diagnosed me with severe deconditioning. I was just out of shape.
Brianne: Yeah. Which is like, you haven’t been using your muscles, so now it’s hard for you to use your muscles, basically. That’s always like, yeah, okay maybe, true. But also I didn’t stop for no reason. Even if I have continued without… Yes. Okay. So, so far that’s what you know.
Belle: And then my bronchodilator came back and they were like, “Albuterol does nothing for you. It actually makes your lung function less.”
Brianne: So that’s when you found that out?
Belle: Yes. And I’d been doing albuterol four times a day at this point, plus inhalers. Four nebulized albuterol treatments plus inhalers.
Brianne: And you didn’t… I mean, probably when you’re doing it that often, you don’t know the difference between that and not doing it.
Belle: Yeah, exactly. And so I saw my doctor the next day and he was like, “Well, I think this is a good start. Let’s do nitrous oxide and an overnight pulse ox and see what your pulse ox is.” Well my overnight pulse ox showed a possible arrhythmia and my mean oxygen level was 96%. Still not terrible. Not exactly normal for a 25-year-old mom who is chasing her kids and homeschools and is not exactly your average soccer mom. Like I don’t go to work and sit on my butt all day, I have kids. And I played sports in college and coming from an active background. I’ve always had muscle tone loss and they attribute that to the lack of vitamins that I was absorbing and getting in my diet.
Brianne: Right, that you seemed to be malnourished for some reason.
Belle: Yeah. And so they did a nitrous oxide test. Well, at that point I was on prednisone and symbicort. Those are two steroids. So a five is normal for somebody on steroids. Okay. Well what about not on steroids? They wouldn’t tell me what that number should be. Five was normal for somebody on steroids. Okay, great. Let’s move on to more. Endocrinology said that it doesn’t seem like I’m not taking in enough calories because I take in 3,500 to 5,000 calories a day with my shakes included, without them it’s 2,500 to 3,000 calories dependent upon whether I’m home or I’m out and about. If I’m out and about, I’m getting fast food and that racks up calories pretty quick.
Brianne: Yeah, like normal things that will cause variety in your diet.
Belle: So he wanted to do a resting metabolic panel to see what my resting metabolic rate was. And then he wanted to test trace minerals and some minerals and metals and vitamins. So I had more blood work and I had to go back early in the morning for resting metabolic, I had to do the resting metabolic because I had to do that before I did anything else. So I had to come back at 6:45 the next day to have that done. And when I had that done my resting metabolic rate was like 1,420 calories or something. So 1,420 calories. Daily intake is supposed to be between 1,500 and 2,000 okay. With adding in activities of daily living and everything, I would need 1600 calories in order to gain weight. And then you add another 500 because I’m breastfeeding.
Brianne: Right, and that certainly amps it up.
Belle: So you’re at 2,100 calories. I’m intaking 2,500 to 3000 so why am I not gaining? So we go from why am I not gaining to what exactly is going on? So they decide to do another fecal fat test. And they find out on my creon I am absorbing everything that I should be absorbing. So we still have no answer as to why I’m not gaining. We have no answer as to why I’m short of breath other than I have a concave portion of my sternum, I have CF, and I have four nodules in my lungs. And I’m extremely deconditioned. So when I head home, I have an exercise prescription. I’m off two albuterol treatments a day because albuterol’s doing nothing. I’m changing my pulmozyme from doing it in the morning to at night. And then I’m supposed to follow up with an allergist and my regular primary doctor because at this point they think the next possible thing would be food allergies. Food allergies even with creon, if you’re allergic to it you’re still not going to absorb it, no matter how much creon you take, you’re just not.
Brianne: Right. Like there’s a different problem that that won’t solve.
Belle: So when I went to my regular doctor, he sent a referral to the allergist and said, “Hey, she’s got low BMI.” And the allergist kicked it back going, “We don’t see patients just for low BMI.” So my doctor kicked it back and was like, “She has low BMI from possible food allergies and she has cystic fibrosis and she has random allergies that pop up that give her anaphylaxis.” Like, this girl needs to see an allergist.” Because I had a random allergic reaction to peanuts. I was eating peanut m and m’s and all of a sudden I felt my throat start to close.
Brianne: And I’m guessing that had not happened before. Like you’ve had peanuts before.
Belle: Nope. So now I go into anaphylaxis with peanuts. That’s not fun. I used to love peanut m and m’s and reese’s and all of this stuff that I now can’t have. And canned tuna, like I can eat fresh tuna. But if it comes in a can I can’t eat it.
Brianne: Yeah. I mean this is something that I feel like is just starting to get real recognition in chronic illness, that a lot of people get weird sudden almost unpredictable allergies. I was traveling last week as I think you know, cause we had to reschedule. And one hotel we were staying at, like an Extended Stay America or something, just some cheap place with a kitchen. But they had lobby coffee and I was allergic to their lobby coffee. Like I drink coffee every day. And two mornings in a row I had, not an anaphylactic reaction, but like a full flushing… Like a full histamine reaction, you know, like hives all over my face and stuff. And that’s the only thing it could have been. And it’s like how does this happen and why is it happening to so many people all of a sudden? With like peanuts you’d expect, but the tuna… Like what’s happening?
Belle: And then I get weird reactions to different forms of rain. Like, I’d go outside in the summer in Arkansas where it rains, and if I got water on my lower legs, they’d start itching like hell. And so I’d scratch my legs to the point like I’d have like stripes where they were bleeding because they itched so bad. And then it’d rain again and it wouldn’t happen. If I went outside when it was dry I was fine. And then when I was little, I’d turtle onto my back because I didn’t want the grass to touch me because it itched.
Brianne: Yeah. So you have like, allergy stuff.
Belle: So there is another chronic illness out there called mass cell activation syndrome.
Brianne: Which is a new diagnosis, I learned recently. Like it didn’t… I think 2016 was when it was classified. So it’s still under the radar a little.
Belle: And so that might be something that may happen. I’ve eaten like three of these oranges already yesterday, and now this is the only thing I’m eating and I’m itchy. Like why, why am I itchy now? I’ve already eaten like three of them. But now I’m itchy. Why?
Brianne: Yeah. That’s my least favorite thing is when it’s like, there’s no pattern here. There’s no pattern.
Belle: And there’s no hives. It’s just itchy.
Brianne: Yeah, something’s happening.
Belle: And like why, why is this happening? Why is it happening now? The only thing is the air in my house and the orange that is sitting on my couch. So that’s something they’re going to look at testing.
Brianne: So have you been to an allergist yet or are you waiting right now?
Belle: I’m waiting on a phone call from an allergist. Again.
Brianne: Okay. Because that’s your next step.
Belle: And so the biggest portion of my story that I want to get out there is even if doctors think they know more than you, do your own research. Always do your own research and push and push and push until you have no more options. I’m still having to push to get full diagnoses of things. Now I’m probably going to have to push at the allergist and be like, “Test me for mast cell, test me for food allergies.” Because there’s no pattern here and it is completely random, to random things not just food. Like food is my only anaphylactic type reaction. But wasp stings broke me out in hives the first time I ever got stung. This orange just made me incredibly itchy, rain made me itchy without hives. I get a lot of brain fog and memory loss. I have a lot of GI symptoms. Could this all be histamine reactions Because my dad has a lot of allergies. My dad probably needs to be tested for mast cell. But he hasn’t been. He probably should. So with cystic fibrosis and having the two extremely rare mutations that I do, there’s only one other girl that has them and she’s 18. And her mom messaged me because she was like, “Hey, what are your symptoms like? What were your sweat tests like?” Because they redid mine at Mayo after redoing them at Children’s. At Children’s they were borderline, at Mayo they were high borderline, almost positive. They were like 51 and 59 or something. So my mutations will be sent back to Johns Hopkins to do full sequencing, deletions, additions, the whole nine, to make sure that there’s not something we’re actually missing. And doctors with CF tend to take the database extremely seriously when diagnosing. So if they see that a mutation is supposed to be benign, they take it that way. When you’re supposed to treat the symptoms and the person because it is completely different for everybody. Mast cell is completely different for everybody. You’re going to have random histamine reactions for literally everything. And you sit there and may not exactly know what type of issues they’re looking at, but symptoms of mast cell disease are: headaches, brain fog, cognitive dysfunction, anxiety, depression, flushing of the face, head, neck or chest, hives, skin rashes, itching with or without a rash, lightheadedness, fainting, rapid heart beat, chest pain, low blood pressure – I have chronic low blood pressure – high blood pressure at the start of a reaction, blood pressure instability, uterine cramping, bleeding, bladder irritability, frequent vomiting, bone muscle pain, osteopenia or osteoporosis, diarrhea, nausea, vomiting, abdominal pain, bloating, GERD, nasal itching and congestion, throat itching and swelling, wheezing, shortness of breath and symptoms can be sudden and unpredictable on onset and patients may have a few or many symptoms.
Brianne: Right. It’s so many things. And I think it’ll be fine tuned a little over the years. But yeah, it’s like a lot of stuff can happen if that’s out of whack.
Belle: Well it’s like you have different levels of cystic fibrosis and each patient is different even if they have the same mutation. So you have class one, class two, class three and class four for mutations and each of them do different things. If they figure out mast cell is a mutant issue and it’s an issue within the actual cell itself, then they could probably fine tune that to saying, “Okay, class one has these type of reactions. Class two has these types of reactions.” But you’re still going to have to treat the person and not necessarily the class of symptoms that they have. Like ehlers-danlos syndrome or EDS. You have different levels. I have PTSD and anxiety from sexual abuse when I was a child and rape when I was a teenager and then physical abuse when I got into my adult years and had bad judgement in men. And some women. But bad judgment and everything caused me to have severe PTSD. Well, I didn’t tell anybody around here, so I waited until I went to Mayo. When I went to Mayo, they diagnosed me with severe PTSD and that looks different for everybody depending upon what triggered it. Did you see somebody die? Were you a military veteran? Did you have sexual abuse? Did you have some sort of trauma, like a car accident? Or you got drunk and you were raped. What triggered that? So you have to treat the person and not, “Okay, this person has PTSD because they were military” and treat all of those people the same. Because they’re all different. And that’s the best advice that I can give to any doctors or nurses, anybody in the medical field, is treat your patients individually. Don’t clump them into a class based on race or color or genes or symptoms. You’ve got to treat the person and I think a lot of MDs tend to forget that. DOs are more likely to get to the bottom of symptoms and what are causing the symptoms whereas MDs are more, “We’re going to treat the symptoms but we’re not going to figure out what’s causing them.” And that’s been the main difference I’ve found between the two. A lot of MDs though, if you find the right one…
Brianne: Yeah, which is always the thing.
Belle: Will be willing to work with you. And the best advice I can give to other chronic illness patients or moms of kids with chronic illness, I have a child who has anxiety and high functioning autism, and my daughter has developmental delays. Find a doctor that’s willing to work with you. Don’t see a doctor that’s going to treat you like you’re stupid. Because you most of the time are going to know more about your own disease then they get in their entire years of med school unless they’re specialty doctors. Those can tend to, but it’s going to depend on what area you’re in. So like at Mayo, your specialty doctors each had subspecialties or things they were interested in helping or learning about. So Mayo tries to match you up with those doctors. Sometimes it works, sometimes it doesn’t, but like here in Arkansas they really don’t, we really don’t have doctors that personally put their interests out there like, “Hey, I’m a gastroenterologist and my interests or subspecialties are malabsorption and malnutrition and eating disorders.”
Brianne: Right, right. But then there’s probably, it’s like a population thing because so many people come through Mayo that they can really choose who they want to see almost versus when you live. Like, I live in Massachusetts, but I live in western Massachusetts, so also a relatively rural area. And I think people do travel to Boston for when you can and need to. But everything else it’s like… I just got a neurology referral and there’s like three people total that I could be referred to. And one of them, my doctor was like, “Nobody really likes this doctor, so I’m not going to send you to them.” You’re like, “Okay, cool. Like these are the options.” So they’re probably not doing a lot of specialization.
Belle: Yeah, it’s like, “These are your options.” We have where I’m at, I’m in what’s called the River Valley in Arkansas. So our capital is Little Rock, it sits pretty much in the middle of the state. Fort Smith is on the Arkansas/Oklahoma border. Fayetteville sits towards the north, towards Missouri. So for me, where I live, I am equidistant to Little Rock and to Rogers. I am 45 minutes from Fort Smith, Fayetteville’s 45 minutes from there. Little Rock’s about two hours. So anybody that I get referred to as either in Fort Smith, or north or south into Fayetteville or Little Rock.
Brianne: Yeah. So one or two hours.
Belle: Those doctors seem to be more… except endocrinologists, most of them don’t have like subspecialties. Our endocrinology team up in northwest Arkansas that’s with Mercy, they have subspecialties. When they get the referral in, they match you with an endocrinologist that is going to be the best to help you. I have to have iron infusions, so I probably need to call him. I have to have more iron in April and I don’t see him again until June.
Brianne: There you go. Lots of thing to keep track of.
Belle: Because they found out my total ferritin stores was a 12.
Brianne: Okay yep, low.
Belle: So, total iron. But my hemoglobin was completely normal so I’m not anemic.
Brianne: Right. It’s like a… is that a conversion issue, something like that? The way that you are using or processing it. Yeah.
Belle: Yeah. So now I need to make sure I continue to get iron infusions. But I only have two good veins in my arms and IVs like to push out and my doctor at Mayo was like, “I don’t think a port would necessarily be a good idea just because if you go under anesthesia then you have the issue of losing lung function and I’d like to preserve your lung function as best as possible. But if you think that is a good idea for you, then you need to go ahead and do it because I don’t know. I’m a doctor, so I don’t draw blood, that’s not what I do.”
Brianne: Right. “I’m not the one doing that operation.” Not operation, but thing.
Belle: “I can’t be the one to tell you: hey, your veins are tiny or hey, your veins are so scarred or hey this or hey that.” When I’ve had several phlebotomists tell me, “Your right arm is extremely scarred and if we keep continuing to draw blood here every three months, that vein could end up getting so scarred that it closes. And your left arm sometimes shows up and sometimes doesn’t.”
Brianne: Yeah. Yeah. I can’t get blood drawn on my right arm. It does not happen. This is an ick thing to think about.
Belle: So he’s like, “Okay, based on that information, depending upon how many times you have to have iron infusions and blood draws and all of that. That might be something you talk to your team about.” I have to have blood draws every three months. I have to have iron infusions every three months. Probably a good idea. So I’m not getting stuck four times a year or more. But I want to see the allergist first because if I’m having a mast cell issue then that contemplates allergy shots and IVIG and at-home IVs and stuff like that if I’m having massive flare-ups. So I’m going to wait until I know more from that aspect and go from there. But like all of my friends have said, “You need a port because you have crap for veins.”
Brianne: I mean it’s a hard thing. It’s invasive. It’s like obviously you know what the benefits are, but it’s like anything that’s medical, it’s not like there aren’t any reasons to be…
Brianne: Yeah. It’s not like there aren’t any cons. Which I think is what’s so hard about managing care, is that anything that feels low risk that may or may not help, even using a nebulizer, you’re like, “Okay, well it’s kind of annoying but it’s always outside my body so at the end of the day, why not do it? Or why not do it four times?” But as soon as things become invasive, it’s easy for somebody outside to say like, “Oh, of course you would do this!” But like, it is a new hole in your body and it’s okay to be like, “This is something that I want to give some time to think about.”
Belle: For me, it’s not me saying no. It’s the doctors going, “Well you have terrific lung function, why risk it?” Well, because if my lung function is so terrific and I go under anesthesia, which I have been under many times before and not had an issue. Like, yeah, I’ve got mucus growing now and anesthesia becomes a risk, but anesthesia is going to be at risk whether you’re healthy or you’re chronically ill.
Belle: Unless you’re 70 years old, is going to always be a risk. I’m 25. I’m willing to take that risk so I don’t have issues with my veins later in life.
Brianne: Like forever.
Belle: Whereas I could get a port, it can be replaced every 15 years or whatever. Until I don’t need it anymore.
Brianne: So that’s pretty much the present, it sounds like. And you’re in the middle of it all, because that’s the chronic part of chronic illness. Just feeling in the middle. So next you’re going to the allergist.
Belle: Yeah. Applying for disability is difficult.
Brianne: Yeah. Oh, I was going to say, because you mentioned a while ago when you stopped working and then have you been not working the whole time? Have you tried to work? What has that been like?
Belle: I’ve been not working since 2016. My last job was as a CNA at a nursing home, which obviously I can’t do that job now because a chronically ill person around sick people is not quite a good thing.
Brianne: Yeah, there can be some like immunocompromising problems. I don’t know if those words go together well. Yeah, that’s fair. Yeah, disability is kind of a very difficult thing.
Belle: And because I can’t use my degree or anything and I have like $15,000 worth of student loans.
Brianne: That’s another fun part.
Belle: Our refund, like taxes, because I’m delinquent because I haven’t worked in two years, they took our refund check. So then you have to go through the process of filing… my husband and I weren’t married. So now we have to go through all of that to try and get our taxes back to try and pay medical bills with. When you’re chronically ill, everything gets messed up.
Brianne: Yeah. It’s like you need the cognitive function to sort through all the paperwork and then you need the money to handle what’s in the paperwork. A lot of stuff can stack up very quickly. And yeah, it’s hard. I haven’t done my taxes yet this year, that’s for sure. And I’ll note we’re recording in March because this probably will come out like after tax day. Or not March, it’s still February. We’re recording in February! Well we just covered a whole lot. Thank you so much for sharing, and for your baby girl for her patience while we’ve been talking.
Belle: Thanking amazon prime for having Creative Galaxy. It’s like #NotSponsored.
Brianne: Yeah, totally. Just a casual endorsement. Is there anything that we missed that you can think of? I know you did a bit of a sum up too, in terms of what advice you’d give.
Belle: Not really that I can think of. Well, I will throw this out there. If you want to support cystic fibrosis research, join a great strides walk near you! Go to cff.org/GreatStrides and find a walk near you! You can join my national team, look for Chronically Belle in the national team name and that is me!
Brianne: Cool! Well, thank you!
Thank you for listening to episode 24 of No End In Sight! You can find Belle on instagram @chronicallybelle, you can find this show on instagram @no.end.in.sight.pod, and you can find me on both instagram and twitter @bennessb.
In the next episode I’ll be talking to a woman about her experiences with cfs/me and mold avoidance.
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As usual, don’t forget that I have a small Facebook Group called Chronic Hustlers for people living with chronic conditions who are self employed. Now that I’m trying to get back on a more regular work-type schedule, I’m hoping to get this group a little more active.
This podcast is supported by my cross stitch company, Digital Artisanal. When I’m up for it, I make simple modern patterns that you’ll actually want to hang in your home. I love to cross stitch as a way to feel productive during flares when I’m stranded in front of the television. One of these days I’m going to get to work on some spring and summer patterns. I’d love it if you checked us out at digitalartisanal.com.