The Paid Leave Podcast

Living with Sickle Cell Disease and Celebrating Motherhood

• The Connecticut Paid Leave Authority • Season 3 • Episode 46

Share episode

In this episode of The Paid Leave Podcast, we are focusing on Sickle Cell Disease (SCD) September is National Sickle Cell Awareness Month. Sickle cell disease affects about 100,000 people in the United States; more than 90% are non-Hispanic Black or African American. Many people with Sickle Cell Disease do not receive the recommended healthcare screenings and treatments mainly because of social barriers. 

My guests are Dr. Genice Nelson. Dr.  Nelson is the Nursing Director at UConn Health-at the New England Sickle Cell Institute, Hemophilia Treatment Center, and Outpatient Behavioral Health Programs.

Daisha Dillon is a mom who was diagnosed at birth with sickle cell disease and has been suffering since she was 6 months old. She always wanted to be a mom and has a healthy daughter who was born without Sickle cell disease. Daisha is seen at the New England Sickle cell Institute and gets her treatments for sickle cell and pain there at UConn Health. We discussed what sickle cell disease is and how it effects the body. Dr. Nelson is aware of CT Paid Leave, and the benefits it has for the patients she treats and their caregivers. Daisha's health has been improving with the newer medications that have become available to combat the symptoms and the pain. 

To get more information about the New England Sickle Cell Institute: New England Sickle Cell Institute | UConn Health

For more information about Dr. Genice Nelson: Genice Nelson, DNP, MSN, APRN, ANP-BC | UConn Health


For more information or to apply for benefits: ctpaidleave.org


https://ctpaidleave.org/s/?language=en_US



https://www.facebook.com/CTPaidLeave



https://www.instagram.com/ctpaidleave/



https://twitter.com/CTPaidLeave



https://www.youtube.com/results?search_query=ct+paid+leave

Nancy Barrow:

Hello Connecticut, and welcome to The Paid Leave Podcast. The title basically says it all. I'm Nancy Barrow, and I will be delving into this new state program and how it can help you and your family. This podcast will give you information you should know about Connecticut Paid Leave and maybe just a little bit more. Connecticut Paid Leave brings peace of mind to your home, family and workplace. Welcome to The Paid Leave Podcast. September is National Sickle Cell Awareness Month. And sickle cell disease affects about 100,000 people in the United States, and more than 90% are non Hispanic, Black or African American, and an estimated 3.3% to 9% are Hispanic or Latino. And many people with sickle cell disease don't receive the recommended health care screenings and treatments, mainly because of social barriers. And my guests will have lots to say about sickle cell disease. Dr. Genice Nelson is the nursing director at UConn Health at the New England Sickle Cell Institute, the Hemophilia Treatment Center, and Outpatient Behavioral Health Programs. And Daisha Dillon is a mom who was diagnosed at birth with sickle cell disease and has been suffering since she was six months old. She always wanted to be a mom and has a healthy daughter who was born without sickle cell disease. And welcome to The Paid Leave Podcast. I'm so happy to have both of you here.

Genice Nelson:

Thank you for having us.

Nancy Barrow:

Let's talk about this. Dr Nelson, tell me what sickle cell disease is.

Genice Nelson:

So sickle cell disease is an inherited genetic disorder that affects the red blood cells, and so it's important for people to understand that it's not something that you can catch, nor is it something that you can develop. Should you have something that's called sickle cell trait. So if you are not born with it, then you cannot have sickle cell disease. But it is one of several different genotypes of a red blood cell disorder that can cause differences in pain, anemia, and just really affect people's lives in a chronic way.

Nancy Barrow:

And there's no cure for sickle cell is that right? Just treatments?

Genice Nelson:

There is a cure for sickle cell disease. It is not universally available, so a bone marrow treatment from a matched donor can cure sickle cell disease. It is a very long proliferative process. Is not an easy process, but there is potentially a cure through the bone marrow transplant. Again, it's not widely available, because you have to have an identical match for the individual. So ideally they have same mother, same father, and then you match them genetically. It's a process that you go through. Then you have to treat the individual who has sickle cell disease, because they have to literally go through a process where you kill off their own bone marrow, because their bone marrow is only producing sickled red blood cells. And once you do that, then you're able to collect the donor, the bone marrow donor, from the sibling that's going to donate, and then they're able, you know, through medicines and different processes, infuse that into the patient. Once that graphs to the individual, then they can overcome their sickle cell disease. They'll have what's literally known as sickle cell trait because their underlying bone marrow disease is still there.

Nancy Barrow:

Wow, that is so fascinating. I didn't realize that there was a cure for that. And is it cost prohibitive? Is it covered under insurance?

Genice Nelson:

There are extreme costs associated with it. But the problem, again, is finding those matches because you made the same mother, same father, but the differences in genetic material is what limits people from being able to have a bone marrow transplant. There are sometimes you're able to find an anonymous donor that may genetically match you, but you have to have a genetic match in order to do a bone marrow transplant. There are some studies where they did half genetic matches, and it hasn't been as effective. But if you're able to be one of those individuals, and there are quite a few individuals who have successfully had bone marrow transplants, we ourselves at New England Sickle Cell Institute have had two patients who have successfully had a bone marrow transplant. So it is possible for it to happen.

Nancy Barrow:

For me, the only person I knew growing up was T Boz, right? So I knew from TLC, she had sickle cell, and she used to talk about how painful it was and just how debilitating it was, and that there was no cure. So I always felt like, oh gosh, there's no cure for her, and I was just devastated for her.

Genice Nelson:

She visited the state of Connecticut a couple of years ago through a concert, and through my association with the National Sickle Cell Disease organization, they put me in touch with her. She actually ended up having a medical event, and I stayed with her the entire night to help her get through. Medical event. And when I tell you she is one of the sweetest, kindest individuals you could ever possibly meet, she is definitely that person, a staunch advocate, had an ability to interact with her, to actually give her some teaching, for some things that she could do for herself, given the lifestyle that she has being in entertainment, and hopefully she's taken some of those things to heart and has incorporated them to help her have a better, less painful lifestyle as well.

Nancy Barrow:

Wow. How fascinating is that? What do you do at the New England Sickle Cell Institute at UConn Health? What do you do there as the Nursing Director?

Genice Nelson:

So as the nursing director, I oversee our clinical and programmatic operations. What it means is that I'm here to help ensure that our patients are getting high quality care, that they're engaged in care, that we're doing the things that we should do for every individual with sickle cell disease. So an individual with sickle cell disease, there are certain screening examinations that they should have so that we can check their heart, their lungs, their eyes, depending on the different kind of sickle cell disease that they have, we're plugging them in into primary care, because as people live to be older, all of the things that plague the general patient population can also plague individuals who have sickle cell disease. So at a certain point, they have to be screened for breast cancer, they need to be having prostate exams, they need to have colonoscopies, all of the things that the general patient population have. And then we really focus on trying to manage their individual sickle cell disease and the different things that they have. So you have individuals who may have a history of stroke, and so there's an indication for a person who has had a stroke, which means that they need to be on chronic blood transfusions. So we manage those chronic blood transfusions. We may have somebody who's pregnant, and Daisha can share her story with you how we help to manage her pregnancy, but we just try to help ensure that our patients get, high quality care, that we help them to have as less symptoms as possible and just really be engaged in their families and their home lives.

Nancy Barrow:

Yeah, and how do you diagnose it? Is it a simple blood test?

Genice Nelson:

So it is a simple blood test an individual who has sickle cell disease should have what's called a hemoglobin electrophoresis test. Yeah, it's a mouthful. It's a mouthful. What it does is it identifies the different hemoglobins that individuals have. So an individual who does not have sickle cell disease has hemoglobin AA, because you inherit one gene from your mom, one gene from your dad. So an individual who has sickle cell disease, they have two different types of or they may have the same type, SS or SC. There's a bunch of different types of sickle cell disease, but it teases out which kind of hemoglobin they have. And so that's how you determine if someone has sickle cell disease or sickle cell trait. And once that individual is identified, then through the state of Connecticut, we notify through newborn screening process, the parents that their child has sickle cell disease because they need to be engaged in care sooner rather than later.

Nancy Barrow:

And do we know why that black and brown people are affected so much more with this disease.

Genice Nelson:

It depends on where you are globally as to who's affected by sickle cell disease. Because sickle cell disease, I know people think that is a black and brown disease, but it affects everyone. So we have in our own program people who are Caucasian who have sickle cell disease as well. So I need people to understand that. So, depending on where you are, globally, depends on what sickle cell disease looks like. Think about sickle cell disease as a natural genetic mutation that occurred as protection against malaria. And so those malaria belts, you know, Africa, Turkey, India, those are the countries that were malaria was prevalent, as sickle cell trait is protective from people dying from malaria, sickle cell disease itself is not and so that's how sickle cell disease was propagated, as sickle cell trait was protective from malaria.

Nancy Barrow:

Let me ask about the treatments, because I think that that that's a loaded question, right? Because I think depending on what you can do, there are different treatments, right? Like, is it normal to get a blood transfusion and IV pain medications, or is gene therapy coming about and stem cell therapies?

Genice Nelson:

So let's take it one step at a time, so an individual who has sickle cell disease may or may not need a blood transfusion. It depends on what their symptoms in or medical problems are. So if you have a person who, like I said, who has had a stroke before, the protocol or treatment for that individual is to prevent them from having another stroke. So how do you prevent them from having another stroke? You. Want to suppress as much as possible the ability of their blood to sickle so you decrease their sickle blood by giving them non sickled blood, and so that will hopefully prevent them from having another stroke. So for an individual who may have a medical emergency called acute chest syndrome, where is like having pneumonia, except it's sickled cells that are clogging their lungs there that's a medical emergency, which may require a full body blood transfusion, and so, depending on what issues an individual has may or may not require them to need blood for a woman who's pregnant, who sometimes their baby may not be developing enough, because, again, that individual has sickled blood and non sickle blood is more beneficial because it can carry more oxygen and has more hemoglobin. You may need to give them blood transfusions to support their pregnancy, the other medications that are available. So for instance, Cascavey and Lefjevy those are the two stem cell or newer therapies that are available. So they are very, very expensive, on average, about three to $5 million but if you think about what this can do, this, these treatments take individual stem cells and they alter their stem cells so that they no longer have sickle cell disease, and then they re-infuse it into the patient. Now I know I'm making it sound very, very simple, but that patient has to go through a deconditioning process, because, again, their body is making sickle cells, so you have to kill off their body's ability to make those sickle cells, which means they may have a very prolonged hospitalization, they may get very weak, they could possibly get sick. You have to keep them in a protected environment, because they're going through this conditioning with some chemotherapeutic medications to prepare their body for these transformed cells that will now hopefully take place and replace their sickled cells. So not necessarily easy decisions for individuals to make, but they can be life changing decisions once individuals have experienced this, and depending on your providers, we don't offer that right now, yeah, but we are process of partnering with our bone marrow treatment program that is in the process for accreditation. So we're looking to next year, be able to possibly start offering these therapeutics to our patients as well, which we're extremely excited about, because these are life changing events and to be able to affect someone's disease to the point where they're no longer hospitalized, they're no longer in pain, they no longer need blood transfusions. It's an amazing opportunity for them to have a different scope and perspective on their life, because now they can go and live as normally as you or I, without the complications that they're currently facing.

Nancy Barrow:

Daisha, why don't you tell me about your journey with sickle cell disease?

Daisha Dillon:

Well, it's been an up and down journey, as you know, I was diagnosed at six months, so that's when I had my first crisis, as my mom says, and I've just been living with it till 35 which I am now, good days, bad days and in between.

Nancy Barrow:

Yeah. And so like a typical day for you, do you have to get hospitalized? Do you get blood transfusions?

Daisha Dillon:

All of the above? So it was a point where I was getting blood transfusions once a month. I no longer get those. Thank you, Jesus. When I do get a monthly medication, I believe the name is Adapvio, that decreases my crises, and I am on my chronic pain medication to help me, well, I'll be able to live as normal as possible. Be able to go to go to work, go on vacation.

Nancy Barrow:

And that's amazing, right? So if you have this medication and you don't need blood transfusions, that's life altering. And so doctor, these are all things, yeah, these are all things that you as a team work on with Daisha.

Genice Nelson:

Absolutely! So the medication that dish is talking about, Adaptvio the generic name is Chrisamizalab. Is a medication that is infused, meaning that it is given through an IV once a month. This medication works kind of like and I when I explained it to a patient, I explained it to think of your body as being a car, and you need oil to protect your engine. So in in this person who's now the car, the oil, or the Adaptvio, encompasses the red blood cells so that they don't stick together. They don't stick together. Now they're able to flow freely, and that helps to prevent the pain from happening. So the medication, by selection, or P selectin specific prevents the cells from sticking together and sticking to the tissue lining, which prevents the cells from clumping together and causing pain, causing pain. So that's why I use that a car and oil, yeah, because that's really a good visual for how it kind of works,

Daisha Dillon:

And it works!

Nancy Barrow:

And it works, and so are you. Are you? Is your pain a lot less than it used to be Daisha?

Daisha Dillon:

100% I have a job. It was a point where I didn't have a job before. It was a point where I was going to the sickle cell clinic maybe twice, three times a week to get a new pain medication. I probably go there once a month, if that, for pain medication.

Nancy Barrow:

That's like altering and being able to have a job is is crucial, right, especially since you're a mom. So tell me about that journey of becoming a mom, because it's complicated with sickle cell.

Daisha Dillon:

Definitely, just because when you have sickle cell, one of the things that they tell you is that, not that you can't have kids, but that would be very, very difficult for you to have kids, that it was something that you can die from, something that you shouldn't do. It's something that would be life altering. So it was one of those things that when I got pregnant, I was like, Oh, what do I do now? And I was very, very nervous, and it was so when I talked to Janice, and I think that what she told me was, you're not the first person to have sickle cell have a baby, and you're not going to be the last. And that just comforted me. And she created a whole treatment plan so that I could have a healthy baby girl, and I did!

Nancy Barrow:

And that's amazing. So did you have to go off medication when you were pregnant? And did that change the pain that you were in, or did it change anything?

Daisha Dillon:

Yes, and no, I think we added medication and I didn't have to go off on my pain medication, because they were able to find a way for me to take it so that my daughter didn't get addicted to medication. If that makes sense, gotcha. Gotcha as a narcotic. And when she was born, they looked after her to make sure she didn't have any withdrawal symptoms, and she didn't.

Nancy Barrow:

So that's is that a concern for you doctor, when there's a sickle cell patient that is pregnant that she may pass that gene on to the baby.

Genice Nelson:

If you have an individual who has sickle cell disease, as a mom, she is definitely going to pass on one half of her gene set. So if she has SS disease, she can only pass on an S if she has sc disease, then she could pass on an S or a C. So it depends on what kind of sickle cell disease the individual has, but they're going to pass on what it is that they have genetically, not necessarily concerned about if mom has sickle cell disease. What we what we tend to focus on, is testing the dad. What does dad have? So when we not sure what dad has, we say, hey, father of the baby, come on in. Let's get some blood. Let's see what you got going on so we can make a determination of whether or not the baby may possibly have sickle cell disease, because a baby has sickle cell trait that, in itself, doesn't necessarily cause health issues, but it also gives mom a peace of mind that she doesn't have to worry about baby having disease versus dad having sickle cell trait and mom having sickle cell disease, then, you know, the likelihood is very high that they could have a baby with disease, so we just focus on testing dad so we can relieve mom's fears.

Nancy Barrow:

What about clinical trials? Do you offer those for sickle cell disease at UConn Health?

Genice Nelson:

We are research Central!

Nancy Barrow:

I know you are!

Genice Nelson:

When it comes to clinical trials. We, we currently have four different clinical trials that are open right now. We, we pride ourselves in trying to ensure that we bring to our patients everything that we can bring to our patients, and in order for them to have access to newer treatments, better treatments, we have to perform clinical trials. And in order for those clinical trials to be successful, you have to have patients who have sickle cell disease. So we encourage our patients to participate. Typically, they're knocking down the doors. Hey, do you have a child that I can participate in, which is fantastic, because the only way that we're going to improve their life the care that they're receiving is through these trial and error processes. Again, we're looking to partner with our bone marrow treatment program so that we can bring the stem cell therapies to our patients, we're always looking for clinical trials that are going to benefit our patient in some way, shape, form or fashion, whether it impacts their hemoglobin, if it impacts their pain, if it impacts their disease in any way, if it's something that we feel is beneficial to our patients, our medical director. Dr berenda Maryam is a very serious advocate and component of making sure that we are viably in the research space on a daily basis, and we have a whole research team of individuals that are dedicated to our clinic specifically for research.

Nancy Barrow:

Yeah, it's amazing. Daisha, you were talking about having a job, and have you used Connecticut Paid Leave?

Daisha Dillon:

Yes, I actually used Connecticut paid leave when I had my daughter. Because when I had my daughter, I think I had her at 36 Well, I know I had her at 36 weeks, and I wasn't ready to leave work. I wasn't prepared or anything. But Connecticut paid me was actually pretty easy. All I did was contact my HR. They gave me the paperwork, and then I brought it to Teresa, which is the sickle cell social worker, and she, like, literally, filled out the paper for me, walked me through it, and it was done. Oh, it's so good. enough to worry about, like, oh my god, I'm gonna get paid, and not to worry about, like, filling out any more paperwork. Literally, I went into the hospital and had my baby girl.

Genice Nelson:

Our social worker does yeoman's work when it comes to taking, yes, patients. She is on top of every possible resource possible. And just like Daisha said, she doesn't just give them the set of paperwork and say, Here you go. Go fill it out, she is hand in hand with them to make sure that the paperwork is filled out correctly, that it is filed and that it goes through to fruition, that she knows that it's been approved and what they have access to. So I give kudos to Teresa. Works every single day, and it is absolute pleasure to work with social workers like her and Emily Baker, who are so dedicated to our patient populations.

Nancy Barrow:

Well, those women, they get gold stars from Connecticut Paid Leave for being able to help people fill out that paperwork when they can't, you know, when it's when it's you know. And the stress too, is is something that can, that exacerbate the symptoms of sickle cell?

Genice Nelson:

Absolutely, it can. Yeah, emotional stressors, physical stressors, the temperature, so when it gets very hot, when it gets very cold, things that you or I don't take a take into account and how it physically bothers us, can trigger an individual who has sickle cell disease to have a painful crisis and end up in the emergency department, or even hospitalized.

Nancy Barrow:

Oh my goodness, yeah. Has that happened to you Daisha?

Daisha Dillon:

Multiple times. Multiple times!.

Nancy Barrow:

Is it the stress that sets you off, or is it the temperature that's really interesting.

Daisha Dillon:

It could be anything. I'm going to Disney World on Saturday, and I'm nervous.

Nancy Barrow:

Did you ever think that you'd be able to go to Disney World and have your daughter with you?

Daisha Dillon:

Have my daughter with me, no! Because, like I said, they for so many years, it was like, Oh no, you shouldn't have a child. So I just, you know, okay, okay, it wasn't one of the things that, like I wanted it, but it wasn't one of those things. I was like, okay, you know, I'm trying to have a child, just because all of the doctors that told me that I shouldn't.

Nancy Barrow:

And so now this is going to be a different experience for you.

Daisha Dillon:

Definitely, and then a lot better predicament, and a lot better space of my health. That's definitely so definitely, I can say that.

Nancy Barrow:

And did you ever go to a support group? Or do you recommend that?

Daisha Dillon:

Yes, I do. I definitely recommend support groups like having friends that have sickle cell disease, because there's some things that or somebody's that you'd like. Okay, what is this? I've never felt this before, and it just feels good knowing somebody Okay, no, I've had that before. That is sickle cell. You can go to the clinic, tell them about it like it just reassures you.

Nancy Barrow:

Yeah, there's strength in numbers, right? Like so and and there's comfort in knowing that you're not alone in this.

Daisha Dillon:

Definitely, it's always good to have support too, because there's some days where you just feel defeated. You just feel defeated, and it's up to that person to say, Nope, you got this the causes, it will go away. You know, it's not gonna be here forever. You know, hold your hand. Get you through it. It's times where I don't want to go to the hospital, and because I'm I'm just tired of going, and I know that I need to go. And then I'll call a friend and like, nope, come on. We're going, we're going.

Nancy Barrow:

Yeah, it's a lot when you get this diagnosis. What about mental health Genice, what do you talk to people about? Because living with chronic pain is exhausting and it's awful, and going to hospitals is is tiring, and you don't want that. How important is it for patients and family members even to get mental health support?

Genice Nelson:

Part of our process for when our patients come in is to ask those questions, are you feeling anxious? Are you feeling nervous? Do you feel depressed? Are you having sleep disturbance? Do you have suicidal ideation? And it is okay to acknowledge that you may have to some degree any of this or none of this, our social worker does some psychotherapy with our patients. We do refer to our outpatient behavioral health psychiatric programs as well, but we do tell our patients there is help should you need it. And we try to screen for that, because we know living with a chronic disease, for one thing is not easy at all. Living with acute, chronic pain is even more difficult on top of it, then when you have a disease where you know people like days of her she shouldn't have a baby, or she could die at a young age, those things start to burden individuals, and as they get older, it can make them more anxious or nervous about what their life trajectory is. And so we try to intervene where we can and say, Hey, let's partner with this mental health group or that mental health group to see where we can we can talk through some of these things. Sometimes people need some medications because anxiety and depression is real, and we as a as a society, have really acknowledged that given covid. But we still need to make sure that services are available to people, and as people acknowledge that there are more and more psychiatric issues that are being uncovered, there are less and less services available. So we really have to be diligent to make sure that we are really serving the whole individual, not just their pain, but their mental health as well.

Nancy Barrow:

I think that's so important. And Connecticut Paid Leave also covers mental health. We tell people to always take that FMLA to get their job back. So take it together. FMLA and Connecticut Paid Leave because we do the income replacement portion. So take them together. It's really important that you know this, since you're working and you're paying into this program, Daisha, that you can take this program for many reasons, and for long periods of time if you need it shorter periods of time, intermittently or from a reduced schedule from work. There's a lot of flexibility with Connecticut Paid Leave, so make sure that you talk to that social worker and and you find out if you need anything from us.

Daisha Dillon:

I'll have to look into that. Yeah, intermittent leave.

Nancy Barrow:

How important are programs like Connecticut Paid Leave to patients and their caregivers?

Genice Nelson:

For many of them, it can be the mind relief that they need to know that if there's a medical emergency or something that takes them out of work for an extended period of time, or affects their income in any way, shape, form or fashion, they have something to back them up. And we do have the information for Connecticut Paid Leave in English and in Spanish hanging in our waiting room so that is visible for all of the patients every single time they walk in the doors.

Nancy Barrow:

What are your hopes for national sickle cell Awareness Month?

Genice Nelson:

Well, I have two hopes. One of the hopes that I have is that individuals who have sickle cell disease, as I've said, need blood. So one of the hopes that I have is as and one of the issues that individuals who have sickle cell disease who need blood is that the black community does not donate, so the blood pool is mostly Caucasian, and so there are some very tiny genetic differences in the blood that causes individuals who have sickle cell disease over time to develop antigens or allergies to blood. So if I could do anything, I would encourage the black community to come out and donate blood and earmark it specifically for sickle cell disease. And then I would just like for people to be really aware that individuals have sickle cell disease are just that. They're individuals. They're living with a chronic disease. They're not fragile. So you can still talk to them, hug them, talk to them, just treat them like normal people, instead of, oh my God, you have this, that or the other? No, they're they're individual who has a chronic illness like anybody who would have hypertension or diabetes.

Daisha Dillon:

I'm kind of going to piggyback on what Genice said, that we are normal people that just have a chronic disease. It's not Oh, my God. Oh my God, you have sickle cell disease. Um, like, No, we have to have sickle cell disease. And I'm living with it, just like anybody else will. Good days are not bad days, just like anybody else does. Awareness that sickle cell is something that you can use CT Paid Leave for.

Nancy Barrow:

That's a great answer. I like that. Well, thank you so much continued good health to you. Daisha Dillon, thank you so much for sharing your incredible story with us on The Paid Leave Podcast. And please use Connecticut Paid Leave for any part of your journey. And if I can help, just you know you get in touch with me.

Daisha Dillon:

Definitely. Thank you so much.

Nancy Barrow:

And Dr Genice Nelson, thank you so much for lending your expertise and being such a compassionate health care worker at the New England Sickle Cell Institute.

Genice Nelson:

Well, thank you so much. I really appreciate that, and I appreciate the time that you've given me to be able to talk about this for for our patients, because I think the more people who know, the better care, the better advocacy our patients can have.

Nancy Barrow:

For more information or to apply for benefits, please go to ctpaidleave.org This has been another edition of The Paid Leave Podcast. Please like and subscribe, so you'll be notified about new podcasts that become available Connecticut Paid Leave is a public act with a personal purpose. I'm Nancy Barrow, and thanks for listening.

People on this episode

Head Shot

Nancy Barrow

Host