A question for the Sickle Cell Disease Community

Take a moment to describe how you or your loved one currently manage this condition, including any medical treatments. How did you or your loved one and doctor decide on this approach?

Answers from the Community

The biggest thing that I do is take a prescription called Hydroxyurea. I’ve been on this consistently for 16 years. Me, my family, and my care team decided that it was necessary because it was at the time, the only FDA approved drug that was available to treat sickle cell disease. And I’ve continued and plan on continuing to stay on this drug. It’s done wonders for me and yeah.

I think I manage my condition very well. I manage it by getting a lot of rest, drinking plenty of fluids, eating healthy the best I can and not stressing myself out. I decided on this approach by just years and years of just trial and error. Back in my college days, I would end up in the hospital once a month. I didn’t want to live that life. I figured out something that works for me.

Currently, I’m managing my condition by taking a couple of medications for sickle cell. I take hydroxyurea, folic acid, Xarelto. I do also take pain medications, but not daily. And my doctor and I decided… We worked together and I’m familiar with outpatient. I usually wait. I usually hold myself, if I’m in pain, I usually try to wait until the clinic is open because the ER makes… They have a long wait time, especially for people with sickle cell. But I do love to take a lot of natural medications. I love my tens machine, I love Bengay, I love muscle creams. I love doing natural remedies like essential oil, deep breathing exercises, massages, heating pads because I don’t like what pain medicine do to my body. They make me tired and they make you have mood swings and it’s not your personality. This medicine is changing your body. So I prefer to do natural remedies because the side effects are overwhelming and they keep you away from having any type of social life. You’re sleeping all the time. You’re just not… It’s a helpful thing, but then over time, your body gets so used to it that you have to take more. What’s the word? Shoot. It’s a word where your body gets so use to a medication. And I prefer it as a last resort. But that’s how I take care of it.

So as per managing this condition, it’s a very tricky situation. And I did have a recent doctor visit with my physician and his primary recommendation was to stay away and definitely be in quarantine as much as I can, try to avoid any social contacting or anything that would involve contracting new diseases or viruses because that would surely be detrimental to my health. The way I’ve coped with this, is just again, taking the medications, taking regular sleeps and naps. Not really involving myself in stressful matters. Stress is another contributor into the demise of my health and poor health for me. So sickle cell is a very tricky situation as I stated before. So it’s just about how you take care of yourself and what you do in that aspect. But I have made sure to drink plenty of fluids. It’s so imperative to do that. Definitely want to replenish yourselves and make sure you’re staying hydrated. So once I just understood how sickle cell works, I’ve always maintained a strict regimen as to my diet, my habits, everything concerning in my life.

We manage the condition of my loved one’s health by making sure that she takes her medication as scheduled, and by making sure that she is well rested and well hydrated at all times, in so much as possible. These are the things that have been suggested by her doctor and by other people that we’ve met along the way who know about the condition and have advised on the things that most helped to keep a person with her condition in optimum health.

One I see a hematologist, and that doctor manages my blood levels. They also have me on Oxbryta, which is a new sickle cell medication that I’ve taken. So just pretty much lab work monitoring my hemoglobin level. If I do go into a crisis and I can’t manage the pain at home, then I usually go to the ER. It gets severely low, then usually they admit me and give me fluids, pain medication, sometimes blood transfusions, but that’s like worst case scenario. I also see a pain management specialist who has me on pain medication, and they manage that and how much and often I take it. So that’s what I do to cope or manage it somewhat.

I manage my condition by basically taking hydroxyurea and folic acid and pain meds when in need. I mean at the start of the year, when one of the new drugs was out I started to take it, as it would probably help me reduce my episodes that I was having, but somehow I think I got a side effect. I started getting chronic priapism and I ended up going to the ER almost every weekend. Almost every day I would wake up with priapism, so I’m now only taking hydroxyurea and folic acid. How did my doctor decide? Okay. Hydroxyurea, because it was the only drug that’s been really available and very accessible. I told my doctor about it when the [new] drug was really out, and I was able to qualify to get it, and they did a waiver, but my doctor also thought of Endari. But then Endari, their access program for Endari, I was not qualifying, and it was really expensive, so I was not able to be able to get the Endari. That’s how I am trying to manage this condition.

I usually manage my pain with medication. I use long-lasting methadone for pain and I use oxycodone for short-lasting. Me and my wife, she usually just asked me to use my medicine on time, or I use study medication also or whatever I need to do, she’ll ask me to do it. She tries to keep up with my appointment. I try not to have her go to every appointment, but what I do is I have the doctor talk to her or I’ll personally call her at my appointment and tell her what’s going on and I keep her informed. And she just makes sure she knows all my medication, she knows where it’s coming from, and she knows how much dosage I use on a regular. And she tries to take it easy with me. She doesn’t let me do too much. Most of the man stuff in the house I try to do, but when she sees that it’s becoming too much, she had me take it easy.

I have been diagnosed with sickle cell disease, SS. My doctors have diagnosed me since I’ve been six years old. I’m currently having issues maintaining my hemoglobin, where I am getting transfusions almost on a monthly basis. I get two units of blood, which has caused iron overload. The iron overload has caused me to get a port so I could get exchange transfusions where the iron levels will not be as affected. I’ve had a stroke in November because of my sickle cell disease, and I’ve been in a pain crisis, severe pain crisis, from my stroke, I’m now suffering from seizures. I’m on seizure medication. I’m on high blood pressure medication. I am on thyroid medication, cholesterol medication, as well as currently taking Oxbryta for my sickle cell disease.

My doctor and I came to the agreement that if I’m tired or feel dehydrated that I can get fluids inside the doctor’s office. Other ways I manage this is taking my medicine every day and eating better.

The major ways that I treat sickle cell disease is that I have to take pain medications, which are narcotic. So they’re pretty strong, because I get pains in my joints of my knees, my lower back. They say that the disease is called pleiotropic, meaning it affects a lot of different areas of the body. Like I mentioned, your lungs can be affected, so my breathing. I have to take oxygen at night and sometimes even during the day. I can have sinus infections. I’ve had several, and my ear is hurting right now, actually. So it arises in different treatments that you may need compared to other people who may have same illness but different conditions. Some things that I’ve had to go through a lot is acute pain crises, where I have to go to the hospital and get actually more medicines, IV, or fluids because I’m dehydrated or going through a sickling crisis. And to combat that symptom a lot, I have had to receive blood transfusion since I was about age, like a child, probably eight or nine, or maybe even six. But at a very young age, I started receiving what they say chronic transfusions. The reason I had to be chronically transfused is to lower the amount of sickle cells in the body, because when I had sickle cells at a certain level, I actually had a stroke, and if you have too many sickle cells in your body, one of the common symptoms is pain, but also you can have strokes. You can have cardiac arrest. You can have what they call chest syndrome, where your chest becomes hard to breathe and you have a fever. So I have received blood transfusions, and my parents agreed to that. Some people with sickle cell disease, their family is against receiving blood products because you can get illnesses or transmit illness through transfusions. I received hepatitis C in, I believe, 1989 or 1990, I think. I have been treated for hepatitis C, but that also was a concern with getting transfusions. You can get exposure to HIV and other serious conditions. So far, I have only had the hepatitis C, and I believe now they have a test for that. They weren’t testing for hepatitis C back in 1990, when I contracted it through blood. Another thing that I use to treat myself, or the physicians have treated me with, is physical therapy. Because of bouts of fatigue and sickle crises, I’m often in the hospitals, even up to three or four times a year, or three or four times in one month, or every … I would say maybe two or three time every six months. There have been periods when I had successful health and stayed out of the hospital. But as I’ve gotten older, specifically, you see that a lot with sickle cell patients, they don’t have as much support as they did when they were in the pediatric care. Programs seem to be more robust, and that means that there’s more nurses, social workers, just more eyes are on you as a child. And then when you go into adult care, because sickle cell patients are living longer, which is a good thing, but they haven’t had the same robust care that they did as pediatric. So many patients, including myself, stay with a pediatric hematologist up until age 26, which is pretty uncommon, but they are in a positive, usually stable, treatment plan at that point. So switching can often arise issues. I know when I switched to adult care, my physician, for a while they didn’t want to do the transfusions the way that I was getting them. I get exchange transfusions, which required me to have a port in my chest to receive medicines. So that is in itself its own condition, because I ended up having to have it taken out because I got infections in the port and blood clots and things like that, which you are at risk for when you have a in-dwelling catheter in your body. The reason that was done is because I’ve had so many transfusions and in my arms, the veins aren’t as strong, they get sclerotic. And just over time, being poked and being punctured so much in the same area can cause scar tissue or it does. I have a lot of scar tissue. So it’s hard for them to get into a vein. Especially when you are dehydrated or feeling like you’re having a sickle crisis, your body, at least mine, you have hard time to get access to veins, because you’re in a dehydrated state and you don’t have a lot of fluid in your blood cells or blood vessels. So that can be a difficult thing. So a lot of patients do have ports, but right now I have to get a line placed every time I go to the hospital for transfusions, which is an extra step. And it is costly having to have that many doctor’s appointments. Insurance does cover some things, but not all. It is also physically taxing on the body.

Currently, I manage my condition with taking daily prescription medications. I was previously on Hydroxyurea, which is the medical treatment for sickle cell disease. We decided on this approach just based off of how often I was being admitted and how often I was having pain crisis and whether or not I could actually benefit from this medication. So that was something I considered, but then later figured out that it was not for me because my platelet count dropped really low and I had to be admitted to ICU. So that was a way of deciding that, that did not work for me, and I just went back to my normal what I was doing beforehand, which was taking my Gabapentin for neuropathy and my folic acid I take daily for my sickle cell, and then I have my pain medicine that controls most of it as well.

My daughter’s medical team actually manages her by me having to administer her routine medication twice a day. She takes penicillin twice a day. We were taking some other medications, but now we have actually reduced that type of medication, which actually included folic acid as well. Our doctors actually decided this based on my daughter’s condition and her reaction to the actual medication itself.

To manage my sickle cell, I drink a lot of water. Every day, I have to drink a lot of water. I have started walking every day for my joints. I’m developing AVN in my left knee. So, to kind of hold off from having surgery or a knee replacement, I have started to exercise. I do get pain from exercising, but I think that would be better than a total knee replacement. I try to stay active so my hips don’t get AVN. I’m trying to eat better, more fruits and veggies in my diet. Try to take different vitamins. Vitamins that people with sickle cell become deficient in.

Currently there are not too many options for sickle cell patients as far as medicine so my hematologist has me under the care of just pain management pills. I will be transitioning into the new medicine that just came out last year for sickle cell patients through a fusion. I believe it’s called Adakveo. I will be starting those infusions, but it’s been delayed because of COVID. Our hope is that in the next couple of months I’ll start the infusions to help with my sickle cell.