Management of SCD: Educating Patients on Symptoms and Lifestyle Modification


Wally Smith, MD, and Payal Desai, MD, spearhead a discussion on patient education surrounding sickle cell disease symptomology and lifestyle modifications.


Ifeyinwa Osunkwo, MD: I’m going to segue into treatment. Dr Smith, you mentioned pain as people get older. How often do people with sickle cell disease require the emergency department? Please talk about the mortality rates. When do we find this mortality? What age ranges are higher risk? Lastly, what would you tell a new patient who’s 30 to 40 years old coming to your practice in terms of what you worry about and how they should approach their condition?

Wally R. Smith, MD: [Those are] good questions. Let’s start by talking about pain. When you say something to an uninformed person about sickle cell disease, often the first thing you hear is pain. Even though pain is the most common reason for presentation to an emergency department, it’s certainly not the only important symptom. Fatigue is a very important symptom. This is, after all, an anemia. Your blood counts are low. People feel badly most of the time, even though they don’t go to the hospital. Only about 12% to 13% of days do people spend in emergency departments or getting treatment for their pain other than what they do at home.

Most pain is treated at home, and most people are dragging around their house and tired. When they come to the clinic, the questions I get are, “What do I do for my everyday pain? What do I do for this fatigue? Do you have a vitamin I can take, Dr Smith? Do you have something that will give me some pep? And by the way, I’m not gaining weight like I’d like to. Do you have anything that will help me gain weight?” We still don’t have good answers to these questions. This is a disease that causes folate deficiency, vitamin D deficiency, renal tubular disorders, pituitary dysfunction. Patients look thin, they often look younger than their stated age, they don’t mature sexually as fast as you wish, and they want to gain weight and gain a mate. They’re often interested in, “What can I do to attract a mate?” It’s tough. They may have a little education and a lot of ambition, but they’re not popular at parties. These are adults that want to know: What can I do to be normal? When they come to me, we try to deal with some of those things as a team.

You asked the tough issue about mortality. Yes, we do have to have patients who face the possibility of mortality, but what we say to them is, “We have things to help you with that. We have disease modifiers that can help you with that. We have little things that you can do to help yourself, such as drinking water.” Why is water so important, Dr Smith? One reason it’s so important is because you can’t hold on to your urine. You pee all the time whether you want to or not because your kidney can’t stop you. You must replace that water. If you don’t and you get dehydrated, you’re more likely to sickle. Patients have come back and said to me that I was right. They started drinking that water started to feel better. How much water? People talk about 96 to 128 oz. That’s a lot of water, but I count anything that has water in it. I get people to try to hydrate themselves as much as they can and to avoid extremes of temperature.

Of course, extremely hot environments and extremely cold environments can cause crisis. We used to say this without much scientific backing, but now we can say with scientific backing to avoid stress. Stressful situations indeed lead to people having pain and sometimes come to the hospital. Whether or not it’s for a vaso-occlusive crisis, they’re coming to the hospital. Maybe they don’t drink as much when they’re worried. I don’t know. I can’t explain it completely. I just know they need to avoid it, so I tell them, “You need to find out how to chill.” Just those few things, much less hydroxyurea, and all the disease-modifying agents can lead a much healthier life. It’s your job to get those messages through to your patient.

Ifeyinwa Osunkwo, MD: One thing we try not to do is to say you’re going to live to be 45 years old because we have a lot of patients who were told you’re going to die before your 21st birthday. Now they’re 45 years old, so that’s it. They’ve been waiting to die, and they haven’t died yet. Dr Desai, can you touch on how do you treat these patients? What’s your strategy for ensuring that they stay as healthy as possible in terms of preventing acute complications? What’s your recommendation for them in terms of what they need to do every day, other than drinking water, to make sure we can give them the best quality of life?

Payal Desai, MD: I 100% agree with you, Dr Osunkwo. Putting that number out there leads to a fatalism, which usually leads to some very poor life choices, including why should I do this? Why should I train? Why should I educate? If I’m going to die, I’m going to live my life fullest—and sometimes in not the healthiest of ways, which can lead to some poor choices. I too don’t put a number on there because all these people that are in their 40s were all told they were going to die before they became adults. Now they’re here and living their life: they’re having children, they’re significant contributors to family and to society, they’re doing all these wonderful things. We need to have them be able to seek that value in their life.

How do I focus on helping them achieve their goals? What is their goal? What do they want to accomplish? We start that conversation with, let me bring you to where you want to be. My goal is to help you live your life and on the adult side, I particularly try to emphasize autonomy in terms of what are your goals. If your goal is reproductive health, let’s talk about that and discuss how some of the therapies will affect or not affect your reproductive health. Let’s talk about the things you can do that we know from data. When is the best time to plan your life? When is the best time to approach things? Afterward, we start talking about those potential complications. Picking a partner. What things do you need to think about? My job is not to tell you whom to pick; my job is let us do some reproductive counseling.

Let’s talk about if your partner has gotten tested. Do they have other traits? What have they’d looked for? I approach it from a standpoint of getting you to your goals, and then we talk about where different pieces can fit in. We talk about disease-modifying therapies, but we also talk about other things, such as sleep. How’s your sleep quality? Are your oxygen levels dropping at night? Do you have sleep apnea? What are you doing in terms of putting your phone away so you can get sleep and rest? Let’s talk about birth control and the risk of clotting with certain birth control pills. What should you be doing? What are the therapies known to reduce pain crisis? When some of our female patients have their menstrual cycle, they have painful episodes. If you can break that cycle for them, if you can talk about things like Depo-Provera or other therapies that might help them, then you can avoid some complications.

Let’s talk about what the impacts are on their life. If they’re trying to go to school, if they’re trying to do things, what are the ways we can try to support you even if you’re having a pain episode? Can we try to optimize your management at home? Can we try to optimize your management even in outpatient infusion, so you hopefully don’t interrupt your studies with hospitalizations if we can try to avoid it? Those are some of the strategies we talk about. We focus on their goal. To get to their goals, I throw in some health maintenance. Let’s check your kidneys, let’s make sure we get your eyes checked, let’s try to prevent complications. We’re not going to shift from the mortality to the next phase unless we start addressing organ complications earlier and targeting them with therapies. We need to come at it on the on the patient’s terms and what they hope to accomplish.

Transcript edited for clarity.

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