Ifeyinwa Osunkwo, MD: I’ll move on to treatment. Dr. Smith, you mentioned pain as people age. How often do people with sickle cell disease have to go to the emergency room? Please talk about mortality rates. When do we find this mortality? Which age groups are at higher risk? Finally, what would you say to a new patient in their 30s or 40s coming into your practice about what concerns you 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 someone uninformed about sickle cell disease, the first thing you hear is often pain. Although pain is the most common reason for presenting to the emergency room, it is certainly not the only significant symptom. Fatigue is a very important symptom. It is, after all, anemia. Your blood count is low. People feel bad most of the time, even if they don’t go to the hospital. Only about 12-13% of days people spend in emergency departments or receive treatment for their pain other than what they do at home.

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

You asked the difficult question of mortality. Yes, we have to have patients who are at risk of death, but what we tell them is, “We have things to help you with that. We have disease modifiers that can help you. We have little things you can do to help, like drinking water. Why is water so important, Dr. Smith? One of the reasons it’s so important is that you can’t hold your urine. You urinate all the time, whether you want to or not, because your kidney can’t stop you. You need to replace this water. If you don’t and you become dehydrated, you’re more likely to get sickle. Patients have come back and told me I was right. They started drinking this water to feel better. How much water? People talk about 96-128 oz. That’s a lot of water, but I count anything that has water in it. I urge people to try to stay hydrated as much as possible and avoid extreme temperatures.

Of course, both extremely hot environments and extremely cold environments can cause seizures. We used to say this without much scientific backing, but now we can say it with scientific backing to avoid the stress. Stressful situations cause people to have pain and sometimes to go to the hospital. Whether it is for a vaso-occlusive crisis or not, they come to the hospital. Maybe they drink less when they’re worried. I do not know. I can’t fully explain it. I just know they have to avoid it, so I tell them, “You have to know how to relax.” Just those few things, a lot less hydroxyurea, and all of the disease modifying agents can lead to a much healthier life. It is your job to convey these messages to your patient.

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

Payal Desai, MD: I 100% agree with you Dr Osunkwo. Putting that number out there leads to fatalism, which usually leads to very bad life choices, including why should I do this? Why should I train? Why should I educate? If I have to die, I’m going to live my life to the fullest – and sometimes in the healthiest way possible, which can lead to bad choices. I don’t put a number on that either because all these people in their 40s were all told they were going to die before they became adults. Now they are here living their lives: they have children, they make a significant contribution to the family and to society, they do all these wonderful things. We must allow them to seek this value in their lives.

How can I focus to help them achieve their goals? What is their goal? What do they want to accomplish? We start this conversation with, let me take you 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 relation to your goals. If your goal is reproductive health, let’s talk about it and discuss how some of the therapies will or will 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? Then we start talking about these potential complications. Choose a partner. What should you think about? My job is not to tell you who to choose; my job is to let us do reproductive counseling.

Let’s talk about if your partner has been tested. Do they have other traits? What did they look for? I approach it from the perspective of getting you to your goals, and then we talk about where different elements can fit together. We talk about disease-modifying therapies, but we also talk about other things, like sleep. How is the quality of your sleep? Does your oxygen level drop at night? Do you suffer from sleep apnea? What do you do to put your phone away so you can sleep and rest? Let’s talk about contraception and the risk of clotting with certain birth control pills. What should you do? What therapies are known to reduce pain attacks? When some of our 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 the impacts on their lives. If they’re trying to go to school, if they’re trying to do things, how can we try to support you even though you’re having an episode of pain? Can we try to optimize your home management? Can we try to optimize your care even in outpatient infusion, so as not to interrupt your studies with hospitalizations if we can try to avoid it? These are some of the strategies we talk about. We focus on their goal. To achieve their goals, I add a bit of health maintenance. Let’s check your kidneys, make sure to get your eyes checked, try to prevent complications. We’re not going to move from mortality to the next phase unless we start treating organic complications earlier and targeting them with therapies. We have to make it happen on the patient’s terms and what they hope to achieve.

Transcript edited for clarity.


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