Impact of SCD on Quality of Life

Ifeyinwa (Ify) Osunkwo, MD, MPH: One thing I’d like to point out that you said is to screen for symptoms. A lot of times patients show up to their clinic appointments, and let me know if you think this is true, we ask, “How are you doing?” They say, “I’m fine.” “Have you had any pain crises?” “Absolutely none.” They’re like, “I’m doing great.” But then they can’t walk a block, they can’t climb the stairs, they’re getting dizzy when they get out of bed too quickly, they’re lethargic all the time. They can’t do any type of sports, or any kind of physical activity, they don’t have a job. And they’re “fine” because they’re not in the hospital, but they really are not fine, they have shortness of breath, they have…on exertion, they have all the symptoms that would suggest either cardiac pulmonary or chronic anemia complications, and it slides under the radar. What are your thoughts about that? I’ll start with Pat and Wally; I want to get your thoughts.

Patrick McGann, MD, MS: Yes, in sickle cell disease the pain crises are so predominant, in your face, and this patient’s quality of life is so poor that they’re just used to it. Unless you dig in and get to it, they may not recognize that they could do things that they ought to do. It’s partly on us as health care providers to ask these important questions because it’s not about the pain at all, it’s about organ function, it’s about quality of life, it’s about doing stuff. These things often go under the radar, and when they go under the radar, it’s hard to do anything about them when pulmonary hypertension or chronic kidney disease are too far along.

Ifeyinwa (Ify) Osunkwo, MD, MPH: Absolutely, Wally what are your thoughts? This whole idea of caring for sickle cell disease being more of an early detection and prevention philosophy vs damage control and acute intervention. What are your thoughts on this?

Wally Smith, MD: We study patients to determine their health-related quality of life, Ify. And we looked at battered women, patients on dialysis, patients with asthma, and the like. And sickle cell is competing for bad quality of life with those diseases, and we published that because both in children and adults, quality of life in sickle cell disease is impaired. It’s impaired in people who don’t have a lot of crises, and it’s even more impaired in people who have a lot of crises. It is important to say to the patient, “The way you feel now may be normal for you, but it’s not normal for life.” What we’re learning, now that we have medications that can improve the hemoglobin, is patients are suddenly going, “I’ve been missing all of this, all my life?” Their quality of life is improving because they’re on medications that help them, and they didn’t realize they could do better than what they were doing. It is important to say to patients, “What’s your fatigue level?” If you run a sickle cell clinic for adults, don’t expect any patient to show up before 10 am.

Ifeyinwa (Ify) Osunkwo, MD, MPH: How true.

Wally Smith, MD: They are not getting out of bed at 8 o’clock in the morning and doing nothing for nobody, because they’re anemic.

Ifeyinwa (Ify) Osunkwo, MD, MPH: Yes. 

Wally Smith, MD: It takes them a while to get going. 

Abdullah Kutlar, MD: Can I share an anecdotal report on a patient of ours to the effect that Wally reported? This is a 56-year-old female who I have followed for over 2 decades, almost 3 decades. She was on hydroxyurea and had a great response, fetal hemoglobin around 30%. But over the years her hemoglobin declined from 8 to about 6 [g/dL]. In August we started her on voxelotor. I saw her last week. I said, “How are you doing?” She said, “I’ve never felt this good in my life.” Her hemoglobin, which was 6, was now 10 [g/dL]. To Wally’s point, we have some success stories with some of the new disease-modifying agents like that.

Ifeyinwa (Ify) Osunkwo, MD, MPH: That quality-of-life matters. They get used to being so poor with their quality of life. 

Wally Smith, MD: They grew to expect a poor quality of life and they adjust, and they use the word “fine” to describe their current quality of life, when what they really mean is, “As good as I can be in the condition that I’m in.”

Ifeyinwa (Ify) Osunkwo, MD, MPH: That is interesting.

Transcript Edited for Clarity