Do Sickle Cell Pain Crises Increase or Decrease With Age?
More Programs Featuring Carla Lewis
In this program:
Do sickle cell disease pain crises really worsen as patients age, or are other factors at play? Dr. Marwan Shaikh, hematology nurse practitioner Maya Bloomberg, and Founder of Kids Conquering Sickle Cell Disease Foundation, Carla Lewis, share challenges that young adult SCD patients may encounter with their care and offer advice for how patients can advocate for their best care.
Transcript
Interviewer:
Dr. Shaikh, is that something that typically happens with patients with sickle cell disease? Do the crises get worse as they get older?
Dr. Marwan Shaikh:
Well, there's a number of factors, I think, that play into that, as people get older though, when they go on to college, now it happens, they're moving miles away, hundreds of miles away. They're all of a sudden taking care of their own finances, trying to figure out the car, they don't have insurance, maybe at the beginning, they don't know where to get up, set up with the school system, the health system over there, or they may be afraid.
It's a new society, the...they're a little timid about making new friends, you have all these transition issues and barriers that happen. And then what happens, people get lost to care. So someone with mild kidney dysfunction three years later presents with later stage renal disease, or the disease doesn't give them control, and so they start having more pain crises. So part of it may not be that the disease is getting worse, it is getting worse, but it's just the way that it's being managed has been dramatically changing...all that support is gone.
Maya Bloomberg, APRN:
But I think it's important to mention that sickle cell is so unique, so I have some patients who got sick all of the time growing up. And then now that they're in adulthood, they have a very smooth sail type of lifestyle, if others who barely got sick, and now all of a sudden they're getting sicker. So I think if you benefit from disease-modifying drugs like hydroxyurea (Hydrea), you have the potential to really lead a normal life, as normal as possible where your sickle isn't interfering with what you can and can't do. But as Dr. Shaikh mentioned, patients are at risk for various complications. And if you have certain complications, those complications can then trigger certain crisis and pain events as well. So I think it's hard to say just a blanket statement as you get older, you're going to have more issues, because it's not always the case, and it really is so unique and so individual.
And I think with the new advancements and treatment options, and the fact that we now have four FDA-approved, before, we've only had hydroxyurea. Now we can actually offer a combination therapy for our patients with sickle cell is definitely a step in the right direction.
We need more, or there's more research coming on and more treatments in the pipeline, which is definitely exciting. But just the fact that we're able to offer a combination therapy which we couldn't have done five years ago is definitely a step in the right direction and allowing our patients to live a better quality of life.
Carla Lewis:
And one of the things I would love to...I just mentioned, my son was actually...we speak about pain. But I have to mention that my son was diagnosed with high blood pressure at the age of 9, so the damage was occurring internally because of the disease. But not necessary, you can have those particular crises that's actually...that's triggering. There are patients on just like...there are patients that have strokes but never had a major crisis, so having those silent strokes. So we have to understand too maybe too, just because just the nature of sickle cell disease, how it's so complex. As you mentioned, we have to take an account that even from childhood until the adulthood there's damage occurring internally. And just like that was mentioned prior, that we have to be cognitive of making sure that child definitely gets the comprehensive care, but not only that, continue that. We have to continue that conversation to these young adults as they are preparing for transition and then in transition, what that comprehensive care did for you back then when mom or dad or another family member was in how that was so important. It's still important as an adult, even though you want to live that normal life, so how do those important conversations is very, definitely important because it's that stage of life that is, it's very, very unique for them. And they have to understand that more so what these tests were producing for the doctors to get information, so they can compare the progression of this disease, and they can better manage their care better that way, and definitely having those conversations about medication and keeping up with doctors’ appointments as necessary.
Maya Bloomberg, APRN:
I think this all boils down to education, and the more education you can provide to the patients, the better the outcomes. Having support and a well-educated mom and just that support is so important, because I know how much education I do for my patients.
But the reality is, is when you are an adult and you're going to get managed for sickle cell, the majority of patients are going to see a heme-onc doctor, a hematologist/oncologist that probably does 90 percent oncology. Because the reality is is that's what brings more money. Sickle cell really doesn't bring that much financial gain to an institution. So if you're connected with a heme-onc physician, chances are that doctor is really just managing your pain and the anemia, not really understanding the head-to-toe effects that sickle cell can have. So if you educate yourself and you really understand what sickle cell does, what the importance of the tests are, what the importance of my doctor's appointment, what they're going to do with these doctors’ appointments, I then feel more empowered to have meaningful conversations with their providers. Because not everybody is going to get connected to a sickle cell specialist, there's a national shortage of sickle cell specialists all over the country. So I think our job is, is just to give our patients the tools that they need to better educate themselves so they can then go to their doctor and have meaningful conversations and say, “Hey, I saw that I should be getting this urine test every year to check my for protein loss and make sure my kidneys are fine.”
Where otherwise they would have no idea, so we need to continue with the education and the awareness and just really empower the patients, because during the transition period, a lot of times is that role identity, and you don't want to have your sickle cell defined when some patients will just neglect their health and that's their coping mechanism. And then three or four years down the road, they have advanced kidney issues and all of this stuff. So I think if we can spend more time...and I think that the chapters and the different foundations and people who are doing the outreach in the community, they have a really good chance of connecting with these patients, so they can better advocate for themselves. Because as providers, we can do so much, but I think it really kind of does a lot in the patient and their support has come to make sure that they're getting the care that they truly need.
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