Adolescent to Adult Sickle Cell Care | Key Advice for Patients
More Programs and Publications Featuring Maya Bloomberg, MSN, APRN
In this program:
What should adolescent sickle cell patients know about the transition to adult care? Hematology nurse practitioner Maya Bloomberg from Sylvester Comprehensive Cancer Center explains issues that can arise during the transition and patient advice for navigating the transition.
Transcript
Deandre White:
Transition from adolescent to adulthood is often challenging, so are there specific tests and assessments to keep up with during pediatric to adult transition?
Maya Bloomberg, MSN, APRN:
Yeah, we know transition can definitely be difficult and an overwhelming time for individuals with sickle cell, because first of all, you're having a pediatric hematologist who probably diagnosed you and is taking care of you your entire life having to switch over, but it also coincides with a lot of major life changes. There is a spike in mortality during this transition period for a number of reasons, including the shortage of providers on the adult side where many will get connected to oncologists, and in addition to that complex insurance landscape in the United States where you need referrals to prove visits, so there's a lot of reasons why we see that spike. But knowing that there is that spike, we start the transition process pretty early. Some places will start it as early as 12, but most will do it at around the age of 16, and it's really just starting to involve patients and shifting the responsibility from the parents to the patient.
So when you're thinking about preparing for transition, just things as an individual of what you should know is, what's your hemoglobin genotype? What's your medical history? Do you have any sickle cell complications? Maybe you have gallstones or had your gallbladder removed. What's your baseline hemoglobin? What medications are you on? What are your triggers? How do you treat a crisis or what do you do when you're having pain? Other things to know are who to contact in case of emergencies.
What's considered an emergency, even? And then I think it's very important to learn about the insurance options and what's required for visits. Because a lot of times when patients transition to me for the first time on the adult side, they get blocked and their visit's not approved because they didn't get the referral from their primary care provider. You ask them who their primary care provider is, they say you are when it's no, we're specialists. So there's a lot of coordination that needs to go into transition just to set up for success, so I think knowing who to contact when you're having issues, start just being coming involved in your actual care and ask questions when you're in the visit.
There's no such thing as a stupid question. And I know parents sometimes might be a little helicopter because you just want to make sure your child is doing everything right, because sickle cell is scary and it's so unpredictable, and you can be doing everything right and something still happen, that you want to do everything for your child. slide 2 But it's equally as important to involve your child in a lot of the, not necessarily decision making, but in your visits and just have them involved in asking questions and understanding what's being said, because ultimately once they become an adult and come to my office for that first time, they're going to be so much more equipped if they've been involved in talking about this for several years leading up to transitioning to that adult service.
Deandre White:
Maya, are there tests for warriors that can measure organ damage and chronic pain?
Maya Bloomberg, MSN, APRN:
So what you're talking about or screening tests, and these are tests that allow for early diagnosis and intervention, so I kind of just give a head-to-toe run down of what you would want to do for sickle cell screening, starting with lab tests, getting your blood count doing a complete metabolic panel, which is gonna check on your liver function, it's also going to check your kidney function. But on a metabolic panel, a creatinine level is typically what we use to measure your kidney function, but it's important to realize individuals with sickle cell actually have a reduced ability to concentrate the urine. So your creatinine's always going to be low. Once we start seeing a high creatinine, this is usually more long-term going on that you've been having some kidney dysfunction in the background.
So a great test to check for your kidney function and is an early, early sign of any type of kidney involvement, is a random urine protein. Now, the best test is a microalbumin creatinine ratio, but if you can't get that one, then at least get a urinalysis, and that way you can see, do you have any protein loss? Now, going to the eyes, we know that sickle cell retinopathy is a complication of sickle cell, and it can actually contribute to going blind. It's more common in hemoglobin SC genotype, and essentially what can happen is you have a sickled hemoglobin that blocks off a blood supply in one of the vessels, your body creates these little webs or collateral vessels that serve no function at all and they can actually bleed and cause vision loss, so it's recommended to get an annual full dilated exam beginning at the age of...to start looking for this. Because if they start seeing any early signs, they can laser it and just come up with an intervention before it ever gets to the point of you losing your vision. Another test to consider is an echocardiogram, this is an ultrasound of your heart. This is to screen for a complication called pulmonary hypertension, where you can have increased heart pressures as a result of just long-term anemia and the increased cardiac output, among other things. Now, we used to have guidelines that would have individuals being screened with an echocardiogram every two years, but unfortunately what was happening is individuals would have high heart pressures, we really will look at the RVSP, or the right ventricular systolic pressure, and if that's above 35 millimeters of mercury, that's indicative of pulmonary hypertension. But once you have a positive echocardiogram or ultrasound of your heart, we then have to confirm it with a cardiac cath, which is where they stick a catheter inside your groin or your wrist, and it goes right inside of your heart to objectively and definitively diagnose and check for pulmonary hypertension. So we found that there were a lot of false positives, meaning they would have a positive echocardiogram, and then you would get the cardiac cath and they would have normal heart pressures.
So now the recommendation is to screen people who have any concerning symptoms for pulmonary hypertension, and the biggest one is, is if you get shortness of breath with little activity. Now that's also a very common sense for anemia, but say you had low hemoglobin, you've got shorter breath, you're having some brain fog and feeling a little tired and weak, you get your blood transfusion and most of the symptoms go away, but you're still having shortness of breath, then that might be something you'd want to consider. So shortness of breath with little activity, definitely make sure you talk to your provider. A lot of times my patients are like, oh, well...
I haven't been working out a lot, it might be endurance. And it very well could be endurance, but people who have advanced pulmonary hypertension have a worse prognosis from a sickle cell standpoint that I will always like to rule out any other cause before just saying, yeah, it's low endurance. Pulmonary function test is another test that could be considered, but it's not something that everybody with sickle cell is going to be screened with, but if you're having any shortness of breath, you might want to consider that. Now, when it comes to chronic pain, that is the million dollar question. I wish that there were lab tests to validate and to quantify pain, but in individuals with chronic pain, it's important to get an appropriate workup, because again, it's so easy to blame sickle cell on everything that we want to rule out autoimmune causes, for example, which can contribute to joint pain. You want to do imaging.
Is there any avascular necrosis which is contributing to the pain? Could they benefit from physical therapy? Could they benefit from a nerve block or a steroid injection, things of that nature. So although we don't have labs to confirm chronic pain, it's important to just rule out other causes of pain and to also just make sure you're having a combination approach when it comes to managing pain. Because narcotics alone should not be the sole management of chronic pain. When it comes to narcotics, you want to use the lowest effective dose. Chronic pain usually has a neuropathic or a nerve component as well, so adding neuropathic medications in addition to your narcotics and anti-inflammatories. We even use antidepressants, not for depression, but in the sickle cell guidelines, there's research that shows duloxetine, for example, which is Cymbalta, known as Cymbalta, the commercial that shows, “Depression hurts, Cymbalta helps,” but that medicine is now recommended in the sickle cell guidelines for use in chronic pain, because it's really good for addressing that nerve pain. So I would just want to make sure that individuals are appropriately managing their chronic pain and working it up to make sure there's nothing else that could be contributing to that as well.
Deandre White:
What is the transcranial Doppler screening test, and when is this test typically ordered for patients?
Maya Bloomberg, MSN, APRN:
So people with sickle cell have a much higher risk of stroke than children without sickle cell disease, and knowing this, we perform screening tests with transcranial Doppler to identify those who are at higher risk or highest risk, I should say, so we can intervene before a problem actually occurs. So it's basically an ultrasound of the vessels in the brain, and if they see that there's high pressure, which means that there's a lot of blood just trying to squeeze out of a vessel, those are the ones who have abnormal TCDs or abnormal transcranial Dopplers and are therefore at higher risk of having a stroke. So once somebody is identified as being high risk for stroke is when they'll discuss starting on blood transfusions, maybe hydroxyurea. We should start screening at the age of 2, and it's usually done until the age of 16, and at that point it stops because your skull ultimately becomes too thick to be able to have a clinically meaningful reading.
Deandre White:
What if patients don't have access to resources to be able to get this sort of screening, what happens later on? Are they less likely if they're an adult to be able to get this kind of screening test ordered? Or is it just kind of as an adult, more so geared towards patients that have strokes?
So if they don't do the screening test, then they're just at higher risk for stroke and it's almost like a ticking time bomb waiting for that to happen. So the TCD is allowing us to be more proactive with somebody's health as opposed to reactive, because once somebody develops a stroke, that's when we're going to start them on blood transfusions right away. Kids are very resilient and a lot of times they recover really well from these strokes, but not everybody is as lucky, so it should be a standard and everybody in the United States really should be making sure they get the transcranial Dopplers, and if you haven't gotten it, you want to ask for it. Now, if you have hemoglobin SC, it might not be done because childhood strokes are much less common in that population compared to hemoglobin SS.
But if you have hemoglobin SS, you really should prioritize getting that done, because it's going to allow you to just, again, be proactive at preventing a potentially catastrophic health outcome.
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