Focal Segmental Glomerulosclerosis and Key Diagnostic Testing
More Programs and Publications Featuring Maya Bloomberg, MSN, APRN
In this program:
What is focal segmental glomeruloslcerosis (FSGS), and what should sickle cell patients know about the condition? Hear from hematology nurse practitioner Maya Bloomberg from Sylvester Comprehensive Cancer Center explains FSGS and how it can develop, symptoms that patients may experience, and key tests and treatments for sickle cell care.
Transcript
Interviewer:
What is focal segmental glomerulosclerosis or FSGS test? And what are the ways it can develop? Because I know there are, it's considered primary and secondary FSGS. It can develop from drugs, or it can develop from having HIV or sickle cell or diabetes or all these different things. But is it true that it can sometimes be from an unknown cause? And what is the FSGS test?
Maya Bloomberg, MSN, APRN:
So focal segmental glomerulosclerosis, say that 10 times faster, FSGS, is a kidney disorder. And as you mentioned, it can occur with certain infections, even obesity. But with sickle cell, what we see is when you have sickled hemoglobin decreasing the blood flow to an organ, in this case the kidneys, it ultimately leads to impaired kidney function. Now, when it comes to any type of kidney dysfunction, early on, symptoms are very rare, they're not that obvious. And as the disease progresses is when a person might start experiencing maybe swelling of the legs or ankles, maybe even face, you might notice having foamy urine, high blood pressure, and when it gets even more advanced is when you have decreased urine output, or you're just not peeing at all. We'll also see different differences in your labs when it's later on. So knowing that sickle cell can affect the kidney, we do screening tests for this. One of the earliest indicators of any type of kidney involvement or FSGS related to sickle cell is you start spilling protein in your urine because your kidneys are responsible for metabolizing and filtering out all of the products and that's what we excrete in our urine. But once proteins, which are larger molecules start getting excreted, that means the kidneys aren't working as well.
So this is an amazing screening test that if you aren't connected with a sickle cell specialist, you should make sure at the very least you should have a urinalysis performed. A better test would be a microalbumin creatinine ratio. And if your ratio is above 30, that is indicating that you're having microalbuminuria. So when I have patients who are identified having proteinuria or microalbuminuria, that's the medical term for protein in your urine, we can actually do something about it because when it comes to kidney disease, again, it's rare to have early symptoms. So when we're catching this just from protein, I always reassure my patients, don't be startled, you don't have severe kidney disease and all of these issues. We're catching it so early where we can actually potentially reverse it and protect your kidneys from future damage. The first step is hydration. When you're dehydrated, your body has this protective mechanism where it diverts or it moves blood flow to your essential organs, which is your brain and your heart. So that means your kidneys can get neglected. So anybody can get an acute kidney injury from dehydration alone. Now, let's add sickle cell. We know dehydration is the number one trigger for a sickle cell crisis.
You have more sickled hemoglobin circulating, which can then decrease more blood flow to your kidneys. So you have two things that can worsen and impair your kidney function. So hydration is the number one thing you want to do because that's what's keeping your kidneys well-perfused or circulating with blood. Another thing, if somebody is found to have protein in their urine, you want to make sure that you're limiting medications that are filtered and metabolized through your kidneys. And the biggest one are NSAIDs, nonsteroidal anti-inflammatory drugs. So this is like your ibuprofen, naproxen, Aleve, Motrin. These medicines, I always encourage my sickle cell patients to take because when you're having a sickle cell crisis, your body's in this pro-inflammatory state. You obviously are going to need narcotics when most…patients with sickle cell are dealing with pain their entire life. So most patients are going to need narcotics to treat this pain, but it's important to realize narcotics are just a band-aid. It's literally just turning off a switch in your brain saying, hey, I'm not in pain. When you take naproxen or an NSAID, this is an anti-inflammatory that's actually addressing the underlying cause of the pain that you're having.
And then it has a great synergistic or an additive effect when you're taking your narcotics with your anti-inflammatories. But if you have kidney disease, you want to avoid the NSAIDs because that is going to further damage or further contribute to insult to your kidneys. You also want to avoid NSAIDs if you have any stomach ulcers, bleeding, to sort of things of that nature. Now, somebody who has persistent proteinuria, or they continue to have protein every time we recheck it, then we consider starting on an antihypertensive medication or a blood pressure medication. There are two classes that we use called ACE or ARBs. These medicines are very rarely used by itself to treat blood pressure. It's usually a combination medication. But when we use it for sickle cell and protein in the urine, we're using it specifically because it dilates the vessels that enter and exit the kidney. And by doing so, it decreases the workload, and it can protect from future damage and can actually reverse from the kidney damage. Hydroxyurea (Hydrea) is also a medication that prevents multiple complications, because at the end of the day, if you're taking a medication that is improving the red cell health and really modifying the disease process, which is that sickled hemoglobin, we're producing more stable hemoglobin, fetal hemoglobin with the use of hydroxyurea, that will also protect your organs, and in this case will help protect your kidneys from further damage as well.
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