Two or More

Time for another reader question, but first, let’s pay attention to what day today is. Many people see today as the day for bar-b-ques or backyard ball games (or, at least, they did before Covid 19). When I married Bear a little more than seven years ago, he explained about Memorial Day. I knew it was to honor those who died protecting us, but it was so much more meaningful when explained by a veteran… someone who didn’t die protecting us and lived on to meet me and marry me. So give some quiet thoughts to these men and woman today, will you?

Now, the question. This reader has both lupus like immune mediated glomerular nephritis and Wegeners vasculitis with kidney involvement. Her question is how does she handle both?  And, here I thought I had it bad with pancreatic cancer (now gone), Chronic Kidney Disease, diabetes, and a whole host of what I consider lesser diseases!

Starting slowly is a must here since I am like a fish out of water with these two diseases. According to the MayoClinic at https://www.mayoclinic.org/diseases-conditions/granulomatosis-with-polyangiitis/symptoms-causes/syc-20351088,

”Granulomatosis with polyangiitis is an uncommon disorder that causes inflammation of the blood vessels in your nose, sinuses, throat, lungs and kidneys.

Formerly called Wegener’s granulomatosis, this condition is one of a group of blood vessel disorders called vasculitis. It slows blood flow to some of your organs. The affected tissues can develop areas of inflammation called granulomas, which can affect how these organs work.

Early diagnosis and treatment of granulomatosis with polyangiitis might lead to a full recovery. Without treatment, the condition can be fatal.”

Whoa! Not good. Let’s see how it’s treated. The Cleveland Clinic at https://my.clevelandclinic.org/health/diseases/4757-granulomatosis-with-polyangiitis-gpa-formerly-called-wegeners/management-and-treatment tells us,

“People with GPA who have critical organ system involvement are generally treated with corticosteroids [Gail here: commonly just called steroids] combined with another immunosuppressive medication such as cyclophosphamide (Cytoxan ®) or rituximab (Rituxan®). In patients who have less severe GPA, corticosteroids and methotrexate can be used initially. The goal of treatment is to stop all injury that is occurring as a result of GPA. If disease activity can be completely ‘turned off,’ this is called ‘remission.’ Once it is apparent that the disease is improving, doctors slowly reduce the corticosteroid dose and eventually hope to discontinue it completely. When cyclophosphamide is used, it is only given until the time of remission (usually around 3 to 6 months), after which time it is switched to another immunosuppressive agent, such as methotrexate, azathioprine (Imuran®), or mycophenolate mofetil (Cellcept®) to maintain remission. The treatment duration of the maintenance immunosuppressive medication may vary between individuals. In most instances, it is given for a minimum of 2 years before consideration is given to slowly reduce the dose toward discontinuation.”

Okay, got it. Now let’s take a look at lupus like immune mediated glomerular nephritis. MedicineNet at https://www.medicinenet.com/script/main/art.asp?articlekey=8064 reminds us about lupus:

Lupus: A chronic inflammatory disease that is caused by autoimmunity. Patients with lupus have in their blood unusual antibodies that are targeted against their own body tissues. Lupus can cause disease of the skin, heartlungs, kidneys, joints, and nervous system. The first symptom is a red (or dark), scaly rash on the nose and cheeks, often called a butterfly rash because of its distinctive shape. As inflammation continues, scar tissue may form, including keloid scarring in patients prone to keloid formation. The cause of lupus is unknown, although heredity, viruses, ultraviolet light, and drugs may all play a role. Lupus is more common in women than in men, and although it occurs in all ethnic groups, it is most common in people of African descent. Diagnosis is made through observation of symptoms, and through testing of the blood for signs of autoimmune activity. Early treatment is essential to prevent progression of the disease. A rheumatologist can provide treatment for lupus, and this treatment has two objectives: treating the difficult symptoms of the disease and treating the underlying autoimmune activity. It may include use of steroids [Gail here: Remember they’re used in treating this reader’s other disease, too.] and other anti-inflammatory agents, antidepressants and/or mood stabilizers, intravenous immunoglobulin, and, in cases in which lupus involves the internal organs, chemotherapy.

But our reader has lupus LIKE immune mediated glomerular nephritis, so she may need to deal with the symptoms, but not the treatment. Wikipedia at https://en.wikipedia.org/wiki/Immune-mediated_inflammatory_diseases informs us,

“An immune-mediated inflammatory disease (IMID) is any of a group of conditions or diseases that lack a definitive etiology, but which are characterized by common inflammatory pathways leading to inflammation, and which may result from, or be triggered by, a dysregulation of the normal immune response. All IMIDs can cause end organ damage, and are associated with increased morbidity and/or mortality.”

That’s as close as I could get to the definition of immune mediated.  We know that glomerular means of or about the glomerulus. Dictionary.com at https://www.dictionary.com/browse/glomerular helped me out here:

“Also called Malpighian tuft. a tuft of convoluted capillaries in the nephron of a kidney, functioning to remove certain substances from the blood before it flows into the convoluted tubule.”

And nephritis? After a decade of writing this blog, we probably all know that’s an inflammation of the nephrons.

Let’s combine the pieces to see what we get.  The nephron’s glomeruli are inflamed in the same way lupus inflames the organs. Remember that GPA also causes inflammation. (By the way, this is the perfect point in the blog to remind you I am not a doctor and have never claimed to be one.)

But how is it treated? Here’s where I admit defeat. There is quite a bit of information available on Lupus, Lupus Nephritis, and the like. But I could not find anything that includes ‘Lupus like.’

The commonality between the two diseases seems to be inflammation. But isn’t that at the root of all Chronic Kidney Disease? I admit to being surprised twice while writing this particular blog:

  • GPA was called by its older name by the doctor.
  • The dearth of treatment information for lupus like immune mediated glomerular nephritis.

Until next week,

Keep living your life!

AKI & CKD

Aha! Dana contacted me and here’s the blog I promised him. (Still looking for the request from the woman who waited so patiently for me to recover from my surgery. Please contact me again.) Dana asked about AKI, Acute Kidney Injury, and how aggressively his nephrologist should be pursuing treatment of this. He and his nephrologist feel that his AKI may have been caused by strep.

I know I write about CKD, Chronic Kidney Disease, so what is AKI? The glossary in my very first CKD book, What Is It and How Did I Get It? Early Stage Chronic Kidney Disease, tells us ‘acute’ means:

“Extremely painful, severe or serious, quick onset, of short duration; the opposite of chronic.” This is what I wrote about AKI and CKD in SlowItDownCKD 2017,

“I’d always thought that AKI and CKD were separate issues and I’ll bet you did, too. But Dr. L.S. Chawla and his co-writers based the following conclusion on the labor of epidemiologists and others. (Note: Dr. Chawla et al wrote a review article in the New England Journal of Medicine in 2014.)

‘Chronic Kidney Disease is a risk factor for acute kidney injury, acute kidney injury is a risk factor for the development of Chronic Kidney Disease, and both acute kidney injury and Chronic Kidney Disease are risk factors for cardiovascular disease. Not surprisingly, the risk factors for AKI {Once again, that’s acute kidney injury.} are the same as those for CKD… except for one peculiar circumstance. Having CKD itself can raise the risk of AKI 10 times.’

Whoa! If you’re Black, of an advanced age {Hey!}, or have diabetes, you already know you’re at risk for CKD, or are the one out of nine (Update: Now one out of seven.) in our country that has it. Once you’ve developed CKD, you’ve just raised the risk for AKI 10 times. I’m getting a little nervous here….

It makes sense, as researchers and doctors are beginning to see, that these are all connected. I’m not a doctor or a researcher, but I can understand that if you’ve had some kind of insult to your kidney, it would be more apt to develop CKD.

And the CVD risk? Let’s think of it this way. You’ve had AKI. That period of weakness in the kidneys opens them up to CKD. We already know there’s a connection between CKD and CVD (Cardiovascular Disease). Throw that AKI into the mix, and you have more of a chance to develop CVD whether or not you’ve had a problem in this area before. Let’s not go off the deep end here. If you’ve had AKI, you just need to be monitored to see if CKD develops and avoid nephrotoxic {Kidney poisoning} medications such as NSAIDS… contrast dyes, and radioactive substances. This is just so circular!

As with CKD, your hypertension and diabetes {if you have them.} need to be monitored, too. Then there’s the renal diet, especially low sodium foods. The kicker here is that no one knows if this is helpful in avoiding CKD after an AKI… it’s a ‘just in case’ kind of thing to help ward off any CKD and possible CVD from the CKD.”

Dana’s nephrologist put him on a regiment of prednisone for two months. Why? Well, prednisone is an anti-inflammatory drug. WebMD at https://www.webmd.com/a-to-z-guides/what-is-acute-kidney-failure#1 offers the following as possible causes of AKI. Notice the very last one and you’ll see how prednisone may be helpful.

  1. Something is stopping blood flow to your kidneys. It could be because of:
  1. You have a condition that’s blocking urine from leaving your kidneys. This could mean:
  1. Something has directly damaged your kidneys, like:

Now we know AKI and Acute Kidney Failure are not the same thing, but it is possible that this nephrologist is using prednisone in an attempt to avoid Acute Kidney Failure.

One thing Dana asked that made me stop cold is “How do you cope with the inevitable aspects?” They are not inevitable, Dana. I am a lay person who has managed to keep my CKD at stage 3 for 11 years. I am also not a magician. What I am is someone who follows the guidelines for keeping my kidneys as healthy as possible.

You’ve already seen a nutritionist – hopefully a renal nutritionist, since a healthy diet is not necessarily a renal healthy diet – so you’re aware of the nutrition aspect of protecting your kidneys. But there’s more. Do you smoke or drink? If so, stop. Do you exercise? If not, start… but with your nephrologist’s supervision. Are you getting adequate sleep and rest? Here’s the hardest guideline: try to avoid stress. Of course, if you have a stressful life, avoiding stress can just be another stress.

As to how aggressively you should expect your nephrologist to treat your AKI (or the CKD resulting from it) really depends upon you and your nephrologist. For example, some think stage 3 is barely CKD and urge you to just keep watch. Others, like my nephrologist, take CKD seriously and have their nutritionists train you re the renal diet and speak with you themselves about the guidelines. As for AKI, again it depends on you, your nephrologist, and the severity of the AKI. Since you have waste product buildup and inflammation, you may need dialysis or a hospital stay… or watchful waiting while taking a medication such as prednisone.’

There seems to be quite a lot of leeway as to the treatment you and your nephrologist decide upon.

Until next week,

Keep living your life!