I Hate Dialysis Message Board
Dialysis Discussion => Dialysis: Transplant Discussion => Topic started by: KT0930 on March 30, 2008, 12:59:20 PM
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This article was in the latest issue of my transplant team's newsletter (Piedmont Hospital; Atlanta). Thought some here might find it interesting. I apologize for any typos; the article is not on the web that I could find, so I typed it in...any spelling errors/typos are my own and not in the original article.
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FSGS accounts for 3.3% of the ESRD population. Studies have shown that approximately 30% of patients with a pre-transplant diagnosis of idiopathic (i.e. unknown) FSGS will develop recurrent disease post-transplant. Individuals at higher risk for recurrence include young children, rapid progression of disease in the native kidney (less than three years), and especially a history of recurrence of FSGS in a prior kidney transplant. "The pathophysiologic etiology of recurrent FSGS is thought to be related to the presence of a circulating factor in the bloodstream that injures the glomerulus, or filtering unit, of the kidney resulting in significant proteinuria. This can occur as early as intra-operatively or as late as several weeks post-transplant. The average time to recurrence is usually 4 days and can be confirmed by a kidney biopsy showing foot process effacement of the glomerular basement membrane on electron microscopy.
Traditionally, those with recurrence of FSGS transplant have had a poor prognosis, with only a 50% allograft survival at five years. Recently, plasmapheresis (PP) has been utilized as a therapy for recurrent FSGS and been effective if initiated promptly once proteinuria in detected. PP is thought to remove the circulating factor responsible for glomerular injury, which leads to proteinuria. This therapy has been supported by studies that have shown a course of PP lead to reversal of foot process effacement on kidney biopsy and remission of proteinuria. A course of PP consists of 9 treatments every other day where we exchange 1.5 liters of plasma volume with 5% albumin replacement. Once the course is complete, we will determine the success of the treatment by re-evaluating for proteinuria.
At Piedmont Hospital, we are developing a protocol to evaluate individuals with native FSGS in the post-transplant period. Specifically, we will be measuring spot urin protein and creatinine, and using these values to estimate 24 hour urine protein excretion. If we see a urin protein to creatinine ratio of greater than 2 (estimating greater than 2 grams of protein in a 24 hour urine collection), then we will proceed with a kidney transplant biopsy and initiate PP if there is foot process effacement. By promptly diagnosing and treating recurrent FSGS with PP, our five year allograft survival in this specific cohort should increase from 50% to close to 70%.
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Interesting
Well if there was spelling errors, I did not notice, but wasn't looking for them either.
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That's me---idiopathic FSGS. I cannot get any neph or transplant surgeon to flat out tell me the chances of it recurring. I always hear "everyone is different". It is a big concern of mine. If you are asking for a living donor and you know there is a chance of FSGS effecting the new kidney, are you being irresponsible in taking the new kidney? I strugge with this alot. Thanks KT for posting the article. I want to read everything there is on FSGS.
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That's me---idiopathic FSGS. I cannot get any neph or transplant surgeon to flat out tell me the chances of it recurring. I always hear "everyone is different". It is a big concern of mine. If you are asking for a living donor and you know there is a chance of FSGS effecting the new kidney, are you being irresponsible in taking the new kidney? I strugge with this alot.
Paris, my answer to that is no. You're hoping to get your life back. If you were to get the kidney and did nothing to take care of it, that'd be irresponsible; getting a kidney and having something happen to it that is beyond your control is just bad luck.
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Thanks KT. As I said, I really struggle with this. None of this is easy, is it? I need to hear people's opinions to help me think this through clearly. I forwarded the article to family and friends because it explained FSGS so well and helped to show them where I am in all of this. Sometimes it helps them to read someone else's story to understand mine----does that make sense? Again, thanks for the article and your reply :cuddle;
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Dustin's is idiopathic also. The transplant Dr told us there is a 50% chance the disease could take out a new kidney. I like yours better 30%!!!!!
Lori/Indiana
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This was very interesting to read - thank you.
I have FSGS but the slow version (it took well over 10 years and possibly over 30 to totally snuff out my kidneys). I asked the doc about the chances of it affecting a transplanted kidney and he said that it wasn't that likely and even if it did it was reasonable to assume that the progression would be as slow as with the current FSGS I have - thus it could affect the kidney but so slow that the transplanted kidney could fail before FSGS killed it off.
It is still a concern to me obviously so thanks for sharing this!
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This was very interesting to read - thank you.
I have FSGS but the slow version (it took well over 10 years and possibly over 30 to totally snuff out my kidneys). I asked the doc about the chances of it affecting a transplanted kidney and he said that it wasn't that likely and even if it did it was reasonable to assume that the progression would be as slow as with the current FSGS I have - thus it could affect the kidney but so slow that the transplanted kidney could fail before FSGS killed it off.
It is still a concern to me obviously so thanks for sharing this!
That is exactly what my transplant doctor and surgeon told me. I was diagnosed in 1990 after my son was born, at which time I was functioning at 30% and started dialysis in 2006, so the disease progressed slowly. Ironically I was also told that since my donated kidney from my sister is such a close genetic match that the chances of recurrence go up. It is a big concern for me as well.
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Thanks RichardMel and Jill for you input. Jill, you are still my guiding light! Part of my struggle is having one of my children donating and then, in the future FSGS effects them. None of this is simple, is it?
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I have FSGS and heard this before, too. I haven't been super-worried about it because my form is very slow-progressing. I was first diagnosed with "some sort of" CKD when I was 9 years old. I'm almost 45 now and still have 25% of my native function left. My younger sister offered to be a donor to me when I need one, but it turns out she's an FSGS carrier. My nephew (her son) was diagnosed with FSGS a few months ago, so that worries me about the possible recurrance, assuming she's still interested in being a donor (she might want to save that kidney now in case her son needs it some day). My whole family sent blood off to a Harvard study to look for the genetic link.
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Thanks RichardMel and Jill for you input. Jill, you are still my guiding light! Part of my struggle is having one of my children donating and then, in the future FSGS effects them. None of this is simple, is it?
Elizabeth, is there a history of kidney disease in your family, specifically FSGS? If you have an idiopathic form, I think that means you do not have the genetic form. I don't know if they can tell the difference with tests, or if they determine the form by your family history. The Harvard study that Deanne and her family are participating in could be very helpful for you if they can find the genetic link and develop a test for it. Regardless, I know what you are saying about the thought of risking your child's health. My daughter was adamant about being tested as a donor for me. I said no way would I accept it, so don't even bother. When she turned 18 she went behind my back and was tested. She is an incredible young lady!
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Hi Jill----no family history. We have everything else in our family, but my kidney disease is a first. My sister-in-law is in a study to determine if there is a cancer link with my husbands family. I think I will look into the Harvard study. If nothing else, I would love to know the results. Every neph or surgeon I talk to gives me a different idea on FSGS. So, I keep researching on my own to find some kind of answer I can live with. Maybe one day, I will get that infamous call and I won't have to worry about it! I would know so little about all of this if I didn't have all of you! I would be blindly stumbling through and not have the strength and power you all have given me. Thank you :grouphug;
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Let's hear it for Epoman and IHD!!! :clap; :clap; :clap;
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My FSGS was not discovered until 6 weeks after my transplant, and my graft had already started to fail. This was discouraging because my transplant had been donated by my eldest son. We did try plasmapheresis, to no avail. We also experimented with rituximab, but I did not respond.
I have 3 other sons who would be willing to donate, but I'm not going there. Unfortunately, FSGS is particularly difficult to treat in Af-Am patients. Since I'm Af-Am my wait on the cadaver list is...forever, so I'm placing my bets on some other emerging treatment.
One of my docs is a leading researcher into FSGS, and I regularly bug him about when he's going to come up with a cure...hehehe...I will keep you posted!
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I did have a scare this fall that FSGS was causing protein to spill. My nephrologist determined it was my native kidney still functioning. I think about this often. I have no family history and one of my daughters had reflux but out grew it.
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I found this info while looking for something else. It's from The National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK):
Recurrence after kidney transplant
Approximately 25% of patients who have FSGS in their own kidneys will experience recurrent FSGS after kidney transplant. When FSGS recurs, it typically does so within days to weeks of transplant, and essentially always within one year of transplant. The cause of recurrent FSGS is unknown but is believed to be a circulating protein. Patients at increased risk for recurrent FSGS include the following: rapid progression (from diagnosis to end-stage kidney disease in less than 3 years, recurrent FSGS in a prior transplant (risk of recurrent FSGS is approximately 70%), and white race (weak risk factor). In patients with FSGS, it is prudent to check the urine weekly by dipstick for protein or every 2 to 4 weeks by measuring a random urine protein/creatinine ratio. The appearance of proteinuria should prompt a transplant kidney biopsy, which would initially show just podocyte foot process effacement and after weeks to months of proteinuria will show FSGS. If the diagnosis can be made within 2 to 4 weeks of proteinuria onset, therapy may include plasma exchange and possibly cyclophosphamide.
NIH research studies
1. Early diagnosis FSGS, children (proteinuria > 50 mg/kg) and adults (proteinuria >3.5 g/d): therapy with intermittent oral dexamethasone. Open-label study, no placebo, duration 48 weeks.
2. Therapy-resistant FSGS, adults (proteinuria <3.5 g/d despite ACE inhibitor or ARB therapy): therapy with oral retinoic acids (alitretinoin or isotretinoin). Open label-study, no placebo, duration 24 week.
3. FSGS with progressive decline in GFR despite ACE inhibitor or ARB therapy: therapy with oral pirfenidone. Open-label study, no placebo, duration 48 weeks, option to continue therapy longer if GFR decline rate is improved with therapy.
4. Recurrent FSGS following kidney transplant: For those at high risk for recurrent FSGS, therapy involves plasma exchange prior to kidney transplant. For those who have experienced recurrent FSGS after kidney transplant and who have had proteinuria less than 4 weeks, therapy involves plasma exchange plus oral cyclophosphamide. Both trials are open-label, no placebo.
More info at: http://intramural.niddk.nih.gov/research/glomerular_diseases/selected.asp
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Thank you Okarol. I want to read everything there is on FSGS. Having it recur is something I think needs to be considered before a transplant. My mind is always weighing the pros and cons. Sometimes I just think too much! :rofl;