Does anyone have NS w/out a cause?

By carrie63
Posted July 8, 2008 at 4:18 am

Dear Shawnsmom

Every case is different but my daughter has been leaking high levels of protein for 3 years (she has FSGS) and is totally unresposive to EVERYTHING. She remains however extremely well, athletic, to all intents and purposes glowingly healthy, with 100% kidney function, near normal albumen levels etc - which mystifies yet rather delights the nephs. All I am saying is they don't really understand this disease and can't predict what will happen and when. I wish you all the best. Thinking of you and Shawn.

By Mika
Posted July 9, 2008 at 11:10 am

Hi. My son was in a similar situation. He was 13 months old when he got sick. He was diagnosed with FSGS, I know that you said that your son does not have it but I know that even if you get a biopsy it might not show up and then if the biopsy is redone in a few months it might show it then. Well either way, my son was leaking huge amounts of protein and had extreme edema. Several medications were tried and nothing worked, he had a very bad reaction to cyclesporine and that could not be used. His kidneys were still working fine but because of the edema the doctors wanted to remove his kidneys in order to stop the protein loss. Fortunately they decided to try Rituximab first and that put him into remission. So now it is a year later. He has been in remission for most of the year, relapsed only once and was given rituximab again. Now he is also on Cellcept and is doing very well. If you are out of options then ask your doctor about rituximab, it saved my sons kidneys and probably his life as well. Good luck.

Mika

By Shawnsmom
Posted July 10, 2008 at 7:42 am

Thanks so much for the reply Mika... they did find out my son's condition is not immune based. I know Cellcept supresses the immune system so it stops attacking the kidneys, is this other drug similar and suppresses the immune system or is it something different? So glad your son is doing well, I am looking forward to that day....

By Mika
Posted July 10, 2008 at 2:41 pm

Hi. I was just wondering that how did they find out that it is not immune based? When my son first got sick they were not sure if it is immune based or not because he did not respond to any drugs (at the time even Cellcept did not make any difference but it's working now). They did send a blood sample to be checked to see if it is genetic. But we had to wait for six weeks for the results and in the mean time they did plasma phereses (not sure how to spell that) and that helped a little so it made them think that it is the immune system that is causing it. The test results for the genetic cause came negative so that confirmed that it is immune system based. Rituximab does affect the immune system (it destroys the B cells in the immune system) so if the immune system is not the problem then obviously it would not work. We did meet one little girl who's FSGS was genetic (although no one in her family has it, strange how that works). She did have to have a transplant and now has her fathers kidney and is doing very well. The one thing good about the genetic kind is that it does not come back in a new kidney the way that the immune system kind does.
Hope they can find something for your little boy soon.

Mika

By louink08
Posted July 11, 2008 at 8:58 am

I find it hard to believe your son's NS is not immune based and very much doubt there is a test for that. Ask if he has "idiopathic NS," which means the cause is not known. And ask how they can know it is not immune-based. And the only way they can determine he is "negative for FSGS" is to do a kidney biopsy, literally punching an instrument into the kidney to take samples. Did he have a biopsy? FSGS means the kidney filters that are misshapen and allowing the protein to leak have finally scarred from continued deformity or "effacement."

By Reneaorr
Posted July 11, 2008 at 11:42 am

My daughter has FSGS and leaks protein all the time. She is on cyclopsporine which only reduces the protein leak. We call it a partial remission, because swelling is reduced and Creatine clearance is stable. We are doing everything we can to save the kidneys. Did the doctor say what his GFR or creatine clearance was? It sounds like you need to try some of the other medications available before the due such a major surgery on your little baby. Have you tried cytoxan, cellcept, cyclosporine? Most did not work at all for my daughter but lowering her leakage at least has slowed the progress of the disease and keeps her from dialysis and removing her own kidneys. Good luck and we will pray for you both!
Renea

By Shawnsmom
Posted July 11, 2008 at 1:38 pm

You know, he did have a biopsy about month after he was diagnosed since he case was very abnormal. He did have scarring so the original diagnosis was FSGS, but they did a "staining" test which showed it was not FSGS. From this they thought it was congenital, so they thought he was born with the bad kidneys. But since his kidney funtion is still perfect, that is not in line with congenital NS either. He really is not fitting into any one diagnosis. We ruled out Allports and Pierson Syndrome also w/ genetic testing done by Dr. Zucker who is a geneticist in Germany who specializes in peds nephrology. I have had pathologists at UCLA & Cedars Sinai in LA look at the slides from the biopsy and they are stumped. He has scarring and thin basement membrane. Still the cause is unknown. I am going to try to seek other opinions.

By Shawnsmom
Posted July 11, 2008 at 1:50 pm

Thank you for your response. His creatine level is .2 and has not changed since he was diagnosed in early Feb 2008. The only med they are giving me to "treat" him is an ACE inhibitor (Enalapril). We tried steroids (he is resistant) which did not work and we were on cellcept for a short time until they said it was not FSGS. I just can not keep doing once a day infusions of albumin through the IV each day forever, need to find an answer.

By Reneaorr
Posted July 11, 2008 at 2:13 pm

Have you gone for a second opinion? There are other meds out there that help with the protein spilling regardless if it caused by FSGS or Lupus, etc. I have read studies about cytoxan, cyclosporine, cellcept, prograf, etc. I would ask them to give you more options or find another doctor. Did Cellcept reduce the protein leakage? If not, then I can understand coming off of it, but if they only took him off because it was not FSGS, that would concern me. We will keep you in our prayers. Renea

By Reneaorr
Posted July 11, 2008 at 2:13 pm

Have you gone for a second opinion? There are other meds out there that help with the protein spilling regardless if it caused by FSGS or Lupus, etc. I have read studies about cytoxan, cyclosporine, cellcept, prograf, etc. I would ask them to give you more options or find another doctor. Did Cellcept reduce the protein leakage? If not, then I can understand coming off of it, but if they only took him off because it was not FSGS, that would concern me. We will keep you in our prayers. Renea

By ALE2003a
Posted July 25, 2008 at 6:36 pm

I noticed somewhere on the website that he possibly had MPGN. I think it was from your Feb. entry. I have MPGN Type 1 and Im about the only one I can find that has this type of kidney disease. MPGN is usually a disease that starts at young ages like your son. I was fortunate enough not to get it until I was 32. I do have the idopathic form so no one knows why or how I got this disease. They tell me I probably will never know. My symptoms from the disease mainly consist of edema and extreme proteinuria. All the rest of the wonderfull things I am expierenceing are from the medications. ( and I mean wonderfull in the most sarcastic way possible) I was put on Pred. right after my diagnosis in Feb. I also am on a ace inhibitor. The pred. did absolutly nothing for the first three months other than cause me to gain 60 pounds in three weeks. Then they decided to try the cellcept in conjuntion with the pred. and this has worked absolute wonders for me. So far it has taken 4 months but my protein has dropped from 17,000 mg to 4,000 mg. I dont see why they removed cellcept of your sons diet just because its not focal segmental, cellcept is used for almost all forms of kidney disease /nephrotic syndrome.

By leh8709
Posted July 28, 2008 at 1:03 am

Dear Shawnsmom,

My son was diagnosed with NS in November of 2008. I can relate to how frustrated you may be feeling. My son has already had several relapses and hospitalized twice. It has been a very rough year and it's not even over. I find myself doing well on somedays but then I just feel so overwhelmed on ther days. I just wish that my son could be free of this disease. It's like a nightmare that never ends. My son is steriod dependant and every time we try to ween him off, he relapses. Regarding your son, there may be some other meds that may help him. I would look into trying some other meds first. Please get a second opinion. Renea has been wonderful to me and she knows her stuff. I agree with her that you should get a second opionion. I will pray for your son. I have an affirmation that I have been using to help me get through the rough patches. I will pass it on to you. "I go beyond incurable. I am healthy. My son is healthy. My family is healthy. All is well in my world". I hope everything works out for. Sending you my love and prayers.

By Shawnsmom
Posted July 28, 2008 at 12:43 pm

Thanks so much to everyone for your responses. I am going to try to get a second opinion out of Boston's Children's Hospital. Hopefully we can put him on some meds that will help with the protein spilling. Right now he has a broviac catheter and has to have albumin iv treatments once a day to keep down the edema. Thanks also for your prayers - please keep them coming as I will continue to pray for all of you,
Much love and appreciation...

By JennaS
Posted July 30, 2008 at 2:57 pm

Hi

I agree, a second opinion is a good idea. Our son Danny was diagnosed with nephrosis about a year ago. He is now 7 years old. Nothing seem to be working at the first Children's Hospital we went to in PA and they never seem to explain things so I could understand it. Gave me different medicines and Danny only seemed to swell more That left me on the internet searching! Finally we found Dr. Heiliczer at St. Christopher's Hospital for Children in Philadelphia. Dr. Heiliczer has been a blessing from heaven! First he explains everything in clear english and he gives you his time with and is very good with Danny. We are finally on the right medicines and Danny is in remission. So even if you wind up staying with your first nephr getting an another explanation helps.

By s-nathan
Posted August 7, 2008 at 3:17 pm

Shawnsmom
Combination of ACE inhibitor + Angiotensin Receptor blocker (ARB) is sometimes used to reduce the amount of protein leakage. You son seems to be on an ACE inhibitor. Have the docs considered adding on an ARB to reduce the amount of protein in the urine?

By beej1962
Posted August 11, 2008 at 4:34 pm

I have idiopathic FSGS. Nobody else in my family has any type of kidney disease and I am the only person in my immediate family that is not diabetic. The doctor told me that I did not cause my disease and I am not sick in any other internal organ in my body. My blood pressure is controlled and I check my blood sugar regularly anyway due to the number of family members who are diabetic. I have been checked for every other disorder under the sun including arthritis and lupus. My internest said that my sedimentation rate is high indicating inflammation(arthritis) but that is all.

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