6 Year old Niece

• By polaris
• Posted October 1, 2008 at 9:40 am

Hello, I'm sorry to hear about your niece . I have had nephrotic syndrome since I was 3 years old,now I'm 43 years old .I still have relapses but my kidney function is normal but thats not the norm. There are several treatments out there prednisone dispite the side effects has always worked well for me. The biopsy will
need to come first to determine the best treatment for her . I wish the best of luck to your family.

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• By levismom
• Posted October 1, 2008 at 11:26 pm

Are they also giving her lasik, to help remove some of the fluid? Iam sorry that she is going thru this. I hope they can get the biopsy done as soon as possible.

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• By Sev
• Posted October 1, 2008 at 11:35 pm

No they are only giving her prednisone. I had no idea what lasik is and that it removes some of the fluid? Should they be giving her lasik aswell as prednisone?
We dont know much about this at all....

She has been booked to have her biopsy late next week .. i just hope her kidney doesnt get worse till then

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• By Rileys-parents
• Posted October 2, 2008 at 2:53 pm

Hello,

We are sorry your neice is having this issue as well. I hope all goes well.

Rileys first trip to the hospital they gave him Lasik almost immediately to reduce the swelling/fluid in his body. After they get that under control they did blood tests to see how his protein (Albumin) levels were. If it is real low then they should be giving her Albumin as well.

The prednisone is suppose to stop the spilling over but sometimes people don't respond well to it and they have to have additional meds to assist.

Riley became steroid dependent because even though the steroids work. He relapses right away. We had a biopsy in March 07 and at that time started a med called Prograf which has worked fairly well and we have had to combine it with steroids as well sometimes.

Hopefully your neice will respond well. Send us a message if you would like and we will try to let you know what we know on any questions you think we might be able to help with.

We will pray for her - Looks like she has some red in her hair? As you might know(rileys picture) we are a bit partial to red heads ;)

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• By satishmk
• Posted October 2, 2008 at 4:46 pm

Right before my daughter was diagnosed with nephrotic syndrome (she was 18months old), we were running around to get various tests done. When we first talked to our nephrologist, she suggested that we control the water and salt intake for our daughter. Water was restricted to 600ml per day and salt was restricted to as little as possible.

The water restriction worked really well for her. The swellings went down drastically. She had swelling in her eyelids, belly and feet. She had put up 4-5 lbs just because of water.

May God bless your niece.

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• By Sev
• Posted October 5, 2008 at 2:47 am

Thankyou for your response everyone. I now understand and have researched into the medications she should be taking. Can i ask also, has anyones child had the side effects from the medication where there child cannot walk. It has been 1 week my niece has not been able to walk. she is still in hospital also. does anyone know? could this be from the medication side effects?

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• By duker
• Posted October 5, 2008 at 8:56 am

My daughter is unable to walk right now, but we have been on very high steroids for essentially 4 yrs and it is due to bone loss. I highly doubt that would be the case with your niece b/c she has been on it for such a short time,

but I have heard of people with tremendous muscle aching right away (seems to go away with time).

My daughter, when she first presented (at 2.5 yrs old) she had so much water weight (edema) that her little legs just couldnt take the huge increase in weight in such a short time. perhaps that could be it?

Lasik(furosimide) is a diuretic that works by bringing the third-spacing fluid back into the "leaky" capillaries and finally excreted by the kidneys. BUT it will not work if the albumin is not high enough. My daughter had no detectable albumin when she first presented, therefore they give 4 hr (most standard dosing) of an albumin (a blood protein) infusion often with a lasix dose at the 2 hr mark and then again at the 4 hour mark. it sometimes takes a couple of times of that regimen, but it works. it treats the symptoms to make them more comfortable, but does not treat the spilling of the protein.

Steroids are the only thing that can GET a person into remission (what I have been told), but once in remission, they try to get you off of steroids and use another autoimmune agent (cytoxan, cyclosporine, prograf, cellept) to HOLD the remission. Those other agents seem to be dosed in varying "cocktails" by different nephs. the above list was out order over 4 yrs.

Just remember, that 98% of kids WILL outgrow this after a 6 week course of high dose steroids. they will have a few relapses, but they should become fewer and far between as they age.

Keep strong.I think it is wonderful that you are doing all this research for your niece, it must be so frustrating to have to communicate this info across so many borders.

Best of luck!

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• By Crowley
• Posted October 5, 2008 at 9:13 pm

Make sure if your niece doesn't have much activity that they give her something to prevent blood clots. My daughter was non active due to the weight gain from fluid retention and was very uncomfortable walking around. This inactivity caused her to develop a blood clot which developed into a pulmonary embolism. This is in no way to scare you because this is rare, but please make sure if she is inactive to have preventive meds given. Best of luck to you and your family as you try to make sense of this disease. Keep posting questions and we will help you through.

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• By s-nathan
• Posted October 5, 2008 at 9:28 pm

Sev

Our son was diagnosed with Nephrotic Syndrome about 2 years ago. He was in the hospital 2 weeks when he was initially diagnosed. He was extremely uncomfortable and was unable to walk (due to severe swelling in the lower abdomen, scrotum and legs).
In the hospital, he was put on Lasix and Prednisone. Lasix administration also has to be monitored since it can cause electrolyte imbalance.
Our son also underwent MRI / deep scans of the leg to check for clot / pulmonary embolism. Fortunately he did not develop any.

A biopsy this year (2008) confirmed diagnosis as Minimal Change.

We will keep your niece in our prayers and we wish her the best

Swami Nathan

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• By Sev
• Posted October 6, 2008 at 1:46 am

Thankyou for your responses and i am sorry you all had to go through this all aswell.

So did your children not walk till they were off the medication?

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• By s-nathan
• Posted October 6, 2008 at 10:07 am

Sev

After enough fluid was out (thanks to Lasix - the diuretic), our son went back to walking - while at the hospital.
SN

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• By kcsmommy
• Posted October 6, 2008 at 11:13 pm

Hello Sev,

My 2 year old son went through the same exact thing... He was given prednisone and it worked at first, but it never worked again. He was first diagnosed on July 10th, and Prednisone put him into remission after 1-1/2 weeks. He stayed in remission for 10 days, but then he started to spill protein again at the end of July and we spent over a month in the hospital because it never worked again for him. Our specialists explained that they have to give Prednisone 6 weeks total to see if it will work. For my son, it never worked again. He is considered to be steroid-resistant. It is rare, but it does happen. Someone earlier (Duker) stated that prednisone is the only thing that will put someone in remission, but this is incorrect. My son was given Cyclosporine at the 6 week point, and he went into full remission within 2 weeks and has been in remission ever since. It sounds like your niece may also be steroid-resistant. Please know that there ARE other meds. that can put her into remission. My son's nephrologists considered 3 (cyclosporine, cellcept and prograf) and we went with cyclosporine because it was not as strong as the other 2 and had less side effects. Luckily, it is working very well for my son.
As far as not being able to walk, it may be due to the amount of fluid in her body. My son had a hard time moving around when he was very swollen. He would receive Albumin infusions and Lasix after each infusion to help bring down the swelling, but his ability to walk better did not improve greatly until he stopped spilling a lot of protein. (His little 2 year old body gained over 6 pounds of fluid!)
I think your niece's doctors may also be waiting the "standard 6 weeks". If she does turn out to be steriod-resistant, please know that there are other medications her doctors can try.

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• By Sev
• Posted October 6, 2008 at 11:24 pm

Hello kcsmommy,
Thanks for that.. you really made me relax a little.
I am sorry your little boy went through that but i am glad to hear his doing well now.
can i ask did they do a biopsy?

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• By kcsmommy
• Posted October 7, 2008 at 3:06 pm

Hi Sev,

Yes, we ended up doing a biopsy after 7 weeks. Luckily, it came back as minimal change. (I believe lots of prayers had a lot to do with this!!) We (my husband and I AND the doctors) were NOT expecting it to be minimal change. We were preparing for FSGS, but his biopsy showed normal, healthy kidney tissue with no damage. My son's doctors started him on Cyclosporine 5 days before the biopsy because Prednisone was not working for him. I don't know which medication your niece's doctors are planning on trying next, but keep in mind that some can take weeks to months to work. Again, we were very lucky because Cyclosporine worked right away for my son.
Please try to keep your head up and think positive. I know how hard it is to wait and wait and wait... It was very hard to watch my son get worse and there was nothing I could do about it except pray, think positive, and trust his doctors.
From our experience and others that I've talked to, it's always the worst in the beginning. It may take some time, but once the doctors can find a medication to control it whether it's minimal change or FSGS, it will get better.
Feel free to ask me any questions. I think your niece's situation sounds very similiar to my son's.
I will pray for her and you and your family.

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• By Sev
• Posted October 7, 2008 at 5:46 pm

Thankyou kcsmommy. Well we hope and pray our situation is the same as yours. Your boy looks like a cute and bright little one. I will keep you updated on how my niece goes. She is in Turkey now and they did a biopsy on her yesterday. We wont be getting the results till 10 days later. is this normal? thats is the hard bit also waiting in all these emotions and worries for results.
So far I dont know if they will try a new medication but i am guessing they will once they know the biopsy results.

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• By kcsmommy
• Posted October 9, 2008 at 3:22 pm

Hi Sev,

Yes, 10 days is normal for the full report. It took a week for my son's complete results.
Please keep me updated. I've been thinking of her!

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• By Pmorris
• Posted October 9, 2008 at 3:31 pm

The treatment for me after prednisone was cellcept. My doctor doesnt want me on lasiks, but i do have fuild restrictions (1L a day). But the medication hasnt stop me from walking, the disease has, my legs get so big that I cant walk. 10 days is normal, it took 2 weeks for me to find out my results. I pray that your niece situation gets better. It breaks my heart to see kids suffer. I can barely handle it and Im 18, so I can just imagine how I would deal with it when I was younger. Please do keep us updated and she and your family will be in my prayers as well as everyone else here.

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• By Sev
• Posted October 14, 2008 at 7:41 am

we will be getting my nieces full biopsy report tomorrow. my fingers are crossed and i am praying her kidney has not been damaged. Yesterday her doctor did say that so far the biopsy looks like it is MCD but wont be sure till tomorrow. They have already increased her medication and she is now back in bed not walking at all. she did start to walk a little in the last few days untill they increased her medication.. does anyone know if this is normal? the swelling in her feet did go down also but now the swelling in her feet are worse since the medication has been increased? she also has rashes on her body.. we do not know exactly what they are yet.. they look like pimples... does anyone know if these could be side effects??

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• By Sev
• Posted October 29, 2008 at 6:27 am

Hi everyone to let you all know. My nieces results have come out from her biopsy and it is FSGS. at the moment they are trying to find out weather the disease is genetic on her or not. they have started her on cyclosporine. she has many side effects such as vomiting and stomach pains ... id ont even know if these are side effects of cyclopsirine? has anyone experienced this before? She has been taking cyclosporine for a week now and a few days after she was taking them she could not breath for a few seconds... is this normal? and when is it normal to start responding to cyclosprine. it has been 2 months my poor little niece has been in hospital... no child deserves to go through this.. its just so painful for the family to watch her go through it and even more painful when im on the other side of the world.. just feeling hopeless!!!

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• By duker
• Posted October 30, 2008 at 8:05 am

Hi there,

Our daughter's next drug after she did not respond to the initial prednisone was cytoxan. It is very powerful and there is a lifetime max. dose. It has worked for several kids that I know - they remain in remission for years... for us, it did not, my daughter was then put on cyclosporine for 1.5 yrs. She did not have any of the symptoms you mentioned - except excessive hair growth. she attained her longest remission (7 months on it) - but was then moved on to prograf b/c she began to spill again..

Amos B knows A LOT about cyclosporine and the side effects - it has worked well for her daughter with fsgs - and has essentially kept her in remission for a very long time- I am hopeful that your neice will respond too! I know that NO child should be subjected to what our's have been, yet they truly are soo resiliant.

It sure sounds like your family is doing everything they can for her too. I am sure it must be extra stressful to have to go to a different country for care. Are they setup in a sort of " ronald mcdonald" house while they are there?Do you think they would ever be able to come to the US? I am sure that is probably a tall order, just wondering... where are you located in the US?

I happen to know that at Montefiore (Bronx, NY) that they have Intl programs where they bring kids from ALL over the world for major procedures.... We were there for 76 days when my daughter first presented. Kids from other countries came and went - with terrific improvement for all sorts of issues...

I know that during this acute phase, she is unmovable though.

How are her renal functions?

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