Background information: My son has a kidney disease MPGN type 3. It seems that it's rather rare and unstudied. It's caused more than just kidney problems, it's also contributed to hypertension which caused an emergency situation involving flying him via helicopter to Pittsburgh (Children's Hospital) to save his life.

Last month I asked my son's nephrologist if there is anything else we can do since every time we tried to lower Marky's prednisone dose his creatinine level shot back up. The specialist said there are other drugs but prednisone is the least toxic and has the fewest side effects. He asked me to get a second opinion.

We went to another doctor and while he said the first doctor was right on track and did everything correct that he is willing to discuss trying other medication.

Cytoxan is the suggested medication. I've read the side effects and both me and my wife cannot decide to just go ahead with it.

Marky won't be on the full dose as for cancer treatment so it's less likely that he'll get side effects but the chance is still there. There are so many frightening things such as bone marrow damage/cancer/failure, bladder damage, immunity failure, et al.

I really wish he had the ability to help in the decision. I've already gone through so many guilt-trips during his life. If the doctor could give any sort of guarantee or percentage of success, that may help.

I'm pretty much at wits end over this at the moment so if there is any advice, suggestions, etc. please throw them my way.

Off board discussion is OK with me at the email in my profile.

I can't speak to the medications as I am not a doctor.

I did want to say that I think you are a damned good dad. May you and yours be blessed.

quote:

Originally posted by lizardman_u:
I can't speak to the medications as I am not a doctor.

I did want to say that I think you are a damned good dad. May you and yours be blessed.

Thanks, friend. Prayers are graciously accepted.

Praying for wisdom for you and your family as you go through this difficult decision. You are a fantastic father and inspire me.

Old grandpaw sending prayers your way too

How much Prednisone is he currently on, Mark?

I'm not a doctor, and the decisions you face are extremely tough, but you can only make the best decision you can make, you can't feel guilty about it.

That being said, if the prednisone is doing a good job and has the least amount of side effects, I'd be very wary of switching it out for the unknown.

Hi Mark,
I just looked up MPGN on UpToDate, the definitive evidence-based reference for physicians.
This is an extremely complex subject with multiple causes, multiple variations of the disease, and multiple complications of the disease.

There is no drug recommended for treatment among the three I saw (prednisone, cyclophosphamide or Cytoxan, and rituximab) that does not have potentially severe adverse effects.

My strongest recommendation to you would be to consider (if you have not already done so) a referral to one of the leading centers in the country involved in treating this disease, and try to get the most experienced and highly recommended specialist. Even if for a second opinion, which might involve a few visits, it could make a large difference.

The takeaway I got from the UpToDate article is that there are few high-quality randomized controlled trials (the gold standard for judging effectiveness of treatment). When that is the case, there's nothing like a caring, highly-trained, and experienced specialist physician to get you the best advice.

Prayers for you and Marky.

I don't feel like I really have the right to an opinion.
Just a question....If the prednisone is working at this time and other drugs may or may not work and may cause other problems....would it be possible to just decide to cruise on the prednisone for 5 years ( or so) and then re-evaluate ?? Maybe they will have better options by then ??
In the meantime, you and your son are in my prayers...mike

quote:

Originally posted by kkina:
How much Prednisone is he currently on, Mark?

He's been on 10mg/day for many months keeping his creatinine level hovering around 2.0 except when it was dropped to 5mg causing the creatinine to shoot up to 2.6 in one week.

0.55 - 1.30 is normal creatinine level.

quote:

Originally posted by sjtill:
Hi Mark,
I just looked up MPGN on UpToDate, the definitive evidence-based reference for physicians.
This is an extremely complex subject with multiple causes, multiple variations of the disease, and multiple complications of the disease.

There is no drug recommended for treatment among the three I saw (prednisone, cyclophosphamide or Cytoxan, and rituximab) that does not have potentially severe adverse effects.

My strongest recommendation to you would be to consider (if you have not already done so) a referral to one of the leading centers in the country involved in treating this disease, and try to get the most experienced and highly recommended specialist. Even if for a second opinion, which might involve a few visits, it could make a large difference.

The takeaway I got from the UpToDate article is that there are few high-quality randomized controlled trials (the gold standard for judging effectiveness of treatment). When that is the case, there's nothing like a caring, highly-trained, and experienced specialist physician to get you the best advice.

Prayers for you and Marky.

I have to agree here. If it is rare, find the group that treats the most. UPMC or Cleveland Clinic is worth a call. If you reach wit's end, reach out to me and I will see what I can do to help.

quote:

Originally posted by sjtill:
Hi Mark,
I just looked up MPGN on UpToDate, the definitive evidence-based reference for physicians.
This is an extremely complex subject with multiple causes, multiple variations of the disease, and multiple complications of the disease.

There is no drug recommended for treatment among the three I saw (prednisone, cyclophosphamide or Cytoxan, and rituximab) that does not have potentially severe adverse effects.

My strongest recommendation to you would be to consider (if you have not already done so) a referral to one of the leading centers in the country involved in treating this disease, and try to get the most experienced and highly recommended specialist. Even if for a second opinion, which might involve a few visits, it could make a large difference.

The takeaway I got from the UpToDate article is that there are few high-quality randomized controlled trials (the gold standard for judging effectiveness of treatment). When that is the case, there's nothing like a caring, highly-trained, and experienced specialist physician to get you the best advice.

Prayers for you and Marky.

Rituxin was also mentioned but an issue with insurance may take that of the table. We did try to get him into an out of state clinic but while fighting with insurance we found the current specialists in town that is highly regarded. He also had contact with someone in NYC that he considers a "kidney guru" (his words) that will go over Marky's records and give an opinion on the new treatment or suggest something else.

That's so much for looking into this for me. I appreciate the insight you've given. The doctor did let me look at UpToDate in the office while we were there.

quote:

Originally posted by Censored:
I have to agree here. If it is rare, find the group that treats the most. UPMC or Cleveland Clinic is worth a call. If you reach wit's end, reach out to me and I will see what I can do to help.

Cleveland Clinic is the place we tried to get to but so far the insurance is the road block.

quote:

Originally posted by mike28w:
I don't feel like I really have the right to an opinion.
Just a question....If the prednisone is working at this time and other drugs may or may not work and may cause other problems....would it be possible to just decide to cruise on the prednisone for 5 years ( or so) and then re-evaluate ?? Maybe they will have better options by then ??
In the meantime, you and your son are in my prayers...mike

The possible future issues caused by long term use is the concern

quote:

Originally posted by mark123:

quote:

Originally posted by Censored:
I have to agree here. If it is rare, find the group that treats the most. UPMC or Cleveland Clinic is worth a call. If you reach wit's end, reach out to me and I will see what I can do to help.

Cleveland Clinic is the place we tried to get to but so far the insurance is the road block.

CC is big enough where they should have dedicated individuals to battle the insurance company's for you. I am blanking right now, but there is another big heart hospital close to CC. I worked on TAVI's there. You may want to reach out to them too.

Most clinics leave the battle with insurance companies up to the patient. The insurance review board in your state is one avenue. The clinic may assist, but it is all about the money with these insurance companies.

quote:

Originally posted by mark123:

quote:

Originally posted by kkina:
How much Prednisone is he currently on, Mark?

He's been on 10mg/day for many months keeping his creatinine level hovering around 2.0 except when it was dropped to 5mg causing the creatinine to shoot up to 2.6 in one week.

0.55 - 1.30 is normal creatinine level.

OK. Not a massive amount, but definitely a long-term concern.

No medical input here but am sending prayers for you and your family.
Dealing with a very sick child is one of the hardest things a parent has to do.

Sending prayers for you and your family. Hope you can get the info you need to make a good decision on the medication.

I cannot offer an opinion about Marky's Meds but can offer a suggestion...Have you checked with Shriner's? While not as serious an issue as you are having with Marky, we took Rotndad Jr. there years ago and received EXCELLENT care.

I have no idea of their experience with MPGN but the care we received was such that they are on the top of the IF-I-EVER-HIT-A
-BIG-LOTTERY-Win list that I will donate lots of money too.

They did take my insurance info and did bill my insurance but refused to even take my co-pay.

They don't just treat childhood cancer.