nephrotic syndrome

My son, Harvey who is now 21 has had nephrotic syndrome since he was 4.  He has always been dependent on steroids, relapsing within 4 weeks of coming off them.  His consultants have tried all the alternatives to steroids. He had a terrible relapse at age 14 when he swelled all over and had to be given intravenous prednisolone - the swelling resulted in stretch marks all over his arms and abdomen.  The longest remission he had was when he was 15 when he was given Cyclophosphamide.  The remission lasted 2 years and he looked and felt great.  Then he had a terrible relapse and was given Tachrylymus.  This gave him a 12 month remission but he then relapsed and was admitted to hospital - this time they got rid of the oedema with frusamide given intravenously.  He was waiting for approval for Rituximab but in the meantime relapsed badly.  He is currently in hospital with terrible oedema receiving intravenous prednisolone, frusamide, albumin and rituximab.  He is being given double the dose of rituximab once a week over 4 weeks.  He is extremely depressed.  He is one of my 4 children - the most intelligent and ambitious.  He is in the middle of a university degree but the last year and all his suffering has resulted in a terrible crush to his confidence .  I am struggling to cope . He has now been in hospital for a week today - the swelling is not going down despite all the medication , he is bed-ridden and in extreme discomfort from the swelling. I am sure there is lots of research going on - however,  because it is not such a common illness and because the kidneys are so complicated and inoperable - funding for research wouldn't be as great as eg. for cancer.  

I am scared to ask his consultant what the prognosis is.  How long was your grandson on Rituximab before he relapsed ?  It is so sad I could cry all the time. Life has been very cruel to me. I lost my mother to a terrible illness when I was 25 and my sister ( who was 20 ) when I was 26.  My sister was diagnosed with Lupus when she was 11 and was treated with steroids. She, like my son was covered in stretch marks.  She committed suicide 9 months after my mother died having suffered with episodes of psychosis.  Perhaps it would be useful for you grandson to get in touch with my son .  One of the things my son can't cope with is that all his friends are healthy and can live a normal, fulfilled life - he feels he is the only one suffering. 

did you receive my message earlier? it disappeared from my screen!

I am re-writing my message to you in case you did not receive it. It disappeared from my screen! Forgive me if I repeat myself.

Your message literally reduced me to tears. I feel SO sorry for you all and know exactly what you are going through, though of course, you also have the other traumatic things going on too which fortunately, I do not have.  

Your poor lad obviously feels he is the only one suffering and when you are that low, it is hardly surprising is it? Robert is in remission at the moment, though still taking low doses of steroids as part of the weaning off process. He therefore still has the 'steroid' face and extra weight. One of the things he has done is try to keep as active as he can. He has recently qualified as a football referee and has joined a gym. This makes his feel 'normal' and helps to keep the weight off a bit. The Rituxumab worked for Robert for about 18 months before he relaped again. They repeated it, and again, he was in remission for some time before relapses again. This drug is expensive and they do not give it lightly. Like all drugs, it has side effects, and I m always torn between wanting him to have it to make him well, and worrying about what side effects it may have. Fortunately for him, his relapses do not sound as dramatic as Harvey's.  He does his urine test fairly regularly so is ready to start in with the steroids if his levels are high for more than a coupe of days.

I don't know where you live, but we are in Sussex and initially he was treated locally, but they had little experience of the problem, so his case was transferred to The Evalina, St Thomas' in London. His Consultant, Dr Cosar [?] is fantastic. She is so interested in the problem and in fact does research as well as treat patients. Unfortunately, The Evalina mainly treats children, so he has been transferred to Guys and the treatment is nothing near as good.  Perhaps you could contact Dr Cosar or get your Doctor to do so?

As for Harvey, I have had a word with Robert and he is happy to have an email chat to him, if nothing else, to assure him that he is not alone.  I asked Robert today how he deals with it, and he just said, pretend it is not there as much as I can. Not always possible of course, especially if you are hooked up to drips and so on. Robert has great friends and his Mum made them aware of Robert's problem before they all went off for their first holiday without parents a month or so ago. This was an anxious time for us, and I am sure that underneath his tough exterior, Robert was also concerned, but they had a brilliant time.

 I have to say, I have never heard Robert say, "Why Me?" and I am amazed as I am sure I would!  Kids tend to be so resiliant, but they do need to be made aware that they are not alone in this.  Perhaps Harvey can write a Blog on this site? He will be amazed at how many people will come forward with offers of support and advice.  This is something I have been made aware since I put my initial message on. There are some truely lovely people out there! My best wishes to you and your family.