FSGS

I have touched here on the fact that Pipo has kidney disease, but have never really talked in depth about it. What he has exactly, is FSGS or Focal Segmental Glomerulosclerosis. Basically, his kidney's excrete too much protein into his urine, instead of into his blood where it should go. This causes scarring on his kidneys, which can interfere with renal function. The prognosis for FSGS patients is usually eventual renal failure with dialysis and a transplant necessary. Depending on the severity of the disease, and how well a patient responds to treatment, this can take many years to run it's course. There is usually a series of remissions and relapses, with the relapses getting longer and more frequent. Probably more than you wanted/needed to know.

We went into all of this knowing (for the most part) what we were getting into. Pipo was very sick when he arrived last year, and there was a fear of him going into renal failure in Haiti just before he arrived. Thankfully, that wasn't the case, and our awesome doctor had him admitted immediately and he and I stayed for a week, getting his situation under control. Looking back, I can't even imagine how frightened he must have been. He had been given about a week's notice to leave everything he had ever known behind, and be dumped in a foreign land with a bunch of stangers, and then brought to a hospital where he was hooked up to all kinds of machines. He didn't speak a word of English, and my Creole was pretty limited. But by the end of the week, we were holding conversations, and he seemed to be accepting me as his support.

When we were released, it was just the beginning. He was taking about 10 different pills, 3 times a day, and in the beginning, there were daily doctor's visits. Slowly, those visits became weekly, and I learned how to do a lot of his monitoring at home. He was incredibly bloated. It's hard to even look back at those pictures and realize how sick he was. We had never known any different though, having never seen him healthy.

Very slowly, over the next few months, I watched the protein level in his urine decrease, and we were able to start lowering his prednisone doses, which helped a great deal with his puffiness and swelling. Towards the end of the summer he was put on a 3 month round of oral chemotherapy, and by the end of this, we were amazed at the difference. It looked like we were headed into remission. By October, his doctors were amazed by his progress, and the amount of meds he was on was minimal. We weaned him off his meds slowly, and by January we were med free, with not even a trace of protein in his urine. Although it is rare, his doctor told us that occasionally, an FSGS patient will go into a 'permanent remission'. I hadn't known this was possible, and didn't want to even dare hope that this was a possibility for Pipo. But He just kept getting stronger and healthier looking, and we were more and more hopeful. In a remission, it's easy to look at a child who is running and playing, laughing like any other child and forget that there is anything wrong. It's easy to pretend that they are like any other child.

But FSGS is a lifelong illness. Even if there was a chance of permanent remission, Pipo would still need to be monitored his whole life. Last week we had our check up in Boston with his specialist. It was a happy day, with Pipo looking great, and the doctor patting me on the back for all our hard work paying off. Two days ago, I got a call from their office. A closer look at his lab results showed a minimal amount of protein in his urine. I love this nurse, and she knows us very well. She has an adopted daughter from Guatemala, and we have had many a long conversation in the waiting room about our families. She is a mom, and she knows exactly how my mind started to spin when she called me that day. She tried to reassure me, and reminded me that the key words were a "minimal amount". But I know what it means. It means our bubble has burst. It means the pretending is over. It's FSGS giving us that little tap on the shoulder saying "hey, I'm still here... I'm not going away."

So we sent in another urine sample to get a more in depth look. And now we wait. I am sitting on my hands, trying to keep myself from calling the lab and screaming "Hurry up with those results!" I know it's not a horrible thing. I know that even if we are headed into a relapse, we have caught it early, and Pipo has shown he responds well to the meds. But this kid has been through so much. His specialist is still amazed that he survived 3 years of this sickness while still in Haiti. He had a wonderful doctor there, who did what he could with what was available. But without adequate labs and testing, Pipo could not be monitored. When he 'looked sick' they gave him meds. When he 'looked better' they stopped the meds. There was never any way of knowing how he was really doing. And when things got really bad, there were no IV meds available. There was no chemo. So they prayed. And somehow God saw a reason to keep this little boy going.

And now we sit and we pray. We wait for that phone call to tell us where we stand. Pipo knows what the extra labwork means. I try to play it down, but he knows there is every possibility that he will be sick again. He has been moody and quiet. I know he is just waiting for that phone call as well. He hates taking meds, though he knows they make him better. But more than anything, he hates being different. And taking a handful of meds several times a day make him different.

He asked tonight if we were going to church in the morning. I said "of course... we always go." He looked relieved. I know part of him is excited to receive communion. But I know the real reason for asking. He knows that now more than ever, we need God. So we will all go to Mass tomorrow, and we will pray. Pray for that phone call, and pray for the strength to move into this next phase of our journey.