The Inevitable Decline

Since we pulled food dyes from Naomi's diet in May 2011 she has enjoyed an amazingly long period of relative health. She does have a cyst in the pineal gland in her brain, which warranted five MRIs at the children's hospital to watch for growth, and a spinal tap that landed her in the hospital for a week in September of 2011, but other than that, things have been relatively calm.

When Matt and I stood over her bassinet in the Neonatal Intensive Care Unit nine years ago and listened to the nephrologist inform us that out tiny new daughter would one day need a kidney transplant, we pushed him for times. He didn't want to make predictions, but his best guess would be around the age of puberty for Naomi because her kidneys had only a limited amount of function in them, and as children tend to add more body mass with puberty, they often outgrow their kidney function at that time. Nine years ago that seemed like the wonderfully distant future.

We have waited and watched, and been constantly thankful each time Naomi's kidney function is checked and shows that it is holding steady at around 60% of normal. Her ultrasounds and CT scans always remark at how abnormal her kidneys look. They are full of cysts and scar tissue, and are far too small for her size. A CT scan report from 2011 reads, "The kidneys are diffusely abnormal. They are malrotated. There are multiple right renal lesions. There are multiple areas of bilateral renal cortical thinning, suggestive of prior episodes of infection/infarction." Her last ultrasound report summarized the findings as, "Atrophic, hyperechoic bilateral kidneys," (which means they have atrophied and are filled with microscopic cysts that make them appear bright on the ultrasound image). And yet her little, browbeaten kidneys have chugged on. We have always known renal failure, dialysis, and transplant were coming, but in some ways, we'd almost forgotten.

Last fall Naomi suddenly began to gain weight. We searched for answers--looking at possible fluid retention, checking her thyroid gland, and even locking up the snack foods at our house so she would have less temptation to overeat--yet she continued to gain. Last Tuesday I took the three girls to the children's hospital for their six-month check-ups on liver disease and celiac disease. There the GI doctor confirmed what I had begun to suspect--Naomi is showing some possible signs of early maturation. (...Insert heavy sigh here...) This could be "normal" as many girls are maturing at an earlier age now, but she only just turned nine, and I have a hard time seeing that as anything close to normal. It is also possible that that cyst in the pineal gland has grown or is causing the secretion of hormones too early, so the GI doctor wants me to check with the neurosurgeon who had followed the cyst and also to send Naomi to see an endocrinologist.

This is bad news all on its own, but grew worse yesterday when the nurse called me with last week's lab results. Naomi's creatinine (a marker of kidney function) has suddenly risen over the last six months, which means that (unless we're dealing with a lab error of some sort) her kidney function has declined quite a bit. When she was a baby her creatinine sat around 0.7 (somewhat higher than normal, but pretty good for her condition). As she's grown it has slowly risen over the past nine years to 0.9 (still not too bad). Last week her creatinine came back at 1.24, which is a sudden 30% increase and could (trying not to jump to conclusions here too quickly) put her kidney function down from her "normal" of 60% to more like 40%. End stage renal failure is between 15 and 20%, where the creatinine level would likely be around 3 or 4.

I am afraid that as Naomi has gained weight (and possibly even entered puberty) that first nephrologist's predictions are coming true--she may finally be outgrowing her limited kidney function. How fast she will outgrow the remaining roughly 40% of function she has is still a guessing game. I have heard that it can happen in a matter of months or over several years. Today I am waiting for a call back from her nephrologist to see if he got these lab results from the GI doctor's office yet or not, and to see what he wants to do now. I am guessing he will want to see Naomi in his office and repeat the blood work, since different lab equipment can sometimes give slightly different results, and that he will need to watch her more closely now.

Today I am re-imagining what going through a kidney transplant would look like for Naomi. She is still so young for that. I am praying that it was a lab error, or that we can slow her weight gain, or that there is a medication they can prescribe to slow down the deterioration--praying for a few more years. I am praying for some sort of good news. And I am waiting.