After school I took both kids to the pediatrician again. Quinn was there last Monday (allergies) and Gage was there 3 weeks ago (scalp-eating rash) and we happened to get the same doctor. I went through my sob story about how we can’t get anywhere in treatment terms meanwhile my son’s hair is falling out and my daughter looks like she is a clown-in-training. The schools are sending home notes (today) about ringworm like we don’t know they have it (kind of) and they are calling us (today) like we should be surprised they (maybe) have it.

I KNOW THEY HAVE SOMETHING THAT LOOKS AN AWFUL LOT LIKE RINGWORM, except for the spots that don’t have rings around them, for starters, like the fist size, scaly patch on Gage’s head.

I gave the doc the transplant clinic number to page one of the nurses (I love them) so we could get an answer about giving him/them an oral med to combat this scalp-eating rash. Turns out that if Gage goes on it he’ll have to go into the clinic a bunch of times for lab draws to monitor the prograf levels. Enough said. We’ll try more treatment with a prescription topical ointment.

This all just made me cry, right there in front of the doctor, who sympathetically handed me some tissue. I rambled something about how it’s all just too much sometimes and how it’s never just a simple rash and a med, 8 people have to get involved to make a decision and it takes time to do all of this, which leaves my son’s head more bald by the hour, and well, it just went on from there. Because nothing says you have it together like sobbing at the pediatrician’s office for a common childhood rash (and an uncommon scalp-eating rash!).

Stressed much?

I’m just not willing to get Gage on drugs that cause him more lab draws unless it is completely necessary because he is hating the needle right now, with the passion of a wild boy with no impulse control. Because besides sobbing at the kid’s doc’s office there’s hardly anything more fun than of convincing Gage to hold out his arm!

More fun to be had as the pharmacist told me that insurance doesn’t cover the $112 tube of ointment. And then the only tube they had was torn. And then the other three pharmacies didn’t have it either.

Perfect.

And…my head and face are kind of feeling itchy.

Gage’s head thing (the not positive culture ringworm) is worse. Over the weekend it just grew. And now instead of a patch on his head having thinning hair the size of an egg, it’s the size of a fist. And the hair is falling out at a greater rate than before. The pediatrician who saw it a couple of weeks ago was reluctant to prescribe anything because of all the drugs Gage is on — she wasn’t sure how the drugs would interact. So she gave us some medicated fungal treating shampoo and we were to hope for the best!

Well now it’s worse and he has a patch of something on his cheek and now Quinn has two little patches of it and it looks like ringworm, kind of. But is the ringworm separate from the scalp fungal-eating patch? Because of Gage’s susceptibility to pick up just about anything, I’m wondering: did he bring it in and now Quinn has it because of the close poximity?

A typical life cycle for anything out of the ordinary just rocks the boat for everyone.

The Life Cycle of a Rash Noticed on a Post Transplant Kid
- One night you are helping your son shower by washing his hair and you notice a patch of hair is missing. Upon further investigation you notice it is scaly and flaky. You call your wife in a panic while she is grocery shopping!
- You call the transplant team because you wonder if this isn’t a rare symptom from an anti-rejection drug, because you think surely, if there is a rare symptom, my boy will get it!
- They tell you to call the pediatrician.
- Which is great, because our pediatrician saves daily appointments for situations like this. So, you get an appointment.
- And it is great, except for when the ped isn’t really comfortable with prescribing meds of any kind to a transplant recipient. Culture for ringworm is sent out and it’s a negative result.
- About a day or two later a rash develops on his nose and you keep (anti-fungal) ointment on it. You think the rashes are connected. When we treat with ointment it looks better. And even goes away.
- About a week or two later a sibling develops a rash. Treat.
- During this time there are calls, emails and notes from both schools. Where you explain about the rash on the head and the culture was negative.
- The scalp-eating fungal patch grows. So you say “Screw it! I’m going to the top!” and call your child’s dermatologist. Who’s appointment gal says can arrange an appointment in May! Call back tomorrow and speak with her scheduling nurse, who might be able to fit you in. You have to wait until tomorrow.
- You call the transplant team, relay that the ped doesn’t want to give meds, but your kid is looking half bald, and she directs you back to the ped or the dermatologist. Yup! You’re already on that!
- The transplant docs want you to go to the ped for things like this! But hey! The aren’t comfortable treating it with any drugs!
- So you must make another ped appointment for the viewing of the scalp-now-invading-the-face-eating rash because to just wait feels wrong, even though you know they won’t prescribe drugs.
- The transplant nurse says she will take the call from the ped if they page her and she can find out what drugs he/she can have. Because you know there ain’t no way the ped will prescribe drugs for both of the kids!
- Rearrange day to make the appointment with both kids.
- Repeat tomorrow with dermatologist.
- Realize that there is no way your mangy looking kid can have his school picture taken on Wednesday.

Gage and Quinn don’t have one physician or one team that treats them, so often it seems that there is no one in charge and it’s painfully apparent during situations like these. Well, I guess that’s not entirely true. I’m in charge.

When I think about the hilariousness of this entire situation it makes me laugh a little bit. Except when it makes me cry a little bit.

It was great to be around The Donor and her family last night. Gage and Quinn were actually kind of charming. Gage sat by me and talked my ear off the entire night and answered questions and was generally pretty pleasant to be around. And we didn’t even threaten him with him missing his first slumber party at a friend’s house tonight. I know, Jody’s eyes are closed in one of these pictures…but Gage is looking like he is so sly. I had to include it. Then there is her girl Becca with my girl.

We’re now into year two.

The scene: Picking Gage up from school in car pool.

Momma: Hey sweetie! How was your day?

Gage: Good, I guess.

Momma: You know what day it is, right? It’s the 1 year anniversary of you getting your new kidney from Ms. Jody.

Gage: It is? Because it seems a lot longer than that.

Momma: It does? Because to me some days it seems like a long time and some days it seems like yesterday.

Gage: So my kidney is one year old? Or months old? I get confused about that.

Momma: Well, your kidney is way older than that because Ms. Jody is older and her kidney is the same age as she is! But to you it is a one year old kidney! Isn’t that kind of neat. You have her kidney! And you’ve had it for a whole year.

Gage: Yeah. But it does seem longer.

Momma: Do you remember how it was, how you felt before you got her kidney? Because you sure couldn’t run or play soccer and you didn’t eat! Now you eat and you are growing.

Gage: What did I eat?

Momma: Like two poptarts a day, a slice of bread with butter, 5 cheetoes and a half a pear. A cup of milk and water. ALL DAY! That’s all you ate ALL DAY!

Gage: I did?

Momma: Well, you didn’t feel well. So you didn’t feel like eating. And you weren’t growing very good. You were little compared to other kids your age. And your brain didn’t get enough of what it needed and it didn’t work as good as it can.

Gage: So is that why I don’t know what I should know, like other 2nd graders?

Momma: You do know a lot! And you have caught up to other 2nd graders, but you have had to learn A LOT in a short amount of time to keep up with your class and you are doing great! Your brain is getting bigger! And you are getting smarter each day because you have energy to learn and that is because of your new kidney.

Gage: I can play. I remember soccer from before and I didn’t like it because it was hot and it made me itch and I didn’t like it. I hated it.

Momma: I know, I hated you itching like that too, it looked like you weren’t very comfortable and that was no fun at all. I’m sorry you went through that. I wish you didn’t.

Gage: But now I itch when it’s hot and I’m sweaty.

Momma: Well when you play you get sweaty and that can happen.

Gage: It can happen when you are dirty too.

It’s been a year. Already. A year since Gage got his new kidney from Jody. And as I sit in the quietness of my house while the kids and Julian are asleep, I can only think about the chance at a better life (and ultimately a better life) that one woman gave to one little, kidney-challenged boy. I type those words while there is a tear running down my face and a lump sits in my throat. I would sob right now out of sheer amazement of her gift, but that would stop me from trying to express my gratitude.

Giving himself a shot.

It’s been a year of firsts for Gage and our family. Since March 27, 2007 we’ve seen a child in our family without high blood pressure, and daily shots. We’ve seen a boy whose energy finally matches his zest for living. We have a boy who has normal kidney function. One who can run. And play soccer. Our boy, for the first time in a very long time, has learned a huge amount in the past 12 months academically, instead of barely, and sometimes not, getting by. Last March was the first time I believed that Gage might survive. For a very long time before transplant I thought ARPKD would kill him; by way of infection from dialysis. I see a happy child, while still behaviorally challenged, he is happy. He feels good. He has soft, curly hair we didn’t know would return after he had good kidney function. He weighs almost what an average 9 year old weighs.

One week prior to transplant, learning what will happen during his transplant

Just the past few months I’ve been able to hug my child and feel health, instead of frailty. You can’t possibly know how it feels to feel weight on his bones. It’s the one thing I marvel at several times a day…while I see him changing, when he is in his PJs, when I notice a t-shirt that hung to his thighs a year ago now shows a protruding belly, when I hug him. When he runs up to me to hug and knocks me over with his weight.

4 days post transplant

Under two years ago (at age 7) they said my boy had the same wrists that would belong to a 3.9 year old. A toddler. That’s what failing kidneys do. They rob you of almost every normal thing in your life. You are tired, feel bad and don’t/can’t eat, you sleep restlessly, your brain is robbed of oxygen that you need to learn. Your body can’t grow. All of this affects every ounce of self confidence you have.

And kidney function can and did, change all of that.

One month post transplant

Our donor, Jody, would have you believe it’s a small gift. That she was “called” and she gave and that was that. Her greatest hope besides Gage’s life being improved, would be for someone else to hear her and Gage’s story and feel moved to donate a kidney. She’s a remarkable woman, our donor. She, along with 3 other remarkable people, decided however they decided that donating a kidney to Gage would be worth the risk. All for a chance to give him a life. A different life. A life full of promise and hope.

Jody, you can’t ever possibly know what it means to this mother to watch a child get weaker and weaker and know that you and his father can’t help him. You can’t possibly know how it feels to be in awe that you followed your heart when you learned you could help Gage. And each and every day I thank God that Gage is alive and that you helped him when he needed it.

It’s impossible for me to tell you how grateful I am for your gift. I am so appreciative of Gage’s ability to participate in life. What I can tell you with certainty is that I am committed to appreciating the beauty in being his mother. Thanks for helping him live and giving me the opportunity to watch him grow.

Happy Kidneyversary. I love you.

Easter 2008

When we go to the NIH for the study, we are staying at The Children’s Inn.. By all accounts this is a lovely place for us to be for the week. People have gone to great lengths to tell us that the place is fun for kids, has tons of activities and that the kids won’t want to leave.

Not one of them mentioned that the 4 of us would be in one room with two double beds. While this isn’t a problem for vacation – because who needs rest anyway – it is for this trip. We are putting the kids through 4 days of medical testing. And I want them to be rested.

Remember when I wrote about sleep training (I know, bad parents!) and their illness affecting how much sleep they need (one of the benefits) each night? Currently Quinn goes to bed at 7:00pm and is still pretty tired in the morning when she gets up at 6:30am. Gage goes to bed by 8:30 each night and he’s tired too, but I think his is more growing, playing boy tired. I’m extremely concerned that they won’t be well rested and thus make the poking and prodding more difficult. Now, you remember that Gage just finished up the worst medical year and 1/2 of his life last year, right? He’s sick of poking and prodding. And he’s got some sensory issues, and well, he’s difficult and can be uncooperative. I’m guessing that he will be more difficult and more uncooperative than his usual difficult and uncooperative.

I called The Children’s Inn about getting adjoining rooms so that we can put the kids down and not have to sit staring at the wall until the fall asleep and tiptoe around them for 3 or 4 hours. They said they would put us down for the extra room, but probably we wouldn’t get it anyway because they will probably be full. “Well, thanks, I guess.”

A day or two later I get an email from the coordinator of the study saying that we can only have one room. That if we want to have two rooms they can move us off “campus” and we will have to travel/shuttle in to the NIH (for something like a 7am start time), and furthermore, she really thinks the kids will love The Children’s Inn, because they have so many activities. I worry they will be so tired we will have to go sit in our one room as to not disturb other parents so the great activities won’t really apply to us.

I told her we’d stick with the original plan, but warned her about the potential problems with the kids when they are sleep deprived, and told her to wish us luck.

So, that is the plan. While I am looking very much forward to what we (and researchers) will gain from knowing more about the kids particular mutation (we hope) and add to the pool of knowledge for the ARPKD+ community at large, I am very worried about the week being an intertwined exhaustive mess of kids and parents. But hey! The kids will have activities!

Maybe I should do some live blogging with photos!

Greeblemonkey (who is ridiculously funny) turned me on to what she calls a “dashboard” of sorts for news and entertainment…started by this Guy. Go here and check it out. I asked them if they could start a sub category for special needs moms. I know, long shot, right? Can never hurt to ask. And well, as a mom to two special needs kids, you kind of learn to ask, or you will never know.

Gage has has about 6 “good” days at school in his homeroom. Keep in mind this mark is on a sliding scale for him, as a “good” day for him is probably a not so good day for other kids. We think probably one of these days was a “gimme” since the teacher also wrote that she had to get onto him several times that day about listening. Because a reward of a Friday play date hung in the balance, we think maybe his teacher wanted to encourage the whole process and give him the stamp that day. Yesterday we learned he’s being very defiant in PE, which is going to totally piss off my sister, who is a PE teacher.

Quinn is doing pretty good at school by all accounts but has fallen into a few of the behaviors at home that Gage exhibits, and so we are on top of getting rid of those as they come.

We’ve been praising good choices A LOT and writing them on a chart. But I think we need a more systematic way of “rewarding” good choices. So I’m starting another chart. And then the stickers/check marks/stars/whatever are cumulative for little treats.

Gage had a hard time listening (sadly is the norm, because of his impulse control problem) over the weekend and Julian had to explain to him that is why he won’t be able to go to Scouting camp this summer where I know he would have a fabulous time. Julian explained that other parents, who would be responsible for taking him (parents would trade off) are concerned enough with his behavior that they won’t take him. I know it makes me sad. I think he really thought about it. That hits home, huh? Well, it hit me. And is further indication how far we have to go. It says nothing for how far we’ve come. It’s hard to remember how far we’ve come in the midst of being mortified (and tearful) that other parents have those feelings about your child. And well, because Gage’s behavior gets in the way of a lot of wonderful things for him.

But we had a great Sunday, with baby Tessa’s family in celebration of her baptism and Gage and Quinn both did awesome. Gage sat in church for over an hour without one behavior incident. Right before the baptism (at the end of hour and 20 minute service) I let him go to our Sunday school room where he did some arts and crafts and waited for us to pick him up. While this might not be a lot for other 9 year olds – sitting through service and then waiting for us in the room until after pictures – it is for Gage. Remarkable actually. Because usually he cannot be trusted to leave ahead and wait.

We talked all day how proud we were of both of them. The two mornings so far this week to get them off to school? In a word: challenging.

It’s a constant revolving door, this thing with behavior. It’s like the pop-up mole game…you get one and another (bad behavior) rears it’s ugly head. We’re never quite sure how we’re going to get them all, because all of them are annoying. But that mole sure is cute.

Regarding the brain abnormality…expressive language is usually affected. I’ve been thinking about this a lot since the doctor mentioned it to me. She told me that she’d read in our notes that at age 3 Gage was using 3 words sentences and that kids who have moderate to severe JB/Brain Thing aren’t expressive, or are slightly expressive. Meaning that they need technology to speak; pointing boards and computers. And their receptive language skills are usually okay.

So. That brings me to think a lot about how the kids express themselves and how it has always been a little slower for them compared to their peers. For 6 years I’ve read and I’ve heard that the kids are behind developmentally with language. So, while I knew it was the case, it was never connected to some brain abnormality. This information is just another piece of the puzzle that is our mutant gene.

The doctor who runs the study the kids are going to be participants in called to say that she had looked at Gage’s MRI from age 22 months. Surprising us, she said that Gage does in fact have an abnormality (“slight” “very subtle”) in Gage’s brain that is an indication of Joubert Syndrome. Although she mentioned that with their other issues, it probably is what Dr. Wonderful has said all along; it’s a hybrid of a couple or diseases, a potentially undocumented syndrome. They didn’t catch it at the time because it is very subtle. Here is information taken from the Joubert Syndrome website. The doctor sent me a copy of an MRI showing a normal brain and one with the abnormality where Gage’s is, but said his abnormality is much smaller.

Joubert Syndrome and Related Disorders are a group of developmental brain disorders characterized clinically by disturbances of breathing rhythm, ataxia, oculomotor abnormalities, developmental delays, and hypotonia. These disorders are characterized on brain imaging by cerebellar vermis hypoplasia and the “molar-tooth” sign of the brainstem. From our review of clinical records from patients in the Joubert Syndrome Foundation, we have found many with co-existing medical conditions at a frequency sufficient to warrant a set of guidelines for the evaluation and monitoring of all patients with Joubert Syndrome and Related Disorders. These evaluations are necessary for two reasons. First is for proper diagnosis of the child, as we find that approximately 20% of patients display features indicating a subtype of Joubert syndrome or a related syndrome. Second is to try to prevent medical complications from these associated conditions, which through proper screening might be avoided. However, because our total knowledge of Joubert syndrome and its complications are limited, our recommendations will need to be evaluated and changed as new information becomes available.

The problems that Gage and Quinn have are both related to ARPKD and JS. If it is a hybrid of a couple of kidney diseases, as Dr. Wonderful has always suggested and this new doctor suggests, maybe there could be other symptoms that manifest that are unknown at this time. What else? Is all I have to say about that now. A document about OMA that the JS site has on it is interesting and one of the few pieces that describes Gage and Quinn’s problems, except for the gene that is mutant – Gage and Quinn’s blood was tested for NPHP1 and was negative.

This new doc (who talks extremely fast…whew) is going to check with a doctor that Dr. Wonderful had sent the kids blood to a few years back to see if any information ever came from testing/research, relating to a location of a mutant gene. Maybe he can shed some new light.

As I gathered all of the kids records (a few hundred pages?) for copying, located MRI films, ultrasounds, CDs with who knows what on them from various examinations like echos and ultrasounds to send to the NIH I thought about how any new information about their specific issues or more problems we might get a peek at might impact me emotionally.

I guess more information is more and always better for us. Right?