Muddy fun, compliments of rain and a few kids who love to dig up the foundation of a house on a hill!

Bullets are all my brain can take right now.

1. One doctor for the entire lot of us for Thursday clinic. I have no idea how many that is but I saw about 13 kids during the time we were there and probably another 2 in the waiting room when we left. Doc (Dr. Kind) was a little stressed, but still took time with us. Love her.

2. The appointment took a long time and Gage didn’t get to school until after 11:00 – lunch, yes lunch – is at 10:55. So we had to stop for him to eat. I think we got to the hospital at 8:30, so it was a long morning.

3. Because it was a long appointment, the numbing cream on his arm didn’t work. And yowza, was he pissed.

4. I think we are going to remove Gage’s other crappy kidney over the summer. I’d been reluctant to but have eased up on that thinking. I had thought that the surgery was a long recovery, much like transplant, but it’s not. She thinks he’d only be in the hospital for a couple of days. Which he thought was fabulous. Need to talk in depth with the surgeon.

5. I got all of Gage’s records today so I can send them to the NIH and there was some interesting stuff to read! Pathology reports with a multitude of interesting items on them! The notes from all the clinic visits with my concerns over the past 11 months and all calls of mine were noted too. It was weird seeing Gage’s transplant live columned up with comments like “Mom called and was freaking out.” Well, they didn’t actually write “freaking out” but I know that is what they were thinking during some of those calls of mine. I know this because I started some of those calls like this: “Hi, this is Gage’s mom, and I am freaking out.”

6. It’s fascinating to have all of Gage’s appointments and notes from the staff/doctors about what was going on at the time…in their words. There was a long entry from the doctor that I spoke to at this incident.

7. It’s also fascinating that Gage’s kidney biopsy “procedure note” from July was recorded on the recap/ without one mention of the mistake during the procedure with the location of the kidney (the doctor told me without hesitation it happened) but nary a word in writing.

8. Before Gage’s appointment we had breakfast at the hospital cafeteria. There’s hardly a time that we walk in the hospital that I don’t see the doctor that did Gage’s 2nd catheterthen ignored me about the problems Gage was having at the time. Interestingly enough, the notes/report from that surgery have disappeared as well. Why do I keep seeing him? He has no idea who we are either, which I guess is telling too.

9. At every appointment we go to several people come up to us and remark on how great Gage looks and ask how we are all doing. They smile, hug and are some of the most caring people I know.

10. Gage’s behavior was awesome the whole time we were there and he had a good rest of the day too, except for a minor bedtime incident, which could be chalked up to regular PITA kid stuff.

I’ve been searching my brain to find a solution to Gage’s bad-mouthing of school. When we say anything like “it’s time to get up for school!” or “let’s do your homework” we hear nothing but disdain spewing from that cute little face of his. And it’s annoying. And no good for the Universe.

It’s wearing me down frankly. And over the last several weeks the homework battle has been horrible. I finally worked out a deal with his special ed teacher to give him a break between subjects (and folks, I’m not talking mounds of homework, I mean just practicing and writing down spelling words and maybe a grammar sheet and a math sheet) and also agree with his input on the amount of time it will take for a subject…say 15 or 30 minutes. This is work that he could do in well under 15 or 30 minutes, but chooses not to, so let me clear that up right here and now. If he doesn’t finish it within the time agreed upon then we are to put it away and he will stay after school to finish it with his special ed teacher. While we do this program we remain calm and collected and follow through. We know how to do that since we’ve been behavior modficationized by Gage’s behaviorist.

Since we started this program a couple of weeks ago he’s not had to stay after school. And his attitude about doing homework has improved slightly. When I say slightly I mean that he has gone from crying, whining, fighting, throwing pencils to just complaining loudly with a contorted face, which I ignore. Sadly, I see this as a vast improvement.

I know. My standards are low. Sometimes that is what gets me through the night. Low standards.

So now I’m moving on figuring out how to squelch the negative comments about school that come out of his mouth with such ease. I’ve tried talking to him about how he has a lot of years left to go of school and homework and we should find a way to make it manageable for him; just like his meds and all of his medical appointments. “We do okay, don’t we?” I say…”We work together to find a way for you to cope.”

Even a homework assignment of a book report (or Internet report as I like to call it) about a famous African-American person that was due Monday is a chance for him to balk. It was a struggle to get him to complete it over last weekend, but he did it after 1 (or was it 2?) trip to timeout. He said what he wanted it to say and I typed it in for him. He did a pretty good job once we got going.

That was until he learned that this particular person- Granville T. Woods – left school at age 10 to work in his father’s machine shop and was working on his own taking lessons from individuals at the age of 16 in his chosen field. Gage asked if he could quit school at age 10.

“Well no Gage, you cannot quit school at the age of 10.”

And then more whining about hating school.

Ignore. Or try to anyway.

So, the new amendment to the current plan is to have a board on the fridge for each child that will be marked up as he or Quinn tell me things they like/love about school. Anything at all. This is Quinn’s chance to get to be a good influence on her brother because the girl loves her some schooling. School is Quinn’s current passion and I hope to capitalize on her enthusiasm. I am hoping that Gage’s voicing some positive aspects about school (even if he doesn’t believe them) might resonate with him a little bit.

So. We are moving on to strategy #628 in the Gage Behavior Modification plan. The boy is truly a lot of work. Hard work. And sometimes not even fun work.

Ahh…mothering the “strong-willed” child.

I was such a better mom before I had kids.

Jody,

Can you believe we are pushing a year since you gave a kidney to Gage? I can and I can’t believe it in this very moment. It seems so very far away but also like it was yesterday.

How is that for being profound?

I can’t begin to tell you what it means to this mom to know that Gage was given a chance at a better life because of you. Whenever I think about your offer and donation I feel unable to articulate inside of me what it means to Gage’s life and our life as a family.

Gage can do many, many things he couldn’t before. While most of them are good, not all of those things are (he has the stamina to argue like you wouldn’t believe!), but I will take them all any day over what his life was before his new kidney.

He can play with the best of them, run, climb as long as he has the desire to, and his current favorite thing that is related to kidney function that he enjoys is watching his weight go up. Not a single day goes by that he doesn’t get on a scale or talk about how much he weighs. He’s up to 62 lbs, which is 20 lbs+ it was when we were sent to his transplant evaluation. He’s very proud that I can no longer swing him over my shoulder with ease. A beautiful smile and giggle lights up his face when he realizes his clothes no longer fit. And he’s proud to tell us his toes are at the end of his shoes.

Never have I been so happy to replace a wardrobe. He’s growing into his older cousin’s clothes that he’s seen tucked away in his closet for years. What a joy to witness. Because of the kidney function he’s eating (hence the weight) but his food list has greatly expanded to foods other than poptarts. He’s more adventurous in every area of his life.

As you know, this past 11 months we’ve not had any major complications with your kidney. We know that we’re lucky and blessed but not as lucky and blessed as we were the day you sent that simple email asking for a donor packet. Thank you for following your heart and soul.

Thank you for giving me an opportunity to watch Gage’s transformation from sick little boy to rough and tumble little 9 year old. His desires finally match his ability.

I look forward to our private celebration with just our families on March 27th…and our public celebration with The Village(s).

Much love – always,

Gage’s grateful mom,
Julia

On an earlier post, Bree asked why were we able to donate Gage’s old kidney…

Here’s some information from the nurse that helped us facilitate getting Gage’s kidney in the hands of researchers* and how doing so is helpful. Teresa sent me pictures of Gage’s kidney cut up in many pieces…fascinating.

Teresa Chacana, RN, BSN, CPN
Research Nurse Coordinator
University of Alabama at Birmingham
Division of Genetic and Translational Medicine
E-mail: tchacana@uab.edu

From Teresa Chacana on donation:
“Thank you for your interest in our research. We have three open protocols relating to ARPKD (listed below) that may be of interest for you. Upon request, we can provide instructions for blood/tissue preparation, urine collection and shipment of sample.

The goal of the research conducted by Dr. Guay-Woodford is to investigate and identify the genetic basis of ARPKD, defective genetic mechanisms, genetic modifiers and mutations that affect ARPKD. UAB is the site of the ARPKD Database, which accumulates clinical information on ARPKD, affected individuals.

The protocols relating to ARPKD are as follows:

1 “Molecular Genetics of Human ARPKD.”
This is a study involving abnormalities of kidney development and function. The goal of this study is to identify the genetic basis of ARPKD. It requires a blood or DNA sample from the affected child.

2 “Analysis of Molecular Determinants in the Development of Polycystic Kidney Disease”.
In order to accomplish this goal, we need to study the function of certain genes in the kidneys of children with ARPKD. This study accepts tissue (kidney, liver, placenta, pancreas, lung, brain, and heart) and urine.

3 “P30 Core A: ARPKD Clinical and Genetic Resource (UAB Recessive PKD Research and Translational Core Center)”: This study offers clinical testing to participants through the UAB Medical Genomics Laboratory. The goals of this study are to establish a clinical database of patients with ARPKD and to collect DNA (isolated from blood or tissues samples) from as many of the clinical database participants as possible to compare DNA information with medical information. This is an internet-based data collection. Our web site is http://www.arpkdstudies.uab.edu
In this study, the participant/representative may choose to give us only medical information (not DNA) and/or medical information and a DNA sample. This study accepts recently or previously collected DNA, blood, placental blood and/or tissue sample for DNA isolation and analysis.”

*University of Kansas Medical Center takes them too…I think UAB sent some to Kansas (but don’t quote me).

In the car, about an hour into our drive home from our appointment with Dr. Wonderful:

Me: “So. That wasn’t so bad. Right?”

Julian: “Well, hearing about our kids’ kidney failure doesn’t shock us anymore.”

Me: “Yeah, your right…the words Acute Kidney Failure just don’t have the same sting they used to.”

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Edited to add later:

Quinn is exactly where we thought she was relating to kidney function. That high BUN (over 9 months) has all docs perplexed. Dr. Wonderful wants some other tests done (like thyroid) just to rule out anything else. Like Dr. Kind here, she can’t predict the rate of the kidney failure, but did remind us that her rate of kidney failure isn’t necessarily the same as Gage’s.

No matter how many times I ask and no matter how many different ways I pose the question, there isn’t a doctor that will predict when Quinn will need a transplant. Dang.

Julian and I are, however, not above guessing. I think within a year that we will be discussing being referred to transplant evaluation. Julian is at about a year and a half.

Am thinking of starting a family pool to see who can guess the closest on timing!

See…we’re pros.

But still. The kidney failure thing sucks. Quinn is very, very fatigued. She’s started the renal failure itching bouts that were so famous with Gage (though not as bad), and her growth has slowed a bit. Her chronically ill child status on the inside officially matches the outside now.

I guess we’re glad Quinn has the experience of Gage’s transplant to look back on and realize that she will be fine. She knows that Gage had dialysis, a transplant, came home to recover, goes to the hospital a lot for labs and that he is okay. This is what is her reality and I’m happy that is what she believes in her 6 year old brain.

And while, for certain, this time is easier for me because I can anticipate some things that will happen, it is not easier on me in many, many ways. For starters, Quinn and Gage are different (obviously) on how they will process this experience. Her disease may be different enough to be perplexing like her current labs. And I fear our good kidney karma has been used up on Gage.

Quinny B. My girl.

I fear for her.

Love us some Gameboy action at the doctors. Keeps them busy, allows us to talk to doc…heaven.

If you are by way of the PKD Foundation’s newsletter released today (or Dawn’s post)…head on over to our about page to the right. That will bring you up to speed.

And welcome. Pull up a chair and stay a while.

Julia

We’re headed when the kids are out of school to the NIH (National Institute of Health) for a study on cystic diseases. For 5 days we’ll be staying at the Children’s Inn while the kids have tests each day. It’s 5 days because they spread tests out so there isn’t pressure on the kids with round the clock poking and prodding.

I know. We’re fun parents, aren’t we?

The study has been going on for about 4 years (I think) and until now we’ve been reluctant in signing the kids up. The doctor in charge of the study has been talking to me for a long time about the study hoping we would consider getting the kids enrolled — you know, the sibling thing was a huge reason they were attracted to us and then there’s the mutant thing we have going. We didn’t do it early on because the study was limited to kids with ARPKD/CHF. It’s no secret that the kids don’t have classic ARPKD. They have the eye disorder (OMA) and they have symptoms from another cystic disease that is tied to OMA. But they have very specific symptoms that are linked with ARPKD. Because of this I had decided that our participation wouldn’t necessarily help the larger ARPKD community.

Last June at the PKD Foundation’s National Convention the doctor approached me on day one to let me know that they had expanded the study to include cystic diseases in children. After the Convention I spoke at length with her and changed my mind. I enrolled them in the Fall for a trip soon. I suspect the kids won’t appreciate the blood draws (2) nor the sedation for the MRIs (I don’t think Gage will need it, but I have authorized in case he does) but I think it will be a low pressure week as medical testing goes. And I’m looking forward to the information they will share with us at the end of the week. We are scheduled to go annually until…well, I’m not sure when, but we will have annual visits.

Even though I struggle with putting the kids through non-necessary testing, I think it’s important for us/them to have more information as time goes on as well as try to help the medical community know all they can, so it is maybe a little easier for a family some time in the future. It’s why we donated Gage’s crappy kidney. It’s why we’ve given blood to research studies. It’s important. And I want Gage and Quinn to grow up thinking it’s important too.

It’s so very hard to know if you are doing the right thing as a parent where something like this is concerned. They are non-necessary tests for their treatment but they are necessary as a parent to be armed with more information. I hope that they understand one day that I understand the position I am putting them in and I do it with careful consideration about the many doctors, children and families that can be served from the knowledge they garner from our little mutant family.

We’re headed soon to see Dr. Wonderful on our day trip with the kids. As always, I look forward to these trips because I always learn something – even if it’s not great news – about the care of the kids, their symptoms, their disease progress, or research.

Recently, I’d sent a note to her nurse about trying to get a bit of Gage’s donated kidney tissue from Dr. Wonderful’s center to send to the NIH for a study the kids are in. Dr. Wonderful sent me a note that she wasn’t sure it would be available for us…consent thing.

She asked (for the third time) if we were ready to discontinue seeing her now that Gage is transplanted – at least with Gage. I sent back an email bluntly asking if she was trying to get rid of us a patients. She called me immediately and said absolutely NOT, she was just trying to give us an out if we were tired of dragging the kids to see her. I said I was trying to give her an out if she was tired of seeing us, or if she just saw it as time for us to move on.

“No!”

“Me neither!”

Then she replied, “Let’s never speak of this again.”

Am looking forward to the appointment, as I always do. Love her.

Our very good friends Linda and Don had Baby Tessa last September. I was lucky enough to be there when she was born – not actually in the room since Linda had a c-section – but I was one of the first to see Tessa when she was minutes old.

It was wonderful to be part of her birth in some small way. It was a reminder to me of the blissful days of ignorance before any diagnosis of any disorders (OMA, Central Hypotonia) and diseases (ARPKD+). I didn’t realize how much being around the joyful birth of Tessa would dredge up my own feelings of sadness how I didn’t get that blissful ignorance time with Quinn since she was diagnosed at 2 days old. I did have a little bit of time of not knowing with Gage as he was diagnosed at a year with OMA and 18 months with low muscle tone (requiring aggressive therapy) and just shy of age 3 with ARPKD.

Before Tessa arrived, I’d forgotten what it was like to show off a beautiful new baby without one thought of a diagnosis or prognosis. I’d forgotten what it was like to only see joy in people’s eyes as they gaze upon a baby and not mixed with pity and sadness, and in some cases, relief (that it’s not their baby who is sick). I know there was a time 8+ years ago before we and everyone knew something “wrong” with Gage because of his slow development. I remember when Gage was itty bitty and I thought he was healthy (and “normal” oh how I hate to say that word in this use) and how much fun we had together, but I don’t remember what it felt like to know him as a healthy boy. I certainly don’t remember me as a mom pre-special needs kids but I know I was one. I don’t remember what the feeling was even though I remember there was a time. I long to remember what that felt like.

Friends I know have said many times that Gage and Quinn’s disease/disorder shouldn’t define me or them. But for me and us, it does. How can something that sends you in certain directions in your life – like where you live, how you arrange your job, your life, how you feel every single day – not define you?

I guess you could deny it does define you and try to live like everything is the same way before, but is that living authentically? For me, it wouldn’t work because I wear my heart on the outside of my body and am unable to separate my feelings for what I am going through with my day-to-day life and what lives in fear inside me. How do people do that? How is it possible to watch your child on dialysis, endure 100s of medical appointments, tests, pain and a have a questionable future at best and not let that define who you are at the core?

It is not possible for the person I am. Gage and Quinn’s struggles define me as a particular mother and most especially a woman. But I’m envious too if I’m honest…at how completely lucky our wonderful friends are.

I’m the person that has realized with the birth of a little girl name Tessa how much I still grieve what was lost. And that makes me incredibly sad. But holding Tessa lessens the blow of not remembering what it felt like to hold my own healthy baby. And who knows? Maybe knowing her will rekindle the feeling in me.