I just got back from the PKD Foundation conference, where I was among my people. The people that truly, without a doubt, understand what we go through. Sure, our problems are different from child to child, but the same too. We’re faced with new territory in the treatment of the problems associated with ARPKD for our kids. We have the same fears. Some we speak of. Some we don’t.

In my ARPKD “family” I’m close to some of the mothers. We greet with warm hugs, smiles, and questions about how the kids are. Oh, no. Not the normal questions…how are they, but "how are they?" – or “how were the last labs?”…”how’s the BP?”…”how’s the liver?”– we talk about all of those things and it’s helpful to hear what others are going through. When we are listening to a speaker and there is shocking information to hear, we look at each other with understanding glances. The ones that say “that scares the crap out of me” and those looks are understood with no words.

I don’t always feel comfortable talking about our daily struggles and long term fears with our friends and families. That isn’t to say I wouldn’t be welcome to discuss it. I just don’t always want to burden the people in our everyday lives. One of the reasons I get energized being around other ARPKD moms is because every word can be said. It’s a safe place to land where every feeling is completely understood. We’re face-to-face at this conference, and it’s an experience we can’t replace with emails and phone calls.

I’m connected to several families, but in particular moms K and L and R. K’s son will get a transplant and double nephrectomy soon, R has to give shots to her son, travels across the country to see Dr. Wonderful for check ups, and has to contemplate bringing another baby into the world with the ARPKD risks, and L’s daughter has more liver and spleen problems of any of the ARPKD kids I know. I’m in awe of them. They inspire me and I learn from them. I can ask questions freely, without feeling like I’m talking too much or obsessing about the kids (which clearly I do). We don’t get sick of talking about things…normal kid things, and the more difficult things as well. No issues are trivial. Being with them, talking about ARPKD issues make me a better mom. I’m more prepared, I’m more committed to always find the right answers for Gage and Quinn and our family. And it’s always an extraordinary gift when I have a reminder to appreciate what we have today.

Some of us are working on getting a new national chapter started with the PDK Foundation – specifically for ARPKD. It won’t take on the form of a city chapter (like I’ve been doing) but the impact is far-reaching. During this process of getting it worked out, we’ve been exposed to more people at the Foundation and the Doctors and care providers that are passionate about treating and fighting ARPKD. I’m continually amazed at the amount of time, talent and service they provide to us. For example, over two days, I had the rare privilege of sitting with two doctors and a pediatric dietician in a social setting. I can’t believe they don’t want to run into hiding after seeing us, but they don’t. See, we tend to ask A LOT of questions in our ARPKD sessions and they still offer their off time to us – “why?” you ask. Maybe to stay connected to the ARPKD community in a way that isn’t usually possible, but really I think they sense we want more, more of what they have. Sometimes it’s hope, sometimes it’s a reality check, sometimes it’s an answer to a simple question. Why ever they do it, I’m most appreciative they do. I’m thankful that one day a long time ago, they decided that ARPKD would be an area they would put their efforts into. They help me feel confident and competent, that I am doing everything I can to be the best advocate for Gage and Quinn so I can help them live the best life they can have. They help me feel like we are normal. I don’t know how to say “thanks for fighting for my child’s life, long before they were born.” I’m not even sure that I can thank them in an appropriate way, other than to say they change my outlook on my small corner of the world, and that they have helped restore my faith in humanity and the good that is in people.

And, in this world, among my people, we think that is amazing and wonderful.

Julia

Quinn got her new braces last week. Her feet are barely a size 5 but her shoe size is a 7 because of the braces and she looks large footed for a 3 year old, and she walks a little bit like a duck. Picture a very tall, large 1 year old learning to walk and you have Quinn. She’s still getting used to the braces – so right now quick turns are dangerous.

Since Quinn was born and we’ve been thrown into the medical vortex that is our life, we’ve had to change in our heads what we thought our life would be like. We’ve had to merge our old dreams with our new hopes. And part of that transformation is accepting what our kid’s limitations are. We used to have the regular dreams that people have for their children…you know, to love God, be smart, successful, and nice – and for it all to come easily. In the new hope mind, we want good kidney function for as long as possible, we want the kid’s doctors to be partners, gentle and compassionate, we want the kids to get great special ed services, and help for their bodies with therapy, we want them to have enough confidence in themselves to be socially comfortable, to handle the medical challenges they face with grace and hope, and to be happy human beings that are nice to others.

The shifting of dreams was slow at first. At just a year old Gage was diagnosed with OMA and it was thought at the time that coordination might be a problem. Okay, not great at sports. Hard at first I suppose for Julian, but not tragic. Then, as he turned 1 ½ we learned OMA really affected all areas of development and he had become delayed. Okay, not great, but we dealt with it – jumped Gage into 5-7 appointments per week for therapy and so it began…the evaluations, the reports, the Medicaid, the appointments, and the scheduling.

It was sad really, coming to the realization that our child was “developmentally delayed.” I remember someone I met in passing (can’t even remember her name) saw Gage doing the belly crawl (he wasn’t yet pulling up to stand) and joyfully asked how old he was. “18 months” I replied with a smile. She was speechless. And horrified. I could tell she was shocked about his development. I cried that night for my son and his delays. That experience has always stayed with me as the first time my IDM (Inner Defensive Mom) felt protective for my child, and also sad about his challenges. I think that experience in part forces me to be so open about what the kids are going through. I don’t want people to be horrified or speechless…I want them to know about OMA and PKD and how it affects Gage and Quinn and our lives. I want understanding and compassion.

As sad as it was to accept in the beginning…a transformation happened while sitting and watching Gage through the one-way mirror at therapy. All of a sudden I was looking in from the outside. The sadness about his delays left me as I watched him be playful, lovely, and joyful. And I was a proud mamma as I watched as the therapists fell in love with him. After a short while it didn’t matter that he was behind. Because helping him reach developmental goals were positive and wonderful and I was thankful to be a part of it. As I watched him meet milestones on his schedule I was proud of him and not sad anymore.

Then Quinn came and an entirely different situation was in front of us: the mortality of our children. It brought us to all kinds of spiritual places. It put all of Gage’s delays in perspective: at least our children were with us. We were blessed and thankful.

Yes, our dreams have shifted. Somewhere along the way, we’ve merged our old dreams with our new hopes and our life has meaning in a way that is different than we expected, surrounded by love and caring in a way that surprised us.

So thank you for helping us find our way.

Love,
Julia

This past weekend we celebrated our 10th anniversary. Thirteen years together, 10 years married. Every Holiday and anniversary that passes, Julian goes to great lengths to tell me what a great mom and wife I am. He constantly tells me in our every day life that I’m the best mom for Gage and Quinn. And this past weekend was no exception. He planned an elaborate trip down memory lane, being chauffeured to each of the spots that were important to our life as a couple…where we met, our first date, a favorite restaurant and ending up where we were married. As we walked in, the first song we danced to as a married couple was playing over the speakers. Sweet man, that Julian.

Yes, those are important reasons why I think he is a great husband, but more so, he transfers those same sentiments and feelings to Gage and Quinn. He’s really there for them. One of the reasons he decided to join me in the business was the fact that he was traveling so much. So, for over three years now, he’s a part of the everyday life at home. Sometimes I’m sure he misses being a million miler on Delta, and the quietness of a hotel room with no "To Do" list, or being able to focus on one thing at a time. But what he gets is time with us. And almost any day of the week, Julian can be seen waking Quinn from her afternoon nap or playing with kids well before other commuters are home, taking care of the evening and next morning meds and doing the regular evening rituals. He relishes in it actually…the act of doing for his kids.

Although Julian would love for Gage to love sports and want to play them all the time (what dad wouldn’t?)…Julian is content watching his son in the yard pick leaves of different colors for me, or get excited when Gage masters a new skill on the swing set. He’s also not afraid to foster in Quinn what might be considered a challenge for her by her therapists. The girl wants to play soccer. And although she is still wobbly on the feet, he doesn’t care; he rolls the soccer ball to her in a way for her to be successful.

Julian coached Gage’s soccer team for two seasons, and had fun doing it. I can’t add up the times that Gage actually played in practice or a game to one hour. But Julian didn’t care. And as I watched him cheer all of the kids on, praise their talents, comfort them when they were upset, I thought that I didn’t know it at the time I married him, but the gentle parenting spirit he has was one big reason.

It was a lucky day that I met this man.

Julia

It’s not a pretty post today. Rarely, when you are discussing urine and the amounts of urine is it pretty.

We’ve had a ton of problems with Gage and his concentration (or lack of) of urine because of ARPKD. I’ve neglected to send an update about it, since we were trying to get a plan in place to deal with it, and really, I was always afraid when Gage is 15 he’ll find all of these updates in the journal of our journey, and he’ll be so mad. Then I started thinking, he’ll be a teenager, he’ll have a million reasons not to dig me. I’ve decided that if he can handle another person’s kidney in his body, a strict regimen of anti-rejection drugs, he can handle a little public pee discussion, back when he was 5.

I call everything that we do, around the pee thing: The Urine Factor. Because it seems to control a multitude of other things. We have to factor in where we are going, how long the drive will be and how long we’ll be there, how many changes of clothes we’ll bring, and who’ll we’ll be around that we’ll have to explain it all to, if in fact there is an accident. Not everyone in our daily routines (school friends, sports activites) knows about their problems, but it’s hard to explain the pee thing without going into all the other details. And my Inner Defensive Mom (IDM), wants everyone to know there is a reason for the accidents. I think it is harder because they look healthy.

It’s been a struggle. 1 day out of 7, no accidents. The other 6 days, 1-4 accidents. He’s been saying for a long time "I can’t feel it coming" and on top of that, he’s had some behavior/testing us about the pee thing, and I frankly, was (am) barely holding on. We’ve been trying everything in the positive reinforcement arena with little success.

I have been saying since late summer last year that his inability to "hold it" was a combination of his behavior (one of the only things he has power over) and the volume of urine he produces, and that my friends, is one bad, smelly mix. So finally, after a desperate sounding email to our Bama Neph (I’ll call her Dr. Wonderful), we are taking Gage there to discuss some medicine options. We would treat it like nephrogenic diabetes insipidus or water diabetes. So we’re headed to Bama, probably in the next month to deal with that. Dr. Wonderful pointed out that he also is just probably angry about the whole thing, hence the behavior part. I guess I can’t blame him can I? I mean, I know how I feel about it and he is only 5.

Hopefully soon, we’ll be able to have less of The Urine Factor in our lives.