Agree to Disagree

I'm planning to agree to disagree with many people.  It has been said here and been stated to me personally very recently.  I do not feel that dialysis and/or transplant are a treatment for PKD.  There.  I said it, and let the attacks come.  But, please, read why I feel this way and realize that you may also decide to agree to disagree--no matter your stand on what defines a treatment for PKD.

A treatment is defined as care provided to improve a situation--especially medical procedures or applications that are intended to relieve illness or injury.  Dialysis is used as a treatment for kidney failure.  Transplant is yet another step in treating kidney failure.

PKD is NOT kidney failure.  It is a genetic disorder characterized by the growth of numerous cysts in the kidneys.  These cysts continue to grow and eventually shut the kidneys down.  Kidney stones, frequent infections, cyst ruptures, extreme pain, high blood pressure.  These are just some of the symptoms of PKD that we endure long before the symptom of kidney failure is severe enough to merit dialysis or transplant.  My thoughts dictate that a true treatment would take care of the cysts causing the eventual problem of kidney failure.

I can't look my 13 year old daughter in the eye and tell her that she inherited this awful disease from me, but that in 35 years she'll have the option of dialysis as a treatment.  I can look her in the eyes and tell her that her participation in the drug study she's in will hopefully help.  That there is research going on to find a treatment.  That the time spent working for the PKD Foundation raises the money to fund research for a cure.  That I pray she doesn't endure the pain I do. 

A treatment would allow me to not have to ask my sister for one of her healthy kidneys.  Dialysis and transplants are treatments for kidney failure; NOT for PKD.  PKD is more complicated than that.  There are years of uncertainty leading up to the point of failure.  I can't live my life thinking, "Well, dialysis is a treatment, so I'll just wait until my kidneys fail."  I live my life thinking, "What can I do NOW to improve my life?  What can I do NOW to avoid dialysis?"

Take it or leave it, that's how I feel.  I will continue my fight for a better life.  I will campaign for a cure, but will settle for a treatment.  If not for me, at least for my daughter.  She deserves it.  She has put herself out there for 12.5 million of us PKD'ers!  Let HER be able to tell her children that there's nothing to worry about.

Yes....it does!

Okay everyone....PKD sucks.....if you think there's not much else to say about it, then you are mistaken.

It sucks to be in pain, it sucks to watch those you care most about live with chronic pain, it sucks to feel tired and ill all the time, it sucks to be saddled with a 'genetic' situation, it sucks to fear for your children's health and well being, it sucks to worry about passing along a tainted gene onto future generations, it sucks to be in the care of doctors who may know as much or even less than you do about your disease, it sucks to watch your waistline mysteriously expand, it sucks to have cyst bleeds, it sucks to have chronic kidney infections and/or kidney stones, it sucks to be unable to obtain life insurance or adequate health insurance, it sucks to be to be diagnosed with a disease that you have never heard of before, it sucks to find out you have a life-threatening disease for which there is no treatment or cure, it sucks to think that your kidneys may fail and you will either have to be tethered to a dialysis machine forever or receive a life saving kidney transplant, it sucks to know that there are not enough kidneys to help those needing one, it sucks to not know when your kidneys might fail, it sucks to live with the fact that your kidneys might fail when you're too old to receive a kidney transplant, it sucks to feel frustrated and helpless all the time, it sucks to not have major financial support for critically needed research.....

Yes, I think you have to agree....it sucks to have PKD.

A transplant story

Throughout the last few weeks, myself and many of other fellow PKD families have been following an amazing story of a wish come true for 8 yr old Quinn Roberts. Quinn and her brother Gage both have PKD and their mother Julia, is very active in the PKD Foundation and heads up the Atlanta Walk for PKD. Quinn's brother Gage has already had a kidney transplant so this is the second transplant this family has had to face in a matter of a couple of years!

Quinn received her transplant on her 8th birthday! Here she is with her kidney donor Cheryl, who is a very close family friend. It is an amazing story of courage...both for Quinn, her family and for Cheryl her donor. What other gift could you give a child that would give them life? What a selfless act all organ donors do when they decide to donate.

**************Warning....the next picture is a little graphic (medically graphic that is)....so those with quesy stomachs may want to scroll past it! **********

The kidney on top is Cheryl's healthy kidney and the one on the bottom is Quinn's dying kidney. All those lumps & bumps you see are the cysts, which overtake the healthy kidney and eventually causing kidney failure. Yes, PKD SUCKS!!

Here is a link to Quinn's incredible story that an Atlanta TV station followed from beginning to end. It's a wonderful piece and highlights the incredible story behind this transplant.

http://www.11alive.com/video?maven_playerId=immersiveplayer3&maven_referralPlaylistId=playlist&maven_referralObject=1282648353

Please consider being an organ donor and put that little orange sticker on your driver's license....you never know who's life you may someday save.

Happy Birthday Quinn! And thanks to the Roberts family for sharing their amazing story with the world!

This Mom's thought process

Today I realized exactly how much we rely on routines, schedules & our calendars. Yep...I said calendars. Plural. I carry a planner that mirrors the calendar on the door at home. Not necessarily a bad thing, and it really helps. It started with a phone call from V this morning. V is in Driver's Ed right now and she called to tell me that she scheduled a drive for Saturday morning. No problem. I pulled the planner out of my purse to write it down and realized (in this order, mind you!!):

1. M & I are both working that day--how are we going to get her there?
2. K will be gone to her cousins' house for the weekend.
3. I need to call her Aunt & Uncle to remind them of her medicine schedule. (PKD Study meds every morning & anti-seizure meds morning & night)
4. I forgot to fill her pill case last night. Oops! I forgot to fill mine, too!
5. Dang! I didn't put a new pain patch on after I got out of the shower....I sure hope I have some pain pills in my purse. (I do!)
6. Dang! I forgot to take my meds this morning. Uh-oh!! Did K take hers?
7. We haven't yet logged her BP's for the month. Better get that done tonight.

Once I realized that the possibility was there that K forgot her pills, I called her. Sure enough. She hadn't taken them with her to her Aunt's house this morning. What to do?? I called M for help and he said he could run home to get them for her. (Have I mentioned how much I love living in a small town where work, schools & home are less than 10 minutes apart?!?) Since her pill case wasn't filled, he called to ask what to do. "Where are her pills? Her case is empty."

To which I replied "With the other pills."

"What does she take in the morning?"

"A whole Pravastatin. A whole Lamictal. And half of a Lisinopril. The pill splitter is right there if you need it."

"Thanks. You need to fill this case tonight."

I didn't have the heart to ask him to grab my meds. I'll just take them when I get home. :o)

Just think....all of that came from V telling me about a scheduled drive. I wonder what else the day holds???? So, we're just a normal family, right? We just happen to have some extra issues to deal with. Every family has their "something". It's all in how you deal with it. Today made me realize that all members of our household need to know what meds are taken when and who takes what. There are many ways that PKD can rear its ugly head. I would hate to end up in the hospital with a major complication and not have M & V know what to do. Both for me and for K. I carry our medical info in my planner...but I don't have it written down at home. I guess this is something I just can't wait for. Just do it! FLYlady Control Journal, here I come. Sis, I hope you're happy!!

Our Story

Welcome to our blog! Much thanks to Veronica for coming up with the idea & running with it!! :) We hope that you will find our blog humorous, insightful and helpful...not necessarily in that order.

Like Veronica, my family is affected by PKD. But my sitauation is a bit different from hers. As Veronica stated, her grandmother was the "mutant" (for lack of a better word) in her family in which the PKD started. Well for our family, this ===> adorable little girl is our "mutant". :) She is the first and only one in our families to have PKD...but it will continue on in generations when/if Payton has children of her own.

Payton was born back in June 2002 with a rare genetic syndrome called Oral Facial Digital Syndrome Type 1, http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=gene&part=ofd1 . This came as quite a shock for her father & I as the pregnancy was normal and we had never heard of this syndrome before in either of our families. Payton has endured 15 surgeries in the first 5 yrs of her life to correct the physical anomolies, the first one being at 1.5 months old! We are on a holding pattern right now for surgery as she needs to get more permanent teeth in before they can continue with the oral surgeries.

Payton also has global developmental delays due to the brain anomolies that are associated with her syndrome, namely complete Agenesis of the Corpus Callosum (c-ACC). The CC in the brain connects your right & left hemisphere and Payton's is missing. This means she has to create the synapses/connections between the hemisphere's when she learns something new. Repetition is the way that Payton learns....lots of repetition until she can finally do something that might come very easily to someone else. She was very delayed in meeting her developmental milestones-didn't sit up until 18 months old, walked at 3 1/2 yrs old and is just now potty training. But Payton is amazingly resilient, courageous, tenacious & it's unbelievable how far she has come even with all the obstacles in her way.

PKD is also associated with her syndrome in over 50% of the cases. At birth Payton did not have any cysts on her kidneys & the Dr's told us that we should start having her kidneys checked when she was around 10 yrs old as this is when most kids start to develop the cysts. Well Payton has NEVER gone by the "rules" so when her blood pressure was showing elevated numbers around age 2 1/2 yrs, I politely demanded an ultrasound. Sure enough, she had cysts on both kidneys! She was started on Enalapril to control the high blood pressure and began her yearly kidney ultrasound & echocardiogram and twice a year blood tests to monitor her kidney function. Luckily, we have only had to increase her medication a few times and we are keeping the BP under control. And so far her heart has not been affected by the high BP.

She did have a liver ultrasound in addition to her kidney ultrasound last April and they did find cysts on her liver as well. This is typical for kids with her syndrome that have PKD. She is also at risk of cysts on her pancreas as well. There is nothing we can do for the liver cysts, just monitor it along with her kidney & treat the symptoms.

PKD sucks as there is NO TREATMENT OR CURE! There is only managing the symptoms, going on dialysis and eventually a kidney transplant. As Veronica said, all we can do is wait...wait for a cure, wait to see what happens over time with the kidneys, wait to see if this disease continues to get passed on through the generations.

Veronica & I, along with many others, don't like to just sit and wait! So we are giving our time, energy & dovotion to finding a cure by teaming up with The PKD Foundation. We both are involved in volunteer positions which we feel brings us closer to finding the cure. To learn more, please see the link on our home page.

Until next time,

Christine

Welcome to the wait

If you have found this blog, you must have a little bit of a clue about what Polycystic Kidney Disease (PKD) is, so I won't go into the details at this point. However, I reserve the right to change my mind on this later. I strongly encourage everyone to visit the PKD Foundation website to keep up to date on the latest in PKD news and research.

My name is Veronica and I am one of the 12.5 million people worldwide who battle PKD every day. Welcome to my little piece of the World Wide Web that I share with my friend Christine and the occasional guest. The goal is to give you information, make you laugh, and have you realize that you are not alone in your struggle to understand this disease. Here's my story:

My Grandma was the first in our family to have PKD. She was, for lack of a better word, a mutant. Grandma passed away when I was in 7th grade. I remember her spending hours hooked up to a HUGE machine in her bedroom. She eventually graduated to peritoneal dialysis, which allowed her to remain closer to the family action while dialyzing. Grandma & Grandpa have 7 children, so the house was always bubbling over with activity. To my knowledge, 3 of their 7 children have PKD as well. Some of my Uncles have not been tested because they just don't want to know. I know that at least 1 of them is PKD free because he donated a kidney to another of my Uncles!! My Aunt is currently going through the process to (hopefully!) find a compatible donor. This brings us to the other PKD child of Grandma's. My Mom. Mom is the oldest of the 7.

My Mom had a successful cadaveric transplant 3 years ago. Prior to that, she had been on peritoneal dialysis. Mom & Dad have 6 children (all girls & I am the oldest) and 4 of us have PKD. While we are fairly certain that it is only 4 of us, we can't yet rule out my youngest sister having PKD. Until testing clear at age 30, you aren't given a free pass. She has 3 years to go.

I have known about my PKD status since I was very young. Until I hit high school, I really didn't have any difficulty that I know of. Even then my only problem was a higher than normal number of urinary tract infections. There were a few years that I was on antibiotics more often than not. That part seems to be fairly well controlled now, but chronic pain has taken its place. For about 10 years now, I have been in constant pain. I'm not talking about it feeling like constant pain...I'm talking NEVER. WITHOUT. PAIN. At all. 24/7/365 (sometimes 366!) Some days are worse than others. There have been many missed days at work and missed sporting events for my girls. Of course, there were also plenty of times that I went about my normal life when I shouldn't have...some people tell me I'm a bit stubborn at times. Shocking, I know! Trying to work around the pain is at times quite problematic. Tylenol only does so much. I have gone through trial and error with many different narcotics over the years. Some worked, some didn't. And let's not forget the ones that didn't work for the pain but I didn't care because I was in LaLa Land. Those got flushed very quickly. I am now finally on a regimen that has me feeling less pain than I have in years. It's amazing!!

Unfortunately, my story with PKD does not end with me. Remember, this is a dominant genetic disease and I have two lovely daughters. V is 15 and K is 13. When V was younger, we decided to have her undergo an ultrasound. At that point (I think she was 3 when we did this) she was found to be clear. WHEW! It is now in her medical chart that she DOES NOT HAVE PKD!! YAY!!

K, on the other hand, was not so lucky. While we didn't seek testing for her, PKD was discovered while undergoing other tests. Beginning when she was about 6 1/2, she was having some digestive issues--or so we thought at the time. Without going into too many details, I will tell you that she underwent many, many, many invasive tests over the next 2 years that ended with a diagnosis of epilepsy. One of those tests was an abdominal ultrasound. While the tech was going over the different organs, I noticed those oh so familiar spots on her kidneys. I knew what I was looking at and asked the tech to go back. She did what I asked but told me that it was absolutely nothing at all to worry about. Uh, yeah. OK. When the radiology report came back, the notes read "Numerous cysts are present on both kidneys. Not consistent with PKD.". REALLY?!? With the family history? The gastroenterologist read that, looked at me and reminded me that this was in her records. She DOES NOT HAVE PKD!! But he also told me that I knew what I saw. Leave it in her records and treat her accordingly. When she was 10, I saw a drug study opening up for children with PKD. I called, told my family history, asked some questions and told the study doctor about the ultrasound. She asked me to send her the radiology report. Diagnosis confirmed. I was told "The radiologist who read these was off their game. Your daughter most definitely has PKD." K decided to join the study and was the first child to sign up. At 10 years old, she made the decision to help 12.5 million people!! She has one final trip to make next summer for the end of her part in the study. Then we wait.

Wait to see if the study is helpful. Wait to see if K stays healthy. Wait for a treatment of any kind. Wait to see if V develops PKD. Wait to see if a 5th sister joins our PKD "club". Wait for a drug study that will accept ME. Wait for my Aunt to receive her transplant. But mostly we wait for a cure. PKD is a lot of waiting. And it sucks!!