Learnings from National PKD Convention

Logan has a new website: http://bumpykidneys.blogspot.com

The National PKD Convention was amazing. They had a dedicated track just for parents with children with PKD. It was great/sad to meet other families who are struggling with this disease in their little ones. Dave and I walked away with a new understanding of what questions we need to ask our nephrologist for treatment processes/policies. This means we need to do a better job understanding how frequently do we look for abnormalities in his liver, his esophagus, his blood pressure, his urine tests, his blood tests, and, of course, his kidneys.

We walked away with a new found understanding of Congential Hepatic Fibrosis (CHF). This has been "explained" to us multiple times but Dr. Ryan Fischer really taught us the differences between, Caroli disease, Caroli Syndrome, CHF, and fibrocystic liver disease. These are all associated with PKD. Liver transplants are also common in PKD patients. We also learned from the other parents that height concerns are common in ARPKD kids. Dave and I have realized that Logan hasn't grown in four months. Our nephrologist responded to us while he was on vacation (I love our doctor) to setup an appt to get him tested. Dr. D would refer us to an endocrinologist to discuss options if they see he has plateaued. If Logan doesn't continue to grow while his kidneys are still growing it can cause pain in his back again due to a lack of room.

We learned a ton of miscellaneous information as well. We also talked to COTA (Childrens Organ Transplantation Association) on starting a savings account for Logan's hospitalization. It was explained to us that this can be used throughout his lifetime and we can use it as a mechanism to fundraise without getting taxed on it. :) Dave and I are still trying to determine the best use for this fund. We were shown how dialysis works for both peritoneal and hemo dialysis. We talked through if dialysis was needed we would go the peritoneal at-home route. We learned from a child social worker from the Kansas city Children's Hospital about how to transition care to your child so they can manage their own health concerns as an adult without it being "dumped" on them when they are 18. We heard from a gentlemen who had a transplant at 10, who did not transition his care into adulthood well, and ended up needing another kidney because he didn't take care of his first gift. It was heart wrenching.

We heard from the NIH (National Institute of Health) on how funding is distributed for research. The foundation is working on getting $1.5 Billion dedicated to kidney disease research over the next five years. The research going on today ranges from stem cell research to grow healthy kidneys, to cancer medications to reduce cysts in the kidneys and other organs, to understanding how the primary cilia communicates with the kidney, and many other areas within the kidney to improve function. These are exciting times.

The biggest take away from the convention was new found friends: a couple local to Cincinnati\Dayton area who were recently diagnosed, a mother who lost her son to ARPKD 3 years ago, and a father whose new set of twins had one diagnosed with ARPKD.

We are so grateful for the community of PKD families to help us figure out what we should be doing to advocate for Logan. We are grateful for our PKD in Children Chapter Coordinators who keep us connected and learning from each other. We are grateful for our family and friends who continue to love us and support us as we take this journey together.

Ann and Dave Wiesman

Annual Ultrasound

Logan's belly has appeared to be reducing in size as he gets taller and taller. When we walked into the ultrasound today I completely expected them to say his kidney had grown slightly not almost a full cm each. All of his other organs were investigated as well and while it took forever, his other organs appear to be ok. The normal size of an adult kidney is between 11-14 cm. Logan's are now slightly larger than 14 cm each. One of his cysts is 1.3 cm which absolutely terrifies me as PKDers have told me it is excruciatingly painful when they burst. Sigh. We still have 100% kidney function for which I am thankful.

Today was rough.  Luckily Christmas and family is right around the corner. Next update will be after the echocardiogram in February.

As always thanks for supporting us,
Ann and Dave

Let that ray of sunshine take all the dark clouds away

While I was on the www.pkdcure.org site today I saw this latest update in research and 
my heart soared. 

Vitamin B3 Research

I need to do more research around vitamin B3 and if I is something I can get my hands on. 

Logan is doing well. Our next appt is aug 16. His blood pressure is down to 110. 
We are still hoping to get it down to 100. 
He got glasses and he is even cuter now. I know I didn't think it was possible either. 

Thank you as always for you love and support.  Currently the status quo remains. 

Ann and Dave

Ramblings

Logan remains the same. High blood pressure and playing around with meds. His kidney function remains normal so we are very happy.  He will be going for an echo-cardiogram this coming Friday to see what the blood pressure has done to his heart. We finally have a goal for it 100/60 which is the 50% of normalcy.  He is currently at 115/74.

On another note, I have been a member of the PKD in Children support group on facebook and I am now faced daily with the severity of this disease.  No amount of research can prepare for the posts from a new mother that just loses her child to this horrific disease. I have gotten to hear from 5 in the course of 2.5 months. It breaks my heart and I cry profusely and then realize how lucky I am.  Lucky that Logan's health hasn't hit bottom, lucky that each day he becomes stronger to be able to fight when something does happen, and lucky that I have amazing family and friends to support me. I have also read amazing stories of children with football sized kidneys at birth living through nephrectomy, dialysis, and transplant. This gives me hope. However, at the National Kidney Foundation walk we met a woman who had just lost her 23 year old daughter. Even with a transplant, there were "complications". I will always have to worry about how this disease will impact him. It doesn't end as he gets older. It doesn't end when he gets a transplant. It doesn't end when the doctor says he is healthy. There will always be follow up visits and checkups to ensure everything continues operating as expected.

I very much dread the day I have to tell him his children will have a 50/50 chance of inheriting this.

Dave's Returns from NYC with too much information

PKD Conference for Parents with Children

This conference was meant for parents with children to come together and learn from the experts on what having AR and AD PKD means for their little ones.  Approximately 20 parents attended with five specialists including: Chief Pediatric Nephrologist (Kidney doctor), Pediatric Hypertension specialist (Blood Pressure), Pediatric Hepatologist (Liver doctor), a renal dietitian, and a pediatric social worker. Dave walked away with so much insight we now have a gameplan for Logan's blood pressure and how we ensure we are part of the next round of genetic testing at the National Institute of Health.  We also walked away with the continuing realization as to how unique this disease is and how there are so many unanswered questions around it. Every form is slightly different which makes it insanely difficult to map the DNA for finding a treatment or cure.

Good news:

1. Talvaptan medicine currently under testing for treatment of ADPKD is basically a crap ton of water being pushed through the system. The recommended amount of water a day is approximately 4 L or 120 ounces for our little Logan.  
2. Caffeine exacerbates the growth of the cysts so we need to cut down on chocolate and coke is completely abolished now from our home. (Haven't let go of coffee yet)
3. Lowering blood pressure to under the 50th percentile can add 2 years of Kidney function for every 5 years of life. *As I said we have a gameplan.
4. The entire conference was recorded and will be posted in the next few weeks. 

The best part of the whole conference was meeting parents with children similar to where Logan is and sharing stories.  Surprisingly we weren't the new kids on the block either.  One couple had found out a month ago their four year old has it.  Dave shared knowledge and we received knowledge. It was a great event.

In other news: September 22nd is the date for the PKD walk this year.  Dave and I are coordinating so save the date.  

PKD Leadership Conf. Day 1

This PKD Leadership seminar has been very eye opening and very heart wrenching. I have been shocked at how flippantly death is discussed here.  It makes me realize how sheltered my life has been up to this point and how completely unprepared I am for what lies ahead.  With stories of a 6 year old going blind from kidney/liver transplant gone bad to a 6 year old maintaining 7 years of 100% kidney function up to his 13th birthday, I am slowly beginning to realize that there is no normal outcome for this disease. There is depression, extreme pain, multiple organ transplants, side effects of the transplants, dialysis, and even death imminent for my beautiful baby boy.  As a mom I am supposed to fix this.  I am supposed to make it better and I am helpless. I have learned a great deal about the disease from the people here and I have enjoyed talking to them. At the end of all this they will make me stronger and able to help Logan more.  They will also provide a great support network for Dave and I when the hard times start to unravel.

Good news -- Logan's most recent set of blood results came back with 100% kidney function. His blood pressure isn't great still but has dropped.  We are going to keep trying to lower it.

As always thanks for thinking about our family and keeping those positive thoughts coming our way.

Ann

More drugs for the little one

This one is a little late.  Last friday we had to go back to the doctor because his blood pressure was still not under control.  We got put on additional medicine to help bring it down.  We were smart enough this time to ask about side effects.  This new medicine can cause a dry cough, increase in potassium levels, and a potential to worsen his kidney function.  So we are still on the big gum medicine and this new medicine that requires us to go next month to check potassium and kidneys again.  He said we are keeping our February appt to check kidney function too.  The next ultrasound will not be for a year.

In addition Dave and I have been asked to coordinate the PKD walk for Dayton/Cincinnati for 2013.  This will be in September.  We are hoping to have it be even bigger than last year. We are very excited about this and will be going to Kansas City in Feb to meet the board of directors and Chiefs of the foundation for a weekend.

If you hadn't heard there was a break through this year for ADPKD with a new trial drug called Talvapan.  It is NOT a cure but it seems to be slowing down the growth of cysts. This is only in ADPKD patients and we are still assuming Logan has ARPKD.  We can do the DNS testing to find out but it costs $5000 and is not covered by insurance. This test would currently not help us as talvapan is not perscription ready.  More tests will continue in 2013. So we are holding off on the test.

As always we appreciate you reading this and keeping up with what is going on with our family.

Love and hugs,
Dave and Ann