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

More good news than bad

The doctor got back to us today and said the size of the cysts were approx the same. They have not spread into any other organs which continues to suggest Logan ha the recessive form of the disease. Logan's blood pressure medicine is still not helping so we will be addressing that with the doctor this week. Logan has started to complain more about his back and his side which hurts my heart a little but mostly he is still in great spirits.

We attended the webinar for children receiving kidney transplants, which I know some family members did too, and gained a ton of insight as to what to expect. There is another one on December 13th regarding obamacare's impact on pkd. If you are interested in attending please let me know.

I still cry a lot but am getting stronger with each conversation I have. Thank you for your love and support.

Ann and Dave