After 3 years am I still in denial?

Logan's last appointment was March 3rd. His kidneys have grown to 15.3cm and 14.7cm. This is double the size of a normal kidney for his age and even larger than an adult kidney. They have grown almost one full cm in a year. I was shocked. I knew they were going to grow but I didn't think by that much. Not sure why I thought that. Maybe because he "seems" healthy. He acts like a typical 5 year old. It is easy to let myself believe that nothing is wrong. That his kidneys are going to be the anomaly and he won't lose function. He won't need a transplant or have to go on dialysis. He is going to somehow 'beat this'. This appointment was reality slapping me back in the face saying "Don't be an idiot".


It breaks my heart thinking about him having to go through what is sure to await him: magnitudes of doctors' appt, multiple surgeries, medicines every day for the rest of his life, potential dialysis, pain from ruptured cysts (which I have heard is so bad most end up in the hospital to get pain killers that are strong enough to help), liver failure, and a whole slew of other potential issues that will make me go crazy if I think about it too much.  Just like any other parent I want him to have the best in life, but this prevents me from fantasizing about his future they way that I want to.


Logan's personality has really taken shape this year. The kid is a kidder and is always looking for the laugh. With friends over he grabbed a microphone and started doing a stand up comedy routine. He was really good. (We need to work on him not laughing at his own jokes.) I know he will take the world by storm in whatever capacity he can.


There is so much research being done for PKD these days. It really does create hope. The PKD Foundation continues to fund 15 research grants, 3 for ARPKD, to identify a treatment. In addition to PKD research, other research areas are making huge strides that offer opportunities for Logan:

• Better criteria for matching organ transplantation donors to reduce anti-rejection and immune suppression drugs
• Better methods for extending the longevity of a kidney transplanted organ to 20 years or more (reducing the total # of transplants that Logan would need)
• Stem cell research that builds a kidney out of the individual's OWN stem cells - this means no DRUGS after transplantation (not certain if an additional organ would need to be grown or if it is a one time and done)
• Artificial kidneys - this machine kidney would eliminate the 100,000 person waitlist for kidney transplants. Should be up for clinical trials in 5 years.

Logan still has 100% kidney function. Dave and I are working with his nephrologist to begin testing liver function this summer. A dear friend of mine suggested that I focus on function and stop getting ultrasounds all together. I think this is sound advice. I can't do anything about how big his kidneys get, and his function is really the indicator for how he is progressing. So if we take out the growth of his kidneys, Logan is doing GREAT! His blood pressure remains around (105-110)/60 and he starts soccer in a few weeks.


Your love and support keep us going,
Ann and Dave

Logan the Fighter

Logan had a doctor's appt. this last week. His numbers are really good. He is still at 100% kidney function. What was really surprising for Dave and I was his blood pressure. He was 98/56. This is the best he has ever been. Logan knew to celebrate as well. I told him that that is close to Mommy's blood pressure and he should be really excited. Logan LOVES his doctor. He was all smiles and even more so when he knew he didn't have to do blood work this time. YEAH!

Stunted growth is another common side effect of PKD. I have been concerned about Logan's growth over the last 6 months. I asked the doctor to show me his growth trend and he is still close to the 75% range so there is no need for action at this time.

My sister, April, wrote a book for Logan called Logan's Bumpy Kidneys. She wrote it for him so he would have a method of communicating to his classmates about his disease. Logan's Bumpy Kidneys  You will need a snapfish account to see it. It is adorably cute though. We are hoping other children with kidney issues find it helpful in having discussions with their peers. We are going to sell them and have the proceeds go to PKD research.

In addition, April and I, have found that the University of Alabama does ARPKD research which is the form of this disease that Logan has. P&G will do matching up to $5000/person for educational institutions only. April has created a direct matching to the research studies of Dr. Bradley Yoder. If you work at P&G, or know someone who does, this is a great way to give to helping Logan directly.

There is so much to be thankful for over the last few months. Today is the day of the Cincinnati/Dayton walk and I am excited about what we have accomplished so far. Hopefully all my posts from here on out will be as positive as this one.

Thanks again for supporting us.
Ann and Dave Wiesman

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.