Well Balanced

The last few months have been spent balancing high blood pressure with kidney function as well as balancing emotions of fear and thankfulness.

High Blood Pressure vs. Kidney Function
A few months ago Logan's kidney function dropped drastically due to PKD's relentless cyst growth and the high potassium blood pressure medicine he was on. After multiple medicines and blood work throughout the last few months we believe we may have found a winner. His blood pressure is currently not as low as we would like but we are still tweaking the dosage to pull it down further.

FEAR

Logan is in stage 2 kidney failure. There is no more denying this disease is in our house and is starting to have an impact on him. He complains about itching, headaches, stomach aches, back aches, and other symptoms common to this disease. The switching around of medicines has made him nauseous a lot. His body hasn't been able to regulate any one of the medicines because we keep putting him on something new every few weeks. We have actively begun talking about transplantation so that he can start to formulating his feelings around it. I have called Child Life at Cincinnati Children's Hospital to get a better idea on how to expose Logan to the hospital before he will be going under the knife.  More to come on this one.

I spoke with Ethan yesterday morning on his concerns for his brother. He said "I wish I could donate my kidney now. They are the right size for him." He also said he was scared to donate his kidneys because it might hurt. I am such a lucky mommy. Logan had his ultrasounds yesterday: renal and full abdominal. His renal showed that his kidneys have grown to 16 cm. I still hate the renal ultrasound as it is a constant reminder that this disease is taking over my child's physical body and there isn't a darn thing I can do about it.

THANKFULNESS

Logan is only in stage 2 renal failure. We have finally found a medicine that appears to be helping with blood pressure. Our pharmacist was proactive and flavored Logan's new medicine with lemon, because it is his favorite, without us asking. Logan's nephrologist has been gallivanting around the world including NIH conferences to keep up to date on all the latest in kidney advancements. He never once missed responding to an email within 2 hours while Dave and I tried to navigate the medicine whirlwind. Logan's full abdominal ultrasound showed that his liver is not fibrotic and that all other organs remain clear of cysts. The Duke Clinical Trial we are participating in for genetic mapping called me for an interview this week and our blood work has been submitted for testing. We should have analysis completed within 6 months. This will tell us if Dave and I have recessive genes that created this or if Logan was a "spontaneous mutation". 

The best news of all is that the PKD Convention in Orlando is in 2 weeks and I am so excited. We will get to hear from the scientist who created the artificial kidney and learn how it works. We will get to have private sessions with two PKD researchers. One of which has shown evidence of REVERSING, not slowing progress of, PKD.

I am, as always, thankful for my family and friends, near and far, who ask not only how Logan is doing but how I am doing. Your hugs and thoughtfulness continue to make me a better mom to my two boys and I sincerely can't imagine going through this without you.

Ann and Dave

The Decline Has Begun

I am surprised at how much I think about Logan's disease now that he is not 100% kidney function. The good news is that the substantial drop from 100% to 60% did readjust itself to 80% after modifying the blood pressure medicine, enalapril, with Chlorothiaz. The bad news is his blood pressure is NOT being managed as well. This whole disease seems like a trade off of different side effects. We try to limit his red meat just because it puts so much strain on his kidneys but he is now anemic. So he is now at stage 2 kidney failure and that has occurred over the last 7 months. We have another nephrologist appt on July 15th with a full abdominal and renal ultrasound scan scheduled for July 8th.

Needless to say we have started family conversations about Logan needing surgery at some point. We have talked to the boys about our hero, Chris Schmidt, who selflessly donated a kidney to his friend and saved his life. Ethan did a verbal report on him at school. We have talked about how we will be looking for our family's hero to donate a kidney to Logan.

The other night we were lying on the floor in the family room and somehow the various scars on my tummy came up. I told the boys about my appendectomy with Ethan at 30 weeks pregnant. Then Logan asked about the "gash" in my right side. I told him that was where mommy had to have some of her kidney removed when she was 7. He gasped and asked "Will I have to have my kidney removed when I am 7?" In that moment I realized that even though I cannot donate a kidney to him due to not having 2 full kidneys (and mutated kidneys) I can be his normalcy. He may be not as scared knowing mommy went through something similar at the same age. For that second, oddly, I felt better. Like I had something to offer him that was maybe just as important as a kidney. Security. Normalcy. Relate-ability.  Not being able to donate has been the worst part of this WHOLE thing. I have felt so guilty knowing I can't help him feel better by giving him  a healthy kidney. I am finally feeling like I can give him something no one else can. I want to believe it will mean more that his mommy went through something similar and it will make him stronger.

Thanks as always for reading and your support.

Ann and Dave

Static?

Logan's doctor's appointment yesterday was pretty good. His blood pressure is staying stable at 105/60 which is very good for him. He still doesn't appear to be growing. He is hovering around 46.5" and has been about that for a year and half. The doctor says he is still in the 50% range and we shouldn't worry. Which I can understand but a kid should be growing at least a little and I keep watching him drop from 80% to 65% to now 50%. His urinalysis came back pretty standard as well. No huge indicators.  However, his first round of blood work came back this morning and his creatinine  level has jumped by 41% which has me nervous. He is way outside the range of what is normal for a 6 year old but in the range for an adult. His BUN numbers jumped also by almost 100% which also has me freakin' out a little. So far my nephrologist is not worried. So I am trying to enhance my calm. 

Given where Logan is now at 6 the doctor has indicated he is feeling pretty confident that not much will hopefully change between now and puberty. The first few years of life needed more attention as Logan's body was growing and changing rapidly. The doctor indicates we should be in a plateau period (again hopefully) until Logan hits puberty. 

We did not do an ultrasound this year as it was making me depressed and there was nothing that could be done anyway. So next year we will have a full abdominal scan to ensure none of his other organs are impacted, mainly his liver. 

I captured pictures from the walk this year and all the support Logan had because the news so far as been so happy and these made me smile.  

Thanks for following along with us on this journey. 

Hugs and love to your family,

Ann and Dave 

Latest Numbers are not trending well

We went to the doctor this week and Logan's kidney numbers are not trending well. His creatitine and bun levels are both out of the normal range which has caused his Bun/Creatinine ratio to be out of the normal range. These three metrics are all indicators of kidney failure. However, his GFR remains barely in the normal range. I have a question out to the doctor as to which of these is the best overall indicator for kidney health, but even with that answer I fear the decline has begun.

If you look over the last three years his overall GFR has dropped 41 points. If that continues he will have complete kidney failure over the next three years. Over the last 18 months the decline has been pretty aggressive as you can see from the chart below. The more cystatin C in the body the less the kidneys are functioning to remove it. He is still considered to have very high kidney function for now but I am bracing myself for the next 6 month appt.

Logan is still a happy, goofy 5 year old who plays tricks on us on a daily basis. He is excited about going to kindergarten at Mason Public in 2.5 weeks. He starts soccer again in a few weeks as well and loves playing defense. He is practicing so hard to ride his bike and hopes to have it mastered by the end of the summer. Logan took a break from piano this summer but will start back up in the fall. At a friend's house last weekend Logan swam the length of their entire pool. We may be putting both him and Ethan in a swim team this fall. 

The whole family is looking forward to the PKD walk this year on September 27 at 11:30 at Voice of America Park. If you would like to join our team you can sign up at the link below. While I am not coordinating the walk this year, I am still organizing the kids area. I may even be sitting in a dunk tank if you would like to take a shot at me. :) 

Walkin' with the Wiesmans team 

As always we appreciate your love and support. Your friendship does make us stronger.

Ann & Dave

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