I get knocked down but I get up again

I was challenged this week to determine what I would say in a letter to PKD. This is what I wrote:

I hate the uncertainty you create for the life of my child. The hardships he is destined to face because of you saddens me. The uncertainty of not only what may happen but which pieces of his childhood will be altered by you. His innocence has already been stripped somewhat from doctors’ appointments and blood work. 

Despite not knowing when your wrath will come we grow stronger as a family every day to stand against you when the time comes. My sons are educating their classmates on what you are and you will no longer be able to operate in the dark. We have acknowledged and accepted your presence in our lives but we will not embrace you. You will be defeated. Your ability to impact future generations is just as uncertain. Be warned.

The very next day we were slapped in the face at Logan's doctor's appt with his kidney function being on the cusp of Stage 3a renal failure. He has 60% kidney function right now. So despite my big tough words from less than 24 hours before my heart sank and I allowed myself to take a break and sob uncontrollably in the waiting room so Logan wouldn't see. According to the doctor's this is his "solid" kidney state. This means in the last 5 months it hasn't improved or worsened anymore than the 60%. I guess that is about the best silver lining I can find at my current emotional state.

As for his blood-pressure, we are maximizing 3 different medicines as of yesterday we are hoping that works. If it doesn't then the options are to put him back on the drug that decreased his kidney function to 60% (but have his blood-pressure under control) or put him on a medicine that makes him grow body hair everywhere at the age of 6. (*This is the medicine they use for Rogaine.)

His anemia has not improved and his growth remains thwarted. We have him on iron supplements now and will be meeting with an Endocrinologist within the next month to get his hormones tested. We also need to put him on whey protein drink to ensure he is getting all the nutrients he needs. His kidneys are 15cm and 16cm each. The standard adult sized kidney is 7 cm. With him being less than 4 feet tall there isn't much room in his belly for food so he eats very small amounts at meals and needs more meals throughout the day. We are on the hunt for a protein shake with no creatine, potassium, or sodium. Can you say "good luck with that"?

On the other side of my emotional roller coaster, the PKD Convention was phenomenal. The work being done by so many researchers is really moving forward. Logan is now part of 2 studies. The first is specific to data mining trends in ARPKD patients to formulate standards of treatment practices by sharing Logan's medical records. The second is a stem cell study where Logan's urine, saliva, and hair cells are converted to stem cells and "encouraged" to become a kidney cell and grow his kidney. This will allow researchers to see the point at which PKD influences kidney growth. To read more about the research occurring: PKD Research Grants (To the right Ethan and Logan are reading about research presented at the convention.)

It is getting harder to stay positive as this disease starts to show itself in new ways. Logan's personality remains unimpaired. He is still my little lovable goof ball who now talks very openly about "getting my new kidneys when I am 7... or maybe 8". We went to Children's Hospital for a pre-operation tour just because we have started saying "when Logan will be transplanted" as his function has dropped so drastically in a year. A 45 minute tour took 1.5 hours because the boys touched and inquired on almost everything. I was very proud of how mature they presented themselves. Mommy needed the tour just as much as the boys. I am doing all I can to prepare myself for when the time comes.

I am looking forward to the Cincinnati/Dayton PKD walk this year as I love feeling the support of my family and friends. If you are able to join us or would like to donate you can do so here: Walkin' with the Wiesmans 2016

It will take me a while to process 60% as our new norm but I have felt so much love and support in these 48 hours. Friends have reached out to hug me and spend time with me (usually without talking about it) to just remind me they are there when I am ready. It is such an odd feeling to feel strong through support but weak within. I am fortunate to have the network I do. I know I will never face any of the challenges PKD brings my family alone and together we will defeat this disease.

Hugs and love,
Ann and Dave