Processing the transplant game plan

Our Hearts

From the responses I received in my last post, "The Time has Come...", I realized I was overly factual and didn't speak to how the family was feeling with this news. That is how my mind works when it comes to PKD and my family. I am black and white. I am either allowing emotion to overrun the practical, tactical, and actionable side of myself or I am solely focused on the “game plan” and how, what, and when we need to take actions to get ourselves from here to there. Let’s see how I do when I try to blend the two together.

Hearing my child has a life-threatening disease really has to be one of the absolute worst feelings in the world. Hearing there is NOTHING I can do to help my child was debilitating for me. I am inherently a person of action. Telling me to wait for anything is going to be met with some anger and frustration. This particular case was no different, and the emotional outrage that I had toward having to wait for Logan’s health to fail was put towards raising awareness, fundraising for research, and reaching out to others to find and provide support. 

Upon hearing we were ready to start the transplant process, joy consumed me to the point of being manic. All of the effort I had put into fundraising and awareness weren’t filling the void I had in my core from being able to tangibly help Logan.  While this direction is not ideal, it is inevitable and I am beyond thankful that we are taking steps to get him healthy.  My entire being is a healthy balance of being excited and terrified. There are risks and uncertainty. What if the 25 named people we have on the list aren’t a match? What if the 3^rd person is a good match and we move forward but person number 17, whom we’ll never get to test, would have been a perfect match and would have decreased his needs for anti-rejection medicine? What if they remove both kidneys and the transplanted kidney doesn’t take and he has to go on dialysis? The ‘what ifs’ are endless.  My mind spends a lot of time mulling these over again and again in my mind. My heart is happy and eager to move forward.

 

As for me being a donor, my mom came to my rescue. Five years ago, when we first found out about Logan, my mom gave me a write up from Aug 6, 1986 (typed on a typewriter) on what my kidney issues were and what procedures I had done to resolve them. My mom had requested this documentation from the nurse and had it signed by my nephrologist at the time. This document that my mother thought to keep for 30 years, in case I might need it, is one of the most thoughtful gifts I have received in my lifetime. This document has been shared with the transplant team and Logan’s nephrologist and they are reviewing whether or not I should be considered for a potential donor.  I have had a partial nephrectomy of my right kidney and my left kidney has two ureter tubes that are tied into one and happen to be flowing the correct direction down to my bladder.  In my head I know I am too risky for an already risky procedure. The parent in me says “take both of my kidneys” as long as Logan is healthy. I would prefer I be on dialysis instead of him.

Logan knows everything that we know. He knows he is ready to start the process to get a new kidney to get healthy. We are presenting this news to him with excitement and happiness. There is no reason for him to worry about things as he doesn’t have another path to choose from.  We want him to focus on the positive of getting healthy. After we told him he immediately asked if the doctors were going to use his ear skin to build him another kidney. Dave and I looked at him perplexed. He went on to explain we had read him ‘a story’ (news article) about scientists that had regrown a pig’s kidney with skin from the pig’s ear. That was about a year ago. The kid forgets nothing. He was visibly disappointed. We told him that his Uncle Jude and Daddy were eager beavers to be the best kidney candidate for Logan. Logan thought about it for a while and decided he would take Uncle Jude’s because he gets to see Daddy every day and this would allow him to have a piece of Uncle Jude with him everywhere. Melt. Logan and I calculated how old I was when I had my surgery (7 years and 128 days) and what date we would need to have his surgery for him to be the exact same age, Feb. 5, 2017.  Logan will be most likely older and wiser than I was when I had my surgery. 😄

Logan has expressed nervousness when it comes to the surgery itself which we have told him is a normal emotion and have reassured him, and ourselves on some level, that the doctors are experts at kidney transplants for kiddos so he is in good hands. Logan has been insistent on getting his picture taken with his bumpy kidneys once they are out of him. Mommy has some work to do to figure out how to make that happen. Logan also believes that once he gets his transplant he will no longer have to take as many medicines.  Currently, he is taking 7 pills a day. We have explained the medicines will be different but he will still be taking meds.  He is very disappointed that the medicines seem to have no end.

Ethan has been a great big brother. He doesn’t understand why he can’t be a donor. He told Daddy his kidneys were too big for Logan and that his would be a perfect fit.  J Melt again. Ethan is struggling to determine how he fits into this kidney picture. He knows he is supposed to look out for his brother but he doesn’t seem to know exactly what that means. He does more than he realizes by giving him very special memories of the two of them playing (and fighting) together.

So overall, the family is trying to determine what this new phase of this disease looks like and how best to navigate it. We are leaning on friends who have been through this already as well as those friends and family who are waiting for us to pick up the phone and tell them how to help. The adventure continues and we are so lucky to have so many people rooting for and supporting us.

Hugs and Love and a happy holiday season to everyone,

Ann & Dave

"The time has come" The Walrus said.

This kiddo is heading for transplantation. We meet this news with uncertainty, fear, and hope. Logan has been taking a very bad turn since July. He is very thin, anemic, uncontrollable blood pressure, and the onset of bone disease. Dave and I got this news on Monday and reacted quickly working with his doctor. 

His doctor is amazing and we are so lucky to live in Cincinnati for Children's Hospital. We had an hour strategy session this morning. Dr. D has expedited all of Logan's paperwork in the course of 2 days and Logan is now at the front of the line for transplant process in Cincinnati. We have a lot of work to do before he can be ready for surgery. He is on a new slew of medicines: minoxidil and amlodipine for blood pressure, calcitrate for his bone disease, and a new iron supplement that hopefully will stop making him throw up or feel nauseous all the time. 

We have a dedicated social worker, a transplant coordinator, and a living donor coordinator to help walk us through this process. The transplant coordinator has started negotiations with the insurance company and should have sign off from them by the beginning of January to begin donor testing. The first week of January we are hoping to get Dave and I tested. I am almost positive neither one of us will be viable candidates as I have mutant kidneys and Dave is close to 50 which is the cutoff age for consideration. However, we both feel like it is important for us to make sure we aren't candidates. We are allowed to have three people in the testing process at once. Logan will go through 2 days of testing as well after we get him ready for surgery. 

After this testing the entire transplant team will decide if his red blood cells are functioning properly. Right now Logan has massive bruising all over his legs. We don't know if they are caused by being a 7 year old or if they are caused by his red blood cells not functioning properly. If it is the latter he will need to get injections to "activate" his red blood cells. The team will also be making decisions on a single or double nephrectomy. This is the removal of one or both kidneys. Both of Logan's kidneys have lengths equal to the length of my head, yes we measured. There is no room for a 3rd kidney.  Currently he still has some function, so keeping one would be a safety net.  If the transplant failed he may not have to go straight away to dialysis. However the bad kidneys are responsible for his bone disease, high blood pressure, and can still grow with cysts while they stay inside him. 

The doctor informed us that sometimes with the new kidney the blood flow will choose the path of least resistance and funnel only to the new kidney. This will cause the PKD kidney to shrivel up over time. This is not guaranteed but would be the best outcome if one of the PKD kidneys is left behind. 

Taking out both kidneys feels ideal but it does make us nervous as we never want him to have to go on dialysis.  So... no safety net. 

We are excited that we are no longer waiting to take action. The next 3 months are going to be stressful and your support will mean more now than ever.  

BECAUSE IT SUCKS!

Hugs and Love,
Ann & Dave

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

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