Control in the Fish Bowl

Hi all, this is Jeff.  I am going to do my best to frame up our lives from my perspective.  There are a couple of things that I struggle with on almost a daily basis:  how public our lives have become and how little control we have over our lives.  There are many more things to be thankful for.

Our lives have become so public.  It seems like our family has been put on display for everyone to see. Facebook and our website have allowed us to reach a large amount of people and spread the word about Charlie's condition and our family's struggle to tread water during all that we have endured.  Don't get me wrong, I think the awareness that everyone has about Charlie and our family is a testament to how much people around us care and to what ends our family and friends are willing to go to support us.  For that, we are truly blessed.  I would like to unplug at some point and retreat to our hide-away and enjoy things the way normal families do, hopefully that is coming in the not-so-distant future.

For the most part, we have a pretty firm grasp on Charlie's dialysis.  Things had been cruising along fairly smoothly until a couple weeks ago.  We noticed that the deliveries of the supplies were becoming smaller and smaller as we went along.  It finally got to the point where we ran out of one of the necessary supplies and had to improvise to get Charlie connected and disconnected.  Our current situation is stressful enough without these inventory related issues causing more grey hairs to show up...When telling the nurse about this shortage, she told us that she would provide the supplies from the hospital and instructed us that we needed to order more than what we need to prevent this from happening in the future.  Hopefully the dialysis phase will not go on for too much long;  Jessica, Kari and John Kral (Sarah's siblings) have been going through the process of becoming a donor.  I have too.  There is a 2-day transplant evaluation process that I will be going through on June 17th & 18th.  If all goes well, I think we can start talking about transplant.  To be completely transparent, this is a scary process.  I know there are no guarantees.  But I will do ANYTHING I can to help ensure that Charlie has an opportunity to live a full life.

God has blessed me with Sarah.  Sarah and I, while struggling through the stress, continue to grow with each other and I believe that we are stronger now than we were last year.

Natalie is becoming quite the tall, vocal young girl.  She is rapidly approaching 4 years old (August 3rd) and is wearing 5T clothing.  She is still my little peanut.  Natalie carried on a 5 minute conversation with her cousin Anthony tonight and I don't think Anthony got more than a "hello" in.

Charlie has been out of the hospital for almost 4 months now.  It doesn't seem like a long time when I say it, but when we were living in the hospital for long stretches of time, it seems like much, much longer.  He has grown more than 2 inches since he started dialysis.

Willa is growing like a weed.  She is 3 months old now and I'm sure Sarah is not looking forward to having a 3rd mobile child to watch after.  It seems like she is recognizing me more and more and seeing her gum-filled smile is really wonderful after a stressful day at work.

I want to mention poor Hank.  He is not receiving any attention from Sarah or myself and has become a jungle gym for Natalie and Charlie.  He is still the same lovable, whiny meat-head.

Thank you for all you do.  We appreciate everything that people have done to support us.  God has put us exactly where we need to be and surrounded us with great people.

-Jeff 

Life

I have thought about writing more often but I find it hard to to catch my breath. Our lives are more busy then I have ever imagined, with Natalie and Charlie running around and baby Willa in tow. Everyday has its challenges but at the end of the day we are grateful we have each other. 

Natalie is growing up so fast and becoming her own person. She knows what she likes and asks over and over to do things that she likes and if she doesn't like something she will let you know with a tantrum. We are working on her to show her emotions in a different way then tantrums but it takes time to train, some days she does great and says "Mom I'm sad we have to go!" When she says that it makes me so proud of her because I know she wants to throw that tantrum and she is trying not to. She is also loving being able to pick out her own outfits everyday. Right now she loves to put little books in her bag and to set up picnics with her play food. She also loves to play with Charlie and to help me out with Willa. At almost 4 she is such a little adult at times, just growing up so fast.

Charlie is amazing! With everything he has gone through the last few months he still manages to find joy in everything he does. I love hearing him laugh when he is really into a movie.  Everyday he is learning more and you can see his pride in it when he does something new. During the day he seems like a normal kid without any problems, he is walking, running, climbing and talking. Just last week he started going up the stairs again now I can't keep him off. When just a few months ago he couldn't do that because he was so sick and swollen that he wasn't moving and had stopped talking. Since starting dialysis he has re-learned everything. Even though Dialysis keeps him going its still challenging for everyday life. Charlie has to be hooked up everyday for 12 hours, that means we have to start giving him his meds and doing vitals by 7:30 so he can be hooked up and in bed by 8 pm so he can be unhooked by 8 am. If we have to leave in the morning everything is rushed, we have to unhook Charlie then give him his meds and do his vitals, not to mention getting everyone else ready fed and out the door. Dialysis helps Charlie to live right now but he needs a kidney transplant to save his life.  When he goes to his Dr appointments he says thank you to the lab tech after he/she draws blood from his arm so they can test it. If its a girl he will blow her a kiss good bye if its a guy he will give knuckles. Even though he knows that the lab people cause pain I want him to still have manors and because he says thank you and blows kisses EVERYONE knows who Charlie is. They also know who Charlie is from his scowl. He hates being touched by medical people and will let you know with a scowl as they walk in the room and a tantrum when the touch him, but he will always say thank you and if its a girl he will blow kisses and if its a guy give knuckles. 

Willa is a easy baby till about 8 pm. She sleeps most of the time. She is a very happy little girl during the day. She is fussy from 8 to 10 or 11 pm then sleeps most of the night waking once or twice to eat. At her 2 month appointment she was 100% in height and 90th% in weight. She is a little blessing.

Q & A: Our Lives

Q. How long has Charlie been on Dialysis?

A. He started on January 27th.

 
Q. How does home dialysis differ from hospital dialysis?

A. Peritoneal Dialysis (PD) is done every night for 12 hours.  This form of dialysis is performed at home, but only Jeff and I have been trained on how to administer the dialysis. Hemodialysis (HD) requires a trip to the hospital 3 or more times a week and be hooked up to a machine that takes his blood out of his body and cleans it. He would have to be hooked up for 4 hours without running around or eating during that time. Not to mention the only hospital that does it for little kids is an hour away from our house at Amplatz.

 
Q. How long is Charlie on dialysis each day?

 A.12 hours every day. 

 
Q.How is Charlie different now that he is on dialysis?

A. He is back to our trouble making little boy. He is such a trooper

 
Q. Is the home dialysis machine portable?

A. Yes, it even has a case that it fits into.

 Q. If you can take it other places, what all do you have to bring?

A. We have to bring the Cycler (dialysis machine) and what ever bags of solution we need. If we go out of state we can have the bags of solution sent to our hotel. 
 

Q. Is Charlie still on so many medications now that he is on dialysis?

A. He is on 3 different blood pressure medications, one thyroid medication, and 5 other vitamins and supplements.

 
Q. Is Charlie cooperative about the dialysis?

A. Yes, we put a TV in his room so he can watch a movies when he is stuck in bed awake. Since he is hooked up for 12 hours a day he often times doesn't sleep for 12 hours and needs some entertainment. 
 

Q. Does Charlie still need a transplant?

A. Yes, there is still a need for a transplant. Unless God heals him there is no other option but a transplant.
 

Q. If yes about the transplant - what is the timing?

A. As soon as we find a donor and can set up date with the surgeon and the donor.

  Q. What are the transplant people looking for as a donor match for transplants?

A. Someone between 18 and 50 in age and in good health. They told us that anyone can be tested to be a donor. Some people may not be a match for Charlie but they could swap with another donor that isn't a match for their kid/ friend then they would give their kidney to Charlie. Its called paired exchange. to be a direct donor you would have to have A+ or O+ blood type since charlie is A+.

 
Q. I know Charlie lost a lot of muscle mass when he was hospitalized so much this winter - how is he doing with re-learning to do things like crawl and walk and play and talk?

A. He has bounced back so fast since starting dialysis. He started crawling again soon after his 2nd birthday (2-9-14). The day Willa was born Charlie started walking again (2-24-14). Ever since then he has been acting like a normal 2 year old. He is talking all the time. 

   
Q. How is your family doing?

A. We are doing really well considering whats going on. Jeff and I have both accepted what is going on with Charlie which makes our lives 'easier'. We still wish that this wasn't going, on and Charlie was healthy. Some days its hard  but most of the time its our new 'normal'. Now that he is on dialysis it is much 'easier' as we don't have to worry so much about going to the hospital because dialysis helps keep him healthy. Although any sign of sickness Jeff and I are talking to Charlie's nurses to see if we need to bring him in. We are all happier not living at the hospital. We have a really hard time thinking about his Transplant. He will have a huge incision, he will be in the ICU for a week after the transplant and then in the hospital for another 2 weeks; so 3 weeks total. That just breaks my heart. I know it means he will be 'better' but what are baby has to go through to get better is something I would not wish upon anyone.

  
Q. What help can people provide to your family?

A. Meals, donations (to help with ALL the medical bills), prayer (pray that god will heal Charlie), company (since we can't leave the house as easy as we once could), support, and help with the house (cleaning, construction, landscaping anything to help us finish our house so we can move into a house that will better fit our family).

  Q. What happens if a donor match kidney is not found?

A. Charlie need's a kidney they will not stop looking till we find one. Right now he is on an inactive donor list that will become active if we can't find a match with in our family and friends that are being tested.

 
Q. Is there an ideal time to get a donor kidney?

A. As soon as possible.

Q. How is looking for a kidney for someone so young different than an adult who needs a kidney transplant (if anything)?

A. I'm not sure except I know that the younger you are the higher you are on the list to receive a kidney. Kids can and do have adult kidneys in there body.

Q. What is the procedure to hook Charlie up to dialysis and also to unhook him?

A. Check previous blog post. A Day of Dialysis

 
Q. In addition to dialysis are there other things you need to do each day to monitor Charlie?

A. We need to take Charlies blood pressure, temperature and weight 2 times every day (morning and night). Then he also need his medications every day.

 
Q. Are there results of some sort you are recording? Do those results get sent to someone who is monitoring Charlie?

A. We record the numbers from the cycler and Charlies vitals every day and once a week we give them to a nurse that talks to Charlie's Dr. about the numbers.

 
Q. Does the dialysis machine automatically send results (is there an internet connection on the machine?)

A. It does not and no there isn't internet on the cycler.

 
Q. How often are you discussing results with those who are monitoring Charlie?

A. Once a week. Unless we are having a problem then we can page them any time day or night.

 
Q. Do you have a normal range for results (whatever those are) so you have an idea that the dialysis is working?

A. Yes we do.

 
Q. Can Charlie roll over in his sleep and stop the machine from working in some manner?

A. He can sleep in any position he wants on occasion he will kink the line or need to be flipped over due to a low drain.

   
Q. Does Charlie have to be in bed when he is on dialysis? If yes, how is he doing with that? Does he seem to understand?

A. Yes, he does need to be in bed. Its normal for him now that he has been on it for a while. When he first started on dialysis he was to sick to fight being hooked up and now that he has been on it for a while he is used to it. Yes, there are times when he doesn't want to be in bed or he wants to be held, but for the most part he knows it his time to be in bed.

 
Q. How is Natalie doing with all of the attention Charlie needs and now with new baby Willa?

A. Natalie is AMAZING! She is my biggest helper. She and Charlie play so well together, it is so awesome to watch. When she wants mommy or daddy she tells us she need a snuggle. 

 Q. Do you receive updates on routine basis regarding Charlie's priority on the donor list? 

A. He is on an inactive list right now as we are testing family and friends first. 

 Q. Does he have a number at this point?

A. Not at this point but because he is so young he will be at the top of the list if there isn't a match withing the family and friends that are being tested.

Q. Does he have any priority because of his age?

A. Yes, he will be at the top of the list if he needs to go on the list.

Q. Is the dialysis frequency difficult to manage, especially with a 3 year old and a newborn?

A. It can be, Jeff and I try to start getting him ready for bed between 7 and 7:30 so he can be hooked up at or before 8. Some times its a challenge with the other kids but for the most part Jeff and I are a good team and can get it all taken care of with time to spare. 

Q. Are you both exhausted?

A. Most days I would say yes we are. Charlie wakes up a few times during the night, Willa is a night owl and also wakes up during the night and Natalie is an early bird. In any given night Jeff and I get out of bed to help kids at least 2 times but often its more then 2 times. Jeff and I do not sleep much at all but surprisingly can function all day. 

Q. How often does Charlie get sick like the other night that you were up with him all night, but he was better in the morning?

A. Because his kidneys are failing at such a rapid rate and he is in end stage renal (kidney) failure we have to be very careful to not expose him to sick people. He has a weakened immune system. Anything could send him to the hospital.  

Q. How often do you need to take him to the doctor(s)?

A. Once a month while he on dialysis. after his transplant it will be weekly.

  Q. Do you need anything? Diapers? gift cards? Money?

A. This is a hard question to answer. Yes we do but if I say we need diapers we will be blessed with to many boxes of diapers to sore them all. Gift card to target to buy diapers would be better. or gift cards to The Home Depot would be good to as we need to finish our house so we are living in a finished house. If you have ever been to our house you will know that is it always under some sort of construction. Money is always helpful. We have so many medical bills its hard to justify spending money on the remodelling that needs to happen.

Q. Has Willa been tested yet?  

A. No she hasn't    

Q. What will life be like after his transplant?

A. Life will not be easy. He will need medications every day at the same time for the rest of his life. It will be to prevent his body from rejecting his new kidney. For the first year every time he is sick we have to go to the ER to make sure its not his body rejecting his new kidney. Some of the medications he will be on could cause skin cancer and other kinds of cancer. Every time he is outside we have to lather him up with sunscreen and keep him out of direct sun light if we can. They say the first year after the transplant is the hardest. I'm not sure what could be harder then the past few months of our lives so I'm not looks forward to the first year after his transplant. He will have to have his blood drawn 2 or more times a week and have to see his Dr. at least once a week. 

Thank you to everyone who asked all these questions. If you think of any other questions just ask in the comments or send an e-mail and I will add them. 

Also please feel free to share our website hoeftfamily.com and or our blog thehoefthomestead.blogspot.com  

Willa Christine Hoeft

Willa was born on February 24th at 2:25 pm.

She was 8 pounds 14 ounces and 19.5 inches.

Labor was fast and had no complications. 

(Photo taken by Danica Donnelly)

I love Willa's Name. 

She is named after my grandpa William Clarence Hearst.

Willa is the female version of William.

Christine is my middle name.

Hoeft is our last name.

WCH

(Photo taken by Danica Donnelly)

This is our first family of 5 photo, thank you Danica!

We didn't find out if we were going to have a boy or a girl but we both thought Willa was going to be a girl.  We were right! Natalie would tell people that she was going to have a baby sister that was a boy. (I'm not sure she knew that sisters are girls and brothers are boys even though we explained it to her.)

Sisters!

(Photo taken by Danica Donnelly)

We are all so happy that Willa is here. She is such a good baby
 Whenever she cries Charlie asks "What happened Willa?" and Natalie says "Mommy Willa is hungry!"

A Day of Dialysis

Peritoneal Dialysis for Charlie.

Morning:: 

We start the morning by disconnecting Charlie from his dialysis cycler. In order to do that we need to put on a face mask wash our hands with antibacterial soap and dry them with paper towels. Then we go into his room and put mild bleach on to clean gauze and then we have to scrub his transfer set (the end of the catheter that is outside of his body) for 60 seconds to make sure its clean and then soak it with the gauze for another 60 seconds. Once that is done we disconnect the tube and put a cap on the transfer set for the day.

Next we draw up his medications and give them to him. There are about 10 different medications and vitamins he gets in the morning. 

Then we do his vitals: blood pressure, weight and temperature. We also have to disconnect everything from the night before and dump all the fluids down the drain and record the numbers from the night before.

Night:: 

We start the night by giving Charlie his night medications. There are only 4 at night and a shot 3 times a week.

Then we take his vitals again before bed. 

Generally, while one of us is doing meds and vitals the other is setting Charlie up for the night. 

Set up we start by putting on a face mask and washing our hands just like we do in the morning. Then we get 2 3L bags of dextrose and 1 1L bag  and we get them all hooked up to the cycler 

Once that is all set up we can hook up Charlie. Its the same way we disconnect him in the morning but in reverse. 

Then Charlie is hooked up in his bed for 12 hours we aim for 8 pm - 8 am.

 

Charlie's Hospital Stay Starting on January 20,2014

On Monday 1-20-14 Charlie got sick with what we thought was a stomach bug.

He had stuff coming out of all ends and had a fever of 101.

Most kids would not have to go to the emergency room for one day of the all of this, as most of you know Charlie is not "most kids". So off to Amplatz Children's Hospital we went. They almost did not admit Charlie but right when they were going to send him home he threw up his medications in front of the Dr.

We were told he would be there over night and then most likely get to go home...

A few days passed and they were still not letting Charlie come home, in that time they had told us he has the virus RSV, but RSV shouldn't keep him in the hospital. We were confused and a little annoyed that we were not able to go home because we wanted our little guy back home. I can't quite remember what day it was but they finally told us why they were keeping him so long and it wasn't because of Charlie's RSV it was because of Charlie's kidney's. Every time they drew blood and tested the blood it was showing that Charlie's kidney's were failing more and more by the day. They were drawing blood twice a day and each draw his kidneys were getting worse. The Dr. told us that Charlie needed to start dialysis as soon as he could. So they set up surgery for Friday the 24th as it turns out Charlie was to sick with the RSV to get general anaesthesia so they decided to put a central line in one of his arteries in his neck so they could give him some medications that are a little much for a regular IV to handle also so they could draw blood from the IV and not have to poke him all the time.

This also meant that Charlie had to go down to the ICU because the Medications needed to be closely watched. Friday, Saturday and part of Sunday Charlie was on oxygen because of his RSV. 

The Dr. also told us that Charlie will not be on dialysis long because he will need a kidney transplant soon. We had genetic testing done on Tuesday the 22nd and that takes about month to get all of the results back. Once we have the results back we can start looking for a kidney donor and get a transplant as soon as we find a match. Once he gets his transplant he could be in the hospital anywhere from a few days to a month after word depending on how well his body takes the new kidney. In most cases they leave the old kidneys and add the new one but because of Charlie's disease they need to remove both of his kidneys. Something else that we learned is that kidneys do not last forever and Charlie will have to have more then one transplant in his life. 

Monday a week into our hospital stay Charlie had a 1:00 appointment to get his dialysis catheters put in. We were told two different times on Sunday so we were not sure what time it would be but it turned out to be 1:00pm and not 8:00am. 

So Monday morning Charlie's Dr comes in and tells us she wants to put in a PD catheter (the one we chose to do) and they might put in the HD catheter (the one we didn't want) if the PD didn't seal up right away during surgery. We agreed that if the PD didn't seal up right away and couldn't be used right away that the HD could be put in. At this point we thought it would be up to the surgeon on the operating table. Well before the surgery the surgeon came in to talk to us about what he was going to do and he told us he was putting both catheters in. We said wait a second we were told it was only going to be both if the first one didn't seal on the operating table. It was poor communication on Charlie's Dr.s side as it turns out. So Charlie ended up getting both catheters put in. On the down side the one Jeff and I did not want put in was off a little bit and didn't work well, so the Dr.s decided they wanted to fix it because the PD wasn't working fast enough for there liking. We were not a fan of the idea that Charlie might go under again to fix something we didn't want in him in the first place. We ended up getting them to wait till today and they decided Charlie's labs were going in the right direction fast enough that they didn't need to put him under to fix the HD catheter. We were so excited.

Right now Charlie is on manual dialysis till they can get him up to a number that is ok to start using the dialysis machine. He is on 24 hour dialysis to start and they have to do it manually every 30 minutes, because of that we are still in the ICU and will be till charlie can go on the PD dialysis machine. Once he is on the machine we can move to a regular room for our training in PD dialysis. Once we are trained we will be able to go home and start doing dialysis at home. 

Once we are home Charlie will need to be hooked up for dialysis for 12 hours a night, my guess is 8pm-8am.

I hope this up date helps you to understand a little bit of what we are going through right now. I am also due to have a baby in February and Charlie's big sister Natalie is spending a lot of time with her grandparents. 

This is hard on all of us so please don't take it personal if we forget to respond to messages of any sort. 

Thank you everyone for all your prayers and support.

The Last Few Months!

In October 2013 our son Charlie started to get a little swollen so we decided to take him to the Dr. to see what was up.

The Dr. had some blood work done and decided that Charlie had hypothyroidism, so we started treating that with some medication. After a week or so on the medication and Charlie getting more and more swollen we took Charlie to the Emergency Department (ED) at The Children's Hospital in Minneapolis. After many different tests is was decided two things one that Charlie was going to be admitted and two that Charlie has some sort of Nephrotic Syndrome. 

Nephrotic Syndrome is a sickness of the kidneys. There are a few different kinds of Nephrotic Syndrome and because of Charlie's age they thought it was Minimal Change Disease. They treat that by giving steroids and it should clear up within 6 weeks. Three hospital stays later the 6 weeks was almost up and Charlie was not getting any better. December 11th it was time to do a kidney biopsy on Charlie. We got the results on the afternoon of December 12th...

December 12th

The Dr walked in to give us the results of Charlie's biopsy our good moods quickly turned to heart break and sadness.

We were told that Charlie has a very rare disease that he has had since birth.

The disease is called  Diffuse Mesaginal Sclerosis.

There is not much information on it as it is so rare. From my understanding it is a genetic disease that in our case is most likely a result of our combined DNA. The hospital we were at U of M Amplatz Children's Hospital which is world renowned for paediatric kidney diseases has only seen what Charlie has 6 times.

At the time of the biopsy Charlie had 30% scar tissue in his kidneys, that is irreversible. The Dr also said that Charlie will need a kidney transplant by the time he is 3 years old. When we got the news Charlie was 22 months old, so two months shy of two years old.

Since that time we have been getting blood work done on Charlie twice a week. Seeing his Dr. once a week and talking to Dr.'s or nurses almost daily. We have been trying to get the correct medication cocktail with it changing a little here or there every week. Charlie currently is on around 13 different medications daily and most are 2-3 times a day. Since his first hospital stay in November Charlie has regressed to the point where crawling was a struggle and walking was out of the picture. He is crawling more and more every day and holding our hands to help him walk. We are so proud of him! It is some times hard to think that a few months ago you couldn't slow Charlie down if you wanted to. He was running, walking, climbing and getting in to everything, then it changed so fast.

Every day we are grateful for our kids!

Natalie has been amazing through all the hospital stays and changes to our lives. It is hard for her that her little brother is sick and that he can't keep up with her any more, but she helps out so much every day. and tells people they have to be careful because her brother is sick. She doesn't understand what Charlie is sick with she just knows that we get to give Charlie lots of medications and that Charlie goes to the Dr.'s office a lot. 

Charlie is also on a strict renal (kidney) diet, he is only allowed 24 ounces of fluid a day, 2 grams of salt a day and he is restricted on potassium and phosphorus. This makes our day to to day meal planing challenging. 

Some of you may or may not know that I am pregnant with our third child.

He or She is Due at the end of February. Both kids are really excited for a new sibling. We are praying that this baby does not have what Charlie has because we were told that there is a one in four chance we will have another kid with the same disease.

If you have any Questions please ask in the comments. 

There is so much more info but this is all my brain can handle for tonight.