BIG news...New Hope

                                               








 On Monday Luke went in for what I promised him would be the last blood draw for at least two months.  It is always a struggle when he needs to give blood, and I always feel like the bad guy holding him down and making him do it.  Well, I feel guilty no more... The blood draw from Monday showed that Luke got "almost all of his function back," and "his function is at almost 100%."  We are looking at this as just short of a miracle.  We knew it was possible after we wiped out the C.diff for his function to come back, but given his history we did not have much hope.  Luke was definitely knocked down, but as his Aunt Katie said, "Wow, he is such a little fighter!"

Luke is still spilling protein, and we are still using diuretics to manage his swelling, but he is pretty much drug free.  Luke feels great.  Luke looks great.  We will still test his function every couple

months, and his Nephrologist is still meeting with other specialists to figure out if we are doing everything we can.  I will post again if the plan changes and I am hoping that post comes well after the holidays.

     

The pictures are what happens when your very smart six-year-old figures out how to use Photo Booth and has a sister that loves the spotlight...

                                                                                                                                                                                           

 Please continue to pray that Luke is strong and breezes through whatever may come next.

Halloween in the Hospital

My guy Luke has had a rough couple weeks. He has been having fever and vomiting off and on for a week, and diarhea for much longer. A stomach bug definitely made its way through our house last week. Alexa, Sean, and myself were all affected at varying degrees.  Luke got hit hard. I took him to the ER Monday morning for dehydration and dangerously low albumin (protein) levels. 4 days later and we are still here. Last night we found out that Luke has C.diff. This is a germ, or bacteria that lives in the colon that is known as flora, or gut flora. It turns to C.diff and becomes toxic when the flora becomes over grown from antibiotics. This made sense to us because about a month ago Luke was on antibiotics for about 10 days and that is when his bathroom issues began. We stopped other medications hoping they were causing the problem, but they weren't and his problems continued. So, we are still admitted and he is now being treated for C.diff (Clostridium Difficile Toxin).

We've learned more about Luke's kidney disease since we have been here. We found out on Monday that Luke no longer has 100% function in his kidneys. Through this whole journey since age 2 Luke has always had 100% function. Even after finding out that 50%of his kidneys were scarred, we still had that silver lining.  He was still functioning at 100%. On Monday he was only at 50%. After treating his dehydration his function went up a bit, but we are not expecting it to return to 100%.  And taking into account all the other factors, such as the scarring, and the fact that he has been relapsing for months, and that he is steroid resistant, the nephrologist is assuming that it will decline further. Luke's kidney function could stay where it is for some time, or it could go down slowly, or it could decline quickly, we have no way of knowing. What we do know is that once the function gets to 30% we will start the process of finding a donor and getting him a transplant.  Like I said, it could be months, it could be years. If we start the process at 30% then we can do an elective transplant with a living donor. He won't have to wait on a list or wait till his function is so low that other organs begin to fail. We will avoid dialysis, and get him a new kidney.

So, here's the plan. Get rid of the C.diff. No more cell cept. It hasn't worked yet and it has had him so weak that he is constantly sick. Luke will only take his Ace Inhibitor, which is believed to stop the progression, and diuretics to manage the swelling. We will test his function every couple months and move forward as the tests suggest.

For now we just want to go home. Luke misses school and his sister.  Alexa has pretty much moved in with Nee and Pa. They watch Dancing With the Stars together and Alexa is running her own hospital for stuffed animals out of their family room. We are so thankful for the flexibility at Nee's job. A big thank you to her bosses for letting her do so much from home! And Thank you to Nee Jojo for hanging with Luke so I could take Alexa Trick or Treating.  Thank you to everyone who has reached out. Our family, friends, and the families at St. Irene. It really means a lot to us to know that we have so much back up and support.

Please pray that Luke will continue to be strong and breeze through whatever may come next.

We're Home!

Luke was released from the hospital yesterday.  He was thrilled to go straight to Nee's (Grandma's) House  to see his cousin, Nicholas, who is in town from California.  What a great way to forget about the hospital and just play and have fun!

His belly went way down an his knees and ankles are completely free of any swelling or bloating.  His eyes were still pretty swollen when we left the hospital, but we are continuing the diuretic treatments at home, and have already seen some improvement in his eyes this morning.

Luke is still relapsing, but we are giving the Cell Cept a good 6 month trial, so we may be dealing with swelling and bloating for a while, as we pray that the Cell Cept will put Luke into remission.  We will be following up with the Nephrologist on Wednesday, and I will post again at that time.

Luke is so happy to be home, and we are hoping that any future swelling will not land us back in the hospital and can be dealt with at home.

Please pray that Luke will continue to be strong and breeze through whatever may come next.

Diuretics at the Hospital

After a couple weeks of carrying around extra fluid the oral diuretics that we started using on Friday did not work.  Luke woke up this morning with eyes swollen shut.  I called the Nephrologist and told him Luke needed something else, he is on day 6 of taking 3 different diuretics and he is still waking up like this.  The Neph explained that when you are as bloated as Luke, sometimes it is hard for the intestine walls to absorb an oral medication.  Luke was admitted into Central DuPage Hospital early this morning and is getting diuretics through an IV.  We will definitely be here over night.  The Neph is planning 2 doses and they need to be 6 hours apart.  Hopefully the 2 doses are enough, but we will see after the second one if the weight has come off.  Let's all pray that 2 is enough because Luke is very upset to be sleeping here tonight, and has asked repeatedly to go home.  He took a nap from about 12 to 2 and when he woke up he asked if he had slept for a night yet, because then he could go home.

Please pray that Luke continues to be strong and breeze through whatever may come next.

The Latest on Luke

Good News: Luke has started Kindergarten and loves it!! He has his moments when he misses mommy (and mommy misses him too!) but for the most part he loves it and looks forward to going each day.

Bad News: Luke already missed a day. Friday morning he woke up and vomited. His eyes were nearly swollen shut, and his belly was so swollen that no pants or shorts were comfortable on him.  He has gradually swelled up from 60 pounds to 75 pounds in the past week.  He is relapsing pretty bad and spilling as much protein as our test strips will test for.  After getting in touch with the Nephrologist it was decided that Luke would take diuretics to release the extra fluid he is retaining. We started the diuretics last night and we are hoping for some signs of relief for him soon.  If the diuretics are not working by late afternoon we will need to admit him to the hospital to get the diuretics in an IV. So, we are praying that the oral medication works and Luke is feeling better soon.  If you refer back to my posts "The Difference a Diuretic Makes" and "My Eyes Aren't Workin" you will see that we have not used diuretics since Luke was 2.  They worked well then and we are hoping they work well now.

Luke is still on antibiotics, and we restarted the Cell Cept a couple of days ago. I am realizing now that the antibiotics are not the magic cure I thought they would be.  It seems to me that it is actually the onset of antibiotics that usually puts Luke into remission.  The antibiotics are done in a couple of days and my prediction is that the next time Luke needs them, whether it be an ear infection, or pink eye, or whatever normal childhood illness he picks up, they will give him an antibiotic. The onset of it will put him into remission and then the remission will fade into a relapse, unless the Cell Cept starts to work.  We shall see.

Please pray that Luke continues to be strong and breezes through whatever may come next.

Pneumonia, Antibiotics, and Kindergarten!

Luke has had a rough couple of weeks.  First he got Hand, Foot, and Mouth Disease. It is a nasty virus that comes with fever, vomiting, and sores that look like Chicken Pox.  He got it bad, and there are still marks on him from the sores.  Alexa got this virus too, but she had about three sores and only ran a fever for about a day.  About 5 days after we were sure he was better he started running a fever and vomiting again.  This time the Pediatrician said it was a virus and needed to run its course. Alexa got a touch of the same virus.  She ran a 99.9 for about 8 hours and was back to playing ponies the next day.  Four days later Luke was still running a fever, that Tylenol would not break, still vomiting, and now coughing a horrible cough that scared me enough to bring him to the ER.  In the ER they found he had pneumonia and his albumin (protein) levels were very low.  He has been relapsing and spilling protein since his biopsy in May.  The Cell Cept (medication started in May after his biopsy) has not yet worked, and was hurting his chances of fighting off disease.

The ER doctor along with Luke's Nephrologist decided to take him off the Cell Cept to give his body a chance to fight the pneumonia. He was given antibiotics intravenously, and breathing treatments to help him breathe and clear up his lungs.  It was also decided that he would need intravenous antibiotics for the next couple days as well. Regular oral antibiotics were not strong enough for him at this point.

So we were released form the ER with an inhaler and appointments to see the pediatrician for shots of antibiotic. I was scared and worried about my son, but at the same time...I was thrilled!  Why? Because luke was off kidney medication and on some super strong antibiotics.  If you've read my past posts you will remember that antibiotics always put Luke into remission.  The day after the ER Luke was swollen in his eyes and belly, and his protein was sky high. He received one more shot of antibiotics and did not need the second shot, they determined he was strong enough to take regular oral antibiotics to get rid of the rest of the pneumonia.  Two days after the ER Luke was not swollen at all and his protein had come way down. ANTIBIOTICS!!!! all he is on is antibiotics!!!!  And he looks and feels the best he has since May.

So what's next?? We met with the pediatric nephrologist yesterday.  I had a lot of questions and unfortunately so does the nephrologist. Luke is still a rare case and they have never seen this type of reaction with antibiotics.   The Neph is on board with giving more antibiotics a shot, but as we all know that cannot go on forever.  We decided to give Cell Cept one more chance along with the antibiotics.  This particular drug usually takes about 6 months to work and we need to give it a real chance.  Cell Cept is a last hope.  The next drugs to try after Cell Cept are "drastic," and "come with serious side effects." According to the Neph, "There are no good answers." Luke is still running on 100% function, but the 100% is coming from 50% of his kidneys.  The working halves of each kidney are working over time to compensate for the 50% that are not working.  We are hoping that the scaring has halted (which is why he takes an ace inhibitor), but can't know for sure, and the Neph has warned us that he could some day be dealing with chronic kidney failure.

So for now Luke feels great (antibiotics), and is starting Kindergarten in 11 days. I know the minutes and seconds too, but won't bore you with the details.  Alexa and I will be lost without him.  We cannot believe he will be gone all day, everyday. He is ready though, even told me he can't wait to ride the bus....bus?  We'll see about that!

Please continue to pray that Luke is strong and breezes through whatever may come next.

Biopsy Results

Luke's Biopsy went smoothly and we got to go home the same night as the procedure.  It's good to be home, but hard to keep a 5 year old boy on the couch.  Luke cannot bump his back for 10 days.  So, this means no climbing, running, jumping, or horseplay, this should be interesting!

On Thursday we received the results of the biopsy.  As I posted previously the reasons for the biopsy were to see if Prograff (the medication Luke has been taking for 3 years) is hurting him, and to see if we are still dealing with same diagnosis. The surgeon called me on Thursday and said that half of the Glomerular filtering units (small holes) on Luke's kidneys are terribly scarred.  The doctors are assuming that the Prograf did this to him because 3 years ago when he had a biopsy there was no scarring at all. Of course, they cannot be 100% sure if the Prograf is to blame, but we are taking him off Prograf anyway.  The only other explanation is that the disease was progressing these last three years and caused the scarring.  I find it hard to believe that it was just the disease progressing because Luke has been so healthy and happy.  No swelling, and up till 6 months ago he was, for the most part, in remission for 2 1/2 years.

His diagnosis is still Nephrotic Syndrome, and they are still calling it Minimal Change (no change to the kidney) despite the scarring.  When I asked how we could still call it Minimal Change the doctor said because half the kidneys are still untouched and he has so few symptoms. In other words they don't know what to call it.  When inquiring further about the scarring the doctor told me that it is permanent damage and we must stop the scarring because now he's only working with 50%.

Once again Luke is an enigma.  The doctors are baffled to say the least and it is extremely frustrating to have most of my questions answered with, "I don't know, we have never seen this before," "we aren't sure, Luke is a rare case," and my personal favorite, " well... that's just not typical..."

The doctors are amazed at how healthy Luke seems to be.  He is never tired, eats like a horse, is active, and has grown 3 inches in the last month and a half!!!  And here's the real kicker... He has 100% kidney function!!!  The kidneys are filtering incorrectly and are "scarred beyond belief" but are functioning 100%! Thank God! The doctors also have never seen Prograf do this to someone's kidneys, especially when his levels were never toxic.  He was given regular blood tests to test the levels in his blood and it was never toxic. And then there is the antibiotics.  Every time Luke is given an antibiotic, whether it be for an ear infection, or most recently, strep throat, he goes into remission. When I point this out to the doctors, which I have done on several occasions, they say they have never seen it before, and that they don't give antibiotics to induce remission.  So, I understand that this is not "typical" but it is a fact! It happens!!  I'm not trying to get the doctors to write Luke a lifetime supply of Amoxicillin, I just want them to think about it and consider what this might mean.  Is there an infection somewhere that he is constantly fighting??  It must mean something!

On Friday, May 18th there is going to be a conference to discuss Luke's biopsy.  Until then he is starting a new medication called Mycophenolate, or Cell Cept.  This is another transplant drug and is also used for Crohne's Disease.  Just like Prograf it will turn off the aspect of Luke's immune system that is attacking his kidneys.  Transplant patients use this drug to stop the immune system from attacking the new organ. The problem here is really with Luke's immune system.  The immune system is working too hard and needs to back off of the kidneys. Our pediatric nephrologist also wants to put Luke on an ace inhibitor.  While this is usually used for high blood pressure, it is also used in kidney disease patients to stop the progression of the disease.  Like I said before, the scarring cannot continue, and while we are hoping that stopping the prograf will halt the scarring, we cannot be sure.  We are starting the Mycophenolate today, and the ace inhibitor will be discussed at the conference on Friday and if there is a consensus among the team of specialists then we will start an ace inhibitor at that time.

LUKE IS HAPPY AND FEELS GOOD!!! I will post again after Friday's conference and give an update on Luke's remission, or lack there of.

Please continue to pray for Luke to stay strong and continue to breeze through whatever may come next.

New Biopsy Date

Luke's biopsy has been rescheduled for Tuesday, May 8.  While we are excited to get this done and over with,  it was nice to have another two weeks to prepare ourselves, and Luke is now able to participate in his school's field day this Saturday!  Luke had strep throat last week, which is why we had to reschedule. He is now on antibiotics, which end right before the procedure date, so the odds of him being sick again are very low.  He is scheduled for 9 am.  The procedure requires 12 hours of observation, so we should be able to go home by 9 or 10 in the evening.  It's late, but way better than sleeping in the hospital.  We will be in the city by 7:30 am for pre-op blood work (oh boy, Luke's favorite!) and then to the biopsy at 9:oo.  I will do my best to keep everyone informed.  If you would like more info on what goes on during the biopsy you can read my posts from the biopsy he had about 3 years ago.  Here and here I explain in detail how the biopsy will happen.  Thank you all for the kind words and prayers. Luke amazes us everyday with his ability to endure and adapt.

Please continue to pray for Luke to continue to be strong and breeze through whatever may come next.

Biopsy Postponed

Luke was scheduled for a kidney biopsy early tomorrow morning. The biopsy is being postponed due to Luke having a fever today. We should find out in the next couple days when it will be. I will post at that time.

Nothing is Typical

In my last post I announced Luke's most recent relapse. We were hoping for a quick remission and then a taper from his medication. Well, he has yet to go into remission, and the doctors think we need to take action. Yesterday, we met with a second nephrologist at Children's Memorial. After talking to our regular neph, the two decided that Luke needs a second biopsy on his kidneys. He got his first biopsy about 3 years ago when he was 2. You can read about it in my 2 posts "Biopsy: Searching for Answers," and "Home Sweet Home." There are two reasons for the biopsy. The first reason is to see if we are still dealing with Minimal Change Nephrotic Syndrome. Has the disease changed or progressed? The second reason is to see if the Prograf has hurt the kidneys at all. We knew going into it that Prograf not only helps but could be toxic to the kidneys. We had no other choice, and for two and a half years the Prograf was the answer to our prayers, keeping Luke in remission, and for the most part, feeling good.

We asked a lot of questions yesterday, and did not get a lot of answers. This disease is rare, every patient reacts differently, and with Luke nothing is typical. I pointed things out to the neph about how Luke's body responds to different things (such as antibiotics, which always reduces the amount of protein in his urine, and have even put him into remission before), and he shook his head, and said "No, thats not typical." Some non-typical things are actually good things. Luke seems to be doing fine. He is happy, energetic, growing like CRAZY, eating like a horse, and only got sick twice this cold and flu season (thanks to the Chicago winter that never was!) His kidney function is 100%! If it wasn't for his blood and urine tests you would never know he was relapsing, and that his kidneys were filtering incorrectly. But it is a serious thing that needs to get fixed to insure healthy kidney function into his future.

The biopsy will be April 24th. It is going to be hard. He is much older and smarter, and he feels good. He surely will be wondering why he is in a hospital bed, and will most likely ask, "Is this about my kidneys?" He knows something is up with the kidneys and often wants to know, "how's my kidneys?" I once heard him telling his sister, "Well, maybe its your kidneys, we all have them, you know!" To which she replied, "yeah, maybe..." and then rubbed her head.

I will post on or after the biopsy day, and hope to have good news to share with all of you.

Please continue to pray that Luke stays strong and continues to breeze through whatever may come next.