So Far So Good

We are on day five of the Prograf and so far, so good. We had one vomiting episode on Saturday (all over Nee and Pa's couch), but I am pretty sure it was due to the taper of the Prednisone again. He got very little Prednisone on that particular day, and ate a lot more than usual. Luke is not yet responding to the Prograf, but the Nephrologist says it could take up to two months.

The biggest obstacle for Luke is the bloating and swelling. When he reaches a certain weight he gets very uncomfortable and irritable. It is hard to watch him try to get on the couch or play with his trains on the floor, because he cannot bend and turn like he used to. This is when I give him the diuretic. The diuretic is very strong and makes Luke urinate out four pounds of fluid over night! When he takes it I need to change his diaper three or four times throughout the night to avoid leaking on the bed sheets. Then, when he gets down to a certain weight the diuretic needs to be stopped to avoid dehydration. If he looses all the weight over night, or too quickly, he could experience a type of shock due to the rapid loss of fluids. Once the weight comes off Luke feels like a million bucks and will run and play, get down on the floor with his trains, and take part in his new favorite thing to do....camping in his tent. The diuretic can not be used as often as he needs it, so unfortunately I need to let him reach that high number on the scale before I can administer relief.

I will continue to keep you all posted. As much as I love to write, it is a good thing when there is not much to say!

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

"May every sunrise hold more promise, every moonrise hold more peace."
-anonymous

A New Start

Luke is starting his new medication, Prograf, today. It took a few days to get it ordered through a specialty pharmacy that will compound medications. We are looking at this day as a new start for Luke. The Prednisone dosage is really low and getting lower every day. He will feel and look like himself again very soon, and has been in a great mood lately. We are praying that this medication works for him, and we can get on with our lives. I am at peace with the fact that he will have relapses, but as long as we have a medication that will put him back into remission I can deal.

I plan to keep you all posted on his progress with this medication, and any other progress or regressions that we may have. Since there is not much else to report on I will share with you a conversation that Luke and I had yesterday. Very funny....

I overheard Luke saying something to his sister over and over again. I asked him what he was saying and he said it louder so I could hear him. "Happy birthday Lex, Happy birthday!" As you all know Alexa was just born in November and has quite a wait until her birthday. "It's not Alexa's birthday," I told him. "Yes it is, where is the cake? Where are the presents mom, where are they?" Now he was starting to yell at me. "Its Lexi's birthday!" I told him he could pretend it was her birthday, but there were no presents or cake. He replied "Awww, poor Lex."

After talking in circles with him I came to realize that he had just watched an episode of "Mickey
Mouse Clubhouse" where there was a birthday party with cake and presents. I told him one more time that it was not Alexa's birthday, and then let it go. As I was walking back into the kitchen he said, "Mommy," "What?" I answered. "Happy Birthday! Mommy, its your birthday!"

This had me laughing all day. He also had me laughing earlier that morning. Those of you that know Luke very well know that he loves Thomas the Tank Engine, and all his friends. He has probably close to 50 of the trains, and they each come with a name and a story. He has all the names memorized and knows there are quite a few that we still do not have. I had been buying trains for him and keeping them hidden until he had a blood test, or something scary concerning the doctors. I found during one blood test that if I talked to him about the new train that was waiting at home for him he was so distracted that he calmed down long enough for them to stick him. So this particular morning I went into the bedroom with a syringe of medicine in hand. He held his lips together tight and would not take the medicine. I told him it was very important and if he did not take it they would have to do it at the hospital and give him another boo boo in his arm. (This is not a lie or an idol threat, it is actually what they would have to do if I could not get him to take the Prednisone orally.) He looked at me and said,"So, what's the new train's name?"

The photo was taken a couple of days ago. If there are sunglasses around Luke will put them on and then look at everyone in the room with a serious face. This kid knows funny.

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

"You can turn painful situations around through laughter. If you can find humor in anything, then you can survive it."
-Bill Cosby

Plan B

We got Luke's biopsy results today and they were what we were expecting and hoping for. Luke's kidneys showed Minimal Change. There is no scarring or damage to the kidneys. So on to plan B. Luke will now begin to take Prograf. Prograf, also known as Tacrolimus, is a transplant drug. It is given to patients who have received a kidney transplant to reduce rejection of the graft. In other words this drug attempts to stop the body from attacking the new organ. It works by suppressing one aspect of the immune system. Why this works for some Nephrotic Syndrome patients is not known for sure, but it does work for some, and puts them in remission.

Luke will need to get a blood test every two weeks. His levels have to be within a very narrow margin for the medication to be safe. In other words there is not much difference between the blood level at which it is safe and the level at which it is not. The side effects are not too scary if we are on top of things. If the medication is given every 12 hours and Luke gets his blood tests at the same time every two weeks, and his levels are safe, then the worst we need to watch for are swelling, vomiting, headaches, high blood pressure, and risk of infection, because of the weakened immune system. Sounds bad, but for Luke this is cake! Long term use of this medication gives higher risk of cancer later in life and could worsen kidney function.

Luke will begin this medication on Wednesday because we needed to go to a specialty pharmacy and they cannot produce until then. Luke will also be taking a baby aspirin every other day to reduce the chance of a blood clot. And of course that lovely Prednisone is still on a taper, and will be until the end of march when he will remain on a very low "maintenance" dose for some time.

Luke has been in great spirits as of late, and the Prednisone is at a low enough level that we are starting to see his little face showing through. He has not been too bloated and all in all feelin' fine.

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

I hate to repeat a quote, but this is a very fitting one, and Lexi is screaming, and Luke REALLY needs to go to bed!

"Life is all about how you handle plan B."
-Anonymous

We Thought It Was Allergies

Since the beginning of this blog people have been asking me how we knew Luke was sick, and how was he diagnosed. Since there is no news about his current condition until Monday, I figured this post could be about how we entered into the world of Nephrotic Syndrome. For Sean and me it is a world of on-line research, support groups, medication fact sheets, and constant discussion about where this came from and what path should be taken.

On October 20, 2008 Luke woke up with swollen eyes. We figured it was allergies because his eyes had swelled before after playing outside, and we had just turned on the heat for the first time in our new house. We figured the vents were blowing out dust and stuff that he could be sensitive to. His eyes looked better as the day went on, but were swollen again Tuesday morning and then again on Wednesday. On Wednesday I called the pediatrician and we got an appointment that afternoon. At the time I was in my 9th month of pregnancy and Sean was working nights, which was nice because he could help with Luke all day (I was so tired!!) and go to the doctor with us.

The pediatrician agreed that it was probably allergies, but wanted a urine sample just to be sure. He said that about a year ago they saw a kid come in with swollen eyes and it ended up being a very rare kidney disease. We thought nothing of it, the nurse put a little bag on Luke and we were to return the bag once he had urinated in it. The doctor also gave us a prescription for children's Zyrtec, to help with the allergies, we never filled that prescription. After the doctor we stopped at Target to pick up a few things. While we were in Target Luke and I made eye contact and all of a sudden I felt a pit form in my stomach. I knew, looking at those puffy eyes, that something was very wrong. Sean thinks mother's intuition is just a card I play when I want to win a debate over what to do with our children. I am here to tell you that it is very real.

Luke did not urinate in the bag until dinner time. This was strange to us because he guzzles juice and milk all day long. We did not know it at the time, but this is a symptom of Nephrotic Syndrome. In hindsight he had been urinating less and I took that as a sign that he was ready to potty train. We brought the sample back to the doctor and Sean ran it in while Luke and I waited in the car. He was gone a long time and then came out to tell me that the doctor wanted me and Luke to come into the office. We were ushered into an examination room and told that the doctor would be right with us. Sean and I were annoyed and just wanted to get home, what were we waiting for? The doctor came in after a few minutes and told us that Luke's urine was loaded with protein. Sean asked how much protein, and the doctor said it was as much as the test could test for. He told us his kidneys were not functioning correctly and that we needed to go straight to the hospital.While we both felt like we had been kicked in the stomach we still had no idea of the beating we were in for. This was only the first kick in the stomach.

The ride to the hospital was silent. I asked Sean what he thought, and he said we should pray that there is nothing seriously wrong with Luke. When we got to the hospital the pediatric nephrologist had gone for the day, but would talk to the staff in the emergency room over the phone, as they examined Luke. They took blood, and tried to get another urine sample, but Luke was not urinating, and Sean and I refused a catheter. We still were not grasping the seriousness of the problem. We were given a lot of information, which was not retained, at least not by me, and were sent home with a prescription for Prednisone. The doctor at the emergency room said that the Prednisone was a steroid and might make Luke hyper. HYPER? REALLY? HYPER? That's all he could tell us? The medication made Luke an absolute mess, he was anxious and shaking, he would wake up in the morning and lie in bed and cry. He would have screaming fits, throw his toys, and most of the day would not leave the recliner in our family room. He began to blow up and look completely different from his old self. That night I phoned my parents, who were in California for the birth of my nephew, to tell them what was happening. This was the first phone call of hundreds, I should have started the blog then, everyone wanted to help, but again we had no idea....

We finally got to meet with the specialist on Friday, October 24th, he diagnosed Luke with Minimal Change Nephrotic Syndrome. He told us that 90% of kids Luke's age respond to the Prednisone within 5-8 weeks. He said if your going to have a kidney problem, this was the one to have. We left feeling relieved and thinking that in 5-8 weeks he would be better. Three weeks later Alexa was born, Luke was still sick and still acting out from the Prednisone. My 3 night stay in the hospital was very hard for him, he had the normal 2 year old separation anxiety, but the medication magnified it. When I returned home he would not let me out of his sight and did not want me caring for the baby. Again, normal 2 year old reaction, but the screaming and crying were out of control. After being on the medication for about 6 weeks he must have adapted to it, because he began to behave like himself again. This is also about the time we began to realize that he might be in the 10% of kids who do not respond to Prednisone, he was diagnosed as steroid resistant, and again we were kicked in the stomach....a biopsy would be needed. Which brings us to today, we now know the seriousness of what we are dealing with, we have been kicked several times, but are still fighting.

The photo with the pumpkin is about 2 weeks before Luke was diagnosed. The other photo is from his second birthday, one month before being diagnosed.

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

"It is not the load that breaks you down - it is the way you carry it."
- Lou Holtz

Home Sweet Home

We are home, and very happy to be here. Luke's biopsy went smoothly, and for now all is well. The nurses and doctors at Children's Memorial were unbelievable, and for the most part, made
Luke feel very comfortable. My fears of him being wheeled away from us and traumatized for life went unrealized. It turns out that coming out of the surgery is way more dramatic than going in. When we finally got to see him he was confused, irritable, and frustrated. He didn't know where he was or even who we were. It was hard to see him like that, but he came out of it within about 10-20 minutes. We needed to stay over night, just because he is so young and the 12 hours of needed bed rest were easier to enforce at the hospital than at home.

The photo on the left is of Luke and his dad in the hospital's lobby before the procedure. He was so excited to walk in and see Melman (giraffe from "Madagascar.") chillin' in the lobby. We also found it very interesting that Melman is a Sox fan! The photo on the right shows Alexa hanging out with her cousin Nicholas. The two are only three weeks apart and got to spend two whole days together at Nee's house while we were at the hospital with Luke.

We will be seeing Luke's Nephrologist on Monday afternoon for the biopsy results and to discuss what should happen next. Until then we would like to forget about doctors and the fact that Luke is sick. There is no point in worrying for now, because there is nothing we can do until Monday.

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


""" " "" Worrying gives the illusion of control. You think that by imagining all the worst things that could happen, you can have solutions in place beforehand. But the reality is that since you’ll never think of all the possibilities, your worrying work is never done."
-Kathleen McGowa, author

Biopsy - Searching for Answers

Luke's biopsy is tomorrow, February 9th, 8:30am, at Children's Memorial Hospital in Chicago. To say we are scared is an understatement. From what I understand, they will not need to cut him open. They will first give him an ultrasound to locate exactly where the kidneys are. They will then use a special kind of needle, which is inserted into his back and into the kidneys. The needle then pulls out a piece of the kidneys that is about the size of the lead in a mechanical pencil. The procedure is to take between 15 and 30 minutes. This does not sound too bad, right? Right! I am not too worried about the procedure itself. A needle in the back is a small price to pay if it means getting him the proper diagnosis and the proper treatment. The scary part is the doctors taking him from us and bringing him to the procedure "suite" (yes, they call it a suite). He is not going to like that one bit, Luke is not big on leaving his mom, especially to go with a stranger. And by now he knows what a hospital is, and what goes on there, no matter how many cartoon characters are plastered on the walls! The nurse has assured me that he will immediately get sedated as soon as they are in the procedure suite.

The Nephrologist is fairly certain that the biopsy will show that Luke's Nephrotic Syndrome is Minimal Change. This is the type of NS that most boys between the age of one and two years usually get. It means there is little to no change in the kidneys. If the biopsy finds that it is not Minimal Change then it could be FSGS, which means there is scaring on the kidneys, and is a bit more serious. Of course the biopsy could also find something completely different. There are many forms of NS, depending on all sorts of factors, so who knows what we may find.

We are hoping to come home tomorrow night and not need to sleep over. The nurse said it depends on how he is doing. I will be armed with trains, books, stickers, crayons, games, and whatever else it takes to get him through yet another day in a hospital bed.

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

"Life is all about how well you handle plan B"

-Anonymous

This Too Shall Pass....

This is Luke's sister, Alexa. She must be mentioned in this blog because without her angelic cooperation we would be in big trouble. There have been many times when she is plopped into the crib or stuck in the swing while we deal with Luke. She stays with Nee (Grandma Denise) whenever Luke has a doctor appointment or needs to go to the hospital. While she may not always cooperate with Nee, she is a perfect angle when she is home, sleeping through the night and taking three hour naps every morning!! I am pretty sure she behaves so well because she loves Luke so much. Every time he looks at her she smiles and coos.

This past weekend we had Luke in the emergency room for a fever and some vomiting. We were told it was a virus that was going around and should be gone in 3-5 days. He was fine on Monday and Tuesday, but began to vomit again on Wednesday. I took him to the pediatrician on Thursday, and since he has no other symptoms besides the vomiting, we have come to learn that this is all a side effect of tapering off of the Prednisone (steroid that did not work for him). This morning he vomited again, and I called the Nephrologist because I was concerned that he was not getting any medication because he was vomiting every time anything went into his mouth. The Nephrologist said to give him a full dose of the Prednisone and wait to see if he keeps it down. If he does not keep it down he will need to go to CDH and take it through an iv.

Prednisone is a very strong drug and while he needs to come off of it slowly, he still needs it. He has had some withdrawl symptoms, such as insomnia, and anxiety. The Neph also decided that he needed a slower taper, something Sean has thought for about a week now, so now his dosage will change a little. We have learned from talking to other parents that the Moon Face starts to go down when you get to a certain level of tapering. We will be at that level next week and are very excited to see his little face again soon!

I am truly touched to see how many people are reading this blog. Sean and I do believe that the more people that pray for Luke, or even just think positive thoughts about him, the better off he will be. Luke is blessed to have so many people that love and care about him.

There will be another post detailing Luke's biopsy sometime this weekend. Not sure when I will get to it because this weekend Luke and Alexa get to meet their cousin Nicholas from California!

FYI - If anyone tried to click on the link for Nephcure, it may not have worked. I have fixed it and you can go back to the post, "Where We Stand," and click on it now.

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

"The only people who think children are carefree are the ones who've forgotten their own childhood."
-Orson Scott Card, author

Where We Stand

Luke has a rare kidney disease called Nephrotic Syndrome. It means that his kidneys are leaking protein into his urine, instead of putting it into his blood. He is extremely bloated and swollen. He has gained close to 20 pounds of fluid and carries most of it in his stomach. His medication has given him what doctors call a "moon face," or "facial mooning," which means his cheeks have swelled up and his face is very large and round. His eyes are swollen as well. There is no cure for the disease, and doctors do not know what causes it, it could have been triggered by a bad virus he had in September, but it could also be genetic. Like cancer, you can fight the disease with very strong drugs and hopefully go into remission. Relapses are close to inevitable because they are caused by viruses, colds, and infections. The only ray of hope is that he will out grow the disease before his teen years. Luke has completed his first round of drugs, a steroid called Prednisone, and did not respond, so now he will get a biopsy next week, and then try a new drug. All the drugs are very scary and have horrible side effects.

Despite all of this, Luke is the happiest guy I know. Besides some mood swings here and there (a side effect of the steroids) he laughs, plays, kisses his baby sister, repeats everything we say, dances, sings, and most important of all keeps us laughing all day long. His favorite movie is "Madagascar," and the other day he told me his breakfast was "crackalackin!" He has even started taking his medications directly, instead of hiding it in his milk. He has endured blood test after blood test, people poking and prodding him, and has proved to be a strong little 2 year old.

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

To learn more about Luke's disease go to http://www.nephcure.org/ or google "nephrotic syndrome"


"The pressures of being a parent are equal to any pressure on earth. To be a conscious parent, and really look to that little being's mental and physical health, is a responsibility which most of us, including me, avoid most of the time because it's too hard."

John Lennon