Tuesday, April 28, 2009

An Enthralling and Informative Visit To Milwaukee

Okay, that is an outright untruth. The latest in a frustrating run of non answers.

The CAIR team in Boston was not willing to change Sam's reflux medication as they wanted him to be seen by someone to ensure there is not more going on than just reflux. Not sure why this came up now since we have been dealing with the vomitting issues since December.

We finally got an appointment with our "local" GI today. After arranging to drop Ryan off at Grandma's and rearranging work schedules for the week we make the two hour drive to Milwaukee, which I just did on Friday.

We left there today more frustrated then when we arrived, no ideas, no answers, nothing. We will be making two more trips to Milwaukee before I leave for Boston on May 13th. They will be performing a Barium Upper GI study one day and then a few days later he will be having a Endoscopy. THese two tests will allow them to take a better look at what is going on in his digestive system. We also need to stop giving him his current reflux medicine so that these tests can be done without any chemical interference. We are in for a long few weeks. If the test show anything is not working correctly it could mean surgery. If the tests show nothing abnormal then we are back to square one, but then they will at least let us try the combination of meds we want.

Through all of this there really has been no change in Sam so we are not overly concerned. He is still gaining weight and growing, getting closer to walking on his own everyday, and generally happy and content most of the time.

Sam's weight has gone up to about 18.5lbs and we have been able to successfully move his "unhooked" time (off of all pumps) to 8 hours a day.

Besides the medical stuff we have been busy, busy, busy. Sam has a new cousin due to arrive any day and his uncle Joe got enganged this past week.

Amanda - there is still time to change your mind, although Ryan is very excited to have you as an aunt.

Tanya - Now that the baby is almost here your stuck with us one way or another for the rest of your life.

I will try to get some new phots and videos uploaded this week and we will update as soon as we get results back from our upcoming tests.

Friday, April 24, 2009

To the ER

I just wanted to let everyone know that we are headed to the ER in Milwaukee. The lumen (end) of Sam's Broviac (Central line that supplies all of his hydration and nutrition) broke off tonight. I will update agtain once we are settled in.

***3:52 A.M. Update***

We are back home! A little cut here, a little splice there, some glue, and a very grumpy Sam made for an interesting evening. The line is repaired and Sam is finally sleeping. Time for Dad to do the same.

Tuesday, April 21, 2009

Grab A Comfortable Chair

This is bound to be a long post. It has actually been coming for awhile, but I needed time to get my thoughts together and have time to sit down and type this all out.

I will start first with an update on Sam. Not much has changed, no decision on what to do about the elevated liver enzymes. We will be finding out if the Protonix was the cause because it has stopped helping Sam's reflux. Over the weekend things progressed quickly from his occasional spit ups to all out, obviously painful, vomiting fits. It is so horrible to watch him go through these that it almost being tears to my eyes each time. By the end of the week we will be switching reflux medications, again, and are actually hoping to use a combination of IV Zantac and Oral Prevacid. We still have to get a doctor to agree to this, but one of them will eventually - we are persistent. This combo is apparently not a standard practice and I could hear the eyebrows raising in Boston today (You know how those crazy parents who think they know everything are). Then we played our Bo card and they seemed to warm to the idea a little. (This is the combination of meds that Bo is currently receiving for his reflux and helped to return some sanity to his parents). We'll see what happens once they get a chance to discuss it tomorrow.

Onto the long part. My rambling thought that have been brewing for awhile. About a year ago we posted the entry below:

"I knew we were headed for bad news when I saw him this morning. His color was terrible, he was very pale, and I could just see that he was not feeling well. His blood gases this morning came back good though and we were hoping that his color just had something to do with the fever he ran yesterday. They also began to have problems maintaining his body temperature today and his temperature kept dropping below normal. Then they did his afternoon blood work.

It turns out that Sam is once again very ill. There was only 12 hours between these two sets of blood work and his blood PH had dropped to 7.20. To give an idea of how drastic this is, a normal blood PH would be 7.41. The last time that they made Sam sick for testing we waited days for his blood PH to drop to 7.28 so they could draw blood while he was in this acidotic state. For him to drop this quickly was devastating for us. To top all of this off his urine output has dropped to almost nothing.

This evening Sam looked possibly the worst we have seen him since the first day that he was admitted to the hospital 40 days ago. Because of the low urine output, they have had to stop administering the antibiotic he was on. Due to his blood PH they have stopped providing the intravenous lipid he was getting as well as removing the simple fat that he was receiving in his diet. Hopefully with these steps Sam will begin to return to normal again on his own. If he doesn’t they will help him along so that he can be “healthy” again and they can determine what our next steps will be."

This was just before we made the decision to move Sam to Milwaukee and a few weeks before his diagnoses. This was one of the episodes that made us think that Sam was never going to make it. If you look back at pictures from that time there is no way you would think that Sam would be the apparently healthy little boy that has been keeping us so busy these days. What a difference a year has made.

In the past year we have been exposed to a completely different world that has always been out there, but we were never a part of it. It didn't exist to us. Just like it truly doesn't exist for a good majority of the people reading this, and I hope it never does. This year of living with a medically fragile child has exposed us to some callous and insensitive people, and it has also exposed us to many more selfless, caring, generous people.

How many times, before Sam, did I ever follow a sick child’s journey? Never. Sam has brought a special connection to the other families out there that are in similar situations that I could not imagine living without. We can vent with each other and compare notes on subjects that most other people just can't understand. We owe a lot to the technology that has allowed us to meet each other over great distances to help each other out. These people can truly understand the complexity of Sam's disease and care like nobody else can.

People who have never been through a situation like this have difficulty understanding Sam's disease, what it takes to care for him, what it is really like. Everyone can imagine what it would be like, but I can guarantee that your imagination is only about 25% correct. I find myself getting angry when people ask me questions that I think are stupid, but they are not stupid questions, they are just trying to understand. I just have to remember to put myself in their shoes and pretend I haven't lived the last year with Sam. Here are a few frequently asked questions:

"Is Sam's disease cured now that you went to Boston?"

No. Microvillus Inclusion Disease is currently not curable. Sam's disease prevents his intestines from absorbing nutrients, so he is dependent on IV nutrition (called TPN) to survive. He is attached to IV pumps for 17 hours a day that provide all of his nutrients and fluid requirements.

"Then what did you go to Boston for?"

We go to Boston to receive Omegaven. The fat content in standard TPN in the US causes liver damage in over 80% of infants that are TPN dependant long term. Sam started showing signs of liver damage very early on. Going to Boston is saving Sam's liver, it is not curing his disease.

"Will Sam disease go away as he grows older?"

After our experiences this year I will never again say that anything is impossible. However, no, Sam will most likely be living with this disease his entire life.

"Why can't Sam just get a transplant?"

Well, he could. We have made the decision to avoid a transplant unless it becomes necessary to save Sam's life. Transplant technology has come a long way, and it continues to improve every year. Currently Sam's prognosis without a transplant is better than if he were to receive a transplant. This would not be the case without Omegaven. Our hope is that transplant technology will continue to improve and Sam will be able to make a decision regarding living life with the backpack or trying for a life without it. This is a personal choice that we have made for Sam. Other families have made the choice to transplant and we respect their decision and understand the difficult position that they are in. Transplants can be lifesavers, but they are also life changing. In our opinion Sam's chances to live a life as close to normal were better with TPN and the risks and limitations that come with it.


For those of you who didn't know the answers to those questions, I hope that they help your understanding of some of the choices we've made this year. Next week I am planning "A Week In The Life Of Sam" post to try to give everyone a better idea of what things are like (The good and the bad) managing Sam‘s care.

We also wanted to give a special thank you to Jose and Kinn (Bo too), for helping save our sanity by going through most things about 9 months before we do so they can work out all of the kinks for us. They have become an invaluable part of our life and we can't wait to see them again in May.

Friday, April 17, 2009

Too Many Cooks In The Kitchen...

If you read our post yesterday, you might as well forget that you read it. We are having an experience that we are actually surprised has not happened before . Our rather large team of doctors in thre cities and two states do not all agree.

We have 1 vote for the Iron beign the reason for elevated liver enzymes. We have 2 votes for the Protonix being the cause. We have 1 vote for neither being the cause. Everyone else is scratching their heads.

The team in Boston will revisit the issue next week during their rounds and we hope to have a plan of action then. For now we just sit back and wait. This is an experience that we are used to, wait and wait and wait some more.

Thursday, April 16, 2009

Theories vs. Answers

We really don't have any answers yet, and did not expect too this soon, but we have a theory or two.

In February Sam started receiving Iron Dextran Infusions once a week to help with his severe Anemia. In March Sam started a different medication for his reflux.

Since that time his Anemia has improved as has his reflux. Unfortunately we can not rule out either one of these as the cause for the huge spike in his liver enzyme levels.

The plan is to stop the Iron Dextran infusions for the next month. If he shows improvement in his next round of labs then we will know the culprit was the Iron. If he shows no improvement or is worse then we will stop the Protonix and wait another month. So in a nutshell we could be waiting at least a month or two before we have an answer. If there is no improvement in two months then we are back to square one and will start with other theories which we discuss in more detail with the team in Boston during our visit on May 14th.

Wednesday, April 15, 2009

Crazy Labs, Second Transplants, A New MID Baby

Things have been rather quiet since our central line incident. Wouldn't you know that we were headed for another round of concern.

Sam's monthly labs showed some unexpected results today. In order to explain I will have to get a little technical, so I hope I do an alright job of explaining. Each month Sam has many lab tests run that measure many things in his little body. All of his labs have been looking better each month. One thing that we watch closely is his hepatic (liver) panel. As you will remember Sam's liver was showing signs of damage that was being caused by the intralipid (Omega-6 fats) that he received as part of his nutrition. This was why we made the decision to go to Boston. We replaced the Intralipis with Omegaven (Omega-3 fats) and is what has enabled us to avoid the need for a liver transplant. Two things that we watch are:

Aspartate Aminotransferase (AST): AST is normally found in red blood cells, liver, heart, muscle tissue, pancreas, and kidneys. Low levels of AST are normally found in the blood. When body tissue or an organ such as the heart or liver is diseased or damaged, additional AST is released into the bloodstream. The amount of AST in the blood is directly related to the extent of the tissue damage.

Alanine Aminotransferase (ALT): ALT is found mainly in the liver, but also in smaller amounts in the kidneys, heart, muscles, and pancreas. ALT is measured to see if the liver is damaged or diseased. Low levels of ALT are normally found in the blood. But when the liver is damaged or diseased, it releases ALT into the bloodstream, which makes ALT levels go up. Most increases in ALT levels are caused by liver damage.

When we arrived in Boston:
Sam's AST was at 98 (Normal is 10-65).
Sam's ALT was at 132 (Normal is 3-54).

When we left Boston in September:
Sam's AST was at 136.
Sam's ALT was at 132.

These numbers have slowly shifted up and down over the last few months. This isn't that abnormal, and it can take a while for these numbers to return to almost normal. For Sam the high side of normal would be perfect.

Until recently Sam's AST was averaging around 70-80. His ALT was usually right around 150. Although still elevated these were "comfortable" levels.

Today Sam's AST was at 117 and his ALT was at 370. Comparing to last months results (which they were hoping were a fluke because the number were increasing) these numbers have been steadily, and dramatically, increasing since January.

So what does this all mean? We don't know yet. Boston is looking into whether other changes in his medication and nutrition could be causing this. This would be an easy fix. Our other options, which we will not go into yet, are not such an easy fix. Sam is really teaching us to be prepared for anything I guess.

Other than that, Sam continues to look and act fine. We does not like to be confined and easily walks around the living room, or any other room for that matter, using walls, furniture, or people for support. I do not see full out walking being very far into our future. That sounds exciting, but there is that matter of his IV that is running. He is down to being connected 17 hours a day, from 8 PM to 1 PM, and we received the OK from Boston to go down to 16 hours a day - but then we got his lab results. I think we will hold off on making any more changes.


Awhile ago we posted about Emerson White. She was the little girl that had received a transplant and rejected, then Colorado tried to cut her Medicaid. As I write this tonight she will be on her way into the operating room to receive her second transplant. Her blog entry form earlier today is below:

Days on transplant waiting list: 14


The surgeon accepted an organ offer for Emerson this morning!! She’s admitted & going through the pre-op process. The team is out procuring the organs as I write - liver, bowel, pancreas, and kidneys! Surgery is estimated to begin between 10 & 12 tonight. All expect her to come out sometime tomorrow late morning.

I have a good feeling about this! It’s Daddy’s birthday today – a great day for a transplant!!!

Check back often for updates through the night. (I won’t send e-mail notification with each one.)

Many prayers & heartfelt appreciation for the donor family on this very difficult day. Thank you for giving the gift of life. Thank you for giving my baby a chance…


In other news we were contacted this week by a family in Canada who's 4 week old son, Hunter, was just diagnosed with Microvillus Inclusion Disease. Hopefully we can share part of what we have been through the last year with them and our experiences can give them hope for their son. Since Hunter is in Canada they were able to start Omegaven right away which will hopefully help his liver stay healthy. I can barely remember the range of emotions we went through a year ago and relived them a little when we read their story.


That's it for tonight, we will post again as we come up with some answers to our latest adventure.

Wednesday, April 1, 2009

What is better than two trips to the hospital in one day?

Leaving without being admitted.

We lucked out and the line placement is still good. The cuff that holds his line in is displaced and he will probably be a little sore for a few days. His site was always in such perfect shape and now he has a bulge there, but that is better than a line replacement. We will have to keep an eye out for any changes over the next few days, but it looks like we lucked out and are going to have to miss being admitted to Milwaukee.

Would it be considered abuse to force your child to sleep in a straight jacket?

What is better than a trip to the hospital?

How about two trips to the hospital.

Sam decided he didn't like his central line today and tried to remove it. Sounds worse than it is. Looks like the line is fine. Mom was pretty panicky as she thought the cuff (that hold his line in) was above the skin. Looks like it is just a sleeve that is above the cuff that is exposed. First chest xray looked OK, but Milwaukee (and Mom and Dad) aren't 100% convinced so we are headed back to the hospital for a second check with a more detailed picture.

We will post more details as soon as we can.