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.

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