Monday, June 30, 2008

One month down...

We have survived a month at home. Some of the days in that month have been very good days. Some of the days in that month have been not quite as good. The past week has been filled with quite a few of the not so good variety, but we will vent about those shortly.

It is hard to imagine what our lives were like 4 months ago. March 1st really wasn't that long ago, was it? We spent the day with friends and all of the kids at Wild Air and we played almost as much as the kids. Even Deb, at the end of her 8th month of pregnancy, got into the action. Everything was so care free, at least that was what we remember. We were about to close on our house and were busy picking out new carpet, paint colors, new appliances. These seemed like such tremendous, important decisions at the time. We spent time everyday talking to Ryan about being a big brother. These were exciting days, full of dreams and plans for the not so distant future.

When we skip ahead a month and remember what our lives were like 3 months ago, you have to do a double take because you can't believe that this is the same family from just four short weeks ago. It feels like out family is falling apart. Ryan is practically living at his grandparent’s house. We can't sleep, eat, or work. In fact we can barely function. Sam has been in the NICU for three weeks. We are living in a half finished house because nothing in our lives went according to plan in the last month. Our days are spent telling each other that everything is going to be ok. Pretty soon they will figure out what is wrong with Sam and no matter what it is we will deal with it and move on with our lives. Just one more test. They should have an answer in just a few more days.

A few more days turn into weeks and we find ourselves now looking back at what our life was like two months ago. Things should have been better by now, but instead they are worse. No matter how hard they try, they have not been able to get to the bottom of what is wrong with Sam. Sam has moved to a bigger hospital, further away from home. Now our family seems even more broken, Ryan lives at the Ronald McDonald house a few days a week, spends a few days with Grandma and Gramps, and a few days a week at home with Dad. We are no close to an answer about Sam, we watch him with sadness every day. Although we don't vocalize it, we have given up hope and are certain that he is not going to survive long enough for them to ever figure out what is wrong with him. We cannot bring ourselves to unpack anything for his room at home. We both begin to worry about the other mental health. The doctors tell us that they should have a better idea of what is going on and may be close to a diagnosis in a few days. I think that we have heard this one somewhere before.

One month ago we found ourselves in a very scary place. Sam had been diagnosed almost two weeks prior and we were going home. After 3 1/2 years it is hard to remember what is was like to have a baby in the house. We quickly begin to read "What to expect the first year", but quickly realize that it will not be much help to us. We will need to learn as we go, and we have a lot to learn. We don't care though, we are just happy to be going home. We can manage this, it won't be that bad. We get to be a family again.

Like I said earlier, "Some of the days in that month have been very good days. Some of the days in that month have been not quite as good." The past week has been very, very trying.

As we said in our last post, Sam's direct bilirubin took quite a jump in the past week. We knew before the lab results were back that this would be the case, his color has been gradually getting worse. The lack of sleep has begun to catch up with us, we are edgy and cranky at each other, but we get over it quickly. It has helped to have other people to take our frustrations out on in the last several days.

If you have ever had to depend on any government agency for anything, we feel very sorry for you. You wouldn't think that it would be so difficult for someone in Sam's situation to get Medicaid to help with his medical care. Your thinking would be incorrect. When we applied for Medicaid on Sam's behalf we were told that they would let us know as soon as they needed any documentation. They did. We received a letter on June 18th telling us that we had until June 20th to provide documentation of our citizenship and identity, and until June 27th to provide verification of our monthly income. We hand deliver citizenship and identity verification on the 19th. We hand deliver income verification on the 26th. We receive in the mail, on the 26th, a letter dated the 24th saying that our application for Medicare has been denied because of our failure to provide the requested documents. To make a long story short our application was denied because the case worker lost the paperwork on her desk and didn't have time to process any of it when she received it because she is so busy. I am going to try this one at work and see how long I last. This situation has since been resolved only because Deb went down to the local office and pitched a huge angry fit - it is amazing how fast they can locate a missing piece of paper when there is an angry mother and crying baby in your lobby.

Social Security payments are another whole story - at least they haven't lost anything yet, they just move at a pace slower than a turtle - to date (approaching Sam's 4 month birthday) he has received a total of $30 in benefits from the federal government.

In preparation for our journey to Boston, we have had to have medical records from three hospitals and the pediatrician’s office sent to Dr. Puder. This is another one of those things that you wouldn't think would be that difficult, but several phone calls later you would understand that nothing is easy. Three out of four sets of medical records went out on the first request. I would be up all night if I told you about the fourth. Everyone has an excuse for not doing their job. Everyone tells you "I will take care of that right away". Then you find out a few days later that it didn't happen.

We have figured out that the only way to get anything done is to yell and scream. It also helps when you throw in "Your stupidity is causing a delay in getting a life saving treatment for my son" a few times. But it really is a therapeutic way to vent your frustrations out on someone who you don't have to sleep next to every night.
As soon as we get through all of our red tape we will be able to set a date to get out to Boston. If we have any updates later this week, we will be sure to post again and let everyone know.

Friday, June 27, 2008

A special Thank You

A special Thank You to Mickie, Beth, Paula, Katie, Marsha, Norma, and Terry from ATW for sending Ryan and Sam each their own special quilt. They quilts are beautiful and the boys both love them.

Not much has changed in the last few days. Sam has been very grumpy and wants to eat and eat and eat.

We got a little bit of a shock today when we got Sam's labe results back. His direct bilirubin jumped from 2.9 last week to 5.2 this week reafirming that our decision to go to Boston was a good one. We are still awaiting the receipt of all of our medical records in Boston and are still hoping to be out there with in the next few weeks.

Tuesday, June 24, 2008

Red Tape

It has been almost a week since our last post so we just wanted to give everyone a quick review of our last few days. We have heard from several parents of "Omegaven Kids" and, since we have not had a chance to respond to everyone personally, we just wanted to say thank you to those that have taken the time to send us messages to let us know about their experiences in Boston. The opinions of other parents who have been in similar situations and have had to make similar decisions has meant more to us than the opinions of the medical professionals that have been supplying us with medical advice.

We are working through all of the red tape that is needed to get to Boston. So far it has not been too bad, just a lot of information to gather and coordinate. Everything seemed so overwhelming at first, but when you take a step back and let the people that are there to help do their job it is not so bad. We are very lucky in the fact that our health insurance provider uses a third party Coordinated Health Care company which helps greatly when you are trying to get everything together and to the right person at the right time. Sam's trip to Boston will make things a little hectic again for all of us, but hopefully we will see good results in Sam like so many other people have. Jason will be flying out with Deb and Sam for the first few days and then heading back to Wisconsin for work and Ryan duty. Poor Deb will be homesick I am sure. By the time she returns home we will have lived in our house for six months and Deb will be lucky to have been here for 2 months of that time. Eventually we might finish unpacking and actually get some decorating done - by that time it may be Christmas decorating though.

Sam has had several very awake and alert days recently. He loves to smile at his brother. His color has worsened a little over the last few days and we are awaiting his latest round of blood work to see if his bilirubin levels have actually taken a spike in the upward direction. This week will be our first week that we do not have to travel out of town for his weekly doctor visit as we will be visiting our local pediatrician instead.

On Monday Sam weighed 8 pounds 3 ounces! We broke that 8 pound mark for the first time ever, and hopefully we continue to have a modest weight gain.

I have added quite a few pictures to Sam's photo gallery. Just a warning that if you look at the pictures from March 10th they are pretty disturbing. This is the day he was sent home from the birthing center and those pictures were taken just a few hours after we brought him home. It is amazing to see his pictures now and think back to that time when we were not sure if he was even going to survive through each day.

We also added a new guestbook to the journal that is a little more similar to how things were set up on our Caringbridge site. Please take a moment to sign our guestbook when you get a chance.

Thursday, June 19, 2008


Today was overwhelming for us. So much information to process and such tremendous decisions to make for Sam. It is hard enough to make decisions that only impact your own life; but when you have someone else depending on you to wade through the mounds of information, listen to several opinions, and then make a decision that will ultimately affect the quality and length of their life; where do you begin?

Sam needs his TPN (total parenteral nutrition) in order to survive. Without it he would not get the necessary nutrients that his body needs to live and grow. The problem, which is not uncommon for TPN dependent individuals, is that his lifesaving TPN is also damaging his liver. They believe that this may be caused by the Intralipids that are added to his TPN. This is where his fatty acids and a good portion of his calories come from each day. Sam's Direct bilirubin (a product that results from the breakdown of hemoglobin) has been slowly creeping up. On top of this, his liver enzyme reading have been increasing as well. This all means that it appears that Sam has begun to experience some level of damage to his liver, which could be very minor at this point, but it will most likely continue to get worse.

We basically have three options available to us right now:

1) Do nothing. See what happens. Maybe the changes in his levels will stabilize and maybe his liver damage will never become severe. If it does and his liver begins to fail, put him on a transplant list. In defense of the person who presented this option, they were just trying to present all of the options and even they do not think we should go this route.

2) Adjust the makeup of his TPN. They can reduce the amount of intralipids in his TPN and replace the calories he would be missing with extra glucose. This sounds simple. Why don't they just do this with all TPN patients and lessen the risk of liver damage? The problem is that excessive amounts of glucose being pumped directly into a persons blood stream can cause hyperglycemia. Let's fix one problem and create another whole set of problems while we are at it. (No anger there, just pointing out what we thought was an obvious flaw in that plan).

3) Adjust the makeup of his TPN. Reduce the amount of intralipids that he receives and replace the calories with a amino acid derived from fish oil called Omegaven. WHile this method is not FDA approved, it has been widely used in Europe, and there are patients receiving Omegaven in the US. So far there have been no reports of any sever adverse reactions to this treatment and in some cases it has corrected some of the decreased liver function that was caused by the Intralipids.

Can we get Omegaven here? The answer was - shockingly - yes. They can have it here and the protocol set up and get FDA approval to administer it to Sam in Milwaukee in 6 to 8 weeks. This was amazing news, we thought we were going to have to go all the way to Boston. Oh wait, there is a catch. Children's Hospital of Wisconsin will only bring in Omegaven if our health insurance will cover this non FDA approved treatment that is considered investigational. They will not be able to fund this for us because if they did it for him, they would have to do it for everyone, and that would be too expensive. Although disappointing, we can certainly understand this, this treatment is not cheap. You will be shocked, I am sure, to learn that our health insurance company will not, in fact, cover a treatment that is not currently approved by the FDA and is considered investigational.

So it appears that will be be making that trip to Boston after all. Dr. Puder's group at Children's Hospital of Boston has the ability to use this treatment and cover it's cost if that is needed. Unfortunately we were also informed by our insurance company that they also will not cover his hospital stay, doctor visits, lab work, etc... if we choose to go to Boston. Apparently it would be more beneficial for them to pay for the liver transplant in a few years and all of the necessary treatment that would entail - even if that cost would be much greater than what we are asking them to do.

It is time for all of us to get some rest after a busy day. I hope we sleep soundly tonight, knowing that we are making the best choices that we possibly can each and every day for Sam.

Tuesday, June 17, 2008

Journal Updates

We just wanted to let everyone know that we finished moving over all of the information from our Caringbridge site today. This means that if you were subscribed to get journal update notifications from Caringbridge, you will now get them from our website as well. To be removed from that list, you just need to reply to the update notification and you will be removed.

If you are not on the update notification list and want to be, simply use the "contact us" page and write "subscribe to journal" in the message.

We do not have any other news for the day. We will be meeting with Dr. Guhday on Thursday and I am sure we will have several update after that appointment.

Sunday, June 15, 2008

Rain and rain and more rain...

We often forget that there are people from all over the country who keep up with Sam's progress; the first part of this entry is for you. We received an email today from someone asking how we were coping with all of the wicked weather we have had and if we had been affected at all by the flooding here.

We have been pretty lucky here. It has been a rough week with Ryan cooped up in the house a lot, and we did get a lot of rain, and everything is mud. We did not get quite as much rain as areas about 20 miles to our South. In Oshkosh at one point over 75% of the roads in the city were under water. My home town of Fond du Lac, about 45 miles south of us, was hit pretty hard by the floods and there are a lot of areas that were affected by flooding. The pictures of the parking lot where my old high school used to be were just incredible. Both of my parents had to deal with flooding at their workplaces and things were pretty messy for them. My mother’s car fell victim to the flood waters and is still sitting in the parking lot where she works, waiting to be towed, like many other vehicles around the city.

On the Sam front, we are doing about the same. We have a routine down for what Sam needs and when. Today we made a trip to grandma’s house and were actually gone from home for over 8 hours. We were a little nervous about traveling that far with Sam for that length of time, but everything went well. It was a little bit of work to make sure that we had everything that we needed for the entire day, but we managed to get through it without any yelling.

We had mentioned the possibility of traveling to Boston in our last post. On that front we have made some decisions and are beginning to make some plans.

Sam's direct bilirubin level hit 2.9 early last week. While there are many TPN dependent children out there with higher levels than that, this still is not good. This is an early indication that Sam will most likely not be one of those miracle people whose liver will tolerate the lipids contained in his TPN without any issue. Sam is very slowly becoming more and more jaundiced, but the changes are subtle and he doesn't look too bad. We have seen a few pictures of babies who were almost orange. They can reduce the intralipids in his TPN, but then of course his growth will slow down and reducing this too low could actually cause him to be malnourished. Without some way to compensate for this there would be very little hope for Sam.

Omegaven is derived from fish oil and is widely used in Europe in TPN as a replacement for intralipids. It is not yet approved for clinical use in the United States. We are still working on trying to get our doctor here to get approval from the FDA to use Omegaven as then we may be able to at least have our follow up visits occur in Wisconsin sometime in the near future. Sam cannot wait that long for Omegaven, so we need to go right to the source and to the expert regarding this. This will mean that Sam will need to go to Children’s Hospital in Boston for an extended length of time. Starting the Omegaven will mean an initial 48 hour stay at the hospital for him, and then he will need to stay locally until his direct bilirubin falls below 2 and the rest of his lab work looks good. This will most likely mean a stay of 1 to 2 months in the Boston area. Unfortunately it also means returning to Boston every 2 months for follow up visits until we can get someone locally to handle Omegaven.

We are still in the planning stages for this next step and are working on trying to get assistance for the travel expenses that we will need to incur to make this happen. Deb and Sam will be heading to Boston for the duration of his visit, while Jason and Ryan wait at home again for Sam to come home. The good news is that with this complication out of the way, Sam's future looks a little more promising and a tiny bit less complicated if we can remove the risk of liver problems with this treatment.

Wednesday, June 11, 2008

More thoughts on medical care...

I was reading our post below and comparing it to another blog for another child who has Microvillus Inclusion Disease and what they went through shortly after the diagnoses. Unlike their situation I just wanted to add that although our doctors may not be willing thus far to apply to bring Omegaven here, they have been supportive and excellent. After reading someone elses horror story, it has made us appreciate all the more the excellent care that Sam and the rest of our family have received from Children's Hospital of Wisconsin. They are the ones who first mentioned Omegaven to us and told us that it was very promising. We are very lucky that Sam has been so healthy this long, and we owe a lot of this to the team of doctors who cared for Sam going all the way back to the NICU (and I can't forget all of the nurses who paid special attention to Sam too!). Our doctors have never told us that we should give up hope for a long, happy, almost normal life for Sam. They have been open and honest and explained all of the challenges that we may face while still telling us that there is hope, even if it many miles away.

June 11th 2008 Update

We have been home for almost two weeks. Things are starting to settle down a little bit and we are settling into an almost normal routine.

Sam has continued to do about the same since we left the hospital. His weight has gone up a little and we were approaching the 8 pound mark on Monday at 7 pounds 13 ounces.

Jason has returned to work on an (almost) full time basis and Deb will be returning to work next week for a few hours. With Sam's condition it is no longer feasible for both of us to work full time, but hopefully the few hours a week that Deb will be able to work outside of our home will give her a little break from the kids and help keep her sanity.

We have been gathering information since the day before we left the hospital in Milwaukee regarding a "investigational" treatment (which is supposed to sound better than "experimental") to reduce the impact of TPN on the liver. Typically TPN contains a plant based fat which is what is believed to cause the liver problems experienced by patients who are on TPN for long periods of time. This treatment replaces the Intralipids with a fish oil based fat called Omegaven. We have been unsuccessful, thus far, in getting support from the physicians here to get FDA approval and create protocols to have Omegaven brought to Wisconsin for our use with Sam. We were feeling pretty disappointed regarding this and are still trying to push this issue. I was contacted today by Dr. Mark Puder from Boston Children's Hospital and spoke to him regarding this treatment. He reassured me that if we can not get anyone to set Sam up with Omegaven here, that he would be willing to help us get Sam seen in Boston to start this treatment.

In an odd coincidence, we were also fortunate this week to be contacted by another family that has already gone through most of what we are experiencing - including traveling to Boston to receive Omegaven. They live in Michigan and have a little boy who is just over 1 year old and was diagnosed with the same disease as Sam shortly after birth. They went through a lot of the same experiences that we did with the testing and retesting and waiting and we hope to be able to stay in touch with them. They sound like they are doing well and it gives us a little more hope.

Although we do not look forward to Sam having to be in Boston for any period of time or the follow up visits that would be necessary, it sounds like this may be his best chance for living a long, healthy life. It was very hard when one of us was in Milwaukee and I can not imagine how hard it will be on all of us to have part of our family relocated to the East Coast for several weeks or months.

Thursday, June 5, 2008

Another Busy Day

Sam had a check up in Milwaukee at the GI clinic today, so far everything looks good. They are going to be increasing his calories in his TPN next week as his weight gain has slowed to almost nothing. Hopefully this will help him put on a little weight and plump up even more.

This evening we made a visit to the NICU in the Fox Valley for their monthly pizza party. It was nice to see everyone again and meet some other parents who had children at the NICU.

Tomorrow we plan on spending a quiet day at home. Sam will be three months old tomorrow and it is hard to believe all that he has been through in his few short months of life.

Tuesday, June 3, 2008

Another Day, A Little Less Sleep

We are still adjusting to everything new at home, but I think we are managing fairly well. If we could just add a few hours to each day so that we had more time to play with the boys and get a little more sleep...

Sam had a rough night last night, he was very cranky and was awake on and off almost the entire night. Since his stool output varies so much we never know if we are going to have a busy day or busy night. Yesterday we had a pretty good idea it was going to be a rough night when Sam had no stool output for almost 5 hours in the afternoon. It all has to come out sometime, but we prefer it to be on the low end of his cycle when we are trying to sleep.

Yesterday Jason took Sam out to a quick visit to the store and stopped at work with him, today Deb went out for lunch and took him along. We are trying to learn, in small doses, what it will be like to take Sam away from home for short and long periods of time and come up with creative solutions to what we encounter. We are determined to live as normally as possible and have Sam out and about as we would with any other baby. We have definitely learned that if you need to be somewhere by 10 AM, and you need to get Ryan ready, and Sam ready, and have all of Sam supplies ready, it is best to just forego sleep and start getting ready overnight :) .

Monday, June 2, 2008

Welcome to Sam's new website

The last few days have been pretty trying, but each day has gotten a little better. We are starting to get into a routine and were actually able to sleep for more than 3 hours last night. Sam's weight is still hovering right aroung 7 pounds. Sam is doing well right now and smiles a lot in the morning. He especially likes it when his big brother reads to him.

More than a few people have asked us to let them know when we are ready for visitors. We're ready. We may be a little tired and still trying to get used to everything, but we enjoy having company - especially if you have children who can play with Ryan. We are pretty busy on some day with nurse visits and doctor appointments, so please call first.

Now that the new site is up and running and we are back at home, we plan on updating the site with new journals and pictures once or twice a week, please keep checking back for new additions.