More Decisions

So some of you are wondering how our appointment went yesterday. Never have we looked forward to appointments as much as we do these Lung Specialist appointments and I'm so thankful that Rob comes with each time.

Yesterdays appointment did not really bring much in the way of surprises. What we heard is somewhat what we expected, although not necessarily what we wanted to hear. The doctor does not feel that the treatment is helping James as it should; She had expected better improvement by this stage. The only encouragement is that James' LDH levels continue to drop: July 16 - 3700 units per litre; August 20 - 1537; September 5 - 1220. We are not in the normal range yet (500-900), but getting much closer. The puzzle is: why are the levels dropping if the treatment doesn't seem to be working? Just because James is clinically struggling, does this mean the treatment is not working? Does he have to struggle through this to reach the better stage? Can we expect him to all of a sudden "pop out of the water"? How long should he struggle before it's too late and his body isn't able to go through the lavage? That and so many other questions remain looming.

The doctor still hopes that there will be a sudden breaththrough and turn-around stage. The other PAP case they had also had struggles with coughing up phelgm at first and then suddenly turned around. This child was older and did not have the vomitting issues, but likely this is because he/she was old enough to control this and just cough out the phelgm. By three months they seen a remarkable difference in this child. So far James does not look like he's heading in this direction. It becomes a questions of how much do you compare? This child turned around in three months ... what if James was to turn around in three and a half months and we don't wait long enough to find out?

Although the doctor says she hopes there will be a break through, she is not completely happy with how he has been doing and has decide to prepare for the next step.... a Whole Lung Lavage. I have previously written about this (click here to read full version) and will just copy and paste so you can get the general idea:

What if GM-CSF doesn't work?? Then we move to the more common treatment of Whole Lung Lavage (WLL). This is where they literally wash out the lungs. When they do this they keep one lung going while they wash the other one out ... with up to 50L (compared to 100ml in a bronchoscopy) of saline water. They allow you to recuperate for several days and then do the other lung. When reading on the internet it all sounds so simple but we are told there are great risks to doing this treatment as you wash the good with the bad and put the lungs under great strain for the process. Even anesthetics are difficult and require a anesthesis who is specifically trained to use double tubes to keep the lungs going seperately. For a normal adult this is a critical procedure and for James ... well it's very critical. They have never done this on someone so young and his lungs are not large enough for the equipment to even go down as they normally would. All she said is that if it came to that point, they would find a way to do it ... but this would be no small procedure. There is also a possibility that even if the GM-CSF treatment is working they will still need to do the WLL in order to get out the build-up that he has accumulated in the past year.

The current plan is to continue with the GM-CSF treatment for another month and then do another CT Scan and some specific blood work. On that day if we see that there is little to no improvement (or degression) then we need to proceed to the WLL. As the doctor believes that this is where we are heading, she is already in the process of getting this booked and it will likely happen within a couple weeks after the decision is made. In this we she that she is very pro-active. On initial diagnosis, she said that without treatment, James had a one year life expectancy. We are now two months into this year with little obvious progress. To give this treatment a fair trial, we should be administering the GM-CSF for three months. Her approach is that if after three months we do not see any improvement, we can immediately proceed with the WLL. NO messing around!

We did not get into great detail of what to expect if they do a WLL. She has already advised us of the risks involved in this procedure and that there is a chance of death. This is another reason why she is already getting prepared for the procedure. It is rare and has great risks. She will be contacting the Doctor in France who has done the procedure on a young child (the only other time that we're aware of) to get guidance. As well she has to find an anethesis who is able to meet the challanges of double intubation. She advised that they will do one lung and then if he is able the other a couple weeks later. She also told us that she expected he would be in hospital for a day after the procedure. We were surprised by this as almost all the information we have read about this have said it's about a five day hospital stay. That being said, we'll be expecting five days (or more) even if they say only a day ... they do not know James as well as we do!!

I have often said I did not know what I would do if confront with having to go through this procedure. Surprisingly at this time I am much more calm then I thought, but that is likely because a final decision and date has not been set. James has been through many procedures already and each time we realized that there was some risk to the anesthetics (more so as time went on), but never have we truly had to kiss him good-bye with the realization that there was a great chance it may be the last time we do so. We remind ourselves that if treatment is not working we have no other choice but to go forward. As hard is it will be that day we know that without the WLL, humanly speaking, his prognosis of surviving is slim, and we are just prolonging the inevitable. We can do nothing more then lean on our Heavenly Father for strength, and submitting to His plan.

With regards to the fevers James is having the Lung Specialist believes they are related to the treatment. Fevers have been reported in relation to the treatment and since James is going in and out of fevers (daily), and not having consistent fevers with high spikes, it is likely the treatment. They would still like blood work done to check his white cell count and see if it's up. If it is they may decide to give him a round of antibiotics.

We have been asked several times about a lung transplant. Although a transplant could be done it would likely not be an answer to the problem so it would only be a very last resort if they thought there was any chance it would help. They have tried this on one other LPI patient and the new lung developed PAP as well. They know little about PAP, what causes it or how it is related to LPI, but this shows that it develops because of some other issue(s) within the body.

So we will be confronted with a major decision next month. Likely the answer will easily come out of the tests, but if it is not an obvious answer we pray the Lord will guide us when the time comes to think clearly. At the same time we are confront with another major decision this weekend. Is it wise for Rob to continue with school with all this uncertainty, or postpone it for a year?

This decision has been laying heavingly on us the past week with this coming Monday being the deadline to drop out and be refunded. Rob has already felt the effects of the extra work involved in taking care of James. Until now Rob has been in charged of James at nights; this means little to no sleep as he is up almost hourly (and often more) with him throughout the night. This week we started with night nursing three nights a week to allow some rest. We hope to get more hours to allow for a couple more nights a week of assistance (won't get into the struggles we are having with that right now). Since I am getting little to no sleep just listen to the constant alarms going off I will likely start taking over James for the nights as Rob just ends up falling asleep at his desk right now. Unlike me, Rob is able to sleep through the monitor and alarms if he knows it's not his responsibilitiy (and sometimes when it is his) :) Matthew is at the stage where I can hopefully balance this, and if we move him into our room at night then hopefully Rob will wake up if he cries too loud because I'm busy with James. Needless to say I have been functioning on survival mode for almost 4 months now and this is only a temporary solution until we can get our nursing battles sorted out. This past week's nursing was nice, but it only made me more tired because my body suddenly realized what it's been missing out on.

Anyways, I was talking about school. James takes up a lot of our time and this has left Rob with little study time and a lot of responsibilities. How will he balance school and assisting with the care of James? We have introduced another change in our house this week to help in this area. We now have someone come in almost daily to help me out with anything and everything. This allows me to focus on taking care of James and hopefully also giving me more time with the other kids, not to point out trying to get some of those things done in the house that only I can do. So if James were to stay home and we have nursing and the extra help would Rob then be able to manage? Likely .. but what if James were in the hospital? Before he was at Mac and it was easy to pop in and out, but now James will be in Toronto. It seems inevitable that he will end up in hospital, it just a matter of how often, how long, how serious ...etc. There are so many factors to weigh out and it's always hard when you have a deadline. Should he just drop out, take an 8-5 job, and post-pone a year? What if that year turns into 2, 3, 4, etc? Should he forge ahead and hope for the best? Should he go part-time and do summer school in order to have his degree before next fall? How much of his responsibility as husband and father can he delegate out, in order to finish pre-seminary education? Sometimes it seems like the answer seems obvious and then it's not so clear.

Through all this, several people have offered to help in many ways, for which we are greatful. We also realize that consistantly having different helpers is in the long run more work and time consuming than if we do it ourself. Any employer can vouch for the expense training new employees can entail, due to inefficient productivity and screw ups. Because it is near impossible to establish a dependable schedule, its also unrealistic to spend all morning on the phone trying to find babysitters and what not else. For this we are optomistic that our new helper will take all the drudgery items off our agenda.

Well this blog entry has become plenty long and it is time for me to go back to work, and muddle through the many thoughts that continue to go through my head.