Soliris Approved! Christian to begin therapy in November.

Wow! This has been an amazing year of updates!

We got word today that Christian has been approved to start infusions of Soliris immediately. With the confirmation that his retinal stroke back in August was a result of a recurrence of active HUS, Dr. S, was able to secure treatment with Soliris beginning in November.

The plan is weekly infusions at TCH for 5 weeks, beginning Nov 14th, then we will go to the standard "every two weeks" infusion schedule.

With this acceptance, Dr S will also begin to prepare Christian's case for submittal to the TCH medical review board for a kidney transplant.

What a year this has been... being able to see coming to fruition the hopes we have had for years - that there would be a miracle breakthrough in this terrible disease, discovery of it's causes and a therapy that can manage it and give Christian back a "normal" life.

We are right there on the cutting edge of all of this becoming reality. The feelings are too much to put into words.

Thanks for keeping up with us and take care.

Kiki's Dad

Update on Christian's retinal stroke

Christian and Mom traveled to Houston to get an MRI and see an opthamology neurologist in order to assess the damage, see what factors caused the stroke and determine a prognosis and therapy/recovery protocol.

The news is a bit mixed. The good news is that the damage appears to be limited to some mild loss of peripheral vision. Christian has recovered much of what was initially lost and that is great. He can see straight ahead just fine but gets blury as he concentrates on the peripherals. The specialist believes that he as recovered all that he will, but this is ok.

The not so good news is that our Nephrologist believes this may be the result of an ongoing episode of HUS. As we often forget, HUS not only affects the kidneys, but it affects the nervous system, the blood system, the brain and many organs in the body.

He is going to conference with some other Nephrologists and get some other opinions.

One side benefit of this is that this could be an opportunity to get Christian approved for Soliris treatments now, rather than being forced to wait until the time of transplant.

Definitely a silver lining, if there ever was one.

We will update with more info as it comes in over the coming weeks.

Take care.

Kiki's Dad

Bad news: Christian has had a mild retinal stroke

Well this is news that has come from left field. As you know, with these kids, things can be going well for quite some time and then seemingly out of nowhere they present with some new issue.

Christian got sick yesterday and then lost vision in his left eye. At first we thought it might be blood pressure related. We got him to a specialist who examined him and determined that he has had a mild stroke in his left eye. He does not think it is blood pressure related as he reviewed Christian's home logs from his daily dialysis and his pressures have been ok.

We are bringing him to TCH to get an MRI done. At this point most of his vision has returned, but he cannot see peripherally - he has essentially a tunnel vision in that eye.

Will update you all with more info as we get it. Keep us in your prayers. Thanks.

Kiki's Dad

Updates for 2011

Well it is hard to believe that more than 2 years have passed since we have posted regular updates. Looking back on the posts from 2008, there were so many changes going on with Christian's care. We were really experiencing daily trials while learning how to handle life with this new method of dialysis + the job loss post-Katrina and the endeavor of self employment.

A lot of water has passed under the bridge in 2009 and 2010 and I can tell you that it has been a tremendous experience of lows and highs. I'll go into more detail with posts to follow.

For Christian's care, there has been some miraculous advancements in the field of study for his disease. The short story is that the wonderful genetics team at the Univ of Iowa have run the protein chain and figured out 3 or 4 'triggers' that cause Atypical HUS. We know now why Christian's body does what it does. Wow.

And almost on divine queue, a new drug emerges, Soliris, that turns out to be a silver bullet for stopping the progression of Atypical HUS in it's tracks! Mega wow.

Soliris has been in clinical trial for about 18 months now and has what I believe is a 100% success rate in halting the bad part of the Atypical HUS progression. It does not stop Christian's body from initiating the progression of trying to fight infection, which is great, but it takes over the role of protecting his body, which happens normally in people without the deficiency of HUS.

In layman's terms I present it this way: It does not stop the team from taking the ball down the court on you (the body's immune system response), but it is like putting Shaq right in front of the goal and he swats away every single attempt of the opponent to slam dunk the ball (killer cells) into the basket (your healthy cells).

Of course, Soliris is the most expensive drug the world has ever seen (more than $400k per year at full dosing, google search Soliris and Forbes magazine for a neat article Forbes Article - World's Most Expensive Drugs) but we'll cross that path when we get there.

So, for 2011 we are working towards transplant + Soliris. And getting our son set up for a long, long run at life. THAT is answered prayer.

Take care,

Kiki's Dad

Lot's of changes - moving forward positively!

It's been a while since my last post. We've had a lot of change in the family these past few months to update you on.

As you know the past 1.5 years have been dedicated to moving Christian to the new in-home hemodialysis. This process has been a larger task than we anticipated.

All of the time missed had Christian running behind on schoolwork at Kehoe and it was decided it would be necessary to hold him back another year to keep going there.

Coupled with the financial crunch we have been working through, it was very obvious we had to make a change to public schools for the kids. Late in the summer, we made the decision when it was apparent some work I was expecting to start got either delayed or canceled.

So we have moved the kids from 1 small school to 3 different larger schools. The kids appear to have adjusted very well - God Bless 'em.

However, we were still having a terrible time with a family routine - Christian would get home at 4:30, we'd eat dinner at 5-5:30 and start getting him on the therapy at 6-6:30.

The problem is, sometimes it was taking as long as 3-4 hours to get his accesses started and so the other 2 kids were left to fend for themselves and Christian was going on therapy until sometimes 2am.

Even on days when things went right, we were having to leave the other kids to themselves and this was becoming a problem.

Well, we have moved Christian to a program at his school called "Hospital / Homebound". We started it yesterday and it has been wonderful.

Essentially Christian goes to school 1/2 a day, and we use the other 1/2 for dialysis time and teachers come to the house to teach him.

We are starting his therapy at 7am - usually have him connected and running by 8-8:30 and the teachers come between 9 and 11.

We've only done this morning routine for a few days now but it is WONDERFUL.

Christian will be going back to the hospital for a week to create a third buttonhole again - replacing the one that we lost at the beginning of the summer. Not sure of the date yet, but we'll let everyone know.

Take care,

Kiki's Dad