and there we have it

So the kids had their appointments at children's clinic today, it was better news. J'naya's numbers went way done from over 2000 to 900 ( still almost double what they want ot at they'd like it at about 550) Tony's numbers didn't really change still just over 900. Plan of action?? Eye appointments.......EKG's ( to see if the oxalate is doing any damage)....... ultrasounds (to see if there are kidney stones forming). so all in all the Dr. does`t think we`ll be able to get the levels back to `normal`so we may just have to live with what we got! She did suggeste i go on the mayo clinic website to `talk` to parents who also deal with PH1 , she also wants me to see if there is anyway we can all go to the `gathering`of the Ph 1 patients that happens every year however the last one was last summer and in new york (yup can`t afford that) so we will see! Now a BIG problem is finding a pharmacy that will make the K-citra for me so far no luck if I can't find one then I'll have to call the doctor and get another new me.

In other news.......the kids brought home report cards this week Good news all around.....Nothing below a 3 (back in the day that would be a C) and we brought home a pile of 1 ( that would be A's) so they all did well. Well the three that still live at home did the one that lives with her Dad did not show me her report card....I'm not allowed to see it....Daddy says so...I'm also not allowed to punish her.....again Daddy says so. I wonder if Daddy forgets that I carried that child in my body for 9 months, I did EVERYTHING for her for 14 years and now I am nothing.....Anyhow I guess you can see how that is going basically she is being "brain washed" by Mr. Wonderful. I'm still not sure how to handle all that.....All I want is my Ashton back....I miss her ALOT!!

meds!

I received a call today from the children's doctor, the news was not good. Both kids had fairly high oxalate output, Tony's was at 900 and J'naya's is 2100. The normal is 500. This is a concern. Now are the levels high because they aren't actually taking the meds ( We have caught J flushing hers, or hiding them in her sleeve) or is it because the meds are no longer working?? The plan is to watch them like hawks to make sure the meds are making into their bodies and retesting them in 1 month. I hope it's cause they aren't taking them. I do not want my kids to have to face a transplant!! I have also brought in a new "tool" to make sure the kids take their meds at the right dose EVERY day. Basically it's a white board/cork board, I used little zip lock bags on the bags is the first letter of the child's name and a number ( I.E. J-2 would be J'naya's for the second) so each day the child takes the bag for that day and it has all the meds they need in it. This way if either one misses I know who!! I hope this will make it easier to do. It is also on the wall which will make it noticeable and harder to forget. I also made some of these baggies to leave with my parents for sleepovers so that even at Grandma and Grandpas they get their dose!!



that's what it looks like. It's one months worth of medication ( we won't mention the cost all of which IS NOT covered by insurance) We will be offering a reward at the end of the month for all the days the meds were taken!! I hope this works!!

what???

So there are only 5 days until Mackenzie's 12 birthday, that's 12 years that I didn't get. I try really hard not to dwell on the fact that I didn't get them, instead it's 12 years that he's gotten to spend with the Lord!!! Wow what a lucky boy, one day I will get to meet him, yup meet him, you see I've never seen his eyes, I've never heard him cry. As I write this the tears stream down my face.

I love him and I miss him.

Alright, I actually got up and walked away I don't want this to be a sad post, it's not like that, I've worked REALLY hard at this. There have been alot of lessons in this the most important one is that I don't have control, these are not my children they are only borrowed to me and when their maker sees fit they are his to take home. Does this mean I'd like to lose more children. That would be a resounding NO!!! Children SHOULD not die before their parents. Or maybe us as parents are selfish and don't want them to go first because we don't want to bear the pain??? If we die before our children they the bear the pain instead of us?? I love my kids, I would do anything for my children, if I had to give my life so my kids would be healed I would in a heart beat. I wish I could have the disease instead of them, I wish it was me being poked and prodded every few months instead of them. I wish I was gone instead of my Mackenzie but that's just not how it is. He's gone, Nothing can bring him back. Trust me I would jump at the chance for just one hug, just to hold him in my arms are tell him how much I love him and have him hear me!! Which brings me to my point I have one child in heaven but I have 4 here do I tell them I love them???? Not Nearly enough!!! They are here. I should hug them and tell them ALL the time!!! I should be shouting it from the roof tops!!


After losing Mackenzie the verse John 3:16 For God SO loved the world that he gave his only begotten son that whose so ever believes in him should not perish but have everlasting life. has had a profound effect on me you see I would not allow my son to die for people who hated me and yet that's what God has done for us. He allowed his only son to die so that we could be with him one day. This brings me full circle. My son is so lucky, he spends everyday walking the streets of gold, spending time in the presence of the Lord. One day I to will join him, what a Day that will be!!!

Ph 1

The kids had their check ups and ultrasounds. In august when Tony had an ultrasound there was spot in his right kidney that looked like it may be a stone. It wasn't shadowing the way stones normally do, so this time the spot was still their but still not shadowing so they have decoded it is not a stone. This means both children are currently stone free. The good news just keeps coming the meds have finally been tweaked enough that they both had good levels. So far so good, as long as we drink LOTS of water and keep taking the meds.

I was so excited to finally get my benefits package so off to the drugstore I go, now I new that the b6 would not be covered and I also new the "new" drug vitamin d wouldn't be covered either but I was sure the K-citra would just because it used to be, Nope I was wrong we now have to pay out of pocket for all of it. It is quite costly, the vitamin D has 100 tablets in the bottle this will last 25 days and costs about $10.00 a bottle (now I could get a cheaper version of this but I opted to get the chocolate flavored chewable ones because the other meds are so big and taste horrible.) the B6 is also roughly $10.00 and has 100 tablets in a bottle but together the kids take 7 of these a day so the bottle will last about 14 days. Lastly there is the K-citra and this is the one that used to be covered and no longer is the cost of these is about $38.00 for a one month supply per child. That's a grand total of $107.00 dollars a month. That's alot now I try to remember that these things are keeping the kids healthy and there for I am VERY thankful that we know about the problem and that the kids a responding to the treatment otherwise we would have some VERY sick kids on our hands!!!



The photo above is the dose of meds that Miss.J takes everyday the smaller ones on the left are the B6 she takes 400mg.and Tony takes 350 once a day, the middle ones are HUGE she takes 2 of these in the morning and two at supper (these are the K-citra) She HATES taking these because they are hard to swallow and they cannot be broken in half or crushed because they will cause ulcers if we do. The ones on the right are the new vitamin D they are a favorite because they taste like chocolate. I try to get them to take them last so that they will "wash" away the taste of the other meds!!!

PH1

My lil bears have PH1, we have just found out (lil sister bear 8 weeks ago, and big brother bear 3weeks ago) Everytime we go to the doc it gets scarier. This week we went for an appointment with a new doc, and left with appointments for EKG's for both bears, it's pretty scary to think that this horriable diease could secertly be damaging all of my precious bears organs, in fact they would like both bears to have their eyes checked (we already had an appoinment), most people actually find out that something is wrong when the optomatrist says that there are crystals in their eyes. I am trying really hard not to worry, but it's really hard when you are told your little bears need EKG's, I thought that people have those when they have heart attacks. Life is pretty scary at our house at the moment, I wish I could go back to the days when we worried about paying the bills and the rent, not watching for signs of renal failure!!!!

today was d-day

Today was the day that the doc called to let us know that, our big brother bear also has PH1 (primary hyperoxaluria) so now not only do we have on bear with this silent but deadly killer we have two. Our big brother bear and our lil sister bear both have it the big sister bear (a.k.a grumpy) and the baby bear do not have it. You see it is one of those things that both parents have to have the mutated gene for the babies to have so myself and daddy bear both have it, and big sister bear and baby bear are carriers of the stupid gene. Which means one of my parents is a carrier or maybe they have the diease who knows? Right now all is up in the air and we are slowly trying to pick up the pieces of our lives that have landed at our feet. Hopefully the doses of vitamin B6 will work for both bears. Then we will not have to face transplant in the near future hopefully never.

There are however a few things to be thankful for inspite of the bad news.
1. It was caught early most people don't catch it till they are in renal failure and we caught it now while their kidney function is still good.
2. Brycen does not have it, he is only a carrier.
3. The kids are really doing well with the amount of meds they take, it can be battle with lil sis bear sometimes but for the amount of horriable meds they have to take they are doing quite well.
4. And lastly they did not find this before my bears were born cause if they had I would not have my beautiful bears and I would not trade them for anything!!!!

I do not feel like doing anything today

I don't feel like doing anything today, I fell like poo and I wish I had stayed in bed today, Our big brother bear has PH1 just like his lil sister bear..

yesterday I ranted today I feel better

Yesterday I ranted but today I feel better, Just so everyoneès on the same page allow me to explain, first Mackenzie Wade is my son who was stillborn 10 years ago. My sweet baby was our ( my husband and myself) first baby together he was born at 39 weeks and 6 days, he had his cord around his neck, and we did not do an autopsy. Now we have been told that PH1 could have killed him but we will never know now. PH1 is what my middle girl bear was just diagnosed with last month, she is now taking 150 mg. of vitamin B6 ( we are working our way up to 250 mg.) and she is taking 15 ml. of polycitra (you should have read the warnings with this) I guess we have to decide between the two evils and this is the lesser. So we are still waiting for results for my two boy bears for thier testing. It has been 3 and a half weeks and waiting really is the worst!!!!! So yesterday I was feeling it all, the chance that my babies will need liver transplant and possiably kidney and liver transplants that is the only cure at the moment. It is pretty scary to think that I can not go to the pharmacy and get something to fix them, but that in order to toally cure them they will need new livers. Livers do not grow on trees and we have been told that myself and the daddy bear would not be able to donate ( I have yet to confirm this as I have also heard that its possiable from people who have done it for their baby bears)

So for now we try to keep from harming our lil sister bears kidneys by druging her with things that make her tummy hurt, and pray that the damage to our brother bears kidneys is not horriable and his kidneys can be saved, and we are praying that our baby bear does not have this horriable, silent killer!!!!!

Now that you have been updated you can see why I have bad days!!! Yesterday was one of them!!!!