Thursday, May 21, 2009

5/21/2009 - Update

I know, I know...why has it taken so stinkin' long to post a post about our little darlin'. Whatever I would say to answer would be pretty lame and I'm fully on board with the fact that this kind of delay is completely unacceptable.

ANYWAY...

It's been so long I'm not certain exactly where to begin except to say -- inn a nutshell, I guess the last eight months have been more of and much the same. So that's it. No need to read further.

But if you must...

Josie is thriving from a growth perspective. She gaining weight and height at a nice clip and has now found herself within measurable range on the growth curve well into the normal range. That's great news. Her strength has continued to ever so slowly improve as well. Now she's taking stairs with little difficulty, running with the other kids (though a little slower than the rest), and generally playing around just like every other kid, just a little slower. She does need to be careful being active as she's still relatively fragile.

In just two short weeks from now school will be done and first grade will be on the horizon. Kindergarten has gone very well for Josie. She's made some great friends and has fit in perfectly. She's progressing both academically and socially right in line with all the other kids. After being so sick for so long and really having an abnormal early childhood, Josie has rebounded so nicely and is developing into a really fun little kid...so cool to see.

Probably the most immediate news is that we're back in the hospital for a short three-day stay. This time, though, it was a planned visit to administer a new drug cocktail which is hard-core enough to warrant constant monitoring during its administration. Josie's disease is still very active, but not acutely crazy like before. The big problem now is her raging calcinosis. Calcinosos is the abnormal deposit of calcium salts in body tissues. Josie has extensive calcinosis all over her skin and within her ankles, knees and elbows. The calcinosis was getting bad last summer and fall but over the last three months she's gotten a lot worse. Her arms and legs appear to have tumors all over them; her knees are so calcified they appear very abnormal. When people see them for the first time most are clearly shocked at Josie's appearance. Luckily the calcinosis has stayed away from her face and back for the most part, but she has developed a couple pretty big nodules under her chin which are starting to draw stares and attention. Josie handles the friendly child questions about her appearance well...and it's interesting observing the reaction of some who see her and try desperately to keep their eyes away. The kids just stare, but the adults just can’t help themselves…whatever.

The question of the moment is what causes the calcinosis and what can fix it? Both have the standard medical explanation; 'Idunno'. And it's a fine answer because they absolutely do not know. We've been trying so many drug combinations to try to get the calcinosis to stop but it's been getting worse. Earlier this month we headed back down to the Mayo Clinic in Rochester, Minnesota (nice to be close) and had a very pleasant visit with a big-shot rheumatologist (Dr. Ann Reed) who we saw three years ago when Josie was getting underway with this. Dr. Reed was impressed with Josie's calcinosis and provided us with some different ideas on new courses of treatment. Dr. Vehe and Dr. Reed have since spoken and discussed Josie’s case at some length and along with some other smart folks in the biz cooked up an interesting treatment regimen that will hopefully show some results sometime soon (soon can have so many meanings, can't it?).

We learned that this calcinosis is a known complication of Dermatomyositis and in the bad cases of the disease (like Josie's) calcinosis follows the really bad part of the disease's courses...kind of like fires after a nuclear blast (not sure if that's an accurate comparison, but there it is). As calcinosis progresses the pockets of calcium can be hard like rocks or firm nubs like erasers or liquefied pockets like pus-filled abscesses, or firm and large calcium sheets. Josie has some of each. Her knees seem to be the liquefied kind and are now starting to break through to the surface. One particularly remarkable one seemed to be getting worse and worse over the period of two months and started to really thin the outside skin as it grew. It looked like a humongous whitehead. One evening during a bath Josie called Mom in because it had finally popped, and what a spectacular and disgusting event that turned out to be.
The liquid calcium oozed out like pancake batter and there was lots of itDeb estimated a quarter cup of the goop came out (See picture). All I can say is WOW, was that something to see! Since then a few more have burst, but none with that kind of drainage. There are others that have broken through which reveal a little rock and now look like small white scabs. Most of the areas are not painful unless they get whacked pretty hard and then they hurt. If Josie spikes a little ...fever then most of the areas flare up, turn red and are painful...especially in her ankles and knees, and at those times it does become difficult for her to walk until her fever comes down.

So what to do? Josie had been on a daily injection of Kineret (rheumatoid arthritis drug which is supposed to stop a particular protein (interleukin-1) from causing more tissue damage and to reduce pain, swelling, tenderness and inflammation in your joints) for a couple months with no clear signs of help so we discontinued it (and Josie likes the idea of not taking a painful shot every bleepin’ day). She's been started up again on a small daily dose of steroids to try and stop the progress of the calcinosis but, again, it's unknown if that's had any effect. One thing for sure is that her appetite has improved since starting the steroids again. She's still getting 750 calories every night through her feeding tube to maintain her weight and calorie intake. Other drugs she's on are:
· Diflucan (Anti-fungal)
· Tacrolimus (imunosuppressant)
· Pantoprazole (Protonix: proton pump inhibitor to decreases stomach acid production)
· Cellcept (Immunosuppressant)
· Metaclopromide (Reglan: increases muscle contractions in upper digestive tract)
· Methotrexate (a chemotherapy drug used in blocking the metabolism of cells)
· Prednisolone (steroids)
· IVIG (Intravenous immunoglobulin: concentrated antibodies from healthy people to boost immune response)
· Sulfamethoxazole (Bactrim: Antibiotics)
· Hydroxychloroquine sulfate (Plaquinil: Antiinflamitory)

We've decided to take a little more aggressive approach to the calcinosis problem by walloping Josie's system with a combination of a drug she's had before and a new one. Rituxan is a chemotherapy drug meant to destroy B cells (lymphocytes that play a large role in the humoral immune response). It seems that those B cells are the buggers causing much of the problems with Josie. She's been on Rituxan before with little measurable effect, but in the experience of some big-shot rheumatologists a few patients have responded well on the second go around with Rituxan after marginal first-round success.

The other drug is Pamidronate which is a treatment for osteoporosis and bone loss. Pamidronate pulls calcium into the bones from the body. The bones have a kind of sponge effect and suck up calcium...sounds like it should work, right? Well...there's a lot of calcium available in the body, which explains where all Josie's skin calcium has come from, but there's no way to know if this Pamidronate trick will pull the calcium out of her skin...which it might. What it also may do is throw the calcinosis a curve and interfere with the calcinosis process. There have been a few known cases where calcinosis patients have been given Pamidronate and have shown their calcinosis start to rapidly melt away.

To administer this cocktail Josie had to be in the hospital. It's a good thing we were there too because within a few minutes of the infusion starting Josie had a bad reaction, changed color, started feeling bad, vomited...classic bad drug reaction. Things were put on hold and then restarted again after a little while. Since then things have gone well. And Josie cannot wait to get out and back home. This time, though, it looks to be a short stay.

So there you have it...eight months wrapped up in a rambling mess of a blog entry. Again...it’s much the same and more of the same, but all-in-all, things are going well.

20 Comments:

Blogger britta said...

Josie!!! Looks like you and I both like to keep on giving challenges to Dr. Vehe! Hope you have a wonderful summer! :-)

12:44 AM  
Anonymous Anonymous said...

Josie, you're a beautiful girl!!!

5:22 PM  
Anonymous Anonymous said...

thank you for sharing your story. I am a new graduate nurse and I think it is wonderful that you post Josie's story.

5:12 PM  
Blogger Michelle said...

I just happened upon your blog and look forward to following Josie's story.

7:04 PM  
Anonymous Casey Bullard said...

I was lookinf for something else and found this! I will keep Josie in my prayers!!

1:52 PM  
Anonymous Anonymous said...

What a beautiful girl you have! And how strong you are. God bless you and give you even more strength. I admire you. Thank you for sharing this story with the World.

3:53 AM  
Blogger Maria said...

I ran across your blog while I was googling something else. The first thing I noticed was the photos on the blog and as I continued I began reading the blog to its entirety...each time crying with each entry...I could never imagine having to experience this with my own kids..and I commend you and your family on the strength and determination that you have had and have to make it through. I look at your daughter at the age of 3 which my daughter is one year older and son a year younger...and feel so terrible that my children have the ability to live normal fun lives at this age and yours does not. God has a purpose in life for all of us, and Josie's is to inspire. You can tell she is a fighter...I am sure you already know this. One day at a time, a lot of prayer and a lot of love. Thank you for making this public and I more appreciate my own family because of this blog. You think that you have everything in the world..and never notice..then something like this sends you back to reality and you realize that any thing can happen to anyone. Stay strong and keep blogging. Josie will be in my prayers.

8:38 AM  
Blogger janine said...

Such a sweet little angel my thoughts and prayers are with Josie you and your family stay strong Take care Janine

5:11 AM  
Anonymous Anonymous said...

Hey Adams Family
The blog helped me to understand Josie's medical situation. She is a real trooper and hats off to you for juggling a full family of four and your menagerie of creatures. As always, my pal Josie is in my prayers. I do so enjoy my time with her on the soccer sidelines.
Julie RB

1:19 PM  
Anonymous Aubrey said...

Oh, what a sweet girl. I also found this, googling something entirely different.
I commend you guys for being able to deal with this. I am a mother, and my heart was sinking, reading all of this, and seeing the pictures. Your poor baby. I cannot understand why a child has to suffer like this.
I am praying that she can find superb health soon!

4:10 PM  
Anonymous Anonymous said...

I also didn't expect to arrive on this blog when googling some business pictures...

But what a story!! We really should take care of our own health, when we are blessed with it and don't complain when we are sick a little.

Josie is a really strong girl, she deserves to be healthy in the end, for the rest of her life as a reward for her long struggle.

May God be with you all always.

2:00 AM  
Blogger me said...

Hi, I was just now searching the web for a strange looking skin-condition I seem to be developing that may be connected to my Lyme Disease diagnosis and came across the blog for your sweet little angel. I just want to say, I do not normally write on people's blogs and never sign electronic guestbooks (esspecially for total strangers) so writing this message is a total rarity for me, however Josies struggles and triumphs moved me into action, compelling the need to say a few things in hopes my 2 cents might help you, as parents of Josie.

As I read the Josie Blog, I could not help but start wondering if Josie's condition isn't in some way, shape or form related to Lyme Disease after all. I know you said she was tested and came out negative, but I currently have full blown Lyme after getting tested for Lyme on 3 seprate occasions through my Insurer's HMO that showed I was LYME NEGATIVE, only to be tested a 4th & 5th through IGENEX one year later that showed I was POSITIVE for Lyme all along!

Many 'Lyme Literate' experts agree there is technically no such thing as "auto-immune," our body isnt designed to suddenly turn on itself and cause auto-immune through self-cannibalization while manifesting a host of symptoms that resemble named-diseases and result in our misdiagnosis and resultant labeling as a sufferer of them, while the true origin for themysterious symptoms, stays ever elusive.

From what I know, "Auto-Immune" is just a catch-all phrase invented to placate the masses and generalize a far more insidious disease that is diminishing the quality of life for so many of us inflicted by it.

So while it may appear that the body is attacking itself and causing the auto-immunity, that's tipically not the case. What the body is actually doing in an auto-immune scenario is attacking a stealth bacteria that has latched onto and begun literally devouring our cells, which makes it appear to be attacking itself, but in reality is trying to take an enemy out who is wearing the disquise of a normal cell.

Lyme is often called by insiders "The Great Stealth Virus" because the numerous bacteria which causes the infections, stealthily cloak themselves within our cells and mimic cellular behavior so they are indistinguishable to the real thing, which confuses our body's macrophage and killer T-cells.

The problem here is that our bodies have a hard time trying to figure out who the real cells are and who's wearing a disguise, thus what appears to be an auto-immune response is actually our body reacting on both offence and defense.

This results in a futile waging of war against a stealth invader as our body begins hunting down the enemy within, feverishly working to identify, destroy and purge billions upon billions of infected cells, and making it appear as if our body has turned against itself, developing into the signatory auto-immune mystery disease sufferers of Lyme are commonly misdiagnosed and labled with. Perhaps Josie's little body is fighting against the enemy within and doing it to the best of its ability. Thankfully there is hope, there are cures and there are answers.

So please, please, please keep an open mind, this may be the missing key to your wonderful daughter's illness. And please understnad, for the most part, the medical community is ignorant about these areas of study because so much of what they do on a day-to-day basis is controlled by corporate politics, Big Pharma and Big Insurance who always put profits as a priority over our health and wellbeing.

For Josie, take 5 minutes and click the following two links, they might just open your eyes to what's really going on inside your daughter's body and become the catalyst to what will provide her with a longterm solution, cure and most probable, the quality of life that she absolutely diserves.

http://www.UnderOurSkin.com/watch.html
http://bit.ly/lOwmX

Keeping your family in my prayers. The answer is out there, soewhere. :)

9:49 PM  
Blogger Jane said...

What a journey you have all been through. I just want you to know that I've read your stories and my compassion goes out to you and your little girl.

12:30 AM  
Anonymous Anonymous said...

Please: check out bacteriality.com and find out the latest science on Josie's conditions including the knowledge on how to cure them.

4:00 PM  
Anonymous Amanda Cardinal said...

Josie you are one tough bear!
You and your family have given me a new perspective on my life and the lives of my little family.

Josie of all the things that have happened to you, i couldn't even begin to imagine!

Next time i feel glum or think i even have the right to complain about my day, i'll have your story to reflect upon and then that'll slap myself out of a funk!

To Josie's family,
Reading this blog has indeed changed me. If i were you, i would make this into a book to get all of your struggles and adventures known.

Pulled on my heart strings as soon as i saw the first picture of little Josie with her lips and face all puffy. I wanted to reach out and hold her. She reminds me so much of our little girl Zoe.

Josie your very beautiful and your quick wit will indeed be a great advantage in the big wide world i have no doubt you'll master!

A Fan For Your Total Recovery And A Day,

Amanda Cardinal :)

9:02 AM  
Anonymous Anonymous said...

first I would like to say to Josie that she is SUCH a couragous young lady. I have such emtapthy for you and what your family must be going through on a daily basis. I was wondering as a current nursing student how you are doing now. I would also like to thank you and your family for posting such an in depth diary of your trials and your daily challanges. My thoughts and prayers are with you. I am sure with gods will and such great health care you will grow up to be a wonderful woman.

11:27 PM  
Anonymous Kate said...

Hello, I'm from Australia and found your blog while I was googling 'blistery rash'. btw, what a beautiful child you have - she has such a lovely face.
I have celiac disease and sometimes get a mild rash even on the diet. I've become certain that it is food/ autoimmune related. My mother and brother are also celiac and my mother also has rheumatoid arthritis. It's amazing what havoc a little gluten can create. Unfortunate genes! My question might have been answered in one of your previous entries (sorry if so) but does Josie also have celiac disease?

4:03 AM  
Anonymous kaia said...

you are great mom. josie is sooooooooooooooooooooooooooo pretty god bless you. my sister has the same promblem

12:28 PM  
Anonymous Anonymous said...

I came across your site after googling for a while because I am about to undergo surgery for the first time. I am scared, but after seeing what Josie goes through on a daily basis, I now feel as though there is nothing to fear. She is such a strong little girl and your blogs have brought me to tears. I pray for her and your family. I hope all the best and that Josie will one day be pain free. God bless you and your family!

5:46 PM  
Blogger Susan said...

Hi,

I also found your site looking for something else, but I was wondering if your doctors tested the oozing calcium for what type of calcium it is?

We've had a woman with fibromyalgia who had lumps all over her body and they went away by reducing oxalate in the diet.

I run www.lowoxalate.info.

Oxalate can bind and redistribute calcium, so I don't know if this could be related to what is causing these lumps, but if I can help at all in exploring this possibility, please let me know.

I run a yahoogroup where people reduce the oxalate in the diet and it is changing all sorts of unexpected but welcomed things. I think there is a possibility that oxalate that gets into circulation because of gut issues (like short bowel syndrome) can egg on autoimmune processes.

I'd love to talk to you, and am so taken by all you've written here.

Here is a cutaneous lesion caused by oxalate that was in this woman's body because of a gene defect. For most everyone else, having too much oxalate is from eating and overabsorbing high oxalate foods.

http://dermatology.cdlib.org/101/case_reports/oxaluria/hsu.html
for comparison.

I promise you that your doctors would never think of this possible link. but it is an easy hypothesis to test for its relevance.

Susan Owens lwo@iadfw.net

5:10 PM  

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