IVIG OMG!!

Today is Infusion Day for Lola. This is our monthly ritual. So as I was laying in bed this morning, it dawned on me that this month marks the 5th year of her receiving monthly IVIG infusions!! While she has been getting Solu-medrol and Methotrexate infusions since day one of her diagnosis and weekly ones for a couple of years at that, I cant believe that it has been a whole 5 years that we have been doing the IVIG dance. That is roughly 60 infusions! With these bad boys costing around $22,000.00 each, that's alot of dinero in 5 years! No wonder my insurance company gives me hell when trying to get a prior authorization for these treatments. Many people ask me what is IVIG, so here is a little tidbit of information.
Intravenous immunoglobulin (IVIG) is a blood product administered intravenously. It contains the pooled IgG (immunoglobulin (antibody) G) extracted from the plasma of over one thousand blood donors. IVIG is a blood derived product that has been used in the treatment of children with JDM. IVIG consists of purified antibodies which are proteins that the body's immune system uses to fight infection. The mechanism of action is unclear, but IVIG has been shown to slow down the inflammatory process. Side effects like headache, fever, and vomiting can occur, but they are usually prevented by pretreating with Tylenol, Benadryl, and sometimes the addition of a steroid.

I have been very fortunate that my dad insists on taking her to her infusions. She feels comfortable and at ease with him and he wants to be there for her. And he is retired so he has nothing better to do!! LOL! When we first started doing these all day long infusions, it was a bit easier, she had a port then. All they had to do was access the port and viola, it was going. Then that port was no good and we had to get another port and then that one lasted a whole 2 weeks. So then we were told that she could no longer have another port as they believed her body rejected them and that was what was causing the damage to the port tubing, the calcinosis that forms in her body. So then it was IV sticks and that was not fun. It used to take me, my dad and an extra nurse to hold her down, that was even after sedating her with some Phenergan. Allorah gave them 2 chances to stick her and if they couldn't get it on the 2nd try, we left, that was the agreement. She runs a hard bargain, that girl. Well, she became such a hard stick that I finally starting asking for the anesthesiologist to come in. We have been so blessed that they have been so willing to come in and start her IV each time we have gone in, so a big thank you to the anesthesiologists at GCRMC, especially Steve. You all have made this a lot less stressful for Lola and for us. Allorah now just goes in and weighs herself and gets settled in and sits by herself while they start her IV and get it going. No complaints or tears anymore. She is truly amazing. I hope and pray that one day there wont be anymore infusions, any more medications and no more doctors. That she will be cured and in remission and this will all be in the past.

Chicago Info

It was a whirlwind trip this time and we barely had time to sit back and relax. It was GO GO GO the entire trip. We are back home and back to our "normal" routine. Her appointment went well and she grew 2 inches!! That is a big deal for kiddos who are on Prednisone or any long-term steroids, as they stunt the growth. As soon as Lola walked in to clinic, everyone commented on how much taller she was! Dr. Pachman was so pleased at how well she looked. We met the new the PA and a few other new faces. I sure hope Doc gets some more help, she is so swamped with JDM cases. There are some concerns that she wants to address, as Allorah is complaining of trouble swallowing food (dysphagia), which is something that is common with the progression of JDM, that concerns Dr. Pachman very much so she will need to have a swallowing study when we come back in January. She also has to have another pulmonary function test to follow the slight pulmonary fibrosis that she has in her lungs. That will be done in January as well as a consult with the pulmonologist that is Dr. P's associate. So far her PFT's have come back normal so lets keep praying for that. She is also scheduled for a bone density scan (DEXA) to help check and follow her osteopenia, that she has developed from the long term use of Prednisone. She has a lump on her chest that she is sending us for scans to check on. It may be calcinosis or a possible cyst, but whatever it is, it can be removed. Her muscle strength is about the same but her range of motion is worse. Her flexibility is tight and less so she stressed the need for her to increase her exercises and therapy at home or they will be putting her back in PT a few times a week. She was not thrilled about that, but either she takes this serious or she will hinder her ability to walk right and have proper posture and have increased pain. The whole gamut of labs were drawn and we should be having those back in about 2 weeks. So its the waiting game until then. We are hoping that we can continue to decrease her Prednisone and possibly the MTX as well. I hate that shot!!
We travel back at the end of January and hopefully by then she will be more flexible and better labs, so I'm crossing my fingers!!

Did She Seriously Just Say That??

The random things that come out of my daughters mouth's from time to time are enough to crack me up and sometimes make me choke on food or whatever beverage that is in my mouth.
Me: You will be grounded until you bring your grades up. Period.
Allorah: I cant work at the church fiestas this weekend then?
Me: No
Allorah: But grandma and grandpa already told the church people that I would work the booth and help them out. So I still cant go?
Me: Nope
Allorah: So, let me get this right, your telling me that I cannot work for Jesus this weekend?
Me: That's what I'm saying, I will have grandma personally tell Jesus that you cant be employed by Him this weekend because your grounded and I'm sure He will understand your dilemma.
Allorah: Boo.

It's gonna be a long weekend so thank goodness my husband will be home this evening! LOL

JDM Milestone

So this may sound like a "no big deal" for most people but for our family, this was a milestone that brought tears to my eyes last night. As most of you know, Allorah has Juvenile Dermatomyositis, which is an auto-immune disease that affects pretty much her entire body including her muscles and range of motion. If you have ever seen her, you will know how tiny and skinny she is, especially her arms. As long as she has been around in our little world, I have done her hair everyday. She is 12 and has the most hair on a person I have ever seen. Because she lost so much at one time because of the chemo shot that she is on, it grew back very course and frizzy, so much so that its impossible to let her hair down without me straightening it, curling it or putting it up. Her arm strength and weakness has prevented her from being able to straighten her own hair, as it takes at least 45 minutes to an hour for this process. She would attempt to but her arms would get too tired and she couldn't keep them held up above her shoulders that long and would ask me to finish the process. Well last night she walked out of her room with the most perfectly straightened hair!! ALL BY HERSELF!! I couldn't believe it, she did it, without any help from Jo or me! This is a huge accomplishment in the journey of JDM!!! Take that you stupid disease!!!

8/12/11 Update

It seems that life is running at about 150 mph these days. No time to even pick my nose! School starts on Tuesday so we are busy trying to get the girls situated and ready for that. My parents are taking them to El Paso today for a day of shopping and lunch and then I will take them on Sunday for the same thing. Spoiled trolls!! :) It was a busy week and a half for me. I traveled up to northern NM for my job and got to see some amazing areas of our great state. I worked in Farmington on Thursday and actually loved the area, then went to Gallup and worked there on Friday. I had to then work in Las Cruces on Tuesday and then work in Los Lunas on Wednesday! I actually enjoy the traveling. I am so lucky to have a great job, wonderful boss and co-workers. It makes it so much easier.
Lola had her 6 month eye exam with her opthamologist yesterday. Her peepers are well and no serious problems can be detected. She still has that small cataract on her right eye, but it hasn't grown or deterred her vision so that is a plus. She has to see her opthamologist on a routine basis because of the 2 medications that she has taken for 10+ years and all the side effects that they can have on the eyes. The Prednisone and Plaquenil are monsters to this area. So she will be back to see Dr. Yang in February and we will discuss contact lenses at that time. I cannot believe that she is gonna be in contacts soon! She is growing up too fast.
Jo should be getting her car out of the shop today. Yikes, hide your kids, grannies and families and get them off the road! She will have wheels and is not afraid to use them! Hopefully JR will have her car done today and she can be on the road soon. So off to DMV I go today for her registration and all that jazz. Thank you Nathan for all you have done for her by getting the car and all the parts and working on it to get it ready for her. You are amazing and I cant put into words how thankful we are for all you do!
So we are 35 days out from the wedding! So many little things to get done. Today I will go drop off his suit and tie at the dry cleaners so it can be pressed and made fancy and Monday I will order the flowers and this weekend we will find Jo her shoes. Yup, still no stress or anxiety and not quit sure if I will really have any, this is just too easy!! :)

Pre-Application for Marriage License

We did it this morning.... we submitted the pre-application for our marriage license in Clark County!!! We are about 56 days away! We cant wait to celebrate this momentous day with the girls and our family and close friends. We have family already making airline and hotel reservations and we are finishing up some last minute details and decisions and its been such a fun process for us both. So far we have kept our sanity and just had a good time working on projects for this day. I just talked to my good friend and photographer, David Thompson, who will be our amazing photographer for the day. His work is totally amazing and I am so thankful for him and I cant wait to see him and catch up. I know he will catch some beautiful images of the first day of the rest of our lives. Thank God for PhotoShop which he will use to make me look tan, tall and skinny!! LOL. I cant wait to see the girls in their beautiful black dresses, all dressed up and even Lola in her surprise "kicks", since she doesnt do dressy shoes. Still have many things to finish and check off "the list" but we are well on our way to our goal.

Lab Results

Allorahs last appointment with Dr. Pachman in Chicago was about a month ago and I finally received some lab results on Wednesday morning and then Dr. Pachman called me herself last night. It seems that she is impressed with Lola's recent labs. Her muscle enzymes are doing well and so is her liver enzymes, which had been on the high side for some time. This last time we were in Chicago, they started to decrease her Prednisone which was necessary to help lower her blood sugar and get her diabetes in control. It has been working and her glucose has been better and we are finally down to 7mg. a day. The Prednisone is a double edge sword, it helps control the inflammation that has wrecked havoc on her little body but at the same time there is so many side effects that she has experienced from being on this medication for 10 straight years, osteopenia, cataracts, diabetes, fatty liver syndrome. It has been tough but hopefully now some of those issues will start to repair themselves with her being on a lower dose and continuing to taper. Lets keep our fingers crossed. I informed Dr. P of this recent issue we have had with swelling and irritated lips. They look like they are just really chapped and they are starting to really concern me. She is starting to get real self conscious of the appearance. We thought maybe she was allregic to something she ate or to a lip balm that she used or lotion or something, but we havent changed anything like lotions or soaps or detergents and it still stays, we even tried giving benadryl with no change. So Dr. Pachman suggested to call her pediatrician or dematologist and see what may be going on as this doesnt sound like a JDM issue. Hopefully this is just a simple problem that can be easily remedied.

Road Trip

Me and the kids will be heading to Santa Fe this evening and meeting Nathan and his parents for the NMSP promotion ceremony tomorrow morning. It is also Jordyn's 17th birthday, so text her, call her or post "happy slappy birfday" on her facebook wall. Her gift is en route and wont be delivered until tomorrow evening, so that stinks, I was hoping to have it today so that we could give to her tomorrow when we take her to lunch at Rio Chama Steakhouse. :) Oh well, we will be back tomorrow night so it will be here when we get back home. I hope she likes it.
So it will be pizza, wings and poolside when we get into Santa Fe tonight. We found a pizza joint in Santa Fe that does gluten-free pizza for Lola and she is just beaming about this. The Rooftop Pizza does GF crust so this is a plus. I can almost taste the Dions and chelada. C'mon 5 o'clock!!!

Sitting Alone

Sitting alone,
Mind racing,
Thoughts a blur,
Seeing the despair,
Watching the questions,
Hearing the fear...

A look around, need only be brief...

The waiting room in any hospital is filled with all these deep emotions, yet a waiting room in a children's hospital seems to magnify them
all around by an exponential value not yet describable.   The parents, friends, loved ones and relatives wait feverishly for any word providing an indication of how things are going.  If only all could share in the strength and hope written on one of these kids faces and seated deep in their hearts, our days would be much simpler and full of appreciation!  Please keep Lola and our family in your thoughts and prayers.

This was written by my amazing fiancee. His ability to convey his feelings and emotions into words truly amazes me.

Relaxation, What Is That?

No one can seem to explain to me what relaxation is. These days its 200 MPH with no end in sight. I cant seem to not ever have anything to do. Its crazy how fast the year has gone and its now almost half over! Today I glanced at my wedding countdown app on my iPhone and gasped when I saw that we are 99 days away!

Holy schnikes, where did the time go? I will be Mrs. Nathan Barton in 99 days! Oh my goodness, I am so excited! My girls dresses have been bought and they look so amazing in them. Jordyn loves hers and she tried it on and I couldnt believe she is only almost 17 years old! Yikes, I'm in big trouble! And Allorah, well, she is no longer my baby girl, she looks so grown up. Now if I can just convince her that converse sneakers are not my choice of shoe, then we will be fine! :) I just wish that Diesel Bentley could make the nuptials. I just want to see him in a tuxedo! He would be our ring bearer but with our luck, he would eat a fairly large amount of jewelry and we would have to be scouring his turds! So, maybe leaving him at home is a more suitable option! We have decided that we are writing our own vows and we are actually already almost done with that. Can you believe how we are just so organized and on top of things? I know I cant! Here is our wedding website if you wanna take a sneak peek on what we are planning: http://www.mywedding.com/shannonandnathan/
Its cheesy and corny but so are we.

Home Sweet Home

We made it home safe and sound and we are beat! My poor Nathan did the entire drive since we all suffer from road trip narcolepsy. :) At least I can say I have the worlds most handsome chauffeur!

We are home for 12 days then we take off to Chicago on the 16th for Allorahs surgery to remove calcinosis on her right hip. Then after that maybe she can finally enjoy her summer. Thank you mom and dad for traveling with us and being there for us during this trip. The kids had a great time minus all the hospital business but it was a nice time with you. Thank you to all our family and friends for all the prayers. Love you all!


- Posted using BlogPress from my iPad

Schools Out!

I cannot believe that another school year has come to a close. I offically have a senior in high school and a 7th grader! Wow! I can say that I officially feel old! The wrinkles and grey hair that I sport are evidence that its true! It will be a very busy summer for my family. We will be leaving for Dallas on Monday morning for a week, then heading to Chicago on June 16th for Allorahs surgery the following day and then her appointment with Dr. Pachman on the the 20th. Then Jo got a car and we will be bringing that home and working on that for her, preparing the house so I can rent it out, packing and cleaning and then moving up north and getting situated up there, then finishing up the summer with a trip to Vegas in September to tie the knot! It will be a hectic one for us thats for sure. Hardly enough time to relax.

Waiting...

As I sit here waiting for our appointment with Dr. Kaufman, the endocrinologist in Albuquerque tomorrow, I am reminded of how much anxiety I have building up. I am also waiting to hear back from Claudia, the nurse for Dr. Garg in Dallas, to see what date we need to be there. It's all a waiting game, waiting to see what the outcome is, waiting to see how this will impact my Lola, waiting to see what new meds they want to put her on, waiting to see how the meds will make her feel and how and if they will affect the disease process, all a waiting game. She's a nervous mess, I'm a nervous mess, my oldest daughter, Jordyn, my parents, Nathan, my family, we are all stricken with this anxiety of what they will tell us, how serious it is. I know that I am a pessimist, but I prefer to think I am a realist, but at this moment in time, I wish I was more the optimist, maybe it will hurt less... I doubt it. :(


So last night Lola wasn't feeling good at all. She said her stomach was hurting and she felt like she did when she was getting the stomach bug. I had her give me a urine sample to see if there was any glucose or ketones spilling out, but thank God, both were negative. Not a sign in her urine, that's a plus! I was so relieved and I know Allorah was too. She keeps asking me questions about the diabetes and insulin and I don't have answers for her, I wish I did. We will get our answers tomorrow. It will be a long drive to Albuquerque but I am keeping my fingers crossed. Dr. Kaufman is an excellent doctor and is so sympathetic and caring. She will know whats best for my baby.

Why?

So this is the latest update on Allorah. I received a call on Monday from Chicago about her lab results from last month. They said we needed to follow up with her endocrinologist in Albuquerque for elevated triglycerides, elevated glucose, insulin and hemoglobin A1C. they were going to send the lab results to Dr. Kaufman and that Dr. Pachman was going to send her a letter as well. So I called and scheduled an appt to see Dr. Kaufman next week on the 17th. The following morning I get a call from Dr. Kaufman, her endocrinologist, telling me that she received the labs and letter from Dr. Pachman, she immediately called her to discuss what she saw. She told me that she now knows why Allorah's triglycerides have been high and it is making sense. She informed me that she has now acquired generalized lipodystrophy AKA Lawrence Syndrome and its very serious and extremely rare. She took the liberty of contacting Dr. Garg at UT Southwestern in Dallas and we need to take Allorah there. He is the leading physician that sees lipodystrophy cases and devotes his practice to this. I have attached a website explaining this diagnosis.


http://www8.utsouthwestern.edu/utsw/cda/dept105805/files/106988.html

She informed me that it is very rare and she knows about this disease enough to know that she needs to see the best. So I told her to please get it set up and we will go. Dr. Garg called me later that day and we talked and he informed me that he wants to get her in. His nurse called me yesterday and told me that we will need to be there for a minimum of 4 days. So off to Dallas we will be headed. We are looking at the first part of June and she is now working on getting her a room for there in the research unit and getting all her tests, scans and appointments scheduled. She is looking at insulin injections, as well as possibly being a candidate for a treatment protocol of a medication that Dr. Garg has initiated and only he can administer. So we will head to Albuquerque on Tuesday to see Dr. Kaufman and to meet with the diabetes educator and see what treatment she wants to start there. So that is where we are at now. We are praying that this hurdle is only a speed bump in her recovery and that once this gets controlled that we can get a better grip on the JDM. Please send her your prayers and I will keep you all informed.

Anxiety Stricken

So in the maddness that is Juvenile Dermatomyositis, a step back has been taken today. Allorah's doctor from Chicago called with some recent lab results from when she had her last appointment there on April 4th. Dr. P is wanting her to see the endocrinologist, Dr. Kaufman, in Albuquerque. It seems the long term dose of Prednisone that she has been on for almost 10 years has caused her to develop diabetes. Her insulin, glucose, Hgb A1C and triglycerides are ALL elevated!!! Why cant my baby get a break? Why does this keep happening? It seems that as soon as we think she is doing better, she is hit with a blow that knocks her down. I HATE JUVENILE DERMATOMYOSITIS, CURSE YOU, FOUL DISEASE!! So we are heading up to Albuquerque on the 17th for a consult as to what course we need to take. I am hoping that we can do this with maybe diet and exercise. It just really stinks because she is already on such a strict diet because of the celiac disease, a gluten free diet can be difficult for an adult let alone a child. So now she has even more restrictions! UGH!! She is such a trooper.

The Waiting Game

So, I have been bad about blogging lately, as this has been a very busy and hectic beginning of the year for us. I just had to take Allorah to Chicago on the 10th of January for her appointment to see her amazing rheumatologist, Dr. Lauren Pachman and her just as wonderful staff. It was a very busy appointment day and these times take so much out of Allorah. Her day started out by being at Childrens Memorial Hospital by 9:15 for her appointment with Dr. P and staff. For the most part, her appointment went ok. Dr. P wants her to have surgery by the plastic surgeon to remove a 5x7 cm calcinosis on her left back hip area. She is wanting to add Cyclosporine to her regimen of medications since the calcinosis is forming again and this is the top treatment for that and I am not happy about that one bit. Its a very strong med and I just wish we didnt have to go this route. She had her regular visit with PT and OT and she has lost a bit of range of motion to her hamstrings. Its so hard to get her to do her stretches and she is so passive about it. I cant get her to take this serious and its frustrating at times. She just wants to be a "normal" kid. So we are sent to the lab for her bloodwork and by this time Allorah is not feeling well. She has a bad headache and her body aches. She get 20 vials of blood drawn and I am trying to hurry over to catch the shuttle to take us to the Clark St. building her her x-rays and DEXA scan, so I rush her out of the lab, we are about 10 steps out of the lab and she stops me and tells me that she doesnt feel well, just then her knees buckle and she just about passes out on me! I scoop her up and carry her back to the lab and sit her down and ask for some juice. It was too much blood drawn and she is weak. So we wait until she downs the juice and she feels better and there we take off for the rest of her tests. An x-ray of her right wrists to try and rule out arthritis and x-rays of her right hip to see exactly how big the calcinosis is there, then its off for a DEXA scan to see the bone density in her little body. By this time, she feels like total crap and we still have to go back to the Ronald McDonald house and clean out room, pick up our luggage, catch our cab and head back to the airport to catch our flight!! Needless to say, it was a mad dash and she passed out on the plane ride home!! So now is the waiting game of waiting to hear how her results are. I hate this part, its brutal. I will keep you posted!

2011, New Beginnings

Its a brand new year and a brand new time for me and my family. The old blog has been brought down and a new one arrives. As we move forward into the future, all I can say is "WOW"! So much has changed in my life in this short time that I am taken back at times at how truly blessed I am. The girls are doing so well in school and Allorah is still fighting her battle with Juvenile Dermatomyositis. Jo has a job now and is driving. Diesel is getting so big and is just a mess! As for me, this princess has found her Prince Charming! We have something that only fairlytales are made of and I will be marrying my best friend on September 17th. The kids are so excited and I can hardly contain myself! So, as you see, happiness and a wealth of love is at my fingertips.