sorry for the delay in updates....

Hannah has bee officially dx'd with Autoimmune Enteropathy, with IPEX syndrome not being ruled out. She is doing well and has kept herself out of the hospital for a wonderful 3 weeks now. This is a record!!
 with this news comes much transition for me... mentally. We have a name for our enemy now, but he is so rare. I am still doing alot of studying, but from what it sounds like I think I need to really push this bone marrow transplant... If anybody can help me get my hands on this book please contact me!!  Volume 1, 4th edition, pediactric Gastrointestinal Desease. I swear to God it is the book her Doc in Cincinnati are reading! The way they are treating her is qouted in here.

But anyway, I will try to snap out of my mental quick sand and get back to work, I promise! :)


I search a quiet place,

For reverent solitude,

One where I can fix,

My strained attitude.



I’m not in the mood,

To think or speak,

I feel kind of tired,

I feel kind of weak.



My brain won’t absorb,

The things you have to say,

I need a quiet place,

So I can find my way.



Back to this place,

Where I can hear your tune,

I’ll take a short detour,

But I’ll be back soon.

is it November already??

Oh my how the time flies by! October was'nt much better than September. 2 weeks of  all us being sick, from an intestinal flu, to strep throat, to H1N1, it has all hit my house.
Hannah spent 4 days in Mary Lanning hosp. after the intestinal flu hit her. while she was there, my 10 year old Ash, had strep throat. Then a couple days later, 3 hours after they released Hannah. I was in the ER with Ash again, who spiked a 103 degree temp... H1N1. That was wednesday. Thursday it hit Anthony and we were all started on Tamiflu. Friday it hit Brittany. but thankfully not as hard. Today is Sunday and Hannah is running a low grade temp of 99.3, nothing to call the dr. for yet, but just enough to make you hold your breath and pray!
 The Dr.s are holding the tachrolimus right now. which is good considering it depletes her already fragile immune system. her left side is still causing alot of pain. They are giving her hydrocodone for pain.. and she is actually asking for it today. I realize how much pain she is in when I see that the medicine they are giving her is the exact same thing I was on after my knee and 2 shoulder surgeries, even same dose. and it barely subsides her pain.
 We have missed everybody this week! Due to our quarintined situation we have not been out and about. Thanks to everyone who made the extra trips across town to help our family! I really don't know what I would do without all of your support! It means so much to us all.

What is Autoimmune Enteropathy?

The diagnosis they have been tagging Hannah's lab's is autoimmune enteropathy. What is that? here is an article I found...

Advances in the understanding and management of autoimmune enteropathy



Summary

There have been real advances in understanding the pathogenesis of autoimmune enteropathy, including determination of specific autoantigens. The most important clinical association is with IPEX (X-linked immune polyendocrinopathy) syndrome, which is due to mutation in the Foxp3 transcription factor, a molecule critical in generation of regulatory T cells. Association of non-IPEX autoimmune enteropathy with T cell activation defects further point to impairment of T cell tolerance mechanisms as the primary underlying cause of autoimmune enteropathy. This also explains the frequency of other autoimmune manifestations. The centrality of T cell responses in autoimmune enteropathy, rather than B cell autoantibody production, as previously thought, is further suggested by the finding of late-onset gut autoimmunity in APS-1 (autoimmune polyglandular syndrome-1), a condition where negative selection of T cells within the thymus is disrupted due to mutation in the Aire (autoimmune regulator) gene. However, this form of autoimmune enteropathy is milder because the immune target is within entero-endocrine cells rather than absorptive enterocytes. There have also been important changes in management, with introduction of more potent immunoregulatory therapy, and more recently the use of bone marrow transplantation, which may theoretically offer hope of a cure in what frequently used to be a fatal condition.



Keywords: Autoimmune enteropathy, IPEX syndrome, Foxp3, APS-1, Aire, Bone marrow transplantation

Warwick Medical School, Clinical Sciences Research Institute, Clifford Bridge Road, Coventry CV2 2DX, UK



PII: S0957-5839(06)00085-6



doi:10.1016/j.cupe.2006.07.008



© 2006 Elsevier Ltd. All rights reserved.

The Climb

Cincinnati has a treatment plan...

After Spending almost the whole month of september in the hospital, I am overwhelmed with the news that Cincinnati has finally given word of a plan! a little nervous and nauseated, but relieved at the same time.
Monday Hannah will start taking Tacrolimus a drug that comes with some serious side effects, and strict precautions. Alot like getting chemo. Hannah's health is so fragile right now... I think now more than ever I will need all the help and support I can get. This is going to make her very sick.
Why are they starting this med? Tacrolimus is an anti rejection drug given to people who have had an organ transplant. Basically they think Hannah's body is rejecting her own organs.
Omaha is cordinating all her care through Cincinnati's guidance and then we will go back to Cincinnati in 6 months.
I agree with this plan, I think it is a long time due to have a plan! I pray this plan does something good for Hannah. But I am also very intimidated. And it takes alot to do that to me! But it time to buck up and take this head on!
WE WILL NOT GO DOWN WITHOUT A FIGHT!!

Hannah's Song

http://www.songsoflove.org/

and these are the days of our lives...

Sometimes my life is so crazy I can't even believe it myself!! It take me back to a therapist I had who said... "Beckie, I think you create your own crisis". Well, all I can say to that is... okay nevermind, I won't say it!

Hannah and I got to Omaha right at 10 am. Got her checked in and prepared for her procedure, the most dramatic part of that was taking her earings out... sorry you were late for lunch, Melissa! During her procedure they came out to tell me that the liver biopsy would not be done at this time due to a (what I thought was maybe a bug bite) on her upper outer thigh. My initial thought was , "you gotta be kidding" but the nurse explained that If it was something infectious it could be in her blood, and they would risk infecting her liver. So they biopsied that! One thing I am learning, When you remain undiagnosed after 7 years of extensive searching, they biopsy everything... even bug bites! and lets not forget the raised eyebrows along with the questions "did you let her outside??" aaaahhh HHEELLOOO!!!!! She is 8! yes, she went out and sat on the porch maybe 2 or 3 times this week. So Sorry for letting her live a little!
I was had a conversation with a dear nurse about the fact on letting Hannah live a little.... It has been such a fine line, and as her Mom, a very horrible decision I have been left to make, and I strongly believe that I am doing what is best for her.
I do my best at keeping her out of populated places, and when we are out she wears her mask and knows her rules of no touching. she has a suitcase of pumps and fluids, so that limits her activity as well, no playground, no slides, no swings... no pools no sprinklers... need I go on??
So here is the Golden Question... do we let her live a happy full 10 years, or do we lock her in this bubble and make her depressed and bitter at life for 30??

well anyway, after her scopes and biopsies of her intestines, she was released shortly after, BTW... Melissa, she was terribly disappointed she had to come home and miss putting new babies out in the playroom!! She was still pretty loopy when she was released and slept all 3 hours on the way home. She has been good, but yet low key since.
G.I. Dr. called from Omaha this morning, seems we have been talking directly about 3x a week latley. He said her potassium was low, we had this problem in June was not happy to hear that 1 again, low potassium cause heart issues. so up we go on the 24 hour TPN concentration which is causing her liver levels to be rising which is why they wanted a liver biopsy... and I think I am getting dizzy!
I can't thank you enough for all the support and prayers, it is what keeps me going... recently heard of someone having to take a vacation to send off over 300 Thankyous due to the recent death of a family member. I'm thinking I have about 3 years or more of thankyou's to send out... vacation sounds good!!

5 am and can't sleep... good time to update :)

woke up this morning feeling sick, came downstairs cause I didn't want to wake anybody up with my puking.... come out of the bathroom and there stands Hannah... her tummy hurts too. she dragged her ladybug bag of pumps down the stairs... sorry neighbors.... oh wait, my stupid neighbors kept me awake till 2 am hanging out under my bedroom window AKA our front porch... so sorry? hhmmmm, not so much!

OK... venting over, let me go get my coffee and glasses and we will get on with this updating thing! :)

Okie Dokie, well, my Bronchitis got better, slowly.

School began, routines been adapted too.

Hannah has been to ER in Omaha twice this month.
1st time because her J-button cracked, overnight,but no surgery, but an awake procedure... they gave her pain medicine and verset ... which made her stutter for 3 hours after the procedure... kinda creepy!
2nd time because there was a pinhole in her central line. no surgery. The surgeon RESIDENT that was repairing her line asked her if she stuck a pin through it... she looked at him like he was nuts and asked "why would I do that?" Hannah is very aware of the fact that when her line is down she spends the night on IV fluids with potassium and dextrose (which burns like crazy going in).
If Hannah is not talking much people have the 1st impression that she is 3 or 4 years old. She is sooo 8, and very intelligent. She is also very sarcastic... hehehe, I have no idea where she gets that!
She still has 2 pumps running 24 hours a day... which explains my lack of sleep, if it isn't 1 beeping it's another, most usually because she rolled over and pinched her tubing. oh and then there is a third... but that is only 1x a week for about and hour. That is her new Vivaglobin. the first and only sub Q infusion. and I believe that she is the 1st around here to receive it.
Monday she is having surgery. Upper and lower scopes and biopsy's, the are also going to do a liver biopsy because her levels are still running high. this biopsy comes with more complications then the scopes so they will be keeping her at least overnight.
Britt's root canals had to be reschedule due to Hannah's surgery, so the dentist has her on call for that... crazy!! I need 4 of me, 1 for each kid!
Ash and Anthony are doing good, they love school so life is good right now! Hannah has a teacher coming out 2x a week. really looking forward to that, she has an amazing mind, it's fun to see it in action!
Well it is after 6 already... got to go start getting things prepared for Hannah, she is going to be running pedialyte through her pump today in preparation for surg, tomorrow.

Love,Blessings & Barefeet, Beckie

Wash your Hands!!

With School starting this week, this is a very big concern of mine for Hannah, my lil bubble girl...did I mention that we live in Adams Co. !?

2nd H1N1 Flu Fatality in State

A spokeswoman with the Nebraska Department of Health and Human Services says the second state resident to die from the swine flu passed away at an Adams County hospital. The Adams County woman in her 50s died late last week. The woman also had several chronic, underlying health conditions, a press release states, including asthma, heart disease and diabetes. Because of state statutes, the woman’s name and other details about her case cannot be released. The state's chief medical officer, Dr. Joann Schaefer, said the virus, also known as the H1N1 flu, can be a very serious illness for those who have chronic health conditions. Federal officials have said the flu strain could kill possibly 30,000 to 90,000 people later this year, mostly young children and young adults. Seasonal flu typically causes 30,000-40,000 annual deaths, mainly among people over 65. Health officials advise only those who are moderately or severely ill or someone with underlying medical conditions see a doctor. Most people who get the virus can recover on their own, they said.

August 26, 2009

Thanks for the prayers everybody, yesterday was a whirlwind! I have a bad case of bronchitis and am struggling to breathe, had extensive conversations directly with Dr. Zapatta, 3 times yesterday, Hannah's liver is failing due to the lack of use, so they r starting her feeding pump back up, slowing weaning her on to that. And still running the TPN . 2 pumps running 24 hours a day. Plus trying to get Ashley and Anthony ready for their 1st day of school today. and to top it off, Brittany missed her 1st day due to a trip to the Dentist who informed me she needs 2 root canals, how do her teeth get that bad so fast? just was there for a cleaning in March!! Not sure if she will be able to go to school tomorrow, this morning her lip was at least 4 times the normal size, poor thing. I have never had a root canal, heck, I have never even had a cavity, so I do not know personally what she is going through, but it sure doesn't look fun!
I have not heard back from Cincinnati yet, so I am making a call, it's been 2 weeks today. (not that I'm counting) Hopefully I can get myself to the Dr. today, but I don't see it being able to fit in my schedule unfortunately. I just need a good ole shot in the butt of antibiotics!! Anyways... off to survive another day!
Love, Blessings, & Barefeet, Beckie

Hope For Hannah Benefit

This Was absolutley awesome!! Thankyou to everyone!

Khas News story

NTV News story

Have you figured me out yet??

okay okay, I'm here to update, as most of you know, I am not good at commincation, unless I can sing how I feel through a song! lol. But here we go....

Went to Cincinnati last week, Lots and Lots of questions!! 7 years worth of history to share so I am sure it is all overwhelming for them. Plus they drew a bunch of blood, then they said that they would be in touch and probably have us come back for some scopes and stuff.
My overall feeling is that they do not have the same sense of urgency that is needed for her. Ever since she has started this 24 hour TPN thing things have been crappy. There is not a day that goes by that she has not had to deal with headaches or abdominal pain, Her blood sugar has been low, her liver and kidney levals have been high, all common side effects of the TPN. She has gone over that hill of more bad days then good, and I am struggeling with my redheaded patience to get somebody to care enough to DO SOMETHING NOW!!
I just keep replaying in my head what the hospitalist in Omaha told me... " Go home and have a normal summer " None of my children have had a NORMAL summer because of this, we all have stayed home watching her get sicker and sicker. I'm constantly watching one of her sisters carry her up the stairs while her brother carries her pump, because she is to dizzy and weak. her burst of energy do not last more than a couple of hours, and they take everything out of her.

I am just tired of having someone so tiny yet strong have to fight so hard to live.

Under Construction

This site is currently under reconstruction. Please check back soon for updates! :)

Updates on Hannah

She has had a very rough couple of months. She now depends on a central line, (a tube that runs in her underarm up through her a vein in her neck and straight to her heart) for all her nutrition. which comes with alot of complications. Complications that apparently our local hospital wont touch and I have to rush her to ER in Omaha... 3 hours away.
Sunday morning on the 9th, Hannah, my BFF Sharon and I will be loading up in my lil Mini van and driving to Cincinnati Children's Hospital to see some specailists that hopefully will bring us some answers to what is the problem and how we fix it, or at least treat it.
Hannah remains strong and full of life. She knows no other way of living. she has lived with this for over 7 years.If it wasnt for her ability to laugh at this crazy life and make the best of it, I dont think I could. She is the one that reminds me everyday to keep fighting. To be strong in my faith, and live with the grace and dignity that has comes with living this life.
Please keep us in your prayers and we battle the road ahead. and the other drivers on it!! lol

Good news from Cincinnati Children's Hospital

We have an itinerary for Cinncinati! We will be leaving August 9th, she has 2 days full of appts. on the 11th and 12th. She is being seen by G.I., Immunology, Oncology, and Hematology. If all goes ok We should be home by the next weekend! Praise the Lord and thank you for all the prayers!

Thanks to Raleigh Kelsey and Dave Worrell for thinking of Hannah and our family for their annual Benefit! There is more to come, I will post info as I receive it.

so far this is what I know...

this is taken from a referral letter from the Doctors in Omaha requesting to be seen in Cincinnati....

Hannah Copley has an as yet undefined condition characterized by elements of immunodeficiency and enteropathy with the result failure to thrive. She has been followed closely and evaluated extensively at Children's Hospital and Medical Center since October 6, 2003.
Hannah's evaluation has included input from multiple specialists. She has seen no less than five pediatric gastroenterologists, two pediatric surgeons, five pediatric hospitalists, two endocrinologists,two behavioral health practitioners, one neurosurgeon, two rheumatolologists, and one immunologist. She has been hospitalized at Children's Hospital, Omaha, five times for a total of 56 days and counting.In addition, she has been seen at Denver Children's Hospital and has been admitted to many times to count at her local hospital.
She is currently being evaluated and managed for common variable immunodeficiency. She has been noted to have poor T-cell responsiveness and a very low mannose binding protein level.
Evaluation at Cincinnati Children's Hospital has been recommended as they are the premier site in the nation for the evaluation of pediatric gastrointestinal disorders.


We are waiting for all the authorizations for Nebraska Medicaid to prove that we have exhausted all other options and have to go out of state. When we went to Denver we went through this process but it was more difficult, but she was not considered "disabled" at that time. I'm guesstimating we are looking w/in the next week or two. But in reality we are on call.
I am not sure yet what I need, I am in the process of paying my bills and budgeting now (or I was b4 I started this!... focus Beckie!!) I received a $250 check from "12 Omaha women who care" no return address or anything (God Bless you all) which I am again guesstimating is at least half of what the trip will cost.
It is at least a 12 hour drive, so I will have to take it in 2 days, Hannah won't be able to do more than 6. I'm having Joe check my fluids and air pressure, stuff like that, tomorrow.
The kids Ashley and Anthony start summer school tomorrow and Brittany starts detasseling any day now. Which will be good for them, and me. Hannah's care has stepped up alot more, though I have no beeping alarm going off every 5 hours, I still find I can't sleep. Just to much to do, and now that I have a baby monitor I can get them done. She was up late with a headache last night, and the night b4 we had lab come back with low blood sugar. I am up to 3 cups of coffee a day... this is not good!

Another Adapt & Overcome moment from the Copley's

Hannah's backpack for her infusion pump is huge!! Her bag of TPN is over 2 liters and is light sensitive. it run 24 hours. no breaks. I went to Wallyworld... I mean Walmart... last night and found this lil rolling suitcase. Much Better!! :)

Univerity of Texas sings Hannah Happy Birthday

Visitors from Omaha Childrens Hospital

Steve Peoples, a race car diver with Holiday Horsepower Drive



What a great Guy!!



Extreme Makeover... lol






The University of Texas and Louisiana State University keep the College World Series excitement going at Children's!





Playing Jenga with LSU



They were singing her Happy Birthday!





Several Husker greats stopped by Children's Thursday. She enjoyed meeting Danny Noonan, Tom Ruud, Barrett Ruud, Bo Ruud, Johnny Rodgers and Jim McFarland. Barrett and Bo still play in the NFL!

Thanks for coming, guys. We had a great time!

June 30, 2009

well I just got out of a meeting with the docs. it looks like they r going to send us home for a "few days" b4 we go to Cincinnatti, so now I have to figure out how the hell we r going to get there. as if being here for two weeks isnt draining my account enough. obviously these people have no idea what it is like to be a single mom of 4. Im so sick of this constant battle... physically mentally emotionally....
3 hours ago ·

2 day training starts tomorrow for me to learn how to care for her central line and feedings at home. probably going home Thursday. Dr. Kocoshis in Cincinnati has been contacted and is receiving all her files. He is the Medical Director of the Pediatric Nutrition & Intestinal Care Center, and Small Bowel Transplantation Program.
8 minutes ago ·

Took these pics this morning, 1st time outside in 2 weeks!

Updated pics