Monday, February 23, 2015

Our Sweet Girl is Sick

Our Sweet Girl is Sick
Well, I'm pretty sure I jinxed us when I wrote in the blog on Saturday saying that we were thankful that Elizabeth's continuous upper respiratory infection had not yet developed into pneumonia, because Elizabeth now has pneumonia. :-(  It was confirmed yesterday with a chest x-ray.  We started her on another dose of antibiotics and hoping that she can fight it off on her own so that we can avoid the hospital. So, she could use your prayers.  We will be following up with the doctor later this week, and if she gets worse or isn't much better in a few days then we will have to come up with another plan of action.

Celtan stayed with Aunt Holly yesterday while we took Elizabeth to the doctor to get checked out.  He loves spending time with his Aunt Holly.  While they were together, they built a snowman.  He was so proud, so I thought that I would share the picture with all of you.  For only being 4 years old, he is a super good sport about everything that goes on with Elizabeth.  He truly is an awesome big brother.



Saturday, February 21, 2015

Benefit Updates

Benefit Updates:

As I posted a few weeks ago, my cousins and sisters are hosting a benefit for Elizabeth.  Everyone in our family are completely overwhelmed with the amount of support that has been shown for the benefit from people offering to donate, help that day, who are planning to attend, etc. Jason and I are not involved in planning the benefit, obviously, it was quite the surprise to us when the girls told us about it.  However, I do have a lot of people who have been contacting me asking how they can help.  So, I decided to post updates on the blog about the benefit and who to contact if you have questions or want to help.  

Recently, a friend of one of the girls offered to host a way to help out with the benefit through her photography business.  The contact information and details are on her flyer.  Thank you to Ashley Binus for offering to help out with the benefit.  We really appreciate it! 


A friend of Jason's cousin is offering to help Elizabeth  through her Premier Jewelry company.  We appreciate Terri offering to help, as well! Here is her flyer if you are interested in ordering. 

There is also a Go Fund Me site that has been set up by a colleague of Jason and I.  We are so grateful for Megan setting up the account.  It can be found at:  http://www.gofundme.com/kkrlog  

The girls in charge have set up a volunteer website to help with the benefit.  If you'd like to sign up to donate items for the benefit or to help on the day of the benefit, you can do so on this website.  If you would like help with signing up, you can contact one of the girls.  I'll post their contact info below.  The link to the volunteer website is:  http://www.volunteerspot.com/login/entry/9978464280103

The benefit will be held on Sunday, March 22nd from 1 p.m. to 7 p.m. at Holy Trinity Lutheran Church in Chicora.  There will be a spaghetti dinner with take-out available and all ages are welcome. Tickets will be $10 for adults and $5 for kids.  You can purchase tickets at the door. There will also be a Basket Raffle and Silent Auction Raffle Items.  Some bigger items that will be there will be a handmade rocking horse, Pens Tickets, and an IPad Mini.  There have been so many wonderful baskets and other items donated for the benefit.  Other details are on the benefit event flyer.  We will be there that day so that people can meet Elizabeth, and also so that we can thank people for their support. 
If you would like to contact one of the Team Elizabeth Jeanne Benefit Committee members, you can do so by contacting them via email.  Their email addresses are:
1.  Holly (Bly) Wilson: hollyjbly@gmail.com
2. Emily (Bly) Rekich:   emrekich516@yahoo.com 
3.  Kellie (Kepple) Johnston:  kckel134@aol.com
4. Katie (Kepple) Callihan:  KT.Callihan@aol.com
5. Ashley (Kepple) Ciafre:  ashleyciafre@gmail.com
6. Laura Kepple:   laurakepple@yahoo.com 
You can also let my parents (Jeff and Amy Bly) or any family member know if you're interested in helping, and they can fill you in on details.

We also received word that the Butler Lions Club is taking an active role in helping the girls with the benefit.  We truly appreciate their support of Elizabeth and the help that they have extended toward the benefit.  If you know a Butler Lions Member, please tell them thank you.  I cannot thank them enough for their support.

Seriously, thank you from the bottom of our hearts to everyone who reads this blog, who has offered to or already signed up to help with the benefit, and who support us in various other ways.  The prayers, donations, support are appreciated more than you can imagine.  We love you all!

Feeling the Love in February

Feeling the Love in February
Well, February has been cold, cold, cold here in PA.  Right now as I type this recent blog entry, it is snowing outside again. We are warm inside of our house, spending yet another day in our pajamas, and we have water now (thanks to my dad).  We couldn't ask for much more.  Truthfully, we are all a bit stir crazy.  School was only in session 3 days this week due to the extreme cold, but hopefully the weather will break soon. Until then, we are operating happily in the realm of super heroes, princesses, Hungry Hungry Hippos and the Teenage Mutant Ninja Turtles version of the game "Trouble."  I love to watch Celtan play, and lately Elizabeth has been really interested in watching him play, which is also simply incredible to see. 

We continue to be overwhelmed and filled with gratitude by the amazing amount of support that has been extended to us from our family, friends and community.  I will be adding another post today to discuss the upcoming benefit. I have had many people contact me and asking how they can help.  We still are humbled and very thankful for everything.  Our secret angels keep storming us with very generous gifts, inspirational notes and cards, and our prayer angels are keeping up the vigil sending prayers our way.  We appreciate it all more than you can imagine.  Thank you to our one secret angel for the continuing gifts of Wal Mart gift cards, which have been a tremendous help to us to get things for Elizabeth, groceries, etc.  Also, a big thank you to the lovely Valentine's themed card from another secret angel in disguise that provided us with kind words, a gift card to Wal Mart AND a gift card for us to have a date night.  We have yet to go out on our date night, but when we do, we will be sure to thank you again then.  The mystery of trying to figure out who these people are is actually a fun way for us to pass the time while we wait for answers and continue on the path.  I hope that one day I find out who all of you nameless angels are and can thank you properly, but part of me realizes that I may never know who you are and may have to continue to thank you regardless of knowing your identity. Celtan particularly enjoys the mail. Again, thank you to all of our supporters.  We truly and sincerely appreciate it.

Elizabeth has recently made some progress and has also been continuing to struggle with infections.  Since mid-January, she has had a recurrent respiratory infection, which has included an abundance of mucous, a cough, intermittent fevers, etc.  We have been using suction to clear out her snot, inhalers, cool mist, elevating her bed, and anything really to keep her comfortable.  We have also been keeping a close eye on her cough and have been to the Pediatrician's office several times in the past month to have her cough checked out. Thankfully, this upper respiratory thing has not spiraled into something worse, like pneumonia, but she still can't seem to fight it off. 

Last week, Elizabeth started spiking some higher-than-usual intermittent fevers. She was not her usual happy self, was refusing to eat, and seemed very lethargic.  So, I left school early one day to take her to the doctor.  They swabbed her for the Flu and for Strep Throat.  They also catheterized her and got a urine sample.  Thankfully, the Flu and Strep tests were negative.  However, the nurse called me the next day to tell me that Elizabeth's urine was again infected and showing growths of E.Coli. So, they started her on another antibiotic. The antibiotic seems to be helping her out.  However, this is her third UTI (Urinary Tract Infection) since November, and now we are being referred to Urology. Her team of doctors feel that a referral to Urology is warranted, because that is a high number of UTIs in a short amount of time.  They want to try to determine if there is something physically that is causing the frequent UTIs, like Bladder Reflux or some kind of malformation in her urinary tract.  So, we are scheduling an appointment in Urology.  

We had a nice, quiet Valentine's Day at home. Since Elizabeth was sick, and we had yet another snow storm that day, we stayed in.  We watched movies, played games and with toys.  Celtan kept giving Elizabeth hugs and trying to give her a kiss while telling her Happy Valentine's Day. At each attempt, Elizabeth would give her brother a dirty look, as if she was saying, "Leave me alone!"  It was rather entertaining, and we call that look, "The Princess Stink-Eye."  At least that is normal, she definitely lets you know when you're annoying her. Celtan really enjoys holidays.  He made up a song about love, and his family, and kept saying, "Happy Valentine's Day, I love you guys!" It was adorable, and I did get a video of it that I sent out to our family. 

On Monday, February 16th, we had several appointments scheduled at CHP and at the Children's Pine Center in Wexford.  I had scheduled the appointments on that day, because we were supposed to be off from school.  However, we were then scheduled to have school that day as a snow make-up day.  Then, that day we received a blessing that school was cancelled, which meant that I wouldn't be taking an unpaid day to take off for the appointments.  It also meant that Jason could bring Celtan home after his Dermatology appointment in the morning, and that I wouldn't have to manage Celtan and Elizabeth all day at CHP.  I knew that either my dear mom or my lovely Aunt Susie would be with me on that day to help, but it obviously is never ideal to have to entertain an energetic 4 year old boy at the hospital all day. Thankfully, we didn't have to.  Jason brought Celtan home with him after the Dermatology appointments, and Mom and I took Elizabeth to CHP for her remaining appointments. 

Our first appointments that day were at the Pine Center in Wexford at Children's Dermatology.  We saw Dr. Kress.  Elizabeth and Celtan both had appointments that day.  Celtan has been seeing Dermatology for his eczema since he was about a year old.  He is currently on a combination of allergy meds, anti-itch meds, steroid creams and moisturizers that are doing a remarkable job of keeping his eczema under control.  We had an appointment with Elizabeth that day about the ever present rash on her cheeks. She has had this rash on her cheeks since she was just a few months old.  It has continued to spread and grow, and it has never gotten better no matter what kind of cream or ointment we have used on it.  It also flares up whenever she is sick, and it even sometimes looks like it is blistered or ulcerated depending on the flare-up. Dr. Kress looked at her and said that what she had was called Keratosis Pilaris (KP), and it is a form of eczema.  It is known to be on your face, arms or butt, it runs in families, and it also is known to flare during illness.  There are creams to treat it, and so he gave us some samples.  So, now we know what the facial rash is on Elizabeth.  It is the only place that she has the rash, and hopefully we can find a cream that will treat it.  Elizabeth also has issues with sensitivity to sunlight.  When she is exposed to the sun, even a tiny amount, she develops red rings around her eyes that look like a severe sunburn.  Elizabeth's team of doctors wanted Dr. Kress to give his opinions on Elizabeth's skin and her sensitivity to sunlight. Dr. Kress agreed that her skin is very translucent and hyper-elastic, which means that her veins are very easy to see through her skin and that her skin is very stretchy. Dr. Kress also said that based on the appearance of her skin and other clinical symptoms that he would like to order a few blood/ urine tests to test Elizabeth for porphyrins, which are responsible for a series of disorders known to cause sun/ light sensitivity.  The issue is that we have to wait for the sun to come out again, and wait for her to have a reaction to sun/ light exposure, because the testing has to be done while she is having a reaction. So, Dr. Kress wanted to see her in two months to see how the treatment for her KP was coming along, and he will order the lab work at that time.  So, once we have the lab orders, and she has a reaction to sunlight, then we go straight to the lab and have them drawn or taken for the testing. We are going back to see him in April. 

We left Wexford and headed to our next appointment at CHP with the Developmental/ NICU Follow-up Clinic. Elizabeth has been seen by them every 6 months since she was born.  This division is provided partially through a grant, and it is intended to serve as a tool to monitor the development of NICU babies after they are discharged and sent home. It is very helpful, but this appointment is always very difficult for me.  Those of you who have been following since I started the blog may recall that it was at our NICU Developmental Eval appointment in August 2014 that recommended an appointment with the Cerebral Palsy Clinic for further evaluation.  At the eval, a team of Developmental Psychologists and a Pediatrician conduct an assessment on Elizabeth to assess her overall progress towards developmental milestones in the areas of cognitive development, receptive and expressive language, and gross and fine motor skills. While we cherish any accomplishment she makes, and we have come to terms with the fact that she accomplishes things on her own time, we do know that she is delayed. This is very clear to anyone, especially when she is around other kids her age and even younger than her.  Typical kids Elizabeth's age, and kids younger than Elizabeth, do a lot of things that she does not do, does not attempt to do, and that we aren't totally sure she will ever do.  That's ok, though, that's what makes her a miracle and makes her special. However, in these evaluations, as I watch her not be able to complete tasks over and over again, it is disheartening, and every single time this appointment ends with me being in tears.  The team always tries to comfort me, and then I feel totally lame and weak for crying in front of them. They always discuss the results with me, and this time Elizabeth's scores dropped in every category, except they remained the same in receptive language. The point is that although we see her making a lot of progress, and the clinic team even acknowledges they have seen amazing progress even since they saw her last in August, the test and skills required for her age keep growing while her skills in those areas are not necessarily growing at the same rate.  This is why her scores drop, remain the same and do not show growth, and it really boils down to the fact that she is severely developmentally delayed and is not developmentally close to her same age or adjusted age peers.  Deep down, we realize this.  I realize this.  It doesn't make it any easier to hear or cope with, but it also just makes us love our little miracle even more.  It makes us appreciate the hard work that she has to put in, the motivation she has, that enables her to accomplish any of her milestones.  She will be able to follow-up one more time in this clinic, and then her team will determine which clinic should continue to monitor her development.  NICU graduates can only be followed by this clinic until they are 2 years old, adjusted age, which is why her next appointment will be her last with this team.  The objective measurements of her development although heartbreaking and tear-jerking for me are actually a very helpful tool in monitoring her development.  Hopefully, in 6 months we see at least improvement on some scores, but if not we will keep plugging away and appreciating the achievements as they come.  

Our last appointment that day was with the amazing Dr. Amy Goldstein.  I affectionately call her, "The Brain," because she is our clinical brain, our neurologist, and she has been immensely helpful to us in a diagnostic sense.  Even though we don't have a diagnosis just yet, she has been a driving force in helping us to get there.  Dr. Goldstein is brilliant, compassionate, personable, and everything that you want as a doctor on your child's medical team.  I just love her.  Anyway, she checked her out.  We reviewed symptoms, recent appointments and developments. I told her about the benefit and all of the generosity that has made taking Elizabeth to Boston and UCSF possible. She is thrilled that we will be taking Elizabeth there this summer, especially to see Dr. Sherr at UCSF.  Then, we talked about the Microarray Genetic Testing, because the results were back.  Genetic Testing is a bit of a gamble, which is one reason why insurance companies are so reluctant to pay for it.  We have known all along that we would have to fight to get the insurance companies to pay for the test, and that even after the fight that these tests may not tell us anything. The reason is that the Microarry and Exome sequencing available only test for known variations that cause known genetic disorders.  The realm of genetics is constantly evolving, and geneticists are always finding new genes that do things that we never knew about before. This is one reason why we may not have answers any time soon. We have known this all along, and we are hoping that we get answers from this testing that we are fighting for with the insurance companies.  However, Elizabeth is a medically complex case, and although she has a variety of medical diagnoses, conditions, anomalies and many delays, the testing may not give us an answer or name a syndrome that will explain all of her symptoms and medical complexities.  It doesn't mean that she does not have a syndrome or a genetic condition, it just means that as of this time the doctors don't KNOW what it is and they don't have a NAME for it. There are thousands of families in this position.  There is clearly something wrong with their child, and the medical professionals all agree on that, just like they do with Elizabeth, however, at this time there is no answer for them, because medical research and technology and testing hasn't found or given a name to that answer yet.  So, families like that and like us are in a holding bay of sorts waiting for answers.  We are essentially waiting for medical knowledge and research to catch up with our mystery kids. It is no wonder why we call our kids who fit into these categories "special."  They are special indeed. So, the position that we are in now is one without answers, because the Microarray was negative.  We have known, and her team of doctor's have expected, that the Microarray was probably going to be negative and additional testing would have to be ordered for Elizabeth. Their predictions were correct, and now we are petitioning the insurance company to pay for a genetic test called "Exome Sequencing." This was actually always our plan, because even if the Microarray had come back with a positive result for some kind of genetic syndrome, the Genetics team wanted to order Exome Sequencing to learn more about the Microarray results.  Now that the Microarray was negative, we really need to have the exome sequencing done to have a chance for answers.  We also realize that even if we are granted the exome sequencing from the insurance company that we still may not have answers, because it is highly likely at this point that whatever syndrome Elizabeth has that it is either unknown at this time or it is very rare.  Dr. Goldstein has a really remarkable way of explaining things, and here is how she explained the difference between Microarray Testing and Exome Sequencing.  She uses an analogy about books.  Dr. Goldstein says that a Microarray is like taking a Harry Potter book off of the shelf and noticing that Chapter 7 is listed twice in the table of contents, or that there is not a Chapter 2 listed because it skips from Chapter 1 to Chapter 3.  It is a broad, quick glance at a person's chromosomal composition.  Are there too many of one chromosome or is one missing?  However, in some cases (like Elizabeth and other rare and complex kids), you look at the Table of Contents and the chapters are all listed in sequential order.  There are no missing or duplicated chapters.  However, when you go through the whole book, you may notice that pages are missing.  For example, as you are reading you realize that the book is missing pages 10 through 25 and later on is missing another 30 pages at the end of the book, despite the fact that the table of contents looked intact and correct. It is for these rare and complex kids that the Microarray is inadequate and often inaccurate, and it is also why for these kids exome sequencing is necessary.  So, Elizabeth's team of doctors at CHP has sent a request to the insurance company with medical documentation and reasoning as to why the Exome Sequencing is medically necessary. We now are waiting for their answer, and if they deny it, then we will appeal as we did before.  The test itself will take about 14 weeks to get results sent to us. Dr. Goldstein said that they wanted to know if I wanted a job there.  We won Elizabeth's Microarray on the first appeal, and according to the doctors this is a rare occurrence.  So, they asked me on my end to keep up whatever I did before to help with the Microarray appeal, and I fully intend to do that.  So, I will begin calling UPMC next week to check on the status of the Exome request. Hopefully, the exome sequencing brings us answers and a diagnosis.  However, if it does not, we will be like thousands of other families who will be waiting, waiting, waiting, and so we will wait along with them.  One day, we will know what this is, and what has caused all of these medical and developmental issues for our Elizabeth.  We wait in anticipation of that day. 


In other brighter and happier news, Elizabeth is continuing to progress with her crawling.  She has even been trying to pull herself up.  She is not steady enough to remain standing for long, and she often doesn't know how to get back down to a seated or crawling position safely, so we have to be close by to help her and make sure she doesn't get hurt.  It is really great to continue to see her make progress in this area. She also has been wanting to sit on the floor and play with toys lately, which is another development. 

Also, both Celtan and Elizabeth have been matched with runners/ athletes through IR4.  I Run 4 Michael  and I Run 4 Siblings the Unsung Heroes are organizations that assign athletes/ runners to special needs children and their siblings.  Their premise is to run for the people who can't, offer support to the siblings of special needs children who often get pushed to the side and are the unsung heroes, and to provide relationship building, inspiration and support to special needs children and families.  Their website is http://www.whoirun4.com/about-ir4/ if you want to read more about this wonderful organization. Celtan has been matched to Susan Osborn through the IR4 Siblings the Unsung Heroes Group, and Elizabeth has been matched to Robyn Oliver Cabaday through the IR4 Michael group. We have made contact through Facebook with both of these amazing women, and we are looking forward to building relationships with them.  I hope that you check out the IR4 website, because it is a really wonderful organization.  Celtan is very excited and had lots of questions for his match, Susan.  He truly is Elizabeth's unsung hero.  He loves and adores her.  He is always cheering her on, and truthfully he does get pushed aside sometimes due to the care and attention that she requires. We will keep you updated on our relationships with these two ladies. We graciously welcome them to our journey and to Team Elizabeth Jeanne. 

I'm also going to create an additional post with updates on the benefit. We have several appointment days in March, so I will update the blog after those appointment dates.  March will be a big month as a follow-up swallow study is scheduled, and the G-Tube verdict will likely be rendered at our GI appointment.  Thank you, as always, for reading and for being a part of our journey.  Elizabeth is stronger, we are stronger, and we are so grateful for all of you.  We love you all!!! Here are some pics of our Elizabeth!





Thursday, February 5, 2015

News and other updates


News and Other Updates
Hello!  I hope that you're all enjoying this bleak and cold winter weather.  I do not like the cold, but I do love the sight of the snow.  I just don't really care to be out in it.  Celtan has really been enjoying all of the snow, and he and Jason built a snow castle and a snowman in our back yard.  We just have a few updates that we wanted to share with our followers.  I cannot believe that the blog has reached over 5000 page views.  It is amazing to me that we have so many people checking and rechecking the blog for updates and to learn more about our journey with Elizabeth. We appreciate all of you!

Insurance Company:
On January 28th, Dr. Vigliotti's office (Elizabeth's Pediatrician) called to let me know that they had received word that UPMC Health Plan had granted our First-Level Grievance and had agreed to pay for the Microarray Genetic Testing.  We were thrilled, because this testing is going to be really important for us to get answers regarding Elizabeth and her many medical and special needs.  So, I spent the afternoon calling and letting her team of doctors know that the testing had been approved.  Her DNA has been waiting in the lab since November when the test was ordered, and now Neurology could tell the lab to go ahead and perform the Microarray testing.  Her neurologist, Dr. Safier, ordered the testing, and so the lab would need for his office to notify them to go ahead even though it is a genetic test.  However, I received a call later that day from Dr. Safier's nurse.  She informed me that she could not contact the lab without written verification from the insurance company. 
I had to call UPMC Health Plan to get some kind of written verification about the testing.  So, I called and after being put on hold for approximately 30 minutes on and off, I was finally transferred to someone who could help.  I was very upset, and less than kind, because I found out that they had reached a decision on the Microarray Testing on January 6th.  It was the 28th of January.  I had called them at least a dozen times to check the status of the grievance between the 6th and the 28th, and each time I was told that they had not reached a decision yet.  Furthermore, my anger at the insurance company increased, as did my blood pressure, when no one could explain to me why I had not received notification from them in writing of a decision that was made by them 22 days earlier.  The customer service person also agreed that she could not understand why I had not been notified.  I told her that I needed verification today, and that she had five minutes to have a copy of the letter sent to my email or faxed to me, and that I would be on the phone with her until I received it.  I also said that I wanted a copy mailed to me.  She did email me a letter immediately.  I received a written letter from UPMC yesterday in the mail.  I printed the email copy of the letter and faxed it to Neurology.  They then sent the verification to the lab, and the testing is currently in the process of being done.
Their inefficient and inept handling of our grievance really upset me.  It takes a lot to make me as mad as I was at UPMC Health Plan.  However, as far as I'm concerned, their careless and inept handling of the notification process cost us weeks and precious time in regards to the medical care necessary for Elizabeth.  I don't mind waiting for them to make decisions about expensive testing, but I do mind them waiting three weeks to notify us about their decision.  Especially since if I had not called, and if the Pediatrician's Office had not notified me, then we STILL may not know that we had won the appeal. The other frustrating thing to us is that this Microarray testing will take 4-6 weeks for us to get back.  If they had notified us promptly when they had made their decision we would be getting results back any day now. There is really no excuse for them wasting precious time. 
I also recently found out that another local special needs parent also had a similar issue with UPMC Health Plan.  She was notified two weeks ago that her child's genetic testing had been approved in NOVEMBER!  That's right, in JANUARY she was sent a letter stating that they had made a decision in NOVEMBER.  This family has been waiting all of this time for notification, and again their inept handling of notification on grievances made a family needlessly wait and waste precious time for answers for their child.  So, I drafted and sent them a scathing letter about my disgust and recent experience. I encouraged this other mother to do the same as well. 
On a positive and happy note, we are getting closer to more answers for Elizabeth.  Also, I was informed by Genetics that it is rare to win a first-level grievance on genetic testing, and that we should be pleased.  I am pleased that the insurance company made the right decision.  I just wish they had been more timely in their notification.  The moral of the story is that persistence pays off!
Gastroenterology (GI)
We did receive more good news from her GI doctor, Dr. Lindblad.  He called me on Friday evening.  Dr. Lindblad reported that Elizabeth's remaining stool samples came back normal.  Also, he decided that we are going to push the scoping back at this time.  He had wanted to do an upper endoscopy and colonoscopy to check her digestive system for signs of inflammation and other abnormalities that may be causing the diarrhea and failure to thrive. He does want to do these tests in the future, but since she has again had so many viruses recently he wants to hold off until she has a period of good health.  Dr. Lindblad said that this would ensure that any abnormal findings would not be due to her digestive tract being irritated by a virus. It was so nice to receive a phone call from him, especially on a Friday night after 6 p.m.  It means a lot to us when the doctor's themselves take time to personally call us.
Hematology:
I did speak to Dr. Cooper, her Hematologist, last week as well.  Right now, we are not going to follow up with Hematology on a regular basis.  We will do so if her anemia becomes more frequent or other issues arise.  He did say that when she reaches adolescence/ adulthood that she should not have any products with estrogen due to her Factor V Leiden.  Estrogen produces increases chances of developing a blood clot, and so does her having Factor V Leiden, so it is recommended to avoid estrogen products. Dr. Cooper also reinforced a discussion that I had previously with Dr. Larkin (her Immunologist).  He said that they have marked in her permanent record that if Elizabeth should ever need to receive a blood product that it needs to be cleaned first.  Since she has low levels of IgA, which is an immunoglobulin, she is at a higher risk for adverse reactions to receiving blood products.  Cleaning the blood products first helps to reduce the risk of a severe reaction to receiving a blood product. So, hopefully she never needs a blood product, but if she does it is something that we will need to alert medical professionals to and remind them that it is in her chart, if they didn't already see it.
Consults and Trips Scheduled for this Summer:
Due to the recent and overwhelming flood of generosity, the amazing people who surround us (ALL OF YOU) have made it possible for us to take Elizabeth to some important consults at leading Children's Hospitals in the country at Boston and at San Francisco.  Her physicians at CHP have recommended consults with them due to their expertise in some of Elizabeth's areas of medical complexity.  I have been working on scheduling appointments in both locations. So far we will be seeing the following providers this summer:
Boston:
Dr. Janet Soul, Neurologist/ CVI/ White Matter Disorders in the Brain Specialist, June 17th
Dr. Andrew MacGinithe, Expert Immunologist, June 18th
San Francisco:
Dr. Elliott Sherr, Neurologist/ Corpus Callosum Deficits Expert,  June 22nd
Dr. William Good, CVI Expert/ Pediatric Ophthalmologist, June 24th
Dr. Morna Dorsey, Expert Immunologist, June 24th.
There may be additional providers added to those lists, especially as we learn more through the genetic testing. There may also be additional scanning or testing done at both locations.  Both locations have been so cooperative in helping us to coordinate multiple appointments over those dates.  We are looking forward to taking Elizabeth to these experts to gain their insight into her many issues.  We cannot thank you all enough for your love, support and prayers for making this all possible.  We truly, truly appreciate it. 
More Movement and Improvement:
Elizabeth received her Gait Trainer this fall.  We had an evaluation at the Children's Institute to get a gait trainer for Elizabeth to provide her necessary assistance with being upright and walking.  Her hypotonia and other neurological issues prohibit her from standing or walking on her own.  The gait trainer is like a scientific baby walker that gives Elizabeth support to gives her the ability to try to walk.  The insurance company did pay for the gait trainer, which was great.  It is clearly a piece of adaptive equipment that increases her quality of life and promotes her independence.  She does take steps in her gait trainer, has walked across our dining room floor in it, and she sometimes just likes to stand in it.  Jason and I love to see her face when she is in her gait trainer.  It is an incredible feeling to see how proud she is that she is standing and walking.  I've included some pictures below so you could see her in her gait trainer. 




Elizabeth has also been attempting to crawl on more occasions, which is great. The Periactin, which is the medication that the GI doctor gave her to stimulate and increase her appetite, seems to be working.  It definitely makes her sleepy, which is good for her.  She has never been a good sleeper, but has mostly been sleeping all night since we started the medicine.  Elizabeth also seems to have an increased appetite on most days.  There are some days when she has very little interest in eating, but on days when she is hungry she eats or wants to eat a healthy amount of food.  We are glad to be seeing this improvement in her appetite.
We are continuing to give her Pediasure to help her gain weight and to replace milk.  Elizabeth coughs and chokes while eating less with the Pediasure, because it is thicker than milk.  She cannot have any other liquids at this time, because until we have a second swallow study in March other liquids are still considered unsafe and an aspiration risk.  She also still only uses a bottle with a medium flow nipple.  Anything that flows faster, especially sippy cups, are a major aspiration risk for her.  So, the only thing she drinks from is a bottle, because nothing else is safe. The Pedisure does a good job of keeping her full, which is good.  We are seeing small gains in weight, but the doctor's and nutritionists would like to see bigger gains.  Right now, she is averaging around 3 ounces of gain per week.  It is better than 3 ounces of loss, but it would be even better if it were a greater gain than 3 ounces.  So, we have started adding a calorie supplement called "Duo-cal" to her foods.  This gives her approximately an extra 250 calories per day.  We can mix it into her food and liquid, and she seems to tolerate it well. Here is a picture of our princess in her princess slippers eating her favorite snack: Gerber Puffs.
We were also finally able to get her Pediasure covered by her Medical Assistance, which is great.  It is about $40/ case, and we go through about a case per week.  This is very helpful to us financially.  The insurance is covering it, because the Pediasure is considered to be medically necessary to help with her poor and slow weight gain and growth. There is a possibility that if the Duo-cal supplement helps with weight gain that we may be able to get it covered as well, which would help because it is also expensive. However, no matter how expensive, we will continue to give her whatever necessary and recommended to help her weight gain.  We want to avoid the G-Tube if at all possible, although some days it feels like a losing battle.
Thank you for reading, following, and for your thoughts and prayers.  I leave you with a few other pictures of sweet Elizabeth.  Enjoy your day!




Sunday, January 25, 2015

A Flood of Generosity

A Flood of Generosity
We have always known that we are blessed with an amazing support system. We have a wonderful support system with our families, and we work with amazing people at Karns City.  We have a circle of friends who have offered their support time and time again, and our church family is perpetually praying for our Elizabeth.  We are part of a community that takes care of it's own.  There is no way to deny that we are simply blessed, and we have recently been deeply blessed by a great flood of generosity. 

Since this fall, when things started to get a bit crazy with our journey with Elizabeth, we have had a team of followers.  Maybe you're reading.  Maybe you're praying for us and Elizabeth.  Maybe you've sent us a card, or a gift, or offered to help us with Celtan, or cooked us a meal.  Maybe you have done all of these things or some combination of these things.  We have an anonymous and very generous angel who sends us a card with kind and encouraging words every month with a very generous gift card to Wal Mart inside.  I cannot thank them enough, because it has been tremendously helpful to us to buy groceries, diapers, and other things that we need for Elizabeth. Every time I receive a card in the mail from the anonymous angel or from anyone, I still can't believe it. We have continued to be overwhelmed by the kindness and generosity that has been shown to us by all of you. 

Last weekend, a friend contacted me and offered to pay for our plane tickets to San Francisco.  The friend asked me why I keep saying "if" we take her to Boston, or "if" we take her to San Francisco, and why we don't say that we "are" going to take her to the doctors in those places. I explained that even though we wanted to take her there to see those specialists that it was a financial issue for us.  Aside from impacting us emotionally, this journey with Elizabeth has impacted us financially in a big way.  We have bills piling up.  We wonder sometimes if we are going to have enough gas between paydays.  We round up change to make a run to the store for milk.  It could be worse, and I'm not complaining, because I'd rather be broke and live in a tent knowing that I have done everything I could to give her and Celtan everything they need.  However, it has impacted us financially. The friend then said that they would like to help us out, and that they would like to help by paying for our flights to San Francisco.  Seriously, anyone nearby probably thought that I had won the lottery.  I was crying and shaking and smiling and saying, "Oh My! Really?  Is this for real?"  I simply could not believe it.  It was a colossal act of kindness that we had desperately needed after a rough week of appointments and news.  I have thanked them several times, but I still will never be able to thank them enough.  They are the first reason that our "if" we take her has now become a "when" we take her to both Boston and San Francisco.

All weekend long, I could not stop feeling like a huge weight had been lifted off of my shoulders.  It was a relief to know that it was now a possibility to take Elizabeth to see some of the world's leading specialists in many of the medical and special needs that she has been diagnosed with by her doctor's at CHP. I was making lists of providers that had been recommended to us so that I could start getting appointments set up with them.  This way, we could maximize our time out there and pick as many brains as possible to try to put the pieces of Elizabeth's puzzle together.  I always feel guilty when anyone offers to help us, and it is just the way that Jason and I are as people.  We are the ones who are usually on the helping end and not the ones being helped, or at least we like to think that we are those people.  We have made it a point to teach our kids the importance of kindness and helping others, because those are things that are important to us.  However, I was very grateful to our friend who so graciously offered to help us in such a big way. 

On Monday, we had an in-service day at school. I was at my desk and received a message from my cousins that "Team Elizabeth Jeanne," which includes my cousins, sisters and other members from my "Kepple" side of the family, will be hosting a benefit for our Elizabeth to help raise money for our expenses to Boston.  I, of course, started crying, because I just could not believe they were doing this. They all have jobs, kids, are married, have a thousand other things to do in their lives but to plan a benefit for Elizabeth.  However, they got together and decided that they wanted to help us out and help us to get her to see these specialists. I love this group of girls and  all of my family more than they will ever be able to imagine, and I'm so proud and honored to have them by our side at all times.  I told Jason and he was again overwhelmed and very humbled that they wanted to do this to help Elizabeth. He just shook his head and smiled, which for him speaks volumes. 

The "Team Elizabeth Jeanne" committee created a flyer with some important info about the benefit, and they posted the information on Facebook and Twitter.  The response was nothing short of overwhelming and amazing.  I still am trying to wrap my head around all of the people who said they would come, offered to help, offered to donate, offered to donate a basket or item.  It was simply amazing.  They are planning a Spaghetti Dinner and a raffle with baskets and other donated items. My sister Becca and her boyfriend, Matt, attend Westminster College, and they both have their fraternity and sorority getting involved to help with the benefit.  It is just amazing the amount of people who have offered to help, and it is so greatly appreciated by Jason and I and "Team Elizabeth Jeanne."  So, please feel free to come and join us that day, if you'd like to! We are looking forward to being there so that people can meet Elizabeth, and so that we can thank everyone for their support. Here is the flyer for the event:  


The "Team Elizabeth Jeanne" committee has created a Volunteer Spot page for Elizabeth's benefit.  If you would like to sign up to donate a food item, raffle items or gift baskets, or if you would like to donate your time to serve that day, you can go to the website and sign up for anything that you would like to help with that day.  The site is very easy to use, and it does not take long.  However, if you are having trouble with the site, or just simply would rather have one of the girls sign you up, just ask one of them and we can get you on the list.  We are all so overwhelmed with the outpouring of support that the girls decided to start the Volunteer Spot page to keep track of who was offering to donate or help in specific ways.  The website for the Volunteer Spot sign ups for the benefit can be found at:  http://www.volunteerspot.com/login/entry/9978464280103.  You can click on the link or copy and paste it into your browser. 

If you would like to help or get into contact with one of the "Team Elizabeth Jeanne" committee members, here are their names/ email addresses:
1.  Holly (Bly) Wilson: hollyjbly@gmail.com
2. Emily (Bly) Rekich:  emrekich516@yahoo.com
3.  Kellie (Kepple) Johnston:  kckel134@aol.com
4. Katie (Kepple) Callihan:  KT.Callihan@aol.com
5.  Ashley (Kepple) Ciafre: ashleyciafre@gmail.com
6.  Laura Kepple:  laurakepple@yahoo.com
You can also let my parents (Jeff and Amy Bly) or any family member know if you're interested in helping, and they can fill you in on details.  We appreciate your support more than you know.  


The next wave of generosity came as a surprise when Jason's cousin, Quinn, contacted me and said that her friend, Terri Boehmig Kriberney, contacted her and was interested in running a fundraiser for Elizabeth to help the benefit through her Premier Jewelry business.  I also could not believe this, because we did not know Terri, and yet she still wanted to help us.  We graciously accepted her offer, and she created a flyer to be linked with the event.  Terri is offering 50% of sales, donating baskets to the raffle at the benefit, and she is also offering $50 to everyone who books a party with her towards Elizabeth. You can place an order at any time by contacting her.  I'm still amazed that Terri would want to offer such a generous portion of her revenue to our Elizabeth, and Jason and I are very grateful for her willingness to help out.  Here is the flyer that Terri created, and if you are interested then you can contact her for details!


As if all of this was not enough, we were surprised again last week with another generous offer to help us raise money for Elizabeth.  Megan Slaugenhoup contacted me and offered to set up a Go Fund Me account for Elizabeth.  Megan had talked to the "Team Elizabeth Jeanne" committee about the idea, and they all felt that the Go Fund Me site may be an excellent way to allow people who want to help by donating money for Elizabeth's traveling expenses who can't come to the benefit due to distance or other circumstances.  Jason and I were shocked again by yet another offer of generosity and kindness, and we thanked Megan for thinking of us.  Megan agreed to set up the site and did so the next day.  You may have seen it on Facebook or Twitter, but I also am going to post the link below for anyone who does not use social media who is interested in checking it out.  The link for Elizabeth's Go Fund Me site is: http://gofundme.com/kkrlog. You can also click on this link or copy and paste it into your browser. 

The generosity still continues to flood in, and it is truly amazing.  We received a very generous donation from a local family who we have known for years.  It blew me away.

In a world full of bad and depressing news, on a journey with our daughter that is sometimes flooded with gray skies, you all are our bright stars in our sky.  Kindness brings about kindness, and you all have proven this to be true.  What started out as an anonymous card, then became a friend offering to pay for plane tickets, and then turned into a Team Elizabeth Jeanne Committee and a benefit, and has in turn spiraled into an overwhelming number of offers to help out our dear Elizabeth.  All of these offers are unrelated and are all offered by different people, but each act of kindness has followed another act of kindness and generosity.  This flood of generosity is proof that there is still so much kindness, goodness, and so many truly wonderful things in the world.  It is true that there is no act of kindness too big or too small.  It is all kindness, and it all matters.  From following the blog, to praying for Elizabeth, to helping to support her in other ways by your generous donations, it is so kind and it matters to us in a big way.  You have helped to keep us going.  You have helped to keep us strong.  You have helped to brighten up our dim days and to make our bright days brighter.  You give us hope, not only because now we can now take our Elizabeth to these specialists who will be able to give us very important insight, but you give us hope in the goodness of the world and the kindness of others. We are so blessed to have you all in our lives and on this journey, and we thank you from the bottom of our hearts. 

Oh, and one more thing...
WE ARE GOING TO BOSTON FROM JUNE 16-18 AND SAN FRANCISCO FROM JUNE 21-24TH!!! THANK YOU ALL FOR MAKING THIS POSSIBLE AND FROM TURNING AN "IF" INTO AN AFFIRMATIVE.  THANK YOU TO INFINITY AND BEYOND!


Recent Test Results and Plans of Action

Recent Test Results and Plans of Action
Our trips to CHP for the appointment days over the past few weeks brought more tests, some more answers, and some more paths to travel down.  The results are starting to trickle in, and the doctor's have been calling to give their take on what we are learning, which is a mixed bag of good news and more leads.  However, so far nothing very alarming is coming back in the results, which is always good!  So, here is a run down of what we are learning, and what our current plan is to meet Elizabeth's needs based on the current round of testing: 

Gastroenterology (GI):
On January 14th, we had an appointment with GI.  I discussed the details of this appointment in an earlier post. Dr. Lindblad, the GI doctor, ordered several lab tests, as well as some scoping and stool sample analyses.  We had the blood tests drawn the day of the appointment, and we are slowly getting results to trickle in from those tests. The good news about the GI blood testing is that she is not showing any signs of inflammation, and the marker testing for inflammatory diseases of the bowel (Crohns and Ulcerative Colitis) are negative.  So, this is really good news, as both of those inflammatory bowel diseases run in our family.  There is still some blood work pending, and so far the results that are in look pretty good, except for her vitamin saturation results.  Dr. Lindblad also ordered several blood tests to take a look at how well Elizabeth's body absorbs and processes certain vitamins that are essential for growth and nutrition.  These results are especially important for us to know, especially since they are considering a high probability of putting in a G-Tube for nutritional purposes. So far, these results are showing that while she has normal levels of Vitamin D, she does not have normal levels of Vitamins A and E.  In fact, she has an obscenely high amount of Vitamins A and E in her system, and she does not take a multivitamin. We tried a multivitamin the past, but it seemed to give her more diarrhea than actually help her, so we stopped using it. So, the high levels of these vitamins could mean a few things, and one of the theories/ plausible causes is that her pancreas/ liver/ kidneys are unable to, or are not efficiently, breaking down these vitamins when she ingests them as part of her diet. We are still waiting for other results to come in, but as of right now Dr. Lindblad has laid out a plan.  The plan is to get the stool samples turned in, and to get the sweat test done that he had ordered ASAP, and determine whether or not the scoping was necessary after we get the rest of the blood/ sweat/ stool results back.  

Dr. Lindblad's office called me a few times on Wednesday (1/21)  to hash out a plan of attack.  On Wednesday, I felt like I was a secretary, because I had been on the phone 8 times with Children's by the time I left school that day. The sweat test was scheduled for Friday, and I had to move it up from the date that I had it originally scheduled in March so that it could be done before the scoping.  Her scoping is scheduled for February 23rd.  I was not planning to have to take off on Friday, but I had to take her down for her sweat test. Since I was also going to be at CHP on Friday, I decided to collect her stool samples on Thursday night and take them with us on Friday.  So, I collected 5 different stool samples and took them down.  (Don't worry... I wore gloves and followed OSHA protocol...haha)  I turned the samples into the lab on Friday, and then we waiting for our sweat test.  

The sweat test took about an hour.  No, she did not have to do aerobics with Richard Simmons or Jane Fonda.  (haha) They put a small disc on her arm that was attached via a bracelet/ armband.  Then, they collected her sweat into a small tube on the arm band.  Dr. Lindblad ordered the sweat test to rule in/ out Cystic Fibrosis as a diagnosis.  Elizabeth did well during the test.  It is not invasive and does not hurt.  Dr. Lindblad's office called us later in the day to tell us that the test was negative, which is great.  So, we can take Cystic Fibrosis off of the table as a potential explanation for some of her symptoms.  His office is going to call when they get the stool analysis results back from the lab, and we will go from there.  

Hematology:
We also found out on Thursday that Elizabeth tested positive for Factor V Leiden, which was one of the blood tests ordered by Hematology.  Factor V Leiden is a blood clotting disorder that increases your likelihood of developing blood clots, and it means that your body does not break down clots as effectively. So, as much as I didn't want Elizabeth to join this club, I am not surprised, because I also have Factor V Leiden.  Two of my sisters (Holly and Emily) and my dad also have it.  I found out that I was Factor V Leiden positive when I was pregnant with Elizabeth, and I have never developed a blood clot.  However, it is a genetic blood disease, and therefore it can be passed down.  I am still waiting to hear back from Hematology as they are expecting a still pending result to come in Monday, which would tell us if she has any other blood clotting disorder, before they make a plan for follow-up, etc.  They also want us to have Celtan tested for it.

Elizabeth, like me, only carries 1 gene for it, which is good because the form where you carry 2 genes for Factor V increases your risk for a blood clot even more.  Since Elizabeth only has one gene, like me, Celtan either has Factor V Leiden or he doesn't.  If Jason was also a carrier for it, then likely  Elizabeth would have 2 copies of the gene and not one.  Since, she only has one copy of the gene, then it is likely that she has it, because I passed it down to her.  The Hematologist, Dr. Cooper, said that it is important for us to know about this for Elizabeth for follow-up, and also because of her hypotonia that causes some inactivity.  Since her muscles are weak, she does not move around like a normal toddler.  She can't walk or stand by herself, and she is not as active as she should be at this age.  Since inactivity greatly increases your risk for a blood clot, and since she does have inactivity due to her hypotonia and muscle weakness, and because we now know that she has Factor V Leiden, then we will have to be alert for signs and symptoms of a blood clot.  It is also helpful for Celtan to know if he has it, because it is genetic.  Factor V Leiden is actually fairly common, especially the form that we have that involves only 1 copy of the gene, in people of Northern European descent, and it is hypothesized that many Caucasians have this disorder but are unaware of it.  It still is just helpful to know.  I will post more about Hematology's plan for follow up once I know more, which will hopefully be early next week.

Immunology
Dr. Larkin, Elizabeth's Immunologist, is really amazing.  When she called me on Wednesday, she talked to me for 45 minutes. It is a wonderful feeling, especially when you deal with so many doctors and appointments, when a doctor calls you back and not the nurse or another office worker.  Not that those other individuals are not helpful, but actually talking to the doctor is like whipped cream on top of a sundae, or icing on a cake, and it is much more productive. I could not believe that I was on the phone with her for 45 minutes, and this conversation was super informative and productive. 

Dr. Larkin ordered several blood tests, which we had drawn the same day as the appointment on Wednesday 1/14.  The results are slowly trickling in, and what we are finding out is promising.  The results are showing that aside from a deficient/ low level of IgA in her immune system, the rest of her immune system is pretty much working normally.  She has some numbers that are a little off of normal ranges, but Dr. Larkin said she will just watch the numbers to see what they do over time.  She ran a DiGeorge Immunology Panel, and it was normal.  Dr. Larkin explained that if she is diagnosed with a 22q11 deletion, then she has a variant of the syndrome that is not disrupting her immune system.  The counts that she is going to keep an eye on are Elizabeth's IgA level, her NK (Natural Killer) Cell level, and her B Cell levels. Dr. Larkin explained that she would like to see her every 6 months to check these levels until she is about 4.  If at the age of 4 Elizabeth is still deficient in IgA or some of the other immune system levels, then they will diagnose her with something called "Selective IgA Deficiency."  They will at that time consider if she would need immune system replacement therapy or a daily regimen of antibiotics to help keep her immune system working efficiently. The reason that Dr. Larkin wants to track these levels until age 4 is because sometimes it takes a child's immune system until that age to fully develop and regulate their levels.  So, after tracking her immune system levels for the next 2 years, if she still has deficient levels, then an official diagnosis can be made for a deficiency.  Dr. Larkin has also said that she would like me to let her know when Elizabeth gets infections, and specifically when she has another infection that requires an antibiotic.  At that point, Dr. Larkin is going to consider putting her on an antibiotic regimen maybe two or three days per week.  This is not protocol, but because she seems to want to eat less when she is sick, and her weight is a major concern, she would consider this plan of action to help her immune system work more efficiently and hopefully help her to feel better more often. 

Dr. Larkin also gave me the names of Immunologists in Boston and San Francisco.  Since we ARE going to both places this summer, thanks to the overwhelming generosity of all of you that will be explained further in another post, we want to make the most of our time there.  She has colleagues in both locations, and she is going to be contacting them to explain why she wants them to see Elizabeth.  In the meantime, I am working on setting up appointments with her colleagues in those cities.  It meant a lot to me for her not only to take the time to link me up with these physicians, and it meant a great deal as well that she is going to personally reach out them about Elizabeth.

Genetics:
We are still at a bit of a stand still in regards to the Genetics testing.  The past few weeks they have been telling me, and her doctors, that they will be releasing their decision in the next week.  Every week, they say the same thing.  So, her doctors now have their nurses and secretaries calling and faxing and doing everything they can to reinforce the importance of the microarray genetic testing for Elizabeth.  I am also going to continue to call, and her Pediatrician, Dr. Vigliotti, is also going to call them.  They have been sent numerous letters and statements and physicians detailing scores of reasons why this testing is important.  Her doctors said that the insurance companies will often drag their feet until the last day that they have to give a decision.  They have 60 days from the day that the appeal is filed, and their window of time is dwindling down, but we still do not have an answer as to whether they have decided to pay for the testing or if we have to appeal again.  Her team of doctors feel that they will win the appeal, but it is just going to be a matter of playing the insurance company's waiting game.  If we should be denied again, which is unlikely, then we will just keep appealing their decision. Ultimately, this test will help her doctors to put a name to a syndrome or disease that is responsible for her many medical issues and will then help them to be able to predict the progression of the disease/ syndrome.  If they know the progression or what to anticipate, then we will be better able to help Elizabeth as she grows older. 

In the meantime, Genetics, Neurology and Immunology are working together to get some other types of genetic testing ordered for Elizabeth.  One of the tests is a diagnostic tool for Ataxia Telangiectasia, and the second test is a FISH analysis for 22q11.  These two additional tests will help us to be able to definitely rule in or out these two diseases/ syndromes. 

Recent Accomplishments
Elizabeth is showing some big leaps in progress lately, which are so exciting for us to see.  As her support system and followers, I figured you would be just as overjoyed and excited by her recent progress.  As  you know from my last post, Elizabeth has started to try to crawl. She does not crawl consecutively, and she does not try to crawl very often, but when she does attempt to crawl we watch with delight.  She crawls in her own inchworm way, and sometimes drags her head on the floor, but it is still amazing to us that she can even crawl in any manner with all of her muscle weakness.  

She has also been wanting to hold her bottle and feed herself sitting up.  However, she can't quite get the hang of tilting her head/ bottle back enough so that she is actually getting something other than air through the bottle. She tries though, and is so determined, and I know that one day she will accomplish this task before we know it!  

Elizabeth also recently has been holding her arms up to reach for people.  She reaches the most for me, which melts my heart.  Elizabeth is definitely a mama's girl. In fact, one of the only words that she says clearly and with meaning is the word "mama."

Our Princess also has been watching Celtan with more intent.  She watches him play, will reach for toys, and she has even tried to take a toy away from him.  With her vision and neurological issues, it is really incredible that she is reaching or interested in other people, especially interested enough to want to take the toy.  Her EI therapists are pleased with this progress. 

Elizabeth also has been making a lot of vocalizations lately, and she has also be responding more to her Communication Book and using her sign language.  She is starting to use the sign for "more" when she is eating.  She also has a book with Velcro pictures on it that represent toys, songs, etc.  She will pick up the picture of what she wants, or point at it, and sometimes that is really what she wants and sometimes it is not.  However, it is a start, because since she is not saying words it is hard for us to know what she wants or needs.  She does say "mama" and makes a lot of sounds, but her speech is extremely delayed. Elizabeth also loves the song "Row, Row, Row Your Boat."  She will sit on your lap and rock, which is her sign language for wanting you to sing the song. 

Speaking of eating, Elizabeth has this week been on an upward trend for weight gain.  This means that the Pediasure must be working.  I think personally that it is a combination of the Pediasure and all of the prayers, but the Pediasure is clinically responsible for the weight gain. She did lose a few ounces towards the end of the week, but overall this week was considered a gain for weight.  So, we will take it! They will continue to watch her and weigh her, and we are continuing to give her Pediasure.  Currently, she is drinking 3-4 milkshakes a day.  The secret angel that we have that keeps sending us the lovely cards and Wal Mart Gift cards will never know how much that has helped us out.  The Pediasure is expense and Wal Mart or Sams Club are the cheapest places to buy it!  So, please know, whoever you are, that your generosity not only means a lot, but it also has helped us to pay for something that she really needs.  We are currently working to have the insurance company cover it as a prescription, and we should be able to start having it be filled as a prescription in the beginning of February. However, I don't hold my breath when it comes to the insurance company.

Overall, we are pleased with her recent progress.  It is truly a testament to her amazing staff of EI Therapists, as well as BC Bachman who watches Elizabeth for us while we are at work.  The EI Team has given us so many tips and ways to help Elizabeth grow and develop, and I can't imagine how different our story would be if I had not decided to bring them into our home when she was 3 months old.  Even though Elizabeth often takes these spurts of growth and development, and she is clearly in one now, these spurts are often followed by periods of little to no progress towards milestones.  In the times when she seems to have plateaued it helps us to reflect on how far she has come.  Even when she seems stuck, and it has been a while since was had a development burst, we look back and are amazed at our little miracle.  The fact that she can do any of these things, especially knowing what we know now, is really nothing short of amazing.  So, thank you for your prayers and support and for following us on this journey.  Elizabeth is proof that miracles exist in the world and that anything is possible.  Even when the cards seem stacked against you, there is always hope. Speaking of hope, we are hoping for continued upward progress as we look forward to a break in appointments.  As of right now, we are not scheduled to be at CHP again until February 16th. However, since we are off from school that day I booked as many appointments as possible, and I think our current count is up to 5 appointments on that day.  We will keep the faith and keep moving along until then hoping for continued progress, and weight gain, and good news.  







Friday, January 16, 2015

There is much to discuss: The Good, The Bad and The Blessings

There is much to discuss:  The Good, The Bad and The Blessings

This is a very long post, but there is a lot to update, a lot to discuss, a lot to process about our recent appointments and findings.  We had several appointments for Elizabeth spanning three days:  Friday 1/9, Tuesday 1/13 and Wednesday 1/14.  Each day has brought us a variety of both questions and answers.  A mix of information that is both good and bad, promising and frightening, and all of this has delayed my updating the blog.  We just needed time to process, wrap our heads around, and just take a few deep breaths about everything that these appointment days have brought our way.  
Friday 1/9  Appointments:


On Friday, January 9th we had three appointments at Children's:  Hematology, Rheumatology and a post-surgery follow up with Cleft/ Craniofacial and ENT. The day started out with a lot of positive news. 

Hematology
At our visit in Hematology, Dr. Cooper ran a lab test on a sample of Elizabeth's blood to reevaluate her anemia.  We were delighted when he told us that her blood counts had completely normalized and she no longer had anemia!  Her blood counts looked great, and Dr. Cooper believes that she likely has a type of anemia that flares up when she is having an period of illness.  We know that Elizabeth has some immune system issues, particularly because of some recent lab results, and Dr. Cooper explained that when your immune system is not working effectively that anemia can often occur, or become worse, during period of illness. He believes this to be the case of Elizabeth.  So, we really do not need to follow up with him at this time, unless there are new developments or the anemia reoccurs and does not resolve itself.

Cleft/ ENT Post-Surgery Follow-up

Then, we had an appointment with the Cleft Team and ENT as a post-surgery follow up for Elizabeth's surgery and other procedures in December.  We have noticed some improvements in Elizabeth since her surgery, which is great.  However, we also have noticed some lingering things, like coughing and choking while drinking. Overall, these scary coughing and choking episodes have been reduced since her surgery, but they do still happen and are just as scary. We discussed the observations of her eating and drinking behaviors post surgery.  The teams checked her out, and they also agreed that her breathing and speech sounds also sounded more clear than before the surgery.  Then, we started into another discussion, which I knew eventually would come, but I still was not prepared for it.  

Our past few appointments with this team, and over the past 3-4 months in general, Elizabeth has had some issues with weight loss and a decrease in appetite. I have attributed these issues to her nearly chronic respiratory and upper respiratory infections, other illnesses and her recent surgeries.  There are many days when we struggle to get her to eat anything, even when she should be hungry she refuses to eat, and she seems uninterested in eating pretty much anything that you put in front of her.  We also have days when she eats great.  We definitely struggle more with eating when she is sick.  You may be thinking, "Well, that's normal.... or that sounds like my toddler."  Even Celtan has days like that.  Even I have days like that.  However, Celtan, nor I, have issues with being underweight.  This is why these weight loss and decreased appetite episodes are so alarming and of such concern to her doctors.  Elizabeth is considered to be underweight, and failure to thrive, because she is below the 5th percentile in weight.  We added a nutritionist to her Early Intervention team in December to help us better monitor her weight and appetite.  Even when she loses a few ounces it is a big deal, because her weight is at such a critical level.  Another drop in weight, again nothing drastic just some ounces, was noted at her weigh-in at this appointment.  

Her Speech, OT, and Nutritionist, as well as the Cleft/ ENT team and her pediatrician have all been concerned with the decreased appetite and weight issues lately.  The other thing that they have been concerned about is the continued coughing and choking while drinking.  Two questions are always hovering around regarding Elizabeth's feeding and swallowing issues:  How much of this is able to be corrected through procedures and surgery, and how much of the swallowing/ feeding issues are related to the hypotonia (low muscle tone) and will never be able to be corrected?  While we are hopeful due to the benefits that we have seen from her surgery in December, and there are still some other things that we can try, we have always known that the possibility remains that all of this may have been in vain, that no amount of surgery or procedures will ever correct her swallowing since she has the hypotonia. It is a fine line, but its a blurred line, and we have to exhaust our options to try to correct the swallowing before we chalk it up to a hopeless cause due to the hypotonia.  The other underlying issue is that if the choking, coughing and aspiration while eating persist, and we have exhausted all of our options to correct these issues, then there comes a point when we have to draw the line and determine that it is simply not safe for her to feed by mouth.  

It was a combination of concern over the weight loss and the swallowing safety issue that led us to the discussion for which I was not truly prepared for that took place in that appointment.  The Cleft/ ENT team discussed the fact that a feeding tube, specifically a G-Tube, is a very likely option in Elizabeth's future.  They discussed a list of benefits that it would provide to her on both a nutritional and safety level.  I appreciate the relationship that I have with them, because they do value our input.  While they were able to convince me of the various benefits, as a parent I feel that discussing the fact that your child needs a feeding tube is NEVER something that you are prepared for.  As much as I knew that this discussion would need to be had sooner or later, and despite the fact that I had discussed this issue with some of her EI team members earlier in the week, I was still not ready for it.  I explained that I feel that the past few months have been full of sickness and then the surgery, and that in my opinion these have all contributed to the issues that are prompting the urgent discussion of the feeding tube.  The team agreed that this was a plausible theory, and the conclusion of the discussion was that they would give me some time.  Instead of repeating a swallow study at this time, as was the original plan, they are going to have a swallow study repeated at her next follow-up with them in March.  This will hopefully give her time to heal a bit longer and to have a period of healthiness.  If she can put on some weight, increase her appetite consistently, and if the swallow study shows no aspirations, then we can table the discussion of a feeding tube for now.  However, in the meantime, her pediatrician, nutritionist and we are going to be monitoring her eating habits, appetite and weight loss/ gain very closely until then.  She will have to maintain a very upward and unwavering curve, plus eliminate her feeding and swallowing difficulties and aspiration for us to completely eliminate the possibility of a G-tube for Elizabeth.  However, the chances of the G-Tube being a reality for Elizabeth and for us is likely unavoidable for many reasons.  

This appointment alone overwhelmed me, and the Cleft/ ENT team were very supportive.  The team recommended eliminating milk from her diet and moving to all Pediasure.  Currently, she is getting 1 Pediasure a day, but they do not feel that is enough.  In order to bulk up her protein, calories and nutritional intake we are moving to Pediasure instead of milk as our new plan.  This should help her weight gain, and Pediasure is slightly thicker than whole milk so it also reduces the risk of aspiration.  They also recommended finding any means necessary to increase her overall calorie intake, which can include:  adding butter to her food, feeding her pudding and other high calorie foods, adding more pureed protein to her diet, etc.  Over the weekend, I traded high chairs with my grandmother.  The high chair that my grandmother has is much more supportive than the one that we have here at the house, so we just swapped chairs.  So, we are hoping, even thought it probably sounds odd, that the different and more supportive high chair will help her eating habits improve.  At this point, no matter how odd it may seem or sound, we are committed to doing anything that will increase appetite, increase her nutritional intake, increase her weight, and reduce aspiration.  We are going to put on a good fight to avoid the G-Tube... by any means necessary!  The team agreed to give us some time, and we are going to use it wisely to do everything we can that we are not already doing for her. 
Rheumatology
After that appointment, we had an appointment with Rheumatology.   Due to some of her rashes and other symptoms, it was recommended that we have a Rhuematologist evaluate Elizabeth. So, we went to the appointment.  After a thorough review of her history and an exam of her rashes and joints, the Rheumatologist concluded that at this time she does not think it is anything that is related to Rheumatology.  So, for right now, we can also eliminate that department from our list.  This is always good news.  As hard as the discussion was about the G-Tube in the middle of our day, the fact that the day began and ended with good news made it a win in my book.  
Tuesday 1/13 Appointments:

Our next set of appointments were on Tuesday, January 13th.  That day, we were scheduled to see a Cerebral/ Cortical Vision Impairment specialist, Dr. Christine Roman-Lantzy, at the Pediatric View Institute at West Penn Hospital.  We were also scheduled for follow-up visits with her Diagnostic Referral doctor and her Neurologist. As we drove to West Penn, I could not help but remember all of the times that I drove my sister Emily down to her dorm at nursing school.  
Christine Roman-Lantzy, Ph.D.-- CVI Specialist

The appointment with Dr. Roman-Lantzy was super informative, and I was so glad that our vision therapist recommended her to us. At this visit, Dr. Roman-Lantzy performed an evaluation to determine if Elizabeth's vision issues could be related to a disorder known as CVI, or Cerebral/ Cortical Visual Impairment.  She had Elizabeth perform several tasks, and we discussed her medical and visual history.  A Neonatologist from West Penn also sat in on the visit, and he provided insight and gave us some opinions on many of the vision and other issues that we have going on with Elizabeth. He was very concerned about the overall white matter loss in her brain.   Both of these providers were very helpful, compassionate and informative, and we have again added some wonderful members to Elizabeth's team.  Elizabeth's Vision Therapist also attended the appointment to share her input and evaluation results.  

Dr. Roman-Lantzy concluded her assessments and shared the results with us.  The results added another formal diagnosis to our list:  Cerebral/ Cortical Vision Impairment or CVI.  Dr. Goldstein, her neurologist and the Mitochondrial Disease doctor at CHP, felt that based on Elizabeth's vision history and the results of her brain MRI that Elizabeth had CVI, and Dr. Roman-Lantzy also confirmed it based on her assessments.  The links below provide additional information on CVI, and if you want more information you can check them out.  CVI is a vision/ neurological disorder that causes visual impairment.  In CVI, the eyes are functional, but the individual has issues with their vision due to problems in the brain and problems with the way the brain perceives and interprets visual stimuli.  Early vision therapy can help to increase Elizabeth's visual potential, but because CVI is neurological there is no cure and there is nothing that will permanently reverse or correct it.  Her glasses do help her to see better, but her glasses may eventually not help to improve her vision.  It is possible that she will be able to have vision that is very close to normal in the future, but it is also possible that she will never have normal vision. 

Children with CVI make the most gains, in terms of visual progress, in the first 5 years of life.  So, after Elizabeth ages out of Early Intervention, we will have to consider transitioning her to a program or preschool that specifically addresses visual accommodations and programs for individuals with CVI.  We will also have to make sure that when she is school age that her IEP (Individualized Education Plan) contains the visual accommodations necessary to help her meet her visual needs.  Her need for visual accommodations will be lifelong, just like her having CVI, but the extent of the accommodations needed will depend on the progress she is able to make.  So, once again, we are going to provide her with every possible opportunity to help her gain visual progress.  We are already starting to check into programs and preschools that have specialized programs for students with CVI.  We are planning to take her to Boston Children's Hospital this summer to see Dr. Janet Soul, who is a CVI expert, for her to consult on Elizabeth and offer additional opinions and suggestions on how we can best help her to make progress with her CVI.  We will also likely be enrolling her in a CVI research study in Pittsburgh. The following links contain more information, if you care to read it:



I was so glad to have had the opportunity to meet Dr. Roman-Lantzy.  She will be a great addition to Elizabeth's team.  She is an expert in CVI, and she was able to not only confirm the diagnosis but to offer us several suggestions for ways to provide Elizabeth with the best opportunities for visual growth and progress.  She was very impressed with Elizabeth's determination, curiosity and motivation, and she praised us for our efforts.  We will be following up with her in August.
Diagnostic Referral Follow-up:
After leaving West Penn, we headed over to Children's for our last two appointments.  They were both pretty mild, and I was glad for that.  Dr. Brown, Elizabeth's Diagnostic Referral doctor, discussed with us the concerns about her weight loss and slow progression of weight gain.  She also shared with us that she would like us to consult again with the Feeding Team for additional suggestions on how to increase appetite and nutritional intake safely.  Dr. Brown agreed to help us coordinate this visit, and she said she will contact us once she has that arranged for us.  It is really great to have her help with everything that is going on.  Dr. Brown not only adds a diagnostic angle to the team, but she also helps to coordinate all of these different departments and all of the information. 
Neurology Follow-up

Our last appointment on Tuesday was a follow-up with one of her Neurologists, Dr. Safier.  We discussed the appeal on the genetic testing, why that testing is important, and some other recent symptoms.  Elizabeth has recently started having tremors in her hands and feet.  Dr. Safier noted that we should record these episodes and email them to him.  He also discussed with us that one of the reasons why the genetic testing is so important is because the results will hopefully shed some light on the overall white matter loss in Elizabeth's brain.  At this point, her doctors are questioning whether the white matter loss is due to a genetic syndrome that is progressively deteriorating her white matter, or is it one that causes white matter loss, or did she suffer some type of oxygen deprivation that caused trauma in her brain.  The overall white matter loss is striking, and it is another reason why we are trying very hard to take her to both Boston and San Francisco this summer to specialists in the field of white matter loss. Dr. Safier also said that we can decide in the future to follow-up with either Dr. Goldstein or him, and that we don't need to follow-up with both of them.  He and Dr. Goldstein are both Neurologists at CHP, and they work very closely together. So, no matter which one we choose to continue to follow-up with they will both still be involved in her case.  I like them both, so I am glad that they will both be involved no matter what. Here is an article about why the white matter in the brain is so important, and hopefully it will help you to make sense of why this is so alarming to her doctors:

http://www.dana.org/Cerebrum/Default.aspx?id=39152

As glad as I was for the formal diagnosis of CVI, and for the relative ease of the two follow-up appointments on Tuesday afternoon, I was feeling very overwhelmed by everything.  The CVI diagnosis was a big step for us in solving Elizabeth's puzzle, but it is also difficult to think about your child having a life long condition that basically will restrict them in so many ways.  The CVI also compounds her Sensory Integration/ Processing Disorder, since vision is such a prominent sense that we rely a lot on as human beings. It could be worse, but it is still something that will be an added challenge to an already growing list of issues.  I knew, though, that Wednesday was going to also be more information.  So, I went home and snuggled with Celtan and Elizabeth, and began to try to process what we had learned in the past few days while preparing for Wednesday.  Elizabeth has been trying to crawl, so it is great to see her try to do those things.  Progress is Progress after all!
Wednesday 1/14 Appointments:

On Wednesday, January 14th we had two appointments at CHP:  GI and Immunology.  By all accounts, several of her doctors felt that Immunology was going to be a wealth of information for us, especially since lab results are showing a deficiency in her immune system.  So, I made sure that I had my head ready to absorb more information, and I made sure that I grabbed an extra pen to be able to have enough ink to write everything down. I always take notes when we are at appointments.  It helps me to remember things and to keep focused on the important points. 
Gastroenterology (GI)

Our day started with an appointment in Gastroenterology. We were referred to them because of Elizabeth's issues with diarrhea, weight loss and appetite issues.  The GI doctor, Dr. Lindblad, reviewed Elizabeth's chart, examined her closely, took a stool sample to check for occult blood in her stool.  Then, he asked me what my major concerns were for Elizabeth.  I told him that I was most concerned about the current discussions about a G-Tube, her recent changes in appetite and recent issues with weight loss. He also concurred that his greatest concerns were about her weight gain.  He said that he feels that she is a very good candidate for a G-Tube, and he said that she would benefit from one sooner rather than later.  Specifically, he said that her slow progression of weight gain over time, her low body mass index, the fact that she has never gotten her weight above the 5th percentile, and that her height to weight ratio was so low indicated that a G-Tube is necessary on a nutritional and well-being standpoint. The safety and aspiration issues aside, the GI doctor feels that a G-Tube is in our future.  He also has concerns about the possibility of Cystic Fibrosis and Celiac Disease due to her symptoms and some lab work results.  He ordered several blood tests and sent us home with various stool collection kits.  The GI doctor also ordered a Sweat Test, an Upper Endoscopy, and a Flexible Sigmoidoscopy to test for Cystic Fibrosis and Celiac Disease.  He also prescribed a medicine for her called "Periactin," which should help to increase and stimulate her appetite.  The GI doctor also agreed that Elizabeth should switch from milk to Pediasure.  I expressed concern that when she has Pediasure regularly then she is so full that she doesn't want to eat anything else.  He assured me that was perfectly fine, because she is getting adequate nourishment from the Pediasure.  He also said that if she is full on the Pediasure to consider anything else that we feed her by mouth a bonus.  I was glad for his through assessment, honesty, input and willingness to try to find additional answers to some of her symptoms.  We will be following up with him in March. 
Immunology

We left GI and headed to our Immunology appointment.  There we met with Dr. Allison Larkin and her team.  She reviewed Elizabeth's lab results and medical history.  She also examined Elizabeth closely.  We were initially referred to Immunology by Dr. Goldstein after lab results showed that Elizabeth's Immunoglobulin A (IgA) was low.  Dr. Goldstein wanted Dr. Larkin to give her input on that result and how it relates to Elizabeth's overall puzzle.  Dr. Larkin said that at this time the low IgA result was not super alarming to her, especially since her other Immunoglobulin counts were normal.  Even though the low IgA shows that her immune system is deficient, it does not mean that her entire immune system is deficient.  Dr. Larkin also explained that they will watch her IgA counts until she is around the age of 4.  It usually takes about that much time for a person's immune system to fully regulate and establish itself.  Dr. Larkin ordered a series of blood tests to take a closer look at her immune system and to determine just how well it is working.  She also ordered testing for some autoimmune diseases that they are suspecting based on previous lab results, other symptoms, etc.  I appreciate Dr. Larkin and her tenacity.  She had a lot of really good insight into some of the testing, further exploration, and other ways into helping us put together the pieces and find some answers.  Dr. Larkin asked if she could petition the insurance company for some very specific and expensive testing to rule in and out some big lingering diagnoses, as well as contact Genetics and Neurology to also get them on board.   I really liked her approach.  Her feet-first, jump right in and help us attitude.  In fact, pretty much all of Elizabeth's doctors are like this.  This is not necessarily unique, but there was something about her energy about it all that was refreshing and gave me hope. 

One of the tests that she wants to run is called a FISH Test. It is a specific type of genetic testing that is accurate and efficient in diagnosing 22q11 deletion disorders.  This is still a type of syndrome that keeps being brought up in different departments based on her features, symptoms and other results.  So, this test would help us to rule a 22q11 deletion disorder in or out definitively. 

Another type of testing that Dr. Larkin would like to order is a set of lab work to test for a disorder known as Ataxia Telangiectasia or AT.  The diagnostic question of AT being an explanation for many of Elizabeth's symptoms and other findings has been brought up in several departments.  So, this was not new information for me, even though I really wish it was not something was going to keep coming up.  Not that we really want any type of diagnosis, some days I still wish that someone would tell me that this was all a big mistake and that she is fine, but we really don't want it to be this.  AT is a rare, progressive neuro-immune disorder that causes rapid cell death and is degenerative.  It also makes you around 1000x more likely to develop cancers, due to rapid cell death feature of the disease.  As much as we want answers, and as much as we know that at this point we aren't likely going to get an answer that is easily or solvable at all, we still obviously don't want AT to be our answer.  However, since it keeps getting brought up, I have decided to educate myself on it.  If this is it, then it is better to be prepared.  If the testing comes back and it is not AT, then I will rejoice even more knowing that this terrible rare disease is something that my child does NOT have at all.  There is some very specific genetic and tissue testing for AT, which is expensive, so Dr. Larkin is going to ask the insurance company if they will be on board to just allow us to test for AT and rule it in or out definitively.  Here is a link with more information on AT, and I'm sure after reading it, you will understand why we are hoping and praying that this is not our answer:

http://www.nlm.nih.gov/medlineplus/ataxiatelangiectasia.html

The genetic testing ordered initially is called a Microarray, and it will take a very broad look at her genetic makeup.  We should have a formal decision by the middle of next week on the approval of this testing.  Even though there is a clear need to run this type of genetic testing, since it is so expensive the insurance company needs documentation to convince them as to why it is necessary, and why it would be helpful to the greater picture of Elizabeth's health.  We have more than provided them of that, and the doctors are confident that they will see it our way.  As tired as we are of all of this lab work and other testing, these very specific tests for AT and the 22q11 deletions are very specific and efficient ways for us to be able to rule these disorders in or out.  So, I appreciate Dr. Larkin taking the reigns and moving forward. 

As we left the Immunology appointment, I was feeling a mix of emotions.  It is good to be finally getting some answers and directions.  However, as we have said all along, sometimes the answers we receive are not the answers we want.  I was telling my coworker, Jen, the other day that I just wish I could crawl in a hole and when I resurface find out that this was all just a bad dream.  Instead, we are tunneling through this seemingly endless rabbit hole of more departments, more tests, more questions, and answers are slowly rolling in but the puzzle of Elizabeth overall continues to remain unsolved by everyone on her team.  This is one reason why we are seeking other experts in these areas of concern, because somewhere someone is going to look at Elizabeth and all of her testing and have that "Ah-Ha" moment that puts it all together.  I believe that in my heart of hearts, and so we are going to do whatever we can to get her to any specialist who can maybe add some insight to our Elizabeth.  Dr. Larkin and I discussed us wanting to take her to UCSF and to Boston Children's Hospital this summer to see some specialists in Neurology.  She has colleagues in both hospitals that she said she will link us up with just to have their eyes and brains pan all of this information and help us, hopefully, to plug in pieces to the puzzle.  It may seem like a hopeless cause, a pipe dream, but we don't have anything to loose, and Jason and I agree that the more insight we have the better.

Mom and I left Immunology and headed to the lab. We finished off our day with some more blood work.  Elizabeth had around 15 tests drawn that day.  Thankfully, it went smoothly.  Elizabeth is such a trooper.  She never cried, never fussed, and was so good as they drew all of that blood.  She is so good, happy, and just never ceases to amaze me. She is truly a miracle and a blessing, and even though this whole ordeal just plain stinks, we are so blessed to have her in our lives. We fought so hard to get her here, and we will continue to fight to get her whatever she needs to give her the best life and health possible. 

Ugh, not again!

When we got home that day, Elizabeth had a total meltdown.  Honestly, I wanted to have one, too.  I ended up having to take her upstairs at mom and dad's house and sit in a dark room and rock her to calm her down.  I think that she was on total sensory overload, was very tired, and I detected the hint of a fever.  As the evening progressed, she started sneezing, eyes started watering, a tiny cough started, and then the sheets of snot started flowing out of her nose.  At one point she was choking on her snot.  It was coming too fast for her to swallow, and she also doesn't know how to blow her nose, plus her coughs are rather ineffective at times due to her hypotonia.  Jason and I spent the rest of the evening cleaning off her face, snuggling her, and trying to make her comfortable.  It was clear that she was getting sick, again.  She has yet another respiratory virus.  They did test her for the flu, and thankfully it was negative.  She had the flu shot, so I figured it actually being the flu was unlikely, but you never know.  Last night, she tried to jump in her jumper, but she mostly just laid her head down.  We are just watching her closely to make sure she doesn't get any worse.

Final Thoughts

So, I hope that your brain didn't explode from all of this information.  I know that it took me a while to write this, because I felt like my brain was going to explode.  Jason and I wanted to wrap our heads around all of this, and inform our immediate families first about most of this, before we posted it on here.  Despite of the good news and the bad, we are determined to remain positive and hopeful.  Yet, we also want to maintain a balance of realism.  Its a fine line to walk being both realistic and positive at times. We also are blessed with our support system of family, friends and followers, as well as our prayer warriors.  We appreciate knowing that many people are praying for Elizabeth.  Many people have asked if we care if she is added to their prayer list at church, and we welcome that idea.  Elizabeth is truly a miracle, and she is proof of the power of prayer, and we appreciate all of the prayers, happy thoughts, positive energy and all of those things that can be sent her way.

I want to close with a recent dream of a family friend that I feel reinforces the abundance of blessings that we have despite all of this uncertainty.  Melissa Campbell, who I have known for several years because her daughter Kayla is friends with my sister Holly, shared a recent dream with me on Facebook.  It is funny how we have dreams about people, and my personal theory is that we do this because what we dream is meant as a message for that person.  Even though the message may not make sense at the time, it is our subconscious mind connecting us to each other.  In her dream, she said that she was making caramels in her kitchen.  She decided to walk outside for a minute to get some sun, and she turned around to discover the caramel was running like a river behind her into the yard.  Melissa was upset about having to clean up the mess, and she said that I came along in her dream, noticed the river of caramel, and said, "No, this is wonderful!  The kids will love it." So, I  started to gather the caramel and give it to them.  Melissa said that other people in her dream saw me doing this and joined to help me.  As Melissa told me of this dream, she said that she realized that the caramel, a river of milk and honey, was symbolic of abundance of blessing, words of encouragement and possibility.  She wanted to pass all of those things on to us and to Elizabeth.

I was more than touched by this dream and by Melissa sharing it with me. I feel that this river of milk and honey in her dream is not only symbolic of the abundance of blessings, encouragement and possibility, but it also is symbolic of Elizabeth.  She continues to amaze us.  She continues to amaze her doctors.  When doctors see the brain damage and white matter loss present on her MRI before they see Elizabeth, they expect to see a totally different kid.  It amazes them that she can do all of the things that she does despite a clear deficit in her brain structure and function.  Elizabeth herself is an abundance of blessings in our lives.  She fills us with encouragement and the possibilities with her are really endless.  Despite what our final outcomes or diagnoses may be, she has filled our lives with countless rivers of milk and honey.  We are so eternally, humbly, and forever grateful that we have her as a daily reminder of blessings.  Even though some of the news is bitter, and hard to swallow, and it makes us sad and angry and all things in between, we continue to see that river of blessings overflow.  This river of milk and honey also goes beyond Elizabeth and it includes all of you.  You are all also a river of milk and honey to us.  Your support, encouragement, prayer, following the blog, asking for updates, etc. all mean a great deal to us.  Elizabeth's possibilities are endless because of the support that surrounds her.  Thank you to all of you for helping us along the way and for being a part of our journey.

Just to add a bit of sweetness to your day, here is a picture of Elizabeth's crawling attempts, and there is also a picture of Elizabeth and her cousin, Jade, in their matching outfits at church.