Saturday, June 17, 2017

Quick Recap

I know...I know....I've been a huge slacker on the blog.  I would try to come up with some sort of witty excuse, but let's be real, life has just been busy.  So, let's do a quick recap of the last few months.  


January and February were relatively quiet months for our family.  The biggest thing we dealt with was reoccurring pink eye.  We enjoyed time at home doing the regular day-to-day activities.  We had our family pictures taken as well.








March brought about a fun field trip for Miss Emma.  When her cousins were on spring break, Gramma took all of us to Fossil Rim to see the animals.  We got there bright and early in the morning when all of the animals were hungry.  We all enjoyed the little trip and Emma really liked seeing the animals up close.






April was a more difficult month for Emma.  She woke up one morning with a diaper full of blood.  😬.  That landed us an automatic admission into hotel Children's.  She was diagnosed with c-diff and was in the hospital through Easter.  We made the most of our admission and even fit an egg hunt on Easter.  April also was full of fun hospital events.  The annual Heart Center picnic was at the first of April and CMC's Red Balloon Run and Ride was at the end of April.  Emma met a big milestone and walked with her walker in the fun run.  We were all so very proud of her.


May was another interesting medical for month for Emma.  We had several issues with her Picc line.  Her Picc line that was a year and a half ind cracked, so we had to get it replaced.  The replacement failed and we had to get it replaced again.  Quite the roller coaster ride for a few weeks.  Emma also had an issue with hypernatremia that landed us in the hospital for a weekend.  We did get to do a few fun things including a trip to see Lion King the musical and family time in Ada


June has started with a bang.  Due to the regionalization of healthcare in Texas we are having to move into another county.  We put our house for sale a couple of weeks ago. It is under contract and we are under contract on a house in Collin County.  During the house showing process, we made lots of trips to Sonic while people are looking at our house.  Emma loved getting to feed Max French fries. Needless to say, the next couple of months are going to be busy.  We are excited about our next adventures.  



Thank you for hanging in through this long overdue update.  We continue to press forward with Emma's healthcare.  We monitor her heart failure and make regular adjustments to several medications.  She is a trooper through it all and keeps us on our toes.  


God is good!  All the time!  God is good! L 









Sent from my iPhone

Sent from my iPhone

Tuesday, January 17, 2017

New Year

 
Well, once again, it has been awhile since I have updated the blog.  I could blame a busy couple of months, the holiday season, or maybe just maybe I forgot…I’ll let you guess.  The last couple of months have been filled with many wonderful memories/moments and a month long hospital stay.  The later was quite an event.  
Emma entered the hospital at the end of November with a UTI that caused her to run a very high fever. This landed us in the hospital for IV antibiotics and observation for hydration issues.  Unfortunately, she became very dehydrated due to her high fever, so we had to hold her diuretics which aid in keeping fluid off of her lungs and abdomen.  She did not tolerate the diuretic hold well and needed extra respiratory support as well as a closer eye on her kidney numbers, so we were transferred to the Cardiac ICU.  She remained in the cardiac ICU for a few eventful weeks.  We increased her heart failure meds to try to help her kidney function and increasing abdominal girth; however, her belly kept getting bigger and her kidney numbers were worsening significantly.  Scott and I were beginning to get frustrated.  No one had answers and Emma was getting worse by the day. Her belly got so big that she was having trouble breathing.  We ran countless tests and kept hitting a wall.  It always hurts my mom heart when Emma is hurting and no one can find answers.  Scott and I truly felt we were dealing with fluid in her abdomen even though the tests were not showing it.  Our suspicions were finally confirmed….almost 2 pounds later.  We had a drain inserted into her abdomen and drained almost 2 liters of fluid.  The fluid in her abdomen was fatty in nature, so we have switched her to a fat free formula.  Draining the fluid in combination with the formula seemed to do the trick.  So after four weeks we were discharged home.    

 


Now for the fun news….
We were able to make it home in time for Christmas which was a wonderful surprise.  We actually made it home the day before I graduated from UNT with my PhD.  We got settled in and tried to regain some sort of normalcy after a month in the hospital.  Emma came home on her increased heart failure medication and a new formula, but otherwise was on a pretty similar routine.
  
   
We began our holiday celebrations by having “Friendsmas” with the Lawry family.  This is a tradition we look forward to each year.  Lots of laughs were shared, good food was consumed, and card games were played.   We were able to celebrate Christmas at home with just the three of us…oh and Max.  We had a nice relaxing day together and Emma was very excited about her gift….a musical fan.  Scott and I took turns attending worship Sunday morning and opened gifts Sunday afternoon with Emma.  We took Emma to see Christmas lights that evening (not her favorite activity) and ended the evening singing and dancing to Emma’s new musical fan.  We traveled to Oklahoma on Monday for Christmas with the Estes family.  It was a quick but fun visit.  Emma enjoyed the time with her aunt, uncles, cousins, and Gramma and Grampa.  






After all of the holiday excitement, we have tried to get back into some sort of schedule.  Emma has started back all of her therapies and school.  We go to the cardiologist once a week for PICC line care and labs.  
We are enjoying having a happy girl back in the house.  She is back to working hard in her therapies and learning more every day.  She got a new sanding frame and SMOs to help her strengthen her legs and have more ability crawl around and attempt to stand. One of her new goals is to communicate more effectively with her communication device, so we are using that daily to get her to tell us her needs and wants.  Most of the requests include: I want IPAD, I want French fries, and I want music.

 


Thank you all again for the prayers, support, and encouragement.  We have been greatly blessed by all of the people who send cards, food, take care of our dog, etc. to make sure we have little worries outside of caring for our daughter when she is hospitalized.  We could never thank you enough.
Remember, February is right around the corner which means CHD Awareness Week activities. J  Get ready to wear red.
God is good!  All the Time!  God is good!  

Friday, October 21, 2016

But If Not (words from my heart and birthday pics)

But if not… I recently read a book called  Fight Back with Joy. And in the book, the author describes times in your life when you are asking, praying or even begging God to work a certain way in your life and yet God chooses not to work that way. For example I pray that Emma's heart will heal, and no longer be in heart failure, but if not… how will I respond to that answer?  This statement, and the answer to it has been weighing on my heart lately so I thought I would just take a few moments and write from my heart. 

Scott and I close all of our blog posts with the phrase: God is good. All the time. God is good. We truly believe that this statement (I mean our faithful God) has aided us in many valleys over the past six years.  There have been countless moments that I have felt angry, lonely, depressed, sad, and unsure.  Without the knowledge of Who our God is and how He walks beside us everyday, I have no idea how we would get thru this journey.  Our journey has been filled with countless "but if nots"   It would be easy to feel forgotten or neglected; however, we seen those "but if nots" turned into beautiful blessings (maybe not right away...but in time). “But I will hope continually and will praise you yet more and more.” Psalm 71:14

 I was driving home from a meeting the other day and a song came on the radio that described so many emotions I have felt over the last six years.   In the words of the song, by Hillary Scott, there is a reminder of Who is in control and Who is walking beside us:"Sometimes I got to stop and remember you are God and I am not, so thy will be done."  There have been many instances over the last six years in which I have questioned God's plan.  Why should Emma have to suffer so much?  Why can't she get to experience more normalcy?  Why must she endure heart failure with no options?  Why must she have so many medical/ developmental difficulties?  He tells us in Romans 12:12 to: “Rejoice in hope, be patient in tribulation, be constant in prayer.”  I am to fully trust in his plan...let go of my worry...and rest in the truth that He is in control.  This is easier said than done unfortunately.  This last month has been filled with many unexpected set backs and disappointments.  I have been living in a fairly constant state of exhaustion which allows for doubt and worry to creep in and consume my mind.  Don't get me wrong, I have so much to be thankful for; however, I'm human and disappointment is a very real thing.  We are working on hospital stay 2 in less than a month.  Once, for a nasty respiratory virus and currently for abdominal edema and low diuretic response.  The first hospital stay directly impacted Emma's birthday party and this stay successfully cancelled a much needed family vacation.  

Let's go back to that "but if not.." statement.  I pray that we can soon figure out these latest medical issues of Emma's and go home...but if not, I will continue to trust in God's plan and work to see the good he weaving into our journey.  "Rejoice in the Lord always; again I will say, rejoice. Let your reasonableness[a] be known to everyone. The Lord is at hand; do not be anxious about anything, but in everything by prayer and supplication with thanksgiving let your requests be made known to God. And the peace of God, which surpasses all understanding, will guard your hearts and your minds in Christ Jesus."Philippians 4:4-7 Thank you to each and everyone of you who have encouraged us in this journey and prayed for us during the "but if nots".   God is Good!  All the Time!  God is Good!

Enjoy some fun pictures of Emma from her belated birthday party and current hospital stay.      










Sunday, August 28, 2016

Getting Everyone Caught Up

Wow! It has been a long time since I have updated the blog. I will apologize for being negligent; however, we have truly been enjoying life with Miss Emma and for that I will not apologize. :) There have been some events in her life since my last post that I will discuss and fill you in on the last few months in this blog post.

Emma had a couple of event in June and July that sent her to the hospital for a few days. While Scott and I attended a pharmacy conference in Arizona at the end of May into June, Emma contracted a respiratory illness. When we returned from that event Emma's respiratory virus that progressively worsened. The virus landed us in the cardiac ICU for several days and then on the cardiac floor for a few days with the need for increased respiratory support and meds.  This trip turned out to be a blessing in disguise and I will explain that later.


Emma was also admitted to the intensive care unit in July for cardiac arrhythmia issues. Her heart was beating way too fast which is called atrial tachycardia. It would then go into heart block which would slow it down way too much. So we were in the hospital for a few days to administer a new medication to help her heart return to normal rhythm. 


So back to that blessing in disguise. When Emma was impatient with her respiratory virus, she was put on a steroid to help with her lungs. While she was on that steroid, we noticed that her abdominal ascites was lessened, she had more energy, and her meds worked better. After discharging from the hospital, we conferenced with her cardiologist, pediatrician, and gastroenterologist. Well, they all agreed that none of this makes perfect sense, but we all agreed that it was worth a shot to try a longer-term steroid regimen to see if it would be beneficial. Whether it is pure coincidence or her steroid actually working, since she has been on the new medication, her belly has stayed small, we've lowered her diuretics, she has way more energy, she is wanting to try more things in physical therapy, and her heart failure number has improved. While we are still very new in this experiment, we are excited with the potential possibilities.

So what is our family been up to.  We have truly been living, staying busy, and enjoying Emma's newfound energy. We continue to see the cardiologist weekly to monitor her blood work and maintain her PICC line.  She has continued to see her physical therapist, occupational therapist, and speech therapist over the summer and makes improvements weekly with each of them.

We took a family vacation to Lake Texoma for our annual family reunion. It was extremely hot but Emma enjoyed the parts of being in the air-conditioned building LOL.  She enjoyed being with her family and Scott and I enjoyed getting to do some things we love like fishing, waterskiing, and tubing.

Scott and I took a little celebratory trip with some friends in July. We took a cruise to Key West and the Bahamas. It was a very relaxing time with great friends… We ate a lot of food, laughed a lot, soaked up some sun, rode horses, kayaked, and Segwayed.

We are now back into the swing of things. Emma started school this week which will add to her normal routine of all of her therapy visits.  She is actively getting on her hands and knees and trying to crawl, standing for short periods of time unassisted, spelling words, simple addition and subtraction, reading comprehension, and so much more.  She is loving time with her friends, family, nurses, and of course her mommy and daddy.

I wish I could say that I have a lot more free time now that my dissertation is complete; however, I seem to find ways to fill that up. I will be teaching Wednesday night Bible class with my friend Whitney and I'm still working with Mended Little Hearts of Dallas.  I am; however, enjoying being more involved in Emma's daily schedule.  Scott is staying busy with work as well as volunteering in several areas at our congregation.  

Thank you again for being patient with us as we strive to live our normal and enjoy every moment with our sassy girl.

God is Good!  All the Time!  God is Good!

Memorial Day muddin'

All smiles at her dr appt
Respiratory illness=no fun
4th of July with Nana Jana
Family fun.

Reunion fun 
Cruisin'
First day of school

Sweet kiddos at school
Cardio checkup 

Sunday, April 10, 2016

The missing puzzle piece


For the past five years it has been overwhelmingly obvious that there is a missing puzzle piece in Emma's medical diagnosis.  A few months ago we submitted a whole genome sequencing for genetic testing. This included my blood, Scott's blood, and Emma's blood.  Last week I received a call from the genetics department at children's medical center in Dallas. The results were back from Emma's genetic testing and the results were that Emma has an abnormality on arid 1B gene.  This syndrome that is associated with this abnormality is called Coffin-Siris syndrome.  When the genetics doctor was reading the list of issues associated with this syndrome it was like she was describing Emma.  Here's where it gets even more intriguing, according to the National Institute of Health, there have only been approximately 140 cases of this syndrome. That's right, Emma is one of approximately 140 people to ever be diagnosed with the syndrome.  

Here is a brief synopsis of medical issues typically associated with CSS:

Signs & Symptoms

CSS is characterized by distinctive abnormalities of the head and facial (craniofacial) region with affected individuals often described as having coarse facial features that become more prominent with age. Affected individuals may have an unusually small head (microcephaly); a wide mouth with full, prominent lips; a broad nasal tip; a low nasal bridge; and an abnormally long vertical groove between the nose and the upper lip (philtrum). Additional features may include thick eyebrows, long eyelashes, and generalized excessive hair growth (hypertrichosis) with the exception of the scalp hair, which tends to be relatively sparse (scalp hypotrichosis). Reports suggest that sparse scalp hair improves with age.

Individuals with CSS also have characteristic skeletal abnormalities. For example, certain fingers and toes (digits), particularly the fifth fingers (“pinkies”) and toes, may be unusually short due to absence or underdevelopment (hypoplasia) of the end bones (terminal phalanges) within these digits. The fingernails and toenails may also be underdeveloped or absent. Additional abnormalities may include dislocation of the inner forearm bone (radius) at the elbow, deformity of the hip (coxa valga), or unusually small or absent knee caps (patellae).

Early in life, infants with CSS typically experience feeding difficulties, vomiting, slow growth and weight gain (failure to thrive) which may have begun while the infant was still in the womb (intrauterine growth retardation), and frequent respiratory infections. In addition, affected infants and children may have hypotonia, abnormally loose joints, delayed bone age (2 to 3 years behind the chronological age), and mild to severe intellectual disability. Affected infants and children may also have mild to severe speech delays, where expressive language is affected more severely than receptive language, as well as moderate to severe delays in motor skills such as sitting and walking. Reports suggest that on average, affected children learn to sit up at 12 months (typically occurs at 6 to 8 months), walk at 30 months (typically occurs at 9 to 18 months), and speak at 24 months (typically begins around 12 months).

Affected individuals may also have eye (opthamologic) abnormalities. This can include drooping of the upper eyelid (ptosis), clouding of the lens of the eye (cataracts), and misalignment of the eyes (strabismus, commonly known as “lazy eye”).

CSS has been reported to manifest kidney (renal) or genitourinary abnormalities in some affected individuals. There have been reports of affected individuals with fused kidneys at the lower end (horseshoe kidney) and the urethra – .

Individuals with CSS may also have gastric abnormalities which may include one portion of the bowel sliding into the next like a telescope (intussusception) or an opening in the diaphragm allowing abdominal organs to push up into the chest cavity (diaphragmatic hernia).

Less commonly, affected individuals may have additional physical abnormalities, such as choanal atresia, a malformation in which a bony or thin layer of tissue blocks the passageway between the nose and throat, leading to breathing difficulties. Some individuals with CSS may also have heart abnormalities at birth. In addition, a brain abnormality known as Dandy-Walker malformation has been reported in some cases. This condition is characterized by cystic malformation and expansion of one of the cavities in the brain (fourth ventricle). Dandy-Walker malformation is usually associated with an abnormal accumulation of cerebrospinal fluid (CSF) in the skull (hydrocephalus), resulting in increased fluid pressure, a rapid increase in head size, abnormal prominence of the back region of the head (occiput), and/or other associated findings. Some individuals with CSS may also have partial or complete absence of the band of nerve fibers that joins the two hemispheres of the brain (agenesis of the corpus callosum) and fewer folds in their brain (gyral simplification). Some affected individuals may also experience hearing loss, seizures and tics. There have been reports of liver cancer (hepatoblastoma) in affected individuals, but the link between CSS and tumor risk needs to be further investigated.

                           (National Institute of Health)

When I received the news from our genetics team, I felt this overwhelming since of relief....this doesn't change Emma's prognosis, but perhaps it will help us better understand how to better care for her.  I'm honestly glad we didn't know five years ago. I'm not sure what the medical team would've done or not done.  Since this is such a rare genetic abnormality, little is known which makes it difficult. 

You see, what's hard is that we have had phenomenal care over the past six years.  Surgeons, doctors, nurses, respiratory therapists, and other medical professionals have worked tirelessly to try to figure Emma out, scratched their heads at her body's responses to surgeries, meds, procedures, etc.  They have consulted other doctors, experimented on her, went against the norms, etc.  It has been frustrating for both them and Scott and I to watch her struggle and reject what should work.  Maybe, just maybe we were/are fighting something that is fighting harder back, creating obstacles that haven't been figured out yet, and adding extra stress on Emma's body.  

I don't want Emma to be one of 140 people in the world; I don't want every aspect of Emma's life to be so incredibly hard; I don't want her to stump all the doctors, I don't want her to not have any more surgical/transplant options; however, that is our life....our obstacles.....our blessings in disguise.

Emma will not be defined by statistics....by what ifs.....You see, Emma knows no different.  She is surrounded by family and friends who love her for WHO she is.  We have never underestimated her...more importantly, we have never underestimate God.  Our God is bigger...no matter the Earthly outcome.  We know.   Our faith gets us up in morning to continue living, loving, and being a light for HIM! 

Thank you for the continued prayers, love, and support.  Enjoy some pics of our sweet sassy girl loving life!


God is good!  All the time!  God is good!

Sarah 

Weekly cardio visit 

Wagon fun

Uncle Caleb is silly 

Easter Egg Hunt with the Cousins

Fun with PaPa and Nana
Fun with the Estes crew
Easter Beauty
Best Buds

Heart Center Picnic Fun

Family Fun



Family Pics by Rustic Images

Cousin Rhett being a big helper!