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.  "⁴ Rejoice in the Lord always; again I will say, rejoice. ⁵ Let your reasonableness^[a] be known to everyone. The Lord is at hand; ⁶ do not be anxious about anything, but in everything by prayer and supplication with thanksgiving let your requests be made known to God. ⁷ 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.      

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 

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! 

CHD Awareness Photo Challenge Days 1-15

 I have not shared anything on the blog for CHD awareness week like I normally do.  However, I wanted to share a challenge that I'm participating in during February.  Each day has a different theme that deals with an aspect of congenital heart defect awareness. I have included days 1 through 15 in this blog.  Thank you again for always supporting our sweet little heart hero. This is a special time for us and we work diligently to try to educate others on what it is like to live with a CHD.

CHD awareness month is here, so I will be participating in a daily awareness photo.  Day 1: diagnosis....Emma was diagnosed at 19 weeks prenatal with a severe congenital heart defect.  We would learn a few weeks later that the defect was Hypoplastic Left Heart Syndrome with intact atrial septum. I encourage all expectant mothers to ask about the heart during their anatomical ultrasound.  There are over 40 types of CHDs. Early diagnosis helps to save lives. ❤️

CHD Awareness Month Photo Challenge Day 2: Surgeries/Meds/Interventions: These five photos represent the five open heart surgeries Emma has fought through. #chdwarrior #mendedlittlehearts #chdawareness #RockYourScar #HLHS

CHD Photo Challenge Day 3: Family: We are truly blessed with supportive Family and friends who are like family. Scott and I truly do not know what we would do without their encouragement, love, and unwavering support.

Day 4: CHD awareness photo challenge: Hospital: Emma has been treated at both Children's Medical Center (Children's Health) in Dallas and Boston Children's Hospital in Boston.  The hospitals varied greatly in their structure and organization; however, what was not different was the care, love, and heart put into taking care of our precious Emma.  #chdawareness #cmc #bch

CHD Awareness Month Photo Challenge Day 5: Cardiologist:  Dr. Lemler has literally been with us since before Emma was born.  He has been with us during extremely hard decisions, sat at the hospital with us in the middle of the night during our hardest moments, celebrated with us during unexpected successes, come to our rescue more than once during inpatient stays and ER visits, answered panicked phone calls, and supported us throughout all of Emma's ups and downs.  We give each other a hard time, but we are honored to have him manage Emma's care. #chdaware #HLHS #cardiologist

CHD Awareness Photo Day 6: Hope: I could go on and on about hope, but I will just leave this here. We continue to hold on to hope and trust in God's plan for our tough little girl who was born with half a heart. #CHDaware #HLHS

CHD Awareness Photo Day 7: Red and Blue: What does red and blue mean to me? The diagram on the left shows Emma's heart defect at the different stages of palliation. Notice how the red (or oxygenated blood) and blue (unoxygenated blood) mix.  This causes Emma's  lower oxygen saturation levels which makes her appear blue. Red and blue are also the colors we use to represent congenital heart defect awareness. #chdaware #HLHS #hearthero

CHD Awareness Photo Day 8: CHD Facts: My Emma is 1 in 110....Are you CHD aware?  Every 15 mins a child is born with a CHD.  #chdaware #HLHS

CHD Awareness Photo Day 9: Awareness:  I am a coordinator for a group called Mended Little Hearts of Dallas.  I do this to share hope, education, support, and awareness.  Emma is the reason I am so passionate about the work I do.  #chdaware #HLHS #mendedlittlehearts #RockYourScar

CHD Awareness Week Day 10:  #RememberOurHearts #CHDaware

CHD Awareness Photo Day 11: Favorite Quotes!  I have so many, but here are a couple. ❤️❤️❤️

CHD Photo Challenge Day 12: What I wish people knew: I wish people knew that CHDs impact more of a child's life than simply their heart. Due to Emma's CHD, she is challenged every day with developmental delays and other organ issues.  Also, I get asked frequently if Emma's heart is fixed. There is not a cure for her CHD.

CHD Awareness Photo Day 13: Heart Family: Being the parents of a child with a congenital heart defect can feel very lonely; however, meeting many other heart families along the way, whether in person or through social media, living next-door to them in the hospital or meeting them at a heart event gives you someone to relate to, vent to, cry with, and celebrate with. #CHDaware #heartfamily #mlh

CHD Photo Challenge Day 14: Heart: When most people think of Valentine's Day , they think of hearts and love.  Don't get me wrong, the 14th of February makes me think of love and hearts and red and pink; however, since 10/4/10 when I think of hearts, I think of my half a heart hero.  Her heart is shown in the top picture.

CHD Photo Awareness Day 15: Nurses:  we have been very blessed with caring, loving, and attentive nurses from the beginning. From our nurses at CMC to our nurses at Boston and home, Emma has been surrounded by amazing advocates that aid in her care.  I don't have pictures of everyone, but please know that we are so thankful for all of her nurses who worked tirelessly to help Emma get to where she is today.

Emma is doing well. She is very busy in her therapies and school. We continue to travel to the cardiologist every week for her PICC line care and lab monitoring.  Thank you for your continued prayers and thoughts.

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