Sleep Study Results

Ha ha.. So yeah I have the interpretation from the neurologist on Arianna's sleep study but the pulmonologist that reads them and than calls us...is out of town till next week. So I get this new diagnosis and can't really tell you much of what that means as far as treatment. But I can explain it to you a little.

Arianna has Central Sleep Apnea, which is a disorder where your breathing repeatedly stops and starts during sleep. She had 19 central sleep apnea events during her study. Central sleep apnea occurs because your brain doesn't send proper signals to the muscles that control breathing.This is less common than Obstructive Sleep Apnea, which most people hear about. But that's our girl...always has to be a little different thanks to that missing piece of chromosome.

So the typical treatments from what I've read is using a device to assist breathing such as CPAP or supplemental oxygen.

They also noted she had increased end tidal CO2 levels. Which basically is the measurement of how much carbon dioxide she breathes out. I'm honestly not sure if CPAP would help that or not. Again..we have to wait and see what the doctor says.

The notes mentioned she had 19 accounts of respiratory effort related arousals (RERA). Often times it is hard to differentiate between RERA and obstructive sleep apnea (OSA). For people with OSA, breathing stops on a number of occasions during the course of an hour due to a partial or complete obstruction of the sufferer's airway. People with RERA labor to breathe due to RESISTANCE and hence this disease is also known as upper airway resistance syndrome (UARS).

The nurse called to tell me about the possibility of an upper airway resistance so we started Arianna on Flonase tonight, which is a steroidal nasal spray.

She did have 3 events where she was significantly under breathing.

So the conclusion was noted that she has decreased sleep efficiency with decreased REM sleep as well as increased awakenings and arousals at night. Plus, significant elevations of end tidal CO2.

So we finally have some answers...now we just wait for the direction on how to help her. I wont lie..it's been a little difficult last night and tonight putting her to sleep and not worry about her breathing. Several times in her life I have gone up to her and she was NOT breathing and I freaked out!! I literally thought she died in her sleep. I yell and shake her and she wakes up looking at me like, "WHAT THE HECK MOM!" But at least now I know why this has happened.

Thank you God for always watching over her and protecting her...now I specifically pray for this protection while she sleeps. 

Cardiology Update - August 2014

Arianna's cardiology check up went AMAZING! Not only did we get to see our favorite cardiologists but also our favorite echo technician! smile emoticon

This may be more info but for my heart mama's I'm gong to throw it out there. Arianna right ventricle muscle has decreased and is normal size!!! Praise God!! This is what we have been waiting to hear since surgery. I wanted to cry I was so happy. Her cardio also said her pulmonary valve conduit looks absolutely beautiful. Even said the best repair he has seen. Just a trace amount of regurg which is completely normal to have. But the function is perfect! He mentioned something new to me..I guess they measure the tricuspid valve annulus as another indicator. Arianna's is on the higher end of normal. So that is good..but a new piece of information for me to be aware of when getting future echos.

Speaking of future echos and appointments..the cardiologist was SO impressed with her results today he gave us the 1 YEAR clearance!!! FIRST TIME EVER!! We have been able to go 3 months and 6 months but never a full year! God is SOOO good!!! He told her to enjoy her life..do all the things she wants to do and that we shall!

Quick little update: She hit the 50 pounds mark!!! WOOHOO!!! She has been at 46lbs for 3 years so we are VERY happy about the weight gain. smile emoticon

On a side note: We signed up for a research study at the UAMC for children with CHDs and sleep apnea. We will be doing a home sleep study this weekend to do our part in the participation. Arianna has never been good at sleeping so we have been curious about this anyways. This will be a good way to get some information and help research! If she doesn't pass than we can go for an inpatient sleep study to look into things further. But I asked Arianna if she wanted to participate in the study and she said yes! So we go Friday to do some cognitive testing and get our equipment for the weekend.

Scoliosis Update - July 2014

The previous scoliosis appointment was not great so we were going in hoping for the best but kind of expeccting to hear the worst.

But GOD IS GOOD!!! Her curve is down DRAMATICALLY!! So much so that the doctor double checked to make sure he had the right persons xray up.

This is the 2nd time God has worked on her back and helped straighten her out! ;-) We are now down to once a year visits. Looks like I should call and get that appointment scheduled in a few months.

I'm BAAAAACK

Every time I wanted to start blogging again I would get overwhelmed with how much I need to update. So you get the really quick recapped version. But I'm sure I will go back for specific situations and do a post dedicated to that topic. But here goes...

In 2013 I began my journey into Nursing School at Grand Canyon University!

I worked VERY hard for 16 months and earned my bacheloretts degree!

Arianna began 2nd grade at Academy of Tucson and did very well with her health until January.

This was such a special day...her FIRST picture day!!!

Oh but can't forget we went on our Make A Wish trip in December. I will do a dedicated post just for that trip ;)

Arianna got extremely sick that landed her in the ER in respiratory distress and than an admission into PICU.

The VEST to help open airways

 She had double pneumonia, pleural effusion, bronchiolitis, and asthma exacerbation. She spent 5 days in PICU and than stayed home for several more weeks. She was out of school for total of 7 weeks..last 2 was simply because her class was still SO sick. But she is finally back in school...caught up and hitting Honor Roll AGAIN!! That is all 3 quarters with NO modifications needed!

She just had a sleep study done last week and still waiting the results.

Cardiac wise she finally was given the BIG thumbs up and said her RV diameter has shrunk which means the pulmonary valve conduit was able to reserve some of the dilation! In Aug 2014 we were given the okay to go a FULL YEAR until our next appointment. First time we've been able to space them out.

We do have some concern about her palate. After doing some research and talking to other 22q moms I believe she may have a sub mucous cleft palate. We have actually been in contact with CHOPs 22q and You center and hoping to get everything set up to go this summer.

Devin is doing really well. He is getting ready to start Kindergarten in a few months. I can't believe how BIG my boy is getting! He did his kinder testing and he is right on track and we are excited to have him join his sister at the AOT!

Nov 28th: Scoliosis Clinic

Today we took Arianna to see Dr. Vincent for her scoliosis check up. For those that dont know her history. She was born with a butterfly vertebra and missing one of her lumbar vertebras. She had an MRI of her spine and brain around 18 months which showed a slight curve which lead us to the scoliosis docs.

We were seeing one doc for a few years. Each time we went in the curve was getting worse until one visit he said it had decreased! Praise God! She had issues with her feet, needed shoe inserts and been told she has very loose ligaments which caused some delays and issues as a younger child. We tackled each issue with therapy and she overcame every one.

Last year her x-rays showed her curve had decreased again and we rejoiced for the good work God was doing in our daughter! Even our pediatrician did a physical and said her curve looks as if its not there. So when we went for her appointment today we were expecting to get the same news....well that did not happen.

The orthopedic doctor did his physical assessment and seemed to be very happy. He checked her leg lengths and said it all looks great. Whatever kind of curve she has is strickly from her spine. Then he proceeded to pull up her x-ray and we were all shocked! You can see a huge "S" curve on the screen. Now this isn't completely new to me. Everytime shes had xrays this year for pneumonia we (Robert & Myself) noticed her curve looked worse but we didn't think about it and continued to thank God for her good news. What shocked the doctor is that at this degree of curvature there should be asymmetry of her ribs during a physical and she doesn't have none, hence why the pediatrician said its seemed to be resolved. He can't give me an answer for why she doesn't show this in her ribs but the x-rays don't lie and her curve is worse.

The doc measured her curves and her upper curve has gone from 13 degrees to 25 degrees in one year. I honetly can't remember her lower curve but its about the same progression.

So...this lead us to a lengthy conversion about what to do from here and what the future looks like for Arianna. Clinically, she has advanced from mild scoliosis to moderate scoliosis in one year. The likelihood of her curve getting worse is high. We just won't know to what degree.

I asked all the "what if's" and got alot of numbers. Basically, the bigger the curve the higher the chance of reoccuring issues with scolisis throughout adulthood. Since Arianna is only 6yrs old and has a significant amount of growing to do this increases her risk.

But what do we do right now? Well the doctor needs to keep a close eye on her at this point. I asked if its possible she doesnt progress any further and he said since shes already at this point its highly unlikely. He wants to see her back in 6 months to see the progression at that point. If she is around 40 degrees then we will have to talk about bracing and if its more than that then we need to talk surgical intervention.

Now let me explain the bracing issue. Typically children do not go in braces until they hit puberty because that is when the advanced growing takes place. If we are to put Arianna in a brace at 6 years old she will be wearing it for 8 YEARS!

Now if her curve gets to about 60 degress then we have no choice but to place rods in her back. A HUGE fear of mine and something I honestly can't even think about it right now.

So many people say how strong I am and how they could never handle all I go through with my daughter. Let me just take this moment to NOT be strong and whine for a second. I HATE DiGeorge Syndrome, 22q11.2 Deletion Sydrome, Velocardiofacial Syndrome, WHATEVER YOU WANT TO CALL IT!!! The unknowns, the unpredictability, the second guessing, the surgeries, the fear, the pain, etc. etc.... I handle all of this because honestly I have no choice. This is my daughter...my precious baby girl who was given to ME! I have to be the BEST mother I can for her. She needs me. I'm not the one physically going through all of this..she is and it breaks my heart. She is the one that is strong and gives me the strength I need to wipe the tears from my face and tell her it's going to be okay. To stand by her side and be her personal cheerleader!

But we are not alone. We have God on our side and when things like this arise we stand together as a family with all our fears and worries and place it in His hands. He is the only one that can get us through this. We have laid hands on her and asked God to once again touch her miraculously and stop this progression of curvature...and reverse it!

In the flesh you can easily be swayed by the things of this world. I've seen the x-rays and got all the information from the doctors. I know what the world has to say about this but I also have a God bigger than all of this. I know...you may say.."Vanessa you've said this before about her heart." Yes, I have and I know now that God's plan for her was to have her heart fixed by man and receive her healing that way. I've been able to grow my faith in this area as I've studied the bible and spent quit time alone with God. A few year ago I couldn't see that. I know I can petition things to God and I will no matter what arises but I also know that God has a plan for her. Some things she is healed from in the super natural and some things she is healed from in the form of surgeries and the guiding of a human hand. Whatever direction we head with Arianna regarding her scoliosis I know that God will be there every step of the way and I put my faith in Him.

Does it hurt emotionally...YES..I'm human and no one wants to see their child suffer in any way. However, I know I have to remember God can see the BIG picture. I know in my heart she will touch many lives and lead many to the Lord. She has already brought me back to God and built my faith. I will not sit here and get mad at Him for allowing her to go through these challenges because I can find comfort in knowing she was put on this earth for a GREAT purpose and she will fulfill all that she is suppose to. This is our journey and we walk it with her...showing her how to trust in the Lord in all circumstances.

November 14th

Arianna's Pediatric Cardiothoracic Surgical Team: Dr. Thompson (Left), Dr. Teodori (Back right), & Richard Burt, P.A. (Front) 

Just one day shy of 4 weeks pos-op we went to see the surgical team to make sure everything was going well. A week before we saw the cardiologist and had a follow-up echo. So far everything looks PERFECT!

They kindly reminded us these valves don't last forever. Dr. Teodori expects about 10 years of out of this valve but there are many factors to consider. It could be sooner than that. Either way we do what we've always done. Go to cardiology appointments every 6 months and monitor her from here. When the time comes again we will replace her pulmonary valve again.

Weekend Update

We took Arianna to a photo shoot to have her picture displayed at Diamond Children's Medical Center! She will be either on D5 or D6! We are so grateful for the new medical facility for our children.

Went to visit Gracie! Gracie wanted Arianna to sit on her couch with her and Arianna didn't hesitate for one second!

Sunday Arianna woke up complaining her chest hurt and just looked tired. I think she's been trying to do too much physically lately and it took its toll on her. I had her stay home from church and rest. I'm going back to a regular schedule on her Tylenol to help prevent the aches and pains at this point. She is doing amazing otherwise!