ZERO

Another amazing ZERO rejection biopsy! Looks like Gabriella bounced back with ferocity this time! Her pressures were back to normal and labs in picture perfect range. I will get further details tomorrow at clinic, but there isn't much that could squash our joy!!

(I apologize for not posting sooner. I did an email update but it never posted and got lost in cyberspace!)

Do not worry...

I love how one of my favorite sections of the bible was todays devotional.... enjoy!

"Therefore I tell you, do not worry about your life, what you will eat or drink; or about your body, what you will wear. Is not life more than food, and the body more than clothes? Look at the birds of the air; they do not sow or reap or store away in barns, and yet your heavenly Father feeds them. Are you not much more valuable than they? Can any one of you by worrying add a single hour to your life?"

And why do you worry about clothes? See how the flowers of the field grow. They do not labor or spin. Yet I tell you that not even Solomon in all his splendor was dressed like one of these. If that is how God clothes the grass of the field, which is here today and tomorrow is thrown into the fire, will he not much more clothe you—you of little faith?

So do not worry, saying, 'What shall we eat?' or 'What shall we drink?' or 'What shall we wear?' For the pagans run after all these things, and your heavenly Father knows that you need them. But seek first his kingdom and his righteousness, and all these things will be given to you as well.

Therefore do not worry about tomorrow, for tomorrow will worry about itself. Each day has enough trouble of its own."

http://bible.us/Matt6.25.NIV

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Test day.... success.

That was mostly painless. Aside from the general emotional and spiritual struggles I am flooded with when it comes time for Gabriella to have procedures or tests done, today was fairly easy.

We got her checked in, into her room, evaluated, IV started, doctors met and Gabriella rolled into the cath lab in the most timely manner I have ever seen at a hospital!

She opted for the IV over face mask for sedation because she doesn't like the way the mask smells. The nurses were surprised but also impressed she is so knowledgeable and aware of her medical care! She went back crying and anxious, but understanding. It took maybe 90 minutes to complete before the doctor came out and told us that things looked great from what he could see! Those high heart pressures she had in June were back into a picture perfect range and he had no issues with valves while going in for the tissue samples for biopsy. We won't know for a few days what the biopsy results say, but the initial feedback was great!

The best part of the whole process is that they chose to go into her right heart this time, entering through her carotid artery, instead of her femoral in her leg. This meant she didn't have to lay still for 6 hours after the procedure!!!!! I was thankful for this, especially since she had such a miserable time coming out of anesthesia this time. Poor kid was angry, disoriented and frustrated for quite a while!

I couldn't be more at peace on a test day like today. I feel like God has His mighty hands so securely around us all that there is no other feeling to feel than comfort. Even in my nerves, my earthly nerves of a mother concerned for her childs life, I have unwavering faith. Gabriella continues to keep strength when I can honestly say I wouldn't be able to if I were in her shoes. Our marriage took a hit recently but has bounced back with such force that I would say we have never been more in tact and strong. As a family unit, we are moving forward, growing and strengthening.

I feel blessed to be alive.

Cardiac Cath/Biopsy Tomorrow

I realize how bad I have been in neglecting my blog. Im so sorry about that! Lots of busy days have filled our last few weeks!

Tomorrow morning Gabriella will have her cath/biopsy at Phoenix Childrens. This will be the main indicator regarding her recent rejection as to whether we fully battled it or if she still has some low level of rejection lingering. It also will tell us how her pressures are in her heart. In June during her rejection, her heart pressures were more than double what they should have been. Her function will take a while to get back, if it does, to normal... but its vital to that recovery that her pressures be close to normal!

They will put her under and perform the procedure, then we will spend the whole day laying low as she recovers the procedure and allows the site to clot. Its a long day for her and we will have PJ with us to boot....

I'll post tomorrow how she does and hopefully a big fat ZERO rejection report by Wednesday. :)

8 weeks and counting!

What happens in 8 weeks???????

MAKE-A-WISH!!!

Brief recap, in August 2009, Gabriella wished for her Make a Wish trip. She had been telling us she wanted to go on the Disney boat (thank you Travel Channel...lol), but she was far too unstable to be out to sea at the time so we had to veer her away from that idea. Make a Wish planned a beautiful week long trip to Disney World instead, since Gabriella also said she wanted to go to Cinderellas castle! Of course, as most of you know, Gabriella suffered a massive stroke 2 weeks before going on her trip. The recovery from stroke, waiting for transplant, transplant and recovery, and adjustment time after there led us to January 2011 when she was finally deemed safe for travels!

When her Wish Granters came back to talk to Gabriella in February this year, almost exactly 18 months after her first visit, she boldly declared she wanted to go on the Disney boat! Make a Wish has put together a lovely 5 day, 4 night cruise out of Florida on the Disney Dream. We leave in a couple of months to enjoy this opportunity of a lifetime! One day of travel, 5 days on a boat and the plan was to fly home that night after we dock.

Gabriella picked 2 excursions from a list about 4 pages long! When we dock at Castaway Cay, Paul & his princess will be in thigh high waters alongside the ocean feeling and learning about sting rays. Gabriella LOVES sting rays... I think it is their velvety feel and how they flow like satin. Then when we dock at Nassau, she chose to do the 6 hour long dolphin encounter where we will have a lunch by a dolphin tank, go through the day to day life of a dolphin trainer and best of all, SWIM with dolphins! All 4 of us get to do this excursion and I don't know which one of us is more excited!!!!

Paul and I have put in SO much of our energy into making Gabriella feel like any other kid, and it shows in her attitude. She never acts like she is different or lacking in anything, because we absorb any of that for her and discipline her just the same. Her doctors, therapies and meds are just a part of her and we move on quickly from whatever we have to do that day medically to put more emphasis on our "normal" portions of life. We have seized opportunities to make memories and been able to give both of the kids more than we believed possible, because tomorrow is never guaranteed for anyone, but especially a medically fragile child. This Make-a-Wish trip is something monumental in that process for us, and I know that is what Make-a-Wish strives for. Something absolutely unattainable for a family like ours. An opportunity out of the ordinary for ALL of us in a positive way, not just out of the ordinary because we are a family with a "sick kid" on a lifetime medical journey. It is an escape, a memory to top all others and a way to "reward" my warrior princess and patient prince for the life they have been pushed into but embraced so well. I can only believe this is what all families feel about Make-a-Wish and that is why it has become such a passionate charity of mine, to which we try and frequently take donations of new toys and activities to. PLEASE contact your local chapter to see how you can help!!!!

Anyways....

Of course, you all have gotten to know us so well, that you know we would sell our souls to the devil for our kids. We encourage them to dream big... because nothing is impossible! In that, we asked Make-a-Wish to send us home from our cruise a few days later than planned, extending our trip at our own expense, and taking the kids to a place we will probably not be able to go to again in the near or distant future.... Disney WORLD! This means we will be responsible for hotel (that was ever so generously provided to us by a friend who has access to the good stuff), Disney World tickets and food for those extra few days. In the grand scheme, this is so minimal. The blessing of our flight already existing saves us that much money on a typical Disney World trip, and the intensity of the week already planned by Make a Wish already filling our cup of excitement, that we see food and tickets as something achievable and sugar on top of this wish.

We have been saving up for it for a couple months now, and I have been trying to do side jobs and sewing to make money. My side job has been SLOW but I am focusing my energy on sewing until my fingers bleed between now and then because it is something I can do any hour of the day without committing my time and risking not fulfilling due to an unexpected appointment or issue. I have rarely used this blog to promote my web ventures, but I think this is a fitting opportunity to do so since all the money will be used towards her trip extension! I have 2 shops online, one that sells my excess fabrics and one that sells my creations. I make custom tutu's, nursing covers, burp cloths, cloth wipes, diaper/wipe cases, etc. and am working on a batch of frilly flip flops that will be sold at a local hair salon! Here are the links to my shop if you would like to check them out:

Fabric Shop: http://www.etsy.com/shop/gabriellasheart
Sewing Shop: http://www.MaggieMooDesigns.com (past items sold can be seen here: SOLD ORDERS)

And as always, Gabriella's donation account through PayPal remains open and accessible from the right sidebar under 'How to Donate to Gabriella' (and PO Box mailing address under the Contact link above). I just ask that if you wish for any donations through there to be used for her trip, you put that in the donation notes so it can be expensed properly & honestly. This is very important!

I can't keep a full time job with Gabriella's demands, so this is my job. Making side money, selling my creations, and being the best mom possible to my little boogers! I thank you for sharing the links and spreading the word! I always believe it never hurts to put the need out there, because how else does God lead people to fill needs if they do not know they exist!!?

Homebound, Homeschool, Charter, SpEd, oh my

Here it is again, the topic of school. Me, the mom who would rather homeschool for the first few years of education, and the battle of the pressure to send her to school. It is a constant stress in my heart.

I searched, interviewed and toured charter schools in April and May to prepare for Gabriella's 1st grade year this fall, fully knowing I was not going to put her in a general public school after what we saw and experienced for Kinder and those overworked teachers. I finally found a brand new school that I felt comfortable with, a place that FINALLY seemed like I could handle sending her to school. They have a dedicated speech therapist ON STAFF and a crazy strong focus on the students physical and mental well being. We got my godson into it and my sister in law even got a job there! Things were looking forward for Gabriella's ability to flow into a school setting and me be comfortable with it.

Then I got a call from the special ed department of the new school while Gabriella was in the hospital last month. Long story short, he proceeded to tell me that because another district wrote her IEP and she was on homebound status for Kinder, she would need to remain homebound through them until they were in a position to reevaluate her again and take her off homebound, then they would transition her slowly into a classroom setting. I was heartbroken, mainly because I had convinced myself this was what was happening, I was sending my princess off to school with kids and germs and self-sufficiency. I got my head wrapped around it, got her excited for it (even through her apprehensiveness) and it was going to happen. Then I was told to just erase my plan, it was all changing. That was a lot of work on my heart to get where I was and I did not take the news well.

Then it dawned on me, she was in rejection and given those hard core meds to stop it, which also meant knocking out her immunity again. Not only was she educationally and socially being held out of starting 1st grade, but now it was also medically necessary. I spoke to our transplant coordinator and she confirmed what I was thinking, and said it could be a few months into the year before her bloodwork shows us she is back at a level thats safe to be in school like that.

Great.

So now we are going to be starting 1st grade, yet again as a homebound student. We will be assigned a teacher, and after a couple months of homebound (medically permitting) she will start mornings only with me going with her, slowly weaning her (or me) into full time school. I don't know about you, but to me this is sounding like she will not be full time 1st grade until January or so.

I am struggling, yet again. Homebound, again. I feel even more now like she would be getting a better quality education if I enrolled her in the virtual home charter academy I have heard such great things about. Committed wholly to home school, local co-op field trips and groups, and the flexibility to meet all doctors, therapies AND school at our time schedule. Instead, we will spend another half of a school year in limbo... which doesn't excite me. Gabriella needs a structure, to be challenged and to be busy... and I feel I can have better control over that if I just commit to homeschooling.

I need to pray about this some more and try and eliminate from my pleas to God what MY plan is. School has been something of a struggle for me from the moment Gabriella was diagnosed, and I wish I had clarity towards one decision or another. Could you drop a line to God on my behalf? See if He tells you anything? :)

Super resilient super girl

The last couple weeks have been pretty steady! Gabriella got her PICC line removed July 1 once her levels hit a more comfortable area, which meant we were able to go to my sisters house and SWIM on July 4! We have spent most of our time inside or at doctors, but have slightly ventured out to a few places with a mask on.

Gabriellas ECHO has been looking steady, and we have been able to increase her Lisinopril to aid in the recovery of the damage her rejection caused. Her bloodwork her been hovering around target ranges as well. We couldn't be more thankful for her recovery! Gabriella is now on weekly clinics again, pretty much up until her Make-a-Wish trip in September, to watch for any slight changes in her function. We will be doing blood work weekly as well, so I am sitting in a very comfortable spot as her mom knowing we are closely monitoring her. We also condensed her medication times and now we give meds 4 times a day verses 7.

No major medical updates besides these. It is just time now, giving her heart ample time to heal and recover. I have some more updates in the coming days on her school year & Make a Wish trip!

God Bless and thank you all for the continued prayers!

Final Genetics Results

In February, I posted preliminary results of Gaberiella's genetic testing. The full blog is here: http://listeningthruthenoise.blogspot.com/2011/02/genetic-testing-results.html but here is the most important excerpt:

"I got a call today from the genetics doctor at Loma Linda. Everything is still in the very early stages of gathering some answers, but I will share a few things here. Gabriella did have a change in the gene associated with cardiomyopathy, meaning she was born with the mutation and it was not anything that happened during my pregnancy that caused her condition. That was a slight relief, because I naturally had little bits of me that wondered. The issue with this gene, which I will call the T gene because the code names won't mean anything to all of you, is that it is the type of mutation that shows in adult onset cardiomyopathy, in ones 20's or 30's. So why did Gabriella fall into heart failure at 4 years old? This take us to a second gene mutation they found, we will call the M gene. On file, this combination of mutations has not ever been recorded in a case of cardiomyopathy. Given that they have not seen this combined, there are no statistics or information available. The genetics doctor says this causes her to consider that the M gene mutation caused the T gene mutation to progress, causing Gabriella to fall into a condition she shouldn't have experienced until later in life and at a much less severe level. This M gene, in my understanding, is like an instigator that made the T gene issues bigger than they needed to be."

And right before this recent stent in the hospital, I got our follow up results!!!!! We were scheduled to go in to genetics at Loma Linda, but this was cancelled due to her hospitalization. We will have to reschedule, but the genetic counselor at the genetics lab spoke to me in detail and explained the results.

Neither Paul, PJ nor myself carry the disease causing gene linked with HCM, mentioned above as the T gene. This means it was a "spontaneous" mutation (per the geneticist) or in  my words, the way God carefully created her. Paul and PJ both carry the M gene, also called a variant gene. But without a disease causing gene for the variant gene to modify, it is a harmless gene.

This means so much. It means we do not have to worry about PJ having this same condition. It means we do not have to worry about this being a genetic condition passed through family lines to our 11 nieces and nephews or hundreds of cousins. It means that if we do choose to have more children one day, the risk of having another CHD baby is that of any other parent, 1 in 100, with only the added risk of the modifier gene being passed down.

I always say to everyone that God gave us Gabriella for a reason. Her story is to be shared as a physical reflection of His mercies and love. Regardless of the results, we would have believed this, but it makes it even more special and isolated knowing she truly is unique and was created with such intricacy, something we were blessed to be able to see through gene testing.

Romans 8:28 And we know that in all things God works for the good of those who love him, who have been called according to his purpose.

Winning. Duh.

What a relief. The weekend went well despite Friday night being a little rough on my girl. We enjoyed being inside, since both Gabriella is on house arrest and it is hotter than an oven out there! Saturday I ran errands... Had to catch up on 2 weeks of neglected groceries and household items and go to my long over due eye appointment I had cancelled while Gabriella was in the hospital. Saturday night, PJ and I went on a mommy son date to my sisters dance recital. Sunday we stayed home and I made a yummy dinner... Gumbo! Things were nice and uneventful, just the way we like it. :)

Gabriellas bloodwork done today by home health was great! Her levels of immunosuppressant are a little high now, which is better than low. Her magnesium levels are being stubborn but we will continue watching it. If her levels stay stable we will remove the PICC line before next weekend. We went in today for an ECHO and that too looked great! Function returning and leakage improving. We have a few more blood draws and a clinic appointment Thursday for this week and I am sure it will only get better!

Our coordinator was telling me how a girl they just did a biopsy on last week showed grade 3 rejection with no symptoms while Gabriella showed a grade 1-2 with total decline. Its amazing how rejection works and how it is so unique for each kiddo.

We don't know what our future holds with the risk of further rejection but I know this was another battle won by Gabriella! This girl keeps pushing on through whatever is handed to her.

HOME!

I'll quickly get to and past the hard part, because harping on my disappointment is not worth it. Long story short, we were moved from cardiac at 1am to the general peds floor. Thankfully it was a private room, but nonetheless it is more of a holding cell for kids like Gabriella. They needed the bed on cardiac, and I cannot blame the nurses, doctors, or any of our medical team. I'm disappointed in the hospital for opening a brand new, state of the art hospital set to cater to all the kids in the southwest at a level of excellence and they still have yet to open a cardiac step down for our kids who need more than general peds.

Anyways....

My lack of sleep last night over my aggrivation built confidence to tell the docs on the unit I wanted out today. She isn't on any advanced monitoring and only here to take meds orally & have blood drawn. Luckily my amazing transplant team agreed and is sending us home on home health and a PICC line. A nurse will come and draw levels daily so we can intricately adjust her med levels. We got her 12 outpatient meds delivered and sorted, my advanced schedule of 7 med times through the day and supplies to get us by at home. One last dressing change and we are waiting for transport to take us out!!!

We will proceed with caution for a while. Since she has a PICC line, just finished an aggressive immunity treatment over the last 2 weeks and her levels are lower than desired of immunosuppressant, we will be semi-hermit for a while. Well worth the trade off to be home and in our element.

I couldn't be more blessed to be sitting here watching her do so well again. Makes these last 15 days away from home, away from PJ & Paul and sleeping on a recliner with infrequent showers SO worth it. I'm so honored to have a fighter on my hands. I need to remember this when she is strong willed about other things... I'd rather have her stubborn than one who gives up!!!

Extended Vacation

The overall idea was that we were going home today. Any of you used to ICU life, you know we don't ever mention a specific day, we don't pack our stuff and we don't make plans around a specific date. Turns out, we are NOT in headed home... Because Gabriellas body is as stubborn as her mind! Her magnesium levels continue to be low, regardless of how much IV or oral replacement we give her. Today, to top it off, her immunosuppression (tacrolimus) levels are HALF of what they should be. This is a big issue because if her levels aren't right she could face rejection again, especially so fresh off of a major rejection episode! So we are staying here to get it all figured out. We have spread out her medications to 6 administration times throughout the day and night (27 actual drug administrations) to prevent interaction or absorption effects and see if we can get her levels on track. She had an ECHO today that I'm still waiting on specifics from. I'm not going to say tomorrow, or Friday, but I will say I hope its before Sunday so I can spend a day with my family all together!!!

We were supposed to be in Loma Linda right now for genetics, biopsy & kidney function testing. Seeing as how its 113 in Phoenix, if we can't be in California, I'll find the positive in being here that we are not outside!

We are now a few rooms down from some transplant friends. Please pray for little L, who is almost 1year post transplant and having lung & intestinal issues! I love seeing our friends, just wish it wasn't in the CVICU. :)

I'm so thankful for this gorgeous new hospital. It truly is comfortable in an odd way. We don't feel cooped up at all and are thankful for the amazing nurses we have had. It has all made a difficult issue tolerable and we will stay willingly until she is safe to leave!

Another wonderful day

Another ECHO today showed more progress! Every millimeter smaller, every droplet less of leakage and every number change is welcomed by us!

They have stopped Gabriellas IV epinephrine and her r-ATG treatments are complete. Today they stopped her IV Lidocaine, magnesium and sodium fluids... And we are hoping she reacts well! The next step is to stop her IV Milrinone.... But that will come in it's own time. Her r-ATG did it's job, depleting her t-cells, and it was successful in stopping her rejection.

So we have gone our first round of rejection, and it was a severe one! We are ever faithful that this will not happen again, but we will not take for granted the healthy times we have and will always be on guard now that we know she is the kind of kid to quietly fall into full blown rejection.

If we can get her levels right of immunosuppressants, get her magnesium and sodium levels back up to where they should be and continue to see progress, we can get out of here! Last night was night eleven here, which could have been miserable in the old unit but is luxurious and tolerable here at the new tower of the hospital!

Thank you all for prayers up to now, and we ask that you do not stop! We believe in complete healing and restoration, and all God wants is for us to simply ask. Prayers to Him are precious not just for him but also us. God is always near, always listening, and does not need fancy words... Just loving meaningful conversation. How lucky are we to serve such an amazing god....

Sparkle, sparkle little heart

Yesterday was another glimmer... As ECHO showed further improvement in her heart function! Her overall energy and morale was also wonderful! She had some bedside OT and PT to help with her weak legs and arm. We managed to be so busy she didn't watch ANY TV so I was relieved! Her bloodwork is also showing that the r-ATG is doing its job and diminshing her t-cell count. We have 2 doses left of r-ATG and will be done by Monday morning with it.

The game plan is to finish the r-ATG and get her off other IV meds, switching most to oral. Since she is moving towards being stable enough to go home mid to late next week, we will be checking blood and ECHO every 2 weeks after discharge to keep her out of here. She also will have another biopsy in 1 month to recheck pressures and rejection. The r-ATG treatment will take 3-6 months to fully remove from her system, so we will watch her blood counts to see when she is 'safe' again, but until then she is back to wearing a mask and keeping home more than we had been. This affects school in August (along with other factors I learned yesterday), something far too complicated and better suited for another blog this week when I have time to go into detail.

Gabriella is going home on more meds than she did post transplant! We are going back to all of the original meds she was sent home on last year, plus supplements of nutrients she is lacking, plus meds for her declined function. Her heart needs a significant recovery time, so we will watch for signs of improvement in the relaxing phase of her heart- the function that was badly affected. One day we will attempt to lower the function medications and see how her heart reacts. We are faithful for full restoration though, and will allow God His timing in doing that.

We haven't wanted for anything in here. Nurses have been wonderful and spoil my girl rotten, the gifts & activities have poured in like candy to keep us busy, I have snacks galore, but more importantly we have felt the love in every visit or message. We haven't really felt lonely up here because there is always someone calling, messaging or visiting to make us feel connected to outside. We do miss our hard working daddy, and little brother PJ, but we are doing what we need to in the short term to have long term time together.

God bless every one of you. I hope your Fathers Day weekend is filled with as much hope and blessings as ours!!!!

IMPROVEMENT

ECHO HAS IMPROVED! Treatments are working and function shows her heart is working slightly better! We couldn't be more relieved for this direction!!!!

Please continue to be fervent in prayer! The cries to heaven are working and we know God will guide the doctors and provide complete healing... Our faith has never wavered!