Pre op at CHOP

Long day today. Macsen had an echo, an EKG, a chest xray and a blood draw. In between we saw a nurse practitioner and his cardiologist. We got some yummy to eat in the hospital cafeteria before heading back to Md. We are exhausted. Hoping for a little down time, some celebrating of the New Year and some time with family before his cath on Tuesday.

Flying from ATL to BWI

Merry Christmas

Merry Christmas everyone <3
This Christmas feels weird because a few weeks ago we were all set to go to Philly for testing. Then all those plans were cancelled by CHOP. Then the possible heart failure diagnosis and everyone else realized Macsen did indeed really need to go for a cath. We had to wait for scheduling all over again. I'm calling them twice a day. We didn't have dates and couldn't plan - flying before Christmas? After? Flying with gifts for the kids and all the extended family up north or just mail everyone else's gifts? Wait - in the middle of all the uncertainty I realize we haven't really shopped for gifts except for the kids. Not even for each other. Not for all the family we may or may not see. Phew - glad they all understand shopping has not been a priority. We are set to fly out on the 27th with testing Dec 29 and the cath Jan 2. I'm worried about flying with Mac and his low O2. I'm worried about flying out of Atlanta - their parking situation gives me major anxiety. I'm worried about what the cath doctor will find:( I'm super worried about germs and Macsen getting sick before his procedure - from strangers and from friends/family. I hope all our friends and family up north understand what a  tremendous strain we are under and that we probably won't be able to visit with everyone. Please love us from afar if you are sick or think you might possibly be getting sick. Rescheduling Mac's cath because he contracts a cold would be such a nightmare. We will also be transporting our last (and favorite) foster kitty Asher to my nephew in MD.
Christmas was very laid back. We went to our Church Service on Friday so we had a  leisurely day at home - opening gifts and making an amazing Christmas dinner. We even found a lost dog and returned it to its owner.

Beeping Funny

Since we started the Digoxin Macsen has been complaining his heart is "beeping funny." He means "beating" but it's adorable the way he says it. His hospital grade pulse oximeter died last month - of course it did- and the company wants $250 just to look at it :( We have a small drug store pulse ox but its pretty crappy. His fingers are so tiny it doesn't pick up very well either. I've contacted both of cards about his complaint. His local card said we can  try an event monitor but she has to order it - who knows how long this will take :(

So sad to be right

Today we went to Pensacola to see Macsen's local cardiologist. I am so upset CHOP cancelled his appointments - I knew something was wrong with Macsen. His card says his heart function does look worse compared to his echo she performed earlier this year. He had to have blood drawn and his  BNP is also elevated. :( She put him on a medicine called Digoxin. He's never been on it before and I pray it helps

Holding up his hurt arm after the blood draw - still terrifying for M

Something is off with Macsen

 Over the past 6 months or so I have noticed a slow decline in endurance, an increase in blue spells, and his oxygen levels have dropped from 94 after his last surgery in 2015 to anywhere from the 50s-80s. I struggled to make his team understand what I was seeing. His teachers and therapists are all saying they see what I see as well.
 We had appointments in Philadelphia set for the end of December but 3 weeks before our flight out they called to cancel - said they felt he was doing well and they didn't need to see us for possibly another year. They based this on the echo he had at CHOP at the beginning of August. They didn't contact me or his local cardiologist.
When I called to explain I felt he needed to be seen the nurse practitioner at CHOP dismissed all my concerns. When I brought up the decreased endurance she said the best thing I could do for that was make him exercise more and not carry him everywhere like he was asking me to. When I said his oxygen sats were falling she gave me a lecture on the fontan circulation like I was a complete newbie to this world. When I said he wasn't  growing well she said he looked great (even though he is not even on the growth chart.)  When I said he was having nightly leg pains she told me to medicate him every day with Tylenol - that it wouldn't harm his already vulnerable liver. On and on she went. I have never been talked to like that - not in 5 years of trying to navigate this world and talking to specialists in 5 states at many different centers. I am so angry. I am writing a few emails to let his CHOP card know what happened. But in the meantime I am bringing him to his local cardiologist. Something is wrong with my baby and I will make them see it.

Make a Wish Trip

Macsen was granted a trip with Alabama's Chapter of Make a Wish. He chose to go to Disney World. We went on Fall Break so the girls wouldn't miss too much school. It was an incredible trip with so many amazing experiences, too many to even list.
Favorite thing #1Staying at the Give Kids the World village - a special resort for Wish families where everything is included.It was truly a once in a life time experience.

 

The salon was a big hit with all 3 of the kids

We watched Wreck it Ralph 

Every night at the Village there is a different party planned

Disney Characters make appearances at the village 

The girls enjoyed riding the therapy horses at the village 

EPIC summer 2017 roadtrip

On our way

Summer camping

Macsen has some appointments scheduled in Philadelphia and we are taking a week to get there. He will be having some exercise testing done in preparation for a special clinic at CHOP called the Single Ventricle Survivor-ship Program - or SVSP. We have a camping spot scheduled at Shenadoah! I am so excited to bring him and the girls there. When Macsen made it through his 2nd surgery and we were able to finally let out the breath we had been holding since his prenatal diagnosis we revisited the list of things we mourned we would never be able to do with all 3 of our children. Camping was near the top of the list. It is something we did a lot of when I was a kid and some of my most vivid memories are of the excitement I felt on family camping trips. I'm so very very excited to be able to bring my kids to one of the spots I remember visiting - and to be meeting up with my sisters on the trip!

Mayo study update

So after speaking to Macsen's 2 cardiologists and the Mayo doctor in charge of the stem cell study we have decided to not participate at this time. His cardiologists feel he is doing well at this time and worry the multiple sedations needed to participate in the study - several MRIs plus the sedation for the bone marrow extraction then injection via cath- will stress Macsen. His cardiologists also question if it will even be worth it - there are no preliminary findings published yet if injecting stem cells at this time helps heart function. Dr.Quereshi,  the Mayo dr, suggested we start Macsen again on a heart function medication. His was started 6 months before his last surgery when his heart function was so terrible. It really helped his function. He was take off though bc his local card felt it was lowering his blood pressure. He wasn't started on an alternate med but Dr.Quereshi thinks it would help. I think so as well but I'm not the expert.

Mayo study - to do or not to do

I had all Macsen's records sent to the Mayo clinic to see if he could be eligible for inclusion in an ongoing research study to strengthen his single heart ventricle. To be eligible he had to show poor heart function and I thought in light of how he's been looking - more energy, less blue, Sats in the low 90s - that he would be deemed ineligible, too healthy. So I was shocked this morning to learn after reviewing all his records Dr. Quereshi would like him to participate in the study.
It is a big undertaking with several trips to Rochester, NY, MRIs with sedation, the marrow aspiration and a heart cath. But it could result in better heart function for M - maybe he will finally start growing if his heart isn't working so hard. Maybe it will put off future heart failure for a while. And maybe it won't help at all and will be a painful, traumatic, expensive and possibly even life threatening (heart caths are no joke ) ordeal Macsen is old enough to remember.  We are discussing everything with his cardiologists and with our families but appreciate prayers to help guide us on the right path Here is a link to more information on the Mayo Clinic's stem cell trial

Happy New Years!!!!