And the verdict is...

Sorry for the massive delay in updating the blog. Lilli did fantastic in Boston and surprisingly we were not there nearly as long as I had feared. The team there was on top of the whole transplant evaluation process and everything ran quite smoothly. Lilli did have to undergo lots of testing and procedures but thankfully the only invasive test was the heart cath. We met with a pharmacist, nurse practitioner, social worker, physiologist, physical therapist, liver dr, kidney dr, orthopedic dr..... Needless to say we were able to learn quite a bit more about what's going on with Lilli.

Last week we had an appointment with our cardiologist at Children's with a phone conference with our cardiologist in Boston to find out the recommendations.... We were told Lilli is not currently a heart transplant candidate. She is considered very high risk as she has countless factors that would increase the mortality rate of attempting or surviving with a donor heart. They did say if at some point she deteriorates to a horribly poor quality of life it might be worth the risk, but not at this point. The crazy thing is there are actually cases of patients still having PLE even after transplant!

We found out she does have some liver and kidney damage from her heart anatomy causing extra pressure as well as from all the diuretics she requires. It's nothing major at this point but increased her risks. They were also able to test her bone density and it was horrible :( again from her diuretics. We found out her oxygen levels are ok while sitting, sleeping, etc. but when she gets up even just to walk they go down to about 85%.

I have to say I wasn't very surprised with this news and in a very weird way it was sort of a relief. Now we won't have to leave our life in MN behind and move to Boston for 3+ years, now we can enjoy life and make the most of each day. I'm beyond petrified of the future..... They said its impossible to know her outlook. She could have one year or 10 years. Unfortunately her oxygen will continue to lower, her liver and kidneys will have progressive damage done,and  she will probably not grow much at all (because her body can't absorb protein and nutrients well). We pray that she can remain healthy enough long enough to the point where there are new and other medical options or treatments.

With all of the generous donations our trio to Boston was fully covered with enough left over to give Lilliana a fantastic Christmas and to save for our continual medical costs. I truly cannot thank you all enough for your support.

So it will be a day by day journey. I'm trying to remain positive as much as I can and not let this be the beginning of the end. I know deep within my heart that whatever miracles God wants to work through her I will be thankful for!

Lisa

In Boston

Hello everyone,

We made it to Boston in one piece.... Even with the snowy weather. Thursday was a busy day at the Hospital with a DEXA scan (to check bone density), echo, stomach ultrasound, X-ray, and appointment with our new cardiologist Dr. Blume. We were there from 9am to 5:30pm.

Dr Blume was great and seemed extremly knowledgeable. Although I nearly had a panic attract when she mentioned the plans I have now settled down enough to give it a try....

We are going to completely deprive Lilli of water and salt :( she said it's going to be a miserable 5 days. Her body CRAVES water and salt because of her anatomy, her heart tells her she NEEDS it, but it's completely counter productive and actually is making her heart work super hard and is making her pressures go through the roof.

As a mother (especially a health nut) at first thought I said NO WAY! How could I possibly tell my daughter she can't drink ANYTHING for 5 days (and then super limited after that, like literally an ounce a day)! Dr Blume said its rough and she will make us feel like we are killing her at times. But if this means NO TRANSPLANT (for now) & she is healthier and happier in the long run I'll give it a whirl. I just emailed Dr. Blume a million more questions like "what about her digestion, her liver, her skin....." But I guess no minor side effects could be worse than transplant and all that's required with that.

She said she actually had a patient with such horrible PLE he was requiring tubes to drain his stomach fluid and was listed on the transplant list.... After the fluid restriction he improved so much they even took him OFF the list as he was too healthy! He was 16 and he said he thought it was going to be impossible but now he feels so much better and has trained his body not to crave it he said it "wasn't so bad".

Prior to me giving up sugar, wheat, dairy, and processed foods and I would of said NO WAY I could do it.... So I'm attempting to draw off those experiences.

We will be admitted on Sun afternoon, Monday will have some testing and lots of consults regarding the transplant evaluation. Tuesday Lilli will have a heart cath, as they can not figure out why her pressures are so high.... Her pulmonary veins actually seem in good shape so they need more info. The rest of our stay will depend on how she recovers from cath and then we will do the "water restriction" the entire stay. I'm praying for a Sunday discharge.

We probably won't know the transplant answer until after we come home. We will have a teleconference with Dr Blume and our MN team when all the results are in. Plus if this water things works we might not even need the answer now :)

Today we had Hospital free day, we went to the New England Aquarium and had a fancy seafood lunch :) tomorrow we have another off day and might find another fun place to visit.

Thank you for all your prayers and finical support! It's been such a blessing.

Headed to Boston soon...

Once again sorry for the long overdue update, sadly we can’t get any local internet providers at our new house (we are quite good a picking the place right out of service areas) J so it’s taken me awhile to find time to get on a computer.  

Lilliana is currently doing fantastic after a little rough patch. She was having a really hard time at school and then got a horrible ear infection. So after a round of antibiotics and much back and forth with doctors and her school we decided that half days are best for her. Now she goes from 8:00 till 10:30 J it’s perfect for her as she gets to be there for the bulk of the learning time and just misses lunch, recess… She is so much happier and LOVES school again vs being a ball of stress and anxiety.  Her school and teacher have been fantastic and super flexible. 

We made the shift to Children’s Hospital in MN and we have been BEYOND impressed. They are so on top of everything and it has made my life so much easier. Our first meeting with Dr. Dummer was exactly what we needed. She was kind but blunt and HONEST about the severity of Lilli’s health. She agreed that we are at the transplant point and that we need to be evaluated ASAP by Boston. The bad news is that things are really not looking so great for her chances to be a candidate but she agreed we NEED to hear it straight from Boston. 

We had an apt there yesterday and she recommended Lilli see a physical therapist and occupational therapist and see if her “delays” are just her personality/ preferences or if there is a physical reason for her avoidance.  We will also be seeing an infectious disease Dr to discuss Lilli’s immune issues (lack of spleen, PLE, etc.)… Which is something that in hindsight probably should have been mentioned the day she was born! 

Children’s has been in constant communication with Boston and we are knocking off the pre-transplant checklist here while we wait to get booked in Boston.  On Oct 14^th Lilli will be having a CT scan of her heart and veins and also a liver scan. 

The current plan is still to get to Boston this October. We are awaiting word of when they can fit us in. 

As a mother I feel like I just know that Lilli will not be a candidate and after tears, anger, and sadness I feel like it might be for the best…. I want Lilli’s life to be normal. I want her to spend the time she has happy, at home, and not feeling worried. No matter what the evaluation shows I know we will be OKAY. 

We know from the last trip to Boston how extremely expensive it will be… gas, lodging, food, etc. We will try to get into the shared family housing close to the Hospital during the transplant evaluation process and then if she is a candidate we have been generously offered a family to stay with while we wait for a heart. 

I have set up a Go Fund Me page for those of you who would like to help us with this finical burden at:

http://www.gofundme.com/Lillianafaith

 

kindergarten and some updates

Hi everyone, sorry for the delay again. We still don't have internet at the new house and updating the blog in my phone is sort of a pain, so ignore the spelling errors :)

Well miss Lilliana has started Kindergarten! After much prayer, debate,  and back and forth we decided that having Lilli be a "normal kid" as long as possible is for her best interest. I was petrified the first day, and seemed to be about 2% less today lol. She is so happy and seemed to grow up even more and has been the happiest little kiddo the last few days.

Speaking of keeping life normal... there is a slight chance (if our team at Boston agrees its best) that we might stay in MN until Lilli is ready for a true transplant evaluation. We know thay currently she isn't a canidate due to her lungs and pressures so we pitched the idea of having her cared for here at Childrens Hospotal of MN until she becomes more "stable". Dr Blume at Boston actually trained the new cardiologist we will see at Childrens and they have a very close working relationship established. Dr Blume was presenting Lillis case to the whole team at Boston today and we should hear tomorrow whay the game plan is.

Switching to a new hospital will be a change, but based on every interaction I've had with Childrens thus far it should be a GREAT decision.

So for now things are looking up as far as day to day life goes.... the long term outlook still needs MANY PRAYERS. No matter how amazing Lilliana apprears on the outside there is still so much "wrong" going in inside of her.

All I know is that i want to make everyday with her a celebration. I need to treat her like she will grow up and I need to let her be a kid :)

I just pray for a safe and HEALTHY time at school for her as long as possible.

Finally some direction

Yesterday I was finally able to speak with Dr. Blume in Boston. She spent over an hour on the phone with me and we finally got some claification on direction and what's going on with Lilli.

First she explanined the transplant evaluation process and all it requires.
1) Lilli will have to have multiple tests and procesdures to find out the overall condition of her body... liver,  lungs, kidneys, etc. And even her emotional state.
2) then they will have to find her spot on the spectrum of how sick she is... is she too sick to attempt a transplant or is she too "healthy" to be listed yet.
3) next they will find her place on the specteum of how long a transplant would last her, a year or 20 years
4) we will have to hear all that comes with a transplant from the emotions to the lifelong meds and all of the side effects.

The plan is to get to Boston within the next month. We will be admitted on arrival and plan to be inpatient for about 2 weeks. The bad news is that based on her cath results her lungs currently could NOT handle a transplant, the good news is Dr. Blume thinks we can get her in a better place with some interventions.

Durring our stay they will do the transplant eval and try some therapies to get her lungs working better such as using oxygen, fluild restrictions, some iv medications, and possibly another cath as her stent us narrowing slightly and every little bit might help.

If she is approved, a transplant is about a year away :( and she would have to stay stable the entire wait.

We discussed school and we all agree its just going to too much for her right now :( it breaks my heart and I have no clue how I'm going to tell her :(

Now we are just waiting.... waiting for insurance to approve the evaluation (which apparently can be a mess), waiting for beds to open up at CHB, and praying for a miracle that makes all of this unnessary.

To be honest EVERY option but the pure miracle sucks.... so I'm voting for that :)

Thank you for all of your prayers and offers to help. We are working on setting up a donation website for those why want to contribute finacially.