"If you made a list of all the things you could be thankful for, the list would undoubtedly be longer than your misfortunes."
Today, Roshi Fekrat and Aram Coyle brought us a humongous homemade Thanksgiving Dinner. Definitely some of the best food and surprises we have EVER had. So many people have shown our family extreme kindness (neighbors, friends, family, and people we don't even know) during the hardest time in our lives. Our family would just like to say THANK YOU EVERYONE...WE ARE SO THANKFUL FOR ALL OF YOU!! Happy Thanksgiving all!!
P.S. We told Eevie to eat her yummy roll...can anyone find it??
Well.....another week done in the hospital, I think this was our 6th week. How many more? We had a team meeting with all of the department teams on Thursday. Here’s a brief rundown of our week.
GI, has given up on TPN finally after Eevie's persistent unexplained fluid sensitivity that we've told everyone about since day one. Eevie has not gained weight on TPN; no matter how concentrated they made it. She actually continued to lose weight and hit 7.115kgs (15.5lbs) on Monday. So after several weeks of attempted 'gut rest' we are going to 'aggressively' attempt to get her gut working. We started her back on Elecare Jr and she consistently has been gaining about 100g a day, up to 7.445 (16.4pounds) today. Unfortunately, we tried to switch her to Peptamin Jr last night - with too many other changes, which resulted in her having explosive diarrhea. Got her back on Elecare and she seems to be doing better. She seems to be absorbing carbohydrates which is great, so further poo tests to be continued and decreasing TPN and increasing gut feeds. Hopefully she'll be on full gut feeds by Tuesday and we can get her line out as long as she is absorbing everything…see below! Thinking another two weeks inpatient at minimum.
GI gut rehab Drs – no answers for Eevie’s issues and not much input in treatment.
ID-infectious disease - Eevie continued to have low grade temps and positive cultures for yeast even after her line was pulled and replaced. The last positive culture to date is from the 16th around 9pm, so hopefully it is being cleared. Antibiotics are to stop tomorrow, so hopefully that will improve her tummy as well. ID wants a CT of her lungs, since she still has crackling and fluid in her lungs and a nasty cough; fungus could be hanging out there. ID said that her line can and should come out immediately if she remains asymptomatic and she can take pill antifungals. Eevie has developed an awful cough that wakes her up as she coughs so hard and long she quits breathing in short intervals, might be viral pneumonia. Eevie had another ultrasound of her abdomen and liver, which did not show any abnormalities or signs of fungus.
IR-interventional radiology Eevie was under general anesthesia for over two hours for her second procedure. IR said that Eevie has very limited if any access for any future lines. The doctor visited with us mid procedure and shared images that they tried her right and left arms. They had to put her new PICC in her left femoral and are concerned that she may not have any access in the future for a PICC, and uncertain about another central Hickman or port if one might be needed in the future and we would need to do a CT to check. Eevie has now had 4 PICCs one in each arm/leg and two central Hickman’s in each chest. We have to clear this fungal infection and get the line out immediately to prevent any other infections.
Derm- Derm still has no conclusive diagnosis. Over the past few weeks they have considered everything from severe genetic or acquired ichthyosis to palmoplantar keratodermas (PPKs) both of which could be lifelong and very debilitating (google them to see what we are planning for). Now they are leaning towards pityriasis rubra pilaris (PRP), another extremely rare and debilitating skin disease. Of course they remind us that Eevie's rash is very severe, uncharacteristic of any specific disease and may be a combination of any of the above. From what I've learned PPK are lifelong and in the case of Olmsted syndrome, officially - Mutilating palmoplantar keratoderma with periorificial keratotic plaques- yes 'mutilating' can cause spontaneous amputation of digits. As in the case of this poor child http://blog.mlive.com/chronicle/news_impact/2009/04/large_00ap_winners.jpg. I asked Derm if Eevie might have this specific disease and was told that it could not be ruled out and that we would know more with the exome testing (4-6months). PRPs are extremely rare and can also be genetic or acquired (possibly following a severe infection) in childhood. The genetic ones seem to be persistent and may or may not improve with age. The acquired ones may improve spontaneously or after years. Of course they remind us that her biopsies are not indicative of any of the above disorders, her rash -onset/course/presentation is different than all of these mentioned. They are tapering her steroids down as guided by Endocrinology.
Genetics – asked to come back after Derm’s possible diagnoses, but they said they won’t have answers or information until exome back.
Oncology – from their point of view Eevie is doing amazing and astonishing. We’ve avoided asking directly but have learned that Eevie’s chance of survival to 2, are much less than 17%, is probably not much better until 3, but that she was probably rarer than 1 in 5 million and definitely is now that she has made it this far. And her GI and growth issues and are expected and probably will be long-term
Cardiology – Eevie had another echocardiogram which did not show any signs of endocarditis – heart infection. Cardiology has no answers as to why Eevie cannot handle IV fluids.
Nephrology – kidney docs- said that they too have no explanation as to why Eevie cannot handle IV fluids and her labs are normal
Endocrinology – said that Eevie is responding to her steroids well and that we should continue to taper them down. Said discussing HGH treatment is only for outpatient and didn’t offer any input.
Metabolic – said they can’t think of any test/labs that haven’t been done that might explain rash or GI issues.
PACT – got to meet the PACT team here…how wonderful, not only for cancer but possible lifelong debilitating skin disease too!
Immunology – said that since Eevie has been on steroids for so long and is still on them they need to wait until she is off a month to re-assess her immune system.
Pharmacology – no real explanation for rash or GI issues or fluid sensitivity. Tolerating meds well.
OT/PT impressed with Eevie physically and cognitively. Eevie actually got down on the mat and played with both without having Brynne nearby!
Did I forget anything……..who cares
I forgot to mention. An acquired ichthyosis can be indicative of an underlying malignancy, such as lymphoma or other cancer. Oncology said that would be extremely rare and only a lymph node biopsy would rule that out, but clinically they did not see any other signs.
Missing our ROCKCHALK Sweetheart already ! My sis bought her this headband to remind us all that before we know it Eevie will be back on Papas lap watching KU B-Ball ! She loved it and gave the "Hawk" kisses !! It was difficult flying home today but it did our hearts good to see the kids and our baby girl this past week . We are very impressed with the doctors , nurses , and entire staff at CHOP ! You can certainly tell that most of them truly care about Eevie and are very impressed with her sweet yet strong spirit !! As one of the chief oncologist's said " She's a miracle " .... It sounds as if feeds are going well and she is absorbing at the current level .. YEAH !!! However she has to go up quite a bit before they will consider it a complete success ! But it's a move in the right direction !! She still isn't feeling well with fluid in her lungs and slight temps ... Not sure if it's a virus or fluid overload or both ! They are watching her closely and treating with diuretics and antibiotics ! The fungal infection is continuing to respond so another YEAH !!!!!
As hard as it was to leave , we truly feel they are in good hands at CHOP ! Stan and I are still savoring those last hugs and the sound of her precious little voice saying " I love you Nana " and " I Love you Papa ! .. Needless to say, I was a tearful mess as we left the hospital . Eevie Elouise WE LOVE YOU more than you can imagine !
Written by Mommy:
Tonight we had to cancel Eevie's "Big Two" Disney Christmas trip. It symbolized so much for our family. It symbolized the end of chemotherapy. It symbolized fortitude, victory, and peace. But most of all, it symbolized the possibility of a LIFE WITH A HEALTHY EEVIE.
After months of planning and anxious "is she going to make it??" anticipation, tonight I heard Ryan tell the Disney operator "Hi, we need to cancel our reservation." The look on his face and the sink in my heart were identical.
As heartbreaking as it was for us, Eevie is still with us, fighting. Oddly, ever since November 10th, she has asked for us to play the "Happy Birthday" song. She wants to hear it over and over again. When I hold her, tucked underneath her blanket asking for Happy Birthday, I can't help but feel that she is trying to tell me something. I can sense her saying "I'm here. I'm 2. I made it this far. Whew." Her own victorious celebration.
It isn't perfect. In fact, our "Big Two" has been less than ideal. But she is 2. She wants to celebrate by hearing Happy Birthday. I can't help but think to myself "How much simpler is a Happy Birthday song than a perfect Disney Christmas trip?" I am realizing that the simple little tune is really much bigger than any Disney trip. In fact, the whole trip was all about that simple little tune. Thank you, Life Warrior, for teaching me that a simple little song is in fact an anthem.
Eevie's Nana and Papa flew to Philly to be with her!❤️From Nana: Absolutely NOTHING better than snuggles from Eevie tonight ! ... She is still battling this horrible rash, but the Drs, here at CHOP have a new plan that we are praying will " kick start " her GI system and start her on the road to recovery . Thank you dear friends for your prayers ! We are staying positive !!
Once again we find ourselves moving from purgatory straight to HELL. Hell is the only way to describe our days. Eevie's skin is peeling off in layers, her skin is splitting and bleeding, and her nose, ears, and eyes are sloughing. She is becoming aware of her skin and constantly looks at and rubs her hands. When her skin peels she says "skin off pease."
In addition to her physical issues, Eevie is now having a trauma response to this 5 week hospital stay. She looks at the door and screams "open" and says "airplane, airplane bye bye!" Eevie asks mommie to put her "bank bank on head" to hide from doctors and nurses.
Nothing is looking good as far as diagnoses. In fact, the worst has been suggested from a new malignancy to very, very serious genetic lifelong problems.
We have spent most of the day in sheer terror of Eevie's new life and possible future. There are not words to describe this level of pain. I would rather die then watch her suffer one more day.
Eevie is getting her line replaced at 9am eastern 8am central time today. Last time it took 3 hours and the line wasn't placed well. Please pray specifically that it will go smoothly and not take long. Our little life warrior needs all of our prayers more than ever right now!❤️
Though Eevie is active and enjoying riding round and round in the hospital car, her status is not good.
She continues to have positive line cultures (enterococcus and fungus), a cough and fluid in her lungs, rash/skin breakage, and no weight gains.
So today she is scheduled for another surgery. We are very nervous as she will be, once again going under general anesthesia today for line removal and replacement. The picc they put in last time took around 3 hours. We are beyond worried as Eevie does not have many options for placement. Her skin is so damaged, and she has already had 5 previous lines. She is also needing to be on antibiotics and antifungal for weeks so another line near her diaper on her left leg would be disastrous. She is at extreme risk for continued blood infection due to her skin and gut, either or both, could be the source of this and future infections... which could lead to sepsis oh yeah and needing to replace this new line.
Dermatology is worried that Eevie has had a reaction to the chemotherapy that may be permanent, resulting from an underlying generic predisposition and requested a genetic screening, which may or may not help guide treatment if any would even be available or beneficial.
We met with a generic counselor and the geneticist who were awesome. They were of course intrigued with her rash, its course, and other symptoms.. and had no immediate thoughts. The geneticist recommended exome testing, which only takes......6 months and costs $20,000.
So, with no answers or improvement, we each gave our blood samples and signed our consent forms.
We feel that we are in a horror movie, the walls are closing in, more problems are erupting, answers are being crossed out, and Eevie is running out of options and treatments. We hope removing this line will help clear the blood infections and the new line will be in a good location and last until her gut is healed enough to discontinue TON and then pull the line.
Eevie continues to baffle the experts. Derm team has given up on diagnosis. Her gut seems to be a little better even though she's on 5 diff antibiotics. She needs a new line so will have to go under again to replace it. This could be a lifelong chronic condition or it could still be a bizarre chemo reaction that will fade with time. Only time will tell. Please continue praying!❤️
Please donate whatever you can give to my precious niece, Eevie Elouise. She was born by emergency c-section November 10, 2013 in Wichita, KS and was flown by Children's Mercy Hospital in KC to be treated fo r congenital acute lymphoblastic leukemia (ALL). Congenital ALL is an extremely rare condition affecting 1 in 5,000,000 ( Only 4 million babies are born in the U.S. every year). Your support and donations are so appreciated. Brynne and Ryan need all the support they can get as this will be a long uphill battle. Eevie is 3 weeks away from her 2nd bday and is battling a mysterious illness most likely chemo induced.