Life is wonderful and difficult... and I am grateful!

Thursday, February 14, 2013


Last night I sat shaking uncontrollably. The mind-body connection is amazing and I guess my adrenaline was rushing as I sat thinking of the upcoming week and my body responded.
On Monday, our Liz will be admitted to Children's Hospital Los Angeles for a while. We were told days, but we've learned to pack for weeks. On Tuesday she will head to the OR for a procedure to place a PICC line again. Originally, we planned on placing the PICC so that I had IV access at home to provide IV hydration to her. This week plans changed, and the new plan is to have Liz go on gut rest for a while, and receive TPN (IV nutrition) for awhile.
While we are at CHLA, the enteral nutrition team will also consult and try and figure out why her zinc keeps dropping so low.
The geneticist will have labs drawn for the two syndromes he asked to test her for almost three weeks ago, but I wanted him to justify his reasoning. After hearing Hem/Onc's impression of her last bone marrow slides and after the geneticists argument provided in chart notes and conversation to Liz's pediatrician, we have decided to agree to the labs. I have hesitated sharing the names of the diseases with others, as they are both terminal and that is a dramatic thought, but prayer is powerful and so I share now and ask for prayer that she will test negative for both Pearson's Syndrome and MNGIE (both mitochondrial diseases). I do not think she has either.
Hopefully Surgery will consult as well during our stay; it has been decided that Liz needs the cecostomy (surgery to provide colon-access from the outside so that we can do daily flushes) and given her complicated case and so much scarring from all of her previous surgeries, it will be important to have surgeons who are comfortable working on her.
Surgery will not take place during this admit. She must first have the colon manomerty studies repeated and a surgical team in place.We plan to come home and return in about a month for the Cecostomy.

Please pray for Liz's tolerance and patience. Being in the hospital is rough. Last year she needed the PICC line moved to her other arm a month after placing the first, so she is hesitant and dreading having the line in. Gut rest will be difficult as well, as she won't be able to eat by mouth for weeks-month.
We all feel the effects of a hospitalization as Liz and I are away from home. Not being at our local hospital is especially hard because I won't be able to see Jackson or Kate while we are in LA. Walter has been on vacation this week and getting away from work will be difficult since he has just had this time off.
We are praying we are making the right choices for our girl-

Thank you for continuing to pray and encourage us.

Below is an entry I wrote two weeks ago but didn't post. I'll attach to this one for those who have asked the same questions.....

It has been a few weeks since I last posted. I decided to write tonight to address some questions I have been asked or heard others say they have been asked.

What is wrong with Elizabeth-
 Hmm, good question and I wish I knew the answer. If you are looking for an overall diagnosis as an answer, there isn't one yet. It is a complicated explanation....
Elizabeth and her identical twin sister Kaitlin both had surgery for severe reflux when they were four months old. Because of lung disease due to constant aspiration, they were both on oxygen for about nine months during their first year of life. After that initial surgery, Katilin (with the exception of a bit of kidney reflux that effected her in her first years) has been healthy. Liz has not been as fortunate and she has required seven surgeries, as her Fundoplication has failed over and over, and her repaired hernias have also required additional repair. She has had a Pyloroplasty as well. In addition to the surgeries, she has had more procedures and hospitalizations than I can count. All of this, as hard as it was, wasn't life-consuming. Liz would have a procedure or surgery and bounce right back. She was considered a kid with a chronic issue (GI) but we never considered her a chronically ill child. Ever.
That changed in January of 2010. After a small, simple surgery I noticed that in the weeks following she wasn't herself. She was overly tired, her hair was falling out more than usual and she was bruising. I thought she could be anemic and requested blood work. Her labs showed she had low white cell, red cell and platelet counts and her pediatrician said we'd repeat them and watch it. Over the next six weeks, additional labs all continued to show low counts and Liz had started having low-grade fevers most often at night. She was referred to Hematology/Oncology and had her first bone marrow aspiration and biopsy which revealed abnormal marrow and cell lines, but no cancer.
Around the same time, Liz started having trouble fighting off infection. She has had recurrent sinus infections and CDifficile (her last bout of CDiff lasted nine months). Infectious Disease and Immunology came on board and early on there was talk that if it wasn't cancer, perhaps she'd been exposed to a virus, her body was hit hard by it and it may take time for her to recover. That isn't the case and it became obvious that something more was happening.
Hem/Onc said maybe something was "brewing" and she continued to have labs drawn every 2-4 weeks to watch her counts.
In the meantime, she was getting sick often and we were spending more and more time in the hospital.
In the last two years her GI issues have overwhelmingly taken over as well. She was diagnosed with severe FTT (Failure To Thrive) and she had a PICC placed to deliver TPN (IV nutrition) for four months. She now has a GTube (feeding tube in her stomach) to provide supplemental feeding via formula feeds. Hydration is a huge issue now as well and she often requires IV fluids.
Her motility issues have lead to chronic impaction (stool) and she has suffered horribly in the last year. In October, she had testing done that shows that her colon isn't contracting on its own which is why she is getting impacted.
She has had a total of 5 bone marrow aspirations and biopsies in the past three years and all show that her marrow is hypocellular.

I wish there was a diagnosis, wrapped in a bow and delivered to us, so that we could have an overall plan. We may or may not ever get that. In Liz's case, it is almost a chicken or egg first kind of question.... what came first, the GI issues, or something bigger causing the GI issues? The marrow and blood being the primary  cause, or nutrition or something else causing the marrow to be abnormal and not recover?
I have sat at tables with the heads of multiple departments as they talked about Liz's case and they too play the "which came first" game.

What is the next step in Liz's care or treatment plan?
Sigh. This too is a tough question as currently, we have some tough decisions to make.
As for the bone marrow and blood issues- We have held off on testing her marrow for about 8 months. Our current strategy is to give her nutritional status time to get better (with GTube feeds and better Prealbumin counts) and we will test her again in May or June. IF poor nutrition was the cause of marrow failing to produce (I refuse to use the term marrow failure as docs have used) then now that her status is improving, her marrow will show recovery. That is our hope. If it doesn't show recovery and shows the abnormal cells that it has, then we have a problem.

Liz does sinus rinses and takes an inhaled antibiotic (nasal inhalation) multiple times a day to try and hold off sinus infections. For her, antibiotics required to treat infection lead to CDifficile, which is devastating on her system.
Some have asked why I am not taking her to other facilities/doctors....
The truth is that I am! First of all, our local children's hospital is not some small, hick-town facility. It is a major, recognized hospital with some amazing physicians and programs. Having said that, we have taken her to UCLA, CHLA, Rady's. Cedars Sinai and we have consulted with out of state hospitals as well. Currently, three of her specialists are at CHLA.
Kaitlin and Liz are identical twins, why is Kate not sick if one of the possible diagnoses is a genetic disease?
This too is a complicated answer. In short, if Liz does have a genetic disease, Kate could be spared even though they are identical twins. Or, Kate could be a carrier and it will take time or an assault on her body (illness, surgery, pregnancy) to show itself. It is much too complicated to explain in written form, but we have asked the question and understand the answer.
Why are we in medical debt when there are government programs and max-spending even with insurance.....
Currently, Liz does not have a diagnosis that is covered by Medical or CCS. SUPER frustrating that she doesn't qualify for these health coverage programs.
We have private insurance but we pay towards every out of network physician that is billed (this includes radiologists, pathologists and anesthesiologists that are on service at the time Liz requires care; we don't get to choose these physicians. Co-pays for each office visit are $45.00 and her monthly prescriptions cost about $300. Those two costs alone add up.

Does Liz see someone to talk to about her feelings?
Yes. Liz has had a therapist for three years. Some months she goes once a week and others not at all. I recognized early on that she needed someone to talk things out with if she would like. It was a proactive choice on my part that I'm so happy I made, especially now that things have gotten so bad.

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