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

Wednesday, July 3, 2013

Surgery is postponed...

Elizabeth's admission and testing last week went well. She was in the OR longer than
expected and had a bit of trauma to her colon due to the procedure of
catheter placement being more difficult than usual. this caused extra bleeding and
pain, but she is fine. once again, she showed us all that
is more than amazing in her strength to get through painful,
horrible things. She did it though and now we can put the
awful testing away until the next time.
We were happy to be able to get an unexpected discharge so that
we could be home for the weekend. We headed back to Los Angeles
yesterday in preparation for her scheduled colon surgery today....
but tonight we are home. She is too sick with a sinus infection (and cough)
and the surgical team felt it too unsafe to put her through the surgery today.
It is hard to be mentally and emotionally prepared for such a big surgery and then
have a change in plans, but there's no way she should have gone to the OR today.
She's on a heavy course of antibiotics and, because she can't seem to stay well for
very long, she will have surgery as soon as this course of antibiotics is finished in order
to head into surgery her best and not risk being sick post-op.
Elizabeth handled the frustration of postponing it better than I did. She did voice to all
that she will now not have surgery before her birthday; she's spent birthdays and holidays
in the hospital and if she has a say -which she does- she will not be behind the glass on
July 16th. Surgery is scheduled for the 17th.
The bone marrow biopsy will be done at that time as well.
We just have to trust that His timing is best and be patient.
I'll update more soon.


Thursday, June 27, 2013


video

a video made for lizzie. turn off the blog music before hitting play.

Sunday, June 23, 2013

Update

I have not written in months..... too much to say with too little motivation to say it.
If you have checked in, in the hopes of praying for my Elizabeth - thank you.
Elizabeth has been in and out of the hospital since I last wrote. She now eats noting by mouth and is on tube feeds 22 hours a day.
Tuesday she will be admitted for a few weeks for another bone marrow biopsy, testing and then major colon surgery on July 2nd.
And still, she smiles.

Thursday, February 14, 2013

Update

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.






Tuesday, January 8, 2013

You Are My Race


http://www.youtube.com/watch?v=OTg1n95--KE

{There is a favorite children's book of mine called You Are My I Love You (many of you have received the book from me as a baby shower gift) and currently lines from the book are making their way around Pinterest and facebook. Seriously, the beautiful words in the story get me every time I read it to one of the kids.
Two of the lines in the book have touched me in a new way over the last few years as they make me think of the journey Liz is on....I am your finish line, you are my race.
I am your way home, you are my new path.
I have said more times than I can remember and I have heard other mamas echo the same, that this feels like a marathon we are running with our sick children. It is exhausting and difficult and I wish we knew where the finish line was. I'm starting to understand that there may be no finish line for Liz medically. She is always going to have to deal with her health and this marathon is going to be a lifetime race.
What I can do as her mom is be her finish line, so to speak. I can cheer her on. I can advocate for her and rally for her so that there are mile markers she can see and accomplish and overcome along the way. When she gets too tired, I can pick her up and carry her until she is ready to run again. I can open my arms and wave her in and towards her finish line- whatever that may be.
We never would have chosen this path, but I will help her find her way. }

The last week has been difficult for Liz. Since my last update, she did end up starting on an oral antibiotic as her Staph just wasn't healing and her sinus infection had gotten pretty bad. We are now just praying that she stays clear of CDifficile infection as a result.
In the past week she has had two clean-outs due to impaction. It is just this never-ending cycle of impaction, dehydration, soiling, not eating, and back around again.
Her stoma (GTube site)  still isn't looking good or healing the way we'd hoped. She had an upper GI last Friday that showed the tube is placed well in her stomach which is good news. Her surgeon is thinking we may have to change to a new kind of tube, but I would hate to do that as right now she has a low-profile tube which means she has a little "button" sticking out of her belly and I hook her to the tube during her feeds. The new kind of tube would be a tube hanging out of her all of the time. Not ideal. The GI doctor and the amazing GI nurse clinician at the hospital are working to see how we can get her into see the inpatient wound care nursing team, as they know tricks and have supplies that even most docs don't.
She has declined in terms of her colon over the last couple weeks. We have started a new plan of weekly clean-outs and enemas in addition to her meds, but two of her doctors have asked me to seriously consider surgery to do a Cecostomy and Appendicostomy. I cannot even go there right now.
Today I took her to see her pediatrician because she has been short of breath over the last few days....alright, more like I noticed it about 5 days ago. She has been saying that she feels dizzy at times, but that is not a new thing and is usually caused by dehydration. The shortness of breath is new though and it has gotten worse, so I took her in..... she has partially collapsed lungs. This has been caused by the stomach pain she has been having and being so full of stool has caused her not to breathe deeply enough to really get air through. She is in no distress and she has no wheezing or chest pain other than when she blows out or takes a deep breath. She is using the spirometer every hour to move those lungs and we  are doing breathing treatments every four hours to try and prevent pneumonia (she has a bit of a drip and congestion now and she has had pnemonia before and is susceptible). She'll be re-evaluated on Thursday. I am sure it will be better by then.
There has been discussion about her next bone marrow aspiration. GI said she should be ready in the Spring to have it done, but her Pediatrician wants to wait until the summer. We want to make sure that if her improved nutritional status is going to help her marrow we give it enough time to work and prove itself rather than testing too early and thinking it hasn't helped in marrow recovery.
Next week she will see a Genetics and Mitochondrial specialist for the first time at the request of a few of her doctors. Honestly, I am too covered in poop (figuratively, but literally too) to give much thought to that appointment or the reasons we are being sent there. I will deal with it on that day.
School.... is up in the air. I need to decide what to do about it. She missed the week before the two week Christmas break and she isn't going back this week. We just need to decide what will be best. For now, she is my shadow again.

Thank you to those who continue to pray for her and support us on this race. I hope you know how much we appreciate you.


Monday, December 17, 2012

Dear Elizabeth Claire....


http://www.youtube.com/watch?v=7UpfatdyFtY&feature=related

Elizabeth Claire,
I know you are sick of being sick. I know I can't feel the exact pain and sickness you feel, but as your mommy, trust me baby, I feel your suffering in ways only you will understand once you have a child of your own.
Every pain, every needle, every excruciating procedure, every minute of lost modesty or embarrassment, every time you feel scared or angry or lost- I feel it as an ache only a mother's heart knows.
And I also understand your frustration, discouragement and even your thoughts of envy. I have felt those same things on your behalf and in my own mind over the last years.
So my Lulu, this letter is for you to remind you of the things we have gained as a result of pain and suffering; your pain alone, and the pain I have had as your mommy as I have watched you take on your trails.....

If you were healthy, you may take the ways your body is strong for granted.
If you and I didn't spend so much alone time together, we wouldn't talk as much as we do.
If we weren't stuck in the hospital room as much as we are, we wouldn't have as many of our own silly jokes or secrets.
If we never had to wait (and wait and wait) for doctors or recovery, we wouldn't be practicing so much patience.
If you weren't sick, you wouldn't have helped put something back together without even knowing it was broken ( a story I will share with you when you are older).
If you weren't sick, I wouldn't be as strong as I am or as good of a mommy as I am to you and your brother and sister. You have given me that gift.
If you didn't have to fight through pain the way you do, you would not be nearly as compassionate towards others as you are.
If you weren't suffering, many of us may not be reminded of humility, endurance, grace and patience as often as we are because of what we see in you.
If you weren't sometimes too tired or sick to do things, you may take for granted the days you are strong and doing fun things.
If you weren't sick, there would be so many less prayers and less people talking to God.
If you weren't a little hero, we wouldn't have been as touched by the lives of your hero friends, and goodness, can you imagine life without having those friends?
If you didn't have to practice mind-over-matter or have to be brave so often you may not be as strong as you are now.
If I didn't watch you endure so much for so long, I wouldn't appreciate much of what I do, in the ways I do- soaking in the happiness you, Kate and Jack bring. Breathing in the yummy way you smell after a shower, just as I did when you were a newborn. Closing my eyes to take in all of your giggles and the sound of your voices singing. Committing the happiest days to memory knowing they will get us through the next trial and bring us smiles again.
If you didn't have to suffer my sweet girl, we may not recognize that life is hard, but it is also so wonderful in even small ways.

These are a lot of "ifs", I know. There are many other ifs or whys that we could list that have nothing to do with your suffering. Everyone has a list and is fine to question things and even be sad about some of them.
I want you to remember that even though life can feel so unfair and even as if God has forgotten to protect you or answer you at times, many of our "if's" have awesome answers and there is always something to be grateful for.
I love you Elizabeth Claire. If only you could grasp how much and how proud I am to be your mom.

Finally, an Update

It has been a long time since my last post. Though I have much to say, I will keep this blog entry to the point and factual as it is prayer for health that I would like most right now.
I last wrote when Liz was in-patient at CHLA for the colon manometry studies. We were a bit surprised when we learned that the tests revealed that her colon is not contracting much at all without the use of medication and enemas. We knew she had some issue going on, as her chronic impaction and other symptoms indicated, but her physician and Walter and I were surprised by the severity of her dysmotility. Unfortunately, the probes placed in the OR before the start of the day-long testing weren't able to be threaded to the right side of her colon or her cecum; frustrating at the time, but very disappointing now that we know how severe her dysmotility is. We need to know what her cecum and ascending colon is doing. The manometry studies will need to be repeated, perhaps in Cincinnati at the amazong colon/rectal/bowel center there.
After the results Liz was put on a new regimen of meds and enemas, but they are not working in the way we'd all hoped. Unfortunately there are no other medications available to try and at this point we can only adjust meds and enema schedules and try to make sense of her poor colon. Surgery is the only other option. We meet with her motility specialist in Los Angeles in two weeks to talk about whether or not it is time to head to Cincinnati to see the specialists there.
Due to the new findings, her Immunologist has also referred her to the genetics and mitochondrial specialist at CHLA for more studies.
She has had ongoing issues with her stoma (the site/opening where her g-tube is placed) and after much pain, treatment by her GI and home nursing, she finally has started seeing a wound care specialist. Two weeks ago her stoma cultured positive for a Staph infection. Of course this sent everyone into a bit of a frenzy as the topical cream antibiotic she'd already been using wasn't clearing the infection and using an oral antibiotic is not an easy recommendation for Liz due to the recurrent CDifficile. Her infectious disease doctor got involved and after many calls between us all, we elected to give an additional topical treatment a try. Last week, her stoma was swabbed again and is culturing less Staph than a few weeks ago, so we are hopeful the medicated creams are working enough on their own. Thursday she will have the stoma cultured for a third time and we are praying the Staph is continuing to decrease.

Liz has had some great days and has even had two weeks of attending school for the whole week! Admittedly, she is exhausted though and currently we are discussing with her doctors the option of pulling her from school for a couple of months during this flu season and in an effort to get better control of her colon (it is difficult to time enemas and hydration around a seven hour school day).
You may remember that we elected to stop the immunoglobulin infussions (IVIG) in May and watch her body to see what it would do on its own, without the help of donor blood to boost her immune system. We knew that the winter and flu season would be telling in how her body would do without that support. I've been praying that was the right choice.

Today I took her to the pediatrician because her cold-like symptoms are much worse and she has chest discomfort and a fever. Liz and I diagnosed her before we even saw the doctor, as we both know exactly what her sinus infections present like. She indeed does have a sinus infection and her chest pain and tight airways are due to possible bronchitis. Again, an oral antibiotic is not an easy go-to option for Elizabeth because of CDifficle. So, we are increasing her inhaled Azithromycin dosage and Dr L. is contacting Infectious Disease and GI before we begin an oral antibiotic. She is pretty uncomfortable tonight.
Today Dr. L and I talked again about pulling Liz from school and we have decided that we will have her labs drawn on January 4th to evaluate her white count and neutrophil count and then based on those labs decide when/if she can return to school. Infection requiring antibiotics is just such a scary thought for us because of the darn CDiff issue.
Speaking of CDiff, we are nearing a year of her being CDiff free! This is huge and such a praise as that last 9 month-long infection was such a blow to her body. Liz smiled today and told Dr. L and me that she needs a sign that reads "One year CDiff free and I didn't need my sister's poop!" (remember, a fecal transplant was on the table and in the works, with Kate being the donor) : )

Of course the last 2+ months since I've written have brought much more to blog, but tonight these are the facts.... these are the issues our girl needs prayers for.

If you are still thinking of her and praying, thank you.