The Very Hungry Caterpillar

Today I sat and did the household budget which is not my forte.  Honestly, dealing with money is not exactly my forte so it took me quite awhile.  Between doing the budget, checking my email, and of course having something cooking on the stove all the while, I sat at my desk for a total of two hours.  The notable thing here is that LillyAnna sat in front of me in her highchair the entire time eating.  Yes, I said eating...for two hours.  She had strawberries, blueberries, cheez-its and chicken nuggets.  She has also been drinking like crazy.  No vomit since Thursday.  We are doing all milk now, I decided to just go full throttle because she does not seem to mind the milk and mixing formula and milk was getting tiresome.  One of the first things people said when I told them about the eating and vomiting issues was "Oh, she is on so much medicine!  I know someone who____" or "I took a medicine like that and it made me ____".  I very much appreciate these connections and attempts at problem solving but I see more of this disease than anyone and I have seen this vomiting/non eating thing happen way too many times.  It always coincides with her disease acting up on the outside.  Some think the issues stemmed from the formula.  Besides the small body of research that actually supports babies staying on formula until two years, Lil was on full formula starting at 7 months after being breastfed and since then, she has had periods of being a great eater and periods of the vomiting/non eating.  Is it the highchair?  The food choices?  Swallowing issue?  Me and my loving, supportive family and friends have covered all the bases.  The one constant is that she stops eating and drinking when the disease is bad.  Not surprisingly, her blood work looked even better this week than last.  C-reactive protein stayed <0.5, sed rate went from 9 to 7, and white blood cell count was 10 and is now 9.7.  To sum up, these are inflammatory markers we follow that are a loose indicator of Lil's disease activity.  All of these numbers are in the normal range and these changes would not be significant in a normal person's blood work but with knowing so little about Lil's disease process, it is one thing I hold on to and the fact that they are going down in conjunction with the fact that she is clinically improved is significant. So I decreased the steroids to 0.8mLs.  The last time she was completely weaned from the steroids was when we discovered the issues with Remicade so it will be interesting to see what happens when she is weaned from the steroids this time.

We do have the Humira back on board and the lesions look better.  I have some ideas to discuss with the newly returned Dr. R.  I want to ask if it is possible that she could have lesions on the outside of her organs which we would not see in a scope.  Also, in the past weeks, two random people asked me if she had been allergy tested.  I am assuming that this is something the docs have already looked into but it does not hurt to ask.  Actually, one of the things I so admire about Dr. R is that I feel like I am truly a partner in Lil's care.  I would hope he would say the same but I am sure I annoy the crap out of him sometimes!  I can never tell though, so that says a lot.  He is very open to hearing my ideas and thoughts on her and her condition.  I know how busy he is with patients who are much higher on the hierarchy than Lil but he always has time for me and more importantly for Lil. 

I will keep everyone updated but it looks like we are in an upswing.  There have been other times that I thought we were finally coming down the other side of this mountain only to get caught on an errant branch.  Chris often says he feels in his heart that she is just going to keep getting better and one day we will look at each other and realize how well she is doing, how easy life has become and that this is over, forever.  I want to believe that too but it is hard.  I will keep my head up, enjoy the good times and wait. 

The Memory String

There was a time during my years in college that I found myself in a sad place.  I had just ended a long term relationship (way too long), my mother was burying our family in debt with her gambling problem and although I had some good friends, I was feeling lost.  In elementary school I met one of my greatest friends.  I fell in love with her immediately and we continue to be friends to this day.  Since college, I have met people who have become invaluable to me.  However, it was at this difficult time in my life that I met a group of people who would have a profound effect on the person I am today.  They gave me a feeling of total belonging and acceptance such that I had never had outside of my family.   At the most important times of my adult life, they have been there for me in their own special way.  In this circle, true friendship means having lunch with cocktails so I could write my grandmother's eulogy, bringing a flask of Jack Daniels to the funeral, doing all the nonsense bridesmaids do (which in this case meant three men had to act as bridesmaids); they have fed me good food, good alcohol and made me feel like the most interesting and important person in the world. 

One of my favorite memories with them is the first time I went skiing. We were all working at a restaurant called the Italian Bistro at the time and it was the kind of job which gave us the perfect excuse to band together in the face of having to wear horribly uncomfortable uniforms, serve lunch shifts in which we would total 20$ in tips, and a list of other atrocities.  We had so much fun.  Our boss was the kind of man who most loved to hate (and we all secretly admired).  I was not so secret in my admiration.  I thought he was a great person and he always treated me with respect and kindness.

Our boss was the one who organized a ski trip for those of us who spent an average of eighty hours a week serving soup, salad and pasta in his restaurant.  I had never been skiing and I was nervous.  I loved it.  I will never forget the total abandon that I felt sliding down the side of that mountain.  At that point in my life, I had very little responsibility beyond paying my rent, feeding myself and passing my classes (which although challenging were not stressful for me).  I remember watching my friends fly by with huge smiles on their faces, falling flat on my ass thinking it was the funniest thing ever and being terrified while standing at the top of a black diamond slope but going down anyway because I knew I was not alone. 

Today I had the opportunity to take my own children skiing for the first time.  Leaving LillyAnna at home has not been easy.  If I thought my stomach was in knots at home, it has been worse since being away from her.  Chris and I are very much enjoying spending time with the twins and I have to admit I enjoy a break from filling syringes of medication, cleaning up vomit and pouring over Lil's little body checking for sign of her illness acting up.  But today while the twins were taking their first ski lessons, Chris and I went to the top of the mountain and I had the opportunity to recall that freedom I felt years ago.  It was a very rare moment of complete indulgence for me.  After getting my 'ski legs' back, I was able to comfortably coast down that mountain; pretty much alone, watching the forest fly past and feeling the wind biting my cheeks.  Memories of my first experience skiing surrounded me and I felt awesome.  It was actually an occasion where my gut was not twisted and my mind was clear. 

My mother in law tells me that LillyAnna is doing well at home.  Apparently she keeps waving and smiling at the video I sent her of us.  She also keeps going to the front door and asking for Net-net.  I am sure LillyAnna is fine but I also know my mother in law wants me to truly relax and so may not tell me all the ins and outs of what is going on so that I will be able to do just that.  I am ok with that.  I know she would tell me if there was a major issue and I would worry if I knew too much.  Ignorance is bliss.  I can't wait to see Lil.  I truly miss her.  For now though, I am enjoying this time and my memories. 

Meet Me At The Moon

Today we had a Modified Barium Swallow Study done.  It was a very simple test where Lil had to sit in what looked like a car seat and drink a bottle.  An x-ray machine showed us what was going on inside while we watched.  Because she had not had a bottle for a few hours, she drank happily and made faces at the docs and techs surrounding her.  It only took about two minutes.  Then they tried to get her to eat a cookie which, not surprisingly, she would not do.  Part of the study consisted of us meeting with a technician who walked us through the x-ray and then observed Lil eating while we talked. I hate to say it but I was glad Lil did what she does at home and only put the food into her mouth, maybe chewed on it, maybe not and then took it out again.   I appreciated the tech's honesty when she told me that she really did not have any strategies for us.  (Here is my pat on the back) She actually said I am doing everything right.  Haha! The swallow study came back fine; there are no issues with her swallowing so she is suggesting feeding therapy.  We will meet with a feeding therapist once a week.  The therapist will check in with us and keep track of Lil's eating and maybe give us some strategies to help her eat more efficiently.  I do not think this will solve our problem because I know Lil can eat just fine.  For some reason, she does not want to.  However, this is part of the reason I am not working.  I would much rather give this a chance than have to do something more invasive.  Dr. R had mentioned using a daily g-tube to feed her as a last resort if she does not start eating on her own and she gets into trouble.  I really do not want to do that.  What I really do want to do is find out why she is not eating.   In the meantime, she is still drinking enough to stay hydrated.  Her bottles are now half cow's milk and half formula.  At this point she takes a break from her daily vomiting routine every once in awhile so that is good but it still happens almost every day.  At this point, her lesions still look good and what I have gotten back from this week's blood work is good also.  Her sed rate is down from 9 to 7.  I am still waiting for the rest. 

As I rocked LillyAnna to sleep tonight, I enjoyed just holding her.  I will not be rocking her to sleep for the next three days and it makes me sad. William Ellery Channing (to be honest, I have no idea who that is) said "Difficulties are meant to rouse, not discourage.  The human spirit is to grow strong by conflict."  Lil and I have been through so much together.  One of the reasons I started writing this blog was to put into words what the last eight months has been like for us.  I thank you all for listening because writing has been cathartic for me.  Tonight I am just too tired to talk about much but some of the things I have had to do to that baby are horrible.  It is amazing that she does not associate me only with pain and suffering, for that I am lucky.  It is the exact opposite actually.  We are very connected and it is going to be hard to be away from her.  She is so full of life and personality.  Everywhere we go, she leaves a wake of smiles behind her.   On the other hand, Chris and I are very excited for the weekend.  This will be the first time we take a trip like this with the twins.  They have never stayed in a hotel or gone skiing.  And although they handle it well, taking care of Lil is hard on all of us so being able to spend time with just them will be good.  I know LillyAnna will be fine.  She will definitely get lots of attention!  But I am going to miss her.

A Surprise for Mama

The most difficult part of LillyAnna's condition is that I never know what is coming next.  Every day surprises me.  After a few awful days of watching her lesions come alive, cleaning up her nightly vomiting routine and checking that the bag I have permanently packed for the hospital is stocked, I am now sitting here watching Lil happily suck on (still not eating but an improvement) grapes and askng for more.  Her lesions have completely calmed down and are no longer red but back to nice, calm purple.  She did not vomit last night and she has had no fever for the past two days.  A nasty cough has developed but she seems to be handling it well.  I think my head cold is actually worse than hers.  Chalk it up to NyQuil or exhaustion but I only woke up twice last night. 

My father in law has graciously gifted us with a weekend in the Poconos.  We are supposed to leave Friday.  Although it makes me feel like I am going to start a nightly vomiting routine, we are going to leave Lil home.  It is not that I think someone else cannot care for her.  I am just so connected to her.  I know every up, down and sideways of her moods and disease.  I have not been away from her for more than a few hours since June.  Also, Chris and Nettie handle all of this so well.  Very rarely do we see them act out of jealousy and with all of the attention lavished on LillyAnna I think that is pretty amazing.  They will update me on her lesions, give her bottles, bring her toys when she is uncomfortable and they watch what she is eating and report back to me.  They know when she gets sick at night and instead of being mad that I cannot share in their bedtime routine, they ask if they can give her a kiss when she is cleaned up.  Their concern for her is constant.  However, it is not fair to them for us to take advantage.  This weekend needs to be about the twins.  My guess is that they will be more than a little upset Lil is not coming with us.  This often happens when we do something without her.  I do not mean to take away from how wonderful all siblings can be to each other but siblings of chronically ill kids have a special understanding that relationship means.  As a family, we have witnessed this over and over again in our new friends from AI to a much greater degree.  One mom and I often talk about how these kids know and see so much more than we would like them to but it is their reality.  These experiences are going to shape who they are and who we are as families for better or for worse.  As moms and dads, we can only do our best to guide them and support them just like every parent does every day.  So although this city girl did not get on skis for the first time until she was 22, my kids are going to follow in their father's footsteps and have their first experience skiing at 4.  Oh boy!

Quick Update

Lil had an awful night last night. She vomited again before bed, finally was asleep and in bed by 9:30 but was up at 1am, 2am, and 3am. Finally at 3am I gave her a dose of oxycodone and she slept until 8am. However, this morning she had no fever and the lesions looked less red. She was much more pleasant this afternoon than the past two days. She maybe ate 3 cheerios today and half a slice of american cheese but drank about 16oz of her new formula/milk cocktail. Good thoughts for tomorrow!

Tell Me Again About The Night I Was Born

My husband Chris is adopted. When I met him (in his very straightforward way) he told me that he was not interested in finding his birth mother. He was not angry or anything like that, but his parents were the people that raised him. When we had the twins, he started talking about finding out about his medical history. As I have watched him grow as a father, I have also seen his interest in his birth parents evolve. LillyAnna's illness forced his hand a bit. Although we have been communicating with his birth mother, Susan, tonight we met the rest of the family. It was simply amazing to watch this group of people interact with each other. They are a very close family. It felt like although they had never met Chris, somehow he had been missed all of these years and we were just catching up.

Of course Lil was the star of the show as usual and there were balloons so she was happy! She woke up with another 101.6 degree fever that again went away on its own. Chris and I keep finding small papules on her skin. None of them have become pustules yet so that is very good but it is scary to see them pop up. Since the summer, I have not seen this many. Her two arm lesions and the big one on her thigh each have one pustule that is broken open but again, they are very small. For some reason, only one part of her bloodwork came back so I will have to call the hospital Monday and see what they want me to do. With all of this activity I am vey interested in seeing her levels but I am also loathe to poke her again particularly because of the activity. She did vomit again tonight when we got home. It is just sad how nonchalant she is about it.  I don't think I saw her eat anything today save for a spoonful of cottage cheese.  However, she is drinking ok and does not even seem to notice the cow's milk added to her bottle.  Dr. R, where are you when we need you?  Oh yes... Haiti.  They probably need you more:)

The Fussy Princess

Lil started her day with a temperature of 101.6 degrees Fahrenheit. It did not get much better from there. She was fussy off and on for the majority of the day but the fussiness definitely took a strong hold starting at dinner time. True to form, she would not eat anything. She was very excited about seeing Daddy and that was a welcome distraction as well as a little walk outside. I always figure that if she feels good enough to walk, it can't be that bad.  The GI docs gave us some Zofran for the nausea she experiences on the nights we give her the methotrexate. It did not help tonight. She drank about four ounces, fell asleep and about ten minutes later sat up and puked. Her new thing is to say uh oh when she throws up and point to her mouth- which she promptly did and continued to vomit for the next 5 minutes. Clean her up, watch a slideshow of the pictures on my phone for about the hundredth time (that is the only thing that calms her down when after she gets sick) and she goes back to sleep. Around 8pm, we hear her crying. Give her another bottle and here we are.

We did get to see some of our girlfriends from 3F today when we went to drop off paperwork and a goody bag for one of our new Chronically Cool Families friends. It is so good to see everyone there. I know that may sound weird but it must be some sordid post traumatic stress disorder thing (no disrespect to those who actually HAVE Post traumatic stress disorder). Those girls and guys took such good care of us and they love Lil and I mean LOVE her.  They ask about the kids and my dad and my life... it is really amazing.  Dr. R even suggests I visit the floor when I am at the hospital because "it will make the girls happy".  He quickly adds that we are not to stay too long and not to touch anything.

I am concerned because the lesions have some red pustules.  Also, it seems like there are new papules everywhere.  It is probably my anxiety but every time I look at her I feel like a see a ton of them.  They do not look like much but it is evidence that the disease is active.  This has happened before although it seems as if there are a lot this time.  It is my non-medical, purely mom opinion that this is the recourse of not having Humira for two weeks. I hope that is all it is and nothing more.  The other evidence that this is the disease is the fact that her temperature was so high this morning but dropped on its own.  As most of you know, body temperature generally rises in the evening.  Last but not least, part of her blood work from today came back and her sed rate (one of the inflammatory markers) is up.  It is not that high but it is much higher than last week.  This particular marker takes awhile to rise and fall so I am interested in seeing the rest of the blood work. 

Unfortunately, the nutritionist did not have much to offer.  She would like us to very slowly transition Lil to cow's milk.  I am thankful she does not want her solely drinking PediaSure for two reasons: one, Lil does not like it and two, it is even more expensive than baby formula.  It is also gross.  She did warn me that if Lil continues to drop weight we may have to switch to PediaSure.  Lil did drop weight again and although her weight is still normal for her age, she should not be losing weight.  This is where I am again praising God for Dr. R.  During our November admission, Dr. R stated that Lil had been in the 125th percentile for her weight.  We all had a good laugh about that considering the size of my other children and that she was in the hospital for not eating.  He still wanted me to increase the calorie count of her formula to "give us a cushion".  At the time I thought he was crazy.  In today's world, you want me to make my kid fat?!?  However, it is that cushion that is saving us from a g-tube right now.  We have a swallow study next week. 
 
This kid cannot catch a break.  I bought her a balloon today.  Someone suggested tying it to the back of her pants so she would not lose it.  The balloon popped off the ribbon.  Luckily we had one at home to replace it!

Lost balloon

 

And so I sit here, rocking her to sleep for the third time tonight (it is not even 8:30) thinking about what kind of night we are going to have and trying not to worry about it.  I am also loving holding this beautiful baby and smelling her sweet skin.  I know how lucky I am that I am home and that my baby is able to walk and play and pull on a balloon.  We'll see what tomorrow brings. 

Quick update

Just a quick update.... Lil started her nightly routine and threw up again tonight. She also threw up today. She seems uncomfortable and there is a suspicious diaper rash-esque thing on her butt. Hopefully just diaper rash but it looks odd to me. Most of you know that Lil has three lesions on her diaper area. Actually, in the babies that had pyoderma it is the most common site. Lil's diaper area lesions did not show up until later for Lil.  Hope our good streak is not coming to an end. I like sleep.

The True Confessions of Charlotte Doyle

For the majority of my life people have been telling me I was a born mother. I seriously question this observation now that I am one and I even more seriously questioned it when the twins were born and it seemed as if they would never stop crying.  However, it is true that one of my great passions in life is bringing friends and family together for good food, good company and occasionally good spirits (ok, more than occasionally).  A great friend of mine once told me that one of her favorite places in all the world is my kitchen where I am known to work diligently at the stove or counter while someone sits and keeps me company.  Admittedly, my kitchen is my favorite place to be also.  It is not fancy and it is not set up for the kind of picture I am trying to create here but it is mine and it works.  I have had visions of my children, husband and I sitting together at the table that my grandmother bought shortly after her wedding enjoying dinners together. 

That was before I became a mom to three kids four and under because now I understand why Ralphie said his mother had not had a hot meal in eight years.  It is hard to confess that I feed my children dinner with the TV on while I work in the kitchen. It makes me feel like a terrible mother and causes me serious anxiety about the future of our dinner table and my children's diets.  But I also have to confess that it is one of my favorite times of the day.  It is during these times that I get to really listen to the kids' stories about school and friends,  I can get them what they need without having to interrupt my mealtime experience and I get to cook and bake happily with them in my sights.  It gives them an opportunity to ask questions about food and for me to give impromptu cooking lessons... one at a time.  After the kids go to bed, Chris and I eat dinner together alone and this is something else I very much enjoy.  It is again one of the only times we get to talk without a chorus of crying, story telling and echoes of "mom/dad" all happening at the same time.  It gives us a chance to talk about things we cannot talk about in front of innocent ears.  Don't get me wrong, we sit together for meals at least three times a week and it is so much fun when we do.  And I grudgingly say that I make it one of my goals to eat dinner together every night.  For now though, this works out perfectly. 

Of course, one of the reasons this has gone on so long is LillyAnna.  It takes me a long time to prepare her medications for the night and make sure I have everything correctly dosed.  Nowadays it also takes me quite awhile to figure out what she will be willing to eat.  In the past week, she has been eating about one meal a day and has only thrown up twice.  This is a vast improvement.  Until today, she has also been very pleasant.  Today has been a fussy day for her.  She woke up with an elevated temperature.  Meemaw noted last night that her arm lesions looked red and swollen.  I agree.  She is chewing on her fingers like a champ and when she cuts teeth her disease seems to act up. I also noticed a small new papule on her belly. We will find out in the next couple of days.  If her temperature normalizes, her lesions calm down, she is less fussy and most obviously some teeth pop through then all is good.  As Dr. R loves to remind me, she is also an eighteen month old who has typical ups and downs.  We are getting routine blood work tomorrow.  Let's hope for the best.

Where The Wild Things Are

LillyAnna has been acting like herself for about three days now.  My "oldest " friend (that is to say the person who has stuck by me the longest) gave me great advice when I became a mom.  She told me you have to trust yourself and be an advocate for your kids because you are the only one who will.  The past three weeks have been terrible for us.  LillyAnna was just a mess.  Medically we could not really see why.  Her bloodwork showed that her inflammatory makers were fairly low, the lesions were calm, she managed to keep herself hydrated despite drinking so little and she was not febrile.  But she was a mess. The tug of war that went on in my brain for three weeks rivals any elementary school field day activity.  Is she really sick, I think something is wrong but so much seems right, but she is crying all of the time, but she is an 18 month old cutting teeth, but babies do not need oxycodone when they cut teeth, but she is throwing up every night, but that could be the methotrexate, but it started before the methotrexate... and on and on and on. The only evidence I had to support my feeling was that she was losing weight and that those who know her best agreed with me that she was not herself.  Living with Lil the last few days is reaffirming.  I was right.  Something was wrong because she is back to her old self.  I don't know what was going on and that is scary.  As amazing as medicine is today and considering how much I love and respect Dr. R- they do not know everything (which he will openly admit).  I need to have more faith in myself next time. 


Back to eating macaroni and cheese!

However, it is when LillyAnna is having one of her 'normal' periods, that my anxiety roars its terrible roar and gnashes its terrible teeth.  It seems as if this is how it is with little Chris too.  As soon as she comes out of one of her down periods,  we both get tired and down and cranky.  Maybe it is because the crisis is averted and I can think as opposed to focusing all of my energy on putting one foot in front of the other.  Maybe it is because I am just tired.  What I know is that I do not know what is coming next or when.  Something is wrong with my baby.  I can't make it better.  It is not the worst thing that can be wrong with a child and I am reminded of that on a daily basis but it has its own horrors. 

I am scared of the dark and always have been.  I make sure I go up the steps before Chris so that I can avoid the dark but if it happens that he is in front of me, I run like hell up those steps.  When I am in the dark, I cannot see what is in front of me and so everything is a threat.  I am scared of what is sneaking up behind me and its weapon of choice, what is by my feet, what is circling my head.  I feel like danger is closing in on me a mile a minute and I have nothing with which to protect myself, not even the knowledge of what that danger is.  It is one of the reasons I cannot watch horror movies; even the really lame ones.  When Lil starts to feel better it is like being at the top of the steps after running from the dark.  The demons are in my mind's eye; my heart is racing, my hands are shaking and the impluse to keep running is almost inescapable. 

In the car tonight, it was just little Chris and I.  We talked about heart feelings and body feelings.  After awhile he summarized our conversation by saying, "So when someone is sad, happy or frustrated, those are heart feelings."  I told him he was right and he went on "So when I am sad about Lil, that is a heart feeling."  Again, I told him he was correct.  I added that it is ok to have other heart feelings about Lil and he immediately came back with "Then it is ok that I am mad that Lil is sick?"  After giving myself a second I said, "All of your feelings are ok.  You just have to be careful about how you act on those feelings."  "I DO get mad about Lil being sick Mom, mad in my heart." was his response.

Lesions this week: 

Today I Feel Silly...

LillyAnna had an absolutely wonderful day yesterday. After waking up, she drank some PediaSure for the first time. We have tried this many times before to replace formula but today she actually drank some! We celebrated a birthday with some special friends this morning. Lil's favorite party activity was licking marshmallows and then handing them to Big Chris!  Since staying clean is a high priority for Chris you can imagine the enjoyment it brought me every time I saw her stick her chubby little fingers in the air towards him to hand him a gob of goo.

 

 No one can resist those beautiful dark eyes though so he would audibly sigh, smile and hand her another one before scrubbing his hands with a baby wipe. At dinner, Lil ate a half of a chicken nugget, some Mac and Cheese and then had a couple of bites of a chocolate Valentine gift from Mommom and Poppop. Wow!

 

 

To top it all off, she went right to sleep and did not vomit. I have to admit, though I am very happy she ate more than two bites of a cheese stick for the first time in three weeks, it was simply so much fun to see her laughing, talking, smiling, running around and NOT CRYING.  She was the baby I am used to seeing.  Her temperature has been running at about 99.9 degrees farenheit.  This is not a fever and could mean something as simple as the possibility she is fighting off a cold.  Chris and Nettie both have colds so I am thinking that is why.  Whenever her disease is active, her temperature runs about there but also spikes in the morning which has not been happening.  We were actually very lucky that she has not had a fever this past few weeks.  It is probably the only reason she stayed hydrated despite drinking so little.  Ironically she woke up with a dry diaper this morning after sleeping for twelve hours.  Of course, I will continue to keep an eye on this but her demeanor tells the story.  I think she is in an upswing.

Getting her hands on another balloon helped a little too!

 

One Foot Two Foot...

If you have visited us at AI you know Lil's affinity for balloons.  Seeing that look of absolute happiness on her face when she plays with a balloon is just fun. Therefore after getting blood work this week and having the phlebotomist notice how many bruises she has from needle sticks, I decided to indulge in this fairly inexpensive treat.  Poor Lil.  She played with that balloon joyfully for about an hour before it popped.  I don't want to say it was funny but it was.  Partly because I got her what looked to be the more sturdy and of course slightly more expensive balloon.  Partly because she cracks me up when she gets mad. 

It has been an interesting week and I am not really sure how to feel about it all.  LillyAnna has been much more 'herself' this week and that has been wonderful.  When she is laughing, making faces and asking for Monsters Inc. the tight band that forever exists around my chest loosens.  She has even slept through the night, twelve straight hours, twice this week!  Of course she followed that up by waking up at one am this morning and basically not going back to sleep.  I truly thank God for the plush rocking recliner we received as a gift when the twins were born.  I have slept in it more nights than I can count.  At least this time I only have one baby sleeping on me!  Restarting the Periactin helps but I know from experience that this boost will be short lived.  As long as it gets me through the next two weeks until Dr. R returns I will be happy!  I know that the Periactin is just a band aid but she is having more wet diapers and she ate two egg yolks and a whole cheese stick yesterday.  That is the most she has eaten in a 24 hour period in three weeks. 

I just about fell out of my chair when I saw her blood work this week.  She finally received the Humira Thursday evening.  Friday's blood work looked very good.  Before this episode Dr. R and I were discussing the efficacy of the Humira for her or possibly shortening the time between shots even though she is on such an extremely high dose because she was having flair ups days before she was due for the shot.  Now that she has not had a shot in a month... her blood work is fine.  So confusing!  My Mommy heart is telling me to be cautious.  I hope I am wrong but sometimes these so called cytokine* storms take awhile to build up and show themselves later.  The other piece of this puzzle is that she is still on steroids so that could be helping to cover us in the absence of the Humira.  But maybe, just maybe, she is starting to get better.  It is a possibility and it is sitting like a treasure in my heart.  However, I am not getting out the bolt cutters yet.  I do not mean to be negative but I am more familiar with her disease patterns than anyone and it just does not feel like this is the end to me. We have scheduled a modified swallow study, a meeting with a nutritionist, and an appointment with a speech therapist. Oh boy....family beware, I will be needing some babysitters so you might want to screen your calls:)   So for now I am just putting one foot in front of the other and waiting for the next big thing or the absence of a next big thing!

 

 

 

*Cytokines (Greek cyto-, cell; and -kinos, movement) are small cell-signaling protein molecules that are secreted by numerous cells and are a category of signaling molecules used extensively in intercellular communication. Cytokines can be classified as proteins, peptides, or glycoproteins; the term "cytokine" encompasses a large and diverse family of regulators produced throughout the body by cells of diverse embryological origin.^[1]
The term "cytokine" has been used to refer to the immunomodulating agents, such as interleukins and interferons. Biochemists disagree as to which molecules should be termed cytokines and which hormones. As we learn more about each, anatomic and structural distinctions between the two are fading. Classic protein hormones circulate in nanomolar (10^-9) concentrations that usually vary by less than one order of magnitude. In contrast, some cytokines (such as IL-6) circulate in picomolar (10^-12) concentrations that can increase up to 1,000-fold during trauma or infection. The widespread distribution of cellular sources for cytokines may be a feature that differentiates them from hormones. Virtually all nucleated cells, but especially endo/epithelial cells and resident macrophages (many near the interface with the external environment) are potent producers of IL-1, IL-6, and TNF-α.^[2] In contrast, classic hormones, such as insulin, are secreted from discrete glands (e.g., the pancreas).^[3] As of 2008, the current terminology refers to cytokines as immunomodulating agents. However, more research is needed in this area of defining cytokines and hormones.
Part of the difficulty with distinguishing cytokines from hormones is that some of the immunomodulating effects of cytokines are systemic rather than local. For instance, to use hormone terminology, the action of cytokines may be autocrine or paracrine in chemotaxis and endocrine as a pyrogen. Further, as molecules, cytokines are not limited to their immunomodulatory role. For instance, cytokines are also involved in several developmental processes during embryogenesis^{[4][nb 1][5][nb 2]}
A cytokine storm is basically when the cytokines go crazy and wreak havoc on our girl's body which is what happened in November/December.

My Grandmother is Great

What is it about layering lasagna, slicing onions and stirring marinara that makes my soul feel peaceful? 
It could be the connection to food that makes all those who love to cook feel happy smelling delicious food cooking, knowing you will get to eat it, and savoring the reactions of people who enjoy it. 
It could be thoughts of my greatest friend, my grandmother. Being in her kitchen is one of the strongest memories I have as a child.  I can see her lithe hands peeling garlic and then giving me the knife and holding her hands over mine so that I could feel the skin and the flesh of the garlic to know where to cut.  I can see her very small Southwest Philadelphia backyard lined with tomato and basil plants.  She would take me outside and hold out a tomato for me to smell, crush basil between her fingers to release their scents and snip the flowers so they would continue to grow.  Those smells stay on your fingers all day (especially on a child:). 
There is a possibility that I feel at peace because all three of my children are sleeping while I am cooking.  That at least adds to it! 
My grandmother was great.  I often think about what she would say about my life now.  I know she would be supportive, I know she would be helpful.  But if I got one more chance to sit at her feet in her living room with a Phillies game playing quietly on TV and a pot of water simmering for tea, I wonder what she would tell me is in her heart.  I know it would be honest and beautiful and true.  I also know it would make me feel.  Feel angry, feel surprised, or feel happy.  Lately I don't feel much but tired and sad.  So I wish she were here.  I wish I were in her kitchen or living room or backyard.  And I wish I could hear her voice.  For now I have garlic and onions and tomatoes and basil.... and memories. 

 

The Wheels On The Bus

It is very frustrating to keep going 'round and 'round.  The Gastroenterologists saw LillyAnna today.  This includes the doctor who did her scope but Lil's reputation obviously preceded her when the resident who spoke with us first said she knew all about LillyAnna and filled in information I left out even though we had never met her!  LillyAnna's previous scope showed a small spot of inflammation in her duodenum however they only found one small spot so it does not tell us much and is probably not the cause of her discomfort right now.  Unfortunately, they could not get into her small intestine with the scope because it was just too small and they did not have instruments that could do it safely.  With Lil's existing Pyoderma, it is too dangerous to try to force something like that and possibly cause more damage.  Even though her only lesions so far seem to be on the skin, it is possible to get lesions on the inside of her body.  Dr. R tells me they are different cells so that is unlikely but it is still a possibility that makes us cautious.  Especially with Miss "I don't follow the rules".  If there is inflammation in either of those places, it should be contained by the sheer amount of anti-inflammatory medication she is taking and her bloodwork does not show evidence of significant inflammation. So that brings us 'round to the fact that she is two weeks late on her Humira due to insurance/ pharmacy issues. The shot should be delivered tomorrow.  Will that help with the stomach issues?  Who knows, but I am not holding my breath. 
Which brings us 'round again to the idea that if it is not infammation, what is it?
We next talked about her relfux.  Although the gastric stomach emptying study showed only slight reflux the doctors think this could be the cause of her vomiting and lack of eating.  I cannot see how that makes sense.  What brings us 'round again is that Lil is already on the highest dose of reflux medicine allowed for her weight and she just began a new medicine in addition to that one that should help with reflux.  We agreed to give that medication (Reglan) some time to work.  All of this information together is poor evidence that her issue is reflux for obvious reasons. 
And those wheels make one more turn when the docs throw out the possibility that this is a behavioral issue.  Reasons I do not agree: one... she is throwing up daily (not behavioral), two... this issue with not eating or drinking has happened twice before and it always comes when she does not feel good or something is brewing, three... I just know my daughter. 
That brings us to medication.  The big question on everyone's lips is "Could it be the meds?"  Followed almost immediately by "She is on a lot of medication."  The mediation she is on is necessary.  As Dr. D stated so strongly today, he has never seen remicade anti-bodies as high as LillyAnna's.  This is saying something due to the fact that he is the chief of his department and her body is so small.  Therefore, it would be quite dangerous to take her off of methotrexate while she is on the Humira.  Methotrexate is the so-called "chemo" drug and it prevents Lil from creating anti-bodies to the Humira. It causes upset stomach.  'Round again, these issues with Lil have been happening a lot longer than she has been on Methotrexate. 
So basically I walked away from this appointment frustrated and with few answers.  We are going to do a swallowing study, meet with a nutritionist, see a speech therapist (they examine swallowing also) and try to get Lil to drink Pedia Sure instead of formula ($$$).  The formula is fine for her but the docs said we will get more bang for our buck with something that is more age appropriate.  I am not excited about this change as Lil is a creature of habit and loves her Similac Advanced.  Switching her might engage us in a standoff and she cannot afford that right now in terms of hydration.  Only time will tell right now.  So Bon Voyage Dr. R.... are you sure your cell phone will not work in the wilds of Haiti?

LillyAnna On The High Wire

Lil is doing an intricate balancing act right now.  She is drinking enough fluid to keep her hydrated... barely.  She is eating what equates to one slice of American cheese or one half of string cheese a day.  Although she did celebrate Superbowl Sunday with half a Hershey Kiss!  Dr. R told me to start giving her a medication called Periactin which is actually an antihistamine but it makes her hungry and helps her sleep.  Sounds great huh?  What mother does not want an excuse to help her baby go to sleep easier!  Don't judge me! They actually have to put a stipulation on the bottle of Children's Benadryl which tells parents not to use it to get a child to sleep.  This is one clue that I am not the only mother in the world who has been tempted to drug her children to sleep (I have never given in to that temptation just for the record).  Anyway, Lil has been on this medication for months and I recently stopped giving it to her in hopes that she might sleep through the night.  How can I not feed her in the middle of the night if I am giving her a medication that makes her hungry?  Cruel and unusual punishment.  I am going to wait until she sees the GI docs on Wednesday before starting that medication.  Stubbornness?  Possibly but we also just started giving her Reglan which may help with her stomach emptying and I wanted to give that a chance to work before adding another med.  Also, I like to sleep at least six hours in a row.  It feels good and it has happen two nights in a row- unprecedented!

Despite being almost two weeks late on having her Humira injection (it is a long story-refer to Facebook for details), her Pyoderma Gangrenosum is actually very quiet.  What does this mean?  No idea.  She will not eat.  What does this mean?  We have no idea.  There are just so many unknowns.  And so LillyAnna continues to walk that tightrope and I continue to obsess over her every mood and every centimeter of skin.  Luckily I have my husband and Dr. R to keep me grounded.  I am in denial that he will be in Haiti for two weeks.  I am just living on my 'happy little coud' that Lil will be perfectly fine the whole time he is away; hey. it could happen.

For now, LillyAnna has been much happier the last three days which is good because that bottle of Benadryl was posing a strong argument which got indcreasingly stronger over the last two weeks when she would not sleep for more than two or three hours at a time and when she was awake she was crying.  I worry so much less when she is acting like her usual sunny self.