The Little Red Caboose

Since my last post, things have been as I would have expected, until tonight.  Since the night we gave LillyAnna the Humira shot, her lesions improved and she has not thrown up.  The rash on her face has stayed but it gets better and then worse for no obvious reason.  I have not received the results from this Friday's blood work but I do not expect it to show me anything surprising.  Last night when I gave Lil the Humira shot I followed the same procedure as usual and as I have been taught; clean with the alcohol swab, squeeze the fatty part of the arm or leg, go in on an angle...

I reluctantly emailed Dr. R thinking that it would be nothing but I told Chris I would so I did and I got the dreaded "if _____ happens, go straight to the ED".  Damn that man is always surprising me.  So if it gets any bigger or she gets a fever we are to go to the ED.  Issues:  She almost always has a fever of some degree, from the time I received this information she was getting ready to sleep for 13 hours straight and why did this happen. 

When Lil built antibodies to the Remicade, the first thing I noticed was that she was starting to have some of the reactions they had warned us might happen the first or second time she was infused.  All of a sudden, after months of infusions, she began to have reactions.  I could tell the nurses and doctors were surprised but they did not seem concerned so I tried not to worry.  I feel like that same thing is happening now.  The Humira is not lasting the whole two weeks, she is getting very flushed with her medications, and now this issue.  There are only three medications we have in our pocket to use.  This is the second.  Humira and Enbrel (Enbrel is option number three) are not FDA approved for her age which is why we have to give her such a large dose already.  In other words, if the Humira is not working, we are running out of options.  I hope I am wrong about all of this.  I really do and I have been wrong before so I am counting on that.  But I am also scared.  One of the reasons we love Dr. R so much is that I feel one hundred percent sure that his mind is running over our other options already.  This is not to say we are always on his mind but he is very intelligent and he is a thinker.  I have faith in him but I hope I do not have to rely on it. 

Chris and I spent the day at the Special Olympics watching his brother and sister and their significant others play basketball.  I was struck by these amazing people and how they have an ability to show what seems to be every emotion so freely and openly. The most wonderful part was that almost all of those emtions were happy.  I was jealous and inspired all at once.  So often I feel like I have to pretend I feel something I do not.  I have to look calm when I am electrified inside.  I have to keep my face passive when I really want to scream.  I have to smile when I want to melt into a sobbing puddle on the floor.  But if the Little Red Caboose can find his the strength to push an entire train up the side of a mountain, I can wake up tomorrow, get out of bed and put one foot in front of the other. 

 

The Fabulous Firework Family

It has felt like a non-stop firework show in this house.  Since Nettie was admitted to AI two weeks ago it has been sickness after sickness in this house.  From what I hear, it has been the same in many other households too!  In that we are similar, the only addition being that Lil's condition adds an enormous amount of stress and fear.  She has not had any of her immunizations since six months of age due to her condition ( I am a mom who follows the traditional schedule under normal circumstances- no judgments intended for those who do not).  It has just seemed like every time someone gets better, someone else gets sick. 

On top of that, Lil developed a rash on her face.  It could be nothing but with Lil we are always cautious especially with skin issues.  I took her to the 'doc in the box' (Silverside Medical) and she was diagnosed with a double ear infection.  The same day I was diagnosed with strep along with little Chris.  Strep kicks your butt let me tell you and it happened on the same weekend that one of my very best friends was here to visit.  You know the kind of friend that can make you laugh at yourself even when you are at your absolute worst?  Well, she is that friend and I never get to see her as she lives in Long Island New York.  My kids were sick, I was sick and she made me dinner, made me laugh, slept on the couch and spent time with my kids.  Love her. 

Since then, Lil has had some lesion activity and vomiting to go along with it.  Things calmed down after starting her antibiotic, her disease acts up whenever she is fighting off another illness.  However, to follow the same pattern, she began vomiting regularly Tuesday.  Sound familiar?  Exactly ten days after receiving her Humira injection she begins the whole cycle until the next injection.  This time, the vomiting has been worse.  I woke up this morning to a crib covered in vomit.  Somehow she managed to wake up in the middle of the night, projectile vomit across her crib but pretty much keep the area in which she was sleeping clean and go back to sleep without waking me up. 

One option we have is to give her the injection every ten days instead of two weeks.  I am so reluctant to do that because she is already receiving a dose much larger than she should be.  There are tow reasons for this.  One is that the injection only comes in two doses and there is no other way to dose it out.  Humira is not FDA approved for a child Lil's age so they do not make it in that dose.  The second reason is that her disease is so bad, she needs it.  Now we may have to increase that even more which just makes me sick.  The lesions look better now but she is complaining that they hurt a lot and I have been giving her Advil around the clock. I hate giving her that much Advil.  I know how bad it is for her stomach.  However, when I compare it to the medicines she was on I guess it is not too bad.  I did have a long talk with someone I know who uses natural remedies.  I am a big proponent of natural remedies.  I am just so afraid right now. Traditional medicine is like a life preserver for me and I am holding on tight.

 

 I have been a little down in my own self pity these past couple of weeks.  Tuesday my aunt graciously invited me and my three kids over.  They spent the day tearing up her house and eating her food.  She did it with a smile on her face and without getting outwardly frustrated.  Yesterday another best friend of mine forced me to get out of the house and take the kids to the zoo.  These may seem like pretty innocuous things but getting the energy up to take all three kids out after cleaning up after LillyAnna and getting her medicines ready and then the normal stuff involved in getting three young kids out of the house is no easy feat and when I feel down in the dumps, it is even harder.  I am so glad I have these people and so many others who do so much for us.  Keeping us on this side of sane takes people who love us that are willing to do these kinds of things and so many others; babysitting, listening, sympathizing, taking the twins to do something special, visiting us and occupying them or holding the baby, did I mention babysitting?  SO many things break up the monotony of vomit, lesion obsession and medication.  Thank you all. 

Chris and Nettie in the petting zoo.

Nettie doing her impression of the tiger!

Charcoal Pit....

 

Esio Trot

According to Dr. R's scale, LillyAnna has gained a whole .3 ounce in the last month and a half!  Does not sound like a lot but we are on the gaining end and that is a good thing.  She is eating like crazy and getting mad when she has to wait for her food.  I just cannot understand the swing.  How she goes from not putting one piece of food in her mouth for almost two months and vomiting every night to yelling at me (no matter how cute she looks) from her high chair because I put the kids' food in front of them before giving something to her.  It blows my mind. 
The other thing that blows my mind is that this kid is the healthiest immuno-compromised kid I know.  I know more of those kids in this life than in my former life.  Nettie had a terrible stomach virus, little Chris now has strep throat and LillyAnna is the happiest I have seen her in a long time... a very long time.  She is continuing to vomit but not as frequently and in smaller amounts since her Humira injection Friday.  I gave her the last dose of steroids yesterday.  Her bloodwork last week looked great.  Unfortunately, for some wild and crazy reason, she developed some papules and a pustule today.  I will NOT be pushing on them:)

I know these do not look that bad especially comapred to the pictures of her original lesions at their worst but to me they mean that we do not have her disease under control and if this medicine does not work, we are running out of options.  And the bottom line is her body is attacking her skin and who knows what else.  Every time I think I understand what is happening or I think I see a patterm, something happens to throw it off.  Dr. R and I suspect that her disease will act up whenever her immune system has to work hard, for example when she is fighting off possible strep or when she is cutting teeth (four coming in right now).  I am very happy she is pleasant.  Even though she gets mad whenever I touch her 'boo-boos' and she is saying that they hurt (she points to them and says owie), she has been very happy and it only takes advil to make her feel a little better.  I can handle that. 

Cloudy with a Chance of Meatballs

If meatballs started falling from the sky I would not be surprised right now.  LillyAnna's blood work looked very good this week.  She is all cleaned out according to her latest x-ray and we can do maintenance support for her bowels.  We saw Dr. R this week and are able to stop Lil's current dose of steroids after tomorrow.  We had two whole nights where Lil did not vomit and slept through the night for the first time in months.  On Tuesday, which was ten days from her last Humira shot, she began throwing up again at bedtime.  Since the Humira Friday, she has not vomited.  Yesterday she had probably the best day I have seen in a very long time.  Other than cutting a couple of teeth and needing a dose of advil, she was happy and hungry.  Last night she happily munched on a plate of spaghetti and meatballs for about an hour and then had a cookie!  Again, it is obvious to me that she has starting eating in earnest even though none of her medications have changed but her disease is in control for the most part.  I spoke to Dr. R about this and he agrees that her vomiting is related to her disease somehow but we do not know how.  It is not worth putting her through any tests (not that there is really much we have not done) because our course of treatment would not change at this point.  He said he really wants to see what happens as we remove the medications she is taking and see what happens but rhuematology was adamant that she stay on the Humira for a whole year after we see no disease activity.  Because she is eating and drinking so well I have stopped giving her the Periactin.  This way, when we need it again it will have the affect we will need it to have. 

 

Of course, the two nights LillyAnna slept Nettie was up most of the night and as many of you know ended up staying in the hospital for a few days.  Antoinette has always complained of stomach upset/pain.  Due to this and her slow weight gain, her pediatrician and I are getting some tests done to figure out what is happening.  In the midst of this, Nettie got a stomach virus which exacerbated whatever is going on and she quickly became dehydrated.  Needless to say, she was not happy about it but I think it helped that we were able to stay on 3F where she knows most of the nurses and doctors.  They are just so wonderful there.  





 

While I was there I had a chance to talk to the ChildLife Therapist with whom Chis and Nettie work.  We discussed how life for siblings of chronically ill kids is just different.  While most kids play house, mine play 'sick babies' and half of the doctors kit they own is made of actual medical supplies.  But that is only half of it.  Little Chris takes on so much worry about LillyAnna.  While Nettie was in the hospital, she and Chris slept apart for the first time since they were born.  Then he had the pleasure of coming down with the virus Nettie had and was up about nine times last night.  Being Chris his fever was gone, he ate and was up playing all morning.  Two down, three to go! 

 

She picked this outfit out herself!

 

Whoever You Are

I realized something today.  A phenomenally large group of people separately mentioned to me all in the past three or four days that I do so much for other people.  When people you love, respect and know you well all say something to you that is similar in such a short period of time you should pay attention.  The funny thing is I feel like I am not doing enough for other people.  I did not get my best friend a birthday present, I forgot to call someone who just had surgery, someone I love  just lost someone very close to them and I did not even think to call them for three days in a row.  I could make this list go on and on.  Probably the most difficult is that I feel like I am neglecting my husband.  I seems as if I just don't have any emotion left.  Having to watch your baby go through something so painful and actually be a part of that pain kills something inside of you.  One of the reasons I started this blog was to write about LillyAnna's dressing changes.  You may want to stop reading here if you have a weak stomach.

 

 

You have seen the pictures of LillyAnna's lesions.  They went all the way down to her muscle.  I once asked the dermatologist how painful they were; I began by stating that the most painful thing I could imagine is a serious burn.  She said this was much, MUCH worse than a burn.  By the time we left the hospital Lil had had a total of 30 lesions.  The biggest was her leg.  Every other day, we would have to change her dressings.  Finding dressings that will stay in place, prevent infection and provide some kind of comfort was not easy.  The dressings we ended up using were slightly sticky.  Every other day, I would spend an hour or more preparing for the dressing changes; getting out and cutting various sized bandages, sterile water, gauze pads, any ointments we were using, extra blankets in which to wrap her to hold her down, and more.  Most importantly I would line up the things we hoped might soothe her: pink and green soft blanket, pink diamond ring rattle, cell phone with video of Poppop and Mommom singing, Monsters Inc. ready on the hospital TV... Even though she was already on Morphine every four hours we had to prep her with extra medications.  I am not even sure any of it mattered because it did not stop her screaming.  Timing this was sometimes difficult because the amount of people who attended these sessions numbered anywhere from six to twenty.  It took at least six of us to do it.  She was 9 months old when it started.


We would start with the PICC line lesion because the IV team had to be there (this was something they had never dealt with before).  The PICC actually caused the lesion and made it worse.  PICC lines go straight to the heart and are so sensitive to infections that can kill.  Because of the risk and the pain of having a tube going into and laying on this lesion, we did this first. 

We started by papoosing her.  She hated it and this is when the screaming would began and the little door that allows feeling into and out of my heart would have to slam shut.  As the nurses held her down, my job was to ineffectually try to soothe her.  After about a month I would sometimes help with the actual changes.  We held her tiny squirming body still while we peeled off this slightly sticky bandage and shot sterile water at her completely open wound with a huge syringe.  It was important to peel very very very slowly so that new skin would not detach.  We were not always successful.  Once they were off, we all examined them and take pictures.  In the beginning we did each one individually because the air hitting them added to the pain but after awhile we did them in groups.  Then back to holding her down to replace bandages and rewrap, cover with babylegs and clean up the bloody mess.  The whole process (excluding set up and clean up) took anywhere from one to two hours.

Everyone involved fell in love with Lil and did an absolutely amazing job, I have never seen anything like it.   Tom would even warm up the sterile water so it would be less of a shock to her skin.  Afterwards they would hand her to me so quickly.  And rush to get me anything that would make her feel better.  They almost tripped over themselves to settle her and me in.  But sometimes I felt like, why are you giving her to me?  I can't fix it?  I can't take away her pain?  You did this and I helped.  She does not want me either.  Sometimes in the middle of it she would simply fall asleep for a few minutes.  I think she was possibly passing out.  I was terrified that she was going to develop post traumatic stress disorder. 

I recently went to a funeral and met a psychologist who worked in a NICU.  She was asking me about Lil's disease and at one point mentioned dressing changes, she simply said it must have been terrible.  That is not usually a topic people think about.  I looked up at her and met her eyes.  She leaned over, put her hand on my arm and said, "I am so sorry."

These memories haunt me.  I remember every second of it.  I will never forget what it felt like to hold her down and cause her so much pain.  I replay it in my mind when I cannot sleep at night or when I am rocking her before bed and she is so peaceful.  I want her to know how very sorry I am for hurting her. 

I think that maybe I have been putting so much pressure on myself to do things for others because I have to prove how strong I am.  I have to prove it to myself, I have to prove it to you, I have to prove it to that baby.  If that strength falters, if I show weakness, then maybe a weakness will actually appear.  If that happens, it will grow and that strength which I have used as a shield around my aching soul will crack and crumble in on itself.  I will be exposed as a fraud.  I am weak.  I hurt my own baby.  I hurt her and I held her down while others hurt her.  I am a piece of shit and I don't deserve your respect, kind words, actions or even thoughts.  This strength is not real.  I am a fraud. 

I Love You Stinky Face

The past couple of days has been pretty awful.  Not to sound like too much of a Wendy Whiner but it just does not seem fair.  For almost a week, LillyAnna has been vomiting at least five times a day.  Now that we have begun 'cleaning her out' she is also pooping.  I am glad we are getting it all out but stinky does not even begin to describe it!

And of course, through all of this I offered to make my sister her birthday dinner.  I know you might think that is absolutely insane but let me explain.  My sister, who turned 31 years old today actually, has been in a nursing home for years.  She is my only sibling and my mother passed away three weeks before the twins were born.  My sister just recently left the nursing home in the hopes if creating a better life for herself.  Because peoples' problems still exist even when you have a chronically ill child, I want to support her as much as I can.  And so, because the only thing more difficult than taking three kids out to dinner (one of which is puking, pooping and has to take six vials of medication on Friday nights) is cooking dinner, I offered to do it.  I offered before the poop fest began and it probably wasn't a good idea.  I told myself that all morning while I was running around trying to find pediatric enemas and waterproof pads to protect my carpets, holding a screaming fussy baby and cleaning up any number of messes all the while trying to hold back my own tears, anger and frustration.  As any good hostess knows, you never leave everything until that day so almost everything was already made which helped.  In the end, it turned out beautifully.  The food was delicious, my cake was awesome and the company was more than pleasant.  Lil perked up and the twins were so exhausted by the end they happily went to sleep. Everyone who came was a huge help too.  My aunt came early to occupy and dress kids, my cousin and sisters ran them around and loved them to death, I had a dishwasher and cleaner uppers... It all worked out so well.

I just felt so helpless, frustrated and alone watching her go through this mess.  She would choke, gag and finally vomit, squirm around and sit up (it reminded me of mornings during my college years) when I knew all she needed to do was throw up, watching her try to push and push to get it out or try to drink only to vomit in her mouth each time.  And on top of it all she was miserable.  As I have said before, that is what gets to me the most.  This baby has been through so much and again I have to mention that I know there are babies and kids who have been through worse but this is my baby and her reality and it has not been pretty. 
She is still not cleaned out as well as Dr. R would like so we are going to continue with the Miralax.  I did have to give her an enema yesterday (boy that was fun).   However, last night she did not throw up and seemed to be feeling much better and this morning she ate some breakfast potatoes and was blowing kisses (see video below).  I am not sure if it was the Zofran (which is an anti-nausea medicine that she gets on the nights she gets her Methotrexate) or the cleaning out but she did not throw up!  She went right to sleep and I was able to lay her in her crib peacefully for the first time in weeks.  I wish I could describe the relief that washed over me.  My strong desire to shove Ben and Jerry's into my mouth each night after being so careful about what I ate all day was one clue that I was slightly stressed after getting her to bed.  When I was able to lay her down with her body relaxed and quiet, walk out of her room and feel confident that she was going to stay quiet I felt like I could finally take a deep breath. As it turns out, those breakfast potatoes are the only things she ate today. Oh, and one tiny bite of meatballs. But she drank and was happy and is now sleeping.

The Giving Tree

Enough said