I'll See You In The Morning

LillyAnna had a very difficult night last night. Basically neither of us slept. She woke up about 4 times between 7 and 10 crying out "owie owie" but them went back to sleep. The rest of the night, she did not go back to sleep without me rocking her.  When she is feeling good, she usually sleeps 12-13 hours a night. Today her left side seemed tender but she was also very constipated. Her bloodwork last week looked virtually the same as the week before. I thought her numbers looked high but Dr. R pointed out that they are within the normal range so that is good even if they are at the high end of normal. 

 

I am wondering if it could be something as simple as an ear infection.  Her lesions act up when she is fighting off something else, the waking up at night could be ear pain (or about ten thousand other things!)  But this is how she fell asleep tonight.  

 

Today I found another pustule in the lesion on her right hip. But she is eating and drinking well... I just hope she sleeps tonight. You will be happy to hear that I contacted the Children's Hospital of Cincinnati and I excercised today so I am starting to meet my goals for the week.  But then again when you tell hundreds of people your goals, you are kind of stuck!

Llama Llama Time to Share

I have mentioned before that my attention is caught when many people in my life start saying the same thing.  In the last couple of weeks, the idea of getting a second opinion (or actually a third since we have already been to CHOP) has been a strong current in my converstations.  It almost feels like cheating to talk about this even though I know how stupid that is.  AI has been so good to us and Dr. R is one of the most amazing people I have ever met.

I was talking to an acquaintence who shared her mother's story of a chronic illness.  Now that we live this life, I hear a lot of these stories.  People want to share them and I enjoy hearing how chronic illness has affected people.  That sounds sick but it is true.  Most of the time when people tell me their stories, they are at a point where it has come to some conclusion and I can feel how they have found peace in whatever that resolution happened to be.  Sometimes it was a happy ending and sometimes it was not; but always, there is an intimation of the way the person's life has evolved from having been through it.  The story went that her mother had been very sick for a long time.  The father and mother were making most of the medical decisions but after ten years of watching their mother slowly die, the children stepped in and took over.  She said that her parents had not had second opinions and when questioned they talked about how much they loved their doctor.  Of course there was much more to the story but my point in mentioning it is that I felt the need to defend her parents' decision to stay with their doctor and in doing so I realized that my reasons for dragging my feet on this same issue just plain suck.  It is safe to stay where we are, it is easier, it is comfortable, she is doing 'ok'.

In my heart, 'ok' is just not good enough.  What really sticks out to me is that she is so young and if this condition could be associated with another disease or does not miraculously go away, we owe it to her to find out as much about it as we can.  Although I understand what the doctors are saying, it is not ok with me that she vomits almost every night, that she pulls on her legs and feet and says owie.  It is not ok that she conitnues to develop pustules.  Having someone else look at this will not hurt anything as long as we are smart about it (I hope).  The National Institute of Health contacted us in December about being a part of their research.  Although not much, there are some things I can try to do to move this along. I also need to call some other children's hospitals and see what happens. 

This is a small pustule she developed this week.

For now, Lil had another reaction to the Humira.  As per my google research, I tried moving the injection site to her leg.  I had been giving it in her arm but moving the site did not help as others stated it helped them.  A dose of benadryl last night seemed to help the swelling and pain.  The same night as her injection she vomited immediately after receiving her Methotrexate even though I had given her Zofran in preparation.  So we had to give her another dose the next day.  Her temps continue to be consistently on the high end although they do not go much higher than 100.7 degrees.  She ate well today and had a great time playing outside in the beautiful weather.  She was very fussy this morning but as I mentioned to Chris, sometimes getting out of the house distracts her and she feels better.  That happened today and if she can be distracted it is not that bad.

Goals for this week: write letters to local politicians, call hospitals in Boston and Cincinnati, excerise at least four times and maybe go to yoga at least once (because it makes me feel good). 

A Wrinkle In Time

I was asked to write this for an extraordinary friend who is doing an extraordinary thing for our family.  I am sharing it here because it gave me some perspective to write it.  I also did not realize how much I am doing until I wrote it down! LOL! I am also very tired and did not proofread yet!

On June 19^, 2012 we found a small pimple on LillyAnna’s thigh.  At the time, she was nine months old.  Until this time, she was a healthy happy baby who ate well and gained weight appropriately.  Within a week there was a lesion about three inches in diameter that went down to her muscle. 

 

As per our regular pediatrician, we were treating her for MRSA.  We were told to take her to the Emergency Department if she developed a fever.  Ten days after the initial pimple, she developed a fever and we were admitted to the hospital.  It took a couple of days to diagnose her condition as Pyoderma Gangrenosum.  PG is associated with adults who suffer from Crohn’s disease, rheumatoid arthritis and a few other auto-inflammatory diseases.  However, LillyAnna’s was only the 17^th reported case in infants.  Most of these infants responded to oral steroids and then followed up with some other maintenance medication with success.  These other infants were not followed past two years although we did find one who was followed for five years with no recurrences.  During the first week in the hospital, LillyAnna developed another large lesion on the side of her abdomen and a few other small ones on her limbs and we were forced to insert a PICC line.  The PICC line was necessary because she was refusing to drink or eat and she was on IV steroids.  We also had a lot of trouble keeping an IV line secure.  Every time there was trauma to her skin, she developed a new lesion.  We were sent home from the hospital about a week after our first admission with a PICC line and oral steroids.

It was difficult to control her pain.  LillyAnna was always a happy and vivacious baby who almost never cried or fussed.  These lesions caused her so much pain that she was on morphine every four hours and was taking another narcotic for breakthrough pain. 

 Unfortunately, in the thirty six hours we were home, LillyAnna developed six new lesions that were about the size of a quarter on her limbs.  We were readmitted to the hospital where we stayed for the next two months.  We immediately began using IV steroids again and added some other medications that are meant to lower the immune system in an attempt to stop the remarkably fast growth of the lesions.  In our first week back, she had developed lesions on her face, scalp, diaper area and most painfully at the PICC insertion site.

 

Every other day we had to change her dressings which took sometimes over an hour.  These procedures were extremely painful for her although the hospital did a wonderful job of trying to make her more comfortable by premedicating her, using very special bandages and trying to distract her as much as possible.  Although, even with all of that, it was horrible for her and she would scream the entire time.  In total she had about thirty lesions.  Finally we tried a treatment called Remicade.  While receiving this treatment, we were able to begin weaning her from the steroids and by the time we left the hospital she no longer needed the PICC line and was taking all of her medications orally.  Her eating and drinking were not totally were they needed to be but she was improving.  It was during this admission that the vomiting began.  I started to notice that the days when LillyAnna’s lesions were particularly sore, she would vomit before bed. 

We were discharged from the hospital on Remicade treatments and a plethora of other oral medications in late August of 2012.  I was unable to work as her care took up the majority of our day and we were at the hospital two or three times a week sometimes for a whole day. 

Soon, I began to notice that LillyAnna was having some of the reactions to the Remicade that we had been warned may happen when a child has an adverse reaction the first couple of infusions.  She would get extremely fussy, had rashes on her face and trunk, and was very flushed.  When we went home, she seemed ok.  However, we were supposed to be lengthening the time between Remicade infusions and we could not.  Her symptoms would begin to worsen after two weeks and we would have to infuse again instead of waiting longer.  When we would wean the steroids, she was uncomfortable and the symptoms were just getting worse.  She began vomiting each night and not eating or drinking again, she was very fussy all of the time and her lesions looked red, sore and like they were growing larger instead of healing. 

Finally we had to readmit her to the hospital in November of 2012.  There we found out that she was building antibodies to the Remicade; that is why it was losing its effectiveness and we were seeing a resurgence of symptoms.  She most likely will never be able to use Remicade again.  At this time we were faced with many decisions and LillyAnna was taking her first steps.  We needed to find out why she was refusing to eat and drink because it was such a problem that she was dehydrated and losing weight. Along with the advice of her doctor, we decided to scope her entire digestive tract.  This is something we had avoided in the past because of the fear that we would cause lesions to develop on the inside by causing trauma. 

 However, at this point it was necessary.  The scope did not show us much.  There were some parts of her intestines that the doctors could not reach because they were too small even though we were at a reputable children’s hospital.  Other than that, they did not see much.  There were small signs of what could be Crohn’s Disease but because she was already being treated with the medications that are used to treat Crohn’s, this was non-conclusive.  What they saw could also be signs of a virus.  We also did a modified barium swallow study which again was not very helpful.  It showed that she had reflux which we already knew and can be caused by some of the medications she is on and some slow stomach emptying.  However, she is now on the highest dose of a medication to treat slow stomach emptying and it has not helped.

 Since this time, LillyAnna has been hospitalized one more time for flu and dehydration.  For a child who is immuno-compromised, she has done well this winter.  However, it is always scary for her because she has not had any immunizations since six months.  We work hard to keep the house extra clean which is not easy because LillyAnna shares this space with our four year old twins.  We keep hand sanitizer in every corner and remind all visitors to use it often.  This also means that she cannot go to daycare which is a problem for us. 

LillyAnna vomits just about every night and sometimes during the day.  We still do not know why but the doctor and I have noticed that it increases whenever her disease acts up.  There is really no way to find out what is causing this right now other than taking her off of all of her medication and repeating the scope and other testing.  This is just not an option right now because we do not know what will happen and the disease works so quickly.  A lot of our time is spent doing laundry from these vomit sessions and cleaning the carpet or car.  We do what we can to prevent a mess but as we never know when it is coming and where she will be exactly, it is difficult.  Also, when I put her down for naps or bed, I have to hold her in an upright position for at least twenty to thirty minutes after she has her bottle in order to hopefully prevent her from vomiting.  It does not work all of the time but it is worth it when it does; however, this means it takes me over an hour to put her to sleep twice a day.  Not an easy thing when you have two four year olds running around!

She also gets very constipated which has caused moderate bowel issues in the past. 

Right now, I am taking her temperature twice a day in order to track trends as per her doctor’s instructions.  At any given time of day her temperatures are between 99.7 degrees Fahrenheit and 101.7 degrees. 

Each day I examine her wounds and when they are not too sore, I have been told to massage them for five minutes at a time three to five times a day.  She does not often allow me to do this.  At nineteen months old, her personality is blossoming and she has very strong opinions about her ‘boo-boos’.  I am not allowed to look at them which makes my daily inspection quite difficult.  I believe it is important to pay close attention to the nuances of her disease activity.  No one knows her disease as well as I do.  I believe keeping detailed notes will not only help her but will hopefully someday be used to help others who encounter this disease.  To further this cause, I keep a blog and post to it often.  Also, I can often catch things early because I am watching so closely.  When a disease progresses as quickly as this one does, that means a lot. 

We are at the hospital at least once but usually twice a week.  She gets blood work every week and we usually have some other appointment. 

We are currently waiting to hear back from the National Institute of Health.  They contacted us and would like to see LillyAnna for a research case.  We will need to travel there for four days.  We have also been considering going to children’s hospitals in Boston and Cincinnati to see if they can offer any advice but these types of trips take time and money. 

There is a possibility that the medications LillyAnna is on can cause cancer if she is on them too long so I feel like we are in a bit of a time crunch and there are not many resources for this disease because it is so rare and she is not responding to treatment the way the other cases did.  The possibility that she could have Chrohn’s would give us a diagnosis but she is very, very young to have that disease although it is in my family.

 As of right now, we are deciding what to do about her current medication.  She is on Humira (an adult dose) and Methotrexate (a chemotherapy drug).   We are not sure that the Humira is covering her disease and are considering whether or not to increase her already high dose.  I give her an injection every other week and on about the tenth day after the medicine, she begins vomiting again, her lesions develop pustules and papules, and she seems to be in a lot of pain.  Sometimes she does not make it ten days. 

Taking care of ordering and organizing her medicine is another job.  Some of them have a shelf life and cannot be ordered too early.  Some take prep time at the pharmacy.  Some have to be refrigerated.  It can be overwhelming. 

Her pain is sometimes the worst part.  She cannot tell us where her pain is or what it feels like.  She often grabs at her arms and legs and says ‘owie’ when her disease is acting up which, as I said before happens about every other week.  She also gets very fussy and just wants to be held.  I know this is not an example of her just being spoiled because her demeanor and behavior are so vastly different when her disease is calm. 

We do not know the future for LillyAnna.  There are so many possibilities.  I am focusing on today.

 

Boy

Since my last post, LillyAnna has been about the same.  She continues to vomit at night although we did get a break last night.  I think this is most likely because she did not eat very much that day and nothing for dinner.  Her intake has decreased in the past couple of days and the techs had some trouble getting blood from her today most likely because she had not had much to drink beforehand.  She has also had some trouble sleeping the last two days.  Her white blood cell count is down to 10.4 from 12.5, CRPs have stayed below 0.5 and sed rate is at 8 from 9.  Although these numbers are down, they are still higher than we would like to see except CRPs.  Dr. R and I expected her numbers to be high last week due to her ear infection.  I am not sure how the doc feels about these numbers yet as I have not spoken to him.  I am taking her temperatures twice a day and they continue to range between 99.7 and 101.2.  She is uncomfortable, fussy and not eating and drinking great but not anything I am panicked about.  This happens sometimes when she is fighting off another illness or starting to get sick.  It can also happen if there is something more serious happening. 

Today I found out that a young boy from our support group has passed away.  He had many health issues.  His little spirit will live on in those children who will receive his salvageable organs.  Chris and I did not know this little boy or his family well although we recognize them from the hospital and have heard their story in our support group.  Their story is sad and I have shed more than my share of tears for this little boy and his family.  Selfishly, it petrifies me.  Lil's disease is not life threatening at this time but we know so little about her prognosis.  We know so little about what her future will look like and tonight when she threw up my heart just broke for her.  I held her a little longer than necessary and I cried a lot.  I took a deep breath filled with her beautiful clean baby smell and gave her a kiss.  Hopefully she will sleep peacefully tonight.  At least little Gavin will sleep peacefully now and I pray that his family will someday too. 

It Is Dark In Here

I am writing these poems
From inside a lion,
And it's rather dark in here.
So please excuse the handwriting
Which may not be too clear.
But this afternoon by the lion's cage
I'm afraid I got too near.
And I'm writing these lines
From inside a lion,
And it's rather dark in here.

By Shel Silverstein, © 2004, All rights reserved.

Dr. R gave me strict instructions not to go on the Internet when Lil was first diagnosed.  Although I am a reader and a researcher, at that time I was not even close to being in a place where I would 'Google' Lil's condition.  I did not want to know.  Since then I have looked up pyoderma gangrenosum knowing I would not find much for infants.  It is pretty gruesome but after what I have seen I could handle it.  Lil's lesions were so much worse than anything I found that it did not really matter.  The Internet is a wonderful thing.  If you think about it for even a second, you will come up with a list of amazing things about the Internet I am sure.  However, when it comes to any illness the Internet can be a very dark place indeed.  More accurately, it can easily put the person searching in a dark place.  After LillyAnna's reaction to the Humira last week, I broke Dr. R's rule.  After reading story after story of people with Crohn's disease and Rheumatoid Arthritis, I am in a bit of a dark place.  It has gotten me thinking about what Lil's future could look like.

Her future could be just fine and that is what I am choosing to focus on, that and getting some semblance of my life back.  I know my life will be forever changed but it is time to pull my head out of the fog that it has been in for the past ten months.  I will not be able to do all of the things I have done in the past.  Lil's care is just too overwhelming.  She has her good days and her bad but even the good days require a lot of brain power and time spent focusing just on her.  Also, I feel like she is still having too many bad days.  There are still way too many uncertainties.  However, this is my life.  This is what it is going to be like until it is not like this anymore.  So I am making some moves.  Horrible times can push you to do things you might not have done otherwise.  I am selling my cakes which is something I have always dreamed of but never really tried.  I am trying to publish some of the children's books I have written which is something else I have always had on the back burner.  I am not naive enough to think that these things are going to save me but right now I have the gumption to give them a try and that is pretty neat. 

 







For Lil's part, she had a good seven days, we did not make it ten days this time.  Since her last Humira injection she has not vomited, has been very happy and has been eating so well.  Until today when she threw up at nap time.  While changing her, I noticed some brand new pustules.  This is unusual because most disease activity I see now is on the old lesions.  These are brand new spots.  She also threw up tonight at bedtime.  She was very fussy yesterday and today but has had no other signs of illness.  I am keeping track of temps as requested. Wednesday am it was 99.7 and that night 101.1.  This morning it was 100.0 and then tonight (two hours after Advil) it was 100.7. 
 

Quick Update 3

LillyAnna's swelling is finally going down and she never developed a fever.  This indicates that she had a reaction to the Humira.  You would think I would be happy that she does not have an infection but unfortunately this news causes me some amount of anxiety.  I am in contact with Dr. R about our next steps.  I asked if we can test her to see if she is building antibodies and somehow stop it before it gets worse.  He said he is going to talk to the rhuematologists and in the meantime, he would like me to take her temps twice a day and note any patterns.  For now, she is happy and not vomiting.  Her blood work looks better than last week.  Happy Baby = Happy Mommy

In this picture you can see how red her cheeks are (which comes and goes), you can see how her face is thinning out and she is eating:)

Oh and in the background is one of the girls I work with who just makes me happy!

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....