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.