Saturday, November 3, 2012

There's a Fungus Among Us (Paecilomyces To Be Exact)

On Sept. 23 Collin developed a pneumonia in his left lung despite the IVIG infusions that he had received.  He was hospitalized and the doctors decided at that time to do a bronchoscope and see exactly what was going on his lungs.  They also did sinus surgery to open them up more and put another set of tubes in his ears. 

The day after surgery
He was sent home with a PICC line in his arm so he could receive IV antibiotics for three weeks to kill the bacteria in his lungs, sinuses, and ears.  They told us a couple of weeks later that a fungus had grown from the mucous they took out of his lungs, but they didn't think it would be an issue.  He began to put on weight and seemed to be developing into a strapping young man.  Then on the evening of Thurs. Oct. 25 he started spiking a fever, among other symptoms.  I was on the phone with the doctor off and on through the weekend thinking it was a viral infection and we could get him through it with Tylenol and Motrin.  On Sun. evening his fever went up to 104.1.  We knew at that point that there was something more going on.  On Mon. we took him back down to Joe Dimaggio's Children Hospital where he was once again diagnosed with pneumonia.  An infectious disease specialist was called in to review his case.  We learned that the fungus they found is actually quite common, it is found in the soil and therefore quite an easy thing for a boy to come in contact with.  The problem is that it has been known to cause severe complications in people with immune deficiencies.  So much for my theory in raising boys that "a little dirt builds the immune system".   The doctors now believe that the fungus is causing the repeat pneumonia's.  He was put on strong anti fungals as well as antibiotics in the hospital.  They are in the process of regrowing the fungus so that it can be tested to make sure the meds he is on are effective to kill it.  We absolutely hate the amount of strong medications he is having to take, but we have no other options.
Red Man's Syndrome from an antibiotic

Collin's Not So Common Variable Immune Deficiency

Our tiny little bundle

On June 23, 2001 we welcomed our second son into the world.  He was born four weeks early due to complications with the pregnancy.  He was a tiny little thing, but from the very beginning he had a drive to survive.  By the time he was six months he had his first respiratory infection and began nebulizer treatments.  Since we were living in TN at the time and it was winter we didn't think much of it.  In June of the next year we moved back to FL and his respiratory issues seemed to clear up for awhile.  At the age 2 1/2 we started becoming concerned with his speech delay.  After consulting with his pediatrician we began some intense hearing tests.  We were told that he had permanent hearing loss and was only hearing about 20%.  Around the same time we were also told that he was autistic, had ADHD, and some other disorders such as sensory integration dysfunction.  While none of the tests showed fluid on his ears, the ENT decided to go ahead and put in ear tubes since he was having to remove his tonsils and adenoids anyway.  When he came out to talk to us after surgery he told us he had never seen so much fluid on a child's ears.  It was packed in so tightly that it wasn't detectable on the tests.  After that he was a new child, behaviour wise.  He continued having speech therapy and we felt like he had been given a new lease on life.  We were dismayed when his ears continued to become infected.  He was the kid who always had cotton balls sticking out of his ears.  A couple of weeks before he turned 3 he had his first verified pneumonia.  We were leaving for a missions trip to Haiti and ended up in and out of the doctor's office the week before we left.
In Haiti 2004
The next few years we dealt with repeated ear infections and respiratory issues.  He was diagnosed as being allergic to just about every environmental trigger possible and with asthma.  We were told that they were the cause of the repeat infections.  The poor guy had mucous pouring out of some orifice on his head at any given time.  He took antibiotics off and on to keep things under control and we were told that was all we could do.  Things leveled out that way until the summer of 2011 when were were on deputation.  While we were doing some sightseeing in Boston he became very ill and ended up being diagnosed with pneumonia in his right lung.  He was treated and we went on our way.
In Boston summer of 2011

  That next school year was miserable for him.  He coughed and wheezed almost constantly.  He was off and on antibiotics and in and out of the doctor's office.  Towards the end of the year Harold flew a pediatrician from Samaritan's Purse to Haiti.  During the flight Harold went back and spent some time talking with him about Collin.  He suggested what we had already been thinking, that we needed to see a pulmonologist.  We made it our goal for the summer to get his pulmonary health up to par.  The first visit we were surprised to see how poor his lung function was.  A chest x-ray revealed lots of mucous pooling in his lungs.  He started on some new meds and seemed to be getting better.  We thought "whew, now we're getting somewhere".  He started the 2012 school year with a bang.  Two weeks into the school year he became very ill and we ended up in the ER where he was diagnosed with a severe pneumonia.  After hearing his history and seeing that he had a pneumonia in the exact same spot as a few months before the doctor ordered a CT scan and lots of extra blood work.    The CT scan revealed  bronchiectasis (scarring) in his right lung and a very suppressed immune system.  They now believe that he had pneumonia in his right lung for over a year.  We were told that we needed to get him in to see an immunologist ASAP.  The next week we saw Dr. Gary Kliener, who is now our hero, for the first time.
Dr. Kliener

  After spending an immense amount of time reviewing his history and blood work he told us that he believed Collin had Common Variable Immune Deficiency, an immune disorder that affects 1 in 50,000 people.  His immunoglobulin level was extremely low, causing him to be unable to fight off all of the germs he came into contact with.  It mainly affects the lungs, ears, and sinuses causing them to be infected almost continually.  This explained why he could go off a round of antibiotics and two weeks later be sick again.  The only treatment for the disorder is to have intravenous immunoglobulin infusions every 3-4 weeks.  While it is hard to know that this is something that, unless God intervenes, he will have to deal with for the rest of his life we are relieved to know that there is a treatment for him and hopefully with time he will grow stronger and stronger and be able to live a relatively normal life. 

During his first IVIG infusion