Wednesday, May 9, 2012

ALPS

It has occurred to me, after explaining Parklen's disease to yet another nurse.....that maybe many of you don't actually understand whats going on with my baby......

As I sit here watching him sleep in this hospital room , I thought I should take a few minutes to explain his condition to you......I am always asked, "whats wrong with Parklen?" and Many people have the understanding that he is just sick a lot.  Not the case.

Parklen was healthy up until he was six months old......at that point he developed chronic fevers....coming every day, him being my second child, I knew that this wasn't normal and picked up on the fact that "something" was wrong.  He suddenly had golf ball sized lymph nodes in his neck, this led to him being hospitalized in our home town, there, he was misdiagnosed with Kawasaki's Disease and treated.  We were released from the hospital and just a day later Parklen was much worse.  Our pediatrician instantly felt the urgency to get him to Denver as soon as possible.

I can still remember that day like it was yesterday........Parklen was laying in his bouncer in our living room as we rushed around packing our bags.  He was almost lifeless, his lymph nodes had grown more in size and his eyes left no doubt of how seriously bad he was feeling.  Our doctor had given us less than a half an hour to prepare for the trip.....knowing that at that time Parklen had no insurance, he said we could drive ourselves down instead of an ambulance so long as we left quickly.  The drive down was torture....I remember doing a lot of crying.  I remember the sinking feeling of the unknown.

Once in the Children's Hospital, the teams acted quickly to try and figure out what was going on inside the body of our now 7 month old son.  A CT scan showed that Parklen had an abscess between his spinal cord and his airway....it was so large that, given another day, it would have stopped his breathing.  They prepared for emergency surgery. 

We had brought Paysen along and because of his age, Devan was forced to leave the hospital at that time due to Visiting hour restrictions. 

Here I sat, in the waiting room......my son in surgery, no idea what was going on......in a huge hospital, after normal hours.....sitting, alone.  I cant remember being that scared before that point in my life.  After what seemed like an eternity, the surgery completed and a Dr explained that Parklen had some major complications during surgery.....they were unable to remove the breathing tube and he was in a drug induced coma.  The nurse escorted me to his ICU room.....as I walked in, the breath was literally taken from my chest, there he lay, a million tubes coming from every direction, my small baby...swollen beyond recognition.....about 7 or so Dr's, nurses and technicians rushing around him.  My legs went numb, and a nurse helped me to a chair.

Parklen was in the ICU for a week, with no known cause of his abscess, no understanding of his condition.  There were teams of Doctors in and out every day....puzzled and trying to "figure" Parklen out. 

After being released from the ICU, it wasn't a full day before the abscess returned, another surgery......another coma, another week in the ICU.  These weeks were torture...we were unable to hold Parklen, he was unable to communicate with us...he lay lifeless.....and the doctors didn't even feel comfortable telling us he would be ok, because they didn't know.

It was explained to us that the medicine given for Kawasakis Disease completely alters your immune system and affects any and all blood cultures and tests.  If there was something really wrong with our baby, it was unknown.....We were sent home after a month, and Parklen's first Christmas spent in the hospital,  with a decision of "bad luck" as to the reason for his mishap.

We were home for almost five months before he had another bad scare.  I took him to the Dr. for what I can only describe as a "wheezing" sound he was making.....almost gurgling with each breath.  A chest x-ray showed what appeared to be an enlarged heart.  Again our pediatrician sent us south.  It took only a couple of hours before they declared that the sack around Parklen's heart was filled with fluid....it was spilling out, into his liver and lungs.....in essence drowning him slowly.  He went in for emergency surgery.  Again, Devan with Paysen and me alone....with only my fear and my thoughts.

Parklen went a few months before his next hospitalization.......and a little over a year after his first hospitalization, we found ourselves in Denver again.  Parklen was having his tonsils and adenoids removed.....should have been cut and dry.  We were at a different hospital than Children's....the wait was too long for the surgery and his primary care physicians thought it was super important to remove them as soon as possible.  After the surgery, Parklen was having issues breathing.....they did a CT scan and found his entire abdomen was covered with what looked like tumors.  After praying and following my gut, I asked to be transferred to Children's hospital......The oncology and Hematology teams went into action.  Parklen went into surgery....they took samples of his lungs, gut, bone marrow and even taking a lymph node.  It took over a week of testing and waiting.....then the results.  Parklen was diagnosed with ALPS (Autoimmune lymphoproliferative Syndrome)  The doctor held my shoulder, squeezing tightly as he gave me the news.  I felt numb, it was strange to be so excited about this.  But finally....15 months of Parklen being in and out of the hospital....no one able to explain....we had an answer.  I knew that knowing WHAT, would make it easier to deal with it and move forward.  Devan had started college at this point and so I was alone with the news....staring at Parklen, looking at the doctor and I just began to cry.....

ALPS is a very rare autoimmune disease....the known cases are just a few hundred....ever.  Here's what it does....  Everyone has "T" and "B" cells...when we get sick, these cells go and attack the sickness...after which, they tell themselves to die.  Parklen's cells never get the message to die.  They continue to reproduce and eventually begin attacking Parklen's body through his lymph system.  ALPS affects everyone differently...Parklens disease manifests mostly in his lungs and GI tract.  The inside of these organs looks like cobble stone....he, in turn, suffers from a chronic cough, breathing problems, constant stomach aches, and chronic diarrhea.  Every day he takes a medication that they would give someone who receives and organ transplant....this is to try and slow down the attacks on Parklen's being.  In conjunction with this medication and many other taken daily, he receives IV treatments of Steroids to further slow down these attacks.

There is no cure for ALPS....just managing the symptoms.....There are a good portion of ALPS patients that eventually develop lymphoma.  If at some point, Parklen's quality of life were to plummet below "acceptable"   the doctors would want to do a bone marrow transplant.  This is not something I think about often.....the survival rate it less than 60% in patients like Parklen.  His main doctors have said that there is no doubt that Parklen doesn't know what it feels like to feel "good" and the pain is his norm. 

So there you have a brief (not really.....) explanation of Parklen's health.  There is so much more that has happened and that we deal with, but this is the meat and potatoes. 

No comments:

Post a Comment