Jack’s “Attacks”

Like everything else with my son this is not a short story to tell.  I apologize, but keep reading if you are interested in the story.  In September of 2011 Jack started attending a local daycare center; he was almost 2 ½.  Within a few days (likely a timing coincidence) Jack was in the ER late one night with a horrible cough.  Croup was the diagnosis, and if you’ve ever had a child with croup you know how horrible that cough sounds.  A breathing treatment, steroids and several hours of waiting to make sure it didn’t start again and we were sent home.  October, almost 4 weeks later and the same thing happened.  November and December, the same diagnosis, the same routine.  Four months, four visits to the ER and a large medical bill accumulating.  When one of Jack’s pediatricians mentioned asthma and allergies I immediately made an appointment with the doctor whose office had treated 3 generations of my family – Jack would be his 4^th generation.  I had great trust in him as a medical professional and hope that he would find a cause for these late night coughing attacks. 

Unfortunately a basic allergy test showed Jack had allergies to cats, but nothing else that might cause such a reaction.  He was diagnosed with asthma (sorry for passing those genes on Buddy).  January and the same event occurs.  This time the ER stops requiring us to wait the requisite 2 hours before leaving and only make us wait an hour (get, now we’re members of the frequent ER visits club).  February and another “attack” as I’ve taken to calling them.  Now I’ve convinced the ER to try using just the breathing treatment and not the steroids; great it still works.  They send us home after the treatment kicks in without making us wait knowing that we will bring Jack back if he “rebounds” either that or they are sick of our company every month.  It was then that I asked our doctor about giving Jack a prescription for the breathing treatment medication (racemicepinephrine).  I cannot afford to go to the ER every time he has an attack, the outstanding balance and accumulating copays are killing me.  Whoohoo, the drug can be prescribed!  Wait one second, don’t be so happy; it’s not covered by insurance.  Out of pocket is still better than an ER visit copay.

The doctor feels the diagnosis Jack receives in the ER is not accurate.  According to him croup is not a recurring malady.  There is something else causing Jack to have these coughing attacks.  Jack is referred to an ENT (ear, nose and throat doctor).  While Jack has seen this doctor before (Jack was tongue tied and had to have that corrected in addition to having been seen by him in the NICU when he had difficulty breathing after having hernia surgery) I am still a little put off when he looks at Jack for a short period of time and says Jack needs his tonsils and adenoids out, tubes in his ears and to have a procedure done to look at the structure of his throat.  No, no, why and what’s that?  Well at least the good news is the attacks have stopped.

Well I fought the doctor about taking his tonsils and adenoids because Jack wasn’t having sore throats, and while he was getting sick fairly often he had only been in daycare for less than a year; he still had an immune system to build.  And as for putting tubes in his ears, again I fought that because he never complained about earaches (just once in fact).  We went ahead with the procedure – a laryngoscopy and a bronchoscopy. “Laryngoscopy is an examination that lets your doctor look at the back of your throat, your voice box (larynx), and vocal cords with a scope.  Bronchoscopy is a procedure that allows your doctor to examine your trachea and lower airways.”  The thought was that because Jack had been on a ventilator after birth that perhaps there was an issue with the structure of his throat.  Even as he was walking into the operating room he was still trying to convince me that Jack had large tonsils and they were likely why he was having coughing attacks.

After having only been in the OR for fifteen minutes the doctor found a cyst near Jack’s larynx.  In the time it took for us to talk with the doctor about removing it (only a few minutes), in the OR Jack’s throat was swelling shut.  After he was done in the OR, the doctor explained that it was only a short period of time and that the swelling was sudden and severe.  He was now of the opinion that it was likely not the tonsils causing Jack’s coughing attacks and that the “attacks” were likely his throat swelling shut!  Can I just tell you how scary it is to realize that for a year you just thought your child was having coughing attacks and couldn’t catch his breath, only to find out it is actually his throat swelling shut?!?  It was possible that with the removal of the cyst that the attacks would cease.  But he had no opinion on what exactly was causing the swelling.  I was hopeful for about a week – until Jack had another attack – his first in six months.  And then four weeks later.  And then four weeks later.  And then . . .

I know this sound like an allergic reaction – throat swelling, but I have been told it is not.  The biggest logic of that conclusion being that if it were an allergic reaction it would be happening more often.  There is nothing remarkable that happens before Jack has an attack.  It’s not like it always happens when we go to the zoo, or go to someone’s house.  He doesn’t try any new foods before the attacks.  They have always occurred in the early morning hours and without warning.  If it were an allergic reaction it would be happening more often because it would likely be being cause by something in the house.

This past summer I took Jack for a second opinion with an ENT at Lurie.  Two things came of this appointment – one, he wanted to do his own laryngoscopy and a bronchoscopy, and two he placed all his money (so to speak) on the fact that Jack had reflux.  I was okay with him doing a second procedure; however I wasn’t so convinced of the reflux.  While I left with a few prescriptions to treat it, I decided against filling them.  The main reason was that during the procedure the doctor would be able to tell if Jack had reflux (he would see evidence or irritation in his throat).  I didn’t want to subject my child to medication without a true finding of reflux.

I can still remember the doctor’s assurance that it would be a minor procedure and Jack would be going home that afternoon.  Oh how cute it was that he truly believed that.  I, on the other hand, was all packed for at least an overnight stay.  I will always remember that doctor coming into the room after the procedure.  Again, without having done anything invasive, jack had sudden and severe swelling of his throat.  Again, another doctor who was so sure of himself was knocked down a peg by my son (just realized that Jack’s been doing that since before he was born).  This time, however the reaction concerned the doctor so much that he sent Jack to PICU.  One thing he couldn’t do though is verify Jack had reflux.  However he still wanted to put him on medication.  Two medications; one once a day and one twice a day.  Um, no thanks.  My child gags, chokes and throws up with any medication.  If you can’t tell me for sure that he has reflux then it’s not worth torturing him. 

In the meantime Jack is seeing a new doctor in the asthma/allergy practice as our beloved Dr. Cavanaugh retired in June.  This doctor confirms my belief that it is not reflux causing the attacks.  Jack presents no other symptoms of reflux.  He never complains of his tummy hurting or his throat burring.  He doesn’t burp a lot or gag when eating before an attack occurs.  And after an attack starts and he receives treatment there are no more attacks for several weeks.  The doctor would like to test Jack for angioedema.  “Angioedema is a reaction similar to hives that affects deeper layers of your skin, the tissues underneath your skin, and the lining of your throat and intestines. Angioedema often appears around your eyes, cheeks or lips, but can also develop on your hands or feet, or genitals, or inside your throat or bowel.  Angioedema and hives can occur separately or at the same time.  Signs and symptoms of angioedema include: Large, thick, firm welts; Swelling of the skin; Pain or warmth in the affected areas; Difficulty breathing or swallowing, in severe cases.  Hereditary angioedema is a rare but more serious inherited (genetic) condition that can cause sudden, severe and rapid swelling of your face, arms, legs, hands, feet, genitalia, digestive tract and airway. Signs and symptoms of hereditary angioedema include: Sudden and severe swelling of the face, arms, legs, hands, feet, genitalia, digestive tract and airway; Abdominal cramping as a result of digestive tract swelling; Difficulty breathing due to swelling that obstructs your airway; Hereditary angioedema is not usually accompanied by hives.”

So, here we are.  Jack has had one attack since the procedure almost two months ago.  It would seem that this fall and winter are going to be no different than the past two.  While I have no answers on what causes the attacks, I do have a way to treat them at home – racemicepinephrine.  However just because the attacks have historically occurred in the early morning hours there is nothing to say they can’t occur when he is at school, daycare, the zoo, a friend’s house, etc.  I carry an epi-pen, but I have no idea if it will work in an emergency.  Instead I know in the back of my mind that if one of these attacks occur when we are away from home, his nebulizer and the racemicepinephrine we will be calling 911 and going to the emergency room.

There is another complication that these attacks cause.  When Jack goes in for hip surgery in November the doctor is planning on putting him under general anesthesia and intubating him.  Given his history this will likely lead to an attack when the breathing tube is removed.  Just another complication my poor little guy doesn’t need.