Gloria's Diagnosis Journey

    Gloria was born in September of 2013 at about 5:26AM.  Gloria was full term and her birth statistics looked good at 7 lbs. 13 oz, and 20.5 inches long.  She struggled right out of the gate with breathing needing additional suction and spent most of the first hour of her life on the incubator machine.  She struggled with feeding and vomited a lot in the early days.  On her first follow up doctor visit after birth the signs that all was not right began to show.  Gloria had a "galloping heart rhythm,"  and that is when the fear set in for me (Gloria's Dad).  We then saw a cardiologist that said Gloria had some heart defects including pulmonic stenosis and atrial septal defect.  The doctor assured us that the problems would resolve themselves.  The largest issue was feeding Gloria and we quickly ran into the problem of not being able to keep enough food down her to keep her going, and Gloria slid into failure to thrive.  Then one of the scariest moments of my life happened on New Years Eve 2013.  Gloria vomited and aspirated and stopped breathing.   We could not get her breathing again for nearly a minute and I eventually manually suctioned her and got her breathing again.  A tumultuous night in the ER later and we were back home with Gloria still not eating enough to gain weight and we were increasingly getting desperate.  Feeding was taking up nearly 15 hours a day.  We called in the cavalry and I made a tough decision to leave my job as a teacher and move my family to Oregon so that we could be closer to Jennifer's family.  We put our house and car up for sale, I gave my notice and we were turning a page in our lives.  

    Then on Valentines Day 2014, and the last day of work for me incidentally, Gloria started to have nystagmus (darting eye movements at her morning feed).  Jennifer immediately called to get her into the hospital and Gloria was transported to the Kaiser Hospital in Oakland, CA.  The doctors ran an EEG, and I set a land speed record driving from the Central Valley to Oakland, CA.  The doctors diagnosed Gloria with Partial seizures put her on Keppra and kept her for observation.  Then the seizures became increasingly more concerning, with longer and longer seizures.  Finally the seizures topped out at about 10 minutes. I was ignorant of how damaging long seizures can be and if I had known I would have been demanding seizure rescue medications be delivered.  Then without a second EEG her medication was changed to Phenobarbital, which masked the complex seizures that were developing.  This medication is not what should be used on infantile spasms patients.   We stayed at the hospital with Gloria's condition worsening.  I got the feeling that there was a conflict among the doctors as to how to handle Gloria's case.  We had also sold our house and basically if we were discharged would have to drive Gloria ourselves to Oregon (she was too unstable to drive that far).  In the end one of the doctors made a decision to transport Gloria via air ambulance to Doernbecher Children's Hospital in Portland, OR.  That doctor saved Gloria's life and gave her a chance at a decent quality of life, and I still tear up knowing that doctor really put himself out on a limb to save Gloria.  People often ask me why I did not pursue Kaiser for all the things that they did that were wrong or down right terrible...my response is that for now I really want to protect that one doctor who took the chance to save Gloria so that the next kid that needs saved they will not hesitate to do it again.  We boarded the lear jet and off we went.

    The plane ride was nerve wracking for me, but the pilots, nurse, and respiratory care assistant were awesome, and I am exceedingly grateful for them getting Gloria safely to the Children's Hospital.  Once we got to the Childrens Hospital I showed the neurologist the Gloria IS Video (I have posted this video with the hope that it will save other kids).  I could tell right away something was very wrong.  My heart sank and I knew the second she ordered an EEG immediately all was not right with the world. In fact I, half felt, we were only going to be at Doernbecher a few days given the push to discharge Gloria at Kaiser in Oakland...it would be another 3 months before we would go "home" for good.  Gloria got another EEG, which led to the bob sled team picture below.  You know when two doctors walk in to give you test results you are not going to get good news.  My heart sank as they told me Gloria has infantile spasms.  I felt that ringing ear, you just got hit in the head feeling (I played a lot of football growing up), and I just cannot put into words how devastating it was to hear this.  I had actually looked IS up before when people had mentioned going to a neurologist, and I just remember not wanting her to have that just not that God, but there it is she has IS.  I then had to tell everyone including my wife, and at this point I was not holding it together very well.  The doctors immediately took her off phenobarbital and said that they were going to start Prednisolone, and I know what that drug does to adults and when they told me the dosing I was floored. I then began researching burying myself in research articles and studies and trying to figure out what to do.  I found that the three treatments that were used are Prednisone, ACTH, and Sabril.  I looked at the success percentages of the studies and chose to do them in that order.  ACTH is exceedingly hard to get, because the company that makes it price gouges crazily the cost of ACTH has gone from a few hundred dollars a vial a decade ago to over 30,0000 dollars.  This NY Times Article shows the level of disgusting greed that lies within this story, not only that but the FDA allowed Questcor to buy out the competition an artificial form of ACTH that is used in Canada and other countries that only costs a few hundred dollars. Questcor is able to sleep at night, because they have a program through the National Organization of Rare Diseases (NORD) that gives the drugs for free to families that can't get it through their insurance etc., but that program can take a week or so to process and in that time kids die and are permanently brain damaged from seizing.  Gloria's delay in treatment due to the misdiagnosis by Kaiser led to her losing her suck swallow and having to be tube fed.  Treatment delay of hours has consequences, and it pains me terribly watching parents who really need the drug ACTH have to go through so much to get it.

    OK off my soap box and on with the story.  Thus, it was going to take time to do ACTH, and prednisone was right there cheap and ready to be taken, and time is so important with IS.  We decided to do Prednisone with vitamin B, because some forms of IS react to vitamin B.  We were then discharged to the Ronald McDonald House in Portland (which is an awesome charity full of caring people that help so many), because we couldn't go far from the hospital.  During the discharge process the social worker walks in and says you aren't going to believe this, but Kaiser is refusing to pay for your suction or feeding tube equipment.  You will have no way to feed your daughter unless we can get this figured out (Translated they can't discharge us till it gets figured out).  Not only is this illegal not to cover enteral equipment, Kaisers misdiagnosis is what delayed treatment causing Gloria to need the feeding tube in the first place.  The irony and anger was flowing pretty well at that point.  We got it "figured out" and by that I mean I paid with a credit card for all the equipment myself, because I had to be able to feed Gloria.  That is just one of the many debacles I would go through with Kaiser over the next few months.  The one bright spot with Kaiser, was the primary care that Kaiser Northwest had...he was so awesome and he was integral in saving Gloria...you know who you are thank you.  Also, many of the residents and primary cares that rotated through the hospital were great as well and took a role in supporting and helping Gloria.  Back to Kaiser though.  Kaiser California also refused to pay for many other things, and said it was because we were not in Kaiser California's area even though they were the ones that sent Gloria to Portland, OR.  It was an angering mess for the next year.  As I sit here writing this nearly a year later Kaiser still hasn't paid nearly 5,000 dollars that they agreed to pay in writing and on the phone with Doernbecher...Argh.  We were readmitted on and off over the next 3 months.  Gloria got her nasal jejunal tube inserted and pulled it out a few times, and we had to go back to have it replaced.  She had some breathing problems that landed us back in the hospital.  We tried to drive back to  Roseburg once on directions from the doctors, and were ambulanced right back after Gloria started retching over a hundred times a day with the NJ tube.  It was a difficult time.  We then had it out with the surgeons over trying to get Gloria's fundoplication and mic key button placed, and I hauled out the football coach in me (I apologize surgeons) trying to save Gloria because she was suffering so much.  In the end we got the best surgeons doing Gloria's surgery, on their advice, and they placed the gastronomy tube and did the fundoplication.  Afterwards Gloria gained a lot of weight, and now she is in the 50th percentile.  We went home after the surgery and have only had to make follow up visits and checks at Doernbecher since.