One Decade

 First update in a little over 5 years.  

Time has just been passing us by.  The kids are all adults. Incredible things have happened.  And although cancer is one of the scariest things you can hear you child has, that time in our lives taught us many things.  Brought us closer to God during that deep valley.  Created relationships.  Made us stronger.  

Jordon is 20 years old and in his third year of college.  He attends school close to home but works hard every summer to earn enough money to rent an apartment near the school.  He LOVES his life as a college student and still maintains his friendships from high school and is a social butterfly on campus. He makes good grades and will begin the Journalism program in the fall. 

I will do my best to update you with what has been going on.  It's fairly new and intense and I don't quite understand all of it, so here goes... 

A little over a year ago Jordon's new immunologist at St. Louis Childrens informed us that Jordon's IgG levels were still stand alone low and wanted to know if we were interested in doing some genetic testing.  Typically individuals bounce back and their immune systems return to normal within a few years of stopping chemo.  She wanted to see if this was a result of something else.  IgG as well as IgA, IgM, and IgE are all part of your immune system.  When one of them is low it can cause frequent infections or illnesses.  Fortunately Jordon has been on gamma globulin or IVIG for several years and it has kept his IgG levels within normal range. After offering the option to do genetic testing a kit was sent to our house and it was a simple saliva test.  The results were back in a few weeks.  The discovered an abnormal X gene (men receive their X from their mother) and without further familia testing they were unable to officially diagnose him.  The doctor then had a saliva kit sent to me (mom).  After my results came it it was clear to doctors that Jordon had x-linked hyper IgM-cd40 ligand deficiency. Our genetic mutation is the cd40 Ligand deficiency. Only men are affected by this because they only have one X gene.  I am considered a carrier but do not have the disease because I have another X gene.  Because I am a carrier all my children were tested.  There is a 50/50 chance that the defective X gene is passed to children.  If it is passed to a male child they will always have disease.  Females can only be carriers.  This X gene was not passed to my daughters.  What this means for Jordon is that he will never have a complete immune system on his own.  Often times boys are very sick and receive an early diagnosis before the age of 1 and have a bone marrow transplant early in life.  Boys are hospitalized frequently and have to take preventive medication(s).  Jordon never was hospitalized like others and was only hospitalized during his chemo treatments when he was 10.  Before that the only hospitalization was for an appendectomy. The doctors were a bit baffled by this so they decided to do further testing to find out why his immune system seems to be working more than what they've seen with this gene.  His doctor connected with other doctors around the US because Jordon is her only patient with this gene.  It is extremely rare and even more rare to be diagnosed at 20. After many many many blood test and many consultations with doctors around the US, it was discovered that Jordon's immune system works 1/3 of the time.  In males that have this gene, they have never had a patient that has a working immune system 1/3 of the time. That means that it may be possible that others out there have it, have lived fairly normal lives without incident, and unknowingly have this gene.  So they plan on writing a medical journal about Jordon that could assist others with diagnosis. Last week we travelled to St. Louis Childrens to visit with a bone marrow transplant doctor that discussed a bone marrow transplant.  Initially the doctors told us this is the standard of care and would be the only cure for Jordon.  Then after discovering that his immune system works 1/3 of the time and he has remained fairly healthy comparatively, they determined that transplant may be riskier.  Well, last week the doctor echoed the risk, given Jordon's health, but is still concerned because he had lymphoma.  He said with all his research, he could not find a single case similar to Jordon.  He said he researched for two days straight with no success in finding anything similar.  So they are still uncertain as to whether or not the cancer was caused by the immune deficiency or whether it was not.  If it was, they feel that it may be best to do the transplant while he is healthy.  If it was not, the risk is too great.  At this point I am not sure what the doctors will recommend, and I don't know what Jordon will choose.  He is an adult now and will make those decisions.  We will continue to go to Children's Hospital every 6 months and they will continue his care for a couple of more years before transitioning him to the adult side.  When we were told about Jordon's diagnosis it was hard to imagine that he wasn't 100% healthy because he looks and feels good most of the time.  Google is not your best friend in times like these and before understanding Jordon's unique diagnosis the information out there is down right scary and heartbreaking.  There was a few sleepless nights and haunting flashbacks.  

What we do know is that Jordon's immune system is not as strong as it should be.  We also know his body does not build titers to immunizations.  And we know that there are certain common infections that he may not be able to fight off without extreme assistance. The plan thus far is to continue Jordon on IVIG and start him on prophylactic antibiotics.  One high risk area is lung infection so males that have this are given prophylactic antibiotics to keep them from getting lung infections.  So we are prayerful that he stays healthy and those frequently around him stay healthy.  Like I said he is a social butterfly!  We will continue to specifically pray that he remains as healthy as he has been.