10/30/14 Update

We went for Jordan's second Rituximab treatment yesterday, it went very well once again (thank god). Luckily we were at our local hospital for the treatment, but unfortunately Jordan is the first patient ever to get this particular chemo treatment at our hospital, so things were a bit slow on treatment. We got there around 10AM and didn't leave until 5:30PM. Treatment didn't start until after 1PM, as they got their information together (the chemo nurses); and also for the fact that the infusion room was overbooked, so we were separated into a private room during treatment, so there was a lot of running around for the chemo nurses (hopefully next week we will actually be in the infusion room). But, it was another good week with no reactions from the treatment, pretty much a repeat from last week's treatment.

On a better note, last week, before the first dose of Rituximab, Jordan's platelet count was at 10,000. This week, before the second dose of Rituximab, his platelet count was at 35,000. So I am keeping my fingers crossed that this is the treatment working, and not the heavy dose of steroids he gets before getting his treatment. I am trying to be patient with this, as it can take up to 12 weeks after the treatment is done, before we see any results, so I am trying to remain confident on this. We shall see, but definitely doing the platelet dance here!

Also his petechia is fading away, so thats a plus, and bruises are also healing nicely. Still waiting on the IVIG blown vein bruise to heal from the beginning of this month, that is taking some time to go away.

Otherwise, Jordan is feeling and doing great, no side effects or reactions from the meds he is on, nor the treatment. So definitely counting our blessings on this one.

Will update again next week, prayers and thoughts as usual. Thanks!

10/23/14 Update

Jordan and I headed up to Chapel Hill Tuesday evening and checked into our hotel room. Uneventful night.

Wednesday morning, got up bright and early and headed to Chapel Hill Hospital for a long day.

First thing was an hour long breathing test. Jordan did fairly well, but they think he may have some inflammation around the tissue (bronchial) area, nothing serious, but he may get an inhaler to help him on days that his chronic cough is bothering him. Otherwise, everything else looked good.

After that appointment, we headed up to the hematology floor, and got situated with vitals and an IV placed. They pretreated him before the treatment started with Zofran, Tylenol, Benadryl, and then gave him a low dose of Prednisolone into his IV. He passed out within 10 mins after that and slept about 2 hrs through treatment.

They started Jordan off slowly with the Rituximab drip the first hour and then every 30 mins afterwards, bumped it up by 25mg. He tolerated treatment very well, no reactions, and even this morning, there are no reactions (other than being exhausted, which is for the both of us, I didn't realize how draining yesterday was going to be for us). The entire treatment took about 5 hrs (they told us that the next three doses will be faster-I am going to assume 4 hrs, but I am fine with 5 hrs too); after the Rituximab treatment was done, he got another dose of Prednisolone that took about 20 mins and then we were allowed to go home. So it was a very long day for the both of us, a good 9 hour day at the hospital, and then a 3 hour drive back home.

Thankfully the next three treatments will be done at our local hospital, since Jordan didn't have any bad reactions with the first treatment. I do know that the next treatment is next Wednesday, but I haven't gotten a time yet on when we need to be there; if I haven't heard from Diana by Monday evening, I'll shoot her an email that night.

Platelets dipped more, hence the steroid booster before and after the Rituximab; Dr. Gold did tell me he will be doing the steroid booster after each Rituximab treatment to help out Jordan's platelets. Fingers crossed this treatment works, so we can come off the medications (especially the steroids) and it gives us a normal life for at least a year or two.

Also had Jordan's 504 meeting this morning to set that up, so that went well, and we can move forward, accommodations are below...

Will update next week after our second treatment, continued thoughts & prayers!

Specific Accommodations - Please indicate subject area and accommodation required:
1.	If Jordan’s medical condition interfers with his attendance during standardized exams and local
	exams/assessments; Hospital Homebound teacher will administer exams
	Position of person(s) responsible for implementation: Hospital Homebound coordinator, student
2.	Extended time on assignments, per county policy. Jordan will be provided extended time until the end of
	the next semester to complete missing assignments.
	Position of person(s) responsible for implementation:Teacher, Student, Hospital Homebound coordinator
3.	If Jordan has a low platelet count (below 75,000) he cannot participate in physical activities (PE/Body
	Conditioning/Team Sports/Beginning Tennis/etc)

10/17/14 Update

Well as I predicted Jordan's platelet counts have dropped. Its been two weeks since he got IVIG, and typically thats how long IVIG lasts. Last week his platelet count was at 41,000 and its now at 25,000 (where we were exactly a month ago). Thankfully he has no symptoms, but that changes once he goes under 20,000 (petechia and bruising on his legs). So we will see how the next few days hold up.

Diana called me about an hour ago and we are FINALLY starting Rituxan (Rituximab), our first dose is scheduled in Chapel Hill on Wednesday morning, bright and early. So we are going to try and get a hotel room the night before, if not then Jordan and I will probably leave around 4:45AM to beat the Raleigh traffic (or else we will be stuck in non moving traffic for 30 mins to an hour).  It will be an all day thing, since we have to start the treatment slow, to offset any side effects. Keeping fingers crossed for no side effects and that this treatment works. Not going to enjoy a 5 hr round-trip day of driving thats for sure.

Its a total of four doses, given once a week, for four weeks. As long as Jordan has no issues from this treatment, the other three doses will be give here in town at our local hospital (keeping fingers crossed on that also). I have heard such good things about Rituxan, so I am hoping it works and puts Jordan in remission for a minimum of one year (although I hope longer). It takes up to 12 weeks (give or take) from the first dose to start seeing a rise in the platelets. This would be a huge blessing on our family, financially and emotionally.

I will update you after Wednesday and let everyone know how it went.

Prayers and Thoughts as always :)

Call from Immunologist-10/15/14

Two weeks after Jordan's Immunology appointment, I finally got a call from Dr. Wu, Jordan's Immunologist. Its definitely confirmed that he does have an immune deficiency called CVID.

Basically to give you a run down on what CVID is, so you don't have to google it (LOL), its a disorder that impairs the immune system. Patients with CVID are more susceptible to infections from bacteria and viruses and they can become recurring; also pneumonia is pretty common for CVID patients, so they can eventually end up with chronic lung disease if the infections continuously happens (luckily we have not dealt with any viruses or infections with Jordan). It can also cause symptoms we have dealt with since Jordan first started this journey, such as enlarged spleen, liver, and lymph nodes.

So as of now, I have no clear answers about the Rituxan, everyone is basically waiting for Dr. Gold (Jordan's hematologist) to give the go on that, on when we will start (or if). I am thinking he is waiting on this week's platelet count to determine on when we will start that treatment. Labs are scheduled to be drawn tomorrow afternoon (had to reschedule this afternoon's appointment). I am going to see if we can start the treatment the first week of November, this way Jordan is at school the next two weeks and will be there for his mid-term exams the last two days of October.

However, Dr. Wu has a plan as far as his CVID goes. Since his IGG (one of his immunoglobulins) are low (well they are all low, but we are concentrating on just the IGG), two weeks ago they were at 443, and she would like to see them maintained around the 800 area; she wants to start doing IVIG every 28 days for a couple of months (at the local hospital, through an IV) to see how his IGG levels do, and eventually transition his treatments to SubQ (Subcutaneous Immunoglobulin) therapy at home once a week. This will maintain his immunoglobulins levels, which will help his platelet levels, and he won't feel so fatigue all the time either. I will be trained on how to do the SubQ therapy at Chapel Hill, but after many hours of research and watching the videos on the websites for SubQ, I am fairly confident on giving him this treatment (I swear I need to go to school for this).

When we do travel to Chapel Hill for his first dose of Rituxan, Dr. Wu wants to do breathing tests on Jordan to check his lung function, she also wants to do some blood tests to check his thyroid levels and other things, since CVID patients are susceptible to other auto immune disorders , which is sounding to me like a domino effect. Start with ITP, move to ES, and then find out you have an immune deficiency, to which is the cause of you having ES. So my advice to those reading this, and been dealing with ITP/ES, go see an immunologist and get tested to see if you have an immune deficiency. Had I known this 8 years ago, when Jordan first started with ITP, I would have pushed for more testing, instead of waiting all these years for answers on WHY he has this.

It most likely is genetic (CVID), but no one has approached Brian or I on genetic testing to see which one of us carries the gene, and frankly I don't see a need to, we aren't having anymore kids, and our other two children are fine. So basically this is something Jordan needs to stay on top of, when he has a family of his own, to make sure these tests are done when his children are first born. Because Jordan has showed signs of low blood counts since he was a newborn, but they chalked it up to him just having severe jaundice (low hemoglobin counts), which is fairly common in babies anyway. So there was no need for alarm. So my personal thoughts, he has always had ES/CVID, and like with most patients, it just takes years to show symptoms, and I think that was the case with Jordan.

As far as life expectancy goes......yes, there is a chance we can lose him, but hell you can lose a loved one by them going to the grocery store; so we don't dwell on that, we are enjoying him in the moment. Patients with CIVD live well into their adulthood, it all just depends on the severity and frequencies of any illnesses he experiences; and we have been very lucky in that aspect, because he is rarely sick. So I fully expect for Jordan to outlive Brian and I. :)

Will update in a couple of days as soon as I get lab results and hear from Diana about what is next. As always, thoughts and prayers please.

Homebound Meeting-10/14/14

The meeting went fairly well, was much longer than I anticipated it to be (over an hour).

The teachers, counselor, and Homebound Director came into the meeting with the intentions of two things. Shortening his schedule to only two classes for the remaining part of his semester (taking away his elective classes) and exempting him from all missing assignments in his classes. I said no to both ideas.

Jordan has been dealing with this since he was 6 years old, he does not use this auto immune disorder as an excuse, because we raised him not to; so my intention is to educate these teachers and make sure they don't use it as an excuse either, just because they feel sorry for him. We are not going to just sweep it all under the rug and let him slide by to graduation, its just not happening; he knows he has to work for it, even if it means working a little harder to get there.

I told them, Jordan can do the makeup work, and is willing and able to: I just need for them to actually work with him on it, be patient as he does the work, and if they offering afterschool tutoring, etc., he will participate on that. Luckily they all stay afterschool Monday-Thursday, so its not like they would be making special requests just for Jordan.

What pisses me off about the situation is their expectations with Jordan, like he can't do the makeup work and remain on the A/B honor roll. Again, this is not an expectation we set for Jordan, its an expectation he sets for himself. We don't do anything to make his life even more stressful than it already is, he does that enough for himself. And I told them at this meeting those exact words, just have faith in Jordan, be patient, and he will get the work done in a timely fashion.

He has qualified for the Homebound Program due to his medical needs, I don't anticipate needing the program, unless his blood counts dramatically drop again (fingers crossed). We are impatiently waiting for Chapel Hill to call us to start the progress of taking Rituxan for four weeks (once a week). I did let them know at the meeting that I don't have a start date on this yet, but when we do start it, he will miss at least two days a week for four weeks (the day of treatment, and quite possibly the day after treatment, depending on any symptoms he will have from the infusion). Other than that, he will miss one or two days a month, for appointments. Unless, of course, his counts drop to where he cannot attend school. The teachers were instructed to not count him absent from here on out, because he is now a part of the Homebound Program; so thats a relief.

So we will be meeting again next month, a week after report cards have been sent out. So Brian and I will have a sit down with Jordan tonight, because we have two weeks to get him caught up on his work, before the final grades for the first nine weeks are put into the computer for report cards. Our goal is for Jordan to prove these teachers wrong, that he can do the work, and remain caught up with his peers. I would love to go into next month's meeting and be like "I told you so".

Blood work tomorrow afternoon, hopefully his platelets haven't decrease, since the IVIG is no longer in his system. Fingers crossed....

Will update when I get his results from that, continued thoughts and prayers as we continue this journey with Jordan. :)

Update: Jordan's counselor called and after talking to the 504 coordinator, they feel that it would greatly benefit him more to place him on a 504 plan. So I have a meeting about that next week.

Moving Right Along-10/9/14

Jordan's school counselor called, she said they have never had to deal with a student that has medical issues like Jordan before, one week okay to go to school, the next week not okay...etc. I have a meeting with her and the Homebound Coordinator next week, to see what we can draw up, as far as a plan goes for Jordan, so he doesn't get behind with school.

She reassured me that although his attendance looks bad right now, they WILL BE excused and he WILL NOT get an attendance failure, because its due to medical reasonings; that they will do everything in their power to get him caught up and work with us from here on out.

She said that I have no idea how pleased and refreshing it is for them, to see a parent be so advocate with their child's education, because once kids get into high school, the involvement of parents is lessened dramatically (which is so sad on a parent's part); so she thanked me wholeheartedly on that issue.

So, I am pretty pleased with Laney, and glad that they are so understanding about Jordan's medical condition and are willing to work with us, instead of just brushing it under the rug. My hard work is paying off, by spending hours and hours in emails and making phone calls to be Jordan's voice.

An Increase-10/8/14

Jordan's nurse just called me to tell me the good news, his platelets have risen to 41 (from the 16 on Saturday). Now its probably because of the boost from the IVIG, and we will probably see a decrease in the next two weeks. But for now, I will take that high count, which means a small break the rest of the week for us.

I filed for disability for Jordan yesterday, that took me hours to do. I didnt realize you have to remember every tiny, tiny hospitalization, when he saw a doctor, nurse, etc etc. Even after filling out the paperwork and electronically sending it, did I realize I forgot to put in that a homecare nurse comes once a week to draw labs and check vitals, and I also forgot the strep incident back in April, UGH. Thankfully I still have to sign medical release forms in person, so I will have to make sure to mention that. Fingers crossed, because its obvious I can't go to work with Jordan's medical issues. I mean he has been out of school for almost two weeks right now, and someone has to be with him, incase he falls, hits his head, starts bleeding, etc. No way would a job understand and hire me.

Still no word on when we are starting Rituxan or Homebound Schooling. I do know Jordan's school is waiting on the paperwork to be faxed back to them from Jordan's doctor. As it stands, he has missed 11 days of school, and is looking at an attendance failure, which I am going to have to fight with the school board if/when that happens.

Hopefully I get a phone call soon, I need to remind them about the paperwork, and I also need to see at what platelet count is Jordan safe to go to school, or if he even has clearance to go back; especially when we will be missing a lot when he starts the Rituxan treatment soon.

I am also working on a binder for Jordan's medical stuff. Important phone numbers, when he was hospitalized, what treatments he got, when he had outpatient stuff. Also need to write in every doctor appointment., etc etc etc. Right now I have a 1 inch binder...I will eventually be upgrading to the 5 inch binder here shortly.

Will update when I know more...thanks for your thoughts & prayers!

**Update**

Talked to Diana from Chapel Hill, he has clearance to go back to school for right now, since his counts are higher. She will fax the Homebound paperwork in the morning. I am going to email the school counselor tomorrow, and let her know what is going on. I would much rather do a 504 Plan for Jordan, so I will see what she has to say about that. Diana also is going to shoot an email to Dr. Gold and Dr. Wu about when we are starting the Rituxan, we all feel most comfortable doing the first dose in Chapel Hill, just to make sure he doesnt get any side effects from it. If all goes well, then the rest of the treatments can be done here in town. We won't make any changes as far as his medications go right now, want to wait a week or two before we decrease the steroids, just to see what his body does, once the IVIG is out of his system.

Discharged-10/4/14

Last night's labs before treatment, his platelets were at a 4, so thankfully he went in to get some IVIG and high doses of steroids.

Brian stayed with him last night, neither one slept good, but who sleeps good in a hospital. Especially when they are coming in every hour to check vitals, since he was on the IVIG drip.

I went up there this morning, so Brian could go to work. He was finishing up the second and last bottle of IVIG (that finished up around 10:30AM) and then they started the IV steroids about an hour later, which took an hour. They took labs from him close to 1PM, and platelets went up to 16, not as high as I was hoping for, but it was enough to be able to get discharged.

We will have Molly, his nurse, come sometime Wednesday, and draw labs again. Hopefully there isn't a decrease again (which IVIG is only temporary, so I am expecting a drop again). And we will go from there.

I talked to Diana, from Chapel Hill, last night. And she said that they talked to the chemo nurse here in town, and he was comfortable with doing the Rituxan treatment for Jordan. So keeping fingers crossed Dr. Gold is okay with us doing treatment down here instead of up there. She also informed me I will be taught how to insert a needle into Jordan, incase he may need a weekly IVIG pump each week (I swear I need my nursing degree mailed to me for all this that I do for this child of mine). So that should be interesting. Also she is going to fax the paperwork back to Jordan's school on Monday, so he can start homebound school (teacher comes twice a week to keep Jordan caught up with school work, so that he doesn't get behind).

Thats pretty much it, I am exhausted mentally..so hopefully we have a quiet few days at home.

Getting Admitted-10/3/14

Got a phone call this afternoon from Diana, up in Chapel Hill. Dr. Gold & Dr. Wu want Jordan to be admitted into Hanover tonight to get some IVIG and IV steroids to give his platelets a boost. No news yet on the Rituxan treatment, where/when we start that. Hopefully I'll know more next week on that.

Hopefully Jordan won't be in there past the weekend (hoping no later than Monday). Brian is taking him tonight, and will be with him overnight. I'll come up in the morning and spend the day with him; I have to leave around 2PM, because Brittany has plans, so Jordan will be by himself for a couple of hours, until Brian gets off work at 4PM. Brian will again, stay with him overnight, and I will come up there on Sunday, once Brittany gets home from her slumber/birthday party (which I told her to come home early if possible).

Will update when I get news....

Second Diagnoses Confirmed-10/1/14

So Jordan and I traveled to Chapel Hill yesterday to see the immunologist doctor that Dr. Gold had referred us to. Loved her (Dr. Wu), she was absolutely awesome and took her time explaining everything to Jordan and I, and answering all of our questions.

So the second diagnoses, that would be the underlying factor for Evans Syndrome, for Jordan, is called CVID (I attached a link so you can read it). But basically in English terms, everyone has B cells in their body, they produce protein, which is a good thing. But sometimes they will produce antibodies in your body that aren't suppose to be there (foreign objects). Now typically when that happens, your immune system will kill off those "foreign objects" and that is that. Well when you have an autoimmune blood disorder like Jordan has, and this happens, your immune system goes into overdrive and just kills off everything, the good and the bad (platelets, red blood cells, white blood cells, etc)...and then you get the diagnoses of Evans Syndrome. Hope I explained that well enough for you to understand.

So now the plan of attack is to do four doses of Rituximab; given once a week (I have heard different things, such as once a week, or once every other week-we will see). He will be given this in the hopes of killing off the B cells in his body. Also on top of that, do IVIG. Now Dr. Wu said that Jordan may be one of those patients that needs weekly IVIG (which can be administered at home as an injection-which will be interesting nonetheless).

Basically right now, its up in the air as far as when we start treatment and where (I am just waiting for a phone call to see where to go from here). Again, I am 2½ hrs from Chapel Hill, which is where they are use to doing the treatment of Rituximab. However, they now have a certified chemo nurse at our local hospital that could do the treatment. The problem is, the treatment has never been done down here in my town, so its a matter of finding out if they can even do it here locally. If not, then we have to make weekly trips to Chapel Hill for the treatment.

Anyway, so thats what is going on this week here in the Jackson household. Here are his numbers from yesterdays lab work.

Platelets: 7 (Last week was at 8)
Hemoglobin: 13.8 (Last week was at 13.2)
WBC: 4.6 (Last week was at 5.5)
Bilirubin: 0.6 (Last week was at 0.8)

IgG: 443 (600-1700 is the normal counts)
IgA: 8 (40-400 is the normal counts)
IgM: 25 (35-290 is the normal counts)

Also tested COOMBS positive

Until next time.....