12/4/14 Update-A Year Ago Today

So I am two weeks behind updating this blog, which I guess is good, because no news is good news right?

Its been a year, since we started all this craziness, not the actual ITP adventure, thats been ongoing for 8+ years, but the Evans Syndrome Saga. This was my post on Facebook exactly a year ago:

Scariest day of my life, finding him like that. Images embedded into my brain for the rest of my life of coming so close to losing him. I only hope and pray to never go through that again.

Anyway, not to dwell....

As I said in the last post, everything went well in Chapel Hill, also we did December's IVIG last week here in town, since Jordan is without insurance until January 1st (thats a whole other story @@)

His platelet count had dipped a little, not by much (went from 222 to 202), but as a parent with a child with his disease, any drop is never a good thing, and puts you on high alert. So I am that hovering parent this month, double checking for bruises and any petechia, ugh, I hate being this way.

I am unsure on when we will have our next blood draw, we did decrease on steroids two weeks ago, not by much, went from 16ml twice a day to 13ml twice a day and everything looked good on last week's blood draw. I am going to assume Dr. Gold will want blood work done before Christmas, which is three weeks away (crazy), but we will see. I cannot wait to get him off all this medication. Once we are completely off the steroids, I will ask about when we will be weaning off the CellCept. I'd like 2015 to be a good year for him and us as a family, but once again, this disease, we can have some good days, but a lot more bad days, so we tend to live in the moment and not the future.

I have a meeting next week for disability for Jordan, please keep your thoughts and prayers with us on that aspect. I dont care about the check every month, but the insurance aspect of it, would greatly help! Fingers crossed!

I will update whenever I have something to update I guess. I hope everyone has a Merry Christmas, and Happy Platelets!

Continued thoughts and prayers as always! <3

11/20/14 Update

I sure hope these weekly updates become lesser and lesser.

We got up at 4AM yesterday morning and headed out to Chapel Hill around 5AM, we had an 8AM appointment with Jordan's hematologist. Everything looks very very well, we are so excited for the first time in eight years, yet my wall is still up, I do know this can all change at any given time, so I am trying to stay off of high alert and enjoy the moment while we can.

That spleen of Jordan's, as you all know, was so swollen back last December, it was down to his waist, they told us that it would take months and months before it would be a normal size again (right underneath your ribcage). A month ago, it was still down to his belly button, BUT, yesterday, it was right where it was suppose to be, could barely feel the tip of it, his hematologist was very very happy about that. Jordan looks and feels great, and we are so pleased with the progress of the Rituximab treatment, I would highly recommend it for anyone dealing with this blood disorder.

We also saw Jordan's rheumatologist, she deals with Jordan's immune deficiency, still going to do monthly IVIG at the hospital each month, until we can get a correct dosage to move to SubQ IVIG at home. She did say if we are not happy with doing SubQ at home, once we start, we can always go back to monthly IVIG at the hospital. So we will see when we get to that point. But she is very pleased with his current IGG levels, her goal is to keep them above 1,000, and before Rituximab they have been hovering around the 400 range. Only thing that was disconcerting was the fact that Jordan will be on IVIG for the rest of his life.

They finally also gave us some meds for Jordan's face, due to the steroids, they have made his acne worse, so hopefully that gives him some sort of relief.

Other than that, it was pretty much an uneventful appointment. I did talk to the social worker there about Jordan getting on disability, I had filled out papers back in September, online, and hadn't heard anything yet, so she made a few phone calls for me yesterday, and I finally have an appointment in a few weeks to finally fill out a claim for that. Fingers crossed on that, because if he gets approved, then he automatically gets placed on Medicaid (state insurance), and that would save us some money on his health insurance and prescriptions each month (which are pretty expensive). But I also know its very hard to get approved for disability, especially with something as uncommon as Jordan has, luckily I have a binder full of appointments, treatments, his symptoms, etc. I have to basically make a good case on how this disorder affects his life, which it does, and always will.

Anyhow, these are his counts, very excited about them, hopefully they stay up and 2015 will be a good year for him.

Week 1: 10
Week 2: 35
Week 3: 125
Week 4: 205
Week 5 (Post Rituximab): 222

Hemo is 16
WBC is 7.4
IGG is 1405

Thoughts & Prayers as always =)

I will update next week before Thanksgiving, since Jordan has his IVIG treatment on Tuesday.

11/13/14 Update

Jordan got his last Rituximab treatment yesterday, and once again it went very well. Took a little over 3 hours as it did last week, Jordan slept throughout his treatment, except the last hour.

Exciting news though, his platelets increased even more, I will list the past four weeks down below.

Next week we head to Chapel Hill to see Dr. Gold & Dr. Wu to go over everything and see what we are doing here on out. I know they talked about starting the wean off of steroids, which will be a slow process, so it will take months before he is completely off them. I am hoping we also talk about weaning him off of CellCept and all of the other medications that he is currently on.

Fingers crossed this means we are heading into remission status...at least for a min of a year. Thats our goal, anything over that would be even better of course.

Platelet Counts

10/22/14- 10,000 (Before the first dose of Rituximab)
10/29/14- 35,000 (Before the second dose of Rituximab)
11/5/14- 125,000 (Before the third dose of Rituximab)
11/12/14- 205,000 (Before his final dose of Rituximab)

205! Very exciting as Jordan has not had counts that high since right before he was diagnosed eight years ago! So we are very happy with the current results. Now we just wait and see if his platelet levels will hold off, as does his hemoglobin and his other blood cells.

Continued thoughts and prayers as always!

11/9/14 Update

Third round of Rituximab went very well, but we had a very long day on Wednesday. Jordan was also due for his monthly IVIG treatment, so we decided to go ahead and do both treatments on the same day. So what would have typically been a 6 hour day, turned into an 11 hour day.

Chemo gown that the nurse has to wear when administering the chemo

We went ahead and started him on his steroids and Rituximab treatment to get that out of the way. Pretreated as we normally do (Benadryl, Tylenol, and Zofran), Rituximab took only 3 hours, so that wasn't too bad.

Once we were done with that, we started his IVIG, also pretreated with meds (Benadryl and Tylenol) again, and that treatment took 4 hours (which is a record for us, typically IVIG takes 8-10 hours); thankfully no reactions from that either.

Most exciting part of our day was his platelet count. They draw blood before we start each treatment, before the first dose on October 22nd, his platelet count was 10,000; before this third dose on Wednesday, his platelet count was 125,000!!! So we are SUPER EXCITED about those counts. This upcoming Wednesday, his counts should be higher, just because of the IVIG alone...so I'm looking forward to seeing what his platelet count is when we go to Chapel Hill on the 19th (typically IVIG only lasts 2 weeks for Jordan), to see Dr. Gold & Dr. Wu.

Will update later on this week, after our fourth AND FINAL dose of Rituximab is done!

Continued prayers and thoughts are appreciated!

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.....

Bubble Boy-9/25/14

Talked to Diana this morning, had a lot of new information to say.

First off, no hospitalization, unless he starts bleeding or has excessive unexplained bruises. The immunology clinic in Chapel Hill wants to see him next week, and they don't want the IVIG to offset any testing they need to do, for correct results. So as long as he remains asymptomatic (aside from the petechia), he becomes bubble boy at home, until his counts go up.

Which shall be fun with the school system on getting these absences excused now that he is in high school. I forgot to ask Diana to fax a note saying he is under a doctors care until further notice, so I will take care of that later on today or tomorrow. Also have to see about getting Brittany to go to his classes sometime next week, and get any work he needs to do. Luckily they do afterschool tutoring, etc., so he can easily make up any work that he misses. This is the part that stresses me out, his schooling.

So, the Immunology clinic will call me later today (supposedly) and try to fit him in next week. I am going to try and pull for a Wednesday travel day, since I will be out of town Sunday-Monday. The later the better of course.

Also we have upped his dosage of steroids and CellCept once again. We were at 8mls twice a day for the steroids, we are now up to 16mls twice a day on that. We were at 850mg a day on the CellCept, we are now at 1,000mg on that. Boy is going to end up eating me out of house and home (not that he doesn't already do that).

His counts:

Platelets: 8,000 (were at 25,000 last week) EXTREMELY LOW
Hemoglobin: 13.2 (the same as last week) BORDERLINE LOW
WBC: 5.5 (they were at 4.2 last week) NORMAL
Neutrophils: 70 (they were at 64) BORDERLINE HIGH
Bilirubin: 0.8 (they were at 0.7) NORMAL
Retic Count: 2.4 (they were at 2.5) HIGH

Until next time....prayers and thoughts as always! :)

Update: Immunology Clinic called me, we have to travel to Chapel Hill on Tuesday, to get testing done.

Count of 8,000-9/24/14

We had labs drawn today, and the doctor on call in Chapel Hill called me tonight. Jordan's platelet count is at 8, which is a drop from 25 from last week. She said because he doesn't have any active bleeding, its not an emergency to take him to the hospital for treatment tonight, just to sit tight until Dr. Gold or Diana call me in the morning with what they want us to do. I am about 99% sure we are heading to the hospital for IVIG in the morning, and will be there for the next couple of days. *SIGH*

I am going to keep him home tomorrow, 1)because his platelets are that low and 2)because of the risk of heading to the hospital in the morning, no sense of taking him to school to be checked out an hour or two later.

Bad timing, since I am going out of town on Sunday. Right now, my fingers are crossed his other blood counts are decent, and that we can do IVIG here in town, instead of traveling to Chapel Hill.

Will update when I know more...prayers & thoughts....

Platelets Taking A Dive-9/22/14

Well its been two weeks since we increased the CellCept, and Jordan's hemoglobin and white blood cells have finally increased (not by much, but no decrease is a good thing in this house). Sadly his platelets are still dropping and he has petachia on his waist and legs. I am keeping fingers crossed that Wednesdays labs will show an increase, even if its by a little margin.

Platelets: 25 (they were at 50 on 9/10/14)
Hemoglobin: 13.2 (they were at 12.2 on 9/10/14)
WBC: 4.2 (they were at 3.9 on 9/10/14)

We typically don't do IVIG unless his platelet count drops 10 or below. So fingers crossed that won't happen. I am going out of town on Sunday for the day/night (taking my daughter to the One Direction concert, and its a 4 hr drive), and I dont want to worry about him or put that full responsibility on Brian with Jordan needing to be hospitalized, and juggling Conner too. So fingers crossed and prayers to the platelet gods that we get an increase this week!

This Week's Update-9/11/14

Jordan has been doing good with the CellCept dosage increase, no issues with it, thankfully.

Bloodwork shows counts have dipped again, which we fully expected due to the CellCept increase; just don't like any type of dips in his blood counts, worries/stresses me out.

Platelets: 50,000 (84,000 last week) LOW
Hemoglobin: 12.2 (12.4 last week) LOW
WBC: 3.9 (4.1 last week) LOW
Neutrophils: 64 (68 last week) GOOD
Retic Count: 2.5 (1.9 last week) HIGH (This one worries me a little bit, so keeping a eye on it)

But otherwise, Jordan is feeling/looking great, no physical signs of any low counts (even when his platelets dropped to 39 at the end of July, totally took us by surprise).

We have an appointment on Monday to see Dr. Gold as per our monthly appointments. Guess we are going to give it another week or two, to see if his counts finally stabilize from the CellCept increase, and hopefully they do, so we can start the decrease on steroids.

September-January always has me on the edge of my seat with Jordan, this is typically when his auto immune flares up. I need to see what is going on with the allergist appointment and see if that has been set up yet. I really want to see if Jordan has an underlying primary immunodeficiency other than ALPS (which Dr. Gold is pretty sure he has, but not definite yet), that and I have tons of questions for the allergist. I have a strong suspicion that Jordan has an underlying PI (primary immunodeficiency) called Wiskott-Aldrich Syndrome on top of Evans/ALPS. But again, we will have to wait and see.

Until next time...thoughts/prayers as usual.

Major Growth Spurt-9/5/14

Well now I know why my grocery bill has increased so much the past month or two.

Diana just called me from Chapel Hill. She said looking through the paperwork and labs the past 3 months, Jordan has grown 3 inches and gained 10lbs since June (5'6 and 110lbs presently). So its obvious that he hit a growth spurt this summer, and due to that, the CellCept needs to be upped according to his weight/height.

So we have increased it to 850mgs a day (it was at 750mg). We will do labs on Wednesday, just to make sure his counts are still good (due to the increase of the CellCept, it will lower blood counts). She said if counts are still holding, we will do a decrease on the steroids.

So fingers crossed this is why his counts dipped in July, and that its not something else going on.

Prayers and continued thoughts please.

Ying Yang Game-9/4/14

Jordan went to see Dr. Gold about two weeks ago, it was a good appointment. We were in/out in about an hour, which was record timing for us. He is impressed with how well Jordan looks and feels, considering his counts are not in normal range.

They just called me this morning about yesterday's lab results.

Hemoglobin is 12.4 (down slightly from 13.0 on 8/12/14)
Platelets are 84 (up from 54 on 8/12/14)

As you can see his hemoglobin is playing the ying yang game. Went from 12.7 to 13.0 back to 12.4. We did labs yesterday, I am waiting for his nurse to email the results to me. Hoping his hemoglobin hasn't dipped again.

Dr. Gold is concerned though, because we just cannot get his hemoglobin up past 13.0 the past 3 months. I don't know if Jordan's body is still trying to get over the shingles incident we had to endure in June, or what. Typically September-January is when Jordan flares up, and his counts start dipping, and then we are doing the hospitalization crap all over again. I pray that will not be the case this holiday season. But due to the fact his counts are like a rollercoaster ride right now, they are going to look into increasing his CellCept, which has me a little worried, since it does suppress your immune system. Only thing I can do as a parent, obviously, is to keep him as healthy as possible, and keep him away from anyone sick (which is hard to do, since he is in school everyday).

We never did do his allergy testing earlier this year, I had assumed (as I mentioned in another post) that it was just regular allergy testing, but Dr Gold told me it wasn't, that its more along the lines of testing of his immune system to see what is going on. So they are going to see about getting him another appointment for that in Chapel Hill, which means a day of traveling in the near future. Jordan and I will probably make plans to stay the night up there when that happens, so it isn't a whole day of traveling (its almost a 6 hr drive round trip); apparently the testing is an all day thing, which shall prove to be fun, since he doesn't have the best veins and its always a struggle to get bloodwork from him.

Until next time.....prayers and thoughts will be appreciated as we continue this journey.

Weekly Update-8/16/14

His labs this week weren't too bad, everything (other than his platelets) are border-lined low.

Platelets-54 (last week it was 43, normal is 150-450)
Hemoglobin-13 (last week it was 12.7, normal is 14-16)
Neutrophils are 68, which is good, means his body is producing the cells it needs to.

His Bilirubin has risen a little bit, not by much, but I am keeping an eye on his skin and eye color for jaundice. Back in December, it shot up within 2 days, and since this is around the time he flares up anyway, being extra cautious.

We see Dr. Gold (for the first time since May) on Monday, will draw labs again too, but I probably wont hear anything about that from them.

Will update after the appointment....

Slowly Coming out of Remission-8/6/14

Chapel Hill just called me, and Jordan is slowly coming out of remission after 3 months of being in the clear. His counts are slowly dropping again, and the doctor feels we are going back into a flare up. So he is being put back on steroids (we have only been on CellCept the past two months).

His hemoglobin isn't dangerously low, but its been at 14 all summer and its now down to 12.7. Platelets have been holding strong around the 136 mark, and they are now at 43 (last week they were at 39). They also said his WBC is starting to drop (but nothing dangerously low) YET. So the doctor feels its best we put Jordan back on steroids and try to nip this in the bud before we have a repeat of this past December.

We will repeat labs next Tuesday, hopefully the steroids are doing something for him, and then the following week we see Dr. Gold.

Will update next week, please keep your thoughts and prayers for Jordan. <3

Overdue Update-8/6/14

Didnt realize its been so long since I have updated (almost two months). Last time I updated, we were on Day three of being in the hospital with shingles. So let me backtrack and update you all....

Jordan ended up being in the hospital for a total of nine days, which was mentally exhausting. He missed the last three days of school, and his 8th grade graduation, but luckily his homeroom teacher stopped by the house to drop off his awards that he received. Dr. Gold did not want him discharged until all the blisters had scabbed over and we knew that it was not spreading.

He received treatment everyday for the shingles, we had only one issue with his veins, and that was with the first IV that was placed...vein blew a couple of days later, had to get a vein specialist in to put another line in, once that was done, we were good with the IV.

He did receive one treatment of IVIG the day before he was discharged, because his platelets were a little low (not sure of the number), it wasn't a critical low number, but better safe than sorry is always the hematologist's motto.

Since then, its been pretty much an uneventful summer, haven't seen Dr. Gold since May. With Jordan being in the hospital in June, we missed that appointment, and never got a rescheduled one for July. But we do see him in two weeks, which we are excited about since its been three months.

I am waiting on lab results from Tuesday's lab work. Chapel Hill called me on Friday and said his platelets were very low (39 and they were at 136 on June 2nd). So we redid labs this week to see if it was a fluke or not. Keeping fingers crossed on that, or else Jordan will go back on steroids and end up hospitalized to get IVIG treatment again (ugh).

And well thats pretty much it on Jordan, will update again if anything changes.

June Update-6/13/14

Well, this isn't how I wanted to start off our summer vacation.

We are on day 2 on being in the hospital. Jordan has shingles, so he is being closely monitored in the hospital as he receives treatment for it. Very painful thing to have, but he is hanging in there like a trooper.

Unfortunately, he is missing his 8th grade graduation today, yet another disappointment for him. But, it is the last day of school, so YAY on that! He is officially a high schooler!

Not sure how long we will be here, Dr. Gold doesnt want him discharged until the shingles have dried up and scabbed over. They are starting that process, but not quite there yet. I'm hoping for a Monday discharge, we will see.


Good news though, all his counts are good, hemoglobin is at its highest number in six months, 14!! So we are super excited about that, finally normal range!

Weekly Update-5/21/14

We saw Dr. Gold on Monday, and again approached him with the request to remove the PICC line, he answered me with, "not until we are completely weaned off steroids". So I showed him Jordan's PICC area, the blisters, the redness, the welts, how he is allergic to the adhesive of the dressing. He was a bit apprehensive about it, but finally agreed that if the lab results come back good, we will remove the PICC line Wednesday morning.

Left the appointment with a new appointment scheduled for Wednesday morning to have the PICC removed.

This morning came, and as I was getting ready to head to the clinic for the appointment, with Jordan, I received a phone call from the clinic nurse saying that no order had been put in to remove the PICC line. That she would make some phone calls and see what was going on. So I turned around and dropped a very disappointing boy off at school.

I spent the entire day off and on the phone with Jordan's nurse and the clinic nurse; all three of us were making phone calls to Chapel Hill, nobody answering, and leaving messages. I was stressed out and very pissed off all day.

Finally around 2:30PM, Diana from Chapel Hill, finally calls me. Says his labs looked good, and said that Dr. Gold did not want to remove the PICC until Jordan was done with the steroids; BUT he would leave it up to me on the decision. I told Diana, I wanted it removed because of the problems we are having with it, and she said okay, then made a phone call to Jordan's nurse with instructions.

His nurse, Molly, came after school, and we removed it. Jordan was very nervous and over whelmed about the entire thing, but he did good, and within 20 mins it was out of his arm.

See his poor arm (left arm), all red, covered in welts.

They also decreased his steroids again from 30mg to 24mg a day; and instead of weekly blood work, we are now going to do bi-weekly blood work. So yet another step into remission. :)

We are suppose to see Dr. Gold next month, on June 16th, but do not have an exact time yet, apparently he is overbooked that day, since its a half day clinic; they said they would try and fit us in, so we will see if that appointment sticks. Until then.

Late Update-5/14/14

I haven't updated in about two weeks, so I figured I would throw in a quick update.

Luckily there has not been much to update on with him. He is doing great, looks/feels great. At least once a week his Evans does take a toll on him, with feeling fatigue. But that just comes with having this blood disorder. Last week he came home from school, was asleep by 5PM and slept until I woke him up the next morning. Of course I checked on him a couple of times, but he was just tired, some nights he just doesn't sleep good and eventually it catches up to him.

He had labs done this past Monday, haven't heard from Chapel Hill on those counts yet. But last week his counts were good, I'd like to see his hemoglobin rise more, but at least its stable.

5/5/14 Labs
Hemoglobin: 12.8
Platelets: 188
Neutrophils: 7

He has his next appt with Dr. Gold on Monday, where, once again, I will request to have his PICC removed. Its very frustrating to keep being told no, not right now. I understand the doctor's concerns, we all lost a lot of sleep back in December and January with Jordan, it was very scary to almost lose him. But its been 5 months since he has received any treatment through his PICC, he only gets blood drawn once a week from it. He has lost a lot of muscle tone in that arm, and IMO its ready to come out. He has only 21 school days left, and I want him to enjoy his summer vacation with his friends and not be restricted due to the PICC line. He has already had to say no a few times to swimming at the beach and/or pool with his friends, and I just do not want that type of summer for him, especially when he starts high school in August, and I want him to have as much as a normal freshman year, as he can. He wants to try out for the swim team in high school (whew on that, no contact sports to worry about).

I pray this fall/winter will be a quiet one for him, since that is when he tends to flare up with his blood disorder. I am hoping the CellCept he is on, will help with that. Not sure how long the doctor plans to keep him on it, but for the meantime, it seems to be working, so I am okay with that.

Will update next week, after his appointment.

Quick Weekly Update-4/29/14

Sorry its been so long since I have updated, not much to really update about, which is great.

Last time I updated, it was about 10 days before we left for vacation. We had a blast, kids really enjoyed themselves, especially Jordan, which was so heartwarming to see. It even snowed on the second day we were there, which was crazy! I think we saw all four seasons that week.

Jordan's counts are still stable, not much change there either, his Hemoglobin has stayed around the 13.2 range, platelets go back and forth from 140-170, still in the normal range. Liver enzymes look good. All in all good counts so far.

We saw Dr. Gold last week, very quick appt, in and out., everything is fine, spleen is still not normal, but its slowly unswelling back to normal size. We have to remember it was severely swollen, all the way down to his waist, so its going to take some time getting back under those ribs. At the present time, its about 6 inches from his ribs, so we have awhile to go. So Jordan is still pretty restricted to what he can do, because we have to be careful of any impact on his left side (and of course him having that PICC in place also).

We go back to see Dr. Gold on May 19th, so I am once again, going to mention removing the PICC line, especially since we have only 33 more days of school left. I dont want Jordan to be too restricted to what he can do, as it stands now, he can't swim in any pools, or go to the beach, because of the PICC. And I dont want him to not have a good summer, especially since the kids want to go to Myrtle Beach for a weekend in July, for their birthdays. So we shall see, again, I am so over it (flushing and dressing changes).

Until the next update.

Quick Weekly Update-4/2/14

Jordan has been sick this week, he came home early from school on Monday not feeling well. Achy muscles and he said he felt like he had a fever. No temperature the early part of the day, but when his nurse came by that afternoon for labwork, he had a low grade fever of 99.9. By evening it went up to 100. So we started Tylenol every 6 hrs. Kept him home from school yesterday, because his fever spiked up to 102.5. 

Chapel Hill called me later on yesterday afternoon, to go over Monday's lab results. His hemoglobin, platelets, and MCV had dipped; not by much, but enough to cause some alarm, because his counts have not dropped since December. Hemoglobin had dipped to 12.8 (down from 13.6 the week before) and platelets had dipped to 161 (down from 191 the week before). I mentioned that he was sick with a fever and achy muscles; so they decided it would be best if I brought him into the ER for some bloodwork and a flu test, to see what was going on. With him being on CellCept, he is prone to more illnesses and sickness, so we have to be overly cautious with him.

They did blood cultures from his PICC to see if there was a PICC line infection, took some blood cultures from his other arm, did a chest XRAY to check for placement of his PICC line, also did a strep and flu test (so not fun). I do not have all the results, as they have to wait to see if anything grows, but right away his strep test came back positive, so that explains the fever; everything else looked good though. So he is now on Amoxicillin three times a day, and home from school, until tomorrow. Luckily we caught it early, because he was not complaining of any throat pain. His hemoglobin had went back up to 13.1 but his platelets had dipped again, down to 141; so we are watching for any petechia. I hope it only dipped because he has strep, keeping fingers crossed that this is not Evans related. Hospital will call me if anything comes back positive (such as the flu), but the doctor nor I anticipate its the flu, its probably just strep. 

(Woke him up this morning to do medications, and his temperature was normal, so thats good.)

As for removal of his PICC line, his doctor does not want to remove it at this time. He is afraid that if we remove it, and Jordan needs treatment again in the near future, we will have to resort to something more long term (such as the port), and thats not something we want to do. So we have to change our plans on what we are going to do during our vacation (still going to the same place, but the things we wanted to do up there, now has to change); but we will make due, the whole point of our vacation is to just get away from "real life" for a few days, and enjoy ourselves out of town. We will figure it out. :)

Until next week.....

Quick Weekly Update-3/25/14

Just a quick update, nothing to really report on. Counts are still stable, not yet comfortable with the "R" word yet (remission), but getting there.

Hemoglobin is 13.6 (two weeks ago it was 13.3)
Platelets are at 190 (two weeks ago it was 216)

Those are the only numbers I have, Diana said that his counts looked really good.

I YET AGAIN mentioned about having his PICC removed. She asked when we were going on vacation (told her in 19 days), told her we wanted him to be able to enjoy himself, his veins are healed, he will be okay (and I also told her I was sick too death of stressing about the blood backflow in his line). She said its a very reasonable request, Dr. Gold just wanted to make sure his counts are good (great doctor, love him too pieces, but he is way too overprotective); which they have been for the past month. So she said she would email him right now, and get some answers for me. At this point I will drive 2½ hrs to Chapel Hill to get it removed, I don't care. So hopefully I hear something about that in the next day or two, or I am going to have to turn into a bitch, call up there, and raise hell myself. Rather ridiculous if you ask me.

But like I mentioned above, Jordan is doing great, feeling great, back to his old self. Have noticed though with the past few PICC dressing changes, he has lost some muscle tone in his left arm, from lack of usage. So once that is taken out, and he gets clearance, he wants to start lifting weights (he starts high school in the Fall-enough said, LOL).

Until next week...

Quick Weekly Update-3/21/14

Sorry that I am updating this late, they just called me a few mins ago about this past Monday's labs.

Short and sweet conversation, didn't even get his lab results. Phone call was basically to tell me that they wanted to decrease on his steroids again. So instead of 60mg a day, we are down to 48mg a day. So I am assuming all his lab counts were good since we are decreasing steroids again, just don't have an exact figure in front of me to compare from the previous week. She did mention that they are wanting to rapidly decrease him from here on out, long as his counts are good. So I am hoping by the time we go on vacation we will be completely off the steroids, which means he wont have to take the other two with it (I hope, unless he needs to stay on them because of the CellCept). I assume he wants him to remain on CellCept for the meantime, but I will ask about that when I talk to them next week, or if he wants to wean him off that too, or is that something he will be on for awhile (because it seems to be working).

We had his appt with Dr. Gold on Monday, here in town. Everything was good, I mentioned removing the PICC line, and Dr. Gold was a bit weary on that (IMO) but said he would do whatever we wanted to do. I guess the weariness is from still doing weekly blood works; but like Jordan and I both told him, he can take getting poked by the nurse once a week; done it for the past 7 years during flare ups, we can do it again.

So I mentioned again to Diana (Dr. Gold's nurse) when she called me a few mins ago, that we wanted it removed. Told her we go on vacation in three weeks, and he has been having some issues with blood back-flowing into the line (which I mentioned at Monday's appt-and no one seemed concerned about that, which I guess its common? hell I don't know), so I have had to up his Heparin from 2ML to 4ML, and also had to re-position the clamp, to stop it from doing that, so far so good, its been about a week since I have seen any blood in the line. Told her it was just ready to come out, its been in for three months now. She said that he had mentioned SOMETHING about it this week, but the conversation didn't really finish, so she would shoot him an email tonight, and call me on Monday about that. I assume they will make us come up there for that removal, which I am fine with it actually. I will make a day trip with Jordan, up there, just to be done with it all. I just want it out before we go on vacation next month, so he can enjoy himself.

Jordan also had his monthly breathing treatment by the respiratory therapist, and that went fine. Go back next month for that.

Anyway, thats pretty much it with Jordan. He still is looking & feeling great, no more muscle aches; I did keep him home from school on Wednesday, because he said he had a sharp pain in his stomach, but by evening time it was gone. So no telling what that was about.

Until next week.....

Quick Weekly Update-3/10/14

Just got his test results in email, everything looks good.

Something new I learned this week about Evans, thanks to the support groups that I am in....

Jordan has been suffering from fatigue the past two weeks and muscle aches, I figured he just wasn't going to sleep when he is suppose to at night (and or not sleeping well), and I took the muscle aches to us weaning him off the steroids (he WAS on a very high dose starting out-120mg a day). Well come to find out fatigue and muscle aches are symptoms of Evans, even when your counts are in normal range. So that has been an adjustment for him (and us). The fatigue is everyday, muscle aches come and go, so its not an everyday thing.

But other than that, he is pushing through it, dealing with it the best way he can at this point. Spirits are high, and he is excited about next week's appointment, because he is going to request to have the PICC removed. I sure hope Dr. Gold gives the okay on that, even if it means we have to come back at the end of the day and do it (we have to do a breathing treatment 2 hrs after our appt with Dr. Gold., and he is also backed up from not doing clinics last month). Not sure how long it takes to remove a PICC, I know the process of it, but apparently its something the nurse can do right there in the room. Keeping fingers and toes crossed! I am truly over it!

Anyway, here are his lab results:

WBC: 4.3 (was 4.8) Still low
RBC: 5.04 (was 4.57) Normal
Hemoglobin: 13.3 (was 12.2) Still low, but he hasn't been this number in months!
Platelets: 216 (was 163) Normal
Neutrophils: 68 (was 73) Normal
Retic Count: 1.0 (was 1.1) Normal
Bilirubin: 0.4 (was 0.3) Normal

Plan of attack is to decrease his steroids again, we were at 66mg a day, and we are now decreasing to 60mg a day. Appointment next week, and will go from there.

Quick Weekly Update-3/3/14

Jordan isn't feeling well today, so he is home from school. Came up to me last night, complaining about a headache, and then this morning he said he just felt icky. Its now afternoon time, and he says he is feeling better, a little achy, but no headache, and he hasn't had a fever yet. Giving him Tylenol (no NSAIDS because of his blood disorder) every 5 hours.

**FLASHBACK** to almost exactly 3 months ago, same type of symptoms before he went into the hospital in respiratory failure two days later. So yeah, anxiety level has been pretty high today, didn't get much sleep last night either.

Luckily today is the day his nurse comes to see him, so I let her know he was home today, and she could come earlier than she normally does (she typically comes around 4PM); she was here by 11AM. Blood Pressure was great, no fever, all signs pointing to okay.

Check my email a little while ago, and got his lab results...as I was reading them and comparing to last week's lab results, his hematologist called me with the results and plan of attack for this week.

Everything looks good, and still stabilized. His platelets have dropped a little bit, but they are still in the normal range, I am hoping they dropped because of this cold he has; have to make sure we stay on top of it, because of the CellCept he is on. Luckily his WBC is in normal range (but barely), so his body can fight this sickness off.

FINALLY got an appt for this month (after two weeks of fighting someone to set it up), so that is in two weeks, and we are keeping our fingers and toes crossed that the PICC line will be removed. We go on vacation in 41 days, so we are very excited about both.

No weaning of steroids this week, everything stays the same. Which I am fine with, I dont want to rush too fast with taking him off.

Here are his test results this week:

WBC: 4.8 (was 4.7) Normal
RBC: 4.5 (was 4.7) Normal
Hemoglobin: 12.2 (was 12.7) Still low
Platelets: 163 (was 206) Normal
Neutrophils: 73 (was 68) A little high, probably due to the steroids he is on-no concern, since it goes back and forth each week
Bilirubin: 0.3 (was 0.4) Normal

Until next week...

Quick Weekly Update-2/25/14

Never did hear from the doctor last week, normally I hear from them on Wednesday's, so if I haven't heard from them by tomorrow evening, I will call Thursday morning and see what is going on with everything.

We need to decrease steroids again, because (thanks to his nurse for sending me the lab report) he is still in stable condition, numbers are still slowly climbing (not as high as I would like, but we will still take it, as long as there is no decrease); he has been on the steroids for over 2 months now, I would like to have him off of them sooner rather than later.

I also need an appointment date for March, since they canceled our appointment this month; we need/want the PICC removed, its just no longer needed. Granted we are still doing blood draws weekly (and I am hoping to move to monthly here shortly), but the whole purpose of the PICC line was because he was having blood drawn every single day (multiple times a day), and his veins couldn't handle it anymore; they have since healed. Plus with us going on vacation mid April to TN, we just don't want to have to deal with it either, I want him to have a great time, in a normal setting, not having to worry about the PICC being in place. So with him being in stable condition, we would like it removed. I am even willing to make a trip to Chapel next week to do it, thats how bad we want to be done with it.

Anyhow, here are this week's counts, compared to last week's counts. No big change, but we are happy with it.

WBC: 4.7 (was 5.3 last week) still in normal range
RBC: 4.7 (was 4.6 last week) still in normal range
Hemoglobin: 12.7 (was 12.5 last week) almost to normal range
Platelets: 206 (was 226 last week) still in normal range
Neutrophils: 68 (was 74 last week) last week was a little high, but this week is in normal range
Retic: 1.2 (was 1.1 last week) still in normal range
Bilirubin: 0.4 (was 0.6 last week) still in normal range

Jordan is doing good, his face has broken out a bit, not sure if its just from being a teenager, or its something he is allergic to. We dont have the allergist appointment until the end of April up in Chapel Hill, so we will see on that. Doesn't seem to be bothering him at all. Otherwise he looks and feels great, ready to get back into his normal routine again. I only pray that his counts stay up for awhile, I know with this blood disorder, he can be fine for a few months and then everything goes back to square one again; so I am hoping for a lengthy period of time for the rest of this year, so he can enjoy his summer, and the start of high school in the fall.

Will update this post once I hear from his doctor about what the next course of action is.

They just called me from Chapel Hill, we are decreasing his steroids to 66mg a day (we were at 78mg); so we are getting there. Told her we needed a refill for CellCept, and I need an appointment date for March, because of the issues with the PICC; so she told me she would talk to Dr. Gold and give me a call later on in the week with the new appointment date. If I haven't heard from anyone by Thursday night, I will call Friday morning to reconfirm that I need an appointment date.

Quick Weekly Update-2/18/14

I haven't heard from the doctor yet, to see if we are decreasing his steroids this week, but got yesterday's test results from Jordan's nurse in email earlier this morning. Everything still looks good, slow and steady his counts are creeping higher.

WBC-5.3 (was 5.9)
RBC-4.6
Hemoglobin-12.5 (was 12.4)
Platelets-226 (was 209)
Neutrophils-74
Retic-1.1
Bilirubin-0.6

We were suppose to have a doctor appointment with his hematologist yesterday, but they called me on Thursday to cancel. Chapel Hill, Raleigh, and that area got hit with some bad weather last week (as we did, we got an ice storm, they got that plus snow). We were disappointed with the cancellation, because we were hoping to have Jordan's PICC line removed, but we understand, and hopefully that appointment will be rescheduled in a couple of weeks.

We did get his Pentamidine treatment yesterday afternoon (he gets this breathing treatment once a month to prevent pneumonia since his immune system is compromised, plus its because of all the treatment he has received, including medication).

I will update this post once I hear from the doctor on what our course of action is for this week as far as decreasing medication and/or just staying on the same course.

Our nightly routine (med wise, is twice a day).

Quick Weekly Update-2/11/14

Hemoglobin is 12.4 (was 12)
Platelets: 209 (was 195)
WBC: 5.9 (was 4.2)

So everything else is in normal range for a boy his age; Hemoglobin is not there yet, but another 1-2 higher and we will be good. Decreased his steroids from 85mg to 78mg; so hopefully no bad reactions, I am getting nervous as the steroids get lower because his emotions start going everywhere due to weaning. We had that moment this afternoon during PICC dressing change, but I don't blame the steroids on that, just him wanting to be done with all this crap & being frustrated (imagine tearing off a HUGE band-aid SLOWLY, while you have to keep whatever is in your arm INTACT and it can't move....yeah, its not the most comfortable thing to have done-the dressing change-nor is being the person doing the dressing change), and go back to some sort of normalcy until his next flare up (please lord give us longer than a few months to be in remission before the next flare).

When they called me tonight with the blood count results, I asked if his PICC could be taken out next Monday (when we see Dr. Gold for our checkup); she said she sees no problem with that, she can remove it right in the clinic room, but lets see what Dr. Gold says, said she doesn't think he will have a problem removing it, since Jordan has been in stable condition the past two months. So keep your fingers crossed! This does not mean the blood-work will stop, just means he will have to get poked just like everyone else; but he said he was fine with that, and his veins have healed finally.

Nothing else to really report on...did have a TINY scare last week, when I went to do his flush, there was some light pink blood that had drained down to his clamp line; it did this for two days (not bad, just a tiny bit, but enough to alarm me). I just flushed with some extra Hemp and he was good to go. Bad stomach bug going around here, thankfully Jordan has not gotten it yet, and keeping fingers crossed it stays that way. 

Appointment with Dr. Gold is next Monday, Jordan will also get his monthly breathing treatment. Lets hope everything continues to improve and we get that PICC line removed!

Quick Weekly Update-2/4/14

Jordan's doctor called me a couple of hours ago, everything looks great:

Hemoglobin: 12 (up from 11)
Platelets: 195 (same from last week)
WBC: 4.2 (up from 4.1)

So he has OFFICIALLY been placed in stable condition (they wanted at least a month's worth of results, before placing him in this area). We are NOT decreasing his steroids this week, will see how the lab results are next week, and will decrease again. Right now he is on 85mg of Prednisolone a day (along with his other med's, including 750mg of CellCept). In two weeks we see his doctor again, and Jordan is going to request for his PICC line to be removed; he told me last night, his veins have healed, he can stand being poked once a week again, with a needle. So hopefully having that removed will be an easy task, and not something we need to travel to Chapel Hill for.

We let Jordan stay at his best friend's house all weekend, they were our old neighbors (no pun intended LOL) for 8 years; so they have been basically on this journey with us, since they are like a second family to Jordan. Been best friends for 10+ years; he came home for a short moment Saturday night, so I could do his flush, but other than that, took his meds with him and had a ball. He definitely needed to be out of the house, he has been stuck with us for the past 2 months. 

Until next week =)

1/29/14 Weekly Update

Sorry I didn't update yesterday when I got the lab results in, things have been crazy the past 24 hrs. We had a winter storm move through that put several inches of ice on the ground; which has shut our town down completely (we live in southeastern NC); kids have not been in school since Monday, and unsure if they will go back Friday or Monday.

Things are looking stable, that's the best word I can use right now. Counts are not going up, and they aren't really going down either (his platelets are going back and forth, but still in the normal range). We have decreased his steroids again, but this time the doctor only decreased it by 5mg (instead of the 10mg as we have been doing the past two weeks); so we are at 85mg right now on that (13 ml in the morning and 15 ml in the evening), other meds are still the same, no mention of taking him off CellCept, I have about 6 more weeks left in my fridge on that, and I guess I will ask at the next appointment about either a refill or are we weaning him off that.

The Allergy and Immunology Clinic in Chapel Hill called me in the latter part of the week, last week and scheduled him an appointment to start allergy testing, which is good news; bad news is, its not until the end of April. Since he had no viruses or infections going on when he was admitted into the hospital throughout the month of December, they just want to make sure its not allergy related causing these flare ups of his counts to rapidly decrease out of the blue. We did mention that we got our first REAL Christmas tree, 4 days before he went into the hospital. They were "hmm" about it, but said its probably a coincidence, but they will test for that just incase. They said to expect to be there for hours for testing, and our appointment isn't even until 12:30PM, going to be a long day. Luckily our family vacation is the week before, so we should feel refreshed and ready to tackle this next hurdle together.

Here are this week's counts:

Hemoglobin: 11 (down from 11.4)
Platelets: 195 (down from 229)
WBC: 4.1 (no change)
Retic: 1.9 (up from 1.2)

His Complete Neutrophil count is also good.

Jordan is doing good, face has broken out a little bit, since we are now weaning him off the steroids (but his face is like that when not on steroids, thanks to teenage hormones). Still has the PICC line in, and I have become a pro on changing the dressing every week, and doing the flushes everyday. But I cannot wait to not have to play nurse anymore, its mentally exhausting. He is definitely a trooper in all of us, but I know he is ready to get back to his normal life. Even with being absent all of December, from school, still got A's/B's on his report card; very proud of him.

Next blood draw is next Monday, continued thoughts and prayers.

1/21/14 Weekly Update

Doctor just called me with yesterday's results, everything has increased, which is awesome! Moving in the right direction.

Compared to last week's labwork:

Hemoglobin: 11.4 (was a 10)-Not in the normal range yet, but getting there
Platelets: 229 (was 210)-Normal range
WBC: 4.1 (was 3.1)-Almost in the normal range, but not quite there yet
Bilirubin: 0.8 (was 1.2)-He is officially in normal range

We are decreasing his steroids again, from 100mg a day, to 90mg a day. So thats a big jump, because we were only decreasing by 5mg a week, but this week we are decreasing by 10mg. So hopefully his counts stay stabilized, and we still get good lab work results next week.

Nurse will come draw labs on Monday, next week. We are not scheduled to see the doctor until February 17th, as long as bloodwork stays good each week.

Jordan, himself, is doing very good. Back to his normal teenage self. Looks and feels great. He cannot wait to get rid of that PICC line, so I am hoping when we see the doctor on the 17th of next month, we can talk about removing it. 

I will update again next week.