June Update-6/26/15

So we headed up to Chapel Hill last week to see Jordan's rheumatologist and hematologist to discuss whether or not Jordan will be doing another round of chemo (Rituximab). Last I had heard from the doctors is that Jordan's hematologist was going to discuss it with the rheumatologist and go from there; so I was thankful our first appointment of the day was with her so I could talk with her about our thought process with it (love Jordan's hematologist, but he tends to jump the gun a lot, very over protective of Jordan since the incident in December ).

Appointment with her went well, no change on his SubQ-Hizentra at home, which is fine, because his IgG levels are holding steady and much higher than they typically have been. Tried to get Jordan to take over the appointment and discuss his feelings on doing another round of chemo; he did okay, but eventually I had to step in and go a little further with our thought process of wanting to wait it out and see what happens. She was in agreement with me completely (thank god), so now I have her backing me up on our decision to wait. Jordan also signed up for the CVID research program while we were there.

Saw Diana and Dr. Gold, discussed with Diana about wanting to wait on doing another round of chemo, and lets see what his body does on its own. Granted we know he will have to do another round eventually, but it could be a few more months, or even a year or two down the road, before his immune system recognizes those B cells as foreign objects and starts attacking. And since we have never done this treatment before, we don't know when that will happen, so we would like to wait and see before jumping the gun. They agreed with us, and said lets see what his counts do over the summer and go from there.

Did labs, and his veins blew twice...they took 10 tubes of blood from him (retesting for ALPS again), and since his veins blew twice, the CVID program was not able to get their five tubes of blood; so we will allow them to do that when we go back in December (pending his counts hold up until then).

So for now, he is borderlined stable, and we will continue monitor his counts and health and go from there.

6/16/15 Chapel Hill Labs

Platelets: 157 (N)
Hemoglobin: 13.6 (L)
IgG: 887 (N)

We decreased his steroids to 1ml twice a day. He also has a hint of thrush in his mouth, so they put him on Nystatin to help with that for a few days.

6/24/15 Labs

Platelets: 162 (N)
Hemoglobin: 13.4 (L)

No decrease on steroids, we will repeat labs on July 6th and go from there.

As always, continued thoughts and prayers! :)

May Update-5/30/15

Sorry its been awhile since I have updated Jordan's page; believe me, it was not by my own doing.

Doctors finally emailed me this past Wednesday (3 weeks after the last blood draw) and wanted me to take him in yesterday for labs. Also told me that his hematologist wants to do another round of chemo (Rituximab) this summer. I was confused by that, because Jordan's counts have been steady since November when he finished the first round. But apparently after 6 months post chemo, the B cells tend to regenerate after Rituximab, and that has been the case for Jordan. Although the level is very low, the B cells have returned.

For those that aren't familiar with the Rituximab treatment/process for ES patients...Rituximab is used to deplete the B cells in their bodies, so that their immune system no longer sees them as foreign antibodies. When that immune system recognizes them as foreign, the immune system becomes overactive, and starts killing off everything (platelets, white blood cells, red blood cells, etc). So with this treatment, the idea is to deplete the B cells, and that the immune system will quiet down. It doesn't work for every ES patient, what one treatment may work for one ES patient, it may not for another. Some see results right away (as Jordan did) and some, not at all. Unfortunately for those that see results right away, it becomes a waiting game to see when their body will regenerate the B cells again, and then to see when the immune system will recognize the B cells and start attacking again.

After talking to Jordan that afternoon, he wants to postpone another round of chemo right now, continue labs as usual; and if any of his blood counts start decreasing at the rapid rate they typically do, (or if the B cell levels increase dramatically), then we will go with another round of chemo. With him turning 15 this summer, and having dealt with this autoimmune disorder for over 10 years; we feel its his body, and he DOES have a say on what goes into it. Of course if any of his decisions affected his overall health, Brian or I would step in and override that. But I don't think waiting to see what his body does on its own is wrong.

We still need to talk our decision over with Jordan's rheumatologist and hematologist and go over a treatment plan, and see what their opinion of waiting is. We go to Chapel Hill in June, so I will have an update in about a month on what went on with that appointment.

Otherwise, his lab results from yesterday, were "okay" not great, but just "okay".

Hemoglobin 13.1 (L)
Platelets 175 (N)

We will leave his steroids at 2ml twice a day (6mg) because of his dip in hemoglobin; he has exams starting next week for school, so we don't want him to risk feeling too fatigue or bad during that. We will repeat labs when we go to Chapel Hill in June and go from there.

Continued thoughts and prayers!

Feeling Defeated-4/30/15

Growing very impatient with the high school.

Jordan received an attendance failure in one class, and an INC in another class last semester (yet both classes have a final grade of an 88 and an 89-which are B's); which means instead of him receiving 4 credits last semester for four classes, he only received 2 credits (why he received these in only two classes, instead of all four, I have no clue). Emailed his counselor early March about this, said she would look into it, but in the meantime to fill out the appeal attendance failure form. Which I did, including attaching all of Jordan's doctor notes, hospital discharge notes, a letter from his hematologist (stating that Jordan cannot attend school if his counts are below 25,000 and that there would be days he would miss due to treatment to keep his counts out of the danger level), and a personal letter from myself stating why Jordan should receive credits in those two classes, and explaining his condition. Haven't heard anything since. So I sent another email this morning to his counselor, asking for an update, and if the appeal was approved, if not, what actions do I need to take. Waiting on a response from her....

Pissing me off, I understand about the county attendance policy. Whether its excused or unexcused absences, its an automatic attendance failure in that class if its 10 or more absences. I don't agree with it, especially if it pertains to a medical condition, but it is what it is.

Back in October, I had a meeting with his counselor, the school nurse, those four teachers, assistant principal, and a homebound school teacher, educating them on Jordan's autoimmune disorder, and got him started on a 504 plan. I was under the impression that the point of the 504 was to protect him from receiving attendance failures if he missed school due to medical reasoning. But was told in March, by his counselor, that the 504 plan does not protect him from that, because its a county policy. That the 504 plan is in place, incase he misses an extent amount of school again, that the homebound school will kick in, and help him keep up with his school work, also allowing him more time to make up any missed work (which in the 10 years he has had ITP, he has never had an issue keeping up with this schoolwork/making up any missed work; and has always remained on the A/B honor roll).

I just do not get it. Jordan's autoimmune disorder is chronic, there is no permanent remission period for him. Our experience is he gets a few months out of the year with good counts, before they drop in the danger level again, and he misses school due to being hospitalized for treatment. It baffles me that I have to deal with this, every single school year, to make sure he graduates on time with his class in 2018. We have considered pulling him out of the school system altogether and homeschooling him, to receive his diploma, but he doesn't want to do that; he wants to be able to walk the stage with his friends in 3 years, and I do not want to take that away from him. But as a parent, what do I do, when I tell him to keep doing what he is doing in school, keep making up the work, stay on the A/B honor roll to prove to the staff that you can still keep up, even with missed school days; and they turn around and punish him with not giving him the credits for the classes he has so rightfully earned.

Update 6/1/15

The school finally revoked the Attendance Failure for his classes and he has received the well earned credits for his classes. 

April Update-4/28/15

Its been almost 3 months since I have updated, which is a blessing, because that means not much has needed to be updated.

Jordan is doing well since my last update in February. His platelets have been fluctuating up and down for the past two months. He got sick at the beginning of March for a couple of days, so his counts took a little dip, and its taken some time for them to come back up. At the same time, we have been slowly decreasing his steroids. Last I updated in February, he was at 8ml twice a day, as of yesterday, he is now on 3ml in the morning and 2ml in the evenings.

He is also doing well on his SubQ therapy here at home, weekly. His IgG levels are currently at 1064, which is a far cry from them being in the 400s back in the Fall, and its right where his Immunologist wants him to be at. It was an adjustment when we started back in January, but now its pretty routine for us on Friday nights. Premeds (Tylenol and Benadryl) and numbing cream applied about an hour before infusion, and we are good to go.

We saw Diana last week at the clinic (saw Dr. Gold for a few minutes), no swollen lymph nodes, and she could barely feel the tip of his spleen, so all good there. Jordan has been suffering from some ulcers in his mouth for about two weeks, one being pretty big on the inside of the bottom of his lip, so they did a culture on that, it came back negative for herpes simplex.

Jordan still suffers from fatigue at least once a week, last week he came home from school and went to bed at 5PM, slept until 6:30AM; but everyone that deals with ES, we all know its just a fact of life with living with this autoimmune disorder. Also doesn't help that he has PE this last 9 weeks of school, and they are pretty active the 90 minutes of class (running 2 miles, soccer, etc); we are allowing him to participate because his counts are good, which I am thankful for, because he needs this class to graduate in 3 years, and I'd like to go ahead and get it done and over with.

On another note, dealing with school; I am fighting with them right now on giving Jordan two attendance failures last semester in two classes. I was under the impression placing him on a 504 Plan in October, this would prevent that from happening, but alas, not the case. In our county, the school board policy is that no child can miss more than 10 days of school in a semester (excused or unexcused), if they do, its an automatic attendance failure in the class. I filled out the appeal forms, sent in all the copies of doctors notes, and a copy of a letterhead from his hematologist, including a letter from myself; that was a month ago, and no word yet. He had a B in both of those classes, so I am not seeing what the issue is here. I will be furious if he does not receive those class credits, and they want him to make up the time that he missed; especially since he made up the work in a timely fashion and kept up his grades in those classes. 31 more school days to go, and it cannot come soon enough!

We are taking a break this week from doing a CBC, will take him back in next week, where they want to run a Rituximab panel to see if his immune cells are still suppressed from the Rituximab that he received in October/November of last year. Hopefully its still the case, since he is doing so well.

Last week's counts are below:

Hgb: 14.3
Platelets: 199
IgG: 1064

As always, thoughts and prayers!

Quick Update-2/4/15

Today marks 12 weeks post Rituximab for Jordan. Getting nervous. Jordan's platelets are slowly decreasing again. Makes me think the chemo treatment didn't work. Granted his counts are still good, and they remain in normal range, and a lot of ITP/ES patients would LOVE to have these counts; but its also a reminder that any dip could possibly be the start of another roller coaster ride with his platelets. Fingers crossed, they don't continue to dip, because I honestly do not know if we have any other options as far as treatment goes. Nothing else has worked so far for him, and I dread that this treatment didn't work and splenectomy will be the next words brought up; and I stand firm that I will save his spleen, unless its a life/death situation. So basically its just a wait and see game, as usual with this disease.

Infusions at home are still pretty hard on Jordan. Last week, he fought me hard on it, basically threw a tantrum in front of the nurse (took everything I had not to lose my cool); but I also know the premeds make him moody (mostly the Benadryl), so I just have to push through it with him and hope it gets easier. We had to bite the bullet and fill the prescription for the numbing cream this week ($54-thanks insurance for not covering it @@). So I am hoping tonight's infusion will go much smoother; nothing I can do for the burning part of it (once we start the infusion, it burns the first few minutes going in), but I am hoping at least the poking part, will be a lot easier. and that the numbing cream does what its suppose to.

Otherwise, same old around here, below are his counts:

240-1/7/15
218-1/21/15
193-2/4/15

As you can see they are dipping by at least 10 each week; hopefully we wont see it continuing to dip in the next two weeks. His hemoglobin and WBC are in normal range, and his neutrophils are also in normal range; so at least we are good in that area.

As always thoughts and prayers.

Quick Update-1/28/15

Its been almost three weeks since I have updated everyone, so I figure it was about time to do so.

We started at home infusions about two weeks ago. Jordan now has SubQ infusions done at home, once a week. He is on Hizentra, 50 grams (50 ml is the dose rate). First two infusions, we have had a nurse come from Walgreens Infusion Services. She trained me on the first week, last week I did everything by myself in front of her, I will do the same this week, and then Jordan and I will be on our own from here on out. Its been a hard routine to get use to, Jordan doesn't like being poked at every week, and there are three sites I have to poke in his stomach. Infusion takes a little over an hour, and we premedicate him with Benadryl and Tylenol about 45 mins before the infusion. After the infusion is done, he takes a good 4 hour nap afterwards, which has been hard on his sleep schedule.

I figure it will take some time getting use to, as it did last year when he and I were doing his PICC line dressing changes every week. As you guys know, that was a stressful routine also to get use to, started off that it would take us almost an hour to do, by the time we had it removed, Jordan and I had it down to a 20 min window of changing his dressing change. So my hopes are that this new routine of doing SubQ infusions at home will be the same situation. Just patience and time to get use to it. I am hoping eventually I can get him to muster up the courage to do these pokes himself, as this is something he will be doing for the rest of his life...and well, I can't exactly go with him to college to do it myself! 

Also got to see our favorite nurse last week, its been three months since we have seen her. Between Jordan doing chemo throughout the month of October and November, then the traveling to Chapel Hill and the holidays, plus switching insurance companies in December, we had to wait before we could see her again. She has been our nurse for almost a year now and we adore her. She came by to draw blood and take vitals, we are on orders right now to do a CBC every other week, as we start the process of weaning Jordan off of steroids. Also Jordan has to have his IGG levels checked to make sure the infusions we are doing at home, are working, and that he is getting the correct amount of the IVIG. Last week's counts, I will post below, they dipped a little from the January 7th appointment in Chapel Hill, but his Neutrophils look good, so the hematologist team aren't worried. But those of us that have been dealing with this disease for years, any dip, worries us. But, thankfully, for the meantime, his counts are in normal range, so we take that as a blessing right now.

Other than that, everything is going good here. Jordan just started his second semester of his freshman year, which means four new classes, which he was excited about. He passed his first semester classes with two A's and two B's, which makes me so proud; especially since the 504 team wanted Jordan to drop two of his classes, and only take a half day of school the first semester (because he had missed so much school due to low platelets). But, like I told them, he will do the work, he will bust his ass to get caught up, and guess what, I was right. :) 

Jordan is doing great, a little worn down more so than usual, but thats from the infusions, which takes a few weeks before his system gets use to it, and builds up the antibodies that he needs again. So we just let him sleep as much as he feels he needs. Right now he has swimmers ear (took him to the doctor this morning, since he was complaining of an earache-only him would get swimmers ear in the middle of winter-LOL), so he is upstairs sleeping, and back to school tomorrow he will go. 

Hopefully we have a quiet year, opposed to last year. Nothing ever surprises me with Jordan though, he keeps me on my toes medically and emotionally. But like someone once told me when I was complaining on how overwhelmed I always am with Jordan. God wouldn't have entrusted Jordan into my hands if he didn't think I couldn't handle it. Special boy for a special mom. :) Who knew almost 15 years ago, becoming a teen mom, again, for the second time, he would make me grow up so fast. Unfortunately he has had do to the same. But I wouldn't ever trade the fact that I am his mom.

We did receive upsetting news in the ES community, online last week. We lost two wonderful women that had been having complications in the hospital for quite some time. It was heartbreaking and gave me a heavy heart, as this was a reminder that tomorrow is never promised, especially with this disease. They both were wonderful for me when Jordan was first diagnosed with Evans Syndrome in 2013, always willing to message me and answer all and any questions I may have had when pertaining to Jordan. So to them, Shannon and Stefanie...fly high our ES angels, and may you now rest in peace.

Here are Jordan's current counts (they are also on the side for those reading this on a computer).

Platelets: 218,000

Hemoglobin: 13.6

Again, his platelets are in normal range (145-450), his hemoglobin is border-lined low (anything above 14 is normal), so not too concerned (yet); as always we keep an eye for any low blood counts. 

We will do another CBC on February 4th, I will update after that on his counts. As always thoughts and prayers.

A Lessson in Blood Counts

A QUICK LESSON IN BLOOD COUNTS...

Red blood cells or erythrocytes comprise a third of all the cells in the human body. With about 20 trillion in the average adult, they're not only the most common cells in the human body, they are also the only cells that don't have a nuclei. The red blood cells' primary function is to carry oxygen to the body's tissues, and because they are 40 percent lighter than they would be with nuclei, it is easier for the heart to pump them through the circulatory system.
Red cells contain a molecule called hemoglobin, which picks up oxygen as the cells travel through the blood vessels in the lungs. After red cells leave the lungs they get distributed throughout the body, passing through ever-smaller vessels called arterioles and capillaries. There they get forced up against the vessel walls like passengers in a crowded subway train. Under that pressure, the oxygen molecules pop off the hemoglobin and get taken into the vessel walls and passed on to neighboring cells. Relieved of their burden, the red cells pick up carbon dioxide, which they then carry back through the veins to the lungs to be exchanged for new loads of oxygen.

White blood cells or leukocytes, are the body's border defense: they are responsible for apprehending and eliminating foreign substances. They're larger but much less numerous than red blood cells, numbering about 20 million in the adult body. While red cells are buoyed along like cargo rafts as the heart pumps the serum, white cells function more like patrol boats. Under their own locomotion, they can change direction to go after an invading molecule or cell.
There are three basic types of white cells-granulocytes, monocytes and lymphocytes-each with its own specialized tasks. Granulocytes, the first line of defense, home in on bacteria or any other foreign substance in the blood. They are filled with granules containing chemicals that destroy their prey. One type of granulocyte, the neutrophil, is an all-purpose search-and-destroy agent that makes up 60 percent of white blood cells. The other two granulocytes, eosinophils and basophils apprehend special intruders such as allergens and parasites and account for 3 percent of the white blood cell population.
About 33 percent of white cells are lymphocytes, the brains of the immune system. They send out antibodies to immobilize foreign molecules and chemical messengers that spur other cells into action. The remaining 4 percent of white blood cells are monocytes, which clean up cellular debris and devour invaders that have been tagged with antibodies. (Like what happens to the red blood cells, platelets and sometimes white blood cells themselves in Evans syndrome when they have been tagged with antibodies.)

Platelets, so named because they are shaped like tiny plates, release chemicals that promote clotting when blood vessels are broken. These chemicals work by assembling a blood protein called fibrin into a meshwork over the damaged area. The mesh serves as a net to trap blood cells and proteins, which eventually form a plug in the damaged vessel wall.

The meaning of the numbers: A complete blood count involves computing the number of red cells, the percentage of red cells in the whole blood, the size of the average red cell (an indication of its robustness), the average amount of hemoglobin per red cell, and how much there is in the blood. In addition, a physician will want to calculate the total number of white cells and the percentages of the different types of white blood cells, which can be distinguished under a microscope by the shapes of their nuclei. A physician will compare the numbers of cells in a sample of a patient's blood, with the average number of cells in the same amount of blood from a healthy person. Having too few red blood cells or too little hemoglobin is called anemia. Having too few white blood cells is a sign that a person's immune system is not functioning properly. Too many white blood cells usually means that the patient has an infection. The type of white cell that is over produced can indicate the extent of infection. An increase in the number of neutrophils, for example, means the person probably has a bacterial infection. An increase in lymphocytes and basophils usually corresponds to an allergic reaction like what you might get from ragweed. If there are far too many white blood cells-in the neighborhood of 100,000-the patient may have leukemia. A physician will also look at the number of platelets in the specimen to assess if the patient may have too few or too many.
While normal values vary slightly from institution to institution, the following values are within the accepted range of normal:

• Red blood cells 4 -5.3 million/mm3
• Hemoglobin 11 - 14.5g/dl
• Hematocrit 34-42%
• White blood cells 4,000-13,000/mm3
• Platelets 150,000 to 400,000/mm3

PROFILE OF A CHILD WITH EVANS SYNDROME

Katie was diagnosed with Evans syndrome when she was 13 months old. For the five months prior to her diagnosis she had been noted to have a lot of bruising of her arms and legs. They were just small bruises most of the time and because she was getting older and was crawling and learning to pull up, they were attributed, by her doctor, to bumping herself. During those same five months Katie had a low grade fever every day. Her temperature rose to 99.6 to 100.6 every evening. She was cutting teeth, so it was assumed that that was the cause of her fevers. She had one virus and stomach flu after another with rarely ten days of good health before being struck by something else. It was odd because Katie didn't attend day care and wasn't out in public very much so it was hard to imagine where she was being exposed to all of these "bugs". Both my husband and myself were lucky enough to be able to stay home with her full time at that time, as we had adopted Katie after ten years of adoption attempts, and she was the center of our universe. We weren't bringing "bugs" home from work.
On her "well baby check up" at 12 months, I had again complained to her doctor that something did not seem right with her. My concerns were passed off as "normal baby problems and nothing to be concerned about." We returned to the doctor's one month later and I insisted that blood work be done at the hospital, as I knew something was very wrong. Despite a normal assessment by the pediatrician, he wrote orders for the blood work and we headed for the hospital with the understanding that he would call me later that day with the results.
Once home from having the blood work done, Katie was crawling on our living room rug and bumped her front tooth. She started to bleed and although the bump had only been minor, we could not get the bleeding to stop. She continued to ooze for an hour when I called the doctor to report my concerns and ask about the lab results.
A few calls later and the doctor was on the line telling me that he thought she had leukemia and that the pathologist who had reviewed the blood work thought that she had some type of bone marrow tumors. Her platelets, red cells and white cells were all so very low that she was rushed to the local hospital for stabilization and then transferred to the university hospital for further evaluation and treatment.
After bone marrow biopsy results showed normal but increased activity, the cancers were ruled out and the diagnosis of Evans syndrome was made. Katie has been treated with steroids both IV in crisis and by mouth routinely since. She used to spend almost a week per month in the hospital with infections, sepsis or crisis status blood levels. After three and a half years of that, I started begging her doctors to try something different. I am a registered nurse and had by then spent a lot of time investigating Evans syndrome and the best ways to treat it. I was convinced that if we tried giving her the immune globulin (IVIG) routinely instead of waiting until her levels crashed, that we might make some progress. Finally in the spring of 1995, they agreed to let me try my theory.
Katie had an infusaport placed which is a type of permanent IV line that goes into a big vessel in the body and is under the skin so normal activities like swimming don't affect it. I started giving her the IVIG at home every three weeks, sometimes every two when her levels would drop more or if she started to get sick. For Katie this was the magic ticket. Until 1998, Katie did not require any hospital stays for infections or crisis levels. That' s not to say that she hasn't been sick, but we've been able to manage her at home. She still has blood work done every week to see how she's doing. Katie is now eight years old. She is in home school became she has poor endurance and has too many problems with low white blood cell counts to be able to fight the normal childhood illnesses she would be exposed to in school. She has a few friends that she loves to see and spends many hours each day playing on her computer. She loves to be out in public with people, and she does get out, but we try to keep her out of anywhere crowded and away from anyone sick. Very tough during flu season!Katie's Evans syndrome was triggered by her DPT immunizations when she was an infant. She had violent reactions to the three rounds that she got, and began with the symptoms (bruising and fevers) of her Evans syndrome within a week of her last immunization. She also had a grand mal seizure and respiratory arrest six hours after her last immunization. When she was diagnosed, I was told that I would never find another child with Evans syndrome and unfortunately, I have found more than 25. Another little boy diagnosed with Evans syndrome around the same time as Katie, I discovered received the very same lot number of DPT vaccine that Katie got!I have done hours and hours of research on this subject, and have found documentation in all of the hematology and immunology textbooks, that the DPT vaccine and some of it's preservatives and additives that make it work better (thimerosal and aluminum salts), have all been found to be related to thrombocytopenia and hemolytic anemia. I do not mention this for the purpose of upsetting anyone, or to convince anyone that their case of Evans syndrome is also related to vaccines, because it may or may not be. It is merely food for thought.
~Lou Addington, mother

Update 1/7/14

Haven't updated about Jordan since right before the holidays....

Headed to Chapel Hill yesterday and got blood work done for Jordan. Its been 8 weeks since his last dose of Rituximab, and everything still looks great! Looks like he responded well with the Rituximab. But in the 8+ years we have been dealing with this, January-July are our quiet months, normal counts, etc., so I am hesitate anyway. Time will tell for me, moreso in the Fall/Winter later on this year.

Week 1: 10
Week 2: 35
Week 3: 125
Week 4: 205
Week 5 (1 Week Post Rituximab): 222
Week 6 (2 Weeks Post Rituximab): 202
Week 14 (8 Weeks Post Rituximab): 240

Hemoglobin is 14.7, WBC is a little low at 3.9, but no concern from the doctors because his neutrophils look good. That and he is always border-lined low on WBC anyhow.

Immunologist is going to make arrangements with Walgreens to get Jordan started on IVIG SubQ at home in a few weeks. His IGG levels did drop again (they were at 1405 in November, they are now at 711), his immunologist would like him around the 1,000 area.

For those that don't know Jordan also has CVID, so he typically gets IVIG once a month (last round was right before Thanksgiving-didn't get it done in December due to the holidays), but we are going to transition him over to weekly subq at home. His hematologist thinks more along the lines of ALPS instead of CVID (the hematologist and Immunologist are in disagreement over this)...so we did more testing and sent that off to Ohio yesterday to confirm or deny the ALPS claim-which btw, was A LOT of blood to be taken, good lord. But thankfully his vein held up very well yesterday while 11 vials were taken from him (typically his vein would have shut down after the 3rd vial). All the green vials were just taken to test for ALPS. Crazy.

Other than the medical aspect of it, Jordan is doing very well, looks and feels great. So I am fully expecting a quiet 6 months from here on out. Like I mentioned above, July-December are the hell months for Jordan. So we are going to enjoy it while we can.

Happy Platelet Dance for you all!

What is Evans??

Best way to explain what Jordan has in "English terms"

Evans syndrome

is a rare autoimmune disorder in which the body makes antibodies that destroy the red blood cells, platelets and white blood cells. Patients are diagnosed with thrombocytopenia and Coombs' positive hemolytic anemia and have no other known underlying etiology. The patients may be affected by low levels of all three types of blood cells at one time, or may only have problems with one or two of them. The specific cause for Evans syndrome is unknown and it has been speculated that for every case, the cause may be different. There have been no genetic links identified.

The course of Evans syndrome

varies by case. The patient may be symptomatic of whatever blood levels are down. If the red blood cells are down, the problems complained of may be weakness, fatigue, shortness of breath and the usual things associated with anemia. With low platelets, they are susceptible to bleeding and major bruising from minor bumps and cuts. A bump on the head could cause severe brain hemorrhage and death. With low white blood cells, the patient has increased susceptibility to infections and difficulty in fighting these infections. The patient may have problems with one, two or all three of these blood lines, at one time.

 Treatment of Evans syndrome

varies and there has been no "magic bullet" identified that will cure this. Steroids are frequently used to help suppress the immune system, or to decrease the production of the "bad antibodies". Intravenous immune globulin or IVIG is often tried as is chemotherapy when responses to other treatments are not satisfactory. Splenectomy has frequently been done, but the benefits of this are usually short-lived. In the last study done, the beneficial effects from splenectomy had only lasted an average of one month. Closely monitoring the patients' complete blood count is crucial to the patients' treatment. Transfusions, of blood products, is done in crisis situations to help stabilize the patient but is not a long lasting solution as these cells are usually destroyed very quickly by the body.

The prognosis with Evans syndrome

is guarded. Some patients have episodes of major blood cell destruction followed by long remissions, while others have chronic problems with no remissions. It has been reported that patients with Evans syndrome have a greater tendency to develop other autoimmune disorders such as lupus and rheumatoid arthritis and there is a tendency to develop various malignancies. Careful monitoring of the patient by a qualified physician is very important.

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....