Need help, may have 2nd disease

    • Anonymous
      March 20, 2008 at 7:29 pm

      Most of you know Dell. We have been on this forum for over a year now, being dx. 12/05 with cidp. He is almost 4 years old and we may be facing another disease, PID.

      For some reason, his CD4 has been going down. If anyone knows anything about CD4 and why it would go down, I really need your help. I am so upset right now and I can’t believe I can type this.

      2/07 his CD4 was 1074, normal for an infant. 2/29/08 it was 500 something and last week it was 295.

      We consulted the immunologist today and he is thinking PID. He thinks the reason Dell has not gotten sick already is because he’s on ivig. The dr. is consulting with Dr. Sorenson from LSU, a guy who he trained under. Supposedly, LSU has one of the clinics/centers for PID patients.

      I know you can have more than one auto immune disease but it is hard to swallow when you are looking at your beautiful baby boy.

      Please help if you can.

      Love, Lori

    • Anonymous
      March 20, 2008 at 9:39 pm

      Hi Lori,

      I just did some research on CD4 and PID. Many things can affect the CD4 count so until you know more and they do this test a couple times, keep the faith. I know it is hard to see your baby ill. What I can get from my reading is that CD4 levels can change dependent on the time of day they are taken and can go down when other infections are present. They are a measure of total viral count in our system. I would assume PID would be one of these. It also lists pneumonia, HIV, and other things that can lower the count so I would be asking my dr. some questions about this. Could this be the result of an underlying and undiagnosed infection? Keep pushing for answers.

      I was also able to find info stating IVIG is the treatment of choice for PID so your little boy is already getting the treatment he would require if it is infact PID.

      Please keep us posted and I hope you can find comfort in talking to people on this forum until you are able to get answers. If I can do more research for you, say the word.

      Take care.

    • Anonymous
      March 20, 2008 at 10:19 pm


      Thank you so much for what you looked up. My husband used our computer when we got home from the dr. and I went to my mom’s house to use her’s.
      Still can’t make heads or tails of anything.

      I did find a few things that could make the CD4 go down but don’t know if any pertain to Dell. The only thing we have always battled with him is a butt rash. It’s really not even a rash, it’s more like he’s been in a wet diaper for awhile. It is a daily thing to put diaper rash ointment on him. Most of the time, he wakes us up in the middle of the night and I have to put something on him.

      There was nothing I saw on the net that stuck out. Again, thank you for your research. I am trying to keep the faith, it’s hard, but I’m just asking God to please let it be something that is easy to fix or easy to deal with.

      Love, Lori

    • Anonymous
      March 20, 2008 at 11:04 pm

      I wonder, would a year of iv solumedrol cause such a low drop in t-cells? Steriods are systemic, affecting both T & B cells. Ritux would affect b-cells directly, but could there be a negative feedback to the t’s as a result?


      can you think back, and remember what immunizations Dell has had? Wondering if titres can be measured – might give a timeline when immune responded (or didn’t) in an appropriate manner.

    • March 20, 2008 at 11:44 pm

      I agree the diaper rash has some connection. I remember reading something about fungus (diaper rash in this case) and cidp.
      Try to ask about that 3g/kg dose for children, I will look for the abstract. Also, if they do decide it is pid, gammaguard s/d(powder) is supposed to be better. I would be very surprised if it is pid, I cannot understand how the immune system could be over active with cidp and also underactive with pid. You would think one would balance out the other.

      I noticed in my readings that pid people get sub-Q injections of ivig, I wonder if he could get both? (Assuming he does have pid) There has to be some connection between the rituxin and the blood work. The doctors will find it out! We are praying for your family.

    • Anonymous
      March 20, 2008 at 11:49 pm

      Compact disc,

      I had Dell’s shot record on my bar. PCV was given 06/04, 09/04 and 11/04
      HIB 06/04 and 09/04
      DTaP/DT/Td given 06/04, 09/04, 11/04
      Hep B 4/04, 6/04, 09/04 and 11/04
      Polio 06/04, 09/04 and 11/04

      To my knowledge, he has not gotten any other shots. My husband and I started seeing a difference in Dell at 5-8 months, which would have been Sept-Dec.

      I’m sure the immu. dr. would run whatever titres you are talking about, I don’t know what your are talking about, too much above me.

      Also, I know I recently emailed you but did I tell you almost all the cd’s have gone down from 2/07 to 2/08. Rituxan was not introduced in that time. Only CD 19 went up.

      Thanks, Lori

    • Anonymous
      March 20, 2008 at 11:51 pm


      T-cell deficiency does not seem linked to autoimmunity – hope this calms your fears til more is known.

      quotes: (emphasis mine)

      Autoimmunity is uncommon in patients with pure T-cell
      immunodeficiency (Rosen, 1987). Children with X-linked
      severe combined immunodeficiency exhibit T-cell maturation
      arrest within the thymus (Sleasman et al, 1994). B-cell
      numbers are normal, and immunoglobulin secretion can
      occur in the presence of normal T-cell help. Although this is
      the most common form of human SCID, autoimmunity has
      not been reported in these patients,…..

      While opportunistic infections are a
      prominant feature of both adults and children with idiopathic
      or congenital CD4+ T lymphocytopenia, [COLOR=”Blue”]autoimmune disease
      has not been associated with this newly described
      immunodeficiency of CD4+ T lymphocytes [/COLOR](Sleasman et al,
      1990; Duncan et al, 1993; Ho et al, 1993; Smith et al,
      1993; Spira et al, 1993).

      In contrast to purely T-cell deficiencies, defects in immunoregulatory peptides involved
      in cell-to-cell interactions are commonly associated with
      autoimmune disease.

      [COLOR=”Blue”]Unlike T-cell defects, humoral immunodeficiencies carry
      a high risk of autoimmunity.[/COLOR] Children with X-linked
      agammaglobulinemia have little detectable immunoglobulin
      production due to a maturation defect in B-cell development
      (Conley, 1985). Children with this disorder have a high
      incidence of polyarticular arthritis and inflammatory bowel
      disease (Barnett et al, 1970). X-linked hyper-IgM syndrome
      results from a mutation within the gene encoding the ligand
      for CD40 (Aruffo et al, 1993). This defect results in
      ineffective immunoglobulin isotype class-switching from
      IgM to IgG or IgA. In addition to antibody deficiency and
      recurrent infections, affected boys have a high incidence of
      autoimmune thrombocytopenia and autoimmune hemolytic
      anemia (Cairns and Rosen, 1986).

    • Anonymous
      March 20, 2008 at 11:53 pm


      You were posting when I was typing Compactdisk’s reply.

      Thanks for your prayers, we need them. There can’t be a correlation between Rituxan and the numbers because these were done 2/07 and 2/08. The February/08 bloodwork was done the day our first Rituxan treatment was started but the blood was drawn before any Rituxan entered his body.

      Now, some of the CD numbers SHOULD continue to go down, I know CD19 should but I’m not sure about the rest.

      The diaper rash has me puzzled.

      Thanks, Lori

    • March 21, 2008 at 12:06 am

      What about thymic hyperplasia. The thymus is where the t-cells are seperated into good and bad, our kids do not do this, hence the cidp. This process is related to cd4. CD4 is a positive t-receptor cell, and our kids does not work. Apparently ther is a drug called septrin that is relative to this, I have not looked it up for Kevin yet. Additionally, I think there is some connection between cd95, fas ligand.

      I read that thymic hyperplasia is defined as an enlargement of the thymus that occurs when stress has occurred via chemotherapy, steroid therapy, irradiation. This causes the thymus to become atrophic. Once the stressors are subsided, the thymus shrinks in most but not in all. there is also a phenomenon called rebound hyperplasia where the thymus increases even with cesation of chemo or steroids. Has Dells thymus been checked?

      Sorry for bouncing all over, so many thoughts! Good luck tonight!

    • Anonymous
      March 21, 2008 at 2:01 am

      The diaper rash is one of the few things that “fit” into the 10 signs of pid – chronic skin infection (and failure to grow). You had mentioned that Dell has been healthy – no colds, sinus, ear infections. That is supposed to be one of the hallmark indicators of pid when combined with low immune cell counts. Dell has always had cell counts at the low end of normal until just recently when they started dipping below acceptable levels. That makes me wonder if his latest low counts are a result of all the different treatments to suppress in the past year as opposed to an inherited deficiency.

      Warning signs of primary immunodeficiency:

    • Anonymous
      March 21, 2008 at 5:25 am

      Hi, I would like to offer a few comments.
      First and foremost, people consider not only the absolute CD4 count, but also the relative numbers and function when thinking about immunodeficiency. The absolute numbers can go down if the number of white blood cells are reduced. This definitely happens after rituxan and can happen after IV IgG. I have seen a lowered white blood cell count after almost every cycle of rituxan given in children in our clinic including given for autoimmune diseases. Please let us know what his WBC count is. He could have an appropriate CD4 percentage, but a low WBC and the absolute number of CD4 cells will be low. For example, if someone has a WBC of 5000, the calculated number for absolute CD4 might be very different than if the WBC was 1000, but the percentage could be appropriate.
      T cells are the generals or directors of the immune response and help make sure than B cells act appropriately. As compactdisc states, often people look at T cell dependent immune responses to see if the rest of the immune system “works” specifically against challenges in order to figure out if the problem is just with T cells, just with B cells, or with both. Often people look at specific antibody titers to tetanus and pneumococcus–which is why he asked about immunizations.
      The fact that Dell is almost 4 years old and has not had lots of significant infections is a very strong argument that he does not have a severe combined immunodeficiency with both B and T cells not working.
      The most important point of this point is that people have to look at the whole immune system to figure out what is wrong and that a low CD4 count does not always mean that the problem is with the T cell system.

      2. Whatever the reason for a lowered CD4 count, it is definitely worth being safe to take bactrim (as I think you meant) or septra (as Dawn meant). this is the same medicine, but two different brand names. It helps prevent “opportunistic infections” particularly a kind of pneumonia for which T cells are important fighters. At our institution, we often put children who are getting high doses of steroids on bactrim (septra) as a precaution.

      3. My third point is that it is REALLY unusual for a tiny baby like Dell to get an autoimmune disease. It happens, but people should always think about if the immune system is able to regulate and balance itself correctly when this happens meaning if there is a primary immunodeficiency problem that leads to dysregulation. Your immunologist is good to think about it. It does not mean that it is there, but you want to know because if it is…
      a. All of you can know other ways to help keep Dell safe in life.
      b. It might give some insights into other ways to treat the CIDP.
      The biggest point of this is that knowledge is not always bad and that if he has a primary immunodeficiency, it does not mean it is a completely separate disease–they are likely related and knowing this might be a good thing because it might help change the approach to them and might even result in better control of the CIDP, but definitely in better safety with treatment.

      4. If the WBC number and the absolute B cell number were okay before the rituxan, it might be a good thing to have the whole picture looked over by someone that specializes in primary immunodeficiencies in children. Many major medical centers with children’s hospitals (typically associated with medical schools) have such departments. We have a really good immunodeficiency clinic here at this very large children’s hospital in Indiana, but I know there are also good ones in New Orleans (through LSU or Tulane) and University of Alabama in Birmingham. I cannot at this late hour think of people in Mississippi, but I could ask around if you would like. I do not know where you live in Mississippi. If you live in northwest Mississippi, St Jude’s probably has primary immunodeficiency experts.

      WithHope for cure of these diseases.

    • Anonymous
      March 21, 2008 at 9:37 am


      I am so sorry to hear there are more problems for Dell. I hope with all of my heart you find out what’s really going on and can get it treated.

      you’re in my prayers,


    • Anonymous
      March 21, 2008 at 9:59 am

      I think I might have found something but not sure. Dell was on Copaxone, an MS drug from May/07 until about Sept/07. His IL2 numbers really shot up which was the idea. I believe IL2 fights micro infections.

      I have copied the below from a MS site:

      “Copaxone administration elevates a particular type of immune system cell
      These cells are of the CD8+ class, known as the killer T cells for their ability to destroy infected cells
      These killer cells, in the presence of Copaxone, regulate the immune system by killing another type of immune system cell known as CD4+
      CD4+ cells are usually considered “helper” cells in that they do not actually kill other cells but “help” the rest of the immune system in doing so by, among other things, activating and directing the killer cells
      Why the killing of CD4+ cells by CD8+ cells turns out to be a good thing is not quite clear. The immune system is exceedingly complex and any number of theories could be presented, such as that the particular “helper” cells that are killed were the ones directing the attack on the central nervous system. However, what can be deduced is that Copaxone has some immune-system modulating effect that is generally beneficial to MS’ers, and a rising number of at least this particular type of killer cell is not detrimental to an MS’er (at least overall)– otherwise, Copaxone would have likewise had a negative effect on patients. For so many years, the mantra has been that boosting an MS’ers immune system would be a bad idea… as research continues to unfold the puzzle that is MS, it is become clear that that is not entirely the case. ”

      Dell’s numbers looked 1/2 way decent 2/07. His WBC has consistantly been normal, around 9-10 his entire life.

      2/23/07 8.2
      2/29/08 9.9
      3/6/08 9.3
      3/13/08 3.5 low
      3/20/08 6.2

      These are the dates of his Rituxan treatments:

      Also, his SED rate has been up for most of his life. One week it will be in the 30’s, then down, then 2 months later, 50. The SED goes back and forth. He hardly ever shows an outward sign that the SED is going to be up. Sometimes he does, he’ll have an elevated temp, around 99.9.

      Thank you to everyone that has been helping. I slept better after I found out about the Copaxone. I will be calling the Copaxone company soon to talk to them about what the counts should be and how long they will stay down.

    • Anonymous
      March 21, 2008 at 11:31 am

      Hi Lori,

      I am so glad to hear you were able to get some sleep. There are so many intelligent and knowledgeable people on this site. I am constantly learning from them. I hope they help you find the path to answers….In the meantime, always remember to take care of yourself. A strong and healthy caregiver is so important to those of us who struggle with this disease. You all mean the world to us and it is hard to remember that some days when the going gets tough. I know I often got caught up in how this affected me but forgot to consider the impact it has had on my husband and family. God bless…


    • Anonymous
      March 21, 2008 at 12:28 pm

      I’m so sorry you have to go through all this. I wished I could be of more help to you. Perhaps a chart would let us see visually what’s going on with the numbers. You did not mention when he was getting Solumetrol and how often the Copaxone. I still think Solumetrol might have an effect on all cell counts. It seems clear from looking at the chart that the Rituxan did have an effect on the white blood count which is to be expected for the B-cell component. As I told you in another post in my case the effect was not very great. But then children often react much stronger than us adults.
      I would be happy to update the chart with more information if you feel it is helpful.
      Take care

    • Anonymous
      March 21, 2008 at 12:44 pm

      One more thing: CD4 is also found on monocytes and macrophages. I am still researching Copaxone. It shifts from Th1 (inflammatory) to Th2 (anti-inflammatory). I’m trying to find out if CD4 appears on either one.

    • Anonymous
      March 21, 2008 at 1:29 pm

      Dell was getting copaxone from May-Sept, 07. He took 1/2 dose every other day. The high dose solumedrol started about March, 07 to present. He’s getting 160 mg of that each week.

      I did contact the immuologist and tell him the connection to copaxone and CD4. He did not think it would still be playing a part in his immune system. He also stated he could not believe Dell has not been sick. He said he’s treated other PID children with ivig but they still continue to get sick. Maybe not as much as without ivig but they still get sick. Dell, knock on wood, has been quite healthy.

      I contacted the distributor of copaxone and spoke with a real nice pharmacists.

      He was interested in Dell’s case and made the statement that Dell’s situation is unique (yes, I’ve heard that many times) but he could not shed light on the CD4 and other CD numbers being low. He does not think copaxone would still be in his sytem.

      He thought I could be on to something but since he did not think it was in the system any longer, he did not know the connection.

      Thanks for your assistance.
      Love, Lori

    • Anonymous
      March 21, 2008 at 2:28 pm


      The good news is that Dell is not sick – just his numbers are not what was expected.

      Here, another explanation for possible causes of low t-cells:

      (in case it becomes relevant to future developements)

      a transient decrease in t-cells occurs that is “likely to be associated with the use of corticosteriods”. This decrease is recovered in about 4 weeks.


      There was no clinically relevant difference between treatment groups in mean changes from baseline
      in total T-cell (CD3+) counts, helper T-cell (CD4+), and cytotoxic T-cell (CD8+) counts.
      [COLOR=”Blue”]The observed transient decrease in mean counts after each infusion (recovered again to baseline levels by Week 4) is likely to be associated with the use of corticosteroids during the initial phase of the study.[/COLOR] The [COLOR=”Blue”]latter seemed to be confirmed [/COLOR]by the impact of the corticosteroid regimen on T cell counts observed in the phase IIb study.

      Additional info from Copaxone Label:

      Adverse Reactions

      Hemic and Lymphatic:
      ◆ Infrequent: Leukopenia, anemia, cyanosis, eosinophilia, hematemesis, lymphedema, pancytopenia,
      and splenomegaly.


      You have such valuable knowledge from your experiences – would you have any info/experiences with

      lymphocyte transformation test (LTT) or DTH Test? The suggestion of looking for titres against vaccinations indicate a state of functionality of th-2 cells, and now I am looking for specific tests that would reveal function of th-1 and th-17 cells.

      Three subclasses of CD4 “effector” T-cells:

      Th1 intra-cellular pathogens
      Th2 extra-cellular pathogens – signals b-cells
      Th17 inflammatory response

      If I find something significant, I will post for all (along w/references).


    • Anonymous
      March 21, 2008 at 4:35 pm

      compactdisc already answered my question about CD4. I did not know about Th17.

      Here a chart showing the interrelationship between the two subtypes of T helper cells. It also shows which one produces what cytokine, IL-2 versus IL-4 etc.


      On the subject of the white blood count: looking at the distribution of different cell types, I don’t think that the Rituxan would have caused the dramatic drop. I still suspect the Solumetrol especially since you are saying that he’s getting it every week. Here’s the breakdown:

      [U]White blood cells percentages[/U]:
      Neutrophils 50 to 70%
      eosinophils 1 to 3%
      basophils <1%
      lymphocytes 28 to 39%
      monocytes 3 to 7%
      NK cells 10 to 19%

      I could not find the breakdown of lymphocytes into T cells, B cells and macrophages. NK cells are also lymphocytes but shown separately in the table above.

      Take care

    • March 21, 2008 at 4:54 pm

      Does anyone think the thymus gland has anything to do with this? Since the T-Cells are separated in the thymus gland, and all of a sudden there are these new problems, could thymic hyperplasia be responsible for the new problems?

      CD, is what you mention regarding steroid treatment the same as thymic hyperplasia?

      Could the thymus be enlarged?

      I hope you find some answers soon from the doctors, we are thinking about you and will pray for you in church tonight!

    • Anonymous
      March 21, 2008 at 5:30 pm

      Lets hope its the Solumedrol. I updated Dell’s chart (see below). In February 2007 his CD4 count was normal. The following month he started getting weekly treatments of Solumedrol and the CD4 count kept going down ever since. It could be coincidental but in my opinion it looks rather suspicious.

    • Anonymous
      March 21, 2008 at 6:26 pm

      The neuro. just sent me an email. He wants to repeat the CD4 with a stick and not use the port.

      Don’t know if it should make a difference but I’m all for it.


    • Anonymous
      March 21, 2008 at 8:55 pm

      I called the hosptial where the flow cytometry is done. I want to find out if he had flows done before 2/07. They are out for the holiday.

      I haven’t written the neuro. about any of this. Yesterday, when the immu. talked to him, he did tell him he was concerned about the CD4 count and would start him on antibiotic. The neuro. agreed it was a good idea.

      I don’t know when we are going to bring the neuro. in more. Poor man, he’s so busy and has so many patients, he does not have the time to devote to research. The other day, at 5:00 p.m, I gave him a donut and he said that was the first thing he had eaten all day.

      The immu. is new and does not have very many patients. I told 2 of my friends, that man is going to curse the day I called to get an appt. in his office, lol. He also didn’t know what he was doing when he gave me his email address, lol.


      Don’t know if this means anything but I’m going to post it. Dell has almost always had high counts of eosinophils. Regarding lymphocytes normal range (35-65%) I am including b/w that was done before high dose solumedrol. Actually, he was probably only receiving ivig at this point.

      2/06 54
      5/06 30 Low
      6/06 33 Low
      9/06 38
      10/06 14 Low:

      He had amino acid qnt, csf test on 11/06 and ALL were normal. There were 25 of them.

      If anyone has any tests that you think are important to the equation, please let me know, I may have results at home.

      Love, Lori

    • March 21, 2008 at 11:07 pm

      Regarding the antibiotic, check a post by Norb, immunosuppressants explained or something like that. He mentions in an article he refered to that cyclosporin specifically works with t-cells. Maybe you could copy the info he posted and ask the doc if it has any relavence to Dell.

      We just returned from the living stations for Good Friday service, I prayed for Dell, Kevie, Emily, Abby, all the children, and all of the adults. I asked God if he could cut the time short that everyone has to bear this cross of cidp and help the scientists find a cure quickly. I didn’t get an answer, but I am not giving up hope!!!

      Praying that tommorow brings a brighter day!
      Dawn Kevies mom

    • Anonymous
      March 22, 2008 at 9:45 am

      Thank you Dawn,

      That is so nice of you to bring Dell up in prayer. We are Catholic and I hold Mary is such high regard. I pray to her to intercede on Dell’s behalf to heal him. Prayer is a wonderful thing.

      I woke up to a good email. The immuo. sent an email at 1:43 a.m. Poor guy, he’s probably lying awake thinking of Dell. This is the email:


      “I’ve been thinking about this alot. I don’t believe Dell has a PID. Like I said before, he would likely be very sick, if these counts are correct, and of course he doesn’t have the symptoms. I believe these labs are an error. Do they heparinize or flush his port before drawing labs? This would dilute his blood counts and make them very inaccurate. The copaxone and rituxan are not causing these low counts. Copaxone would still have to be in his system, and it’s not. Also, his counts were quite low before the rituxan was given, so diluting the blood draw is the most probable explanation. I’m sorry if I have worried you, but I didn’t think about the port draw issue earlier. I still have not heard from Dr. Sorensen, but I’m sure he would agree with this. Still keep giving the bactrim daily for now.”

      I sent him an email explaining they take out at least 5 ml of blood before they do the test so I don’t know how much error there is. We are going to draw in the elbow on Monday. I’m excited but those results won’t be back for about 5 days. It will be 5 days of agony.

      Dawn, we tried cyclosporin on Dell about 1-1/2 years ago, may 1 year. That was the nastiest thing I’ve ever put in my mouth. You could not hide the taste. It was about 10 times worse than fish oil. It took my breath away.

      Kirk and I are going on a much needed lunch today. We have not had a sister since New Years and can’t remember when the last time was we went out together.

      Love, Lori

    • Anonymous
      March 22, 2008 at 9:57 am

      So I was up thinking about Dell too last night. When were the blood draws taken? Were they shortly after getting Solumedrol? If the Solumedrol can affect C4’s then maybe you should wait until the day before the next Solumedrol infusion to do a draw. Just to see if that makes any kind of difference. The dr might order a series. I would think that he would want to get the levels before, a few days after & then right before the next dose to see if there is any change.


    • Anonymous
      March 22, 2008 at 11:54 am

      we have had 4 cd panels drawn. the first being in 2/’07 and the other 3 beginning in 2/’08 which is when we began the rituxan. in 2/’07 his absolute cd4 was 1074 and in relative order are 560, 523 and 295. the range given is 500-2400 (but the dr. states a small child should be at 1000, those ranges are for adults). he always completes the solumedrol prior to rituxan (usually the day before if not the same morning) prior to receiving the rituxan.

      He did not have a port on 2/07 so we are keeping our fingers crossed the dr. is correct.

      Love, Lori

    • Anonymous
      March 22, 2008 at 12:20 pm


      This sounds promising, it makes sense. Enjoy your lunch date, no jello.