IDF Patient Education Meetings Throughout the Country!

IDF Patient Education Meeting Join us for a one-day educational meeting presented for individuals, families and health professionals. IDF’s Local Patient Meetings offer an introduction to IDF, and feature local immunologists and allergists addressing the treatment and management of primary immunodeficiency diseases. These meetings are the perfect occasion for patients and families to meet and share ideas. Meetings are happening soon in different locations throughout the country! RSVPs seem to be required 3 days in advance, so go to the website and find one in your area. (Sorry, I didn't realize that one happened yesterday, but others will be happening Nov 6th and later). http://www.primaryimmune.org/IDFCalendar/default.htm Update The meeting was great! It turned out that my specialist was the guest speaker, and it was great to meet other CVID patients in my area. My doctor spoke about the various problems that CVID and PIDD patients encounter, different conditions associated with the disease, and the genetics involved. I video taped a lot of her presentation, but I wasn't feeling strong enough to hold my phone up to record, so the video isn't great, and the main reason to "watch" it would be to hear the information. However; I didn't get her permission to release it online, so I will have to do that before posting it here. If I'm unable to get her permission, I may be able to transcribe some of it.  The IDF invited representatives from pharmaceutical companies who had free gifts for attendees. Some of the things they gave away were visors, water bottles, pens, thermometers, and antibacterial hand sanitizer. In addition, the IDF representative provided pamphlets and books with information about PIDD, including a children's booklet that is very informative. I believe these books and pamphlets can be ordered from the IDF at the their website (see my reference page).  The meeting included a buffet continental breakfast as well as lunch. All in all it was a very enjoyable and educational experience, and I'm very glad I went!

I Just Got Approved for Gammagard SD!

My nurse from Accredo called today to schedule an appointment to train me to use Gammagard SD as subQ! I'm so excited! She will come next Thursday 11/4. She said to expect her late, which is fine with me because I will be working that day.

The pharmacist from Accredo also called and she said that I will be getting 10g of powder once a week. I don't know what volume that will reconstitute to, but she said I will still be using the Freedom 60 pump (which only holds 60ml of fluid, the same dose of Hizentra that I'm on).

Update

The specialist called and told me that they've started the process of authorizing the switch from Hizentra to Gammaguard SD with my insurance company. They are planning to have me start at one treatment a week. She also said that the specialty pharmacy (Accredo) said that if my insurance is cooperative then I could be transitioned in a as little as a few days. I'm planning to just skip treatment this Friday since even if it all gets approved this week, they won't be able to get the supplies to me in time.

If my insurance company causes problems or drags their heels too long, I might have to go back to doing Hizentra, but the doctor said that they'd give me steroids so I could get through it without a reaction. I really hope that doesn't happen! Wish me luck people! :)

Even though it's now days after my last infusion, my legs where I inserted the needles are still very itchy, and I'm getting a rash there. Last night the sites were lumpy, not from a build-up of medication this time, but from the rash.

Hizentra Reactions

I did my 4th Hizentra treatment on Friday, and I think it will be my last one. Despite taking 2 Benadryl in advance, I started itching all over my body and having breathing problems within about 20 minutes of starting my infusion. 

The bright note of my infusion was that I did it in the outside of my leg...on my thigh, about half-way between my hip and knee. the swelling was minimal, and went out about 2". Today (Sunday) it's itchy and sore, but better than on my stomach. My stomach tends to bloat and get really painful afterwards, but I haven't experienced that with my legs.

The specialist from Stanford called to say that my IgA is below 6--which is virtually 0, and when I'd had my appointment there he said that if my IgA is that low I could be building antibodies to IgA. Anyway, they believe that my low IgA is what's causing my reactions to Hizentra and Carimmune. I think I will stop taking the Hizentra until they can get my insurance to approve the switch to Gammaguard SD.

Two Days After Third Hizentra Treatment

For the Third treatment I took 2 Benadryl as recommended by the specialist, and also took 1 Tylenol. By the next morning all of the swelling in my belly was gone and I only had 1 tiny knot. I was so excited because that was so much better than the previous 2 treatments! I felt like I had some energy and got up, showered, and went about my day. By about 4:00 PM I felt really tired, and my belly had swollen up again and was starting to hurt. I put ice on it, and it really didn't seem to help other than soothing the itching. Knots started forming at the injection sites, and increased in size and pain until they were about 1.5" in diameter. Only 1 site did not develop knots, and I have no idea why that is. I wonder whether the ice contributed to the knots forming, or if my jeans irritated my stomach and caused them? I have no idea...

I realized that I probably should have taken more Benadryl the night of my infusion, that might have prevented the swelling and itching the next afternoon.

Appointment with Primary Immunodeficiency Specialist

Today I had my first appointment with a true specialist in immunology at a local research hospital.

He's a pediatric immunologist, but sees adults once a week. I saw a fellow first who was very nice and also seemed very competent, she asked me a lot of questions about my history, especially regarding infections. Then the doctor came in with another fellow and sat down, talked to me in-depth, and very patiently answered my questions.

The doctor is concerned that the original tests that I had at diagnosis were not sensitive enough to detect how low my IgA actually is. My test results show <25, but that just means that my IgA is lower than the test is capable of showing. Because I have a history of bad reactions to both Carimmune NH IVIG, and have had a few bad reactions to Hizentra SubQ, he suspects that I might have an IgA level below 6. He also said that if that's the case, and I keep having Hizentra or other high-IgA gammaglobulin treatments, my body could make IgA antibodies which would mean that I could eventually have very serious reactions like anaphylaxis to the treatments.

He plans to run more in-depth and sensitive tests on me to check out my IgA level, see what antibodies I have for various diseases, test for RA and Lupus, and see what I have in terms of Candida. There was probably more, but I'm having trouble remembering.

I am very excited about being able to see this doctor. I believe he will help me a lot! He recommended that for my next Hizentra treatment I take 2 Benadryl, and 1 Tylenol and see if I do better.

I will keep the Fioricet as an option later if I get a migraine, but because it has Tylenol in it, I would have to wait hours after taking the Tylenol before doing that.

He kept reiterating that CVID is actually a "blanket name" for many different diseases, and they will test me to find out more about what I actually have. I'm so excited! :D

My Questions for the Doctor, and his answers:
(Note, I may have not fully understood his responses, or not written fast enough and can't remember exactly what he said (I took notes as he answered my questions). This is my best summary of what he said to me today. I hope it helps, but check out what I have here with your own doctor)

Questions for PI Specialist

Question: Should I take steroids (or any drug that lowers the immune system) ever? (eg: nasal sprays, inhalers, eczema creams, prednisone, etc)

Answer: Yes, as needed for asthma, allergies, autoimmune disorders, etc.     Avoid over-use of oral steroids due to them affecting the blood

 Question: Should we get treatments while sick?

• What about with an infection or while on antibiotics?

Answer: Yes, unless you have a high fever which might mask a reaction to the medication

Question: Should I stay away from children (some doctors have said that I should)?

Answer: No, but use caution. If they are sick, wear a mask, keep distance, and wash hands frequently

Question: What is your "goal" IgG level for me?

Answer: Based on individual, but around 800 for me. He also said that some people are fine at 600, but he doesn't think that I'm one who would be.

Question: Will you test me for IgA antibodies?

Answer: Possibly, if I have very low IgA (under 6.0)

Question: If I have IgA antibodies is it still safe for me to have Hizentra?

Answer: No because I will make antibodies to the IgA, and will then be more likely to have a very serious reaction to my treatments.

Question: Is it safe for me to receive a blood transfusion?

Answer: It is unless I have very low (under 6.0) IgA, in that case a blood transfusion could be very dangerous

Question: Can/will you do genetic testing? I think there’s a family history...

Answer: In my case, yes because I’ve been sick for so much of my life. A very small percentage of people have the defective TACI gene, and the people with the other associated defective genes are even more rare. He will test me for the TACI defect.

Question: Is it possible to have CVID your whole life, but have your immune system crash at some point where your numbers fall, or will they always be low?

Answer: Yes, but because I’ve been sick for so long that’s probably not the case.

Question: Do you treat the patient or the IgG levels?

Answer: He cares about the patient’s well-being, and some patients are good at lower levels and there are treatments other than IgG replacement that work for them. He does not think that is the case with me.

Question: Are you currently researching CVID?

Answer: Yes, he has a budget for researching CVID and is accepting patients for the research

Question: How will you help me regain my quality of life?

Answer: By finding a more appropriate treatment for me.

Question: Can CVID patients make antibodies?

Answer: Yes, especially for things like Lupus and RA

Question: Can CVID patients  test positive to things like Lupus, RA, etc?

Answer: Yes (see above)

Question: Should CVID Patients wear a Medic Alert Bracelet?

Answer: Yes, especially if they have very low IgA, then they should specify CVID – No IgA (or something like that)

Question: Should I have antibiotics for dental appointments? (Fillings, etc)

Answer: No, the primary reason for that is to avoid heart valve infections.

Question: Can we eat honey safely? I read that it’s an issue w/ babies because of them not having enough good bacteria in their intestinal tract, but ours can be wiped out by antibiotics and other drugs...

Answer: Yes

Question: Should we get vaccinations?
Answer: There's not much point because of the vaccinations being covered by IVIG/SubQ--except the most recent flu vaccination. You especially need it if you're on Hizentra.

Question: Is a problem with the innate immune system (limited/no swelling, low body temp, diminished pain response) related to CVID?
Answer: Yes, the innate immune system is (can be? don't remember which he said) linked to CVID, but they don't know how yet. I also don't remember whether he said for all CVID patients, or just some. I do remember him saying that they see things like low body temp, and not swelling in some CVID patients. He also said that some patients don't heal at the normal rate.

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If any doctors who saw me today disagree with the information I provided here, please either send me an e-mail or write a comment below. I will correct the information right away. Thank you! :)

Reactions since 2nd Hizentra Treatment

I haven't posted since 2 days after my last treatment, and I'd have to say that this most recent treatment was amazingly better than the first one.

I had a few headaches since then, but none as intense as last week, and I made a point of checking that my muscles weren't tense, and taking a clonazepam if so. I saw my chiropractor again on Tuesday and he said that most of my pain was due to muscle spasms or knots, and worked many of them out. I'm starting to think that the Hizentra (and other gammaglobulin treatments IVIG or SubQ) cause the muscles to tighten up, and may even contribute to knots. Just my theory, and I don't have any kind of medical degree, so take it with a grain of salt.

Overall this week has been much better than last week.