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
- What about with an infection or while on antibiotics?
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
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.
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.
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.
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.
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! :)
Love this! It is illuminating. Found myself imagining I am the one asking my doctor those questions. Could not help but share the post on my facebook and twitter.
ReplyDeleteThank you Walter, your support means a lot to me, and I appreciate you spreading the word about my blog (which helps spread awareness of this disease). :)
ReplyDeleteI also asked some of the same questions to my doctor that you did. Some of his answers (doctor at Johns Hopkins) were different. He was not at all concerned about the Hizentra. My IgA is less than 5. My local doctors are very concerned and so am I. The local doctor wants me to go back on IVIG... but I like the sub-q. It's so much easier and I don't get the extreme headache. It's hard for me to weigh all the information and make right choices.
ReplyDeleteThanks for the blog. There is so little personal info on this. Technically, I have Good Syndrome(thymoma with immune deficency) I have IgA >4, BUT it just means I am not making it. I was tested to see if my body was destroying it(and that was why it was low) but that is not the case. So, I can have IgA without a risk of anaphylaxis. Unfortunately, they don't replace IgA...only IgG (mine was at a level of 52). I receive infusions of gammaguard monthly. It's helped a lot.
ReplyDeleteThank you for this information. I just received my diagnosis and find this new world very overwhelming. I start my first Hizentra treatment this Friday. If you don't mind my asking, what was the bad reaction you had to it? My dosage is low, but I am still scared! Thanks!
ReplyDelete