So…. we met Moses’ new immunologist. New because, as many of you know, we recently moved across the country back to the city where Moses was born. We spent Thursday at the children’s hospital, meeting the team and getting blood work done. They collected 15 vials of blood from Moses. Goodness!
I’ve been having a hard time emotionally over the last couple of days. I’ve been grieving and sad. Nothing rocks my world like spending the day at the hospital and going through Moses’ complicated medical history. Again. The appointment was all the more difficult because Moses currently has 7 abscesses in three places, which means that the prophylactic antibiotic that Moses is on (Doxycycline) isn’t doing its job. Ugh.
His immunologist recommended a couple of changes to Moses’ treatment. He doesn’t want him going on and off his antifungal (Diflucan); he wants him to be on it all the time. It’s a long story, but 4 years ago, Moses’ immunologist was open to Moses stopping Diflucan to see how he does (since Diflucan can be hard on the liver). Since then, Moses is probably on Diflucan about half of the time – 2 months on it and 2 months off it. He goes back on it when he has a fungal flare up (typically thrush) or the corners of his lips are badly cracked. He’s back on it right now.
His immunologist also highly recommends that Moses receive monthly IVIG infusions, which means spending a day at the hospital once a month to receive a blood product made up of immunoglobulins from 1,000 to 10,000 people. His immunologist in our last city had recommended it too, but didn’t strongly push it and our family wasn’t ready to go this route. To me, accepting this treatment feels like admitting that, yes, my son has a difficult-to-treat immunodeficiency (which, of course, he does, but I try as hard as I can to ignore that fact) and he needs an extraordinary intervention to be well. It means a monthly appointment at the hospital, when my son needs to be hooked up to an IV. It means one day a month of missed school. It means fitting in yet another appointment, while DH and I are both trying to manage fulltime jobs and responsibilities. It feels like a whole lot. And of course, there’s risks involved in receiving blood products. And I even feel some guilt about Moses needing immunoglobulins from so many people to be well.
I’ve known for a long time that approximately 50% of individuals with hyper IgE receive monthly IVIG infusions or weekly subcutaneous IVIG therapy that can be administered at home. Prior to this, we weren’t interested in going this route for the above reasons, and we also didn’t think he was ill enough to warrant this treatment. Also, prior to this, no one seemed very confident that the IVIG would help reduce the occurrences of abscesses. We knew that it could help reduce the occurrence of lung infections (which, thank goodness, Moses hasn’t had for awhile), but would it help with abscesses?
In any case, something has changed and my gut is now telling me, “Say yes to the IVIG – it will help”. And so, we are saying yes. But that doesn’t mean that I can’t grieve. I’m writing this blog post now because it helps me to process and give voice to what I’m feeling. So these feelings can pass, and we can go back to living our very full lives.
I just took a break from writing and dropped Moses off at his friend’s house. He mentioned to his friend’s mom about how he will be needing monthly IVIG infusions. She commented, “But you are feeling well and your energy is good…” Well, yes, that’s true. He looks healthy, well and strong. His main symptoms right now are abscesses, and they are troublesome because a) they can be painful, b) they can burst at anytime if they get to be a certain stage (e.g., he missed swimming today because he was afraid that one of them would burst in the pool) , c) they sometimes need surgical intervention, and d) they sometimes interfere with activities (for example, he missed both of his wrestling practices this week because he wasn’t feeling well enough to participate).
In any case, her comment reminded me that overall, Moses is doing so well. He is. We just want these abscesses to stop. We hope that the IVIG will help his body to fight the infections that cause the abscesses. May it be so.
More reading about hyper IgE syndrome and IVIG
- Wakim et al. (1998) found that IVIG was of no clear clinical benefit in 9 patients, including one with hyper IgE syndrome , and did not significantly decrease IgE levels, IgE synthesis, or other measures of immunologic function.
- Krassilnikova et al. (2007) found that IVIG decreased the frequency of infections for an individual with hyper IgE syndrome.
- Bilora et al. (2000) found that “A moderate dose of IVIG resolved the clinical-radiological signs of the S. aureus bronchopneumonia and improved cytologic and biohumoral parameters,” and represented a useful treatment for pneumonia for individuals with hyper IgE syndrome.
- “Patients with the hyper-IgE syndrome usually have normal serum IgG, IgM, and IgA levels, but some have been reported to have various defects in antibody responses. These include poor anamnestic antibody responses to booster immunization with
FX174, diphtheria and tetanus toxoids, and pneumococcal and Haemophilusinfluenzae vaccines, as well as poor antibody and cell-mediated responses to neoantigens, such as keyhole limpet hemocyanin. There is significant phenotypic variation in the severity of pulmonary infections that is not necessarily predicted by deficits in antigen-specific antibody responses. Despite this, some patients with hyper-IgE syndromes with recurrent respiratory infections mightbenefit from IgG replacement therapy.”- Orange et al. 2006
- This scientific presentation, “What to do when nothing can be done?” mentions IVIG as the most frequently used therapy for individuals with hyper IgE syndrome (besides prophylactic antibiotics and antifungals).
General articles about IVIG
- Anderson et al. (2007) – Guidelines on the Use of Intravenous Immune Globulin for Hematologic Conditions – good general article about IVIG, including costs for our healthcare system in Canada and the types of conditions it should be used for (the articles doesn’t mention hyper IgE syndrome, since it’s a very rare immunological condition).
- Medscape’s article about IVIG.
- IVIG side effects
- Adverse events of IVIG – retrospective study
- Side effects