Ask the Mito Doc – November 2022: Q&A: Maintaining Immune Health in Mitochondrial Disease

Ask the Mito Doc- November 2022

Maintaining Immune Health in Mitochondrial Disease


Clinician: Dr. Peter J. McGuire, MS, MBBCh National Human Genome Research Institute


Watch the full video:

Ask the Mito Doc: November 2022 – Maintaining Immune Health in Mitochondrial Disease – YouTube


Q: Is that OK for a Mito patient on IVIG to do the IgG infusion while they are sick with the flu, virus or have an infection?

A:  IVIG is essentially pooled immunoglobulin or antibodies from anywhere between ten to one hundred people. These are antibodies are quite diverse in terms of their ability to recognize different viruses. Because you’re getting it from a whole bunch of different people and they’re normally adults. Adults have a lot of different exposures during their lifetime. So, it can be quite helpful or protective.

This question should be discussed with the immunologist or the neurologist who is doing the infusion. But what I can say is the antibodies that are in that infusion are quite good for protecting or resolving infection. As an example, back in 2009 when we had the H1N1, one of the treatments that they used was immunoglobulin. In the ICU for severe individuals, they used immunoglobulin therapy to help during an overwhelming flu infection, it can be quite effective. Obviously, these things need to be taken on an individual basis and discussed with your provider. But the medication itself should be actually helpful.


Q: Do you see adults with Mito to have shingles more or less frequently than the average population? Or does it see it occur at a younger age? 

A: First, shingles is a reactivation of the virus which causes chicken pox.  It is varicella virus that belongs to a family called the herpes viruses.  Herpes viruses are things that cause the cold sore. They’re all within the same family.  Herpes viruses remain in our bodies for life. Essentially, that’s what shingles is. Essentially It is the reactivation of the varicella virus, a type of herpes virus within us tends to happen in older individuals.

Because, as it turns out, the half-life of your protective antibodies against varicella, only last about to 40 to 50 years.  So, by the time you know you hit 40 or you’re above the age of 50, your antibodies have decreased. significantly. You need a booster.

We don’t know the answer to that because we don’t see adults. But here’s what I can tell you. We published a paper a couple of years ago in kids looking at their protective antibodies against vaccine preventable diseases. They were low in their antibody titers for a number of different viruses, but the one that was the greatest was varicella. It turned out that 55-60% of our children that we surveyed there’s about 25 children were negative for protective antibodies against Varicella.  For individuals who truly have mitochondrial disease, that means that they’re not starting out with a good pool of their cell antibodies and by the time they reach 50 or so they could be at an increased risk for shingles. But we don’t have this data because we don’t see adults. We only see children, but I can only comment on the protective antibodies and children, which some children don’t seem to have.


Q: Would you please be able to provide a source or website where to read more on IPMDS? 

A: (IPMDS) international pediatric mitochondrial disease scale. This scale that was developed specifically for children to be able to kind of score their mitochondrial disease. For clinical reasons for research reasons. In other words, if the individual’s disease either progressed or even got better with certain interventions, you had kind of a way of object scoring it. You can find this on the internet.


Q: What’s known about the impact of the ongoing and longer-term effects of C19/Sars2 on the immune system and any other systems for patients with Mito, and what are some steps to take to potentially mitigate those? 

A:  Steps to take to mitigate as mentioned previously such as diet and exercise to maintain health obviously within reason because certain individuals with mitochondrial disease may have limitations as to what they can do.

Long term effects and the effects to the immune system; viruses have developed mechanisms for evading the immune system or doing funny things to the immune system we don’t understand.

We don’t know yet in the mitochondrial disease community what these long-term effects may be. I would imagine it’s still possible for individuals with mitochondrial disease to have some similar symptoms to those who have exhibited neurologic effects from long covid. Covid seems to get into the brain.

There was a paper that was published by a group at NIH that looked at post-mortem brains, essentially from individuals who are passed away, and we’re able to detect Covid in the brains of these individuals.  It does seem to be associated with this kind of cloudiness or fogginess, or inability to concentrate. But we don’t know yet what kind. Because obviously individuals with mitochondrial disease, especially the younger kids, tend to have a lot of CNS disease. We don’t yet understand what that interaction. But we have seen, as I mentioned in individuals without mitochondrial disease. These kind of long Covid symptoms, which I don’t know they would be exacerbated in children with mitochondrial disease or even adults, we don’t know yet. 

Q: Would you still recommend masking in public places? And/or is constantly wearing a mask weakening our immune system? 

A: The mask is not weakening our immune system. The virus doesn’t survive very well outside. It is not very resistant to heat and cold. The sun destroys the RNA. If you are outside and not around large groups of individuals it would be ok to unmask and get fresh air. Times to be concerned are in large groups and inside. Although people seem to be doing less masking, I would recommend doing a self-assessment. When I get viral infections, what happens to me?  Do I end up in the hospital? Or someone who has minor symptoms for a few days. I would say individuals with mitochondrial disease with a history of not doing well with infections should wear a mask in large groups and indoors.


Q:  Are people with mitochondrial disease and frequent infections candidates for IVIG? Are there special concerns for Mito patients during the administration of IVIG? How can they be supported during IVIG?

A:  This is something that we’ve been starting to study because we’re hearing more and more that individuals with mitochondrial disease are being put on immunoglobin and replacement therapy it’s for several reasons, one of which is because they qualify as having immune deficiency. That’s one of the diagnostic criteria. You must have primary immunodeficiency to receive immunoglobulin replacement therapy, and then there are individuals being put on for other reasons.

What we have heard is that individuals have been showing some benefits from this.  The one thing that is of concern when you are receiving a IG replacement therapy is that some individuals may have adverse reactions. It really depends on the individual. 

Some individuals can have allergic reactions, or something called serum sickness to receiving immunoglobulin. In which case they can receive premedication beforehand. I do know of several patients who receive IG replacement therapy, and do quite well, and I have heard of several individuals who have, and it’s not because of mitochondrial disease. It’s just that their immune system just doesn’t seem to like it where they have adverse reactions and require premedication like Benadryl and stuff like that to help prevent having those adverse reactions.



Q: I noticed, when I become overly fatigued or when I overdo it. I become more susceptible to a bacterial infection. – especially something I normally would fight off when well rested. — – I know when fatigue my eyes and other organs don’t function properly. I just wonder what would be going scientifically with the immune system when mitochondria are not functioning properly.

A: This is not unique to mitochondrial disease. Stress in general can have negative effects on the immune system and the ability to fight infection. It’s not surprising that someone, either stressed or not getting much sleep, various kind of things happening in their life. The reason why this occurs is quite complex, but one of the things with stress is the secretion of steroids in the body or corticosteroids in the body because of stress. And unfortunately, those Corticosteroids help you with various parts of metabolism and blood, sugar, and stuff. But one of the things corticosteroids do is they suppress the immune system. It is a normal kind of endocrinologic response to stress. But the unfortunate part is, it suppresses the immune system.  it’s not surprising when an individual experiences those types of things that they might be more susceptible to certain types of infections, we should all be as stress-free as possible, exercise, eat a good diet. Stress, I mean, stress is a part of life, finding outlets to help control that stress can be very helpful.



Q: Besides Mito, I have dysautonomia. The 3 vaccine doses negatively affected my blood pressure. the first dropped it, but the other too made it go dangerous high – when going sitting to standing 220/120 – I was bed bound due to effects of the vaccine. we had to adjust my medications -each time. — why would the vaccine affect the blood pressure like that? I’m concerned about getting the new vaccine due to my reaction to the first 3.  I finally got my blood pressure stabilized and can be out of bed more now and move about some.

A: Normally, you would see blood pressure fluctuations in older adults. We have not heard of this being that we follow mostly kids. We have been monitoring adverse events from the Covid vaccine for a while now in children, we haven’t heard this in the pediatric population. In the adult population we have heard, some individual that become lightheaded and feeling faint. Blood pressure dropping can be a part of that. I don’t know the answer to that yet.