All answers today are based on personal experience of the participants. As always, please consult your personal physician prior to taking any action.

Clinicians:

Todd J. Kilbaugh, MD, Children’s Hospital of Philadelphia, Philadelphia, PA

Amy Goldstein, MD, Children’s Hospital of Philadelphia, Philadelphia, PA

Q: Are there any problems with dental anesthetic injections in the gums or on the roof of the mouth in people affected with Mito?

A: Amy Goldstein, MD: I have a little bit of a particular bias with dental procedures, and I get a lot of people that send me forms asking to sign off on this dental procedure, which is being done in an outpatient office where somebody wants to put my child to sleep and this scares me. I want my patients being taken care of in my hospital, and it’s incredibly biased of me to say that, but I don’t know who’s putting your child asleep in your private dental office. I want my anesthesiologist putting your child asleep where I know that they’re going to be safe, and that all of these precautions that we’ve put so much time into, that they’re going to be in safe hands. Now, having said all of that, if it’s an adult or an older child where we just need a little Lidocaine or Novocain to numb up a little local area, so you can have a cavity filled, I don’t have a problem with local anesthesia. I have a problem with nitrous. And again, I’ll let Dr. Kilbaugh answer that.

A: Todd J. Kilbaugh, MD: Yes, I agree with Amy. First of all, I have no problems with local anesthetic. I personally don’t like nitrous oxide for any patient whether they have mitochondrial disease or not, and we don’t actually have it in the hospital. I wouldn’t let my kids have nitrous oxide at a dental office. If you’re an adult and you want to have it done there, because you have a particular provider, I think it’s something that you need to have a very good conversation with the anesthesia team at that dental practice and make sure that they’ve taken care of patients with mitochondrial disorders, and they have a clear and cogent plan, and can even work with your primary mitochondrial physician as well. But in general, these types of procedures that if you do have to have sort of oral max or facial surgery I would suggest that it’s better done at a tertiary center that handles patients with mitochondrial disease than to have that done in someone’s office.

Q: What anesthetic do you advice for a colonoscopy?

A: Todd J. Kilbaugh, MD: I think it’s safe to use propofol for this, for less than a 1-hour period, or for short procedures, like if you are having a short colonoscopy or something like that. I think that is still a fairly safe, good, tolerated drug by patients.

Q: What is the recommended anesthesia for elderly patients with Mito? I was overdosed with propofol my last surgery.

A: Amy Goldstein, MD: We do not want to use propofol in a prolonged way, and prolonged means more than an hour. So prolong use of propofol should be avoided. But we can use that during induction, meaning, if it’s less than an hour to help somebody get into that anesthetic mode, but not to use it for the whole case. And one more caveat, there are children and adults that have fatty acid oxidation disorders, and we know propofol is contraindicated for patients with fatty acid oxidation disorders because it does interfere with fatty acid oxidation.

Q: What types of anesthesia medications should be avoided?

A: Amy Goldstein, MD: For children and adults that have fatty acid oxidation disorders, we know propofol is contraindicated for patients with fatty acid oxidation disorders because it does interfere with fatty acid oxidation.

Q: What do we do if we should have fluids while fasting but have a heart issue and this could overdo the fluids?

A: Todd J. Kilbaugh, MD: So I think this is really critical. This is sort of a cheat answer, but it’s just actually the truth: You need a good anesthesiologist. You need people that take care of patients that are complex. I’m not going to give you a succinct answer in one step because it really depends on that patient and what their heart dysfunction is like: Is it global? Is it just their left ventricle or their right ventricle? Do they have valve problems? Is it a child with mitochondrial disease that tends to have lower cardiac output from cardio myopathies? These are all things that are absolutely critical to watch, and I might be more conservative with the amount of fluid that I would give them. That might be a patient where I would make their hemoglobin 8 to 10 and not use as much crystalloid and use things that are more colloid-based fluids like blood or FFP, depending on what their cardiac or renal disease is. These are complex kids that don’t follow the rules. Anybody that’s on here is here because they understand that these kids are complex.

You need to go to a good center. You may have good primary doctors around you that are invested in the care and that are talking to the anesthesiologists. The surgeons need to be aware of the risks and being invested in that case and not just say, we’re just going to do this case because it’s just a G tube, or we’re just going to do this with the peanut case. They need to know that needs to be the first case. They need to know that these kids will go to the ICU afterwards. They need to know about the consultants that they’re going to need around their care. And you really need to have everybody on the same page. And when you have that, these kids do well.

Q: Are there institutional protocols for anesthesia and post care dosing recovery for young adults requiring it for short periods, but allergic to propofol? Our situation has always required extra hydration post procedure, extra inpatient day.

A: Todd J. Kilbaugh, MD: To maintain hydration they need glucose in their fluids. But your glucose needs to be checked, so how much glucose depends on what a patient’s glucose is, and that can be modulated over time. But certainly most of these patients come out on maintenance fluids. And then, like we discussed earlier, we’d want to switch to some type of parenteral nutrition if they can’t tolerate nutrition quickly.

Q: Should I consult with the anesthesiologist prior to the day of my, or my child’s surgeries? I’ve been told they don’t choose an anesthesiologist until the morning of.

A: Todd J. Kilbaugh, MD. Almost all programs have a perioperative medicine group or anesthesia resource center that you should be able to talk to. And just before you have a procedure or at least a nurse practitioner that works with the anesthesiologist before you have the procedure. It’s actually the day before surgery, required for most of us to call and ask patients whether they have any issues or concerns for the next day, or if someone has a more complicated history. It’s always important to try to work with your primary care physicians and especially your surgeon.

If you’re beginning to book for a surgery, I would say the cheap code in any hospital to get to anesthesia is to talk to your surgeon and say, my child or myself has a particular issue that I need to talk to somebody before I have surgery or I’m not going to do it. And if you do that, in certain offices that will get you in touch with someone. So if you’re having a hard time, I would go through your surgeon to make sure that, you can get in touch with the anesthesiologist, but I think it’s critical that you have these conversations upfront. But again, at a place like CHOP, a place like University of Texas, most of these major centers, we all have anesthesia perioperative teams that are there to answer questions and to make sure that there’s a full cohesive plan going into surgery.

Q: How can you unfreeze a colon faster after dental anesthesia? And if you have gastroparesis should you should fast for longer?

A: Todd J. Kilbaugh, MD: This is a common problem for all people, but especially for children that suffer mitochondrial disease or adults that suffer from mitochondrial disease for that matter. So very important, if you have a surgery that requires you to be on opioids afterwards for pain medication, you need to be on some type of stool softener right away. You know, things like Senna and other things. Then I think it’s important to start parenteral nutrition, so IV nutrition soon afterwards. A little bit of feeds even if it’s 1 to 2 CC’s an hour and see how much their gut will tolerate it. Because I do think that that helps the gut to sort of wake up a little bit and also tends to bathe the lining near your gut so you don’t lose as much of the good bacteria that tends to be in your gut. So as an ICU doctor, we start what’s called our trophic feeds or TROPHIC. Very early, even as 1 to 2 CCs an hour.

I think that’s also pretty important and things that I would talk to my anesthesiologists and my ICU doctors afterwards. So I think it’s important to be aware of it, stool softeners, early IV nutrition, and to think about early trophic feeds as well.

Q: I have an RYR1 mutation as well as mitochondrial myopathy. I have to avoid volatile gases, so they have gone to using propofol. What should they use if the anesthesia has to be given for longer than 1 hour?

A: Todd J. Kilbaugh, MD: We know that almost all anesthetics inhibit complex one. Almost all of them, take your pick – Dexmedetomidine and volatile anesthetics cause more complex one dysfunction than the other anesthetics. So I think it’s safe to use Propofol for this less than 1 h period or for short procedures. It is a very good induction medication. It’s a very good maintenance medication for anesthesia. But now in the world of that, we can use things like dexmedetomidine, for example. Ketamine is another example, of a drug that we can use, in combination with TEA.

Q: Are there any gasses to be avoided during surgery?

A: Amy Goldstein, MD: Nitrous Oxide.

Q: Are there fluid protocol for post op recovery in Mito patients with RTA on high dose bicarb daily.

A: Todd J. Kilbaugh, MD: Yeah, I think if it’s to maintain hydration. They need glucose in their fluids. But your glucose needs to be checked, right? So how much glucose is dependent on what a patient’s glucose is and that can be modulated over time. But certainly, most of these patients come out on maintenance fluids, and then like we discussed earlier, we want to switch to some type of parenteral nutrition if they can’t tolerate nutrition quickly