I have 3 neuroendocrine tumors in left lung: Can I handle radiation?

Hi Teresa,
I have 3 mutant genes, two of which encode proteins that are located in the mitochondria. The first is SDHB Ile127Ser, c. 380 T>G, which means that at position 127 of the protein, the Isoleucine amino acid has been replaced with the Serine amino acid. This is a pathogenic, i.e., disease causing mutation. The protein is located in the respiratory chain of the mitochondria inner membrane, and it is one of a chain of proteins responsible for producing ATP from food. The second mutant gene is FBXL4 Cys547fs*, c. 1641_1642, which means that at position 127 of the protein, there has been a frame shift mutation, so everything downstream in the protein is incorrect and the protein most likely terminates early. This is pathogenic as well. FBXL4 is important for fusion of mitochondrion to form more productive mitochondria, and it also regulates the degradation and recycling of defective mitochondria via lysosomes. I am heterozygous (one good copy, one bad copy) for each of these genes and both of my mutations is recessive for mitochondrial dysfunction. I've been told that I have only half the amount of normal SDHB and FBXL4, which means that my mitochondria aren't functioning at full capacity. (If I was homozygous for either of these two genes, i.e., it would be fatal in infancy). So, it is the combination of the two proteins, each operating at 50% capacity, that is likely causing mito dysfunction and neuropathy, along with a number of other symptoms. These two mutant proteins cause over time damage to the mitochondria, so that the mitos eventually are degraded by lysosomes. Ultimately, this can produce mitochondrial depletion which means symptoms including neuropathy, chronic fatigue, GI issues, etc. which vary from one person to the next. If your doctors suspect mitochondrial myopathy, genetic testing is important. To cover all the bases, they may do whole genome sequencing or if they are looking for suspected culprit mutant genes, they may only do a panel. Re the neuroendocrine tumors, these potentially could be caused by mutant SDHB. SDHB Ile127Ser is dominant for tumors/cancers, and I've read that there's about a 20% chance of developing a tumor if you're a carrier. I had one at age 9; it was not cancerous and never recurred. So it's my mutant SDHB that gave rise to the NET, and the combination of my mutant FBXL4 and SDHB that likely is responsible for mitochondrial dysfunction and neuropathy. (It's strange, but true, that mutant SDHB is dominant for tumors, but recessive for mitochondrial dysfunction.) There are 100s or 1000s of other genes that encode mitochondrial proteins, and if mutant, might cause mitochondrial dysfunction. I would appreciate learning from you how it turns out with Univ Michigan. Best of luck!

I definitely will

@arleighna I hope the treatment goes well next week and the anti-nausea med helps you.
Will you post again and let me know how you are feeling?

I restart this coming week. The dr did precribe a new nausea med, but I'm a little leary of it because it's a psych med and can have some serious side effects. But on the bright side, I'm definitely looking forward to feeling better this month and being more active.

I understand what you mean, @arleighna. How long do you have to wait before beginning the immunotherapy?

Yes. I was on immunotherapy for almost a year and my last pet scan showed only some small glows. The dr gave me the option of continuing immunotherapy or trying the lanreotide to try to control the nausea and diarrhea, it had a 50/50 shot. I never thought I'd be glad to be back on an infusion. At least I know it can be worse and be grateful for the 3 bad days after infusion verses a whole month of really bad days

Hello @arleighna,
I am sorry to hear of the problems you are having with lanreotide. It sounds like restarting the immunotherapy might be a good plan for now. Did you have immunotherapy before trying the lanreotide?

I've stopped the lanreotide and am restarting the immunotherapy. My dr told me we would know by the 2nd injection whether it would work for me or not, and it actually made my nausea, vomiting, and everything else worse. It gets a little frustrating sometimes but it eases my mind to know there are others out there who can relate. So thank you again .

@arleighna - it gets better with time! My first 6 mo I was in bed for 2 days, nauseated. Now I get tired and slight headache on the day. I also take Zofran 30 min or so before injection. The day of the injection I eat light - crackers and swiss cheese.
Hang in there! There are benefits to Lanreotide!
I also take bitter melon and watch my carbs, and my glucose seems to stay stable (~130 in the morning)