#men2a

I finally went to my doctor and talked to her about all of my anxiety. I have finally accepted that I need more help than just breathing exercises.

Dad was doing a lot better at Christmas. He and Husband A spent the entire time in the kitchen having a full conversation. It did my heart happy to see that. Whenever we left, I could tell Husband A was excited that he got to have that time with Dad. It hasn’t happened it a while. He still has cancer and it’s not getting better, but we’re enjoying the time that we have left.

My niece was born. My SIL has a chronic pain disease that meant she had to continue with pain medicine throughout the entire pregnancy or lose Harlee, so Harlee came early at 36 weeks, but had to spend a week in the NICU. She was home for Christmas, though.

My Mom finally got Genetic testing done for MEN2a, but she came back positive. She will be having her thyroid removed this year. Her Calcitonin levels came back okay, though. They were elevated, but not cancer elevated. My brothers and I all have to get tested now. If I come back positive, then I will definitely be getting my Thyroid removed this summer. If I don’t, then I have a 98% chance of developing cancer. I’d rather take medication for the rest of my life than risk that.

Due to all of that stress, I’ve now started Paxil. It took about 2 weeks to get used to it, but I’m actually started to feel better.

Continuing with Multiple Endocrine Neoplasias (MEN). We will continue with the men of Kuroko no basket and tackle MEN2A. 

MEN2A aka Sipple’s Syndrome has 2 P’s: Parathyroid tumors and Pheochromocytomas (tumors of adrenal glands). So these patients get  hyperparathyroidism, adrenal medullary tumors (pheochromocytomas) that produce catecholamines, and as a bonus can get medullary thyroid carcinomias. 

To remember MEN2A - think Himuro. He always sticks together with Murasakibara - so they’re always 2 men ie MEN2.  He loves pheochromocytoma with all the catecholamines, metanephrines, palpitations, and sweating. It’s almost like he has a thyroid problem. He also gets PARAnoid (parathyroid - kidney stones and increased amyloid) when he’s away from his boo Murasakibara.

In summary:

MEN2A = Sipple’s Syn

Parathyroids

Pheochromocytoma

Medullary thyroid cancer

MEN 1 = Wermer’s Syn

Pituitary

Parathyroid

Pancreas (gastrinomas, peptic ulcers)

Make sure to check serum Ca++ esp if kidney stones/peptic ulcers

As MEN1 ,MEN 2A is also familial. It occurs due to mutation in RET gene on chrom.10 .Also called Sipple Syndrome. It mainly affects 3 organs :

Thyroid:(Medullary Thyroid Carcinoma)

Medullary thyroid carcinoma affects nearly all patients with MEN 2A & it’s the first expressed abnormality. The medullary thyroid carcinoma in patients with MEN 2A is typically bilateral and multicentric, in contrast to sporadic medullary thyroid carcinoma, which is unilateral.

Adrenal medulla: (pheochromocytoma)

This causes increased release of epinephrine & norepinephrine which can be detected by lab tests (also metanephrine, which results from them breaking down). These are vasoconstrictors, so the pt will have paroxysmal hypertension with headache, palpitations, sweating....etc.

Parathyroid:

Hyperparathyroidism is also an abnormality caused by MEN 2A. This abnormality leads to increased Ca levels & thus can cause nephrolithiasis .

Many cases of familial lichen amyloidosis were reported in families with MEN 2A. The lichen amyloidosis in this syndrome is usually localized to the interscapular region consisting of lichenoid papules, with hyperpigmentation and fine scaling. 

Surgical removal of tumors is the main Tx. Prophylactic Thyroidectomy is recommended since MTC occures in virtually 100% of cases.