In which I answer your questions (part 2)

“sm-ahnmd disability”

Systemic mastocytosis with associated hematologic non mast cell lineage disease can be disabling, as can most other types of mast cell disease.  Unfortunately, due to in part to the rarity of mast cell disease, applications for disability are sometimes rejected.  I have not had to apply for long term disability, so unfortunately I am not a great resource for this.  I will reach out to some people and try to do a future post on application for disability.

“mast cell disease and steroid use"

Steroids can be a helpful tool in managing mast cell disease, but they have a laundry list of side effects and long term use can be risky.  The steroids used to treat mast cell disease and anaphylaxis are usually glucocorticocoids.  These medications have a wide range of actions, including suppressing synthesis of some prostaglandins and cytokines, which are associated with inflammation. 

Steroids cause fluid retention and weight gain, including the very typical “moon face” (which I currently have), anxiety and increase in appetite, among other things.  With longer courses, you run the risk of bone loss, joint pain, GI bleeding, and a whole bunch of other stuff.  Steroids are serious medications. 

One of the most serious risks of long term steroid use is suppression of the adrenal gland.  Your body naturally makes steroids, but when you are taking steroids, your body stops making them.  For this reason, steroids often have to be weaned off.  General rules of thumb for when you wean steroids:

-more than 40mg of prednisone (or equivalent of another steroid) daily for at least a week

-steroids have been taken at night daily for at least a week

-steroid treatment has lasted at least two weeks

-several recent courses of steroids.  This is important for people with mast cell disease, because when you have a flare, some people take a Medrol dose pack.  If you do this several times in a short period, you may suppress your adrenal gland.

-steroid treatment within a year of stopping long-term steroid treatment

Steroid withdrawal is real and terrible.  Weaning should be done slowly and cortisol levels should be measured after weaning to ensure that steroid levels return to normal levels.  If you wean too quickly, or your adrenal gland is permanently suppressed, you can develop Addison’s disease or have an Addisonian crisis.  Having had an Addisonian crisis, I assure you it is unpleasant and dangerous.  Our bodies use steroids to do a lot of things and they provide mast cell coverage for us. 

Long term steroid treatment should be avoided if at all possible.  However, this is not always avoidable.

“mast hypertension”

Low blood pressure is more common in mast cell patients, but high blood pressure does happen.  This can happen for a few reasons.  One reason is that hyperadrenergic POTS often coincides with mast cell disease, and this can cause high blood pressure.  Another reason is that mast cells release a compound called chymase.  Chymase is tryptase’s less famous relative, and it is involved in the inflammatory response. Chymase converts angiotensin I to angiotensin II.  Once this happens, angiotensin II increases blood pressure through several mechanisms.  I’m realizing that I have never discussed chymase on the blog so I’ll do a post about this in the near future.  But for some people, the release of chymase by mast cells can cause hypertension.

“lymphoid and mast”

I’m guessing this search came after a bone marrow biopsy with a finding of lymphoid cells and mast cells.  Lymphoid aggregates in the bone marrow can be benign.  If they are mostly T cells, they are more likely to be benign.  If they have central cores of B cells in the aggregates, they are more likely to be malignant.  Any lymphoid aggregate should be evaluated for malignant features, but it is completely possible to have benign lymphoid aggregates in the bone marrow.

Occasionally in people with SM, there are both lymphoid aggregates and mast cell aggregates in the bone marrow.  This seems to occur only in the bone marrow and not in other infiltrated organs.  The first mention of this in literature was in the late 80’s. 

“deactivate mast cells”

I’ll let you know when I figure it out.  Ha!  Maintenance medications, low histamine diet and trigger avoidance to the extent it is possible.  Try not to get stressed.  Yes, really.

“can a person die from mast cell disease”

Yes, but it is not common.  Most people who die from mast cell disease have ASM, MCL or SM-AHNMD when the other blood disorder is a more aggressive cancer.  The vast majority of people with mast cell disease have normal life expectancies. 

“histamine and heparin when scratched”

I assume this is a reference to Darier’s sign.  Darier’s sign is when you see a wheal and flare reaction to lightly touching the skin of a person with mast cell disease.  This is due to the release of histamine by mast cells in the skin.  It is usually associated with urticaria pigmentosa or TMEP, but I have heard a few doctors refer to the reaction in people with systemic disease but no skin involvement as “non-lesional Darier’s.”  I had no skin involvement until recently, and every time I saw my mast cell specialist, he would run his thumb down my back and say “positive Darier’s.”  Darier’s sign is considered to be indicative of mast cell disease. 

 

"can i have a baby mastocytosis"

You can do whatever you want with mastocytosis, but some things are inherently risky.  Fortunately, having a baby doesn't seem to be one of them.  Premeds should be taken at the onset of labor but otherwise many women have found themselves able to have safe pregnancies and deliver healthy babies vaginally or via Caesarian section.  Symptom profiles are super variable during pregnancy when compared to before conception and after delivery.  Some women have complete resolution of their symptoms due to pregnancy.  Some women feel it was the onset of their symptoms.  Like I said, super variable.