Disease Awareness #6 - Mast Cell Activation Disorder

Tonight when I was in the Today Show's website, I came across a story that warmed my heart.  A girl, name Kaelyn Krawczyk, (Nickname is KK), she has Mast Cell Activation Disorder and had surgery.  I have never heard of Mast Cell Activation Disorder before seeing it on the Today's Show website.  I wanted to know more about it.  According to the article, Mast Cell Activation Disorder is a rare condition that causes reactions when KK gets too hot, too cold, or gets stressed.  KK's biggest trigger is fatigue.  KK's service dog, JJ, was trained to tell when KK will have a reaction.  JJ was in the OR with KK.  The first ever dog allowed in the OR with a patient.

To see the video from the Today Show, go HERE.

So off, I went searching to find out what is Mast Cell Activation Disorder.  I found:

Mastocytosis is a rare disorder characterized by abnormal accumulations of mast cells in skin, bone marrow, and internal organs such as the liver, spleen and lymph nodes. In the disorders of mast cell activation, mast cells are easily triggered to release their mediators, resulting in many of the same symptoms as systemic mastocytosis.

{Information from HERE}

Mastocytosis Society is a place where parents, patients, caregivers, and physicians can go to get more information on Mast Cell Activation Syndrome.  Mastocytosis Society does research, educate, and are advocates for this disease.  On their website, they said this:

"Mast cell activation syndrome (MCAS), where even the normal number of mast cells are too easily activated by a trigger to release their contents, called mediators. These mediators can cause a variety of unpredictable symptoms in both children and adults, including skin rashes, flushing, abdominal pain, bloating, nausea, vomiting, headache, bone pain and skeletal lesions, and anaphylaxis. Triggers can be heat, cold, stress (physical or emotional), perfumes or odors, medications, insect stings, and foods. These symptoms are treated with medications including antihistamines, mast cell stabilizers, and leukotriene inhibitors, while anaphylaxis is a medical emergency requiring epinephrine. Mastocytosis can affect skin and internal organs such as the bone marrow, gastrointestinal tract, liver, and spleen. Most patients with mastocytosis have cutaneous (skin) or indolent (benign) systemic forms, but aggressive disease can occur, which may require chemotherapy. 

A diagnosis of mastocytosis is confirmed by a bone marrow or skin biopsy. MCAS patients do not fulfill all criteria for mastocytosis but exhibit symptoms, may or may not have increased measurable mast cell mediators (commonly tryptase, histamine or its metabolites) during or shortly after an attack and do respond to the same medications that patients with mastocytosis do."

Common Triggers that cause degranulation 

include: 

 insect stings including mosquito, 

wasp, hornet and bee

 pain medications such as nonsteroidal anti-inflammatory drugs 

(NSAIDs) and narcotics 

(codeine, morphine)

 extreme temperatures, both hot 

and cold

 food and drink that are high in 

histamines or are known to trigger 

histamine release (i.e. shellfish, 

alcohol, fermented foods)

 exercise

 friction, pressure or vibration on 

the skin

 strong scents including perfumes 

and chemicals

 emotional and physical stress

{Information from HERE}

Another place for parents to go for help is a site called Mastokids.

Thanks for reading,

Melissa