Wednesday, August 20, 2014

Common Mast Cell Degranulation Triggers


While there is a large variability factor concerning things that cause the mast cells to degranulate in patients with pediatric mastocytosis, many triggers have been found to have a commonality among the general mastocytosis community and as such, it is wise to avoid these factors or to carefully monitor a child when such triggers cannot be avoided.
  • Factors with a High Probability of Mast Cell Degranulation
Factors in this group should be avoided. If medications are deemed to be essential, medical supervision must be provided and emergency interventions immediately available.
  • Anything the individual has previously reacted to
  • Venom, coming from snakes and insects such as bees, wasps, and fire ants
  • Biologic compounds released by intestinal worms, jellyfish (on contact), ingested crayfish, and lobster
  • Dextran: used in some IV solutions and eye drops
  • Compound 48/80---not commonly used, but with a high degranulation ability, all patients should stay aware
  • Iodine-containing radiographic dyes
  • Non-steroidal anti-inflammatory drugs (NSAIDs), including aspirin and ibuprofen
  • Scopolamine: used in pre-operative procedures and in some eye drops
  • Papaverine: found in some heart medications
  • Dipyridamole: used as an anticoagulant
  • Thiamine (vitamin B6):  found in vitamin supplements
  • Trimethaphan: anticoagulant used in surgical procedures
  • Narcotics: codeine, morphine, meperidine (pethidine, Demerol), and all derivatives
  • Neuromuscular blocking agents:
       D-tubocurarine
       decamethonium
       gallamine
       metocurine
       pancuronium.
  • Sympathomimetics: isoproterenol, amphetamine, ephedrine, phenylephrine
II.  Factors with a Common Probability of Mast Cell Degranulation
Factors in this group should be approached carefully, under close supervision by a medically trained person, parent or caregiver who is prepared to administer emergency treatment if necessary. Reactions to these triggers may vary in degree of severity, so caution and supervision are consistently required.
  • Extreme temperatures, heat or cold
  • Sudden changes in temperature, such as entering a hot car, jumping into cold water, etc.
  • Exercise and exertion
  • Friction
  • Alcohol: includes alcohol taken internally through food and medication and applied topically, such as hand sanitizer or wipe cleaners
  • Polymyxin B: such as is found in many antibiotic ointments and some vaccines
  • Dextromethorphan: found in cough suppressant medications
  • Amphotericin B: commonly found in antifungal treatments
  • Quinine: found in certain medications and in some tonic waters
  • Local anesthetics: including lidocaine, tetracaine, procaine, methylparaben preservative
III. Factors with a Moderate Potential for Mast Cell Degranulation
Factors in this group should be approached carefully, using only a small amount at first, administered by a parent or caregiver who is prepared to administer treatment if needed.
  • Overly warm bath water
  • Hot foods
  • Spicy foods
  • Preservatives and additives such as alcohols, MSG, sodium benzoate, and artificial colors.
IV. Factors that May Increase Mast Cell Activity
  • Emotional stress and anxiety
  • Sleep deprivation
  • Pain
  • Some bacterial and viral infections, including upper respiratory and urinary tract infections, bronchitis, pneumonia, and others
  • Vaccinations-*Please note that Mastokids does not discourage or dissuade against routine and mandated vaccinations for children with mastocytosis. Mastokids suggests discussing with your health care provider the usefulness of antihistamine treatment before and/or after vaccinations are given.
For More Information Please Visit MastoKids/Degranular

References:
Castells, M., Metcalfe, D., & Escribano, L. (2011). Diagnosis and Treatment of                             Cutaneous Mastocytosis in Children. American Journal of Clinical Dermatology 12 (4), 259-270.
Greenblatt, M.K., & Chen, L. (1990). Urticaria Pigmentosa: An Anesthetic Challenge.      J Clin Anesth 2, 108-115.
Hannaford, R. & Rogers, M. (2001). Presentation of Cutaneous Mastocytosis in 173 Children.Australasian Journal of Dermatology 42, 15-21.
Longley, J., Duffy, T.P., & Kohn, S. ( 1995) The mast cell and mast cell disease. Journal of the American Academy of Dermatology 32 (4), 545-561.
Marone, G., Spadar, G., Granata, F., & Triggiani, M. (2001) Treatment of mastocytosis: pharmacologic basis and current concepts. Leukemia Research 25, 583-594.

Emergency Medical Information


Because Pediatric Mastocytosis is a very rare disease, caregivers and patients have found it useful to carry medical information relating to their condition on their person at all times. Many parents of children with mastocytosis have opted to order Medic Alert bracelets for our children. Others have put together kits that they keep on them at all times when traveling.
Below you will find a list of helpful items to carry with you when you are traveling, or for everyday use, as well as information that many of us have included on our children’s MedicAlert bracelets.
 Travel Bags  
  • Any medication your child is currently taking.
  • A dosage chart for your child’s medications.
  • A list of contraindicated medications and activities for your child.
  • Your child’s MedicAlert card, if he or she has one.
  • Names and phone numbers of emergency contact people authorized to make medical decisions on behalf of your child.
  • Names and phone numbers of your child’s health care provider and any specialists he/she may see for mastocytosis.
  • Photocopy of both sides of your child’s medical insurance card.
  • A copy of the anesthesia protocol for mastocytosis patients.
  • A copy of Pediatric Mastocytosis article(s).
  • An Epi-Pen Jr., if one has been prescribed for your child.
  • Age, height, weight and blood type of your child.  

MedicAlert Bracelet Information
  • Diagnosis: Mastocytosis.
  • Subject to Anaphylaxis.
  • Treat with EpiPen.
  • Contraindicated medications.
  • Parent contact information.
  • Pediatrician contact information, as well as any specialists your child sees for mastocytosis.
  • Names and phone numbers for you and anyone else responsible for making healthcare decisions for your child.
  • All medical insurance information.
  • Cell phone numbers.
  • Any medication your child is currently taking.
If you live in the United States, visit MedicAlert online to find out about their products and services.  
If you live in Europe, here is a link for MedicAlert which provides medical bracelet services in the UK and Europe. 

For more information please visit MastoKids/ Emergency Medical Binder

Getting Ready For School

At TX Children's Hospital going to see her allergist for her mastocytosis check up and updated school forms.  

Wednesday, August 6, 2014

Mastocytosis Awareness




Our youngest daughter was born with this rare orphan disease.  Please help spread awareness of mastocytosis and mast cell diseases!  In Hope Of A Cure!

Monday, August 4, 2014

EPI PEN INFORMATION


EPI PEN

Life happens. Be prepared.


Anaphylaxis is unpredictable. When it comes to potentially life-threatening allergic reactions (anaphylaxis), you need to have a plan. Your plan should include avoiding known allergens, recognizing the signs and symptoms of anaphylaxis, having access to two epinephrine auto-injectors at all times and seeking immediate emergency medical care should anaphylaxis occur.

EPI PEN $0 Copay offer!!!

Candice Mastocytosis Triggers and Reaction Information Sheet

Known Allergens and Triggers

NSAIDS (such as Advil, Aleve, etc)
Sulfa Drugs
Mosquito Bites
Dramatic changes in temperature- from hot to cold or cold to hot
Any Amount of Humidity
Emotional Changes
Acidic Foods and Drinks (lemons, limes, pineapple, tomatoes, and their juices, etc)
High Histamine Foods and Drinks (avocados, brussels sprouts, cabbage, etc)

Reactions

Candice can usually feel when a reaction is about to happen and will inform someone that she doesn't feel well and that it is starting.  She will say she is having a masto reaction.  The sooner she gets the proper medications to stop the reaction the sooner she will feel better and will increase the possibility that she will not progress to the serious category of anaphylaxis.  In the event she complains of headache and/or bone pain please give Acetaminophen.

Mild Reaction- Minor skin changes such as her "spots" on her skin will appear to look like a mosquito bite.  She may become irritable and mildly feverish and flushed.  Her eyes and nose may become reddish to purple and swell (looks as though she is tired or was recently crying).
-if this happens please give Cetirizine (Zyrtec) and Ranitidine (Zantac) as well as use her cooling towel and any other cooling products that are available (cool mist fan, cool vest, cold water) to attempt to stop further progression of more severe symptoms and monitor for progression of more severe symptoms

Moderate Reaction- She may have some of the above plus...major skin changes such as the "spots" on her skin will raise and fill with fluid, be painful, itchy, etc.  She could have flushing, this is where she turns red and it feels hot to the touch (like a fever).  She may become lethargic, fatigue, not interested in doing things she normally would enjoy doing.  She may have a headache.  She may have bone pain (especially leg/feet).  A temper tantrum that happens in a matter of seconds with no apparent reason could occur.  She may have diarrhea or complain of GI (tummy issues), bladder control issues,  or even begin vomiting.
-if any of the above happen she must be given her emergency mediation Benadryl to attempt to stop the progression of more severe symptoms

Severe Reaction- Anaphylaxis may occur.  If this occurs immediately give EPI PEN and call 911 to transport to the nearest facility.  Then call parents.


Mastocytosis Flare Pictures

 Knuckles turning red (flushing, burning, painful)

 Facial flushing

 Beginning of a flare up with facial flushing, overall feeling unwell, lethargic

 Facial flushing

 Hand flushing

Candice mastocytoma flare on wrist.  Mastocytosis flare eyes, looks as though tired or has recently cried.

Mastocytosis "spot" flare on thigh

Mastocytosis "spot" flare on back of leg

Mastocytosis "spots" flare on back of leg

Mastocytosis "spot" flare and flushing on back of thigh

"spots" on back of leg

Mastocytoma "spot" flare on ear

Flushing face

Start of flushing face

Flare of "spot" on leg

Flare of "spot" on leg

Flare of "spot" on leg

Tummy (GI) issues from flare up and flushing face and left arm

Flaring "spot" on top of leg and hive 

Mastocytosis flare up reaction "spot" on back of leg starting to fill with fluid

Mastocytoma flare on face

Mastocytoma flare on wrist and face

"spots" flare on back of leg

"spot" flare on thigh

Mastocytma flare on wrist and face

Mastocytoma flare on toe