Wednesday, August 20, 2014

"Magic Masto Lotion" Recipes

“Magic Masto Lotion”

The name of this lotion does not do it justice — it’s better than magic. And if you're looking for information on “Magic Masto Lotion” (MML), you've come to the right place!

Nancy Gould, of New Zealand, developed a recipe for a lotion to apply to the skin which helps the “itchies” go away. Nancy is a nurse, originally from the US, who has systemic mastocytosis. She was hoping to find a way to increase the level of cromolyn in her system by applying it to the skin, and made this happy discovery instead. Thank you, Nancy, for sharing!

The Original Recipe for “Magic Masto Lotion”

1 tsp glycerin
¹⁄4 cup of Vanicream¹ (ordered from the pharmacist, approximately US$15 for a 1-lb. tub)
5 ampules (tubes) of Gastrocrom or 500 mg of cromolyn powder²

¹ Vanicream can be ordered from the pharmacist. It costs about $15 for a 1-lb. tub. Other thick lotions may also work. Suggestions people have shared for other lotions are:

Trader Joe’s has a moisturizing cream that is thick and does not have mineral oil or lanolin, runs approximately $3.99
Desert Essence daily essential face moisturizer is a face cream that might also be a good option due to the thickness. It has no colors or synthetic perfumes, detergents, animal fat, animal ingredients or testing, runs approximately $4.
Aqueous Cream can also be gotten from your pharmacist. There is a thick base that is used for mixing preparations of water-based or water soluble medicines. It is definitely no-frills, with no perfume or color. If you mix it with Gastrocrom, it should turn into a somewhat manageable lotion.

² Gastrocrom (Cromolyn Sodium) is a prescription that will have to be written by your doctor. It comes 96 plastic vials, or ampules, to a box. If your pharmacist can get the powdered form of cromolyn (Nalcrom), it makes a more manageable cream.

If you cannot get Gastrocrom, here are two recipes for making MML lotion — one using NasalCrom, which is available in the United States over the counter (OTC), and another using Nalcrom, a powdered form of Gastrocrom that is available outside the US:

Recipe for “Magic Masto Lotion” using OTC NasalCrom®

NasalCrom® is an over-the-counter nasal spray that contains cromolyn.

2 tsp glycerin
1/2 cup of Vanicream or other thick lotion
1 whole bottle (0.44 fl. oz. size – 13 ml.) of NasalCrom®

Recipe for “MML” using Nalcrom powdered cromolyn

Nalcrom powdered cromolyn is available by prescription.

1 ½ ounces (just less than ¼ cup) Vanicream, or other mild skin cream
1 teaspoon Glycerin
Five 100mg capsules of Nalcrom
Mix, allow to sit 20 minutes, and mix again.

For More Information Please Visit MastoKids MML

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.