Treatment focus' on controlling as many symptoms as possible ;-)
IN Anaphylaxis
First Line
Epinephrine 300mcg ( 0.3mls 1/1000) in auto injection
( EPIPEN / ANAPEN )
Doses in secession dependant on symptoms – 2 are commonly needed
This is the adult dose for individual over 30kg ,
Below this childrens doses given
Chlorphenamine mealate 10mg IV ( intravenously )
Hydrocortisone 200mg IV
Ranitidine 40mg IV
IV fluids ( no plasma expanders )
Nebulaised Salbutamol – 5mg –
can be back to back on neb 3 consider further interventions
Patient advice – if alone
Administer EPIPEN
Call 999
Symptoms –
Having difficulty breathing – can’t speak in whole sentences
Dizzy
Fast heart
Any serious symptom
Every patient is different. You will know if you need an epipen .
Medications work by blocking histamine receptors and stopping mast cells
putting the chemicals into the blood . Receptors are on cells . Histamine attaches
and this causes symptoms . The drugs block these recpetors preventing
histamine causing symptoms.
These are the treatments commonly used ;-)
H1 receptors are in skin , lungs , bladder ( can have mast cells ), mouth , brain
and blood vessels . The drowsy ones are stronger and work better and have the
ability to stabilizing mast cells .
H1 blockade - drowsy making
Benadryl – USA ( not ceterizine )
Hydroxyzine - 25 mg up to 4 times a day ( I take 2 doses )- this has
revolutionized my brain fog and bladder and other spasms ( treatment of choice
for interstitial cystitis )- Known as Atarax
Piriton 4mg up to 6 times a day ( I take 4 doses and keep extra for emergency 8mg ) Generic Chlorpheramine melate . USA - clomtron
Ketiofen 1-2mg 3 times a day - also stabilses mast cells . Available in Canada ,
Italy and UK . Its a safe drug widely used with immense success
Doxepin - stabilizes mast cells and is widely used . Some people don’t tolerate it .
H1 blockade - Non drowsy
Ceterizine hydrochloride - 10 mg once a day ( I take 2 doses )
Fenofexidine 180mg once a day ( common dose 2 times a day some 3 ) I don’t
tolerate this .
H2 blockade - digestive system, Mouth to bottom
Ranitidine 300mg twice a day - drug of choice - Reduces bowel pain on a daily
movement much better , reduced frequency , swelling to pseudo obstruction and
bleeding – from tears from swelling
PPI - proton pump inhibitors
Omeprazole , Lansoprazole and the like to control stomach acid , which is at very
high levels . So these reduce it to normal levels .
Leucotrine receptor agonists ( blocks )
Singulair 10mg once a day ( 2 doses in 24 hours off license this is common)
reduces over all swelling , improves airway symptoms
Parcetamol - sailcilates
1g 4 times a day - reduces effects of prostaglandins
NSAIDS / Aspirin cause mast cell activation so may not be tolerated but can
lead to consistent degranulation aiding symptom control. Often not tolerated.
Opiates. Are not advised, some people tolerate them .If its required, commonly
for bone pain and abdominal swelling pain – consistent dosing with 12 hour
preparations is favored as stable dosing reduces mast cell activation. Oxycodone
is best tolerated.
Cholesterol – fenofibrate 160mg once a day . Mast cell tryptase interferes with
HDL action at a cellular level. Leading to high LDL . Insulin resistance may be
more common in mast cell disease . It has been connected to insulin metabolism
in rats .
Mast cell stabilization drugs –
Stabilizing mast cells reduces the number of mast cells splitting with chemicals
going into the body .
Gastrocrom / Nalcrom - sodium, cromlyn . Is a good drug. It can be expensive
but different preparations are available. Also available is nasal crom, reduced
dose but a large percentage of the oral dose is metabolized on the first pass
through the liver . So nasalcrom in real terms dosing is ok to begin. Available
OTC
Gastrocrom is used for mastocytic entrocollitis . A 6 week course is prescribed .
In mast cell disease / mastocytosis gastrocrom is used as a daily prescription to
stop mast cells splitting in the first place .
Up to 200mg 4 times a day . Of 800 mg taken orally , 57 mg is retained in
circulation to stabilse mast cells .
Stabilizing mast cells reduces mast cell mediators circulating and so
complications such as osteoporosis
Stress / Anxiety
For stress and /or anxiety from brain fog , prostoglandins , serotonin and daily life
Benzodiazepines are used . Diazepam ( valium ) Temazepam , Clonazepam are
all examples of this drug class. They should be used with caution as they are
addictive . But in patients who are experiencing anxiety in a serious way they
have therapeutic benefit .
Depression
Patients with mast cell disease are at risk of clinical depression. Due to the
change in the patients life . Depression is caused by multiple factors. It should
not be assumed that a patient will be depressed. Histamine acts on the brain
causing anxious behavior . H1 blockers can improve this . Prostaglandins in high
levels over time can cause low mood . Measures used to help this include regular
Paracetamol , as this reduces prostaglandin production . ~caution~ in patients
who have not tolerated these drugs in the past .
If antidepressant medication is required . Tricyclics should be avoided as they
interact with epipens causing arrhythmias ( irregular heart beats ). In mast cell
disease some patients do not tolerate serotonin being altered . In many it is high .
In which case use of SSRI’s can increase symptoms . It being low does not
naturally include a clinical depression . So in this matter MOAI’s are preferred .
patches are best tolerated, but if the glue is a trigger then oral medicating is
suitable .
It should be noted that patients will be experiencing a grief process on diagnosis.
In which they will be experiencing many emotions .
Osteoporosis
Osteoprosis is loss of calcium from the bones according to set measurements
High heparin from mast cells is a key cause in osteoporosis in young patients . A
baseline bone density scan should be undertaken at presentation.
Vitamin d should be supplemented as it is poorly absorbed in the gut in
Mastocytosis .
In clinical osteoporosis or osteopenia . A vitamin d and calcium combination is
advised . In advanced osteoporosis , Biphoshonates can be used , but patients
report increased bone pain and increased bleeding so may not be tolerated .
Stabilizing mast cells to reduce total heparin circulation will reduce the possibility
of osteoporosis
When the list of meds is exhausted . Immunomodulation can be used , these
include methotrexate , cellcept , interferon , Gleevac .
Steriods were used routinely and do work , but have serious long term
implications . So are favored for emergency only .