Hi Judy ,

I am glad you are in touch with nancy At  10 days these will be seen as baseline . Tryptase only lasts in the system for up to 6 hours in its measurable form . So at 10 days these results are too be expected .

What it does do , is make full mastocytosis less likely as due to the number of mast cells , tryptase is in general terms , high all the time .

This needs confirming with in incident measurements . Will it rise ? No way of guessing .

I shock and am very ill in this and symptomatic day to day . My tryptase is 3 at rest and so far , 3 in a reaction .

So ;- full mastocytosis is unlikely for me , as it is for jarrod

BUT :-
Mast cell activation syndrome / disorder ( mcas / mcad ) and monocolnal mast  activation ( mmas ) are possible or an idiopathic condition . Idiopathic anaphylaxis or similar .Mine , is idiopathic angiodeama ( don't know why but your swelling ). I am in the process of educating a new medical team on my reactions .

For Jarrod , he has high histamine . So he is nearly fitting the criteria for mast cell activation syndrome , which was suggested by Dr castells recently ( dec 10 ) .

This is the criteria :-

Proposed criteria for the diagnosis of MCAS*


1. Episodic symptoms consistent with mast cell mediator release affecting >2 organ systems evidenced as follows:
a. Skin: urticaria, angioedema, flushing
b. Gastrointestinal: nausea, vomiting, diarrhea, abdominal cramping
c. Cardiovascular: hypotensive syncope or near syncope, tachycardia
d. Respiratory: wheezing
e. Naso-ocular: conjunctival injection, pruritus, nasal stuffiness

2. A decrease in the frequency or severity or resolution of symptoms with antimediator therapy: H1- and H2-histamine receptor inverse agonists,
antileukotriene medications (cysteinyl leukotriene receptor blockers or 5-lipoxygenase inhibitor), or mast cell stabilizers (cromolyn sodium)

3. Evidence of an increase in a validated urinary or serum marker of mast cell activation: documentation of an increase of the marker to greater than the
patient’s baseline value during a symptomatic period on >2 occasions or, if baseline tryptase levels are persistently >15 ng, documentation of an increase of
the tryptase level above baseline value on 1 occasion. Total serum tryptase level is recommended as the marker of choice; less specific (also from basophils)
are 24-hour urine histamine metabolites or PGD2 or its metabolite 11-b-prostaglandin F2.

4. Rule out primary and secondary causes of mast cell activation and well-defined clinical idiopathic entities in Table I.
PGD2, Prostaglandin D2.

I know it is a bit medical worded .

!1) But Jarrod has multiple symptoms in different areas .

2 ) Meds wise , I do feel antihistamines at a good level are worth trying again as a improvemrnt is a criteria .

3 ) He has high urine histamine
he needs he tyrptase in a reaction

4 ) all the other things we mentioned need excluding .


For a while I felt like I wasn't a proper masto patient if I didn't have mastocytosis . That is not the case at all .

I remain concerned about the beta blocker .

Many of us have been told its all in our heads , or have had barely veiled accusations of Munchhausens . This is where doctors go when they don't know what is wrong . With Jarrods positive histamine level , I feel this is what has happened with you . I eventually asked for a psych exam , because I was so fed up of not being listened to . So , I am officially sane not many ppl can say that

I know how frustrating this path is . I am not finished on it myself .

Tryptase over 1 is abnormal , but not abnormal enough for mastocytosis .

With the set of symptoms Jarrod has , the tryptase becomes relevant .
You weren't looking in the wrong place with mastocytosis . It is just Jarrod is looking like he has a very closely related condition . You must have done loads of research .

Anaphylaxis and reactions in mcad , mmas and IA , can present differently and have a different path to typical allergy anaphylaxis . This is termed atypical . This confuses non specialist doctors further

many many hugs and much love

Josie

Hi Judy,

I absolutely understand being emotionless is not normal

Regards the treatments . Say this is mcad . having Jarrod on a good mix of antihistamines at the right level will aid diagnosis

I have had to move on specialist wise and it is hard , almost like you are reliving it all . But it has payed dividends for me .

The dermo / masto / immunology team believed me . Really believed me . They said they may not find the cause , BUT they would treat me and they ahve been good to their word .

They have now passed me to a local immunologist who I am collating all my information for . They will see my reactions . We can go from there . Anaphylaxis can present in many ways . Jarrod may not ahve anapylaxis but many symptoms , just not to the life threatening level . Doctors don't like diagnosing anaphylaxis .

The bucket theory fits with any skin or systemic mast cell disease . As you keep putting in the histamine . You can also aggravate it with stress, which in me , leads to some more serious symptoms . It takes major emotional upset to shock me .  

Has he a asthma doctor ?? as a clear picture on his chest will help . Beta blockers are avoided in asthma

A detailed diary of everything jarrod eats / washes it / has contact with and all his symptoms will help reveal patterns .

As even with a diagnosis jarrod will have to watch his triggers and how he reacts , so he can identify when he is being triggered and medicate / remove it . I went to my GP's this morning . They had sprayed an air freshener . i flushed and went thirsty +++++++++++. I went outside , had chlorpheramine , a drink of water and settled . If i had stayed there I would have been far worse .

My background antihistamines protect me so much . I have only got to a right level for me in the last 2 months .

Dizziness , is a fave thing for doctors not to investigate in young people . Did jarrod have any tests for causes of dizziness  ??

I know teary days sweets , just let it run . many many hugs

Jose

Hi Ruth,

I'm sure Josie's asleep right now since she's several hours ahead of us.  I'll try to answer a few of your questions.

How do we know whent he medas are adequate, when there are no breakout reactions going on.  I've been very impressed as to how much better I feel and how much more STABLE I am with my increased meds.  Once Singulair was added to my medications things improved GREATLY!!

As to the doses, Ruth, you need a doctor's help with this especially since your son is still a child.  The pediatric doses and forms are different than for us adults and although you, yourself can fiddle with this, it's best to have a doctor doing this for you - working with someone like Dr. Escribano or Dr. Castells.  The children's bodys are different from ours and their needs are different and since each of us are working with different things here, it's hard for you to go by our situations.  For example, Josie is working with angioedema.  Her angioedema may be mast cell related, but until her doctors can pin this down, she's got a different situation from the rest of us.  You see, because Josie's situation is a bit different, she doesn't respond to the very same medications that an SM patient would.  

You see, Ruth, this is part of what is so challenging for all of us, for our doctors must personally tailor our medications to meet our body's particular needs.   Yet, as though that isn't a big enough challenge, you've also got differences between us in that now that the WHO consensus has recognized the diagnosis of MCAD, it opens up the consideration as to other forms of mast cell related disorders.  From what Josie knows as to her investigation, she's so far restricted to being an angioedema patient.  Angioedema has a few different mechanisms behind it, one being autoimmune, another being hereditary.  Well, her angioedema may be mast cell related, but she's not a Systemic mastocytosis patient.  This is another form of mast cell disorder.  

It's rather confusing, Ruth and so let me see if I can set you straight - if we put all of the mast cell disorders on a line, you will put on your far left hand side Systemic mastocytosis.  This is a MC proliferation disorder.  There are too many mast cells.  It's a neoplasm and it invades tissues.  The next on the list sits right smack in the middle - MMAS or Monoclonal MCAD.  This is a mixture of the neoplasm with possibly the non-clonal form.  It is recently discovered and it produces very severe reactions in the patient, but it is not as damaging as the SM form is.  Then to the right of the spectrum you have MCAD and within this group you have the autoimmune form but you also have the non-clonal form of the disease.  The non-clonal form means that although there is MC activation seen, it appears that there is a dysfunction of the mast cell but that it is not a neoplasm and that it is not invading tissues.  Within this form you will find the form of angioedema that Josie seems to have.   This is why Josie's medications are different than for some of us as well as the behavior of her disease - it attacks her body differently.  She gets the classic angioedema where the lower portion of her body has extreme swelling and she has all kinds of reactions but she also has the anaphylaxis that masto patients have.  Josie's case is the missing link between the true angioedema patient and masto.  You see, Angioedema, the classic angioedema is NOT a mast cell disorder.  it doesn't involve the mast cell.  It has other mechanisms behind it.  But a case like Josies has a foot in both areas, masto and angioedema and this is why the same kinds of meds she needs and doses may not work with her like it would work with an SM patient.  

Here, let's take my case.  I'm suspected of the MMAS form of masto.  Well, I'm also autoimmune!  I don't have a great deal of issues with foods or with chemicals and the such, yet, that's most of the time - I will have it some of the time.  But my problems are major cardiovascular reactions and I do a lot of fainting.  My doctors have to tailor my medications to help deal with my particular problems and doses accordingly.  

If you take Heather, she's purely autoimmune and totally non-clonal but she's not got the angioedema that Josie has.  So her medications are different from Josies but also from my own.  And if you speak with Joan, she'll pipe in with other medications since she's an SM patient.  

So, Ruth, in talking about your son's medications, and your feeling uneasy about upping those doses, you should be.  Antihistamines can be cardio-toxic if they are in the wrong doses and mixtures and your doctors really have to have an understanding of how to adjust these meds properly.  

Now, as to forgetting his meds, Ruth, if you had this disease, you would never forget to take your meds for if you could feel how miserable you felt without them, you'd know how vital it is to take them on time every day.  If I pass a coupld of hours I begin to feel this gnawing in the pit of my stomach and the malaise rises up and I begin to feel so awful that it's not funny!!   I was in the middle of the pharmacy stocking up on my meds and I got to the point that I couldn't think straight!!  it was AWFUL!  I knew I needed my extra meds just to get myself out of the pharmacy!

Zantac and Zyrtec are non-sedating.  Yet, insomnia is a recognized problem for some masto patients, Ruth.  If he is sleeping more heavily but also more soundly then perhaps he's finally having a normal night of sleep and yet it seems strange to you because of how badly he has slept all his life.  

As to the exhaustion and your description of how he is when he's off the meds and on bad foods, that is very normal!  He's REACTING and it's very, very taxing for his body.  It takes about 3 full days for the mast cells to replenish their stores once spent and all of that takes a great deal of energy.  Then you add on to it the normal growing that his body is demanding of him and you have a truly exhausted child!!

Ruth, make sure that Cameron does not skip his meds!  He needs them badly and you've got to make sure he doesn't miss a single dose!

As to his high IgE, has he been properly tested for worms??  Joan can give you some excellent information for a type of worm which will skyrocket the IgE levels.  

As to the beta blockers, they won't elevate the histamines high enough to matter, Ruth.  This is proven MC activation and this is masto!!   So, don't worry about the bleta blockers but he really should not be on them since they can interfere with the way epinephrine will work in a crisis.  My cardiologist wanted to put me on it too, but Dr. Castells intervened and said NO WAY!  

Dizziness is easily dismissed because 1 - it's not a serious symptom,  2 - it's caused by so very many minor problems that it's not seen as anything important.  You can get dizzy from dehydration!!  Granted, dehydration can be life threatening, but doctors won't put it into the same category as a tumor or something and so unless you've got something that seems really "serious" behind that dizziness, they expect you to "live with it"!  Sad, I know, but I think that it's more of a defense for their not knowing what to do to improve it.  

AS to those foolish doctors and their comments, Ruth and Judy, THEY ARE FOOLS!!  They are merely trying to turn the focus off of their own inability to recognize and deal with these kids symptoms and illness!!   I recommend that you try turning the tables on them and ask they why they can't seem to recognize and treat the symptoms?!!   It's becoming all too easy these days put the blame on the patient or on the mother when in reality the doctor is totally CLUELESS and yet won't admit this openly!!  I have learned to trust those doctors who open up the game with me and ADMIT that they don't know what they are dealing with!!  These doctors win my trust right away!!  They won't put me into danger knowing that they don't know what they are working with!!  They go cautiously!!

I hope I've helped a bit.  I know that Josie will pop back in here and address your questions too!!

Don't worry ladies, keep pressing for answers with doctors.  I'm certain you will find them!!

Hugs!

Lisa

Hi everyone,
                  Thanks everyone for your support, and definately very sorry about yesterday. Having a much better day today.Having you all there for support yestday was good too.

Ruth his sleeping has been an issue since he was a baby, just thought that was him, now its all making sense. I read that bit about 2am being the peak of histamine. Thats making sense. I would say he probably gets his hours but at the wrong time. He much prefers to go to bed at about 11pm. Thats alright if he is sick or school hols, but on school nights its hard to get him to bed earlier. He has an earlier bed time at 9.30 on school nights, which sometimes works and sometimes
he cant sleep. When he is sick and really bad, scared etc he can sleep with me and Ive always said he gets better nights sleep in the later half of the morning. It, after 2am but I just dont know what time. Keep more notice on that one. The first half he is just so restless and moaning and groaning ,waking up all the time.

His IgE  was first detected in feb 09, after I asked  for it and it was the first test that ever showed anything.That was the highest  1854 and the lowest has been 922. Yes mothers instinct has played a part here,
and always has been behind pushing and getting answers. I must say with all their negativity  you  start to doubt yourself though. I have episodes before we got  one positive test that everyone thought I was mad, that I was thinking maybe I am, but then I thought of everything that has happened in the past and thought NO theres always been evidence all along.

Now I must clarify some things girls, all those sympoms I have mentioned dont happen every single attack. The main ones are: pallor,malaise, fatigue (cant sleep)
Nose issues, stomach problems and headaches are frequent though.
Skin lesions like those ulcer, burn like things are  a recent addition.
We would like to get a biopsy done if it happens in the right place. He has had one very nasty one around one eye (like that youtube vid)
two on his tummy button, and one on the top of his ear(cartilage)
The giddiness has happened about 2 or 3 times. The asthma is not really an issue since he had been hospitalized when he was nearly 3, that was particullly  bad. We have noticed that before he got that damn awfull dry cough, he would get a bit weasy. He gets weasy the last 2 or 3 episodes of so called anaphylactic looking thing. He has had that anayphlaxis (whatever it is) about 5 or 6 times. We have noticed that cordial can set that off sometimes.

We really do feel that food and medicines are not really the problem here . His main triggers are wind, pollen , grasses, trees, and some chemicals like , his brother came home from school with headlice so I treated the whole family and that night he reacted. It had eucalyptus in it, its not the first time hes reacted to that. Imagine living here in Australia and being allergic to that.He couldnt seem to tolerate the menthol type cough lollies for his throat either. It seemed to help with the nose stuffiness, but he would prefer to put up with that than take those. Cordial is a problem with the anaphlaxis. Night time seems to be the time he would come down with a 10 day stint. The 3 days to recover makes sense too. On the 8th day you can see a slow pick up in energy, bit more brighter, and more color in his cheeks. By the 10th day he would be back at school not 100 percent but able to handle school. Thats when those petechulal bruising appeared on his upper arms. That hasnt happened for a while now. They seemed to signal the end of an attack. By the way do you know how they said he got those bruises, he sucks them dont you know! (courtesy of the shrinks)

Ruth, I hope your son is OK, Jarrod is well at the moment except the daily symptoms enjoying the school hols here at the moment.

Yes I know you really are still worried about the beta blockers, but everyone has seen an improvement, and he has been at school more often.Still does get sick but less often. Yes i think he was undermedicated in the past for antihistamines etc. Talk more about that next time.

Thanks for replying ladies.
Happy Easter
sorry for such a long post
Judy

Hi Ruth ,

I agree with some of what Lisa has said and disagree in others.

I do agree conditions are on a line . But we can't say where I fall on it . I have not had a 24 hour histamine collection or prostogalndins for the reasons you have worked out . Also my current medical team have not witnessed my shock .

My tryptase remains 3 . My CRP has been high from my first week of illness .

My angiodeama is not confined to my lower body . I have swelling all over inc my face , mouth and throat ( epi pen level ) . I also have a large number of other symptoms .
diarrhoea
vomiting ,
bowel spasms
flushing ++++
pallor
blueness on exertion
pain - abdominal , back and other places - chest wall , bone pain
shakiness
spams of any muscle group all over my body
headaches from mild to migraine
extra bleeding = bowel and vaginal , gums
dry eyes
mouth ulcers
daily wheeziness increases to full throat close in shock
Rashes - little red dots and freckles which urticate in a reaction
night sweats
pule 90 at rest , high in a reaction
high BP in a reaction , lowers quickly

There is more , but thats most of it.

I have found that getting onto 3 antihistamines at maximum does has helped me significntly .

Although my meds read different , that is because I am in the UK . I am on typical mcad meds currently .

Sleeping wise , I am much more settled on this dose of medications . I was waking hourly , especially when I had corn flour in my meds as sulphites give me drenching sweats . Hydroxizine is the one that has made the biggest difference to me .

I have fatigue every day , but far worse after a reaction . I call it the ton of bricks landing . I have a lul , where i don't feel as bad as I should , then it hits .  Any change in meds or any accidental food reactions will have this happen .

I agree with lisa that you need to work alongside your doctors with dosing . But I do think getting a stable level will reduce the ups and downs . I have taken a while to get used to my medication load . I know I need it , in no doubt at all , but sometimes I just want to be normal . I know this is me . And I don't miss any meds . Its just there Maybe cam feels this ?

Any insinuation direct or otherwise that the problem is yours is very hard to deal with . We have all been there . You are a good mum and you are fighting for your sons health for good reason . You are not paranoid or making it worse .
I ahve found family , freinds , medical staff go through stages of accepting I have an illness.
This is :-

It can't be she's young / the same age as me

shes depressed

shes in denial

shes obsessed

finally - yes , its a fact , yes she is coping .

This seems universal . You know cam and you can see where he has problems . You are a good mum . I did , carry on as normal , now I do what I need to to give me my time My illness is here I found before I wanted to talk but I was met with platitudes and this stopped me . So i felt very alone . Once others understood , I have been able to talk and things run so much better as we can all say what is really on our minds . My family didn't want to upset me by asking , but it wasn't them . It was my illness .

Dizziness is a difficult symptom . It can be nothing , but can be something . I know my dizziness . This sounds mad , But I do . I know when its daily stuff against a syncope / near syncope - where I go vvvv pale and have to lie down . This happens even sitting up.
I am very sure cam knows his as well .

With reaching a good level of background meds , my daily dizziness is much controlled .

Dizziness from a trigger comes at rest .

My take on this is , with triggers I have degrees of symptoms . Dizziness comes from reduced circulating volume , once ear problems have been excluded . In my daily life I have many things which can reduce my volume . minor reactions , heat triggering mast cells , any other trigger , diabetes . Removing myself from the trigger ( heat , perfumes etc ) and drinking plenty and medicating is my stock plan .

Occasionally I will have a shock where I haven't been able to act quickly enough . But my epi pen works for that .

Caution should always be taken with children . BUT . I think they still need medicating for stability . This just needs more frequent review as needs change with age , size and reactivity

Histamine and compliment make blood vessels pours , then leak protien and fluid , this leads to the reduction in volume . When you are having regular events , minor or major - this loss will need compensating for . We are lucky that this makes us thirsty , so if your thirsty , drink We are also prone to losing fluid through prostoglandins making our kidneys filter out more and sweating . High pulse adds to this .

Shock thirst , is like your mouth goes bone dry instantly .

I am likley heading into hospital , my cholesterol is off the scale . So If I go quiet , thats why ;-(

many hugs
Josie

OK a few points here.

First, having a Tryptase level drawn when having a reaction, it more precise than mentioned. You wait one hour and have it drawn BEFORE the third hour after the reaction (thats when the tryptase levels goes down fast) Any longer than that, just consider it a baseline tryptase.

Next. that petechei that shows at the end of a reaction comes from a heparin "dump" into the bloodstream by mast cells. That is the "other" job of mast cells, they are in control of the bodys natural heparin. I bleed under the skin on my arms after reacting, its very frequent and thats one of the points meant when you see that Masto often has blood dyscrasias (problems) associated with it. I was just in the hospital and they wanted to give  me a blood thinner to prevent blood clots in my legs--that is the only med I refused to take and that is why. I explained to them that my blood can go from too thick to too thin quickly when I am reacting, so your son has a concrete reason for that happening.

Lisa, that is THE BEST explanation of the different types of masto I have ever read! You taught me also, how to tell the difference. Nicely done.

hugs to all
Ramona

It is really helpful isn't it, hearing others' experiences, so much that you just can't get from a doctor.  

Cameron has had some severe bruising in the past, now I can see the heparin connection.  The deramatolgist was horrified at the sight of the bruise left when one of her colleagues removed the dressing over his patch test - bruising from pulling ofF adhesive bandage?!  The latest unusual thing on Cameron's skin was a small spot that appeared on his arm, unlike anything he has had before. It was in the crease of his elbow, right about where he had blood taken about a week before, and looked like blood beneath the surface, but not a blood blister. It was about 1cm diameter, quite round, and very dark red. It disappeared after a few days.  Unfortunatley any time we try to photograph things like this, or his face during a reaction,  they never seem to look as obvious as in person.

I know how you feel about wanting tests, and hoping for an opportunity to get some answers,  I have taken him a few times now when things have flared wanting tests, but my doctor has thrown her hands in the air and said she doesn't know what to test, and she won't just take a stab in the dark. So frustrating, but that was before I had found this site and had more understanding of it myself.  Now I know what I would be suggesting to her.