Chester County Allergy Blog

I was talking with a colleague the other day about developing an allergy/anaphylaxis education program for a local school district. As these conversations go,we talked about the various aspects of allergy care and how the goal of such a program would be to try and make everyone in a school as comfortable as possible in recognizing and then, providing assistance to a student who may need help. We talked for few more minutes and then she said to me that her daughter, who is not an patient of mine so HIPPA police calm down, has anaphylaxis and for a long time never felt comfortable if she had to use her epinephrine. Before everyone starts screaming, “she needs more education” she did not. She saw a very good Allergist and was instructed in the proper use, storage and indication for epinephrine use. Her mother, the colleague I was speaking with, is a nurse and very comfortable around injectable epinephrine. So I asked how did she, meaning her daughter, get comfortable about having to use her epinephrine. Her response: “An orange”.

“Excuse me”.

She repeated ” An orange.”

“Yeah, that’s pretty funny.  Seriously how did she get more comfortable with her epi”, I replied.

After a few minutes of staring and smiling at me — I think she enjoyed having stumped me — she relayed her story. Her daughter, a teenager, never felt comfortable if she had to ever use her epi. She had been taught the right things by her Doctor. She had an epinephrine trainer. She knew how to remove the cap, inject herself in her outer thigh, count to ten and then call 911. She knew when to use her epinephrine as well. But she was still afraid if it ever came time to use her injectable epinephrine she would not be able to inject herself.

Then one day, my colleagues daughter decided to use her injectable epinephrine — despite not have any symptoms of food allergy and anaphylaxis.

“Tell me she didn’t just inject herself for kicks”, I chimed in.

Well, I must have had an interesting look on my face as, my colleague, just starred at me. Now I have known her for a long time and I thought she was just messing with me and having some fun — at my expense. But I also knew she, from past experience, she usually has some good ideas and comments.

“She used an orange” my colleague casually throws out there.

“Really”.

“Yup, she used her expired injectable epinephrine on an orange”. She explained, her daughter was always afraid that the real epinephrine would be more difficult to use than the trainer. Once she used her expired injectable epinephrine on a orange she realized that the it really was not that difficult and now she has no fear or worries about using her injectable epinephrine.

Well this idea, use your expired injectable epinephrine on an orange,  just became apart of my injectable epinephrine education program.

Don’t you hate it when teenagers teach us lessons!

I recently became aware of some information that may provide improper advice for those with food allergies. This information suggests that food allergic individuals need not be as vigilant as they should be as long as they have their injectable epinephrine with them. Well, this is just plain wrong!

 

Let’s review some basics about food allergy management. Now this all assumes that one has been properly evaluated and diagnosed as having a food allergy.

 

1.Know what you are allergic to.

2.Read labels and understand what you are eating.

3.Make sure others know what you are allergic to. Don’t accept or eat any food if you are not 100% sure of the                                                           ingredients. 99.999% is not good enough — if you are not sure don’t eat it. Remember food allergy can kill!

4. If you are going to a party or some other function and you are not sure of the foods that will be there, bring your own food.

5.Have an Anaphylaxis Treatment Plan. Know what do in the event of an exposure to your food allergen or if you develop allergy symptoms even if you don’t think there was an exposure to your food allergen. Treat yourself first then figure out what the exposure was later.

6.Always have your injectable epinephrine with you. Remember, if you have an exposure to your food allergen and develop allergy symptoms the treatment of anaphylaxis is use your epinephrine. Benadryl, albuterol and  other medicines can be helpful to treat your symptoms after you use epinephrine.

7.If you use your epinephrine, call 911. Food allergy reactions and anaphylaxis are life threatening reactions and require emergent care, evaluation and treatment. Epinephrine can be life saving but even with the prompt administration of epinephrine and calling 911 there can still be bad outcomes.

 

If you have questions about your medical condition or the medication(s) you are taking speak with your physician.  Always remember, your physicians ethical and professional responsibility is to take care of you!

Sarbjit Saini, MD – Associate Professor at JHU. CIU and  response to Anti – IgE therapy. Notes from presentation at 51st Swineford Conference at UVa

 

General Overview about CU:

CU > 6weeks of hives; 80% are idiopathic; 1% prevalence in US; 2F>M

50% better at one year; >20% will have disease >5yr

angioedema predicts more severe disease.

 

Coexpression of angioedema in CIU:

50% Urticaria; 40% have Urticaria and Angio;  10% just angioedema

 

Evaluation of CIU:

exclusion of drug/food (rare);

1/3 with NSAID intolerance;

exclude secondary causes (25% have abnormal thyroid test; thyroid ds is 2 times nl frequency);

consider rare disease (chronic hepatitis, cryoglubulinemia, systemic vasculitis, chronic infections – sinus, dental, H pylori etc)

JACI Feb 2012:hypothyroid 10% CU vs 0.6% in NL

hyperthyroid 2% CU  vs 1%   in NL

20% of thyroid ds before dx with CU

3-4% dx with thyroid disease with 6 months of Cu

 

Systemic W/U of 6000 cases of CU: 

1.6% of cases had other disease with extensive lab w/u

? do we need to do lab w/u in CIU if only rarely find other diseases?

 

Approach to CU:

Non sedating  anti histamine at normal dosing, if not controlled then updose to 4x nl dosing. If not helpful then add H2 blkr, LTRA, and / or change anti histamines. If still not  controlled then consider prednisone burst for 3 – 7 days.

If hives then still persist then into refractory CIU and consider skin biopsy to confirm what is going on and then possibly alternative agents

 

Why Biopsy?

1) confirm histology ( should see edema, vascular dilation, perivascular infiltrate to include lymphocytes, eosinophils and basophils)

2)Characterize infiltrate:

lymphocytre predominant urticaria +/- eosinophils ( ? use sulfasalazine)

neutrophil predominant  (? dapsone or colchicine)

if use alternative agents will need to monitor CBC, LFT’s

1. Exclude other disorders ( leuokocytoclastic vasculitis, mastocytosis )

 

CYA for CIU:

lymph predominant disease; CYA will inhib T Cells as well as MC and basophils

monitor BP, Cr

 

CIU Proposed Pathophysiology:

trigger of some sort to MC (allergen autoab unkn)

histamine release and other med recruits leukocytes

Basophils are depleted in those with more severe CIU – see basopenia

Suppressed Basophil Histamine Release

Auto ab mechanism:

trigger IgE receptor on MC/Baso; 40% of pts have autoab

looking for autoab assay:

autologous ST, sera for auto ab, HRA with pt sera and donor basophils

these assays don’t cross compare

 

CIU index:

if some possible HRF with see Baso release

or see expression of new markers

25% of nl have positive CIU index – problem

even pt who go into remission still have positive CIU index – problem

no assay is superior; problems with specificity

 

Skin MC in CIU:

release to 48/40 in increased in active CIU vs in CIU remission which suggests reversible skin MC hyper- responsiveness.

MC numbers are not increased in CIU biopsy sites. Nor is tryptase elevated in CIU patients. However, tryptase is slightly higher in CIU vs atopic patients. The skin MC in CIU patients have increased spontaneous histamine releasability.

CD34+ MC have increased spontaneous histamine release

Basophils in CIU:

decreased in blood and are recruited to lesional and non lesional tissue

Basophil Histamine Release via FcER1 is reduced during disease and rises in remission

 

Two functional phenotypes of basophils seen on CIU

Responders (R) and Non responders (NR) – based upon histamine release to polyclonal anti IgE: CIU – R and CIU – NR

Longitudinal follow up of these pt – those with active disease stay in the same phenotype but with remission: baso fxn changed from suppressed to non suppressed state as get better

Also will see # of blood basophils increase with remission – ** blood basophils counts are based upon 120cc of blood. This amount was needed to be drawn and basophils counts done to see difference. Typical CBC basophil count probably not enough volume.

 

Why Xolair in CIU?:

Xolair impairs ability of IgE to bind to receptor; dec # of FcERI, decrease meds and decreased symptoms. We know this from asthma, AR.

 

Xolair decrease IgE within a few days then decreases basophil surface IgE and FcERI and function. Then see MC change with tissue MC taking months to drop b/c so longed lived take a long time to drop receptors

 

Reasons why Xolair might be helpful in CIU:

We know that there is MC activation with late phase infiltrate. This is accompanied by altered basophil FcERI HR, basophils being recruited to skin lesion and basopenia.

 

Therefore, omalizumab could cause shifts in MC and Baso FcERI function. Be able to distinguish the roles of MC and Baso in CIU based upon onset of action and possibly reduce targets for autoantibodies ( IgE and FcERI)

 

Random DBPC trial: (Goober AAAAI 2008)

20pt; 18-80yo; symptoms >12 wks; can have angioedema; daily H1 blocker; no steroids or other immunosuppresants < 1 month prior to study

Results:

decreased MD urticaria severity score, within first weeks – with rebound as tapered off

pt reported increased symptom free days

basophil # rise with tx within a month and decrease after stopping drug

 

Kaplan Xolair in auto immune urticaria: JACI 2008

pt with posit Baso histamine releasing activity

Phase 2 study of Xoalir in resistant CIU:

within one week see decreased symptom score; can see a dose effect as well ( to some degree)

Some conclusions:

This brisk effect of xolair is much faster than would be expected if due to MC alteration

Xolair helps in both CIU and autoimmune CIU

? long term disease benefit – what happens when they stop

risk of anaphylaxis. malignancy, carry epi, high rate of local rxn

Novel role of IgE as opposed to IgE that sits on cells and binds allergen(s)

Basopenia and basophil FceRI mediated response improve

basophil shifts like natural remission

 

 

Pork – Cat Syndrome

Below is a summary of a presentation on Pork – Cat Syndrome by Jonathon Posthumus, MD, an allergy fellow at the University of Virginia, presented at the 51st Annual Swineford Meeting held at the University of Virginia April 2012.

 

Pork – cat syndrome was first reported in France, by Drouet et al,  in pt with FDEIA after eating pork. RAST inhibition confirmed shared allergen identified as albumin (67kD) with further characterization revealing that the cross reactivity between cat dander and pork meat are due to shared epitopes on albumin. In European literature, Pork – Cat syndrome symptoms are typically immediate in onset ( OAS, FDEIA and fatal anaphylaxis) with specific IgE to cat epithelium, cat serum albumin and pork meat. Symptoms may be inconsistent and related to co factors such as exercise, alcohol ingestion and dose.

 

Patients are exposed to animal serum albumins in potentially three way: inhalation, ingestion and dermal exposure. For inhalation, house dust contains high levels of cat albumin, even more than Fel d 1. For ingestion, pork, beef and lamb contain large amounts of albumin. As dermal exposure, children with AD have higher incidence of beef allergy.

 

IgE cross reactivity against albumins in pt allergic to animals. 200 subjects allergic to animal dander. 30% had specific IgE for various species albumin. Most had high level of cross reactivity between cat, dog and horse. A few albumin sensitized patients demonstrated IgE highly specific for one species.

UVa study to determine specificity of IgE to serum albumin studies. IgE adsorption studies done with porcine, canine, feline and human serum albumin. Sera of 3 patient with cat – pork syndrome used. Studies revealed that IgE to pork meat and porcine serum albumin was completely bound by cat serum albumin. In addition, IgE to cat serum albumin was not completely adsorbed by porcine nor dog serum albumin. All suggesting that sensitization to cat serum albumin is primary event.

Further UVa studies looked at epidemiology of sensitization to cat cat serum albumin in birth cohort of 200 US children and 963 subject from Sweden. Of the 200 US subjects: 31% sensitized to mite, 8% to egg and 3% to cat serum albumin. Of the 963 in Swedish cohort: 242 had IgE to cat epithelium and of those 215 screened for cat serum albumin with 25 being positive for a prevalence of 3%.

Conclusions:

Pt with immediate reactivity to mammalian meats may have IgE to cat and dog albumins.

The structure of albumin is highly conserved through mammalian species which likely accounts for cross reactivity between pork and other meats.

UVa adsorption data suggests that primary sensitization in these cases is feline and/or canine serum albumin rather than pork serum albumin.

 

Some other clinical pearls:

Cat serum albumin: Fel d 2

Dog serum albumin: Can f 3

 

In the presentation of three patients clinical history, one patient they used a milk challenge as a surrogate for albumin as milk is very high in albumin. Pt had OAs and urticaria from the milk challenge.

 

References:

Drouet et al Allerg Immunolog 1994;Oct 26(6) 305-6

Drouet et al Allerg Immunolog 1994; 166-68

Spitzeuer et al JACI 1995;96:951-9

Drouet et al Allerg Immunolog 2001; Apr 33(4):163-5

I know I am not suppose to watch TV but I do. Let’s face it there are some pretty good shows on and well it is LAX season and I am a big LAX fan. So I watch tv. But I have to say that the advertisements on TV drive me nuts. One of my biggest pet peeves are the drug ads that are starting to dominate the advertisement slots. What is most annoying about some of these advertisements is the misinformation they contain.  There is one advertisement in particular about seasonal allergy that, in my opinion, presents incorrect information. Now before I get in trouble with the makers of any ad, I am in no way making a comment on their medications. In fact I have no opinion one way or the other about the efficacy of their medication. However the content of the advertisement about pollen and seasonal allergy is misleading, in my opinion.

 

We all have probably seen the ad of the little bee that is buzzing around the flowers and talking about seasonal allergies. As this bee buzzes around the flowers, there are suggestions of people having seasonal allergy problems. Now while the bee, with its accent, is cute and all the flowers are pretty, bees and flowers are not responsible for seasonal allergies! That image is just not correct. I am sure that is not the intention of the marketing group that developed and produced that ad, but cute and cuddly does not make medical fact. As discussed in my previous blog, spring seasonal pollens, in this area, are trees, grass and weeds. These pollens are produced by their respective plants, that are not flowering or have very small flowers, and are dispersed by the wind. Because they are dispersed by the wind, they become airborne and are able to come in contact with our eyes, nose, lungs and skin. Consequently, these airborne pollens can, in susceptible individuals, cause season allergy symptoms. Bees have no role in pollinating trees, grasses or weeds. Flowers have no role in causing significant airborne pollen levels that can cause seasonal allergy.

 

Bees do play a part in moving pollens from plant to plant. However, bees do this by being attracted to flowering plants and physically having the pollen become attached to their bodies. When they move on to another flowering plant they transport the pollen with them and some of that pollen is deposited on the new flower. This pollen the bees are transporting does not become airborne and cannot cause seasonal allergy as it is not airborne! The suggestion of the bee and flowers as playing a role in seasonal allergy  is not correct.

 

Enough ranting. Lesson here, take what you see and hear in advertisements with a grain of salt. If you or a loved one have a medical condition and you see or hear of a medicine that may be of benefit, ignore it. What? That’s right I said ignore it. If you have a medical condition, talk with you Doctor about how best to treat YOUR medical condition. A cute buzzing little bee, a bathtub with a wonderful view or a bunch of pipes walking around, does not know your other medical problems, does not know what may have been tried before and has worked or not worked, does not know what you can afford or not afford, does not know what your insurance coverage is. Most importantly, these marketing efforts do not know you! Speak to your Doctor for medical advise don’t listen to your tv.

As we enter into pollen season, I thought it would be helpful to review some plant biology and give a very broad overview about pollen and the allergens that are produced in the spring. This information will be presented over the next several weeks in my blog.

Pollen, a fine to course powder,  contains the microgametophytes of seed plants which will develop into the the male gametes (sperm cells) of these plants. Each pollen grain consists of a few cells which are the vegetative cells and reproductive cells. The reproductive cells has two nuclei, the tube nucleus that forms the pollen tube and the generative nucleus that divides to form two sperm cells. Pollens vary in size from 5 – 200 um.

The wall of the pollen grain has two layers: the exine ( outer layer ) and intine (inner layer). The exine, composed mostly of sporopollenin, is further divided into two layers called the tectum and the foot layer (which is just above the intine). These two layers are separated by the columella, which provides some structural support to the exine.   It is the exine that gives pollens grain their unique features and allow identification of the pollen type (grass, tree, weed).  These features can include thinning, ridges and pores, which serve as an exit for the pollen contents and allow shrinking and swelling of the grain caused by changes in moisture content. The pollen grain can have furrows which are called colpi (singular: colpus) the orientation of which classify the pollen as colpate or sulcate. Additionally, the exine often bears spines or warts, or is variously sculptured, and the character of the markings is often of value for identifying the type of pollen

The intine is a cellulose rich cell wall

The structure of a pollen grain. Source: www.tutorvista.com

The transfer of pollen grain (male) to the female reproductive structure (pistil) is called pollination. Pollination, i.e. pollen dispersal, is accomplished in two ways: through the wind and by insects.  Wind dispersed pollen is called anemophilous and insects dispersed pollen is called entomophilous. For allergy sufferers, anemophilous pollinated plants are the ones that cause seasonal allergy and these are typically trees, grass and weeds. These plants in general do not have flowers or they have very small flowers.  Exposure to particular aeroallergen pollens depends on the plants growing in a particular area. In addition, pollen specific factors such as buoyant density, ease of dispersion and profusion effect local pollen concentrations.

 

Future blog posts will discuss the allergens in grass, weed and trees.

 

 

     I was at a meeting the other day and was listening to a report about the performance of a hospital. All these metrics were being measured and we, the physicians, were being told about how good we were doing meeting the various metrics but there was still work to do. Now, one would think these metrics were about patient care — and they were to some degree – and improving patient care – and the quality care  people certainly think these metrics improve patient care.  But the real reason the metrics were being measured was because the hospital was losing money or would lose money if the metrics were not measured and met some goal.

 

The focus of healthcare has now changed, in a very dramatic way, from caring for patients to measuring healthcare metrics that may or may not benefit patients. This is a very dangerous change. Doctors are no longer giving aspirin to heart attack patients because it is good care,  we are giving aspirin to heart attack patients because some administrator some where has decided that this metric must be measured and if not followed there will be a financial penalty. So much for caring for patients, we now care about heath care metrics.

 

This mindset, that quality metrics will improve healthcare,  has insiduously infiltrated medical care and is irreparably harming the physician – patient relationship. Top notch medical care traditionally has required physicians to do what is best for their patient.  Top notched medical care now requires the physician to understand what metrics are being measured – did you give the right medicine? did you give it at the right time? did the patient give you a good evaluation? did you document that you gave the right medicine at the right time? are you communicating appropriately with the patient? do they understand all the complex decision you are making in order to help them? Do the patients like the bed they are sleeping  in? Is the hospital food good? and oh by the way how is your patient doing. I am not sure I can care for my patient and at the same time care about my metrics.

 

The continued erosion of the patient – physician relationship with the insertion of insurers, administrators, quality metrics analysts and the myriad of others is having a direct negative impact on healthcare. Medicine has become a business focused venture that measures outcomes and metrics. As highlighted by Ryan et al in a recent Health Affairs article ( Health Affairs 31, NO. 3 (2012): 585–592) Medicare’s public reporting on quality measures has had a minimal impact on patient mortality. In fact, there was no improvement in mortality rate for heart attack and pneumonia and only a slight improvement for heart failure this, despite the institution of public reporting of quality metrics. So much for quality metrics improving health care and saving lives.

 

Physicians must change this paradigm. To continue to go down this road and adopt policies that clearly do not improve healthcare is idiotic. Physicians must stop being sheep and start being the leaders, the true leaders,  in health care for our patients. Physicians must challenge the current, in vogue, healthcare reform paradigm. The health care reform experiments of the past 40 years have clearly been ineffective – and there is no indication that the latest iteration of health care reform will do any better.  Will challenging this paradigm du jour be difficult and challenging? You bet it will. But I  for one would prefer to work to develop healthcare reform measures that actually improve improve patient care and save lives. What do you want to do?

I just got done reading an article from Omaha about a child who had a severe reaction to peanuts (http://www.ketv.com/news/30307603/detail.html#ixzz1kcSdIHui). The headline “Boys survives Delayed Reaction to Peanut” piqued my interest. I was expecting a story about a patient who had a reaction 4 or more hours after ingesting peanut. This was not the case. A child with asthma, as well as gluten and peanut allergy, had an accidental exposure to a peanut butter granola bar. According to the story, he immediately had symptoms of itchy mouth. The school called the parents and then they drove him to the hospital.  At that time “ The boy complained of a stomachache but was talking and breathing comfortably” After another thirty minutes in the ED he then developed hives and his throat was tightening. His condition then rapidly deteriorated and required very aggressive medical care. Fortunately, he recovered and is doing well.

 

This story raises some concerns and questions. First off, as described this was not a delayed reaction to peanut allergy. This child has a known exposure to a known allergen and “Max said he knew there was trouble when after just a penny-sized bite, his tongue started itching.” Then over the course of the next thirty plus minutes his symptoms progressed to severe anaphylaxis. Anaphylaxis is an immediate reactions to an allergen that can progress over time. This is exactly what was described in this article – the child had a known exposure to a known allergen developed symptoms immediately that progressed to more severe symptoms over the course of at least 30 minutes. This was not a delayed reaction to peanut exposure this was classic anaphylaxis.

 

Second, it does not appear from the article that this child ever received epinephrine for his symptoms. After he had the peanut exposure the school called his parents and he was taken to the emergency department. No where was it noted that he received epinephrine. Furthermore, according to the author of the article, “The boy complained of a stomachache but was talking and breathing comfortably. After 30 minutes in the emergency room, the child broke out in hives and his throat was tightening.” Subsequently more symptoms developed and he become very ill and came close to death. The statements as reported above, suggest that this child with a known peanut allergy and asthma may have gone as long as 30 minutes without receiving epinephrine despite progressive symptoms.

 

There are several lessons to be learned from this article as reported:

1) peanut allergy can be quite severe and life threatening; 2) an asthmatic with peanut allergy and known exposure to peanut and develops symptoms should be treated with epinephrine immediately; 3) delay in treatment of peanut allergic individual will result in progressive symptoms that will be more challenging to treat;  4) asthmatics with peanut allergy are at risk for more severe reactions when exposed to peanuts and 5) schools need to have good guidelines for treating asthmatic peanut allergic children who are exposed to peanuts – not just calling the parents.

 

 

Peanut allergy is a serious disease. Peanut allergy patients with asthma are at particularly increased risk for serious reactions to peanut exposure. Prompt recognition of symptoms and aggressive use of epinephrine is required to treat these patients. Are you prepared?

 

 

Do you have asthma? Does your child have asthma? Do you think your asthma is under control? Do you think your child’s asthma is under control? Think again. The reality is that most patients and most parents of children with asthma consistently overestimate the level of asthma control they have. While it has been known for  almost a decade now that adult patients are not very good at “perceiving” their asthma. Recent data published in the European Respiratory Journal suggests the same is true for parents of children with asthma. In this study parents typically overestimated the level of asthma control their child was able to obtain. Incredibly, almost 70% of these patients altered their lifestyle because of their asthma.

This is a incredible difficult “nut to crack”. Patients and their parents are perceiving their asthma to be under control and then they are told this is not true. Talk about an difficult conversation. “Yes Mr Jones I know you think you are doing well but I can tell you I know you may not be”.

So what to do? First off realize that your perception of you asthma control or your child’s asthma control is probably not right. This is not to say you are doing the wrong thing treating your asthma but your perception may not be consistent with breathing test data or detailed questioning. This is why it is important to have regular consistent follow – up with your asthma Doctor. They will asked detailed questions trying to ascertain how you are doing and then check your breathing test to make sure your FEV1 is the best it can be. Adjustments may be made to your treatment and there may be more intensive follow – up visits. Remember the goal of asthma therapy is to be able to do whatever you want to do without limitations.

Do you want to be apart of the 70% who have changes their lifestyle to accommodate their asthma?

 

 

Severe Combined Immunodeficiency (SCID) is a congenital immunodeficiency that if not detected in the first few days of life invariable is fatal. Fortunately this is a very rare disease BUT for those with SCID that is not detected the consequences are devastating. Fortunately over the past several year new technology has emerged that allows the detection of SCID babies at birth. The SCID screen is done off the blood spot that is used for other diseases that are screened at birth. Do you know if your hospital screens for SCID?