They say that we need to control the Eczema first before they could do anything .
I have left some messages with some other Dr's to see how we can get him in for a second opinion. I will let you know how it goes, when we get to go to another Dr. They did switch his creams around . It is a waiting game, until we get a call back! Thanks for the support,Lisa

The Immunologist did change his meds to some other creams and told us to put wet pj's on him and a dry ones on top of them . We are getting a second opinion at Duke , hopefully we can get him in sooner than the apt I setup Mon. , The Allergist left a message for the Dr so hopefully the Allergist can get him in sooner than I did. June 25th.

Lisa

Yes we had to do that too with Sam for some time at bed time, it's called wet-wrapping. After washing and applying the steroid potions, he'd be absolutely slathered with Diprobase moisturiser and then we'd put him into damp tubi-grip, different sizes for arms, legs and body. Didn't think of using damp pj's - good idea.

Sam's skin has been relatively problem free for the past 18mths, but just lately things are starting to flare up again and he's getting more and more infected excema. Yesterday we requested to see a Dermatologist as a matter of urgency, one who's reasonably clued up on skin infections and excema with Job's Syndrome, expect they're rather thin on the ground.

June 25th is an awfully long to wait! Maybe you can get your son on a cancellation list, and see the Doc at short notice?

Ax

Hi Everyone

Just come across this forum, my daughter Caitlin was diagnosed with Hyper IgE Syndrome when she was 2 1/2 with an IgE count of nearly 30,000 she is now 11. She has been ill from the day she was born.

At first the Health Visitors and Doctors said her severe eczema was all my fault, wrong washing powders etc. she saw a dematologist at our local hospital who gave her a skin wash and discharged her after 6 weeks, I kept going back to our GP (I saw every one in our surgery) the last one was not happy that we had been discharged from the hospital and she referred us back to a private consultant who is marvelous. He took biopsies and gave us all sorts of lotions and potions he also referred us to a Cranial Osteopath as Caitlin didn't sleep (20 mins at a time if we were lucky) this was a godsend. When she was about 15 months old she started to get boils on her head, she went to theatre 3 times to have them removed, this was when our Consultant tested for HIES, during the first 2 years of her life we must have seen at least 50 Drs as we used to go to special consultation days.

We have been going to Bristol hospitals eversince the diagnosis, we have not met anyone else with this condition. Caitlin has broken the same arm twice in 9 weeks, had numerous chest infections, loads of ear infections, bad skin constantly, been a hospital inpatient on a number of occasions and been until last May on Co-Amoxiclav and Flucloxicillin all the time. Since stopping the anti-biotics she has had two operations on her eye for infected cysts, an absess under her arm which burst, another on her neck and two more on her head all of which have been treated as an inpatient at our local hospital not Bristol. Three of these episodes she was diagnosed and treated for MRSA with IV Vancomycin and oral Fucidin. The Drs can never seem to agree how long she should have the IV's for! She has been decolonised 3 times but it doesn't seem to work.

She has only had one of her baby teeth fall out naturally all the rest so far have had to be surgically removed, she has had a brace for the last 2 years. Caitlin also has hyper mobile joints and complains of aching joints especially her legs but nothing shows up on xrays as yet. She also has the facial features for HIES, on the whole she is a happy little girl but at times I wonder how much more she can take. I can see another trip to the Drs looming as she is complaining of pains round her chest and back.

Its nice to know that there are other people in the same position as us.

Hello Floss

Nice to meet you, sorry it's over having poorly children. Try and get your daughter referred to Prof. Cant at Newcastle General, he's the top man for this condition. If you've been following this thread you'll see that our son Sam (15) has been seeing him and he was the one who diagnosed Sam with Job's and since following all his medical advice Sam has been leading a relatively healthy life. Sam has to take prophylactic antibiotics and anti-fungals every day. Please contact me directly on alice@quay-design.co.uk if you need to chat about this further.

Ax

Hello Lisa, my daughter is the one (in the thread above) who was taken to NIH in Bethesda, MD. Since you are right there in VA, I highly recommend trying to see them. I have the email address to the chief doc. in charge of the Job studies. Her name is Dr. Alexandra Freeman. If you qualify financially, they will pay for your cost to get to there, and they will arrange for you and your child to stay (free) in the Children's Inn right next door to the clinic. They will do a FULL work-up of your child (blood, urine, dermatology, immunolgy, allergy, asthma, dental examinations, x-rays, MRI's, CAT scans, dexa-scans (bones density scans) and a genetics consultation). They will examine your child's DNA, genetic coding, genetic history, medical history....you name it, they research it. They will answer ALL of your questions and concerns. And they don't make you feel like your child is a science experiment. They are very personable, very sympathetic, very friendly, very helpful. We were there 3-4 days and I have absolutley no complaints at all. And, since they are a government funded organization, no health insurance is ever required. They won't even ask you about it. Everything it FREE. In the event that you don't qualify financially for them to pay for your travels and hotel costs, then your only expense will be getting there, and your hotel. The hospital and all the medicince they prescribe you are totally free. They will do an initial work-up at your first visit. IF they determine that your child is truly a Job patient, then they will want to see him back no less than once a year for follow-up, and those subsequent visits are totally free (they will pay for your travel and hotel). When they release you back home, they will work closely with your doctors back home to make sure that the doctors there know what your child's diagnosis is and how to properly care for him.

My Son Hunter has just been diagnosed with HIES, Job Syndrome. What have y'all been using for moisturizers for the face any suggestions to help the scaring and the outbreaks?

any advice would help.

we are also working with the NIH--is there any plans for experimental drugs/treatments.

has anyone read the articles linking the STAT-3 Gene mutation to the TH-17 cell production or white blood cell production?

Please share

Pete in Texas

FYI Y'ALL___




NIH Clinical Research Studies

Protocol Number: 06-I-0032
Active Accrual, Protocols Recruiting New Patients

Title:
Pilot Study of Omalizumab (Xolair) in Hyper IgE (Job's) Syndrome

Number:
06-I-0032

Summary:
This study will evaluate the safety and effectiveness of a laboratory-made antibody called omalizumab in patients with Job's syndrome, or hyper-IgE syndrome (HIES). Patients with HIES have very high levels of IgE antibody, a protein made by white blood cells. IgE plays an important role in starting allergic reactions in the body and may be related to some HIES symptoms, such as skin rashes and asthma. Patients also have frequent lung infections, easily broken bones and other symptoms. Omalizumab, which is approved to treat allergic asthma, is directed against IgE. This study will see if blocking IgE with omalizumab in HIES patients is safe and if it can reduce patients' IgE count. It will also look at how the body handles omalizumab and how it affects patients' symptoms.

Patients 6 years of age or older with HIES may be eligible for this study. Each candidate is screened with a medical history, physical examination, skin examination and blood test.

Participants receive an injection of omalizumab under the skin once every 2 weeks for 6 doses. At the time of each injection, patients are examined by a doctor, answer questions about their symptoms and have a blood sample drawn. After the sixth dose, patients have a physical examination, blood tests, skin examination and lung function tests. At follow-up visits scheduled 2, 4 and 6 months after the last dose of omalizumab, patients have a physical examination, answer questions about their symptoms, and have a blood sample drawn. Patients who show a significant response to omalizumab stay off the drug for 3 months after the last dose and then discuss with their study doctor and referring doctor about continuing the medicine.

Sponsoring Institute:
National Institute of Allergy and Infectious Diseases (NIAID)

Recruitment Detail
Type: Participants currently recruited/enrolled
Gender: Male & Female

Referral Letter Required: Yes

Population Exclusion(s): None

Eligibility Criteria:

INCLUSION CRITERIA:

1. All Subjects must be at least 6 years of age and no older than 76 years of age (as per original safety studies).

2. All subjects must meet the established diagnostic criteria for HIES as determined by the NIH scoring system, with a score greater than 40.

3. Baseline values within the following laboratory ranges:

White blood cell count greater than or equal to 3,300 cells/microliter.

Absolute neutrophil count greater than or equal to 1,000 cells/microliter.

Hemoglobin greater than or equal to 10 g/dL.

Platelet count greater than or equal to 100,000 platelets/micoliter.

4. Women of childbearing potential only: negative serum beta-hCG. Both men and women and their partners must agree to practice abstinence or effective contraception from initiation of the protocol and for 2 months following the last dose of the study drug (effective contraception methods include abstinence, surgical sterilization of either partner, barrier methods such as diaphragm, condom, cap, or sponge, or hormonal contraception).

5. Weight (Kg) x serum IgE (IU/mL) less than or equal to 63,000 (per dosing restrictions) for initial safety study, then, as above less than 2,000,000, greater than 63,000, then as above greater than 63,000, less than 125,000.

6. Patients in tier IV must be 16 years or older.

7. Preference will be given to patients with dermatitis.

8. Subjects (guardians for younger patients) must be able to give informed consent (or assent as appropriate).

9. Subjects must already be signed to other NIH studies on HIES.

10. Subjects must be willing to maintain their current regimens for skin care, prophylactic antibiotics (if applicable), and any asthma related oral or inhaled medications. Albuterol rescue medications may be used as needed.

11. Patients participating in protocols at the National Institutes of Health are expected to have a primary physician outside of the NIH.

EXCLUSION CRITERIA:

1. Pregnant or nursing women.

2. HIV positive diagnosis.

3. Use of any other investigational agent within 30 days of the study.

4. Any condition that, in the investigator's opinion, places the patient at undue risk by participating in the study.

5. Previous anaphylaxis to the study medication.

Special Instructions:

Currently Not Provided

Keywords:
Phenotype
Monoclonal
Immunoglobulin
Immunodeficiency
Dermatitis

Recruitment Keyword(s):
Job's Syndrome
Hyper-IgE Syndrome
HIES

Condition(s):
Hyper-IgE syndrome
Job's Syndrome

Investigational Drug(s):
Omalizumab (Xolair)

Investigational Device(s):
None

Intervention(s):
Drug: Omalizumab (Xolair)

Supporting Site:
National Institute of Allergy and Infectious Diseases

Contact(s):
Patient Recruitment and Public Liaison Office
Building 61
10 Cloister Court
Bethesda, Maryland 20892-4754
Toll Free: 1-800-411-1222
TTY: 301-594-9774 (local),1-866-411-1010 (toll free)
Fax: 301-480-9793

Electronic Mail:prpl@mail.cc.nih.gov

Citation(s):
Lee M, Hodges WG, Huggins TL, Lee EL. Eosinophilic gastroenteritis. South Med J. 1996 Feb;89(2):189-94.

Kelly KJ. Eosinophilic gastroenteritis. J Pediatr Gastroenterol Nutr. 2000;30 Suppl28-35. Review.

Park HS, Kim HS, Jang HJ. Eosinophilic gastroenteritis associated with food allergy and bronchial asthma. J Korean Med Sci. 1995 Jun;10(3):216-9.

Active Accrual, Protocols Recruiting New Patients

If you have:

* Questions about participating in a study, please contact the Patient Recruitment and Public Liaison Office, CC.
* Technical questions regarding the Clinical Center web site, please contact the Department of Networks and Applications, CC.

Hello,
Sam uses Diprobase moisturiser. He gets it, by the tub(!) on prescription from the chemist. In our experience the "plainer" the moisturiser the better. Over the years we went down the route of trying various fancy potions from herbalist shops and High Street stores, but for him they were just a big waste of money.
Ax