Hi, I am now 43 and have a severe form of Job's with IgE count >20,000. The older I get the more unexpected problems I develop. One of the most recent is cataracts (cause unknown) and I need to have surgery. It is believed that the infection risk will be very high and I may lose my sight as a result (I am going to lose my sight anyway due to the cataracts). Does anybody know of anyone with Job's who has had cataract or any type of eye surgery, either successful or unsuccessful?

It is a difficult syndrome to live with because as a patient you generally know more than the doctors treating you. Every new treatment you try is fraught with worry because of the number of allergies you suffer from. I have found that the only way to deal with this problem is to follow my "gut instinct" with regard to new treatment and advice. Decision making is also difficult because there is no-one who can advise you as to what the outcome will be - because you are usually the only patient with this condition as well as your new problem.

I think it is very important to have a good rapport with doctors who support you and it has taken me many years to find a team of doctors who will listen to me as well and take my views in to account. I now have a good team. The other thing you need is a sense of humour! I have had many, many infections. operations and serious problems but it is vital to stay positive and try not to get too "down".

I would be happy to exchange information, views, opinions via this forum.

Dear Carole,

I am from Hungary, and I have a 3.5 year-old son with Job-syndrome. He is the fourth person with this disease in Hungary, but we don't know the other three patients. This is the first time, that I've found somebody, who wants to get in touch with somebody else who has the same problems. It would be nice to discuss our problems with somebody, who knows what we are speaking about.

Thank you, and I wish you a Merry Christmas

Hello Heni, sorry for taking so long to reply but I have had various things going on at home and have only just logged on to the site. If I can be of any help/support to you I would be happy to do so. I have had Job's since I was born but at that time it was unheard of, so although I was constantly ill and in and out of hospital as a child, I remained undiagnosed until in my 20's.

If you would like to let me know some information about your son (symptoms, how he was diagnosed etc), I would be happy to pass on my experiences and any treatments I have tried that have been successful or at least helped.

With very best wishes, Carole

Carol:

I just logged into this for the first time. My name is Amy and my daughter Kameron who is 10 has Job's Syndrome. I understand your frustration with the limited information out there regarding Job's.

My daughter is currently in a research study at the National Institute of Health and they have been great.

Being only ten, we have not had any information given to us about cataracts. I would like to ask you if you have had any problems with your teeth.

Kameron has had 8 baby teeth pulled and will be put in braces next month. Her front two teeth on top will not erupt so they are going to have to cut her gums open to attach brackets to them to pull them in.
A minor thing considering all the other illnesses we have had, but it is just one more thing to deal with.

She has had 2 bouts with empyema, over 10 broken bones, horrible skin infections, repeat ear infections. She was first diagnosed at the age of 4. We have fought one infection after enough.

She currently takes an antibotic every day, antibotic creams for her skin, and Advair for her lungs.

Are you taking an current medications? I am very curious to find out anything about your symptoms and illnesses.

I look forward to hearing anything. Thanks.

Take Care,

Amy

hello carole my name is peter i have been diagnosed with jobs as my three daughters i am 48 but have not had any eye problems to date but have lots of others wish you luck with your surgery if you have not had it yet

Hi Amy,

Thank you for your email. Sorry to hear about the problems your daughter is having but I can relate to them all totally. She sounds as if she is going through many of the same symptoms I suffered as a child, but I was not diagnosed until I was 22 so everyone just thought I was sickly! All my problems were treated as and when they occurred without any prophylactic treatment inbetween.

Currently I take three different antibiotics each day and will continue to do so always. However, I do feel that these are beneficial and I also take other medication, such as anti-fungals, to combat thrush as I am sure you know candida is also a problem with Job's. I have put myself on a regime of vitamins and minerals which I feel suit me and I also take Manuka honey orally (the highest purity) which is currently being used in a famous cancer hospital here to fight infections. I haven't tried it topically yet but will do so when I need to.

Having had numerous chest infections and pneumonias as a child this does leave you susceptible to other infections as well as staph aureus. I currently have pseudomonas which I cannot shake off. Unfortunately repeated chest infections do cause lung damage. I now have bronchiectasis as a result but hopefully your daughter may fair better as she has been diagnosed earlier and is receiving treatment.

The abscesses and skin infections are a nuisance! I tend to see them as two separate features of the disease. The skin condition is similar to eczema and causes constant itching 24/7. The abscesses occur anywhere and it is time-consuming having the post-op dressings. From experience, I always insist that the dressings are changed daily (even if the staff are busy) and my preference is to have a gauze wick inserted to keep the wound open as long as possible. Try and avoid having the wound stitched as the abscess generally recurs.

As a child I had a lot of dental problems and needed quite a lot of first teeth extracted. I also had fixed braces top and bottom. I have had gum abscesses (currently have one)and a large dental one as a child which was undetected initially.

I have had some bone fractures in particular my shoulders and feet.

Whilst I understand this seems like a long list of problems, it is possible to enjoy life and make a success of it. I would say the key is knowing your limitations (not that I always take my own advice!!) I trained for two careers, worked full time and married a very supportive man who is my rock! We have good family and friends who are there if we need them.

The above symptoms are from my own experiences, maybe some of which will not apply to your daughter, but I would be happy to correspond with you and give you any help or advice that I can.

Incidentally do the doctors at NIH know of anyone who has developed cataracts and/or had surgery with this syndrome? As yet, my doctors have not been able to trace anyone.

Best wishes to you and your daughter, Carole

Hi Peter,

Thank you for your email and for offering your support.

I would be interested to learn when you were diagnosed and whether you are suffering the same problems as myself and their severity? (if you would like to share the information of course).

Thanks again and best wishes, Carole

Carole:

I don't know if they know anything about cataracts, but I will forward a question to NIH and ask. So many of the things you have experienced sound so familiar to the illnesses Kameron has suffered.

From going to NIH, we have found that Clorox Bathes or swimming helps keep skin infections down. During the Winter, we take clorox bathes twice to three times a week and during the summer, she goes swimming every day.

Currently Kameron has a pneomaticile in the right lung. She has had it since she was 1. We haven't had a bout of pneumonia since July of 2006, but everyday I worry about it.

Do you have any children? Kameron is one of three children and my other Daughter, Jordanne, is also in the study for Job's at NIH. She is considered in the grey area for Job's since she has some of the symptoms, but has not been as sick as Kameron.

I will let you know if I get any information from NIH on cataracts.

Take care and thanks again,

Amy

Carole:

I checked with NIH and they did not have any information on Job's patients with cataract problems. Sorry.

I hope everything goes well.

Amy

Hi Amy,

Thank you for checking with NIH, I do appreciate it. I will just have to hope some information is found or I will have to decide whether to be the test case for the surgery and keep my fingers crossed!!

Have Kameron's doctors suggested subcutanous immunoglobulin therapy? I am on this treatment once a week and was just wondering if it had been suggested to you.

You asked if I had any children, well unfortunately by the time I was diagnosed the amount of lung damage I had suffered was considered to be far too risky for me to chance pregnancy. My husband and I decided to adopt and my condition had to be discussed by a medical panel before we could be accepted. They could not see any contraindications to this and we have one adopted daughter. We adopted her when she was 8 as I did not feel I had the energy to cope with a very young child. This turned out to be the right decision for us and we have not regretted it.

Thanks again for your emails, please let me know how Kameron progresses.

Kind regards, Carole

Hi Carole



Me and my daughters 19,15,10.were diagnosed about 8 years ago.It was quite a relief for me and my wife,after years of Dr appointments, even though it was quite traumatic at the time to think they got it off me.
My eldest suffers mainly with her lungs, boils and abcesses and my 15 year old with her skin and the youngest touchwood is hardly affected.I have in the past have had T.B ,joint infections and skin infections which my old mum tried her best to give me all sorts of remedies, numerous chest infections which has left my lungs damaged and a lot of fungal infections.It probably sounds worse than it is.
Like you say carole it is important to be positive and to stay as active as you can.

Thanks Peter

Hi Peter,

Thank you for replying. I definitely understand the problems you have suffered as I have them all too. Interestingly, I have also had TB (miliary) and this left plaques at the back of my eyes, although it is thought that the cataracts are not related to those. They are possibly the result of medication or maybe just another new symptom of the disease. Do you find that as you get have been getting older the disease is changing itself? Whereas I used to have lots of abscesses and infections, I now seem to be suffering with joint problems, neurological problems and bone fractures. Also chronic fatigue is another symptom and some days just getting out of bed can be a real effort! Do you have any of these symptoms?

I can also understand your feelings about passing it to your daughters. Whilst I have not passed it on, I always feel guilty about the effect my problems have on the rest of my family and the worry they go through. I tend not to say if I'm feeling unwell until I really feel as if I have to. I feel I should suffer in silence!

However, there is no point feeling sorry for myself and I just try to live the life I've got and am grateful that I can still go out and do things (even if it is limited). Could be a lot worse!!

Best wishes, Carole

Hi Carole

Yes i know what you mean about feeling guilty about your problems, i used to feel like i was always moaning or a being like a hypochondriac.So i tend to just go into myself and get depressed.
It was interesting to hear about the joint and neurological problems i have been putting that down to a side-effect of one of the drugs i have been taking for a fungal infection (posaconzole).
Though when i am not well old fractures seem to hurt which cant really be drug related.
Are you on any medication like that Carole?


Thanks Peter

Hi Peter,

I agree with you about the pain from old fractures having broken both my shoulders (I did one just closing the front door!!). I'm still having pain and am still being investigated with scans, but surgery would have to be a very last resort as I'm sure you know joint surgery is not something the doctors really want to contemplate because of the infection risk.

I take an anti-fungal every day (Fluconazole) and will be taking it forever. I don't think I hsve any side-effects from it but then there is so much going on with this syndrome it's difficult to tell!! The neuro problems are affecting both my legs (very painful to touch) and my hands at the moment.

I occasionally get fed-up with things but on the whole I am quite a positive person and make sure I occupy my time as best I can. It's just that the pain is always there in the background nagging away at you and it can be difficult to ignore.

It is really helpful for me to correspond with you so that I have someone to compare symptoms with, especially as we are of similar age. It also makes me feel I'm not quite so alone with this. I was just wondering if the doctors have given you a prognosis based on what they know?

Kind regards, Carole

Hi Carole

I agree Carole i think the correspondence is really assisting me as well.
As regards the Drs, I am not sure they know what is happening,they say i am so difficult to treat they tend to rely on what i am telling them as regards to how i am feeling i have my appointment tommorrow and i intend to have a good chat about what to do future wise please God.I too have been on the anti fungals for the past 6 years but it seems the itroconzole and voriconzole did not work for me and still became allergic to the fungi apergillus.
I came out of hospital about 4 weeks ago after antibiotic and antifungal treatment and i feel great the best i felt in twelve months, but like you carole i been having nerve pains in my arms and legs especially after putting them under preessure walking up inclines or stairs.Last year i also seemed to pull quite a few muscles and tendons.
Have your Drs said anything Carole about what to expect? How are your eyes i hope they are not giving you to much problems.

Best Whishes Peter

Hi again Peter,

Glad that these messages are helping you too. How did you get on at your appointment? Have the docs decided to try anything new? It's good to hear that you have been feeling a lot better recently, do you think you have found a treatment regime that is right for you?

I am currently having another flare-up of infection with my bronchiectasis and had to spend last night in hospital starting IV therapy again. I usually have the first dose in hospital and then give myself the treatment at home. I sometimes seem to have more problems getting the administrative staff to understand that I need to be seen today so I can start treatment, because if they don't know you they just think you're trying to queue-jump. Oh! to have no problems at all!!

Difficult to know what to expect from a prognosis point of view with this disease. I don't think there are enough cases for the docs to make an accurate assessment and I would imagine that it also depends upon the severity or degree to which you have the disease. I know I have a very severe form and would guess that based on my previous track record, I won't be a great-grandma!

Anyway, let me know how you've got on.

Bye for now, Carole

Carole:

Have you every checked into the studies available for Job's? I know that at least for Kameron's situation, it has been great to see people that have seen the syndrome. Sometimes it is nice to have people to talk to that know what you are going through.

From what I have been told, the prognosis for Job's just depends on how soon and how agressive you treat each illness.

Kameron has days where she says she just doesn't feel good. She has aches and pains that are just there. It has been tough and we have had several talks about the fact that she has to go on each day even though so doesn't feel well. We try very hard to not miss school.

I hope that you get well soon.

Amy

Hi Carole

Nothing really new from the hospital Carole,they are just monitoring me at the moment with this new anti-fungal drug posaconazole which seems to be keeping the aspigillus away at the moment.Which is very good news but on the downside the side effects are worsening.
Sorry to hear you had to spend the night in hospital how do you get on with giving the iv treatment to yourself? I asked if I could give the IV treatment for the aspigillus myself rather than take a bed up for two weeks but they would not let me i think its supposed to be quite toxic.
Its sounds like you suffer a lot more than me with the Jobs syndrome Carole. I was wandering what nationality you were and where you were getting treated if you dont mind me asking?
I am English and i am being treated at Addenbrooks Cambridge.
Best of luck for now Carole
Peter

Hello Carole, how are you? How are your eyes?
It is very kind of you, that you want to help. You know, that it is a great help if you can speak to anybody, who knows what you are speaking about.You was the first I've found on this site with this desease.But just imagine what happened! A Hungarian father with an eight year old son with Jobs had found my e-mail address, and wrote me. It seems, that our sons have the same symptoms, and nearly the same age. And of course, the same language makes easier the contact.
I will read this forum more, because ican learn a lot from here, and of course, if I can help anybody, I will do it.Perhaps later I will have questions.
Thank you, and best wishes
Heni

Hi Peter,

Sorry for the delay in responding to your last message, I have had a lot of problems at home just recently and have wanted to find time to reply to you - this is the first opportunity I've had.

How are ths side-effects from the new drug? How are they affecting you?

I hope you've been well and are managing to stay out of hospital - that is always one of my aims in life!!

I am also English and am being treated near Manchester. I do feel as if things are just static at the moment with not much happening on either the research or the treatment front (unless somebody knows something that I don't!) I haven't had my eyes operated on yet - I'm very reluctant as I know I'll be the first but I also know the day will come when I have to make a decision and I feel it won't be too long now.

Anyway, we have to keep hoping and fighting - my motto is "never give in".

Kind regards, Carole