Lung Damage Due to SJS

[retiredrn]

My son had Stevens johnson syndrome last june (05) from amoxicillin. He has had a chronic cough ever since. He was recently diagnosed with Bronchiectasis ( lung damage) he is curently 16 years old. I believe the 3000 mg/day dosage Rx'd by the D.O. was excessive for a 119 lb. kid. Very frustrated with ALL he has had to go through and now he has sustained lung damage. Do I have a case? we live in Idaho

[rmet4nzkx]

SJS and the damage resulting from the adverse drug reaction can take as long as one year from the discontinuation of the RX after the initial reaction and manifestation, so while your son may have had the initial reaction there may be more damage than seen. Most skin damage heals fairly well, damage to lungs and eyes not so well, it is not the amount of Rx, per se, it is a reaction or hypersensitivity which may build up over time and other factors. While 3000 mg per day is about twice the reccommended dose, there are indications of the 3000mg dose and since you didn't say what your son was treated for, or the actual dosing instructions I can't comment further. I am interested in when did he present with symptoms, what were the symptoms how long before the Rx was D/C, when was he diagnosed with SJS, who diagnosed him? What other medications has he taken since then?
Please see this site for more information and support www.sjsupport.org/index.shtml They can help you further with this issue and questions you might have. According to this link, http://www.springerlink.com/content/k312w4315k532525/ almost 1/3 of children with SJS have lung involvement. You might also get your son tested for A1AD which might make him at higher risk for lung involvement, this testing is free and confidential but may be helpful in his treatment. Alpha One Foundation For more information or a test kit please email the Registry Coordinator at alphaone@musc.edu, or call toll free at 1-(877)-886-2383.
www.alphaone.org/alpha1/get_tested.html

[retiredrn]

thanks for your response.His Rx was 500 mg 2 tabs TID for an ear infection. However, he presented with a deep cough, fever ~102. I questioned the Dr re: his cough and he explained that it was due to the drainage from his ear infection, which (according to an ENT I have worked for) is not possible. No chest Xray was performed. (however, he has a history of bronchitis)The medication was D.C'd after 48 hours by me, and took my son to the ER when his fever reached 103-104 without relief with Tylenol. He was by then ,sloughing mucous membranes, had what appeared to be conjunctivitis, and c/o pain in his testes. Dr.s never have confirmed the Amoxicilin was the culprit, however ALL other possibilities were ruled out. The prescribing D.O became very defensive when I was trying to get a full understanding of what was going on. As a RN I had NEVER heard of SJS! he was hospitalized for 8 days. They finally diagnosed him with Pneumonia also (not mycoplasma) ~3rd day of hospitalization. He was given a course of Zithromax followed by Levoquin. Since his ordeal last year he has not had any medication whatsoever.

[retiredrn]

forgot some things you asked for. They came up with the SJS dx for sure when he started to develop the target lesions the next day. dermatologist was NOT consulted until 6 days into his hospitalization. By then he wasn't developing any nw lesions. an opthomologist was immediately involved. He had minimal scarring without visual impairment. However, his inner lower lids look inflamed always. he is still being followed by an opthomologist. The prescribing Dr was enjoying the memorial day weekend off, so by the time he was back he had already been diagnosed with SJS.

thanks for your help

[rmet4nzkx]

As a RN you know that the appropriate place to take him based on the clinical signs was the emergency room, whether or not it was a holiday weekend, whether or not his treating DO was away for the holiday or not and even so, they failed to Dx SJS for how many days? In actuality, you were lucky to get a DX that quickly as it is often missed and even though you D/C the Rx many reactions can be delayed so you are left with a vexing set of signs and no Rx to blame because it was D/C. There could be an error related to the Rx, did you actually look at the script or only what was on the bottle? If the doctor Rx 250 2 tabs TID 1500 mg the normal dose for severe ear infection/chronic bronchitis, it is possible that the actual fill was substituted, 2tabs@250mg=1tab @ 500mh TID making it 1500 not 3000mg which would be the daily dose from 6 not 3 tabs. This error should have been caught by the pharmacist, did you have a consult with the pharmacist when you filled the Rx? Get a copy of the Rx.

As stated in the link, approximately 13 children with sjs have lung involvement, contact SJSsupport. If you son had Bronchitis it is very important you get the genetic test for A1AD not the serum test because it is an acute reactant and you might receive a "Normal" test result, even variants may have more difficulty than the normal population and ATT affects more than lungs and liver disorders which we can discuss off line via PM.

At this point it is possible that there was some error in theRX, however, your son would have eventually reacted to something so it is better to know now so you can avoid other possible ADRs this may also mean avoiding high sulfur foods and adding some supplements that bind with sulfur metabolites and at least a natural vit E supplement. I will give you some more referals later. You were fortunate to receive the Dx so soon.

[retiredrn]

Thanks for your response. I may have misinformed you, my son has had bronchitis before, not chronic bronchitis. So the actual diagnosis was ear infection and the RX was correct because I asked the Dr about it.
I am familiar with the SJS foundation website, I appreciate the information re: The possible genetic link. my son currently takes a multivitamin daily and omega 3 caps, 2000mg.
Please let me know of anything else I could do for him. you have been very informative

thanks again

[rmet4nzkx]

Thanks for your response. I may have misinformed you, my son has had bronchitis before, not chronic bronchitis. So the actual diagnosis was ear infection and the RX was correct because I asked the Dr about it.
I am familiar with the SJS foundation website, I appreciate the information re: The possible genetic link. my son currently takes a multivitamin daily and omega 3 caps, 2000mg.
Please let me know of anything else I could do for him. you have been very informative

thanks again

ATT affects elastin, collegan, vW VIII & XIa, (66% action of thrombin) etc so you can see how a history of Bronchitis comes into play any child with history of lung or liver ailments should be tested and the testing is free, natural Vit E can help raise ATT levels in deficient individuals including varients so you are looking for about a 150 level if he is recovering. Cal mag & zinc, zinc TID helps bind the sulfur metabolites, limit high sulfur foods and soy products, If he is deficient he may qualify for Prolastin, also the SJSsupport has Refresh available from producer. Walnuts and flaxseed oil are good additions.

[rmet4nzkx]

The other antibiotics and also cause SJS.

[rmet4nzkx]

While this is an old thread, it seems appropriate to post this educational information about SJS and the support group mentioned in this thread.

Stevens Johnson Syndrome Foundation and Jean & Julie McCawley will be featured on the Montel Williams Show on Monday October 30th. Please check your local TV listings for channel and times.

[mstrsjff]

While this is an old thread, it seems appropriate to post this educational information about SJS and the support group mentioned in this thread.

Stevens Johnson Syndrome Foundation and Jean & Julie McCawley will be featured on the Montel Williams Show on Monday October 30th. Please check your local TV listings for channel and times.

This sounds like an interesting topic! I will check it out! Thanks Rmet!