Dr. Frank Shafer

To: frank.shafer@drexel.edu
Subject: Noonan's conference

Dear Dr. Shafer,

Thank you so much for attending the Noonan Syndrome conference in Baltimore. There has been much discussion this morning on the net about how helpful "the blood guy" was. Your message was well received.

We spoke briefly after your presentation regarding the uses of Fibrin glue and DDAVP as they would apply to those with neurological disorders.

In brief, I wondered if the Fibrin glue might be useful in stopping the bleeding early on in a scratch or open small bite that a child might have. I thought if the bleeding were stopped and healing began more immediately, the child that might not have the obsession to continuely scratch therefore keeping the sore open might have a better chance of healing before the wound became more serious. This would be only used in those already have coagulation problems where wounds take longer to heal, etc.

I also questioned whether or not the DDVAP might be contraindicated with those individuals having seizure disorders.

Would you please respond in this forum so that I may pass the post along to the general NS online list? I didn't want to risk improperly quoting you.

I did understand, I think, that the Fibrin glue couldn't hurt in small scratches, etc, just as a trial run. Correct? I would like to see if it would help my daughter who has cronic heightened PT and PPT levels as well
as OCD. I think it might be worth a try if it runs no other risks other than a new sensation that might also be irritating.

Thank you for your interest in our children.

Martha Kate Downey

p.s. Is the Fibrin glue an over the counter medication? My pharmacist is not familiar with it. Brand name???

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From: "Shafer,Frank" <fs27@drexel.edu>
To: 'Martha Kate Downey' <mdo651@airmail.net>
Subject: RE: Noonan's conference
Date: Wed, 10 Nov 1999 07:36:41 -0500

Dear Ms Downey,

It was a pleasure to address your group. I am happy that I was able to provide some information regarding coagulation problems faced by individuals with Noonan Syndrome.

To try to answer your two questions:

1)Fibrin sealants - Purified forms of fibrin sealants have been available in Europe for 15 years. Their use in the US is not as widespread. These are usually freeze-dried products that contain virally inactivated human fibrinogen and human thrombin. The FDA approved the use of one of these products in 1998. Tisseel, produced by Baxter-Immuno in Vienna, Austria is marketed in the US by Baxter Healthcare and Haemacure Biotech. The indication is for patients undergoing coronary bypass surgery or who have bleeding from sites not controlled by sutures. Studies have been done using fibrin sealants in cardiac surgery and trauma. Sealants have also been used with dental procedures in hemophiliacs. There are ongoing clinical studies using other fibrin sealants (Baxter/American Red Cross (US); and Vitex (US)). This is a relatively new agent so clinicians may not be familiar with it and its uses.

2)DDAVP and the possibility of inducing hyponatremic seizures - DDAVP can be a very useful agent for individuals with most types of von Willebrand's Disease and some of the platelet function defects. Since it
is a potent antidiuretic agent fluid and electrolyte imbalances should be considered after its use. One way to avoid dilutional hyponatremia is to limit oral fluid intake to "maintenance fluids" for 24 hours after its
use. I have safely used DDAVP in patients with seizure disorders. We educate families regarding the appropriate usage of the drug and usually follow sodium levels at 24 hours after our "Stimate Challenge"

I hope this information is helpful.

Frank Shafer, MD

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