Inhibitors : leave the inhibition and know about it, you could be the next one

 Rohan/21/Male,  a known case of Hemophilia A, presented with severe bleed of the left knee.
Infused 1000IU of Factor VIII  and was discharged
He reported again the next day complaining of severe pain and swelling at the same site.
Infused factors according to his weight as per the dose chart. Gave maintenance doses too at the required intervals.
But the results were not as good as expected.
What could be the reason?
Is he deficient in factor VIII or some other factor?
But as per history, he used to respond well to Factor VIII.
 Has he developed inhibitors (antibodies)  to the Factor?
What should be done until we get an inhibitors screening done?
Should we continue infusing Factors because we are not sure of the inhibitor status or can we infuse inhibitor by-passing agent FEIBA or Activated Factor VII ?

Will it be a good idea to wait for results of screening tests in case of serious bleeds?
Does these agents have any detrimental effects if infused in persons without inhibitors?
Can we use them when we are in doubt?

If the bleeds are not getting better after infusion, it may be because you have not infused the required dose and not maintained the desired Factor level.
But if the response is not good even with infusion as per the dose chart, chances are high that you have developed antibodies / inhibitors against the factors.
You will have to use by -passing agents FEIBA or Activated Factor VII.

FEIBA ( Anti-inhibitor Coagulant Complex) can be used for control and prevention of bleeding episodes, perioperative management and routine prophylaxis in cases of Hemophilia A and Hemophilia B with inhibitors
should not exceed a dose of 100 units per kg body weight every 6 hours and daily doses of 200 units per kg body weight
Side effects :Thromboembolic events, Hypersensitivity and Allergic reactions , Blood-borne infections
Coagulation Factor VIIa Recombinant is intended for promoting hemostasis by activating the extrinsic pathway of the coagulation cascade.

It is indicated for treatment of bleeding episodes and prevention of bleeding during surgery in persons with Hemophilia A or B with inhibitors to Factors VIII or IX and in persons with Acquired Hemophilia
Treatment of bleeding episodes and prevention of bleeding during surgery in persons with Factor VII deficiency.

If you are not responding well to Factor therapy, inhibitors screening should be done immediately. However,  in extreme emergency, Factor VIIa may be used.
Inhibitors screening should be done by all persons with Hemophilia every 6 months.
In India, most of the centres which have facilities for aPTT  and Factor Assay do inhibitors screening too.
The major testing centres in  India  are:

AIIMS, New Delhi

Address: Ansari Nagar East, New Delhi, Delhi 110029
Phone:011 2658 8500

King Edward Memorial Hospital, Mumbai

Address: Acharya Donde Marg, Parel, Mumbai, Maharashtra 400012
Phone:022 2410 7000

Christian Medical College & Hospital, Vellore

Address: Bagayam, Thorapadi, Vellore, Tamil Nadu 632002
Phone: 0416 228 4255

St.John's Medical College , Bengaluru

Address: Sarjapur Road, Opp BDA Complex, Bengaluru, Karnataka 560034
Phone: 080 4946 6029

Apart from these most Hemophilia Societies and Hemophilia Treatment Centers conduct Inhibitors screening camps.

By

 Hemant Naidu Pulijala ,Dr.Anupama Pattiyeri &Team RedLegacy !