You matter! Your voice matters!!

Tears may go unseen,
Your cry may not be heard...
Oh dear mothers, sisters
And darling wives..
Raise your voice,
And you sure will be heard!!

Let not fears crumble you down,
Let not timidity keep you away!
Shake them off and wake up,
Rise!! Today, Now!!

C'mon together we shall dream,
Together we shall work,
Together we shall build...
New rays of hope,
New wings of hope...

And then, sure shall come the days,
When our brothers, sons..
Sisters and daughters
And our dear love too...
Shall fly high in the sky!!

By-Dr.Anupama Pattiyeri 

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 !

The precious 32 pearls!!

Dental care -A very important thing in the lives of persons with hemophilia and other bleeding disorders.
Dental problems are a menace to hemophilics.
  How do I know that?  Well, I am a dental practitioner and my little brother is a person with hemophilia(Hemophilia A,severe). And yes,he did face difficulties due to dental problems.
  So, here are a few tips on how to take good care of your precious 32 pearls   and decrease the chances of dental problems. A few measures to be taken right from infancy.

• Proper nursing of infants: Seen yellowish to brownish - black front teeth of kids?? Those are teeth decayed due to improper feeding of breast milk/ formula.Breastfeeding is essential, but should be done in the proper way. Feeding at night just to make the baby stop crying and go to sleep is not a good idea. A pool of milk remains in the mouth and causes decay of the teeth. Also, take care to wipe clean the teeth and gumpads after feeding using a soft cloth.Remember milk teeth, though are shed as you grow, are very important for proper chewing of food and thus your overall health. Take good care of the 20 little pearls

• Daily home care: Brush twice a day, but not in a hurry, take a few minutes, covering all surfaces of teeth and don't forget to reach to the last tooth.Floss to clean the interdental areas, but don't force it through tight areas, and do not injure your gums. Clean your tongue and cheeks as well. Follow by a gentle massage of the gums.
• Avoid frequent snacking: Frequent snacking increases the risk of tooth decay multifold. Frequency matters a lot more than the amount consumed. Rinse after every snacking. Avoid sticky and sugary food. Sucrose is known as "arch criminal " because it causes tooth decay.If you just love sweets, try to have it at the end of your  meals.Include more fiber into your diet.
• Regular dental check up:Visit your dentist every 6 months.Early dental decay and other problems can be detected at an early stage and corrected by non - invasive or minimally invasive treatment methods.
•  Professional methods to prevent or reduce tooth decay: Your dentist can help you prevent decay to an extent by methods like Fluoride application, Pit and fissure sealants etc which are applied at specific ages corresponding to the eruption of teeth.

    Follow these easy and simple tips, keep your 32 pearls healthy and sparkling!!
A healthy, happy smile means a more confident you!!

----  Dr Anupama Pattiyeri & Team RedLegacy !