THE R YAL LINE

Hemophilia Without Disability, Children Free of Pain

Look beyond Hemophilia for a life full of success…

A periodic newsletter from Hemophilia Society Cochin Chapter

An

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March 2012

Featured in this edition:

“Royally” expensive!!!

Issue 1 24 pages (including cover)

You might be a PWH…You might know a PWH…

But do you know the factor that is lacking?Coming soon… Meet the different clotting factors in a way you have never seen before. Come and explore the complexities involved in every clotting protein… We take you in depth into your body (or

what’s not in your body…)

The Royal Line

Hemophilia Society Cochin Chapter is a non-profit, voluntary charitable organization established to

support people suffering from Hemophilia (PWH). We aim at both emotional and physical support for

those who need it, when they need it. We also provide medical attention in association with our

treatment centre –Medical Trust Hospital.

Revived in 2005, we have a current strength of 54 members, with hundreds left to be located. One of

the biggest challenges we face is the attitude itself. Many PWHs are not ready to come out and shout it

loud. They fear an isolation in the society, and hate sympathy more than anything. Again, if they can

afford the medication, the won’t feel the need of working with a charitable society. But what they don’t

know is that they are indirectly doing a big injustice to the economically backward PWH families, who

are struggling even for their daily bread!

Coming back to the chapter, we were lacking in the necessary manpower until the day of 11th Dec

2011, when the whole chapter was reorganized, with the active members at the top. This day also saw

the formation of the women and youth groups, to further strengthen the chapter.

The youth group primarily aims at empowerment and awareness creation. It also takes over the

activities of documentation and event planning from the chapter’s core groups. When we were

exploring different ways to spread the word about the disorder, the first thing that came to our mind is

the power of social networks. And to give more updates, we have given life to the newsletter – THE

ROYAL LINE.

Through this, we aim to make others aware of the plights faced by every PWH, and update them with

some information, which they may find interesting and useful. This should indirectly boost the

manpower for the chapter in the form of new volunteers.

On 11th Dec 2011, we had taken a pledge to make this chapter one worth noting. Right now, when we

are working on the newsletter, we feel that this will take our point across loud and clear.

-Editors

Hopefully, we will be successful in changing how the society perceives the disorder,

and make the world a better place to live for PWHs…

Hemophilia Society Cochin Chapter was "revived" in 2005 under Mrs. Syamala Ramaswamy, who was

previously in Trivandrum. During revival, the chapter was not in a good state. From then, it was the

single-handed efforts of Mrs. Syamala that brought the chapter up. A team consisting of a set of loyal

members were along with her in the process. But there is a huge manpower limitation that has been

hampering the activities.

Recently, there has been a tremendous improvement in the activity level of the chapter. The chapter got

affiliated to HEMOPHILIA FEDERATION (INDIA) at New Delhi. Youth and Women group have been

established who are taking care of the activities.

The people behind

• Dr.K.Hari - President

• Mrs.Syamala Ramaswamy - Secretary

• Mr.K.J.Joy - Treasurer

What we do

Our mission covers the following aspects:

• Medical support for PWHs

• Emotional support for PWHs and their families

• Psychological help for recovery & rehabilitation

• Awareness creation among general public

• Fund collection for subsidizing AHF

When we prioritize our tasks, we look for funding since the AHF should be made affordable to the

members. Only when this is done, we have to go for availability of AHF.

Excerpts from HFI’s website:

Established in 1983, Hemophilia Federation India is a self-help NGO run by Persons with Hemophilia

themselves, with help from medical fraternity. We represent India as National Member Organization at

the World Federation of Hemophilia based in Canada. We also work in close collaboration with World

Health Organization (WHO) and National Aids Control Organization (NACO).

We have a network of 72 chapters (48 affiliated and 24 Non-affiliated chapters) spread across India.

We are the only organization in India that provides structured training to our medical and paramedical

practitioners. We have two International Hemophilia Training Centers at CMC Vellore and KEM

Hospital Mumbai. Physiotherapy College in Pune trains physiotherapists for hemophilia care and St.

Stephens Hospital in Delhi trains laboratory technicians in running standardized hemophilia tests.

We have made WHO standardized diagnostic tests available to our PWH. In addition, all Chapters are

attached to diagnostic facilities locally. Our Special Needs Cell for HCV/ HIV positive PWH supports

them by providing lifelong subsidized treatment including Anti-Retroviral Therapy Drug.

We also have started National Hemophilia Registration (Record) to locate undiagnosed persons and

children with hemophilia. We have been able to update records of support to more than 16,000 Persons

with hemophilia in our national hemophilia records.

Hemophilia Society Cochin Chapter is affiliated to Hemophilia Federation (India),the national level body at New Delhi. It is in-turn under the World Federation ofHemophilia at Montreal. Established with a long-term vision to help PWHs aroundthe country, HFI has been the flagship organization leading the chapters under ittowards success.

Below, you will see what HFI is all about, from their own perspective and words...

In this edition

“Royally” expensiveHemophilia features in Forbes list ofmost expensive medical conditions.What is making this rarely seendisorder so hard-to-afford?

Pg 10

Hemophilia Society Cochin Chapter

Dr. P. V. Louis - Patron

Dr. K. Hari - President

Mrs.Syamala Ramaswamy- Secretary

Mr. K. J. Joy - Treasurer

Youth Group

Mahalingam.P.R

Benjoy K.J

Russel Mendez

Amal Suresh

The Royal Line

Concept - Mahalingam. P. R

Editors

- Mahalingam. P. R

- Benjoy K.J

Design & Illustrations

- Mahalingam. P. R

Medico Intro Pg 1

THE INCEPTION Pg 3

THE BEGINNING Pg 4

For those who are hearing it the

first time Pg 5

First aid in Hemophilia Pg 8

Continuous care in Hemophilia Pg 15

The

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Chapter Activity Pg 16

Contributors Pg 17

1

Hemophilia refers to a group of bleeding disorders in which it takes a long time for the blood to clot. It

is a rare bleeding disorder, and if you have Hemophilia, you may bleed for a longer time than others

after an injury. You may also bleed inside your body (internally), especially in your knees, ankles and

elbows. This bleeding can damage your organs and tissues, and may be life threatening.

Causes and Risk factors

When you bleed, the body launches a series of reactions that help the blood clot. This is called a

coagulation cascade. This process involved special proteins called coagulation factors. When one or

more of these clotting factors are missing, there is usually a higher chance of bleeding.

Hemophilia is commonly due to a lack of enough factor VIII or IX (other factors numbered I, II, V, X

and XIII are also present, but less in number compared to VIII and IX). In most cases, Hemophilia is

hereditary. ie, it is passed on from parents to children down the family line through defective genes. It

commonly affects males.

The clotting proteins work with the platelets to help the blood clot. Platelets are small blood cell

fragments that form in the bone marrow – a sponge-like tissue in the bones. Platelets play a major role

in blood clotting. When blood vessels are injured, clotting factors help platelets stick together to plug

cuts and breaks on the vessels and stop bleeding.

As mentioned above, two deficient factors are predominant in hemophilics. They are denoted as

Hemophilia A and Hemophilia B. If you have Hemophilia A, you’re missing or have low levels of

clotting factor VIII. About 9 out of 10 hemophilics are of type A. If you have Hemophilia B, you’re

missing or have low levels of factor IX.

Rarely, Hemophilia can be acquired. “Acquired” means that you are not born with this disorder, but

you develop it during your lifetime. This can happen if your body forms antibodies (proteins) that

attack the clotting factors in your bloodstream. The antibodies can prevent the clotting factors from

working.

Symptoms & Bleeds

The main symptom of Hemophilia is bleeding itself. Mild cases my go unnoticed until later in life, when

they occur during surgery or after trauma. In more severe cases, serious bleeding may occur without

any cause. Internal bleeding may occur anywhere. But bleeding into joints is most common.

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Dr. K. Hari, Physician at Medical TrustHospital, Cochin, and President ofHemophilia Society Cochin Chapter gives amedical outlook into the disorder. He hasyears of experience working withHemophilia, and is one of the most seniorconsultants in Medical Trust Hospital.

2

The extent of bleeding depends on how severe the hemophilia is. Children who have mild hemophilia

may not have signs unless they have excessive bleeding from a dental procedure, an accident, or

surgery. Bleeding can occur on the body’s surface (external bleeding) or inside the body (internal

bleeding).

Signs of external bleeding may include:

• Bleeding in the mouth from a cut or bite, or from cutting or losing a tooth.

• Nose bleeds for no obvious reason

• Heavy bleeding from a minor cut

• Bleeding from a cut that resumes after stopping for a short time

Signs of internal injury may include:

• Blood in the urine from bleeding in the kidneys or bladder

• Blood in the stool from bleeding in the intestines or stomach

• Large bruises from bleeding into the large muscles of the body

Signs & Tests

Most often, hemophilia is diagnosed after a person has an abnormal bleeding episode, or when there is

a known family history of the condition. Blood tests to detect the protein levels of VIII and IX are

usually done, and then a person is classified as mild or severe (there are moderate hemophilics also,

which lie between mild and severe).

Treatment

Standard treatment involves replacing the missing clotting factor through a vein (intravenous

infusions). Diagnosing a bleeding disorder is important so that the doctor can take extra care if you

need surgery, and can test or warn other family members who might be affected.

The main treatment for hemophilia is called replacement therapy. Concentrates of clotting factors are

slowly dripped or injected into a vein. These infusions help replace the clotting factor that’s missing or

low. Clotting factor concentrates can be made from human blood. The blood is treated to prevent the

spread of diseases, such as Hepatitis. With the current methods of screening, the chances of contracting

a blood-borne infection is very low. To further reduce the risk, clotting factor concentrates are make

from non-blood sources. They are called recombinant clotting factors. They are easy to store, mix and

use at home, and takes only about 15 minutes to administer.

Replacement therapy can be done regularly to prevent bleeding, called preventive or prophylactic

therapy. Otherwise, replacement therapy can be done to stop bleeding as it occurs (as-needed basis).

This is called demand therapy. Demand therapy is less intensive, and cheaper than preventive therapy.

However, there is a risk that bleeding will cause damage before you receive the demand therapy.

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-Dr. K. Hari

Benj

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3

Hai Readers, Introducing myself, I am

Benjoy, doing M.com at MG university.

Moreover a member of Haemophilia society

Cochin chapter`s youthwing & co-editor of

Royalline. Myself and my friends,

Mr.Mahalingam - the author & co-editor of

Royal line & Mr.Russel Mendez are

haemophilics. There are many more like us in

our society. But only a few of us fall under the

youth category. An year before, we rarely met

only when a formal meeting was conducted

by society & we knew ourselves only as being

the same Royal, that’s all. But as time passed,

we realised that it would be better to form a

group of royals & thereby share our

experiences, knowledge & do something

creatively for all haemophilics. By keeping

this as our basic aim, we formed the Youth

Wing under HSCC, a couple of months back

Now, let’s discuss about the relevance &

importance of youthwing & the purpose of

this magazine .I guess, many of you know

what haemophilia is. As you have already

read in the magazine, it is a rare, expensive &

dangerous disease. From our life’s experience

of fighting with haemophilia, we all have

realised & admired at one point that, in

today’s competitive world, without sufficient

knowledge, attitude & perfect planning, a

disease like haemophilia will be extremely

hard to survive, I mean physically, mentally &

financially.

Let us consider the knowledge aspect first.

Knowledge of this disease plays the most

important part. It stands as a pillar to attitude

& proper planning. Let me illustrate this with

an example. Just imagine, ten glasses of juice,

of which two are poisoned and you got no

idea on the poisoned ones. What would you

do? Definitely, you will ignore all ten glasses,

out of fear. But remember ,out of it ,eight

glasses are good to taste & enjoy. What if u

got the right knowledge about the glasses

which you should avoid. It is sure that you

won’t have fear & can utilise & enjoy the

other eight. The life of a haemophilic is like

that. There are many things, attitudes, the way

of doing things, thoughts which may be

hazardous to a haemophilic in many ways.

And the same life also offers things which is

essential & good to have. As long as you don’t

have ample knowledge of this royal disease,

you may be forced to ignore the good things

too, that may suit you & may have been

achieved by you ,to lead a better life .It is a

universally accepted truth that ― knowledge is

the ultimate power”, as it helps you to

picture your life with a perfect planning &

thereby develop a right attitude to everything.

Through this magazine & youthwing ,we

intend to improve the above mentioned

knowledge aspect, from our own learning &

experiences & thereby shape the existing &

upcoming royal generations in all means

,most importantly in wisdom

Let hope the Royal line will serve the real

purpose for what it has been framed.

Wel

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The Inception

This is the first edition of the newsletter. Lets start it off with a few

words from the editors. Benjoy, one of the editors of the periodical,

is an active member of the group, and shares a lot of innovative

ideas. He will be telling more about himself in the following

welcome atticle…

Mah

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When the youth group was initiated on 11th

December 2011, there was only one thing in

my mind – to make this group a force to

reckon with. So, innovation was on top of my

list. I wanted to take steps where others have

rarely taken, and come out on top.

Thankfully, I got a good teammate in

Mr.Benjoy, who has stood by my ideas and

suggested a lot to make it better. The idea of a

magazine itself started off like that.

Every time I get a copy of the Hemophilia

World or Hemophilia News, I used to realize

how much we can benefit from knowledge

sharing. A magazine would be an easy way to

disburse the updates in the field of

hemophilia-related research. I also thought

that I can add real-life experiences from

people around the world, that can inspire

others when they feel they are down.

Finally, one day I couldn’t take it anymore. I

had to get this done, and that too, fast. I have

experience writing blogs, through which I am

telling the world what I am going through.

You can find the blog at

http://prmahalingam.wordpress.com/

This gave a head start to the idea, and in no

time, the design was on. Throughout the

design process, Benjoy has given me valuable

feedback regarding the layout and

backgrounds. He has played a commendable

role in helping me organize the whole thing in

just a week.

This is a proud moment for me, as I type

inside my own design, and telling you all

what I have been doing. But I would surely

say that all these have been possible only

through the hardwork of the whole team.

I also would like to thank all those who

helped me select the initial topics, and

illustrate them within the short time frame.

Hope you would enjoy going through the

newsletter and give your suggestions.

You can expect a variety of interesting

materials in this issue, and the upcoming ones,

which are carefully chosen to provide a mix

of experience, technology and possibly, a bit

of entertainment.

So, simply read on!

Wel

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The Beginning

The author is a PWH himself, suffering from Hemophilia A (severe

category). He is currently pursuing his Masters in Technology from

Rajagiri School of Engineering & Technology, Cochin. After

forming the Youth Group in Dec 2011, he has been innovating ideas

for making it a group worth noting…

5

In layman terms, Hemophilia is a medical

condition in which the blood fails to clot. Inmore medical terms, a certain ―clottingfactor‖ is missing in the blood, due to whichthe clot that is formed during the bleed,simply dislodges and disintegrates.

Now, the condition might look quite simplewhen you hear it the first time. But there arethings that lie hidden in that definition.Bumps and scrapes are a part of every child’slife. For most kids, a tumble off a bike or astray kick in a soccer game means atemporary bruise or a healing scab. However,for kids with hemophilia, these normaltraumas of childhood are reason for extraconcern.

Clotting factors

Human blood contains special proteins,known as clotting factors. Identified byRoman numerals, clotting factors help stopbleeding and allow a blood vessel to heal afteran injury. The last step in the clotting process(also called coagulation) is the creation of a―net‖ that closes the torn blood vessel andstops the bleeding. This part of the processinvolves clotting factors VIII and IX. Peoplewith hemophilia are deficient in one of thosefactors due to their abnormal genes and, as aresult, their blood can’t clot properly.

Hemophilia A, also known as factor VIII

deficiency, is the cause of about 80% of cases.

Hemophilia B, which makes up the majority

of the remaining 20% of cases, is a deficiency

of factor IX. Patients are classified as mild,

moderate, or severe, based on the amount of

factor present in the blood.

A patient whose blood tests suggest severe

hemophilia will usually bleed frequently,

whereas another patient with a milder form

will usually bleed only rarely. However, there

is a range of severity within each group. The

reasons for this variability may relate to other

clotting factors or to differences in behaviours

that present different risks of injury.

Signs and symptoms of hemophilia vary,

depending on severity of the factor deficiency

and the location of the bleeding. Few babies

are diagnosed with hemophilia within the first

6 months of life because they’re unlikely to

sustain an injury that would lead to bleeding.

For example, only about 30% of males with

hemophilia bleed excessively when

circumcised and only 1% to 2% of newborns

with hemophilia have bleeding within the

skull (called an intracranial hemorrhage).

Art

icle

“Hemophilia”?Never heard of it!

This is a statement which we have encountered quite a lot. Most people have limited, or no

knowledge of the disorder, and are unaware of the threat it poses. “Awareness creation” was

one of the primary aims in mind when we thought of the magazine. This section is dedicated

for people who are yet to realize the threats posed by Hemophilia, and what they can do to

help the needy.

6

Although hemophilia is a lifelong condition

with no cure (other than liver transplantation),

it can be successfully managed with clotting

factor replacement therapy — periodic

infusions of the deficient clotting factor into

the child’s bloodstream. Factor replacement

may be given through an intravenous (IV) line

either at the hematology clinic or at home by

a visiting nurse or by parents (and even older

patients) who have undergone special

training. Once the clotting factor is ―infused,‖

it begins to work quickly and helps prevent

joint damage.

Factor replacement may begiven through anintravenous (IV) lineEven with all this, there is little awareness

about the disorder. In western countries, a fair

amount of advocacy is present, which takes

the name high up in the hierarchy, both

political and medical.

But when we consider the situation here, it is

much different. At this point we are moving

back to the title itself…

“Hemophilia”? Never heardof it! Whats that???

The question might look funny in the

beginning. But once we realize who the asker

is, the gravity of the situation becomes clear.

Art

icle

Hemophilia is primarily a hereditary

condition. But nowadays, more cases of

spontaneous mutation are rising, which

creates more mystery of how it is actually

caused.

The genetic transmission occurs because a

critical blood clotting gene is carried on the X

chromosome. Since males only carry one X

chromosome, if that is defective, hemophilia

will immediately show up. An early death is

likely. Females, on the other hand, carry two

X chromosomes. If only one is defective, the

other normal X chromosome can compensate.

The woman will have normal blood clotting;

she will simply be a carrier of the recessive

defective gene. This fact will be discovered if

some of her children are hemophiliacs.

Naturally, women hemophiliacs are rare

because it takes two defective X

chromosomes in order for the condition to be

seen.

This is how it can show up in a family tree.

7

Why the concern?

The situation is much worse in India than that

compared to other western countries. When

we go back to the title, we have to realize that

it is the doctors who are asking that question.

Doctors are having littleinformation about thedisorder!The problem lies in the basic training itself.

Here, only the hematologists and oncologists

are involved in hemophilia-related training.

For the others, it is as simple as a small

passage. So, they have insufficient

information regarding the kind of treatment to

be undertaken in case of emergency.

So, the duty falls on the shoulders of the

affected. They have to ―educate‖ the doctor

about the condition before getting the

treatment. The main issue is that many pain-

relievers like aspirin actually aggravate the

bleeding!

Drugs like Aspirin andIbuprofen can aggravatethe bleeding.The same issue is faced by the patients

themselves. This is since they themselves

have to avoid these drugs even in the utmost

emergency.

The most effective method to treat the bleeds

is to use Anti-Hemophilic Factor (AHF),

which can be expensive.

Common misconceptions

Now, there are a lot of misconceptions when it

comes to the disorder.

It is our duty to clarify themisconceptions and makethe public understand thereal deal!• Hemophilics can bleed anywhere in the

body, including the brain (IC bleed)

• Joints are the most affected part of the

body, since prolonged bleeding can cause the

tissue surrounding the joint to break down and

cause permanent damage, leading to

disability.

• Hemophilia’s effect isn’t limited simply

to the outer skin. Any part of the body that has

blood flow can bleed, and the blood can build

up internally, leading to further damage. Art

icle

8

Note: The following noteapplies only to joint bleeds,and any other bleed sitemight react differently!The first aid for joint bleeds is abbreviated as

RICE. RICE stands for

• Rest

• Ice

• Compression

• Elevation

Once first aid is given, the patient has to be

monitored, and if the pain doesn’t subside,

factor replacement therapy might be needed.

Rest: The bleed spot should get enough rest.

Immobilize the joint immediately so that the

tissue doesn’t get stressed, and aggravate the

bleed.

Ice: Apply ice packing. Ice increases the

viscosity (thickness) of the blood, and slows it

down. The reduced flow will slow the

bleeding down. Also, the drop in temperature

will freeze the nerves and relieve pain.

Compression: Use crepe bandage to secure

the bleeding joint. This tightens the blood

vessels and restricts the blood flow. It also

restricts movement and avoids further

damage.

Elevation: Keep the joint in an elevated

position. This avoids too much blood from

going to the affected area, and controls

bleeding.

Art

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First Aid in Hemophilia

Even if Hemophilia can get serious in many cases, with quick action, the damage can be

minimized. The first aid for bleeds are critical in the eventual outcome. Here, we will see

what is to be done as a first aid for internal bleeds.

The Royal Line

10

When we hear the name ―Royal Disease‖,

we feel kind of proud. This is since thedisease is largely hereditary, and if a royalbloodline had this disorder, we might actuallybe part of it!

But recent studies have established that thedisorder can arise due to spontaneousmutations also. So, a person with Hemophiliamay not actually be part of the royalbloodline. But still, all of us considerourselves as ―royals‖…

While this has been a matter of personal pridefor many, there is a big burden working in thebackground. The name ―Royal Disease‖ haschanged its meaning over time, from found inroyal bloodline to one which can be affordedonly by people in a royal bloodline. This itselftells the impact it has on the financial status ofpeople.

The meaning has nowchanged to “one which canbe afforded only by peoplein a royal bloodline”

This is precisely what Forbes magazine has

said. It has published a list of the 10 most

expensive medical conditions. The list goes as

follows.

1. HIV $25,000

2. Cancer $49,000

3. Transplant $51,000

4. Stroke $61,000

5. Hemophilia $62,000

6. Heart Attack including Cardiac

Revascularization (Angioplasty with or

without Stent) $72,000

7. Coronary Artery Disease $75,000

8. Neonate (premature baby) with extreme

problems $101,000

9. End-Stage Renal Disease $173,000

10. Respiratory Failure on Ventilator

$314,000

Now, things get obvious.

Feat

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“Royally Expensive”

Hemophilia is popularly called the “Royal Disease”. The name primarily originated due to its

presence in the British and Russian kingdom hierarchy. But in this century also, the name

still holds. But it is not due to the kingship, but the cost involved. Nowadays, it has come to

a condition where only people with royal background can afford it. In this feature, we

examine why the name is still relevant.

11

You can see that, at $62,000 per year,

Hemophilia scores above other medical

conditions like Stroke, Transplants, HIV and

even Cancer! This is contrary to popular

belief that cancer is one of the most

expensive. Even if not that common, there are

a lot other disorders that burn a deeper hole in

the patients’ pockets.

The situation is not at al different in India. To

add to that, Hemophilia is not covered under

any medical insurance here, whereas some

western countries provide such support. So,

every last drop of medicine has to be

―bought‖. When we calculate the medical

cost, the cost of AHF (Anti-Hemophilic

Factor) outweighs the complete diagnosis and

hospitalization cost by a factor that can’t even

be estimated! Even if AHF is a short-term

medication, it demands more from the patient

than the complete course of supplementing

medication.

In India, AHF is imported from countries like

USA (Baxter, Bayer), Italy (Kedrion), etc. The

availability of AHF is limited by the stringent

standards imposed on the manufacturing and

sterilization.

AHF is a blood-based product. It is made up

of clotting factors extracted from human

blood, which is donated by a pool of donors

worldwide. Since the blood is natural, there

might be a variety of components that might

make the drug unfit for use. As a matter of

fact, during the 1980s’, millions of PWHs in

USA and Canada were affected by blood-

borne diseases, including HIV, which was

transmitted through the medicine.

One that was brought to light, more stringent

measures were adopted to make AHF safer,

and fit for use. So, AHF was brought out as a

dried, sterilized preparation, which removes a

lot of the infectious components. Nowadays,

the AHF is tested for a variety of infectious

components like HIV, BVDV, HCV, PRV,

HBV, CPV, B19V and HAV. Once that is

complete, the AHF is maintained in a

Albumin base.

All these take a lot of time and effort, and

pushed up the cost of manufacturing by a

huge margin. Accounting for all these, the

AHF costs around Rs.3750 for a minimal

dose.

AHF is brought out not in terms of

conventional volume, but as IU (an

internationally accepted unit of

measurement). In India, the open market rate

per IU of AHF comes to around Rs.15. So,

you can infer the amount to be spent in case

of a bleed.

Now, the dosage depends on the level of

factor present in the body, the required rate of

increase and the weight of the person.

Depending on the level of factor available in

the body, PWHs are classified into 3

categories:

Severe (<1% available)

Moderate (1-10% available)

Mild (>10% available)

The fall in availability of AHF is primarily

due to the low liver activity, which results

from the genetic deficiency. For the severe

category, even the most ―simple‖ bumps and

bruises can lead to a torrential bleed episode,

which may require factor replacement. Also,

the level of factor increase depends on the

type and location of bleed.

Feat

ure

Hemorrhage

Type of hemorrhage Required peak post-infusion

AHF activity in blood

Frequency of infusion

Early hemarthrosis or muscle

bleed or oral bleed

20-40 12/24 hours for one-three days,

until bleeding arrested, or resolved

More extensive hemarthrosis,

muscle bleed or hematoma

30-60 12/24 hours for three days, until

resolved

Life threatening bleeds like head

injury, throat bleed, severe

abdominal pain

60-100 8/24 hours until healed

Surgery

Type of operation

Minor surgery, including tooth

extraction

60-80 Single infusion + oral anti-

fibrinolytic therapy

Major surgery 80-100 (pre & post-operative) 8/24 hours, depending on healing

12

So, you can see that there might be multiple

doses required for a single bleed episode. But

before deciding the frequency, the dosage per

administration has to be determined.

The dosage required is calculated like:

So, for example, a peak level of 70% required

for a child of 40 kg, the dosage would be 1400

IU. If it has to be repeated once every 12

hours, the daily dosage would be 2800 IU.

The cost then goes to Rs.42000!

Also, the half life of the medication is just

around 14.8 + 3.0 hours. So, the effect of the

1400 IU will go down to 700 IU (35%

effective) after 15 hours, and to 350 IU

(17.5% effect) after 30 hours. So, proper

supplementation has to be provided in terms

of subsequent dosages, so that the level is

maintained at 70%.

Now, there are variants of Hemophilia like

Hemophilia A (Factor VIII), Hemophilia B

(Factor IX), Factor V, Factor X, Factor XIII

and so on. For each variant, the medication

will be different, and costs will vary.

And there is another problem faced by PWHs.

If the AHF dosage becomes too high, the

immune system of the body might react, and

produce antibodies, called Inhibitors. In that

case, there is a specialized drug called

FEIBA, that contains activated agents to

bypass the inhibitors. But for that, the cost is

many fold, going up to Rs.24,000 for 500 IU.

An alternative is NovoSevenTM , which is also

as expensive as FEIBA.

Adding all this, the cost incurred by an

average family with hemophiliacs can be

inferred. This will take a severe toll on them,

and in cases where there are multiple PWHs

in the same family (which is also getting

common these days), the condition worsens.

Feat

ure

So, the disease truly becomes a “Royal Disease”!Thinking in the financial sense, Hemophilia can make even a royal family a ―Below Poverty Line‖

family, unless dealt with properly.

13

Mr. Jithin Jose is just another person in a

suburb in Cochin for many. But for people

like us, he is an inspiration by all means. As a

fellow royal, he has faced all the hardships

like everyone else among us. But what sets

him apart from us is that he has gone to the

extreme of it, and stays there with a smile on

his face!

Jithin was like all other royals initially. He

had a childhood like all others, and had

occasional bleeds like others. Diagnosed with

Hemophilia A, bruises and bleeds were a part

of his life from the very beginning.

He was not the kind of a person who simply

took AHF for every other bleed. He took

decisions judiciously, and chose when he

wanted a dose of AHF. But still, there was a

fair amount of infusions needed.

Once, when down with haematuria, he had to

take more AHF than usual to arrest the bleed.

Since his father could reimburse the medical

expenses, finance was not a big burden. But

for subsequent bleeding episodes, this was not

the case. The bleed failed to come down

despite repeated infusions of AHF.

Finally, during a physiotherapy session in

CMCH, Vellore, they confirmed the suspicion

through an assay. They reported that his body

had developed inhibitors (antibodies) to the

AHF, and regular infusions won’t work on

him anymore. His only option was to either

double the dose of AHF (which can in turn

push the antibody levels up), or switch to the

activated AHF, called FEIBA.

Rea

l-lif

e st

ory

Impossibility – A myth

Following are the excerpts from a visit by Mrs. Syamala Ramaswamy and Mr.

Mahalingam.P.R to the house of Mr. Jithin Jose, a PWH with Severe Hemophilia A and

Inhibitors. There we had a first hand account of the hardships faced by a person, and

realized how lucky and comfortable we are compared to him.

This is just a short account, and we might bring out more elaborate versions soon…

14

After finishing school, he joined for B.Com,

which he could attend only for the first day.

Still, he continued studying at home, and

wrote exams. And now, he is writing exams

for CA (Chartered Accountant).

The real scale of hissuccess comes only now.Despite all this persistent issues, he scored the

42nd rank in the country in the INTER exams

for CA. This shows the world that you needn’t

be physically the best to achieve success. He

couldn’t attend subsequent exams due to

bleeds, and right now, he is preparing for the

final round of exams (which fall in May).

Rea

l-lif

e st

ory

To add to that, they also confirmed the

presence of HCV (Hepatitis C), which he

might have contracted during a earlier

infusion of blood (to balance the hemoglobin

levels).

Hemophilia, Inhibitors andHCV don’t form quite agood combination.HCV, even if dormant, still poses a threat

since Hemophilia itself, which originates from

a liver-related genetic change, can cause the

virus to turn worse. But treatment is not that

easy and effective.

After all this, he had a fall, which dislocated

his left knee cap, and caused a joint problem.

As a result of that, that leg stopped growing

along with the other, and now, his left leg is

shorter than his right. So, when he tries to

walk, the hip joint gets stressed, and causes

problems.

All this has weakened his calf muscles, and he

is totally bed-ridden. When he tries to walk,

the hip joints cause problems, and the legs

can’t take the weight of the body. (Even when

we were there, he had a bleeding episode in

his left arm). Frequent bleeds also have

caused range restriction in both arms.

But studies are differentfrom disability!He had to stop going to school at 3rd standard

itself. But he studied from home, and passed

the exams with flying colors. He also gets 45

minutes extra time for exams.

An endnote

-Mahalingam.P.R

When we entered his room, he received us

with a smile on his face. The smile says a lot.

He is staring fate in the eye, and moving

forward.

When I met him, at the first glance itself, I got

reminded of Stephen Hawking – the man who

rules the science world from his wheel chair.

Jithin’s body may not be helping him, but his

mind is clear and sharp enough to tackle and

cut through any obstacles that is put in front of

him.

With confidence backing him, how can he

lose???

His situation will be an inspiration for all the

people, whether they are hemophilic or not.

The next time you think your life is miserable,

just think of him, and you can understand how

comfortable your life is, and how can sail

above it…

15

If you have hemophilia, you can take steps to avoid complications. Some are:

• Follow your treatment plan exactly as your doctor prescribes.

• Have regular checkups and vaccinations as recommended.

• Tell all your healthcare providers – doctor, dentist and pharmacist – that you have

hemophilia. You may also want to tell people like your employee health nurse, gym trainer,

and sports coach about your condition.

• Have regular dental care. Dentists at the treatment centers are experts in providing

dental care for PWHs. If you consult another dentist, tell him/her that you have hemophilia.

The dentist can provide medicine that will reduce bleeding during dental procedures.

• Know the signs and symptoms of bleeding in joints and other parts of the body. Know

when to call the doctor or go to the emergency room. For example, you’ll need care if you

have:

– A heavy bleeding that can’t be stopped, or a wound that continues to ooze blood.

– Any signs or symptoms of bleeding in the brain. Such bleeds are life threatening, and

requires emergency care.

– Limited movement, pain, or swelling of any joint.

It’s a good idea to keep a record of all previous treatments. Be sure to take this information

with you to medical appointments and to the hospital or emergency room.

Hem

op

hili

a C

are

Continuous care in Hemophilia

The Kremlin Konnection

The British royal family transferred the Hemophilia gene to German andRussian royal bloodlines.

But what went horriblywrong in Russia!?What led to the fataltwist of events???

- Dr. K. Hari

16

After the resurrection in December 2011, Hemophilia Society Cochin Chapter has been

abuzz with activity. With the financial condition not at its best, the chapter has been

working on raising funds in all circles. Corporate organizations were approached, and we

managed to collect a considerable amount of money. Using this fund, we were able to

subsidize a good amount of AHF for those who couldn’t afford it.

The resurrection was literal in all ways„ New members came into the group, and were

given positions depending on how active they are willing to be. New ideas on fund raising

are coming up, and with active members in the front, we are sure of a successful future„

Ch

apte

r ac

tivi

ty

AHF subsidized: 3275 IU (worth Rs.32750/-)

Spread the word…We are on Facebook…http://facebook.com/HemoYouthCochin

Donate generously…Help us to your capacity… Donations can be made as Cash / Cheque / Demand DraftIf Cheque / DD, they can be issued favoring Hemophilia Society Cochin Chapter, payable at Cochin…

Want IT exemption???

Donate via HFI… Issue Cheques / DD favoringHemophilia Federation (India) payable at New Delhi…Donations will have 100% IT exemption under Section 35AC.

Cheques / DD can be mailed to the chapter’s address given in the last page…

17

Co

ntr

ibu

tio

ns

We would like to express our endless thanks to those who contributed to lift the chapter from rough

seas„

ContributionsStudents of M.Tech CSESIS, RSETMrs. Roopa MenonMr. Homi P UstadStaff of HLL Lifecare LtdMr. Madhava PriyanMr. Narayan ShankarM/S Mediatronix Pvt LtdMr. K Gireendara BabuMrs. Serena MathewM/S Destination HealthM/S Basheer & ShahidM/S Cannanore HandloomsMr. Sathyan C SMrs. Padma BalasubramaniamMr. Vipin AntonyMrs. Sinna P JMr. SreenivasM/S AryabhangyM/S CSD Adenwala TrustM/S Cochin RubbersMr. Sreeraj Choorakkad

Students from M.Tech CSESIS, RSET

Mr. Aravind Krishnan RMrs. Arifa AzeezMrs. Aswathy M CMrs. Dhanya SudarsanMrs. Geethu ThomasMs. Geethu WilsonMs. Jeeva Susan JacobMs. Josna JosephMrs. Lithewmol MathewMs. Merin SebastianMrs. Neethu MohandasMrs. Nithya JoyMs. Saranya D KrishnanMrs. Soumya Alias

Staff of HLLLifecare Ltd

Mr. KesavanMrs. SreekumariMs. Jemi RajuMr. AnoopMs. AthiraMr. Raghul CMs. Junitha C RMs. Sindhu CMr. Jayesh KumarMr. Harish TMs. Nejj ThomasMr. Manoj

Hemophilia Society Cochin ChapterFlat – A, Ground Floor, Thripthi Apartments,

Near Agasthya Medical Centre, Old Bus Stand, Tripunithura, Cochin – 682301

Phone: +91-484-27740838547988083 (Syamala Ramaswamy)

9895243239 (Mahalingam.P.R)9349315905 (Ramaswamy.P.M)

Mail:cochin@hemophilia.in

hemoyouth.cochin@gmail.comWeb: http://hemophilia-cochin.co.nr/

With a membership strength of 54, Hemophilia Society Cochin Chapter was established in 2005 for supporting PWHs in and around the city of

Cochin, including the suburbs.

We have the following mission to fight out…•Provide medical assistance•Emotional support for the needy•Give provisions for adequate financial support

Affiliated to Hemophilia Federation (India)www.hemophilia.in