The quarterly magazine of Hemophilia Society Cochin Chapter... An initiative of Youth Group Edition: January-March 2012

1. March 2012
THE R YAL LINE
A periodic newsletter from Hemophilia Society Cochin Chapter
An initiative of Youth Group, Hemophilia Society Cochin Chapter
Featured in this edition:
“Royally”
expensive!!!
Hemophilia Without Disability, Children Free of Pain
Look beyond Hemophilia for a life full of success…
Issue 1 24 pages (including cover)

2. 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

3. 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…

4. 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.

5. 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 of
Hemophilia at Montreal. Established with a long-term vision to help PWHs around
the country, HFI has been the flagship organization leading the chapters under it
towards success.
Below, you will see what HFI is all about, from their own perspective and words...
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.

6. In this edition
Hemophilia Society Cochin Chapter
Dr. P. V. Louis - Patron
Dr. K. Hari - President
“Royally” expensive Mrs.Syamala Ramaswamy- Secretary
Hemophilia features in Forbes list of Mr. K. J. Joy - Treasurer
most expensive medical conditions.
What is making this rarely seen Youth Group
disorder so hard-to-afford?
Pg 10 Mahalingam.P.R
Benjoy K.J
Medico Intro Pg 1 Russel Mendez
THE INCEPTION Pg 3 Amal Suresh
THE BEGINNING Pg 4
For those who are hearing it the
first time Pg 5 The Royal Line
First aid in Hemophilia Pg 8
Continuous care in Hemophilia Pg 15 Concept - Mahalingam. P. R
The story of an
Editors
achiever…
- Mahalingam. P. R
- Benjoy K.J
Design & Illustrations
Pg 13 - Mahalingam. P. R
Chapter Activity Pg 16
Contributors Pg 17

7. Dr. K. Hari, Physician at Medical Trust
Hospital, Cochin, and President of
Hemophilia Society Cochin Chapter gives a
medical outlook into the disorder. He has
years of experience working with
Hemophilia, and is one of the most senior
consultants in Medical Trust Hospital.
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
Introduction by the President
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.
1

8. 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
Introduction by the President
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.
-Dr. K. Hari
2

9. 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,
Benjoy K.J
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…
Knowledge of this disease plays the most
Hai Readers, Introducing myself, I am important part. It stands as a pillar to attitude
Benjoy, doing M.com at MG university. & proper planning. Let me illustrate this with
Welcome from the Editorial Team
Moreover a member of Haemophilia society an example. Just imagine, ten glasses of juice,
Cochin chapter`s youthwing & co-editor of of which two are poisoned and you got no
Royalline. Myself and my friends, idea on the poisoned ones. What would you
Mr.Mahalingam - the author & co-editor of do? Definitely, you will ignore all ten glasses,
Royal line & Mr.Russel Mendez are out of fear. But remember ,out of it ,eight
haemophilics. There are many more like us in glasses are good to taste & enjoy. What if u
our society. But only a few of us fall under the got the right knowledge about the glasses
youth category. An year before, we rarely met which you should avoid. It is sure that you
only when a formal meeting was conducted won’t have fear & can utilise & enjoy the
by society & we knew ourselves only as being other eight. The life of a haemophilic is like
the same Royal, that’s all. But as time passed, that. There are many things, attitudes, the way
we realised that it would be better to form a of doing things, thoughts which may be
group of royals & thereby share our hazardous to a haemophilic in many ways.
experiences, knowledge & do something And the same life also offers things which is
creatively for all haemophilics. By keeping essential & good to have. As long as you don’t
this as our basic aim, we formed the Youth have ample knowledge of this royal disease,
Wing under HSCC, a couple of months back you may be forced to ignore the good things
Now, let’s discuss about the relevance & too, that may suit you & may have been
importance of youthwing & the purpose of achieved by you ,to lead a better life .It is a
this magazine .I guess, many of you know universally accepted truth that ― knowledge is
what haemophilia is. As you have already the ultimate power”, as it helps you to
read in the magazine, it is a rare, expensive & picture your life with a perfect planning &
dangerous disease. From our life’s experience thereby develop a right attitude to everything.
of fighting with haemophilia, we all have Through this magazine & youthwing ,we
realised & admired at one point that, in intend to improve the above mentioned
today’s competitive world, without sufficient knowledge aspect, from our own learning &
knowledge, attitude & perfect planning, a experiences & thereby shape the existing &
disease like haemophilia will be extremely upcoming royal generations in all means
hard to survive, I mean physically, mentally & ,most importantly in wisdom
financially. Let hope the Royal line will serve the real
Let us consider the knowledge aspect first. purpose for what it has been framed.
3

10. The Beginning
The author is a PWH himself, suffering from Hemophilia A (severe
Mahalingam P.R
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…
This gave a head start to the idea, and in no
When the youth group was initiated on 11 th
time, the design was on. Throughout the
Welcome from the Editorial Team
December 2011, there was only one thing in design process, Benjoy has given me valuable
my mind – to make this group a force to feedback regarding the layout and
reckon with. So, innovation was on top of my backgrounds. He has played a commendable
list. I wanted to take steps where others have role in helping me organize the whole thing in
rarely taken, and come out on top. just a week.
Thankfully, I got a good teammate in This is a proud moment for me, as I type
Mr.Benjoy, who has stood by my ideas and inside my own design, and telling you all
suggested a lot to make it better. The idea of a what I have been doing. But I would surely
magazine itself started off like that. say that all these have been possible only
through the hardwork of the whole team.
Every time I get a copy of the Hemophilia
World or Hemophilia News, I used to realize I also would like to thank all those who
how much we can benefit from knowledge helped me select the initial topics, and
sharing. A magazine would be an easy way to illustrate them within the short time frame.
disburse the updates in the field of Hope you would enjoy going through the
hemophilia-related research. I also thought newsletter and give your suggestions.
that I can add real-life experiences from
people around the world, that can inspire You can expect a variety of interesting
others when they feel they are down. materials in this issue, and the upcoming ones,
which are carefully chosen to provide a mix
Finally, one day I couldn’t take it anymore. I of experience, technology and possibly, a bit
had to get this done, and that too, fast. I have of entertainment.
experience writing blogs, through which I am
telling the world what I am going through. So, simply read on!
You can find the blog at
http://prmahalingam.wordpress.com/
4

11. “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.
Hemophilia A, also known as factor VIII
In layman terms, Hemophilia is a medical deficiency, is the cause of about 80% of cases.
condition in which the blood fails to clot. In Hemophilia B, which makes up the majority
more medical terms, a certain ―clotting of the remaining 20% of cases, is a deficiency
factor‖ is missing in the blood, due to which
the clot that is formed during the bleed, of factor IX. Patients are classified as mild,
simply dislodges and disintegrates. moderate, or severe, based on the amount of
factor present in the blood.
Now, the condition might look quite simple
Article
when you hear it the first time. But there are A patient whose blood tests suggest severe
things that lie hidden in that definition. hemophilia will usually bleed frequently,
Bumps and scrapes are a part of every child’s whereas another patient with a milder form
life. For most kids, a tumble off a bike or a will usually bleed only rarely. However, there
stray kick in a soccer game means a is a range of severity within each group. The
temporary bruise or a healing scab. However,
for kids with hemophilia, these normal reasons for this variability may relate to other
traumas of childhood are reason for extra clotting factors or to differences in behaviours
concern. that present different risks of injury.
Clotting factors Signs and symptoms of hemophilia vary,
depending on severity of the factor deficiency
Human blood contains special proteins, and the location of the bleeding. Few babies
known as clotting factors. Identified by are diagnosed with hemophilia within the first
Roman numerals, clotting factors help stop 6 months of life because they’re unlikely to
bleeding and allow a blood vessel to heal after sustain an injury that would lead to bleeding.
an injury. The last step in the clotting process For example, only about 30% of males with
(also called coagulation) is the creation of a hemophilia bleed excessively when
―net‖ that closes the torn blood vessel and
stops the bleeding. This part of the process circumcised and only 1% to 2% of newborns
involves clotting factors VIII and IX. People with hemophilia have bleeding within the
with hemophilia are deficient in one of those skull (called an intracranial hemorrhage).
factors due to their abnormal genes and, as a
result, their blood can’t clot properly.
5

12. Hemophilia is primarily a hereditary Although hemophilia is a lifelong condition
condition. But nowadays, more cases of with no cure (other than liver transplantation),
spontaneous mutation are rising, which it can be successfully managed with clotting
creates more mystery of how it is actually factor replacement therapy — periodic
caused. infusions of the deficient clotting factor into
the child’s bloodstream. Factor replacement
The genetic transmission occurs because a may be given through an intravenous (IV) line
critical blood clotting gene is carried on the X either at the hematology clinic or at home by
chromosome. Since males only carry one X a visiting nurse or by parents (and even older
chromosome, if that is defective, hemophilia patients) who have undergone special
will immediately show up. An early death is training. Once the clotting factor is ―infused,‖
likely. Females, on the other hand, carry two it begins to work quickly and helps prevent
X chromosomes. If only one is defective, the joint damage.
other normal X chromosome can compensate.
The woman will have normal blood clotting;
she will simply be a carrier of the recessive
Factor replacement may be
defective gene. This fact will be discovered if given through an
intravenous (IV) line
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
Article
Even with all this, there is little awareness
seen.
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 heard
of it! Whats that???
The question might look funny in the
beginning. But once we realize who the asker
This is how it can show up in a family tree. is, the gravity of the situation becomes clear.
6

13. Why the concern? Common misconceptions
The situation is much worse in India than that Now, there are a lot of misconceptions when it
compared to other western countries. When comes to the disorder.
we go back to the title, we have to realize that
It is our duty to clarify the
it is the doctors who are asking that question.
Doctors are having little misconceptions and make
information about the the public understand the
disorder! real deal!
The problem lies in the basic training itself. • Hemophilics can bleed anywhere in the
Here, only the hematologists and oncologists body, including the brain (IC bleed)
are involved in hemophilia-related training. • Joints are the most affected part of the
For the others, it is as simple as a small body, since prolonged bleeding can cause the
passage. So, they have insufficient tissue surrounding the joint to break down and
information regarding the kind of treatment to cause permanent damage, leading to
be undertaken in case of emergency. disability.
• Hemophilia’s effect isn’t limited simply
Article
So, the duty falls on the shoulders of the to the outer skin. Any part of the body that has
affected. They have to ―educate‖ the doctor blood flow can bleed, and the blood can build
about the condition before getting the up internally, leading to further damage.
treatment. The main issue is that many pain-
relievers like aspirin actually aggravate the
bleeding!
Drugs like Aspirin and
Ibuprofen can aggravate
the 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.
7

14. 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.
Note: The following note Rest: The bleed spot should get enough rest.
Immobilize the joint immediately so that the
applies only to joint bleeds, tissue doesn’t get stressed, and aggravate the
and any other bleed site bleed.
Ice: Apply ice packing. Ice increases the
might react differently! viscosity (thickness) of the blood, and slows it
down. The reduced flow will slow the
bleeding down. Also, the drop in temperature
Article
The first aid for joint bleeds is abbreviated as will freeze the nerves and relieve pain.
RICE. RICE stands for Compression: Use crepe bandage to secure
• Rest the bleeding joint. This tightens the blood
• Ice vessels and restricts the blood flow. It also
• Compression restricts movement and avoids further
damage.
• Elevation
Elevation: Keep the joint in an elevated
position. This avoids too much blood from
Once first aid is given, the patient has to be going to the affected area, and controls
monitored, and if the pain doesn’t subside, bleeding.
factor replacement therapy might be needed.
8

15. The Royal Line

16. “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.
When we hear the name ―Royal Disease‖,
we feel kind of proud. This is since the
disease is largely hereditary, and if a royal
bloodline had this disorder, we might actually
be part of it!
This is precisely what Forbes magazine has
But recent studies have established that the said. It has published a list of the 10 most
Feature
disorder can arise due to spontaneous
mutations also. So, a person with Hemophilia expensive medical conditions. The list goes as
may not actually be part of the royal follows.
bloodline. But still, all of us consider
ourselves as ―royals‖… 1. HIV $25,000
2. Cancer $49,000
While this has been a matter of personal pride
for many, there is a big burden working in the 3. Transplant $51,000
background. The name ―Royal Disease‖ has 4. Stroke $61,000
changed its meaning over time, from found in 5. Hemophilia $62,000
royal bloodline to one which can be afforded
only by people in a royal bloodline. This itself 6. Heart Attack including Cardiac
tells the impact it has on the financial status of Revascularization (Angioplasty with or
people. without Stent) $72,000
7. Coronary Artery Disease $75,000
The meaning has now 8. Neonate (premature baby) with extreme
changed to “one which can
problems $101,000
9. End-Stage Renal Disease $173,000
be afforded only by people 10. Respiratory Failure on Ventilator
in a royal bloodline” $314,000
Now, things get obvious.
10

17. You can see that, at $62,000 per year, dried, sterilized preparation, which removes a
Hemophilia scores above other medical lot of the infectious components. Nowadays,
conditions like Stroke, Transplants, HIV and the AHF is tested for a variety of infectious
even Cancer! This is contrary to popular components like HIV, BVDV, HCV, PRV,
belief that cancer is one of the most HBV, CPV, B19V and HAV. Once that is
expensive. Even if not that common, there are complete, the AHF is maintained in a
a lot other disorders that burn a deeper hole in Albumin base.
the patients’ pockets.
All these take a lot of time and effort, and
The situation is not at al different in India. To pushed up the cost of manufacturing by a
add to that, Hemophilia is not covered under huge margin. Accounting for all these, the
any medical insurance here, whereas some AHF costs around Rs.3750 for a minimal
western countries provide such support. So, dose.
every last drop of medicine has to be
―bought‖. When we calculate the medical AHF is brought out not in terms of
cost, the cost of AHF (Anti-Hemophilic conventional volume, but as IU (an
Factor) outweighs the complete diagnosis and internationally accepted unit of
hospitalization cost by a factor that can’t even measurement). In India, the open market rate
be estimated! Even if AHF is a short-term per IU of AHF comes to around Rs.15. So,
medication, it demands more from the patient you can infer the amount to be spent in case
than the complete course of supplementing of a bleed.
medication.
Feature
Now, the dosage depends on the level of
In India, AHF is imported from countries like factor present in the body, the required rate of
USA (Baxter, Bayer), Italy (Kedrion), etc. The increase and the weight of the person.
availability of AHF is limited by the stringent
standards imposed on the manufacturing and
sterilization. Depending on the level of factor available in
the body, PWHs are classified into 3
categories:
AHF is a blood-based product. It is made up
of clotting factors extracted from human Severe (<1% available)
blood, which is donated by a pool of donors Moderate (1-10% available)
worldwide. Since the blood is natural, there Mild (>10% available)
might be a variety of components that might
make the drug unfit for use. As a matter of The fall in availability of AHF is primarily
fact, during the 1980s’, millions of PWHs in due to the low liver activity, which results
USA and Canada were affected by blood- from the genetic deficiency. For the severe
borne diseases, including HIV, which was category, even the most ―simple‖ bumps and
transmitted through the medicine. bruises can lead to a torrential bleed episode,
which may require factor replacement. Also,
One that was brought to light, more stringent the level of factor increase depends on the
measures were adopted to make AHF safer, type and location of bleed.
and fit for use. So, AHF was brought out as a
11

18. Hemorrhage
Type of hemorrhage Required peak post-infusion Frequency of infusion
AHF activity in blood
Early hemarthrosis or muscle 20-40 12/24 hours for one-three days,
bleed or oral bleed until bleeding arrested, or resolved
More extensive hemarthrosis, 30-60 12/24 hours for three days, until
muscle bleed or hematoma resolved
Life threatening bleeds like head 60-100 8/24 hours until healed
injury, throat bleed, severe
abdominal pain
Surgery
Type of operation
Minor surgery, including tooth 60-80 Single infusion + oral anti-
extraction fibrinolytic therapy
Major surgery 80-100 (pre & post-operative) 8/24 hours, depending on healing
So, you can see that there might be multiple Now, there are variants of Hemophilia like
doses required for a single bleed episode. But Hemophilia A (Factor VIII), Hemophilia B
before deciding the frequency, the dosage per (Factor IX), Factor V, Factor X, Factor XIII
administration has to be determined. and so on. For each variant, the medication
Feature
will be different, and costs will vary.
The dosage required is calculated like:
And there is another problem faced by PWHs.
If the AHF dosage becomes too high, the
immune system of the body might react, and
So, for example, a peak level of 70% required produce antibodies, called Inhibitors. In that
for a child of 40 kg, the dosage would be 1400 case, there is a specialized drug called
IU. If it has to be repeated once every 12 FEIBA, that contains activated agents to
hours, the daily dosage would be 2800 IU. bypass the inhibitors. But for that, the cost is
The cost then goes to Rs.42000! many fold, going up to Rs.24,000 for 500 IU.
An alternative is NovoSevenTM , which is also
Also, the half life of the medication is just as expensive as FEIBA.
around 14.8 + 3.0 hours. So, the effect of the
1400 IU will go down to 700 IU (35% Adding all this, the cost incurred by an
effective) after 15 hours, and to 350 IU average family with hemophiliacs can be
(17.5% effect) after 30 hours. So, proper inferred. This will take a severe toll on them,
supplementation has to be provided in terms and in cases where there are multiple PWHs
of subsequent dosages, so that the level is in the same family (which is also getting
maintained at 70%. common these days), the condition worsens.
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.
12

19. 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…
Jithin was like all other royals initially. He
Mr. Jithin Jose is just another person in a had a childhood like all others, and had
suburb in Cochin for many. But for people occasional bleeds like others. Diagnosed with
like us, he is an inspiration by all means. As a Hemophilia A, bruises and bleeds were a part
fellow royal, he has faced all the hardships of his life from the very beginning.
like everyone else among us. But what sets
him apart from us is that he has gone to the
Real-life story
He was not the kind of a person who simply
extreme of it, and stays there with a smile on took AHF for every other bleed. He took
his face! 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.
13

20. To add to that, they also confirmed the After finishing school, he joined for B.Com,
presence of HCV (Hepatitis C), which he which he could attend only for the first day.
might have contracted during a earlier Still, he continued studying at home, and
infusion of blood (to balance the hemoglobin wrote exams. And now, he is writing exams
levels). for CA (Chartered Accountant).
Hemophilia, Inhibitors and The real scale of his
HCV don’t form quite a success comes only now.
good combination.
Despite all this persistent issues, he scored the
42nd rank in the country in the INTER exams
HCV, even if dormant, still poses a threat for CA. This shows the world that you needn’t
since Hemophilia itself, which originates from be physically the best to achieve success. He
a liver-related genetic change, can cause the couldn’t attend subsequent exams due to
virus to turn worse. But treatment is not that bleeds, and right now, he is preparing for the
easy and effective. final round of exams (which fall in May).
Real-life story
After all this, he had a fall, which dislocated
his left knee cap, and caused a joint problem. An endnote
As a result of that, that leg stopped growing -Mahalingam.P.R
along with the other, and now, his left leg is
shorter than his right. So, when he tries to When we entered his room, he received us
walk, the hip joint gets stressed, and causes with a smile on his face. The smile says a lot.
problems. He is staring fate in the eye, and moving
forward.
All this has weakened his calf muscles, and he When I met him, at the first glance itself, I got
is totally bed-ridden. When he tries to walk, reminded of Stephen Hawking – the man who
the hip joints cause problems, and the legs rules the science world from his wheel chair.
can’t take the weight of the body. (Even when Jithin’s body may not be helping him, but his
we were there, he had a bleeding episode in mind is clear and sharp enough to tackle and
his left arm). Frequent bleeds also have cut through any obstacles that is put in front of
caused range restriction in both arms. him.
But studies are different With confidence backing him, how can he
lose???
from disability! His situation will be an inspiration for all the
people, whether they are hemophilic or not.
He had to stop going to school at 3rd standard The next time you think your life is miserable,
itself. But he studied from home, and passed just think of him, and you can understand how
the exams with flying colors. He also gets 45 comfortable your life is, and how can sail
minutes extra time for exams. above it…
14

21. Continuous care in Hemophilia
- Dr. K. Hari
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
Hemophilia Care
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.
The British royal family transferred the
Hemophilia gene to German and
Russian royal bloodlines.
But what went horribly
wrong in Russia!?
What led to the fatal
twist of events???
The
Kremlin Konnection
15

22. 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„
AHF subsidized: 3275 IU (worth Rs.32750/-)
Chapter activity
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 Draft
If Cheque / DD, they can be issued favoring Hemophilia Society
Cochin Chapter, payable at Cochin…
Donate via HFI… Issue Cheques / DD favoring
Want IT Hemophilia Federation (India) payable at New Delhi…
exemption??? Donations will have 100% IT exemption under
Section 35AC.
Cheques / DD can be mailed to the chapter’s address given in the last page…
16

23. We would like to express our endless thanks to those who contributed to lift the chapter from rough
seas„
Contributions
Students of M.Tech CSESIS, RSET
Mrs. Roopa Menon
Mr. Homi P Ustad
Staff of HLL Lifecare Ltd
Mr. Madhava Priyan
Mr. Narayan Shankar
M/S Mediatronix Pvt Ltd
Mr. K Gireendara Babu
Mrs. Serena Mathew
M/S Destination Health
M/S Basheer & Shahid
M/S Cannanore Handlooms
Contributions
Mr. Sathyan C S
Mrs. Padma Balasubramaniam
Mr. Vipin Antony
Mrs. Sinna P J
Students from Mr. Sreenivas Staff of HLL
M.Tech CSESIS, RSET M/S Aryabhangy Lifecare Ltd
M/S CSD Adenwala Trust
M/S Cochin Rubbers
Mr. Aravind Krishnan R Mr. Sreeraj Choorakkad
Mrs. Arifa Azeez Mr. Kesavan
Mrs. Aswathy M C Mrs. Sreekumari
Mrs. Dhanya Sudarsan Ms. Jemi Raju
Mrs. Geethu Thomas Mr. Anoop
Ms. Geethu Wilson Ms. Athira
Ms. Jeeva Susan Jacob Mr. Raghul C
Ms. Josna Joseph Ms. Junitha C R
Mrs. Lithewmol Mathew Ms. Sindhu C
Ms. Merin Sebastian Mr. Jayesh Kumar
Mrs. Neethu Mohandas Mr. Harish T
Mrs. Nithya Joy Ms. Nejj Thomas
Ms. Saranya D Krishnan Mr. Manoj
Mrs. Soumya Alias
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24. 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
Hemophilia Society Cochin Chapter
Flat – A, Ground Floor, Thripthi Apartments,
Near Agasthya Medical Centre, Old Bus
Stand, Tripunithura, Cochin – 682301
Phone: +91-484-2774083
8547988083 (Syamala Ramaswamy)
9895243239 (Mahalingam.P.R)
9349315905 (Ramaswamy.P.M)
Mail:
cochin@hemophilia.in
hemoyouth.cochin@gmail.com
Web: http://hemophilia-cochin.co.nr/
Affiliated to Hemophilia Federation (India)
www.hemophilia.in