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Transcript of THE ROYAL LINE
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
init
iati
ve o
f Y
ou
th G
rou
p, H
em
op
hili
a So
cie
ty C
och
in C
hap
ter
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
sto
ry o
f an
ac
hie
ver…
Pg 13
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.
Intr
od
uct
ion
by
the
Pre
sid
ent
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.
Intr
od
uct
ion
by
the
Pre
sid
ent
-Dr. K. Hari
Benj
oyK
.J
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
com
e fr
om
th
e Ed
ito
rial
Tea
m
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
alin
gam
P.R
4
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
com
e fr
om
th
e Ed
ito
rial
Tea
m
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
icle
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
ure
“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:[email protected]
[email protected]: 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