March 2012THE R                          YAL LINE                        A periodic newsletter from Hemophilia Society Coc...
You might be a PWH…                      You might know a PWH…             But do you know the factor that is lacking?Comi...
Hemophilia Society Cochin Chapter is a non-profit, voluntary charitable organization established tosupport people sufferin...
Hemophilia Society Cochin Chapter was "revived" in 2005 under Mrs. Syamala Ramaswamy, who waspreviously in Trivandrum. Dur...
Hemophilia Society Cochin Chapter is affiliated to Hemophilia Federation (India),the national level body at New Delhi. It ...
In this edition                                               Hemophilia Society Cochin Chapter                           ...
Dr. K. Hari, Physician at Medical Trust                                                              Hospital, Cochin, and...
The extent of bleeding depends on how severe the hemophilia is. Children who have mild hemophiliamay not have signs unless...
The Inception   This is the first edition of the newsletter. Lets start it off with a few        words from the editors. B...
The Beginning   The author is a PWH himself, suffering from Hemophilia A (severe                                          ...
“Hemophilia”?                                Never heard of it!   This is a statement which we have encountered quite a lo...
Hemophilia is primarily a hereditary               Although hemophilia is a lifelong conditioncondition. But nowadays, mor...
Why the concern?                                   Common misconceptionsThe situation is much worse in India than that    ...
First Aid in Hemophilia   Even if Hemophilia can get serious in many cases, with quick action, the damage can be        mi...
The Royal Line
“Royally Expensive”   Hemophilia is popularly called the “Royal Disease”. The name primarily originated due to its       p...
You can see that, at $62,000 per year,              dried, sterilized preparation, which removes aHemophilia scores above ...
Hemorrhage       Type of hemorrhage             Required peak post-infusion            Frequency of infusion              ...
Impossibility – A myth   Following are the excerpts from a visit by Mrs. Syamala Ramaswamy and Mr.         Mahalingam.P.R ...
To add to that, they also confirmed the           After finishing school, he joined for B.Com,presence of HCV (Hepatitis C...
Continuous care in Hemophilia                                                                                   - Dr. K. H...
After the resurrection in December 2011, Hemophilia Society Cochin Chapter has beenabuzz with activity. With the financial...
We would like to express our endless thanks to those who contributed to lift the chapter from roughseas„                  ...
With a membership strength of 54, Hemophilia Society Cochin Chapterwas established in 2005 for supporting PWHs in and arou...
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THE ROYAL LINE

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

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THE ROYAL LINE

  1. 1. March 2012THE 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 PainLook beyond Hemophilia for a life full of success…Issue 1 24 pages (including cover)
  2. 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. 3. Hemophilia Society Cochin Chapter is a non-profit, voluntary charitable organization established tosupport people suffering from Hemophilia (PWH). We aim at both emotional and physical support forthose who need it, when they need it. We also provide medical attention in association with ourtreatment centre – Medical Trust Hospital.Revived in 2005, we have a current strength of 54 members, with hundreds left to be located. One ofthe biggest challenges we face is the attitude itself. Many PWHs are not ready to come out and shout itloud. They fear an isolation in the society, and hate sympathy more than anything. Again, if they canafford the medication, the won’t feel the need of working with a charitable society. But what they don’tknow is that they are indirectly doing a big injustice to the economically backward PWH families, whoare struggling even for their daily bread!Coming back to the chapter, we were lacking in the necessary manpower until the day of 11th Dec2011, when the whole chapter was reorganized, with the active members at the top. This day also sawthe 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 theactivities of documentation and event planning from the chapter’s core groups. When we wereexploring different ways to spread the word about the disorder, the first thing that came to our mind isthe power of social networks. And to give more updates, we have given life to the newsletter – THEROYAL LINE.Through this, we aim to make others aware of the plights faced by every PWH, and update them withsome information, which they may find interesting and useful. This should indirectly boost themanpower 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 weare 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. 4. Hemophilia Society Cochin Chapter was "revived" in 2005 under Mrs. Syamala Ramaswamy, who waspreviously in Trivandrum. During revival, the chapter was not in a good state. From then, it was thesingle-handed efforts of Mrs. Syamala that brought the chapter up. A team consisting of a set of loyalmembers were along with her in the process. But there is a huge manpower limitation that has beenhampering the activities.Recently, there has been a tremendous improvement in the activity level of the chapter. The chapter gotaffiliated to HEMOPHILIA FEDERATION (INDIA) at New Delhi. Youth and Women group have beenestablished who are taking care of the activities.The people behind• Dr.K.Hari - President• Mrs.Syamala Ramaswamy - Secretary• Mr.K.J.Joy - TreasurerWhat we doOur 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 AHFWhen we prioritize our tasks, we look for funding since the AHF should be made affordable to themembers. Only when this is done, we have to go for availability of AHF.
  5. 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 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...Excerpts from HFI’s website:Established in 1983, Hemophilia Federation India is a self-help NGO run by Persons with Hemophiliathemselves, with help from medical fraternity. We represent India as National Member Organization atthe World Federation of Hemophilia based in Canada. We also work in close collaboration with WorldHealth 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 paramedicalpractitioners. We have two International Hemophilia Training Centers at CMC Vellore and KEMHospital 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 areattached to diagnostic facilities locally. Our Special Needs Cell for HCV/ HIV positive PWH supportsthem by providing lifelong subsidized treatment including Anti-Retroviral Therapy Drug.We also have started National Hemophilia Registration (Record) to locate undiagnosed persons andchildren with hemophilia. We have been able to update records of support to more than 16,000 Personswith hemophilia in our national hemophilia records.
  6. 6. In this edition Hemophilia Society Cochin Chapter Dr. P. V. Louis - Patron Dr. K. Hari - President“Royally” expensive Mrs.Syamala Ramaswamy- SecretaryHemophilia features in Forbes list of Mr. K. J. Joy - Treasurermost expensive medical conditions.What is making this rarely seen Youth Groupdisorder so hard-to-afford? Pg 10 Mahalingam.P.R Benjoy K.JMedico Intro Pg 1 Russel MendezTHE INCEPTION Pg 3 Amal SureshTHE BEGINNING Pg 4For those who are hearing it the first time Pg 5 The Royal LineFirst aid in Hemophilia Pg 8Continuous 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. RChapter Activity Pg 16Contributors Pg 17
  7. 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. Itis a rare bleeding disorder, and if you have Hemophilia, you may bleed for a longer time than othersafter an injury. You may also bleed inside your body (internally), especially in your knees, ankles andelbows. This bleeding can damage your organs and tissues, and may be life threatening.Causes and Risk factorsWhen you bleed, the body launches a series of reactions that help the blood clot. This is called a Introduction by the Presidentcoagulation cascade. This process involved special proteins called coagulation factors. When one ormore 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, Xand XIII are also present, but less in number compared to VIII and IX). In most cases, Hemophilia ishereditary. ie, it is passed on from parents to children down the family line through defective genes. Itcommonly affects males.The clotting proteins work with the platelets to help the blood clot. Platelets are small blood cellfragments that form in the bone marrow – a sponge-like tissue in the bones. Platelets play a major rolein blood clotting. When blood vessels are injured, clotting factors help platelets stick together to plugcuts and breaks on the vessels and stop bleeding.As mentioned above, two deficient factors are predominant in hemophilics. They are denoted asHemophilia A and Hemophilia B. If you have Hemophilia A, you’re missing or have low levels ofclotting factor VIII. About 9 out of 10 hemophilics are of type A. If you have Hemophilia B, you’remissing or have low levels of factor IX.Rarely, Hemophilia can be acquired. “Acquired” means that you are not born with this disorder, butyou develop it during your lifetime. This can happen if your body forms antibodies (proteins) thatattack the clotting factors in your bloodstream. The antibodies can prevent the clotting factors fromworking.Symptoms & BleedsThe main symptom of Hemophilia is bleeding itself. Mild cases my go unnoticed until later in life, whenthey occur during surgery or after trauma. In more severe cases, serious bleeding may occur withoutany cause. Internal bleeding may occur anywhere. But bleeding into joints is most common. 1
  8. 8. The extent of bleeding depends on how severe the hemophilia is. Children who have mild hemophiliamay not have signs unless they have excessive bleeding from a dental procedure, an accident, orsurgery. Bleeding can occur on the body’s surface (external bleeding) or inside the body (internalbleeding).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 timeSigns 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 PresidentSigns & TestsMost often, hemophilia is diagnosed after a person has an abnormal bleeding episode, or when there isa known family history of the condition. Blood tests to detect the protein levels of VIII and IX areusually done, and then a person is classified as mild or severe (there are moderate hemophilics also,which lie between mild and severe).TreatmentStandard treatment involves replacing the missing clotting factor through a vein (intravenousinfusions). Diagnosing a bleeding disorder is important so that the doctor can take extra care if youneed 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 areslowly dripped or injected into a vein. These infusions help replace the clotting factor that’s missing orlow. Clotting factor concentrates can be made from human blood. The blood is treated to prevent thespread of diseases, such as Hepatitis. With the current methods of screening, the chances of contractinga blood-borne infection is very low. To further reduce the risk, clotting factor concentrates are makefrom non-blood sources. They are called recombinant clotting factors. They are easy to store, mix anduse at home, and takes only about 15 minutes to administer.Replacement therapy can be done regularly to prevent bleeding, called preventive or prophylactictherapy. 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. 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 mostHai Readers, Introducing myself, I am important part. It stands as a pillar to attitudeBenjoy, doing M.com at MG university. & proper planning. Let me illustrate this with Welcome from the Editorial TeamMoreover 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 noRoyalline. Myself and my friends, idea on the poisoned ones. What would youMr.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 ,eighthaemophilics. There are many more like us in glasses are good to taste & enjoy. What if uour society. But only a few of us fall under the got the right knowledge about the glassesyouth category. An year before, we rarely met which you should avoid. It is sure that youonly when a formal meeting was conducted won’t have fear & can utilise & enjoy theby society & we knew ourselves only as being other eight. The life of a haemophilic is likethe same Royal, that’s all. But as time passed, that. There are many things, attitudes, the waywe realised that it would be better to form a of doing things, thoughts which may begroup of royals & thereby share our hazardous to a haemophilic in many ways.experiences, knowledge & do something And the same life also offers things which iscreatively for all haemophilics. By keeping essential & good to have. As long as you don’tthis 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 thingsNow, let’s discuss about the relevance & too, that may suit you & may have beenimportance of youthwing & the purpose of achieved by you ,to lead a better life .It is athis magazine .I guess, many of you know universally accepted truth that ― knowledge iswhat haemophilia is. As you have already the ultimate power”, as it helps you toread 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 ,werealised & admired at one point that, in intend to improve the above mentionedtoday’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 meanshard to survive, I mean physically, mentally & ,most importantly in wisdomfinancially. Let hope the Royal line will serve the realLet us consider the knowledge aspect first. purpose for what it has been framed. 3
  10. 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 noWhen the youth group was initiated on 11 th time, the design was on. Throughout the Welcome from the Editorial TeamDecember 2011, there was only one thing in design process, Benjoy has given me valuablemy mind – to make this group a force to feedback regarding the layout andreckon with. So, innovation was on top of my backgrounds. He has played a commendablelist. I wanted to take steps where others have role in helping me organize the whole thing inrarely 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 typeMr.Benjoy, who has stood by my ideas and inside my own design, and telling you allsuggested a lot to make it better. The idea of a what I have been doing. But I would surelymagazine 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 HemophiliaWorld or Hemophilia News, I used to realize I also would like to thank all those whohow much we can benefit from knowledge helped me select the initial topics, andsharing. 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 thehemophilia-related research. I also thought newsletter and give your suggestions.that I can add real-life experiences frompeople around the world, that can inspire You can expect a variety of interestingothers when they feel they are down. materials in this issue, and the upcoming ones, which are carefully chosen to provide a mixFinally, one day I couldn’t take it anymore. I of experience, technology and possibly, a bithad to get this done, and that too, fast. I have of entertainment.experience writing blogs, through which I amtelling the world what I am going through. So, simply read on!You can find the blog athttp://prmahalingam.wordpress.com/ 4
  11. 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 VIIIIn 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 majoritymore medical terms, a certain ―clotting of the remaining 20% of cases, is a deficiencyfactor‖ is missing in the blood, due to whichthe 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 Articlewhen you hear it the first time. But there are A patient whose blood tests suggest severethings 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 formlife. For most kids, a tumble off a bike or a will usually bleed only rarely. However, therestray kick in a soccer game means a is a range of severity within each group. Thetemporary bruise or a healing scab. However,for kids with hemophilia, these normal reasons for this variability may relate to othertraumas of childhood are reason for extra clotting factors or to differences in behavioursconcern. that present different risks of injury.Clotting factors Signs and symptoms of hemophilia vary, depending on severity of the factor deficiencyHuman blood contains special proteins, and the location of the bleeding. Few babiesknown as clotting factors. Identified by are diagnosed with hemophilia within the firstRoman numerals, clotting factors help stop 6 months of life because they’re unlikely tobleeding 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 andstops the bleeding. This part of the process circumcised and only 1% to 2% of newbornsinvolves clotting factors VIII and IX. People with hemophilia have bleeding within thewith hemophilia are deficient in one of those skull (called an intracranial hemorrhage).factors due to their abnormal genes and, as aresult, their blood can’t clot properly. 5
  12. 12. Hemophilia is primarily a hereditary Although hemophilia is a lifelong conditioncondition. But nowadays, more cases of with no cure (other than liver transplantation),spontaneous mutation are rising, which it can be successfully managed with clottingcreates more mystery of how it is actually factor replacement therapy — periodiccaused. infusions of the deficient clotting factor into the child’s bloodstream. Factor replacementThe genetic transmission occurs because a may be given through an intravenous (IV) linecritical blood clotting gene is carried on the X either at the hematology clinic or at home bychromosome. Since males only carry one X a visiting nurse or by parents (and even olderchromosome, if that is defective, hemophilia patients) who have undergone specialwill 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 preventX 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 bedefective gene. This fact will be discovered if given through an intravenous (IV) linesome of her children are hemophiliacs.Naturally, women hemophiliacs are rarebecause it takes two defective Xchromosomes in order for the condition to be Article Even with all this, there is little awarenessseen. 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 askerThis is how it can show up in a family tree. is, the gravity of the situation becomes clear. 6
  13. 13. Why the concern? Common misconceptionsThe situation is much worse in India than that Now, there are a lot of misconceptions when itcompared 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 theit is the doctors who are asking that question.Doctors are having little misconceptions and makeinformation about the the public understand thedisorder! real deal!The problem lies in the basic training itself. • Hemophilics can bleed anywhere in theHere, only the hematologists and oncologists body, including the brain (IC bleed)are involved in hemophilia-related training. • Joints are the most affected part of theFor the others, it is as simple as a small body, since prolonged bleeding can cause thepassage. So, they have insufficient tissue surrounding the joint to break down andinformation regarding the kind of treatment to cause permanent damage, leading tobe undertaken in case of emergency. disability. • Hemophilia’s effect isn’t limited simply ArticleSo, the duty falls on the shoulders of the to the outer skin. Any part of the body that hasaffected. They have to ―educate‖ the doctor blood flow can bleed, and the blood can buildabout the condition before getting the up internally, leading to further damage.treatment. The main issue is that many pain-relievers like aspirin actually aggravate thebleeding!Drugs like Aspirin andIbuprofen can aggravatethe bleeding.The same issue is faced by the patientsthemselves. This is since they themselveshave to avoid these drugs even in the utmostemergency.The most effective method to treat the bleedsis to use Anti-Hemophilic Factor (AHF),which can be expensive. 7
  14. 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 theapplies only to joint bleeds, tissue doesn’t get stressed, and aggravate theand any other bleed site bleed. Ice: Apply ice packing. Ice increases themight react differently! viscosity (thickness) of the blood, and slows it down. The reduced flow will slow the bleeding down. Also, the drop in temperature ArticleThe 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 fromOnce first aid is given, the patient has to be going to the affected area, and controlsmonitored, and if the pain doesn’t subside, bleeding.factor replacement therapy might be needed. 8
  15. 15. The Royal Line
  16. 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 thedisease is largely hereditary, and if a royalbloodline had this disorder, we might actuallybe part of it! This is precisely what Forbes magazine hasBut recent studies have established that the said. It has published a list of the 10 most Featuredisorder can arise due to spontaneousmutations also. So, a person with Hemophilia expensive medical conditions. The list goes asmay not actually be part of the royal follows.bloodline. But still, all of us considerourselves as ―royals‖… 1. HIV $25,000 2. Cancer $49,000While this has been a matter of personal pridefor many, there is a big burden working in the 3. Transplant $51,000background. The name ―Royal Disease‖ has 4. Stroke $61,000changed its meaning over time, from found in 5. Hemophilia $62,000royal bloodline to one which can be affordedonly by people in a royal bloodline. This itself 6. Heart Attack including Cardiactells the impact it has on the financial status of Revascularization (Angioplasty with orpeople. without Stent) $72,000 7. Coronary Artery Disease $75,000The meaning has now 8. Neonate (premature baby) with extremechanged to “one which can problems $101,000 9. End-Stage Renal Disease $173,000be afforded only by people 10. Respiratory Failure on Ventilatorin a royal bloodline” $314,000 Now, things get obvious. 10
  17. 17. You can see that, at $62,000 per year, dried, sterilized preparation, which removes aHemophilia scores above other medical lot of the infectious components. Nowadays,conditions like Stroke, Transplants, HIV and the AHF is tested for a variety of infectiouseven 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 isexpensive. Even if not that common, there are complete, the AHF is maintained in aa lot other disorders that burn a deeper hole in Albumin base.the patients’ pockets. All these take a lot of time and effort, andThe situation is not at al different in India. To pushed up the cost of manufacturing by aadd to that, Hemophilia is not covered under huge margin. Accounting for all these, theany medical insurance here, whereas some AHF costs around Rs.3750 for a minimalwestern 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 ofcost, the cost of AHF (Anti-Hemophilic conventional volume, but as IU (anFactor) outweighs the complete diagnosis and internationally accepted unit ofhospitalization cost by a factor that can’t even measurement). In India, the open market ratebe 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 casethan the complete course of supplementing of a bleed.medication. Feature Now, the dosage depends on the level ofIn India, AHF is imported from countries like factor present in the body, the required rate ofUSA (Baxter, Bayer), Italy (Kedrion), etc. The increase and the weight of the person.availability of AHF is limited by the stringentstandards imposed on the manufacturing andsterilization. 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 upof 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 mightmake the drug unfit for use. As a matter of The fall in availability of AHF is primarilyfact, during the 1980s’, millions of PWHs in due to the low liver activity, which resultsUSA and Canada were affected by blood- from the genetic deficiency. For the severeborne diseases, including HIV, which was category, even the most ―simple‖ bumps andtransmitted 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 themeasures were adopted to make AHF safer, type and location of bleed.and fit for use. So, AHF was brought out as a 11
  18. 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 healingSo, you can see that there might be multiple Now, there are variants of Hemophilia likedoses required for a single bleed episode. But Hemophilia A (Factor VIII), Hemophilia Bbefore deciding the frequency, the dosage per (Factor IX), Factor V, Factor X, Factor XIIIadministration 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, andSo, for example, a peak level of 70% required produce antibodies, called Inhibitors. In thatfor a child of 40 kg, the dosage would be 1400 case, there is a specialized drug calledIU. If it has to be repeated once every 12 FEIBA, that contains activated agents tohours, the daily dosage would be 2800 IU. bypass the inhibitors. But for that, the cost isThe cost then goes to Rs.42000! many fold, going up to Rs.24,000 for 500 IU. An alternative is NovoSevenTM , which is alsoAlso, the half life of the medication is just as expensive as FEIBA.around 14.8 + 3.0 hours. So, the effect of the1400 IU will go down to 700 IU (35% Adding all this, the cost incurred by aneffective) 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 PWHsof subsequent dosages, so that the level is in the same family (which is also gettingmaintained 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. 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. HeMr. Jithin Jose is just another person in a had a childhood like all others, and hadsuburb in Cochin for many. But for people occasional bleeds like others. Diagnosed withlike us, he is an inspiration by all means. As a Hemophilia A, bruises and bleeds were a partfellow royal, he has faced all the hardships of his life from the very beginning.like everyone else among us. But what setshim apart from us is that he has gone to the Real-life story He was not the kind of a person who simplyextreme of it, and stays there with a smile on took AHF for every other bleed. He tookhis 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. 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, andinfusion of blood (to balance the hemoglobin wrote exams. And now, he is writing examslevels). for CA (Chartered Accountant).Hemophilia, Inhibitors and The real scale of hisHCV 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 examsHCV, even if dormant, still poses a threat for CA. This shows the world that you needn’tsince Hemophilia itself, which originates from be physically the best to achieve success. Hea liver-related genetic change, can cause the couldn’t attend subsequent exams due tovirus to turn worse. But treatment is not that bleeds, and right now, he is preparing for theeasy and effective. final round of exams (which fall in May). Real-life storyAfter all this, he had a fall, which dislocatedhis left knee cap, and caused a joint problem. An endnoteAs a result of that, that leg stopped growing -Mahalingam.P.Ralong with the other, and now, his left leg isshorter than his right. So, when he tries to When we entered his room, he received uswalk, 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 gotis totally bed-ridden. When he tries to walk, reminded of Stephen Hawking – the man whothe 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 hiswe were there, he had a bleeding episode in mind is clear and sharp enough to tackle andhis left arm). Frequent bleeds also have cut through any obstacles that is put in front ofcaused 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 howthe exams with flying colors. He also gets 45 comfortable your life is, and how can sailminutes extra time for exams. above it… 14
  21. 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 horriblywrong in Russia!?What led to the fataltwist of events??? The Kremlin Konnection 15
  22. 22. After the resurrection in December 2011, Hemophilia Society Cochin Chapter has beenabuzz with activity. With the financial condition not at its best, the chapter has beenworking on raising funds in all circles. Corporate organizations were approached, and wemanaged to collect a considerable amount of money. Using this fund, we were able tosubsidize 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 weregiven positions depending on how active they are willing to be. New ideas on fund raisingare 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. 23. We would like to express our endless thanks to those who contributed to lift the chapter from roughseas„ 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 17
  24. 24. With a membership strength of 54, Hemophilia Society Cochin Chapterwas 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

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