Disclaimer: Writing these notes first as a patient then as an associate member of Thalassemia Federation of Pakistan
Finally after so long delay national thalassemia conference took place in Fatima Memorial Hospital College of Medical & Dentistry in Lahore on 29th-30th November 2014.
Delegates from all over Pakistan started reaching Lahore on 28th November, there was an executive meeting held on 28th November in Carlton Hotel where executive members were invited and few patients from Punjab were also there as observers.
The conference kicked off on 29th November at FMH College of Medical & Dentistry at 9AM after the member’s registration. Starting from the Quran recitation and welcome address by president TFP Punjab, activities of TFP were shared by general secretary TFP Dr. Yasmin Rashid, with few words from Dr Joveria Manan, Chief Guest, Actor Adeel Hashmi and President TFP the ceremony for gold medal to outstanding thalassemics was started.
The below mentioned patients were given gold medals:
Rumaisa Noor, Amna Abdul Hakeem, Mehvish, Shahzaib, Mujtaba and Noman.
Sara Ehsan, final year student of MBBS also got recognition from TFP.
Gold medals are given to the patients who keep their ferritin within 1000 to encourage them and to motivate other patients.
After tea break the plenary session was started on topic Emergencies in Thalassemia by Prof Joveria Manan. The talk was as usual on old stuff regarding emergencies caused after transfusion and iron chelation drugs, such as blood count drop by ferriprox, joint pains by kelfer / ferriprox etc.
Dr Jovaria shared with us that patients are getting HIV Aids infected blood and recently few cases were diagnosed in Pakistan and this is alarming situation for patients & parents. She also shared about kits and blood bags from China are being used in many NGOs and are giving reactions in many patients and thalassemia centers are suggested to stop using them…
After the plenary session scientific session on follow topics:
1) Diagnostic dilemmas in thalassemia
2) Non transfusion dependent thalassemia
3) Role of iron deficiency anemia in the propagation of beta thalassemia gene in Pakistan
Call it a sad point or negligence that till date we are only aware of thalassemia minor and thalassemia major.
Mostly patients, parents and even doctors are not aware of thalassemia intermedia and due to that many intermerdia patients are misdiagnosed and bound to be tagged as thalassemia major and are forced to get transfusion just like thalassemia major.
The criteria few people used is if the patient got first transfusion under 2 years of old is thalassemia major and if above 2 years old then thalassemia intermedia. I would say it is better to get electrophoresis done and checked by some renowned hematologist.
Non transfusion dependent is basically thalassemia intermedia which is less severe stage than thalassemia major. Intermedia patients are most likely to get transfusion in 3, 6, 9, 12 months unlike thalassemia major who have to get transfusion in every 15-20 days.
Many intermedia patients from Pakistan are now on drug called hydroxyurea which helps them increase transfusion gap more and many patients are maintaining their hemoglobin on 7-8 gram with the help of hydroxyurea.
As a patient point of view I personally think hemoglobin should be maintained at 10 gram and many doctors are trying hydroxyurea on thalassemia major which is not helping them. I would appreciate if any hematologist is reading this please do share your point of view too.
Personally my hematologist has told me that hydroxyurea is basically for cancer patients and it can work for intermedia patients but it is dangerous for thalassemia major, according to him he can shift me to hydroxyurea if I want but instead of helping me it will worsen my condition and my life span will be affected. Another verification I got from a patient that he asked the doctor who is doing trial of hyrdoxyurea on thalassemia major, doctor literally told him that he is not willing to do trial on major but he is bound/forced to do so.
Hence I will again say get your mutation and electrophoresis checked by renowned doctor before jumping for the cure, you might hurt yourself by the so called cure and doctors shouldn’t risk patients life like this.
After the lunch few patients were selected to speak on issues they are facing regarding treatments etc. I was selected by Hussain Jafri, the issues I raised were:
1) No / less representation of patients from Sindh in conference
2) Non availability of blood and iron chelation drugs in thalassemia centers of Karachi
3) Thalassemia Federation Pakistan need to be more strong for patients unity and issues.
Fellow thalassemics Faseeh and Batool talked about blood infections and jobs issues of thalassemics.
I really wish TFP let the patients come forward and voice their issues; we need 5 representatives / patients for 5 provinces in executive meeting. And I wish TFP take notice of bad screening, HIV cases and non availability of ferriprox.
The day ended with general body meeting and elections, where a resolution was passed that one of patient of a province will be attending committee meeting, province was selected by lucky draw and patient was selected by societies from that province. Federal was selected first and Rumaisa Noor will be the first patient to attend executive meeting inshaAllah next year.
Personally I and few other fellows think one patient from each province will not harm the federation but it will definitely help patients and parents and above all thalassemia community.
Anyhow second day of conference was on these topics:
1) Non transfusion dependant thalassemia
2) Iron chelation in NTDT
3) Role of hydroxyurea in thalassemia intermedia
4) Complications specific to NTDT
5) Puberty issues of thalassemia
Since thalassemia intermedia patients receive less transfusion their ferritin remain in control too, they don’t need aggressive iron chelation like thalassemia major, just like major their iron chelation drugs are same too, if they are on hydroxyurea then they need to be careful otherwise they can lead a much longer and better life.
Role of hydroxyurea I have discussed earlier too, if you have thalassemia intermedia you should give a try to hydroxyurea.
Complications of NTDT as I learnt are less severe than major, less transfusion, less iron, fewer troubles.
The interested and much needed topic was puberty issues. How a patient should take care of his/her self. How to maintain their growth, girls for breast development and menstrual cycles and boys for testicles growth etc?
If there is a delay in growth then it can be treated with medicines now. It is no longer a big issue. Patients with the hormones treatment are getting married, having kids and if they get proper care then no one can believe they are thalassemia patients.
Later on the societies from all over Pakistan shared the progress of thalassemia prevention programs.
Dr Gerald Mason (UK) discussed about recent advances in prenatal diagnosis. Since last few years we have the prenatal screening facility in Pakistan. I just hope parents take advantages of such facilities and don’t increase the number of thalassemia patients in Pakistan.
Lastly all the NGOs/societies working for thalassemia were invited to share their achievements and work with the audience and indeed it was a great pleasure knowing so many NGOs/societies working tirelessly for us/patients all over Pakistan.
FAiTh was given a chance too to showcase our work. Being a patient myself I think running the cause and generating funds for fellow needy patients is a very small contribution to the thalassemia community.
Below are the changes I think TFP should make:
1) Advertise about conference as much as possible so that more and more patients/parents/doctors can attend and get benefit.
2) Invite international speakers on different topics, we have gone too far from the phase of “what is thalassemia”, we need more knowledge please.
3) Societies should forward their outstanding patients name to get recognition, don’t give gold medal but at least sharing their success stories/achievements will boost other patients moral.
4) Invite media, so more awareness can be created.
5) 1 patient from each province should be given place in the meetings.
6) Patients group should be formed [in process after long discussion]
7) Arrange workshops all over Pakistan, it’s saddening when I get calls from different places of Pakistan and they ask me how can they save their kids lives and when I get to know they know nothing about iron chelation. Please give them awareness.
8) Keep check and balance on all the societies for proper screening, safe blood transfusion, iron chelation and growth assessments.
9) Thalassemia centers from all over Pakistan MUST be member of TFP and if any center is not a member then they must not be given permission to work in Pakistan for the safety of patients.
All doctors and fellow thalassemics who attend the conference please do share your notes/views/pictures or any information / knowledge you want to share 🙂
HYDERABAD: The next time somebody from your family requires blood transfusion, cross your heart. The chances are that the blood procured from a blood bank will not be free of viruses that can cause deadly infections. “It’s a bloody mess at the state’s blood banks and only a few well-known names are free of problems,” said a source familiar with the state of affairs. “Many unsuspecting patients contract deadly disease like HIV and Hepatitis B and C because of blood transfusions,” he added.
The worst hit are those who need blood routinely, such as children suffering from thalassemia. In the last four years as many as eight thalassemic children contracted HIV and Hepatitis C & B infections during the course of blood transfusion. Rajesh Popli, secretary, Society for Thalassemics, says that at least five thalassemic children tested positive for HIV in the last two years alone. “As per our information, these children are from Hyderabad, Visakhapatnam, Nizamabad, Medak and Krishna,” said Popli.
That the stock of blood in many banks is suspect is corroborated by the government itself. The Drug Control Administration’s recent state-wide survey of 219 blood banks found that just about a handful of them met the quality standards. The lapses included non-functioning air conditioners and the use of “rapid testing techniques” by most banks (to reduce the cost of screening) which could miss detecting these fatal infections.
“We found that around five blood banks of the total 219 were following the quality standards,” said a DCA top official, adding that notices were being issued to the erring banks to beef up their standards. And that’s all in the form of punishment for these banks!
As per norms, blood should be screened for communicable diseases including HIV I & II, Hepatitis C & B, malarial parasite and Venereal Disease Research Laboratory (VDRL) test. “The problems are cropping up because of the absence of technically qualified persons. Pathologists are mandatory but many are unwilling to work in blood banks. Besides, blood screening costs are high,” said Dr V Saraswati, who is with NTR Blood Bank, which doctors say complies with most standards (along with Chiranjeevi Blood Bank). Some blood banks, as per the DCA survey, were even found lacking equipment.
While pathologists are meant to declare blood ‘safe’ for use, they prefer working with hospitals over blood banks. The banks are also wary of hiring them because of the high salaries they demand. Focused on cutting cost of screening processes, blood banks flourish thanks to an indifferent government. Worse, the government shies away from introducing tests that could help such as the PCR test which can detect infections even during the window period (the period from the time of contracting infection to testing positive for it). “The PCR test charges range between Rs 2,000 to 3,000 per test and it is not feasible to do this test on lakhs of units,” said an official from AP State Aids Control Society.
Courtesy by: expressbuzz.com
HYDERABAD: The Thalassemia and Sickle Cell Society, a non-profit group that seeks to provide relief to the tens of thousands of children afflicted with the diseases annually, will inaugurate a blood bank at Chatta Bazar here on March 20.
Thalassemia and sickle cell are inherited blood disorders that affect haemoglobin production. More than 40,000 new victims are diagnosed yearly. The diseases require frequent blood transfusion, often once in a fortnight, for the patients to live. This requires children with the disorders to receive dozens of blood transfusions before their third birthday.
At a press conference here today, parents of afflicted children explained the stresses under which they had to operate.
“We feel like no one will help. We constantly have to worry about where the next transfusion will come from,’’ said a parent. To mitigate these concerns, the Thalassemia and Sickle Cell Society aims to bring several operations, including diagnosis, transfusion and storage of blood under their new facility. It also seeks to pair patients with regular donors to reduce the chances of bloodborne infections. Any person can adopt a thalassemia child and become what they call `blood brothers’.
The new blood bank’s location was stragetically chosen to cater to Hyderabad’s most disadvantaged children. Most of Hyderabad’s blood banks are situated in the new city, and very few are south of the Musi.
Courtesy by: cnylink.com
Thirteen-month-old Sophia Stagnitta, of East Syracuse, will grow up receiving blood transfusions.
Little Sophia was diagnosed with Beta-Thalassemia Major, also known as Cooley’s Anemia, when she was 11 days old, her mom Jennifer said last week at Skaneateles High School. Jennifer and Sophia were in town for a blood drive being held in Sophia’s honor at the school where her dad, Scott Stagnitta, is a middle school teacher.
Beta-Thalassemia Major is a genetic blood disorder that prevents or greatly reduces the body’s ability to produce adult hemoglobin and causes anemia, which is a lack of iron.
Jennifer said Sophia thankfully has not had to have any blood transfusions yet, but each month the family goes to University Hospital’s Pediatric Hematology/Oncology department to have the toddler’s hemoglobin level tested.
“Her hemoglobin level has been up and down, but in the normal range,” Jennifer said.
While Sophia’s levels have fluctuated between 10 and 11, once the levels drop to a 6 or 7, she will need at least one transfusion each month. But with transfusions also come more concerns as receiving blood can cause an iron overload, which in turn will mean Sophia will have to endure therapies to remove iron from her blood. The two methods of removing iron are either through taking a pill orally, but not everyone can take it, or through a method called Chelation Therapy, which requires a needle to be inserted into the body to aid in removal of heavy metals.
“The only way to cure this is a bone marrow transplant,” Scott said, but even then the marrow has to be an identical match from a sibling.
The entire process for a bone marrow transplant would be seven months or more.
“If we were going to do it that way, they want to do it while she’s young,” Scott said.
The younger she is, the less likely it will be that her blood is overloaded with iron, and she would have to be closely monitored.
One issue that stands in the way of even looking into bone marrow transplant is Sophia is Scott and Jennifer’s first child — she has no siblings to be matched to.
“We definitely want another child,” Jennifer said, adding she and Scott have met with a genetic counselor and are aware there is a 25 percent chance of having a second child with Beta-Thalassemia Major. “It’s a lot to think about.”
According to Jennifer, after being diagnosed, her hematologist said Sophia may need her first transfusion at 6 months old but has been fortunate to come this far without having to receive blood.
“I think the hardest part is looking at her and knowing something is wrong,” Scott said.
But for the unknowing person to look at Sophia, she appears to be a normal, active and perfectly healthy 1-year-old. She’s full of energy and on target as far as height, weight and other such benchmarks.
“She’s very active. You’d never know what’s going on,” Jennifer said.
While the blood disorder is rare in the United States and Canada with only around 700 people being affected by it, Jennifer said that’s not the case in places like Italy where a large portion of the population has some form of Thalassemia. Both Jennifer and Scott are of Italian descent and therefore there was a chance they may pass the disorder on to Sophia.
There was no prenatal screening for the disorder or much reason for concern when Jennifer was pregnant, though, as both parents are young and in good health.
For the time being, the young family is living life as normal with a monthly trip to the hematologist.
“We’ll try to keep it normal for her,” Jennifer said.
One thing they have learned is not to dwell on it. Another is to be thankful for things like the Red Cross blood drive on Thursday, where there was an overwhelming amount of support from the community for Sophia.
Sophia may not receive blood donated last week by Skaneateles’ teens and teachers, but the donations will help replenish the blood supply and will go to those who need blood now.
According to statistics from the American Red Cross, at some point in our lives, 80 percent of us will require a blood transfusion and only 4 percent of eligible donors donate.
“We’re honored they would think of Sophia,” Jennifer said.
Courtesy by: beaumontenterprise.com
Larry Tidwell had been to the Whataburger in Winnie at least a dozen times on the way back from recruiting trips to Houston, and it had never taken him this long to get his food.
With a bus full of schoolchildren inside that October day keeping the kitchen staff busy, all the Lamar women’s basketball coach could do was stare at a flyer on the drive-through window that had a picture of a young boy in a cowboy hat on it.
The flyer was for a benefit to help defray the cost of medical expenses for 3-year-old Lane Hoffpauir’s blood transfusion.
“I couldn’t stop looking at it,” Tidwell said. “I’m an old cowboy myself, so the hat really got to me. I knew I just had to help this kid, so I took down the number and called his mom.”
Lane, now 4, has had more than 100 blood transfusions in his life, battling beta thalassemia, an inherited blood disease in which the body makes an abnormal form of hemoglobin, the protein in red blood cells that carries oxygen, leading to destruction of red blood cells and anemia.
Tidwell and the Lady Cardinal basketball team decided to adopt Lane for the season. After adopting Lane, the basketball team sold different ticket packages, which included tickets to Saturday’s game and extras such as golf and gumbo, to help the fundraising efforts at his benefit on Oct. 17. The basketball team’s efforts raised almost $15,000 and so far the family has received almost $100,000 in donations to help with the cost of his bone marrow transplant.
Courtesy by: wggb.com
WEST SPRINGFIELD, Mass. (WGGB) – When you meet Essa Khairi you can’t tell that beneath his playful spirit and infectious laugh, the 11-month-old suffers from a rare genetic disorder with potentially life-threatening complications. He was recently diagnosed with thalassemia major. His body produces dangerously low levels of hemoglobin, depriving his tissues of crucial oxygen.
To maintain his health, Essa is dependent on monthly blood transfusions. While effective, the high levels of iron he receives have potentially dangerous consequences for his heart and liver. The only cure for his condition is a bone marrow or cord blood transplant. Essa’s parents were discouraged to learn that there are very few people of Southeast Asian descent in the National Bone Marrow Registry. It is unlikely they will find a bone marrow donor so the Khairis have decided to proceed with a cord blood transplant.
Dr. Talal Khairi contacted a doctor at Duke University specializing in cord blood transplants. He was advised that while there are potential unrelated donors, Essa’s best chance of a match is a sibling. Essa’s parents are now preparing to have a third child in hopes that the umbilical cord blood will be a match for Essa. If not, they will proceed with looking for an unrelated cord blood donor.
While the Khairis are no longer actively looking for a bone marrow donor for their son, they are still hoping to organize a local bone marrow registry drive. They are calling on people of Southeast Asian descent to come forward and register, so that if someone is in need of a transplant there is a potential match waiting.
Dr. Khairi’s colleagues at Baystate Medical Center in Springfield are helping him organize the drive. Stay tuned for more information.