Hoyeraal Hreidarsson syndrome

30-month-old Girl Child Survives Hoyeraal Hreidarsson Syndrome

2 Feb 2021 Health Care, Medical , , ,

“Never lose hope, storms make people stronger and never last forever”- Roy T. Bennett. 

Meera’s (name changed) sufferings had no end. An 8-month premature girl born in Jan 2016, was admitted for 8 days in the hospital. She barely weighed 1.78 kgs. After six months, severe fever and rashes led to a 10 day-long hospitalization. Complete blood count (CBC) tests were performed to evaluate her health. In the CBC test, her platelets were exceedingly low.  Post Bone Marrow aspiration, Meera was diagnosed with Thrombocytopenia – abnormal low-levels of platelets. Eventually, the fever subsided and she was discharged. 

As Meera’s condition was not improving, parents consulted the family pediatrician and were suggested to transfer her to a corporate hospital in Hyderabad. Another CBC test was done. The platelet count was low – 50,000. They were advised to follow up once every month with CBC reports as no treatment was available. 

Meera’s condition was getting worse. She had a shocking weight loss after which the doctor recommended Bone Marrow Aspiration/ Biopsy for correct diagnosis. Diagnosed with Thrombocytopenia with Anemia, Meera’s parents were suggested to wait till her platelet dropped to 20,000.

In August 2017, Meera had a high fever and a severe case of Thrombocytopenia with Anemia. SDP platelets were transfused due to extremely low platelet count. Platelets/blood were transfused every month. It was a very hard time for the family. They could not watch their daughter’s suffering. Fortunately, Meera’s parents got in touch with AHIRAA donors on WhatsApp. A doctor at a corporate hospital referred Meera’s parents to Dr. S.K.Gupta for the possibility of a Bone Marrow Transplant. 

A doctor from the Genetic Department at a public hospital proposed a Genetic Test. The doctor advised them to go ahead with the Bone Marrow Transplant. The possibility of a BMT frightened Meera’s parents. With the risks involved and the finances, the chances of BMT looked bleak. Meera’s grandparents were afraid as no one gave a 100% guarantee of the procedure’s success. 

Meera’s parents contemplated and tried homeopathy medicine. 12 platelets transfusion and 8 blood transfusions were performed between Aug 2017-May 2018. During this period Meera had a high fever and caught infections.

The family finally consulted Dr. S.K.Gupta who explained the procedure and assured them that the BMT has a 70% success rate. 

The HLA match of the parents was negative. Both of them were half-matched. After the tests, the family was heartbroken. Dr. S.K.Gupta advised the HLA testing of Meera’s 16-month-old sister. The parents were panicking as they couldn’t process the thought of a 16-month-old baby donating stem cells. Dr.S.K.Gupta assured that the procedure will cause no harm to their younger daughter. The 16-month-old sister was a 100% HLA match. Meera’s family’s happiness knew no bounds. 

On 13 May 2018 Meera was admitted to the Hematology BMT Institute International located in Hyderabad. The parents were asked to arrange 5/5 donors for platelets and WBC. 

Chemotherapy began and lasted for a week. It was a difficult time for the family as both of their daughters were involved in the BMT. Stem Cells from Meera’s sister were collected and the procedure started. After 12-13 infusions, Meera showed positive signs. Unlike other transfusions that were performed in different hospitals; this time Meera showed no signs of fever or infections. No platelets or WBC were transfused during the 20-day hospitalization. Parents were delighted as they finally took a breath of relief.

According to Dr. S.K.Gupta, the initial 6 months- 1 year period is crucial after BMT. The patients might need re-admission. After 45 days of the procedure, Meera had a severe infection and her platelets dropped to 40K-50K. She recovered quickly. It’s been six months now and she has been doing great.

Meera was suffering from Hoyeraal Hreidarsson syndrome which was diagnosed by the team at Hematology BMT Institute International. 

What is Hoyeraal Hreidarsson Syndrome?

Hoyeraal Hreidarsson syndrome is a rare disease that is even more critical and complicated than cancer. It is an extremely rare multisystem X-linked recessive disorder.

Meera’s parents hold Dr. S.K.Gupta and the team at HBII in high regard. Parents were apprehensive about the cost of the BMT as they came across hospitals that charged an enormous amount. A government employee at a police department could only dream of saving his child with a BMT procedure. 

Dr. S.K.Gupta, the founder of Hematology BMT Institute International believes that every life is worth saving. Hematology BMT Institute International advocated no unnecessary admissions, medications, and blood transfusions. Promoting affordable healthcare and superior quality, Hematology BMT Institute International is making procedures like BMT more accessible to the needy.

 

Haploidentical bone marrow transplant

What is Haploidentical bone marrow transplant?

12 Jan 2021 Health Care, Medical , ,

A haploidentical bone marrow transplant is performed when the donor exactly matches half of the recipient’s DNA. Haploidentical bone marrow transplant is a type of allogeneic transplant. In an allogeneic transplant, the cells from donors are transplanted in the recipient to suppress the disease-causing cells or elements and restore the immune system of the patient. The key to a successful allogeneic transplant is finding the right donor. In most cases, the donors are the blood relatives of the patient. The match is proved through tissue typing. Human Leukocyte Antigens (HLA)  are the proteins that are used for tissue typing. These proteins are present in white blood cells and tissues. HLA is present almost all over the body. Donors closely matching your HLA or umbilical cord blood can be transplanted. Healthy, blood-forming cells from a half-matched donor are transplanted to replace the unhealthy ones of the recipient. This is a Haploidentical bone marrow transplant. The donor is typically a family member in Haploidentical bone marrow transplant.  The donor matches exactly half of the HLA of the donor. 

Who can be a haploidentical donor?

The patient’s mother, father, or offspring are half-matched or haploidentical donors. Siblings have a 50% chance of being haploidentical for each other. Parents are always haploidentical to their children. It is highly unlikely for uncles, aunts, and cousins to be half-match of the patient. 

Benefits of a haploidentical bone marrow transplant

For patients suffering from rare disorders, haploidentical stem cell transplants are a blessing. It is a boon especially for patients suffering from blood disorders that do not have any cure yet. Since this medical procedure does not need a complete HLA match of the donor’s tissue, it is a treatment option for 70% of the patients suffering from rare and complex disorders. Haploidentical bone marrow transplant gives a chance to live for the patients who do not have an HLA-identical sibling donor. 

A half-match donor saves a lot of hassle for the family and doctors. It avoids the need to find an alternative donor who is unrelated to the patient. It also expands the donor pool who are not a complete match for the receiver. 

In a bone marrow transplant, the patient’s immune system is wiped out so that the immune system does not reject the donated marrow. It is done to avoid the host -versus- graft effect as the response of the donor immune system can harm the new immune system during the marrow transplant. In haploidentical bone marrow transplant, the bone marrow suppresses the immune system instead of wiping it out. It is viable to perform this procedure on children with benign hematologic disorders if no donors are available with engraftment rates of 70%, durable graft in 67%. The overall survival of haploidentical bone marrow transplant is 60%.

It is generally assumed that the side-effects of a haploidentical transplant are higher than a full-matched transplant if the procedure is performed in a busy institute. In Hematology and BMT Institute International every procedure is performed with precision and patients are given utmost care. Dr. S.K Gupta, the founder of Hematology and BMT Institute International has 15 years of experience and is an expert in Haploidentical bone marrow transplant. He started his medical career as a nursing student and has been trained at various reputed & prestigious healthcare Institutions in India.

If you are looking for a medical institute that provides quality healthcare with utmost dedication and care, head to Hematology and BMT Institute International in Hyderabad. It is one of the most advanced and efficient healthcare centers in India. 

 

Bone Marrow and Bone Marrow Transplantation

BONE MARROW AND BONE MARROW TRANSPLANTATION

27 Oct 2020 Medical , ,

Bone marrow is the thick, spongy tissue within the bones that develops red and white blood cells and platelets. As bones are present everywhere in the body so bone marrow will also be present in all parts of the body. Unspecialized Hematopoietic Stem Cells are also present in the bone marrow. These stem cells or mother cells have two properties – 

  1. Differentiation into different blood cells or daughter cells, and 
  2. Renewal into new stem cells or mother cells.

Many cancers may hinder the average production/ renewal and differentiation of such stem cells.

The bone marrow transplant is essential if the bone marrow is not healthy enough or cannot work properly due to hematological disorders or blood cancer. Bone Marrow Transplantation (BMT) is done to replace weak bone marrow with healthy bone marrow. Unlike solid organs (kidney, liver, heart) transplant, this is not a surgical process as the majority of us know/ understand. This is purely a medical procedure in which transplantation of new blood-forming hematopoietic stem cells replaces the existing weak bone marrow hematopoietic stem cells. As a result, new blood cells will be formed and will ultimately contribute to new marrow development that will cure and prevent other disorders. For bone marrow transplantation, healthy stem cells can either be extracted from the patient’s own body (autologous BMT) or from an HLA matched family or unrelated donor (allogeneic BMT). Frankly, if we speak autologous BMT is not at all a BMT as here we are using patients own hematopoietic stem cells. In autologous BMT use of the patient’s own stem cells is done to rescue him from the side effects of high-dose chemotherapy.

Dr. S.K. Gupta is a renowned Hematologist in India, practicing as a hematology consultant at Hematology and BMT Institute International with extensive experience of over 15 years in the field. He has performed over 250 Bone Marrow Transplants for patients suffering from various Hematological blood disorders in India and across the globe. He is a specialist in the treatment of all blood, bone marrow, lymph nodes, and spleen disorders. He and his team are comfortable in treating children’s also. Let’s look at some interesting points that he came across while treating one of his patients.

In 2015, a girl was diagnosed with blood cancer and had relapsed three times.  A curative therapy was used through Allogeneic Bone Marrow Transplantation as it was the mother who donated the stem cells which was only half matching  (Haploidentical )and they did not have a fully matched HLA (Human Leukocyte Antigens) donor. Due to this reason, all the other oncologists were reluctant to go on with the procedure because Allogeneic BMT/HSCT is more complicated and demanding than Autologous BMT/HSCT. This is a recent and much-needed advancement in the field of HSCT since the first successful HSCT was done 40 years ago (1968). It was even more challenging to conduct regular services due to COVID-19, especially when the hospital is treating active COVID-19 patients. Dr. S.K. Gupta not only provides excellent treatment but also patiently answers all the queries related to the illness. He motivates and urges people not to give up. 

 

There are few myths about BMT which need to be busted:

 

Patient perspective:-

  •  It is a Surgical Procedure.
  •  Surgery is the last resort in treatment, we should try some medicines and if they don’t work only then should we go with this treatment.
  • A matching blood group is a must.
  • I will receive lifelong treatment.
  • It’s a very high-risk procedure

Donor perspective:-

  • The donor will have a lot of side effects, the blood-producing capacity will go down, immunity will go down, the capacity to fight COVID19 will go down, etc.
  • Umbilical cord Hematopoietic Stem Cells are stored for the child, so there is no need to worry.

Hematology and BMT Institute International is a global healthcare hospital that provides quality care to the people. We provide guidance and treatment as per recent developments across the world. We follow strict protocols when using the equipment and while handling patients by keeping in mind the sensitive nature of the patient’s medical conditions.

Visit our website https://hematologybmt.com/ or contact us on +917780297660 to know more about hematology and the kind of treatments that Dr. Gupta provides to his patients. 

 

 

 

 

 

Search

+
Open chat