BLEEDING IN PATIENTS WITH HEMOPHILIA. CME#2

BLEEDING IN PATIENTS WITH HEMOPHILIA

Hemophilia includes bleeding epitomized by limb- or life-threatening bleeding symptoms, such as hemarthrosis, soft-tissue bleeding, muscle hematomas, retroperitoneal and intracerebral hemorrhage, and postsurgical bleeds. To some degree, the type and the site of bleeding are age dependent (owing to characteristic developmental milestones, such mouthing of objects and mobility) and severity of disease dependent. Neonates with severe hemophilia most commonly present with bleeding after circumcision but may also present with intracranial hemorrhage. In toddlers, bleeding from minor mouth injuries and intracranial and extracranial hemorrhages may occur after minor injuries. As the toddler starts to become more mobile, bleeding into soft tissues, such as buttock hematomas and muscle and joint hemorrhages, may become evident.

Kulkarni and colleagues [1]analyzed infants younger than 2 years of age and found that of 580 children with hemophilia studied, nearly 60% were diagnosed within 3 days of birth, 75% in the first month of life, and 90% by 8 months of age. The diagnosis was established earlier in infants whose mothers were known carriers (median, 1 day) or who had a documented family history (median, 2 days) than in those who presented with bleeding (median, 7 days) or whose maternal carrier status was unknown (median, 152 days). Postcircumcision bleeding was the most common site of first bleed (27.4%), followed by head bleeds in 17% (of which 36.4% had an intracranial hemorrhage).[[1]

Because hemophilia A and B are X-linked conditions, the disease occurs in males and is transmitted by females who may be heterozygous for the gene mutation. Historically, it was assumed that carriers were asymptomatic for bleeding; however, it recently has come to light that many carriers do experience bleeding symptoms. Hemophilia A and B carriers, even those with normal hemostatic levels (>40%), have an increased bleeding tendency, including prolonged skin bleeding, heavy menstrual bleeding, oral bleeding, and excessive bleeding after dental procedures and surgery.[2-4].  Additionally, Sidonio and colleagues[5] showed that carriers of FVIII or FIX deficiency enrolled in the Universal Data Collection project had a reduced mean joint range of motion compared with historic controls from the Normal Joint Study. The data from this study suggest that subclinical bleeding may occur as early as adolescence.

1. Kulkarni R, Soucie JM, Lusher J, et al. Sites of initial bleeding episodes, mode of delivery and age of diagnosis in babies with haemophilia diagnosed before the age of 2 years: a report from The Centers for Disease Control and Prevention's (CDC) Universal Data Collection (UDC) project. Haemophilia. 2009;15:1281-1290. Abstract
2. Olsson A, Hellgren M, Berntorp E, Ljung R, Baghaei F. Clotting factor level is not a good predictor of bleeding in carriers of haemophilia A and B. Blood Coagul Fibrinolysis. 2014;25:471-475. Abstract
3. Paroskie A, Oso O, Almassi B, DeBaun MR, Sidonio RF Jr. Both hemophilia health care providers and hemophilia a carriers report that carriers have excessive bleeding. J Pediatr Hematol Oncol. 2014;36:e224-e230. Abstract
4. Plug I, Willemse J, Rosendaal FR. Bleeding in carriers of hemophilia. Blood. 2006;108:52-56. Abstract
5. Sidonio R F, Mili FD, Li T, et al. Females with FVIII and FIX deficiency have reduced joint range of motion. Am J Hematol. 2014;89:831-836. Abstract

Hemophilia management guidelines CME .#1

INTRODUCTION

Hemophilia A and B are X-linked recessive disorders of coagulation characterized by deficiency of Factor VIII (FVIII) and Factor IX (FIX). Across all ethnic groups, the prevalence of hemophilia A is estimated to be 1 in 5000 males, and that of hemophilia B is 1 in 30,000.[ 1] It is estimated that hemophilia affects approximately 400,000 individuals in the world. Hemophilia A is more common than hemophilia B and represents 80% to 85% of the total hemophilia population. This article reviews the latest understanding of hemophilia and the significant progress that has been made in diagnosis and management during the past several decades.

CLASSIFICATION OF SEVERITY

The severity of disease is classified based on the residual plasma levels of circulating FVIII and FIX. The disease is classified as follows: severe if levels are <1%, moderate for levels of 1% to 5%, and mild for levels between 5% to 40% of normal.^[2] There are some limitations to this classification in that it does not recognize the clinical heterogeneity in bleeding observed in individuals with levels <1%.

It has been observed that 10% to 15% of patients with severe hemophilia as defined by factor levels may have a milder clinical profile.^[3] Additionally, this classification may not distinguish between the bleeding profiles of hemophilia A and B.^[4] Based on inpatient hospital admissions, factor consumption, and rates of joint arthroplasty, some reports suggest that hemophilia B may have a milder bleeding phenotype than hemophilia A,^[5-7] although a more recent study demonstrated that age at first bleed, age at first joint bleed, and age at first factor exposure were similar in patients with hemophilia A vs B.^[8] An additional issue that is unresolved is the classification of individuals with levels of 40% to 50%. Another important point that the current classification does not consider is the potential discrepancy between the 1- and 2-stage assays for FVIII (FVIII levels were lower with the 1-stage assay) in some patients with mild hemophilia A.^[9]

Despite these limitations, the current classification is widely accepted, and there is good correlation between age at diagnosis, age at first bleed/first joint bleed, and age at first treatment and the classification of disease severity based on circulating factor levels in most patients.^[ 10] In general, individuals with mild hemophilia bleed only in response to trauma, such as that experienced during surgery, tooth extractions, or major injuries, whereas patients with moderate hemophilia bleed excessively after relatively minor trauma, and those with severe hemophilia may bleed spontaneously or after trivial trauma.

Long walk to ...freedom. Part One: Getting admitted

Some believe that you become a doctor only after you have got an MBBS degree. In reality, from the time you are admitted into a medical school you become a part of the doctor’s community… the people in white coats!

 ’I solemnly pledge myself to consecrate my life to the service of humanity.

I will use my best judgement to help the sick and do no harm.

I will not give fatal drugs to anyone, even if asked, nor will I suggest any such thing.

I will not divulge the secrets of my patients regarding them as holy……’

There is something very hypocritical about this Hippocratic oath. Hippocrates, the famous Greek physician, must have formulated the oath for his disciples in the field of Medicine in the true interest of humanity and to uphold the sanctity of this noble profession. But now this oath-taking ceremony has been glamorized to befit white coat ceremonies to appease nostalgic minds, or worse still to harass doctors in court.

But then nostalgia and harassment run high in any Medical school. The Professors love to recall the days when they, as medical students, had studied till late in the night and spent the better part of this glorious period of one’s life in the dissection halls, the hospital wards, operation theatres or the library. Having been through this gruesome phase, some of them are sympathetic to the plight of medical students. But then there are some who take it out on this fast emerging strain of stress-resistant medical students who believe they can bunk classes, download video lectures and amazing new mobile apps about the Anatomy of the human body, attend concerts, have a nice time at McDonald’s and study Pharmacology with music in the background.

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http://healthmad.com/medicine/in-white-coats-2/

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Metabolic theory of septic shock

Septic shock is a life threatening condition that can develop subsequent to infection. Mortality can reach as high as 80% with over 150000 deaths yearly in the United States alone. Septic shock causes progressive failure of vital homeostatic mechanisms culminating in immunosuppression, coagulopathy and microvascular dysfunction which can lead to refractory hypotension, organ failure and death. The hypermetabolic response that accompanies a systemic inflammatory reaction places high demands upon stored nutritional resources. A crucial element that can become depleted early during the progression to septic shock is glutathione.
Glutathione is chiefly responsible for supplying reducing equivalents to neutralize hydrogen peroxide, a toxic oxidizing agent that is produced during normal metabolism. Without glutathione, hydrogen peroxide can rise to toxic levels in tissues and blood where it can cause severe oxidative injury to organs and to the microvasculature. Continued exposure can result in microvascular dysfunction, capillary leakage and septic shock .
Read about the Early Goal Directed Therapy EGDT for sepsis management.
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Read about the link between iron and vitamin B12 deficiency and panic attacks. http://adf.ly/1AHkuI

On the off chance that you experience the ill effects of tension or get occasional panic attacks marked by episodes of hyperventilation, you could only be encountering the symptoms of a basic supplement inadequacy that is effortlessly correctable, as per Jonathan Benson of Natural News.

This unquestionably seems to have been the situation with 21 individuals who took an interest in a late study based out of Japan, which recognized an absence of both vitamin B6 and iron among members who experienced panic or hyperventilation episodes.

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Be like the river

Sometimes all  you can do, is not to
dream, expect or obsess.
All you can do, is go with the flow,
And hope for the best.

Going with the flow....

Finding work-life balance is like juggling responsibilities. As long as you keep doing it, nobody cares. Once you stop, it all comes crashing down. And you are the only one to be blamed. You chose to live life that way!

  I often wonder, is there such a thing as the perfect balance, the perfect woman, the perfect family, the perfect food,or the perfect relationship?

Maybe in some fairytale.  But real life is not a bed of roses but a soap opera. Everything changes. The key to survival is 'going with the flow'.

Priorities change daily and as full time working mothers, you have to flow through the demands and challenges as best we can. It won't ever be perfect. And it won't ever be balanced. However, if you are flexible and let things flow as they need to, you won't be worked up over ensuring everything is perfect.

'There are times when my kids or husband take full priority and I have to take care of them while work has to wait. Then there are other times that work is priority - whether it's a big project, meetings or travel, that's what comes first. And there are times that friends or extended family need my help. Balance really doesn't fit any of these scenarios....

 At times, I think I have nailed it and I'm doing really well in all areas. However, there are other times that I feel I haven't spent enough meaningful time with my kids or my husband and I'm falling behind at work. There have also been times that I have felt I haven't been there for my extended family or friends the way that I should be. The constant stress of balancing all of the above can certainly take a toll..' 

Be like the river.

Sheryl Sandberg, COO of Facebook, wrote in her book Lean In:

“Trying to do it all and expecting that it all can be done exactly right is a recipe for disappointment. Perfection is the enemy.”

Discussions or documentations? The way forward.

Discussions or documentations
Surveys suggest that time constraints push doctors to spend less time with the patient and more time documenting records. Important as documentation may be, it ends up with a waste of unnecessary investigations that could have been avoided had the doctor spent more time with the patient; taking a detailed history and doing a thorough examination. Many times we have been forced to look for Malarial parasites in smears from afebrile patients, or do bone marrow aspirations of thrombocytopenic patients (with low platelet counts) only to find platelet clumps on peripheral smears.
Have you ever done an investigation that you thought was unnecessary?

The doctor-in-law phobia