jueves, 21 de julio de 2016

CONTROLLED DRUGS


The Misuse of Drugs Regulations 2001 (and subsequent amendments) define the classes of person who are authorised to supply and possess controlled drugs while acting in their professional capacities and lay down the conditions under which these activities may be carried out. In the regulations drugs are divided into five schedules each specifying the requirements governing such activities as import, export, production, supply, possession, prescribing, and record keeping which apply to them.
  • Schedule 1: includes drugs such as lysergide which is not used medicinally. Possession and supply are prohibited except in accordance with Home Office authority.
  • Schedule 2: includes drugs such as diamorphine (heroin), morphine, nabilone, remifentanil, pethidine, secobarbital, glutethimide, the amfetamines, sodium oxybate, and cocaine and are subject to the full controlled drug requirements relating to prescriptions, safe custody (except for secobarbital), the need to keep registers, etc. (unless exempted in Schedule 5). 
  • Schedule 3: includes the barbiturates (except secobarbital, now Schedule 2), buprenorphine, mazindol, meprobamate, midazolam, pentazocine, phentermine, temazepam, and tramadol. They are subject to the special prescription requirements. Safe custody requirements do apply, except for any 5,5 disubstituted barbituric acid (e.g. phenobarbital), mazindol, meprobamate, midazolam, pentazocine, phentermine, tramadol, or any stereoisomeric form or salts of the above. Records in registers do not need to be kept (although there are requirements for the retention of invoices for 2 years).
  • Schedule 4: includes in Part I benzodiazepines (except temazepam and midazolam, which are in Schedule 3), zaleplon, zolpidem, and zopiclone which are subject to minimal control. Part II includes androgenic and anabolic steroids, clenbuterol, chorionic gonadotrophin (HCG), non-human chorionic gonadotrophin, somatotropin, somatrem, and somatropin. Controlled drug prescription requirements do not apply and Schedule 4 Controlled Drugs are not subject to safe custody requirements.
  • Schedule 5: includes those preparations which, because of their strength, are exempt from virtually all Controlled Drug requirements other than retention of invoices for two years

Standards and safety checks

NMC(standards for medicine management)


-Controlled drugs are kept in a locked cabinet and the keys are carried by the nurse in charge (access to the keys and the CD cabinet is restricted to authorised registrants). CD stationery which is used to order, return or distribute controlled drugs should be kept in the locked cabinet. No other medicines or items to be stored in the controlled drug cupboard.
-There should be a list of the CDs to be held in each ward as stock items, only the CDs listed may be routinely requisitioned or topped-up.
-A copy of the signature of each authorised signatory should be available in the pharmacy department for validation.
-When CDs are delivered to a ward they should be handed to a designated person, under no circumstances should they be left unattended. After the delivery, the registrant in charge and a witness (another registrant) should check the CDs against the requisition, place the CDs in the CD cupboard, enter the CDs into the ward controlled drug record book, update the running balance and check that the balance tallies with the quantity that is physically present.
-All entries in the controlled drug record book must be signed by two registrants.
-Checking the balance in the controlled drugs register against current stock should be done twice a day, recording stock checks along with the date and signature of both health professionals carrying out the check. 
-The registrants should record the amount given and the amount wasted (if there is any) when administering a CD.
-Audits should be carried out to ensure that the record keeping is up to date and correct.



Sources:
-http://www.evidence.nhs.uk/formulary/bnf/current/guidance-on-prescribing/controlled-drugs-and-drug-dependence
-Controlled drugs: safe use and management(2016) NICE guideline NG46
-https://www.nmc.org.uk/globalassets/sitedocuments/standards/nmc-standards-for-medicines-management.pdf
Images: kwizoo.com, www.officestationerywarehouse.co.uk, www.davidwolfe.com, www.opiates.com.

martes, 12 de julio de 2016

OBESITY


Obesity means having too much body fat. It is not the same as being overweight, which means weighing too much. A person may be overweight from extra muscle or water, as well as from having too much fat. Both terms mean that a person's weight is higher than what is thought to be healthy for his or her height.

Causes
Taking in more calories than you burn can lead to obesity. This is because the body stores unused calories as fat. Obesity can be caused by: 
-Eating more food than your body can use 
-Drinking too much alcohol 
-Not getting enough exercise. 

Many obese people who lose large amounts of weight and gain it back think it is their fault. They blame themselves for not having the willpower to keep the weight off. Many people regain more weight than they lost.
Today, we know that biology is a big reason why some people cannot keep the weight off. Some people who live in the same place and eat the same foods become obese, while others do not. Our bodies have a complex system to keep our weight at a healthy level. In some people, this system does not work normally.
The way we eat when we are children can affect the way we eat as adults.

The term eating disorder means a group of medical conditions that have an unhealthy focus on eating, dieting, losing or gaining weight, and body image. A person may be obese, follow an unhealthy diet, and have an eating disorder all at the same time.

Sometimes, medical problems or treatments cause weight gain, including: 
-Underactive thyroid (hypothyroidism) 
-Medicines such as birth control pills, antidepressants and antipsychotics. 

Other things that can cause weight gain are: 
-Quitting smoking: many people who quit smoking gain 4 to 10 pounds in the first 6 months after quitting 
-Stress, anxiety, feeling sad, or not sleeping well 
-Menopause: women may gain 12 to 15 pounds during menopause 
-Pregnancy: women may not lose the weight they gained during pregnancy.



DRUGS USED IN THE TREATMENT OF OBESITY
Obesity should be managed in an appropriate setting by staff who have been trained in the management of obesity; the individual should receive advice on diet and lifestyle modification and be monitored for changes in weight as well as in blood pressure, blood lipids and other associated conditions.
An anti-obesity drug should be considered only for those with a body mass index of 30kg/m or greater in whom at least 3 months of managed care involving supervised diet, exercise and behaviour modification fails to achieve a realistic reduction in weight.
Drugs should never be used as the sole element of treatment. The individual should be monitored on a regular basis; drug treatment should be discontinued if the individual regains weight at any time whilst receiving drug treatment. 

1. Anti-obesity drugs acting on the gastro-intestinal tract:
-Orlistat (Xenical).

2. Centrally acting appetite suppressants:
-Rimonabant (Acomplia)
-Sibutramine Hydrochloride (Reductil).

In July 2012, the FDA approved two new medicines for chronic (ongoing) weight management. Lorcaserin hydrochloride (Belviq®) and Qsymia™ are approved for adults who have a BMI of 30 or greater. 


SURGERY
Weight-loss surgery might be an option for people who have extreme obesity (BMI of 40 or more) when other treatments have failed. It is also an option for people who have a BMI of 35 or more and life-threatening conditions, such as:

-Severe sleep apnea 
-Obesity-related cardiomyopathy
-Severe type 2 diabetes

Two common weight-loss surgeries include banded gastroplasty and Roux-en-Y gastric bypass. For gastroplasty, a band or staples are used to create a small pouch at the top of your stomach. This surgery limits the amount of food and liquids the stomach can hold. 

For gastric bypass, a small stomach pouch is created with a bypass around part of the small intestine where most of the calories you eat are absorbed. This surgery limits food intake and reduces the calories your body absorbs.
Like any surgery, there are risks associated to this interventions, side effects and lifelong medical followup is needed after both surgeries.



Sources:
-https://medlineplus.gov/obesity.html
-BNF 57 March 2009
-https://www.nhlbi.nih.gov/health/health-topics/topics/obe/treatment
-http://mhadegree.org/obesity-and-your-healthcare-career/
-http://lifescienceevents.com/2016-obesity-summit-12th-14th-april-2016/

lunes, 27 de junio de 2016

BREAKING BAD NEWS

Clinical communication with patients, relatives and care givers can be a challenge to healthcare providers. However, the use of communication skills and a therapeutic relationship may help seriously unwell patients and their families achieve the best way possible to cope with the illness (Finset 2015).

The first thing to be done whenever a clinical interview takes place or very important medical information is going to be disclosed to patients and/or family is planning and preparing the interview. 
Breaking bad news is reported to be one of the most stressful communication tasks (Porensky et al. 2015), this is why preparation is so important, including where and how the information is going to be given and anticipating possible questions. Preparation includes preparing oneself, the patient/family and the environment (Barnett 2013). 

Allowing enough time is key to enable successful communication. As the Regional Guidelines state “that it is strongly recommended that the person (named or specialist nurse) accompanying the professional who delivers the news, remains with the patient or family member if appropriate and help provide continuing support” (Department of Health, Social Services and Public Safety 2003). 

Different strategies for managing difficult situations like the Ten-step approach, SPIKES (Box 1) and Seven-step approach have preparation as its first step (Lewis et al. 2010).



Use a “warning shot;” that is, say something to prepare such as, “I do not have good news.”
Regional Guidelines state “that warning a patient or family member that bad news is coming may help lessen the shock and may help to process the information they receive” (DHSSPS 2003). 
Social psychology research has suggested that this approach may be most effective in reducing shock and distress of news with potentially serious implications (Porensky et al 2015). 

The news should be given in small chunks with pauses in between allowing time for information to be processed and encourage questions (DHSSPS 2003). Although the medical team will always try to make decisions in the best interest of the patient, the involvement of the patient and the family is decisive as the evidence suggests that patients increasingly want additional information regarding diagnosis, prognosis and treatment (DHSSPS 2003). It is important to remember the use of simple words and short sentences to ensure that the information provided is as clear as possible.
Patients and family often express feelings without talking about them (Egan 2013), their reactions and non-verbal communication can give us an idea of what the patient and family think and feel. 

Planning and preparing includes establishing goals and objectives, select method of disclosure, select the content and arrange resources (Hargie 2010).  Patients and family who have a clear plan for the future are less likely to feel anxious and uncertain (DHSSPS 2003). Furthermore, communication has a direct impact on the degree to which patients cope (Hawthorn 2015).
A study has shown that individuals who hear the negatively-framed prognosis are significantly more distressed than individuals who hear the positively-framed prognosis (Porensky et al 2015).

Breaking bad news is often a continuing process, it is important not to offer unrealistic hope but it is equally important that patients and family can discuss, consider options and come to terms with limitations without feeling abandoned by the health professional team (DHSSPS 2003).
Every person is different and the degree of information desired can vary as well as their expectations and their coping mechanism.
Evidence suggests that tailoring the amount of information provided according to the level of information a patient desire is associated with lower anxiety and better problem-focused coping (Porensky et al. 2015).

Breaking bad news can be extremely stressful for the professionals involved, emotions such as anxiety, a burden of responsibility for the news and fear of a negative response can have an adverse effect on those receiving and those delivering the news (DHSSPS 2003). Preparing the interview and following steps is a useful way of gaining confidence. Being clear about care goals may also assist staff who may need formal support in order to deliver high quality of care (Lewis et al. 2010).







Sources:
-Egan, G. (2013) The Skilled Helper. 10th Pacific Grove, California Belmont Brooks Cole/Cengage Learning.
-Hargie, O. (2010) Skilled interpersonal communication: Research, theory and practice. Hove Routledge.
-Porensky, E.K. and Carpenter, B.D. (2015) Breaking bad news: Effects of forecasting diagnosis and framing prognosis. Patient Education and Counseling, 99, 68-76.
-Lewis, D. and Kitchen, C. (2010) The role of communication skills in end-of-life care. Journal of renal nursing, 22(2), 69-74.
-Hawthorn, M. (2015) The importance of communication in sustaining hope at the end of life. British Journal of Nursing, 24(13), 702-705.
-Barnett, M. (2013) Breaking Bad News – Pointers and Pitfalls. Scottish Universities Medical Journal, 2(2), 54-57.
-Leonard, M., Graham, S., and Bonacum, D. (2004) The human factor: the critical importance of effective teamwork and communication in providing safe care. Qual Saf Health Care, 13(1), 85-90.
-Department of Health, Social Services and Public Safety. (2003) Breaking bad news  Regional Guidelines. DHSSPS, Belfast.
-Finset, A. (2015) The elephant in the room: How can we improve the quality of clinical communication during the last phases in patients’ lives? .  Patient Education and Counseling, 99, 1.
-http://www.ama-assn.org/ethic/epec/download/module_2.pdf
 http://www.jpalliativecare.com/article.aspissn=09731075;year=2010;volume=16;issue=2;spage=61;epage=65;aulast=Narayanan

sábado, 5 de marzo de 2016

SKILLED INTERPERSONAL COMMUNICATION

The importance for health care professionals of having a "good bedside manner" has long been realized. In 400 BC, Hippocrates noted how the patient "may recover his health simply through his contentment with the goodness of the physician". In recent years, this belief in the power of communication to contribute to the healing process has been borne out by research.

Rider and Keefer (2006) and Tallman et al. (2007) have shown that high levels of practitioner interpersonal skill are positively correlated with increases in the quality of care and effective health outcomes, while ineffective skills are associated with decreased patient satisfaction, increased medication errors and malpractice claims. These findings are corroborated in the field of nursing, where effective interpersonal communication has been shown to be related to improved health outcomes, such as greater patient satisfaction and quality of life (Klakovich and dela Cruz, 2006).



The nature of interpersonal skills

Hargie (2006a: 13) defined interpersonal skill as "a process in which the individual implements a set of goal-directed, inter-related, situationally appropriate social behaviours, which are learned and controlled", it emphasises seven separate components of skill:

1. Skilled performance is part of a transactional process which involves: formulating appropriate goals, devising and implementing related action plans, monitoring the effects of behaviour, being aware of and interpreting the responses of others, taking into account the context in which interaction occurs and adjusting, adapting or abandoning goals and responses in the light of outcomes.
2. Skilled behaviours are goal directed: goals both motivate and navigate the interpersonal process (Berger, 2002; Oettingen et al., 2004).
3. Skilled behaviours are interrelated and synchronised.
4. Skills should be appropriate to the situation.
5. Skills are defined in terms of identifiable units of behaviour: verbal and nonverbal.
6. Skilled behaviours are learned.
7. Skills are under the cognitive control of the individual: learning when to employ behaviours is just as crucial as learning what these behaviours are and how to use them.



Zimmerman (2000) identified four key stages in the learning of skills:

-Observation
-Emulation
-Self-control
-Self-regulation: the person learns to use the skill appropriately across different personal and contextual conditions.

Communication and interpersonal communication
Communication is a transactional process, inevitable, purposeful, multidimensional and irreversible.

Conceptual model of skilled interpersonal communication 
This model builds upon skill models developed, inter alia, by Dickson et al. (1997), Bull (2006) and Hargie (2006c), based upon early theorising by Argyle (1983). It identifies six elements of skilled interpersonal interaction:

1. Person-situation context: personal characteristics, knowledge, motives, attitudes, personality, emotion, age, gender, situational factors and culture.

2. Goals: 
-Task and relational goals
-Instrumental and consummatory goals
-Implicit and explicit goals
Goals are hierarchically structured and have a temporal dimension (short or long term). They differ in their level of concreteness and compatibility, goals may be similar, complementary or opposed.

3. Mediating processes: these processes mediate between the goal being pursued, our perceptions of events and what we decide to do about them.
-cognitive processes: Nelson-Jones (1996) recommended a seven-stage framework for rational decision making: confront, generate options and gather information, assess the predicted consequences of options, commit to the decision, plan how to implement the decision, implement the decision and assess consequences of implementation.
-affective processes: skilled communication must always be adaptively and reflexively responsive to the emotional needs of the other.

4. Response: plans and strategies decided upon are implemented at this stage, there is no guarantee that their translation into action will be flawless or successful, When people fail to achieve an interactional goal but persist, they tend to adjust low-level elements of the plan (e.g. volume or speed of speech) rather than more abstract higher order elements (e.g. general strategy)(Knowlton and Berger, 2007).

5. Feedback: enables us to assess the effects or our communications, convergence towards mutual understanding and shared meaning is proportional to the degree which feedback (verbal or nonverbal) is put to effective use.

6. Perception: not all information potentially available via feedback is perceived, and not all information received is perceived accurately. Perception is an active and highly selective process (Eysenck, 1998). Skilled communicators have the ability to make accurate perceptions of self and how one is being perceived by others.




Sources:
-http://www.redtierabbit.com/what-are-interpersonal-skills/
-Hargie O. 2010 Skilled interpersonal communication: Research, theory and practice. Hove Routledge.

jueves, 25 de febrero de 2016

EMERGENCY OXYGEN THERAPY IN ADULTS: PROFESSIONAL COMPLIANCE AND EDUCATION

In the last few months, I have been introduced to the passionate and sometimes stressful world of research, specifically in research in health and social care. As part of my learning I developed a research proposal which is partially shown here. 


Although I am still far from expert, I think the literature review  brings up some interesting questions. Also, constructive feedback is always welcome.


EMERGENCY OXYGEN THERAPY IN ADULTS: PROFESSIONAL COMPLIANCE AND EDUCATION.

INTRODUCTION
Oxygen is probably the   most common drug used in medical emergencies and its inappropriate administration can have serious or even fatal consequences (BNF 70). The literature review carried out by Ingrid Nippers and Andrew Sutton in 2014 states that there is evidence that oxygen therapy is not being prescribed, administered and monitored correctly. They suggest the implementation of planned audits and educational programs for staff to ensure adherence to both local and national guidelines.
The aims of this study are to measure the level of professional compliance with local guidelines when administering oxygen therapy and to assess the effectiveness of educational interventions in the acute settings of the Belfast Health and Social Care Trust.
The objectives of this study are to obtain information about the current situation of oxygen administration, to provide high quality training tackling all aspects of oxygen therapy and to evaluate the success of the educational program in practice.
In order to achieve these objectives, a quantitative approach has been chosen, it is an experimental study before and after a training session. It will take place in the Emergency and Medical Admissions departments at the Royal Victoria  Hospital and the Mater Hospital, both under the Belfast Health and Social Care Trust policies and guidelines.
Although the Belfast Trust is in process of developing a new policy, audits (as per Health & Care Audit Activity Report May 2015) and has a mandatory training in Medical Gases, there is no information available about professional compliance regarding oxygen therapy at present or evidence of the actual success of the Medical Gases training. Also, no audits are carried out (at the moment of developing this study) which means that oxygen use is not being evaluated.



BACKGROUND
In 2008, the first formal guidance on emergency oxygen use was produced by the British Thoracic Society. These guidelines have been already criticised by Smith et al. (2012) regarding the target saturation of oxygen for actively treated patients not at risk of hypercapnic respiratory failure. Nippers et al. (2014) states that it has limited evidence, which affects its reliability, and the National Patient Safety Agency recommends further documents for clinical guidance as the BTS (2008) does not cover critical care or children under 16 years. There is a clear need for consensus, clinical guidance should be developed covering all areas of oxygen therapy and kept up to date with recent research. It is beyond this study to develop latest up-to-date guidelines so, the policy from the Belfast Trust regarding oxygen therapy will be the one used to develop the instrument for data collection.
Organizations like the National Institute for Health and Care Excellence do not have any clinical guidelines about oxygen.
Also, the medical use of oxygen needs to be further examined in search of solid evidence of benefit in many of the current clinical settings in which is routinely used as stated by Sjoberg et al. (2013).
A rapid response report from the NPSA was issued in 2009 after receiving 281 reports of serious incidents related to inappropriate administration and management of oxygen, also, Kane et al. (2013) states that oxygen therapy is often used incorrectly and the dangers of over-oxygenation are unappreciated.
The literature review suggests an incorrect administration tendency in oxygen therapy and a lack of awareness in its dangers, this project has developed an approach that will study both issues.
A quantitative approach has been chosen as it allows to measure objectively the level of compliance, the collected evidence can be transformed in numerical data which can be statistically manipulated to obtain the answer for the research question (Gerrish and Lacey, 2010).

In order to assess the administration of oxygen before and after an educational intervention, a pre and post intervention study will be done as it is an experimental design that reports a change in an outcome following a change in an intervention (Gerrish and Lacey, 2010). Being able to manipulate and   compare the pre and post intervention data will give an overview of the professional compliance and the success of the intervention in practice.




References
-Gerrish, K. and Lacey, A. (2010) The Research Process in nursing. 6th Edition. United Kingdom: Wiley-Blackwell.
-Kane, B., Decalmer, S., & Ronan, O., B. (2013). Emergency oxygen therapy: From guideline to implementation. Breathe, 9(4), 246-253.
-Nippers, I., & Sutton, A. (2014). Oxygen therapy: Professional compliance with national guidelines. British Journal of Nursing, 23(7), 382-386.
-O'Driscoll, B.R., Howard, L. S., & Davison, A. G. (2008). BTS guideline for emergency oxygen use in adult patients. Thorax, 63 Suppl. 6, vi1-vi68.
-Kor, A.C., Lim, T.K. (2000) Audit of oxygen therapy in acute general medical wards following an educational programme.  Annals of the Academy of Medicine Singapore, 29, 2, 177-181
-Sjöberg, F., & Singer, M. (2013). The medical use of oxygen: A time for critical reappraisal. Journal of Internal Medicine, 274(6), 505-528.
-Smith, G. B., Prytherch, D. R., Watson, D., Forde, V., Windsor, A., Schmidt, P. E., et al. (2012). S(p)O(2) values in acute medical admissions breathing air--implications for the British thoracic society guideline for emergency oxygen use in adult patients? Resuscitation, 83(10), 1201-1205.
-Formulary Committee Joint Cover: Paperback. (2015). Oxygen. British National Formulary, 70th Edition, September 2015-March 2016. United Kingdom: Pharmaceutical Press, page 214.
-National Patient Safety Agency. (September 2009). Rapid Response Report: Oxygen safety in hospitals. Available at: www.nrls.npsa.nhs.uk/alerts.


Note: this is just a proposal done for an assignment, it will not be developed any further.

martes, 29 de septiembre de 2015

ANAEMIA II

The thalassaemias
The thalassaemias are a heterogeneus group of inherited disorders of haemoglobin synthesis. They are characterised by a reduction in the rate of synthesis of either alpha or beta chains and are classified accordingly. Unbalanced synthesis of alpha and beta-globin chains can damage red cells.
The basic haematological abnormality is a hypochromic microcytic anaemia of variable severity. The clinical severity of any thalassaemia is proportionate to the degree of imbalance of alpha and beta-globin chain synthesis.



α-Thalassaemias:


-Hb-Barts hydrops syndrome: there is a complete absence of α-chain synthesis which is needed for fetal and adult haemoglobin, the disorder is incompatible with life and death occurs in utero.

-HbH disease: there is a moderate chronic haemolytic anaemia with splenomegaly and sometimes hepatomegaly. Severe bone changes and growth retardation are unusual.

-α-Thalassaemia traits (carrier state).


β-Thalassaemias:

-β-Thalassaemia major: the characteristic severe anaemia is caused by α-chain excess leading to ineffective erythropoiesis and haemolysis. Anaemia first becomes apparent at 3-6 months, child fails to thrive and develops hepatosplenomegaly. Compensatory expansion of the marrow spaces causes atypical facies with skull bossing and maxillary enlargement. If left untreated further complications can include repeated infections, bone fractures and leg ulcers.
Blood transfusion remains the mainstay of management, splenectomy can reduce its frequency. With such regular transfusion iron chelation is necessary to minimise iron overload (accumulation of iron damages the liver, endocrine organs and heart with death), the common regimen is subcutaneous desferrioxamine given for 5-7 days per week. Compliance may be problematic but where good there is a considerably improved life expectancy.


-Thalassaemia intermedia: the clinical features are less severe than in β-Thalassaemia major. Patients usually present later (often at 2-5 years), have relatively high haemoglobin levels, moderate bone changes and normal growth. Regular transfusion is not required.

-β-Thalassaemia trait (minor): is a symptomless clinical disorder which should not be confused with iron deficiency. Genetic counselling is required in selected patients.




Sickle cell syndromes
The sickle cell syndromes are a group of haemoglobinopathies which primarily affect the Afro-Caribbean population. The common feature of these diseases is inheritance of an abnormal haemoglobin beta-chain gene, inheritance of two of these abnormal genes leads to a serious disorder termed sickle cell anaemia.


Sickle cell anaemia (HbSS): the classic form of sickle cell syndrome is enormously variable in severity.

-Haemolytic anaemia: the haemoglobin is usually in the range 60-100g/L, the symptoms of anemia are often surprisingly mild. Intercurrent infection with parvovirus or folate deficiency can cause a sudden fall in haemoglobin ("aplastic crisis").

-Vascular-occlusive crises: acute, episodic, painful crisis are a potentially disabling feature of sickle cell anemia. They may be triggered by infection or cold. Patients complain of musculoskeletal pain, hips, shoulders and vertebrae are most affected. Attacks are generally self-limiting but infarction of bone can occur. Other organs are vulnerable to infarction; most serious neurological damage which may manifest as seizures, transient ischaemic attacks and strokes. Vaso-occlusion in infancy is responsible for the "hand-foot syndrome", a type of dactylitis damaging the small bones of hand and feet.


First line treatment is rest, increased fluids and adequate oral analgesia. Constitutional upset or pain not relieved by oral analgesia requires hospital admission with continued rest, warmth, intravenous fluids and opiate analgesia.

-Sequestration crises: these arise from sickling and infarction within particular organs. Specific syndromes include "acute chest syndrome" with occlusion of the pulmonary vasculature, "girdle sequestration" caused by occlusion of the mesenteric blood supply, and hepatic and splenic sequestration.

-other complications: papillary necrosis with haematuria, loss of ability to concentrate urine, nephrotic syndrome, priapism, lower limb ulceration, proliferative retinopathy, glaucoma, liver damage and pigment gallstones.

Management:
Prophylaxis is important, patients should avoid factors known to precipitate crisis, take folate supplements and be prescribed penicillin and pneumococcal vaccine. Infections require prompt treatment.
Treatment options are blood transfusions, hydroxycarbamide and stem cell transplantation when indicated.

Prognosis:
The risk of early death is inversely related to fetal haemoglobin levels. The most common causes of death are infection in infancy, cerebro-vascular accidents in adolescence and respiratory complications in adult life.

Sickle cell trait (HbAS): normally causes no clinical problems. However, haematuria occasionally occurs as a result of renal papillary necrosis and additional care is required during pregnancy and anaesthesia.




Sources:
-http://www.slideshare.net/giridharkv/thalassemia-drkvgiridhar
-https://www.pinterest.com/kedillard/thalassemia-awareness/
-Haematology, 3rd Edition, Martin R. Howard, Churchill Livingstone, Elsevier, 2008.
-https://www.linkedin.com/pulse/sickle-cell-disease-scd-metrohealth-hmo
-http://www.slideshare.net/sandipgupta77770194/sickle-cell-disease-sandip

jueves, 24 de septiembre de 2015

ANAEMIA I

The term "anaemia" refers to haemoglobin or red cell concentration below the accepted normal range in the blood. With the widespread introduction of automated equipment into haematology laboratories the haemoglobin concentration has replaced the haematocrit as the key measurement.
The normal range for haemoglobin is affected by sex, age, ethnic group and altitude.

Normal haemoglobin (Hb) value:
-male (adult): 13 - 18 g/dL
-female (adult): 11.5 - 16 g/dL





Iron deficiency anaemia (Microcytic)
Iron is a constituent of haemoglobin and rate limiting for erythropoiesis, its metabolism is dominated by its role in haemoglobin synthesis.
Iron deficiency can usually be diagnosed with the clinical history and simple laboratory tests but, a cause for the deficiency must always be sought.

Iron deficiency can be caused by long-term blood loss (e.g. gastrointestinal or uterine bleeding), Hookworm infection, poor diet, malabsorption or increased demand for iron as in pregnancy.


Clinical features
Patients with long-standing deficiency may develop nail flattening and koilonychia (concave nails), sore tongues and papillary atrophy, angular stomatitis, dysphagia and gastritis. 
Patients may complain of heavy periods, indigestion or a change in bowel habit.
Iron deficiency in young children can contribute to psychomotor delay and behavioural problems.

Management
-Investigation of underlying cause.
-Correction of iron deficiency:
The normal regimen is oral ferrous sulphate 200mg three times a day for at least 6 months to replete body stores (side effects are best managed by reducing the dosage rather than changing the preparation). Parenteral iron (IV or IM) can be used where oral therapy is unsuccessful.

Megaloblastic anaemia 
Megaloblastic anaemia is a common cause of a macrocytic anaemia,  In clinical practice it is almost always caused by deficiency of vitamin B12 or folate.

-Vitamin B12 deficiency (Pernicious anaemia): 
The classic cause is an autoimmune disorder. Patients usually have symptoms of anaemia and the generalised epithelial abnormality can manifest as glossitis and angular stomatitis.
Patients complain of unsteady gait, and if B12 deficiency is not corrected there can be a progression to irreversible damage of the central nervous system. Also, there is a possible increased incidence of carcinoma of the stomach and colorectal cancer.
Vitamin B12 levels are usually replenished by intramuscular injection of the vitamin. Several injections of 1mg hydroxycobalamin are given over the first few weeks and then either one injection every three months or daily oral vitamin B12 1 to 2mg daily for life.
Other causes of vitamin B12 deficiency are mostly abnormalities of the stomach and ileum.


-Folate deficiency:
Folate deficiency is caused by dietary insufficiency, malabsorption, excessive utilisation or a combination of these. Patients may complain of symptoms of anaemia or of an underlying disease.
Folate deficiency is treated with oral folic acid 5mg once daily, the precise duration of therapy depends on the underlying cause.
Before folate is prescribed, vitamin B12 deficiency must be excluded (or corrected) as subacute combined degeneration of the spinal cord can be precipitated.

Haemolytic anaemia
The term "haemolytic anaemia" describes a group of anaemias of differing aetiology that are all characterised by abnormal destruction of red cells.

Classification of the haemolytic anaemias:

  • Inherited disorders:
1. Disorders of the red cell membrane: hereditary spherocytosis and hereditary elliptocytosis.
-Hereditary spherocytosis: fluctuating levels of jaundice and palpable splenomegaly are common features. No treatment is required in patients with mild disease, in more serious cases the spleen is removed.
-Hereditary elliptocytosis: has many similarities to hereditary spherocytosis, splenectomy helps in the rare severe cases.

2. Abnormalities of haemoglobin: thalassaemia syndromes and sickling disorders.

3. Abnormalities of red cell metabolism: glucose-6-phosphate dehydrogenase and pyruvate kinase deficiency.
-Glucose-6-phosphate (G6PD) dehydrogenase: common triggers are fava beans, drugs and infections. Treatment is to stop any offending drug and to support the patient, blood transfusion may be necessary.
-Pyruvate kinase (PK) deficiency: all general features of haemolysis can be present, but clinical symptoms are often mild for the degree of anaemia. Splenectomy may help in reducing transfusion requirements.
  • Acquired disorders:
1. Immune: warm and cold autoimmune haemolytic anaemia.
This disease can be divided into "warm" and "cold" types depending on the temperature at which the antibody reacts optimally with red cells.

-Warm autoimmune haemolytic anaemia (Warm AIHA): is the most common form of the disease, appoximately half of the cases are idiopathic but in the other half there is an apparent underlying cause e.g. lymphoproliferative disorders, other neoplasms, connective tissue disorders, drugs or infections.
Splenomegaly is a frequent examination finding in severe cases.
Management: identification and treatment of the cause, stop any offending drugs (e.g cephalosporin antibiotics), where the haemolysis itself requires treatment, steroids are normally used. Splenectomy is usually indicated when steroid resistance is present.

-Cold autoimmune haemolytic anaemia (Cold AIHA): the cause can be cold haemagglutinin disease, lymphoproliferative disorders, infections or paroxysmal cold haemoglobinuria.
Circularoty problems such as acrocyanosis, Raynaud´s phenomenon and ulceration may be present,  haemoglobinuria may occur when red cell destruction is intravascular.
Where the Cold AIHA is chronic the mainstay of treatment is keeping the patient warm, especially in the extremities. In forms associated with lymphoproliferative disorders, cytotoxic drugs or rituximab may be helpful.

2. Isoimmune: rhesus or ABO incompatibility.

3. Non-immune and trauma: valve prostheses, microangiopathy, infection, drugs or chemicals and hypersplenism.
-Microangiopathic haemolytic anaemia (MAHA): is the intravascular destruction of red cells in the presence of an abnormal microcirculation. Common triggers are the presence of disseminated intravascular coagulation (DIC), abnormal platelet aggregation and vasculitis.
Clinical syndromes associated with MAHA include haemolytic uraemic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP).




Sources:
-https://themedicalpost.wordpress.com/2010/07/18/the-general-concept-of-anaemias/
-Haematology, 3rd Edition, Martin R. Howard, Churchill Livingstone, Elsevier, 2008.
-http://justinhealth.com/symptoms-of-anemia/
-http://www.spinabifida.net/cobalamin-vitamin-b12-folic-acid-deficiency-in-spina-bifida-neural-tube-defects.html
-http://www.thecambodiaherald.com/health/splenomegaly-%E2%80%93-symptoms-and-causes-688
-http://health.kernan.org/patiented/articles/000541.htm