Solved Paper 2007

7/31/2019 Solved Paper 2007

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also known as Mediterranean

Anemia, Cooley's Anemia orHomozygous Beta Thalassemia, is agroup of inherited disorders in which

there is a fault in the production ofhaemoglobin

THALASSEMIA


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PATHOPHYSIOLOGY

Deficiency in the synthesis of beta chain ofHb molecule

Compensatory increase in the alpha chain

production

Defective Hb production

Hb breaks down

Severe anemia,Cardiacfailure,Hypersplenisim


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PATHOPHYSIOLOGY

Compensation of the hemolytic process

Overabundance of the erythrocytes

Tremendous marrow expansion

Frontal bossing,Maxillary prominence


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PATHOPHYSIOLOGY

Marrow fails to maintain an effective Hblevel

Tissue anoxia & repeated infections occurs.

The body compensates by absorbing Fe from

the GI tract

Hemosiderosis occurs.


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PATHOPHYSIOLOGY

Hemochromatosis of pituitary gland

Retarded growth & delayed sexual maturation


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Medical management

Blood transfusion-Blood transfusion is the mostcommon treatment for thalassemia. In this treatment,red blood cells are transfused into the patients,supplying them with healthy red blood cells containing

normal hemoglobin, allowing sufficient oxygen to becirculated throughout the body.Patients with thalassemia major receive bloodtransfusions every three to four weeks. This makes sure

that body tissues receive sufficient oxygen so thatpatients may grow normally.


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Bone marrow transplantation: Bone-marrowtransplants are the only way to completely cure apatient with thalassemia. In bone-marrowtransplantation, a thalassemic's bone marrow, which

is unable to make a sufficient amount of red bloodcells, is replaced with a normal bone marrow.

Chelationtherapy-Deferoxamine(desferal),Kelfer,Desirox,Asunr

a.


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Wheet grass tablets (Deerghau)

120 tablets,1 tab=500 mg,Rs.235

Dosage:2-3 tablets in the morning & 2-3 tablets

in the evening,empty stomach


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BENEFITSOF WHEET GRASS TABLETS

Increase resistance power

Increases Hb level

Purifies blood

Reduces high B.P Improves blood sugar disorders

Improves digestion

Aids in prevention & curing of cancer Is a good energier


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SURGICAL MANAGEMENT

Spleenectomy


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NURSING MANAGEMENT:

Early assessment

Preparation for diagnostic studies

Administration of chelation therapy,transfusion

therapy Care during surgery-Spleenectomy

Prevention of infections

Observation for complications Education & support of the parents & child.


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Education & support of the parents & child-

1.Help the parents to overcome the guilt feelings.

2.Encourage the parents to help their children lead anormal life as much as possible.

3.Avoid being overprotecting as it can lead todependency.

4.Encourage mutual sharing & introduce the child toother thlassemic child.

5.Encourage the child to express their feelings.


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Prevention

Prenatal counseling

Genetic counseling


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QUESTION 2

Role of nurse administrator in PICU.


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Role of nurse administrator in PICU

Overall responsibility for nursing patient care

Establishing and documenting administrative procedurefor the nursing team

Promoting the development of nursing staff

Budgeting and maintaining practice and standardguidelines

Communication between practice and nursing staff

Scheduling and supervising of nursing staff

Analyzing nursing treatment and diagnosis decisions Troubleshooting and patient consultation

Team building exercises and employee counseling.


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Outcome related to limiting iatrogenic injury andcomplications to therapy acknowledge the potentialhazards inherent in illness and the healthcare

environment. Nurses are to create healing environments that

provide safe passage for vulnerable individuals.

Strict adherence to infection control measures incare delivery by care providers would help toreduce the risk of hospital acquired infection.


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Therapeutic environment in PICU

Minimize noise levels educational program tomaintain awareness of various things that cause soundin the unit that affects on the children and families.

Evaluation of equipment loud or annoying alarms

and noises emitted.

Reduce bleep sound of monitors when there is of noadded advantage.

Use headphones for music player volume pitch atchild comfort level.

Avoid knocking on incubator.


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Dim lights as much as possible at night to createnight cycle.

High intensity light use for procedures and

phototherapy can be irritating to child eyes shieldwith eye covers would be essential.


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Importance of family centered care

The unique contribution by parents in the care of sick

children is recognized by many pediatric health centers.

Involvement of parents in the care of their sick child has

positive therapeutic effects upon the health recovery of thechild.

The goals of care should be patient-and family-centered.

It is the family where baby will go home with and as often itis the family that are managing the long-term consequencesin the care of the child.


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A stronger alliance with the family in promoting eachchilds health and development

Improved clinical decision making on the basis of

better information and collaborative processes Improved follow-through when the plan of care is

developed collaboratively with families

Greater understanding of the familys strengths and

caregiving capacities


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More efficient and effective use of professional time andhealth care resources (eg, more care managed at home,decrease in unnecessary hospitalizations and emergencydepartment visits, more effective use of preventive care)

Improved communication among members of the health careteam

A more competitive position in the health care marketplace

An enhanced learning environment for future pediatriciansand other professionals in training

A practice environment that enhances professionalsatisfaction

Greater child and family satisfaction with their health care


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Imperforate anus

is the absence of a normal anal opening. Thediagnosis is usually made shortly after birth by aroutine physical examination. Imperforate anusoccurs in about 1 in 5000 births and its cause isunknown.


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Pathophysiology of ARM

The embryogenesis of these malformations remains unclear.The rectum and anus are believed to develop from the dorsalpotion of the hindgut or cloacal cavity when lateral ingrowthof the mesenchyme forms the urorectal septum in the midline.This septum separates the rectum and anal canal dorsallyfrom the bladder and urethra. The cloacal duct is a smallcommunication between the 2 portions of the hindgut.Downgrowth of the urorectal septum is believed to close thisduct by 7 weeks' gestation. During this time, the ventralurogenital portion acquires an external opening; the dorsalanal membrane opens later. The anus develops by a fusion of

the anal tubercles and an external invagination, known as theproctodeum, which deepens toward the rectum but isseparated from it by the anal membrane. This separatingmembrane should disintegrate at 8 weeks' gestation.


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Interference with anorectal structure development atvarying stages leads to various anomalies, ranging fromanal stenosis, incomplete rupture of the anal membrane,or anal agenesis to complete failure of the upper portionof the cloaca to descend and failure of the proctodeum to

invaginate. Continued communication between theurogenital tract and rectal portions of the cloacal platecauses rectourethral fistulas or rectovestibular fistulas.

The external anal sphincter, derived from exteriormesoderm, is usually present but has varying degrees of

formation, ranging from robust muscle (perineal orvestibular fistula) to virtually no muscle (complex longcommon-channel cloaca, prostatic or bladder-neckfistula).


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Posterior sagittal pull-through with colostomy: Thisapproach is used in boys with rectourinary fistula(bulbar, prostatic, or bladder-neck fistula), in girls withcloaca or vestibular fistula, and in patients of either sex

who do not have a fistula when the rectal pouch is furtherthan 1 cm on 24-hour lateral prone abdominalradiography.

Colostomy closure: Once the wound has completelyhealed and postoperative dilations have achieved theirgoal (ie, the neoanus is at the desired size), the colostomymay be closed in traditional surgical fashion.


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Nursing Management:

The first nursing responsibility is the identification of undetectedARMs. A newborn which does not pass stool within 24-36 hours ofbirth requires further assessment, and meconium that appears at aninappropriate orifice is reported.

Postoperative nursing care usually presents few problems and isprimarily directed toward healing of the anoplasty without infectionor other complications. in situations where the infant hasundergone a pull through procedure with anoplasty, special nursingcare involves maintaining the anal area as clean as possible withscrupulous perineal care. The preferred position is a side lyingprone position with hip elevated or a supine position with the legs

suspended at a 900 angle to the trunk to prevent pressure onperineal sutures. The infant is administered regular formula as soonas the peristalsis returns. In the mean time there may be an NG tubefor abdominal decompression. Care of the infant with a colostomyinvolves frequent dressing changes, meticulous skin care, andcorrect application of a collecting device.


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Nursing Diagnoses:

Alteration in comfort (pain) r/t the perineal sutures as

evidenced by recurrent cry and irritability.

Altered nutrition less than body requirement r/t to NPO

status as evidenced by weight loss.

Altered elimination pattern(via colostomy) r/t the

stepped correction of underlying congenital defect as

evidenced by continuous faecal excretion.

Potential for fluid volume deficit r/t to continuous RT

aspiration and NPO status.


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Potential for infection r/t the perineal sutures.

Potential for altered skin integrity r/t the presence of

colostomy without a bag.

Knowledge deficit r/t care of perineal sutures, and

dilatation programme.

Anxiety r/t the prognosis of the child.


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Purpura

Purpura is the result of hemorrhage into the skin or mucosal

membrane. It may represent a relatively benign condition or

herald the presence of a serious underlying disorder. Purpura

may be secondary to thrombocytopenia, platelet dysfunction,

coagulation factor deficiency or vascular defect. Investigation to

confirm a diagnosis or to seek reassurance is important.

Frequently, the diagnosis can be established on the basis of a

careful history and physical examination, and a few key

laboratory tests. Indicated tests include a complete blood cell

count with platelet count, a peripheral blood smear, and

prothrombin and activated partial thromboplastin times.


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Causes

Nonthrombocytopenic purpuras may be due to: Amyloidosis Blood clotting disorders Congenital cytomegalovirus Congenital rubella syndrome Drugs that affect platelet function Fragile blood vessels (senile purpura) Hemangioma Inflammation of the blood vessels (vasculitis), such

as Henoch-Schonlein purpura, which causes a raised type ofpurpura

Pressure changes that occur during vaginal childbirth Scurvy Steroid use


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Thrombocytopenic purpura may be due to:

Drugs that prevent platelets from forming

Idiopathic thrombocytopenic purpura (ITP)

Immune neonatal thrombocytopenia (can occur ininfants whose mothers have ITP)

Meningococcemia -- a raised type of purpura


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Management

The treatment of purpura should always be directed at itsunderlying cause.

Children with bleeding tendencies generally should notparticipate in strenuous activities or contact sports. Inmost instances, they should not receive intramuscularinjections. The use of aspirin and other NSAIDs alsoshould be avoided. Transfusion of platelets orcoagulation factors may sometimes become necessary.Genetic counseling is useful in families with inherited

bleeding disorders.


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Risk of injury related to increased bleeding tendency

Bleeding precautions;

Bleeding reduction;

Fall prevention; Environmental management; Safety;

Health education; Surveillance;

Medication management


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MID DAY MEAL PROGRAMME

The mid-day programme is also known as schoollunch programme.This programme has been inoperation since 1961 .The major objective of thisprogramme is to attract more children for admissionto schools & retain them so that literacyimprovement of children could be brought about.


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Objectives

The objectives of the mid day meal scheme are: Improving the nutritional status of children in

classes I-V in Government, Local Body andGovernment aided schools, and EGS and AIE

centres. Encouraging poor children, belonging to

disadvantaged sections, to attend school moreregularly and help them concentrate on classroom

activities. Providing nutritional support to children of primary

stage in drought affected areas during summervacation.


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The following broad principles should be kept in mind- The meal should be a supplement & not a substitute to

the home diet.

The meal should supply at least 1/3rd of the total energy

requirement,& half of the protein need. The cost of the meal should be reasonably low.

The meal should be such that it can be prepared easily inschools,no complicated cooking process should be

involved. Locally available foods should be used.

The menu should be frequently changed to avoidmonotomy.


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Mental retardation (MR)

is a developmental disability that first appears inchildren under the age of 18. It is defined as a level ofintellectual functioning (as measured bystandard intelligence tests ) that is well belowaverage and results in significant limitations in theperson's daily living skills (adaptive functioning).


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Mild mental retardation Approximately 85% of the mentally retarded population is in the mildly

retarded category. Their IQ score ranges from 5070, and they can oftenacquire academic skills up to about the sixth-grade level. They can becomefairly self-sufficient and in some cases live independently, with communityand social support.

Moderate mental retardation About 10% of the mentally retarded population is considered moderately

retarded. Moderately retarded persons have IQ scores ranging from 3555.They can carry out work and self-care tasks with moderate supervision.They typically acquire communication skills in childhood and are able tolive and function successfully within the community in such supervised

environments as group homes .


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Severe mental retardation About 34% of the mentally retarded population is severely

retarded. Severely retarded persons have IQ scores of 2040.They may master very basic self-care skills and somecommunication skills. Many severely retarded individuals are

able to live in a group home.

Profound mental retardation Only 12% of the mentally retarded population is classified as

profoundly retarded. Profoundly retarded individuals have IQ

scores under 2025. They may be able to develop basic self-care and communication skills with appropriate support andtraining. Their retardation is often caused by anaccompanying neurological disorder. Profoundly retardedpeople need a high level of structure and supervision.


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Causes

Genetic factors

Prenatal illnesses and issues

Childhood illnesses and injuries

Environmental factors


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Treatment

Federal legislation entitles mentally retarded children to free testing

and appropriate, individualized education and skills training within the

school system from ages three to 21. For children under the age of

three, many states have established earlyintervention programs that

assess children, make recommendations, and begin treatment

programs. Many day schools are available to help train retarded

children in such basic skills as bathing and feeding themselves.

Extracurricular activities and social programs are also important inhelping retarded children and adolescents gain self-esteem.


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Training in independent living and job skills is oftenbegun in early adulthood. The level of training dependson the degree of retardation. Mildly retarded people canoften acquire the skills needed to live independently andhold an outside job. Moderate to profoundly retarded

persons usually require supervised community living in agroup home or other residential setting.

Family therapycan help relatives of the mentallyretarded develop coping skills. It can also help parents

deal with feelings of guilt or anger. A supportive, warmhome environment is essential to help the mentallyretarded reach their full potential.


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Role of voluntary agencies?????

Child marriage act?????


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Documents

Solved Paper 2007