Sickle Cell Anemia Case Study

Posted by: Daisy Jane, RN, MN October 16, 2010 Comments (0) Definition Sickle cell anemia is an inherited disorder on the beta chain of the hemoglobin resulting to abnormally shaped red blood cells. RBCs assume a crescent or C-shape that decreases the cells life span and ability to function thereby, producing various complications. In some cases, these sickled cells may block the flow causing pain and organ damage. Incidence

Equal male-female ratio

Increase incidence in African Americans Etiology

Autosomal recessive disorder Review of Related Anatomy and Physiology Red blood cells or erythrocytes carry oxygen to the different parts of the body. Different from other cells, RBCs do not contain a nucleus (anucleated). These small cells are circular and flattened with depressed centers on both sides resembling to that of a doughnut when viewed under a microscope. Their size and shape provides a large surface area for carrying oxygen in relation to their volume. The normal RBC count is 4-6 million/mm3. RBCs has the life span of 120 days. Hemoglobin is a form of protein that contains iron which is responsible for transporting oxygen that is carried in blood. Adult hemoglobin contains a beta chain (HBB) while a fetus hemoglobin has a gamma chain. Hemoglobin is comprised of four protein (amino acid) components. It has two beta-globin and two alpha-globin. The subunit betaglobin is located inside the RBCs. These amino acids carry an iron-bearing molecule called heme. Heme molecules, which are only found in mature RBCs, enables the erythrocytes to pick oxygen from the lungs and transport it throughout the body. Once oxygen attaches to hemoglobin it gives the blood its bright red pigment. The more hemoglobin molecules the RBC contain, a higher amount of oxygen will they be able to carry. If the hemoglobin is defective, the erythrocyte will also malfunction. A red blood cell is just a vessel; the one that performs the oxygen transportation is the hemoglobin. Normal hemoglobin is 13-18 grams/100 ml of blood in males and 12-16 grams in females. Pathophysiology Erythrocytes in sickle cell anemia contain abnormal hemoglobin that affects the beta-chain producing hemoglobin S or HbS. In this disorder, the beta-chains (beta-globins) are replaced by Hemoglobin S. Valine (an amino acid) takes the place of the normally appearing glutamic acid in beta-chains. Replacement of glutamic acid with valine causes the polymerization of HbS components to cohere forming long and insoluble particles. These distort the red blood cells, which assumes an inflexible crescent or sickle shape.

The abnormally shaped cells become sharp and spiky when the RBCs are discharging oxygen molecules and in cases where the oxygen content in blood is low such as performing vigorous exercise and being in high altitude areas. Typically, a sickled cells lifespan is only 20 days. The deformed erythrocytes also rupture easily and they tend to be trapped in the microcirculation, obstructing blood flow and oxygen transport that might lead to painful episodes of ischemic injury. Sickle cell crisis refers to episodes of acute and severe sickling that blocks the circulation posing a threat of extensive organ damage. Severe pain is noted during these incidents caused by occluded vessels in the bone possibly resulting to bone necrosis. The crisis is triggered by hypoxemia, acidosis, or other conditions such as dehydration, infection, vigorous exercise, pregnancy or cold weather. A condition called a sickle cell trait is identified by the presence of a single defective gene, instead of two. Individuals with this trait are essentially normal however, they are carriers. Meaning two sickle cell trait carrier parents can contribute a defective gene to a child that will carry the sickle cell disorder. Diagnosis Prenatal:

Chorionic Villi sampling

Amniocentesis (blood from the cord) At birth: Newborn Screening Hemoglobin electrophoresis Signs and Symptoms The following manifestations are observed in children with a sickle cell disorder, at about 6 months of age (because

fetal hemoglobin contains gamma, not a beta chain): Fever

Anemia Swelling of the hands and feet (hand-foot syndrome) caused by blood stasis and infarction Protruding abdomen due to enlarged spleen and liver secondary to trapping of sickled cells in microcirculation and obstruction of blood flow Icteric sclera caused by bilirubin release during hemolysis (sickle cell destruction) Priaprism (males) due to pooling of abnormally shaped erythrocytes in the blood vessels of the penis.

Chest syndrome symptoms same with pneumonia that is the major cause of death in sickle-cell patients Management Conservative Management Pain relief with Acetaminophen (Tylenol) or IV of morphine to reduce metabolic demand of oxygen thereby, terminating cell sickling. Adequate hydration IV fluids and electrolyte replacement Oxygenation Antibiotics if the cause of sickling is infection. Blood transfusion with packed RBCs Hydroxyurea antineoplastic agent that increases production of fetal hemoglobin in children.

1. 2. 3. 4. 5. 6.

7. Exchange transfusion replacing sickled with normal cells If the patient does not respond to the usual therapies STEM CELL TRANSPLANTATION is done. Nursing Management 1. 2. 3. 4. 5. Monitor vital signs. Assess for pain. Obtain blood and urine culture, chest x-ray and CBC results if infection is the cause of sickling. Monitor childs nutritional intake with hydroxyurea. If taken orally, this drug can cause anorexia. Assess for kidney function by noting if the child has urinated or not. (Kidney infarction may occur) Do not administer potassium if the kidney function is not verified. Potassium if not excreted by the kidney may cause arrhythmia.

Possible nursing diagnosis 1. 2. Ineffective tissue perfusion R/T decreased hemoglobin concentration in blood Acute pain R/T impaired blood flow due to obstruction of sickled cells

Placenta Accreta, Increta, Pancreta

Posted by: Daisy Jane, RN, MN October 8, 2010 Comments (1)

Definition Placenta Accreta is an intrapartum complication characterized by the abnormal implantation of the placenta. Normally, chorionic villi attaches to the uterine endometrium. With this complication, the point of attachment extends to the layers of myometrium. Review of Related Anatomy and Physiology Uterus, the pear-shaped hollow muscular organ, houses the fetus until delivery. This structure has three layers

namely: the perimetrium, myometrium and endometrium. Perimetrium the outermost layer that covers the uterus. (Peri means outside) Myometrium located at the middle part of uterus. Myo denotes muscle, thus, this layer contains thick muscular layers. Endometrium the innermost layer that responds to hormonal variations (estrogen and progesterone) during the menstrual cycle. The chorionic villi (finger-like projections that attaches to the uterine wall) penetrates into

this portion of the uterus during implantation. (Endo means inside) In a female reproductive cycle, the uterine endometrium normally sloughs off the thickened vascular surface that precedes the actual secretion of blood flow. With fertilization, it continues to be crammed with blood to accommodate and nourish the embryo. Following this condition, uterine endometrium is now called decidua, which has three separate areas:

Decidua Basalis point of attachment between the maternal vessels and trophoblast (structure that forms the placenta and membrane). Lies under the embryo. (To remember easily Basalis comes the word base meaning under/below) Decidua Capsularis part of endometrium that expands and encapsulates the implanted trophoblast. . (To remember easily Capsularis comes the word capsule meaning to enclose/above) Decidua Vera (Parietalis) remaining portion of the uterine endometrium. (To remember easily Parietalis

comes the word parietal meaning wall of a hollow organ) Types of Placenta Accreta This obstetric complication is categorized depending on the depth of its attachment: Placenta Accreta chorionic villi attaches deeply into the uterine wall but does not penetrate the

myometrium. This is the most common form of the condition. (Accreta starts with letter A, so it Attaches) Placenta Increta chorionic villi invades or infiltrates the muscular layer. (Increta starts with letter I, so it Invades/Infiltrates) Palcenta Pancreta/Percreta chorionic villi penetrates beyond the myometrium into the entire uterine wall and possibly to other adjacent organs such as the bladder. This is the least common of the three conditions. ( Pancreta starts with letter P, so it Penetrates)

Pathophysiology Predisposing Factors 1. 2. Scarring of tissues from previous infection Previous uterine surgery (Dilation and Curettage, Cesarean Section, Myomectomy)

3. Thin decidua or absent deciduas basalis 4. Presence of tumor Signs and Symptoms Usually signs and symptoms are not detected until labor and delivery. However, for some third trimester bleeding would be noted. During labor and delivery massive bleeding is observed. In cases when deciduas basalis is absent, the placenta will not loosen and fails to be delivered. Complications

Uterine rupture Massive bleeding

Disseminated intravascular coagulation (DIC) Diagnostic test Ultrasound MRI Medical Management

Conservative treatment is done if the woman wants to maintain her fertility under the condition that no active bleeding is present. This treatment saves the uterus but poses higher risk of complications and low successful rate. Techniques for this treatment are as follows:

The placenta is left in the uterus and the cord is ligated. Closure of the uterus is performed. Methotrexate (an antineoplastic agent) is usually given to the woman to destroy the still attached placenta. WBC and platelet count (thrombocytopenia and leucopenia may occur 7-14 days after the initiation of treatment) Blood Urea Nitrogen (BUN), Creatinine, and urine pH (should be above 7.0) Presence of dry and nonproductive cough may be an early sign of pulmonary toxicity Symptoms of gout must be assessed frequently (increased uric acid, joint pain, edema). Methotrexate causes

Women taking Methotrexate should be monitored for:

increase serum uric acid. Allopurinol may be given to decrease uric acid levels. After the techniques are implemented, prophylactic antibiotic is started to prevent infection. Follow-up includes frequent or daily ultrasound sessions to monitor uterine involution and placental condition. Surgical Management Early detection of placenta accreta will prevent serious complication. The safest modality is a planned cesarean section and hysterectomy (surgical removal of the uterus). Nursing Management 1. 2. 3. Obtain a detailed obstetric history. Assist with modalities implemented. For clients taking Methotrexate, instruct the woman to increase fluid intake to at least 2 L each day as uric acid

formation is increased with the drug use. 4. Provide emotional support to the woman and family. Possible Nursing Diagnosis Diagnosis of Placenta accrete with a massive blood loss is an emergency; objective should consider the brief time frame of the emergency condition. 1. Impaired tissue integrity R/T deep attachment of the placenta 2. 3. Fear R/T pregnancy outcome secondary to placenta accrete Grieving, dysfunctional R/T loss of a body part after hysterectomy

Talipes Deformity Case Study (Clubfoot)

Posted by: Daisy Jane, RN, MN October 7, 2010 Comments (0)

Definition

Talipes deformity is a disorder of ankle and foot. It comes from the Latin wordstalus meaning ankle and pes meaning foot. Incidence Commonly called clubfoot, it is a congenital anomaly occurring at approximately 1 to 2 in every 1000 live births. Male-female incidence ratio is 2:1. Bilateral deformity involvement accounts 30%-50% of cases. True Talipes Disorder Talipes deformity could either be unilateral (affecting a single foot only) or bilateral (both feet are affected). Regardless of which extremity is affected, some newborns have developed a twisted foot appearance due to intrauterine position. However, with manipulation the foot can be brought into a straight position. This temporary abnormality is called a pseudo-talipes disorder. A true clubfoot cannot be aligned properly without further intervention. Skeletal Anatomy of the Foot Two essential functions of the foot: 1. Reinforces body weight 2. Allows the body to move forward when running or walking Facts about the foot bone: 1. The weight of the body is carried by the largest tarsal bones, calcaneus (heelbone) and talus (ankle bone). 2. To create a strong arch of the foot it is arranged longitudinally (medial and lateral) and transverse. Parts of the Foot Bone: Tarsus the posterior half of the foot composed of seven tarsal bones: 1. 2. 3. 4. 5. 6. Medial cuneiform Intermediate cuneiform Lateral cuneiform Cuboid Navicular Talus

7. Calcaneus Metatarsals form the sole and are composed of 5 bones. Phalanges form the toes and are composed of 14 bones. Each toe has 3 phalanges with the exception of the great toe having only 2. Ligaments connects bones. Tendons attaches bone to a muscle allowing movements or a specific amount of elasticity. Pathophysiology Etiology The exact cause of this deformity is unknown. But suggestions or hypotheses of its disease process are the following: Genetic factor Abnormal tendon insertion Anomalous tendons may affect the alignment of the foot. Retracting fibrosis (myofibrosis) Collagen found in all ligaments and tendons are coiled and could be stretched with the exception of Achilles tendon (made up of tightly coiled collagen and cannot be stretched).

Thickening and scarring of fibrous tissue could cause the twisted foot appearance. Neurogenic factors

Innervation changes during the prenatal period could be due to the presence of neurologic events or disorder such as, spina bifida. Studies show that 35% of children with clubfoot have neurologic impairment. Oligohydramnios

Fluid leak during the prenatal period could cause restriction of fetal movements thereby, predisposing to a deformed

foot. Developmental arrest of fetal development Disruption of the medial rotation of the fetal foot could result to a clubfoot condition. Diminished Vascular Circulation Disruption of the branches of the vascular supply of the lower extremity could contribute to misalignment of the foot. Types of True Talipes Deformity

1. 2. 3. 4.

Equinus (plantarflexion) Calcaneus (Dorsiflexion) Varus (foot turns inward) Valgus (foot turns outward)

Some children with this deformity have a combination of the types listed. For example, a child who walks on the heel with the foot turning outwards hascalcaneovalgus disorder while the child who tiptoes with the foot inverted has equinovarus deformity. Diagnostic Evaluation: Physical Examination

Twisted foot appearance should be assessed and gently manipulated. If the straightened foot does not move to a

normal position, true clubfoot is present. Radiography

Use of x-rays is definitive diagnosis for clubfoot as it determines abnormal bone anatomy and assesses the treatment efficiency. Management Categories of treatment:

1. For mild cases: manipulation, cast and splint application (nonsurgical management) 2. For severe cases: surgery Nonsurgical management

denis browne splint

Ponseti Method Applies certain techniques to reduce and correct the deformity to promote normal foot mobility and position. Methods used are the following: 1. Manipulation - Slightly pivoting the bones and stretching the soft tissue 2.

Placement of above the knee cast Frequency of changing the cast is every 5-7 days to accommodate the rapid growth during the first year of life. In most cases, severing of Achilles tendon (tenotomy) is done before the final cast is applied. The reason for doing this is to loosen the foot. The procedure is usually done in a clinic where a local anesthetic is used. A small cut (about 3 mm) is made above the heel of the foot to lengthen the tendon. After the procedure final

casting is done. Final cast is removed after 2-3 weeks when Achilles tendon is already healed. After the final cast is removed: 1. Denis Brown Splints (shoes or boots attached to a bar) are used 23 hours each day for 3 months to maintain the normal foot alignment. For the next 2-4 years the splint is fitted during naps and nighttime only. 2. Passive foot exercises (full range-of-motion) are executed by the primary caregiver to further maintain the position.

Post-tenotomy management Observe for the following:

Drainage on the cast Foul smelling odor from inside the cast. Swelling, redness and irritation at the distal portion of the cast. High fever

ilizarov frame

Ilizarov Technique Method used for complex ankle-foot deformity. Ilizarov frames, the circular structure placed around the limb, are used in this technique which are attached to metal pins and are inserted through the bone. A frame is individually made for each patient and weighs approximately 7 lbs. Placement of the frame requires the administration of a general anesthetic and the procedure may last for several hours. Surgical Management Posteromedial Release The last option for a clubfoot is the release of all tight tendons and ligaments in the posterior and medial parts of the foot. The structures are then put back together in a lengthened position. Tendon Transplant Done at 4-7 years of age when other corrective measures have been ineffective. Complications

Rocker bottom Foot

Vertical talus results from a forceful manipulation causing bone breakage. This then will give rise to a flat foot. Recurrent deformity The corrected foot may return to its deformed state if the parents or primary caregiver fails to apply the methods to further correct the position (e.g. passive foot exercises and Denis Brown splint). Nursing Interventions

1. 2. 3. 4. 5. 6. 7. 8.

Obtain a family and obstetric history for risk factors. After delivery, assess the ankle and foot for a true talipes deformity by straightening the foot. Pseudo-talipes can be realigned to a normal position. For infants with cast assess for circulation, redness and swelling distal from the cast and foul odor. Monitor the infants temperature (for those who underwent tenotomy or surgery). Fever is the first sign of infection. Cautiously evaluate crying. Infants cannot voice out pain. Crying may mean hunger, wet diapers, abdominal pain or tingling sensation from a tight cast. Keep the cast clean and dry by changing diapers frequently. Use a damp cloth and dry cleansers in wiping. Water and soap causes breakdown of cast particles. Place a pillow or padding under the casted area to prevent cast damage and prevent sores from heel pressure. For children with traction, check and cleanse the pin sites frequently.

9. Explain to the parents the importance of passive foot exercises after the final cast is removed. 10. Maintaining the aligned position after the cast application is essential to prevent reoccurrence. 11. Administer analgesics as ordered for pain relief after a surgical correction. 12. Assess coping mechanisms of family and resources available for long-term treatment. Discharge Plan Medication

Acetaminophen (Tylenol) is an analgesic and antipyretic given for pain relief after traction or tenotomy. Do not use Tylenol with NSAIDs or salicylates. Combined use predisposes the child to experience adverse renal

effects. Exercise

Execution of passive foot exercises several times a day for several months to maintain the corrected foot alignment.

Never forcibly evert or pronate the foot during clubfoot casting. This can cause damage to the bones. Treatment Cast application Physiotherapy

Surgery (last option) Health Teaching Cast care:

Frequently change the infants diaper to prevent soiling of the cast. Use dry cleanser in wiping the cast. Ongoing Assessment

Assess the circulation of casted foot. Diet Breastfeeding for infants younger than 4-6 months. For older infants, introduction of solid foods must have the interval of 5-7 days. Spiritual The mother or the primary caregiver is the significant person for the infant; therefore, she should be at the infants side most of the time. Convey expression of parents towards the childs condition. Possible Nursing Diagnosis Risk for Peripheral neurovascular dysfunction R/T mechanical compression (cast or brace) Risk for impaired skin integrity R/T cast application, traction or surgery Acute pain R/T muscular and tissue damage secondary to surgery

1. 2. 3.

4. Risk for Impaired Parenting R/T maladaptive coping strategies secondary to diagnosis of talipes deformity NCP for Clubfoot images from health.allrefer.com, nenky6.mx.ma, advancedfootandanklesd.spaces.live.com andsteps-charity.org.uk