Cleft Lip and Cleft Palate

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Client's father asks the NICU nurse what could have caused this to happen to their daughter and asks what the mother did to cause the baby to look like this. The NICU nurse understands that the spouse is upset and may be in shock and disbelief about their daughter's condition. This type of reaction is normal, especially when the parents are not aware of the condition prenatally and do not have time to prepare for this outcome. Which therapeutic response is appropriate for the NICU nurse to give to Jose?

"I understand this can be upsetting. this deformity occurs early in the pregnancy, often before a woman knows she is pregnant *This therapeutic response acknowledges the client's feelings, and does not place blame on the mother. This is something the spouses need to communicate about at a later time.

Health Promotion and Maintenance Client's postoperative transition goes smoothly and without incident. The nurse prepares the parents for discharge. In client's discharge instructions, the nurse tells the parents that client will need to be seen by orthodontists and prosthodontists. The mother asks the nurse, "Why does she need to see these doctors?" Q: What is the best initial response by the nurse?

"Maria may have improper positioning of her teeth" Answering the mother's question in terms she will understand should be the nurse's first response. Orthodontics and prosthodontics may be needed to correct problems involving the malposition of teeth and maxillary arches.

Psychosocial Integrity/Support Group The parents attend a support group they find after contacting the American Cleft Palate Craniofacial Association. The meeting is conducted by a pediatric psychiatric nurse. A woman in the group tearfully tells the nurse, "I don't know what to say to my 7-year-old daughter who cries when the kids at school make fun of her. We had the best surgeons but she still has a scar, and it is difficult for people to understand what she is saying. She hates to go to school." Q: What should be the nurse's 1st response?

"That is difficult. There are no easy answers when others are ridiculing your child" The nurse should be honest and acknowledge that this is a difficult situation to handle. This openness and honesty will help to establish a trusting relationship that may then allow for the discussion of possible recommendations to solve the problem.

Physiological Integrity/Health Promotion and Maintenance The nurse explains to the mother that she can pump her breast milk and then bottle feed it to her baby using a special bottle nipple. After instructing her on the use of the breast pump, the nurse asks her for a return demonstration.The nurse starts the mother on the pump and returns to the NICU to retrieve the nipple used to feed the newborn. The mother pumps 5 minutes on her left breast then 5 minutes on her right breast. The nurse returns from the NICU approximately 15 minutes later. The parents are concerned because the mother is only able to pump 10 mL of clear, yellow fluid. Q: Jose asks the nurse if that will be enough milk for Maria, or if they should add formula to the breast milk. Which responses by the nurse are the best instructions to give Jose and Blanca about breastfeeding Maria?

-"The breast milk does not need formula supplementation" The breast milk is concentrated with protein and antibodies. The milk produced for the first 2 to 3 days after birth is called colostrum. It does not need to be mixed with formula. -"The sucking of the neonate stimulates the production of milk" The neonate is generally put to breast immediately or shortly after delivery. Current research supports this practice because it appears to increase early maternal infant attachment, increase milk production, and decrease the chances of engorgement. The sucking of the neonate stimulates the production of milk. "This milk is called colostrum and contains immunoglobulins, vitamin K, and a higher protein content than mature milk." Secretory immunoglobulin A (IgA) is found in high levels in colostrum, but levels gradually decrease over the first 14 days of life. Secretory IgA is an immunoglobulin that prevents viruses and bacteria from invading the intestinal mucosa in breastfed newborns, thus protecting them from infection. This whey protein is also believed to play an important role in preventing the development of allergies. Colostrum, for example, is rich in immunoglobulins and vitamin K and has a higher protein content than mature milk; however, it has a lower fat content. Transitional milk replaces colostrum when the mother's milk supply starts increasing, and eventually mature milk becomes the primary milk source.

The nurse explains to the parents that client will swallow excessive air as she suckles. She explains that it is important for her to be burped at least 2 to 3 times during each feeding. The mother asks the nurse why is it important to burp her. The nurse explains that client will swallow excessive air as she suckles. Q: Which facts should the nurse include in her explanation to Jose, on the importance of burping Maria at least 2-3 times during each feeding?

-Prevent gastric distress Due to swallowing excessive air, an infant may experience gastric distress if not burped. -Encourage adequate intake As a result of swallowing air, an infant may experience gastric distention and feel full, and not want to eat enough.

Physiological Integrity The NICU nurse gives the parents approximately 20 minutes to bond with their baby before asking about the plans for feeding client. The mother expresses her initial desire to breastfeed, but she now has hesitations since client has the cleft lip and palate. Q: Which are the best responses by the nurse?

-"Your breast milk is the best option and is customized to meet Maria's physiological needs." Breast milk contains the mother's antibodies that are passed to the infant to protect the infant from viruses and bacteria. The breast milk is customized to meet the infant's needs. The mother's body knows when the infant was born and will produce breast milk composition based on the gestation of the infant. -"The breast milk production is physically beneficial to you and Maria" Breastfeeding and/or pumping the breast milk is beneficial for the mother immediately following delivery because it helps the evolution of the uterus. In addition, breastfeeding and/or pumping the breast milk helps the mother burn more calories as her body produces the breast milk. Long term benefits include lowering the mother's risk of developing type 2 diabetes, certain types of breast cancer, and ovarian cancer. The infant benefits from customized nutrition and immunity found in the maternal antibodies. Long term benefits for the infant include a lower risk of developing asthma, childhood leukemia, obesity, eczema, respiratory and ear infections, necrotizing enterocolitis, SIDS, and type 2 diabetes. -"I am happy to teach you how to pump your breasts so that you can feed your baby your breast milk." This is a good option, as it shows the nurse's support of the mother's desire to breast-feed.

Physiological Integrity Client's five minute APGAR is 10. She is transferred to the intermittent neonatal intensive care unit (NICU) nursery. Her parent accompanies her while the mother is transferred to the recovery room. Client's birth weight is 7 lbs 8 oz (3.40 kgs); her length is 19 in (48.26 cm). She is placed on a radiant warmer to continue with her transition into extrauterine life.The NICU nurse conducts a Ballard exam and her score equates to 40 weeks' gestation, which correlates with her estimated date of birth (EDB). As client continues to adjust to extrauterine life under the radiant warmer, the NICU nurse completes the electronic medical record (EMR) of her physical assessment. X Present/Noted ////. 0 Present/Not noted or observed General Appearance X Color pink X Acrocyanosis present X Flexed posture X Alert X Active Respiratory system X Airway patent X No upper airway congestion X No retractions or nasal flaring X Respiratory rate, 30 to 60 breaths/min X Lungs clear to auscultation bilaterally X Chest expansion symmetric Cardiovascular system X Heart rate strong and regular X No murmurs heard X Pulses strong and equal bilaterally Neurologic system X Moves extremities X Normotonic X Symmetric features, movement X Reflexes present X Sucking X Rooting X Moro X Grasp X Anterior fontanel soft and flat Gastrointestinal system X Abdomen soft, no distention X Cord attached and clamped X Anus appears patent Eyes, Nose, Mouth X Eyes clear 0 Palates intact 0 Nares patent Skin X No signs of birth trauma X No lesions or abrasions Genitourinary system X Normal genitalia Other X No obvious anomalies Comments: Bilateral cleft lip and palate noted; difficult to assess sucking reflex d/t cleft, but infant responds (+) to rooting reflex; lung fields clear to auscultation; have not attempted to feed as of yet; glucose stable at 62 mg/dL (3.44 mmol/L); mother desires to breastfeed; will instruct mother to pump breast milk for infant.

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Client is ready to be discharged. The parents have shown an understanding of the discharge teaching concerning her physical and developmental care. They have lists of the healthcare team members' contact numbers and numerous scheduled appointments. Client will be back in 9 to 12 months for another surgery to repair the cleft palate.

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Client's parent stays in the NICU with her for about one hour. During this time, the NICU nurse explains that the radiant warmer helps client maintain her temperature. The nurse provides additional information about the transitional process period during which she adjusts to extrauterine life. The parent talks to and lovingly strokes his daughter, and she responds appropriately to this loving attention. The parent comments that client acts as though she knows her parents. The nurse explains that around the 5th gestational month a fetus' hearing is developed enough to hear outside the uterus. Therefore, there is a high probability that she recognizes the voices as her parents speaks to her.

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The nurse explains the proper technique for feeding the newborn, stating it is important to feed her in an upright position with her head supported by the feeder's hand or cradled in the feeder's arms. The Haberman nipple is long enough to help the expressed breast milk (EBM) reach the back of client's mouth, utilizing gravity to help it flow down the throat. Client can use jaw compressions to control the EBM at her own pace. The nipple has a one-way valve to prevent blowflow and provide an extra chamber to store expressed breast milk if the feeder feels the need to assist the infant by squeezing the chamber. The nurse successfully initiates client's feeding with the Haberman nipple. She is able to suckle approximately 5 mL, then the nurse stops to burp her. The nurse then places client in the mother's arm to finish the feeding. The mother smiles and talks to client for the remainder of the feeding. The souse appears upset and states concern that client is making a lot of noise while feeding and that some expressed breast milk is leaking out of her nose.

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The parents demonstrate how to safely care for client and are taking her home. She will be seen every 2 weeks in the pediatrician's office to assess her progress and to coordinate with the other members of the healthcare team who will be caring for her.

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How should the nurse respond to Jose's reaction?

-Acknowledge his concerns and educate him that the open cleft cannot muffle the sounds of suckling and swallowing An infant with a cleft means feedings will be noisier than an infant without a cleft. -Acknowledge his concerns and explain that all infants will leak a little bit of their feedings All infants with or without cleft lip and/or palate present will leak a little of their feedings, whether it is from the pursed lips or through clefts.

Cleft Lip Repair Client is now 3 months old and she is considered normal for her height and weight. She is developmentally on target, and her parents exhibit normal bonding behavior. She undergoes successful repair of her cleft lip, spends an hour in the postanesthesia care unit, and then returns to the pediatric surgical unit. Q: Which interventions should the nurse implement?

-Feed Maria w/ a Breck feeder and then rinse her mouth The Breck feeder has a long rubber tip that should be placed in the side of the mouth to avoid the operative area and prevent the infant from sucking on the tubing. -Report bleeding from the suture site to the primary HCP Although pink tinged drainage is normal, bleeding is a sign of a complication and should be reported to the HCP.

Cleft Palate Repair/Client-Centered Care A week after client's first birthday, she returns to the hospital for repair of her cleft palate. She is a thriving and rambunctious toddler who is physically and developmentally on target. She is now 6 hours postoperative after her cleft palate repair. Q: Which interventions should the nurse implement?

-Feed soft, bland baby food mixed w/ water Initially, a child should be fed soft baby food mixed with water and then gradually be fed foods with a thicker consistency. The child should not be given any foods or objects that can tear surfaces of the palate. -Sit the child in an upright position when feeding An upright position will facilitate swallowing and help prevent aspiration.

Based on the newborn assessment that the NICU nurse records in the EMR, which areas need to be addressed?

-Sucking reflex. *The sucking reflex will still be present in a newborn that has a cleft lip and/or palate, due to an intact central nervous system. The infant will demonstrate this reflex, but will not be able to create suction, due to the presence of the cleft palate. -Palates intact Due to the cleft palate present at birth, the nurse marks this as "not present," based on the physical assessment and visible appearance of the cleft. -Sucking reflex -Nares patent Due to the cleft palate and cleft lip present at birth, the nurse marks this as "not present," based on the physical assessment and visible appearance of the clefts.

Physiological Integrity Recent research suggests that the genes responsible for this condition are unknown. Some studies suggest that this condition occurs as a result of some inheritance and environmental factors, but these results are inconclusive. Q: Which causative factors should a pregnant woman eliminate or avoid to prevent cleft lip and / or palate in her unborn child?

-Use of retinoids Research suggests that the absorption of retinoids that a pregnant woman may use on her skin to control acne can cause the formation of cleft lip/palate on a developing fetus. -Consumption of alcohol Consumption of alcohol in any amount is discouraged during pregnancy. There appears to be an increased occurrence of cleft lip/palate in children whose mothers consume alcohol, especially during the first trimester of pregnancy. -Smoking / 2nd hand smoke Smoking in any amount is discouraged during pregnancy. There appears to be an increased occurrence of cleft lip/palate in children whose mothers smoke or are exposed to secondhand smoke during the first trimester of pregnancy.

According to Maslow's hierarchy of needs, which nursing dx for the pediatric pt w/ cleft palate needs to be addressed 1st?

Altered nutrition, less than body requirements *This falls into the first tier, "Physiological Needs." The infant's nutritional status needs to be addressed first, due to the impairment of the cleft lip and palate.

Therapeutic Communication/Client-Centered Care/Psychosocial Integrity Empathy is the source of a nurse's compassion for a client and therapeutic communication is the bridge to get there. As the postpartum night nurse completes rounding, the mother can be seen in her room holding her baby and crying uncontrollably. She states, "It is my fault my daughter is deformed." Q: The client asks the nurse why her baby must suffer punishment for her discretions. Which response by the nurse is likely to be most helpful?

Allow Blanca to cry and verbalize her feelings of grief and guilt The typical parental reaction to an infant with an obvious malformation is guilt, disappointment, grief, sense of loss, and anger. The most therapeutic and helpful intervention the nurse can demonstrate is listening and allowing the mother to express her emotions. Postpone further discussions of treatment options for another time.

When discussing the long-term requirements for Maria's health maintenance, the nurse emphasizes the need for Maria to have frequent hearing tests. Which rationale supports this nursing intervention?

An altered eustachian tube contributes to recurrent ottis media Improper drainage of the middle ear, resulting from abnormal development (cleft palate) and inefficient function of the eustachian tube, contributes to recurrent otitis media with scarring of the tympanic membrane. This can lead to hearing loss if not assessed and treated early.

A Complication Occurs "Push, push, push!" the spouse urges while holding the client's hand and watching as their first-born child makes an entrance into the world. As the infant is delivered, the entire room becomes quiet and the mother sees a look of concern on her spouse's face. The baby girl is crying loudly, but the upper lip appears to be missing and her face looks deformed. The infant is quickly placed under the radiant warmer and dried. Her 1 minute Apgar score is 8. The mother is frightened, and she has not seen her newborn daughter yet. "What is wrong with my baby? What is going on?" she cries. Click for Image Blanca is frightened, and she has not seen her newborn daughter yet. "What is wrong w/ my baby? What is going on?" she cries. Which action should the L&D nurse take?

Answer Blanca's questions and allow her to see her daughter *The nurse should answer the questions and allow her to see her daughter since she is crying and not in immediate danger. This will allow for bonding between the mother and her baby. The nurse should stay with the couple in case they have any questions or need emotional support.

The nurse teaches Jose and Blanca how to care for Maria's incision site after discharge from the hospital. Which instruction will the nurse discuss w/ Maria's parents?

Apply a thin line of antibiotic ointment to the suture line The suture line must be cleaned of formula and any serosanguineous drainage, then antibiotic ointment must be applied to help prevent an infection that can lead to scarring.

Management On the mother's second postpartum day, an unlicensed assistive personnel (UAP) is assigned to help care for the mother. Upon entering the room, the nurse hears her tell the UAP that she will not take a sitz bath. The UAP tells her that she must take a sitz bath as directed by the HCP. Q: What action should the nurse take?

Ask Blanca why she does not want to take a sitz bath The nurse should first address the client and determine the reason for refusing treatment. Some postpartum women may not wish to sit in a bathtub, take sitz baths, or wash their hair for 6 weeks after delivery. In such cases, warm compresses can be used instead of a sitz bath.

Jose calls the nurse's station and tells the nurse that Maria is crying, and he thinks she is in pain. What action should the nurse take first?

Assess the infant's breathing pattern and her incision site The first step of the nursing process is to assess. The nurse must first assess client to rule out any complications of the surgery, such as hemorrhaging or dehiscence prior to medicating for pain. The infant should receive medication if no complications are noted.

Physiological Integrity/Safe and Effective Care Since they are first time parents and client has special needs, the nurse is concerned about the parent's ability to feed their daughter correctly. Q: Which goal must be met before discharging Blanca and Maria from the hospital?

Both parents will feed Maria to demonstrate the proper feeding method Return demonstration is the best means to determine the successful learning of the skill.

Physiological Integrity/Growth and Development The nurse notices that when the door to the mother's room slams, client extends and adducts her arms, and her fingers fan. She looks startled and then begins to cry. Based on Maria's behavior, how should the nurse respond?

Document this behavior in the chart as normal Moro reflex This is a description of a Moro reflex, which is strongest during the first 2 months of age, but begins to disappear after age 3 to 4 months. An infant's developmental reflexes should be routinely assessed and documented.

The nurse discusses w/ Blanca how to provide care of Maria's mouth until her palate repair heals. Which instruction should the nurse include in the teaching?

Encourage Maria to drink water after each feeding Drinking water after eating helps to rinse away any food particles and removes residual sugars from the suture line, thereby reducing the risk for infection.

The L&D nurse knows that many infants w/ cleft lip also have cleft palate. Which assessment technique will determine if the infant has a cleft palate?

Insert a gloved finger and palpate the top of the infant's mouth *The palate is the roof of the mouth, and palpating this area with the finger will determine if the palate is open (cleft) or closed (not cleft).

Physiological Integrity/Safe and Effective Care Client and her parents return to the pediatrician two weeks after discharge. Client appears happy and content and her parents report no problems, with the exception of lack of sleep due to her waking up every 2 to 3 hours to eat. Her vital signs are T 98.9° F (37.2° C), HR 120 beats/min, and respirations 36 breaths/min. Her birth weight was 7 lbs 8 oz (3.40 kgs) and her current weight is 7 lbs 10 oz (3.45 kg). Her skin appears dry and intact and her mucous membranes are pink and moist. Q: Which assessment provides the best indication to the nurse that Mari is thriving in her environment?

Maria's current wt is 7 lbs 10 oz (3.45 kg) Given client's impairment, her weight is the most important indicator of thriving nutritionally. Healthy newborns initially lose some birth weight upon discharge from the hospital. Client lost approximately 8% of her birth weight, but by two weeks of age, she exceeds her birth weight.

Nursing Diagnosis The healthcare team consists of nurses, physicians, and other specialists that must provide accurate information and outline a plan of action for client's future health care. The pediatric nurse notes that the client's health maintenance is an issue. Q: Which intervention should be included when addressing this nursing diagnosis?

Obtain referrals for language-based learning development Speech and language learning impairments are common complications for the child born with a cleft lip and palate. Early intervention can teach parents how to listen for developing speech abnormalities that can be corrected early.

In preparation for the future surgical repair of the cleft lip, the nurse discusses Maria's needs during the early post operative period and tells Jose and Blanca about actions they can begin now to help prepare Maria for that time. Which intervention should the nurse discuss w/ Maria's parents?

Place Maria in elbow restraints for 15 minutes 5 X day so she will be less resistant to the restraints after surgery Following lip repair surgery, infants are placed in elbow restraints to prevent them from rubbing or disturbing the suture line. Practicing this activity will reduce the irritability and resistance associated with the change in routine.

Later in the day, the nurse joins Blanca and Maria in their room to discuss possible outcomes of Maria's disorder. Blanca asks the nurse, "Will Maria ever look normal?" to prepare the client for surgical repair of Maria's cleft lip, which outcome explanation by the nurse is the most important?

Show Blanca pictures of infants who have undergone cosmetic surgery Showing parents photographs of the possible cosmetic improvements that result from surgery, may help relieve the parents' anxiety and provide a sense of hope.

The nurse continues to share info and answer questions about the psychological dvpmt of children w/ cleft lip/palates. Throughout a child's dvpmt, which goal is a priority?

The children dvp healthy personalities and self-esteem Throughout children's development, the development of a healthy personality and self esteem is most important. This must be a priority goal of the multidisciplinary team and families who provide children with a safe and sound physical and emotional environment.

Education/Health Promotion and Maintenance The plastic surgeon meets with client's parents and explains that the initial surgeries to repair the cleft lip and palate are performed in two stages, and that client may also require more surgeries as a teenager, as her body grows. The first surgery to repair the cleft lip occurs at the age of 2 to 3 months. Depending upon client's growth progress, the cleft palate surgery will be performed at approximately 6 to 12 months of age.Although the parents talked to the plastic surgeon about surgery for client, they ask the nurse to explain the information more clearly. The parent asks the nurse, "Why can't the repair of the cleft lip and cleft palate be done at the same time? A year is a long time to wait to repair the palate." Q: How should the nurse respond?

The cleft palate repair is delayed to allow normal palatal changes Cleft palate repair is usually done between 6 to 12 months of age. Surgeons understand the need to repair the palate before onset of babbling at 6 months, other factors (size of the cleft and the child's health) may not permit early palatal repair. Cleft palate repair is usually delayed until 6 to 12 months of age to accommodate palatal changes that take place with normal growth. Delaying the closure of the palate past the time the child begins to speak (after 12 months of age) can result in severe speech difficulties.

Client is now three days old. Her family and the healthcare team are preparing her to be discharged home. The UAP who weighs client that morning reports to the nurse that her weight is now down to 6 lbs 15 oz (3.15 kgs) from her birth weight of 7 lbs 8 oz (3.40 kgs). Q: What is the appropriate nursing action?

The nurse needs to document the weight loss in the EMR. It is a normal finding for newborns to lose a percentage of their birth weight by the third day of life. Most newborns will lose an average of 6 to 8% of their birth weight. Weight loss over 10% of the birth weight should be further investigated. client's current weight is an 8% weight loss from her birth weight.

Psychosocial Integrity The mother reaches out for client and cradles her. The other parent kneels down next to them and explains how the infant recognizes their voices and likes when they talk to her. The spouse tells the mother that the baby will recognize her voice too and encourages her to talk with the baby. Q: The nurse observes Blanca and Jose identifying the likeness of maria to themselves and other family members. Based on this response, which action is the best approach for the nurse to implement next?

The nurse should allow Jose and Blanca time to bond w/ Maria *This is referred to as the claiming process. It gives the parents a changes to find likenesses client has with family members, such as similar eyes or hair and her uniqueness. This process is usually done in the immediate postpartum period, but because client was taken to the Intermittent NICU due to her cleft lip and palate, the mother did not have this opportunity.

Psychosocial Integrity The mother transfers to the postpartum unit and her spouse sits at her bedside. The NICU nurse brings client to the room to instruct and demonstrate how to feed client. As the nurse hands the baby to her mother, she does not reach out, and appears to be hesitant to accept and hold her daughter. Q: Which nursing action would best help facilitate the bonding process and ease Blanca's hesitation to hold her daughter?

The nurse should comment on Maria's beautiful hair while holding Maria *Commenting on client's beautiful, headful of hair helps normalize her and helps the mother focus on her daughter's positive attributes.

Physiological Integrity/Psychological Integrity Due to Blanca's limited prenatal care, she and her husband are unprepared to have an infant born w/ an abnormality. Which prenatal screening could have detected the presence of a cleft lip at 13-14 wks gestation?

Ultrasound screening *A 3D ultrasound can detect cleft lip and palate. A 2D ultrasound can also detect this condition around the 13th to 14th weeks of gestation. Cleft palate by itself, without the presence of a cleft lip, is difficult to diagnose. Twenty to thirty percent of cleft lips are diagnosed by ultrasound, in the U.S.

The nurse has three different feeding devices to try with client. The first device is a feeding bottle with a Haberman nipple, which is often referred to as a "special needs feeder." If the mother appears to have difficulty, then the nurse will attempt a different device. Q: The nurse prepares Blanca's colostrum using the Haberman nipple and attempts to feed Maria. Which action is the most appropriate for the nurse to take?

Utilize Standard Precautions during the procedure. The mother's colostrum is a bodily secretion. It is imperative that the nurse uses Standard Precautions to avoid exposure to potentially infectious agents.


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