Materiały źródłowe

• #1 Hirschsprung Disease (Aganglionic Megacolon) Nursing Care Management – Nurseslabs

  https://nurseslabs.com/hirschsprung-disease-aganglionic-megacolon/

  Hirschsprung disease is a congenital condition that affects the large intestine, resulting in a lack of nerve cells in certain segments of the bowel. This absence of nerve cells leads to a functional obstruction, causing difficulties in passing stool and potential complications like constipation and abdominal distension. […] Nursing care management for a child with aganglionic megacolon includes: […] Assessment involves: […] Based on the assessment data, the major nursing diagnoses for Hirschsprung disease are: […] The major nursing care planning goals for patients with Hirschsprung Disease are: […] Nursing interventions for a child with aganglionic megacolon include: […] Goals are met as evidenced by: […] Documentation in a patient with aganglionic megacolon includes:

• #2 Hirschsprung’s Disease | Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/h/hirschsprung

  Hirschsprungs disease (aganglionic megacolon) is a benign congenital (present at birth) condition that affects how the lower intestine works. […] Each child with Hirschsprungs disease has unique needs. The care team will make a treatment plan for your child’s condition and overall health. Based on your child’s needs and stage of treatment, the care team may include: Specialized pediatric colorectal surgeon and nurse, Pediatric gastroenterologist specializing in motility (motion of the digestive system), Registered dietician, Pelvic floor physical therapist, Psychologist, Social worker, Child life specialist, Other experts as needed. […] Almost all children with Hirschsprungs disease need a surgery called a pull-through procedure. This involves taking out the diseased part of colon and rectum. Then the end of the colon above the diseased part is pulled down and connected to the anus.

• #3 Hirschsprung Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK562142/

  Hirschsprung disease (HD) is a congenital disorder characterized by the absence of ganglion cells (GC) at the Meissner’s plexus (submucosa) and Auerbach’s plexus (muscularis) of the terminal rectum that extends in a variable distance proximally. […] This activity reviews the evaluation and treatment of Hirschsprung disease and highlights the role of the interprofessional team in the care of patients with this condition. […] The diagnosis of Hirschsprung disease (HD) almost exclusively demands surgical intervention. Pediatric healthcare providers should possess a comprehensive understanding of the most popular surgical procedures to assist in the bridging referral phase between the surgeon and the patient’s family. Rectal irrigation before the surgery and in the management of HAEC is highly recommended. It might have a couple of crucial advantages, including colon size decompression and preventing the most devastating complication, enterocolitis.

• #4 Specialized Care for Hirschsprung Disease – USA

  https://universitysurgical.com/specialized-care-for-hirschsprung-disease/

  Hirschsprung Disease is a complex colorectal disorder affecting approximately 1 in 5,000 live births. […] Children affected by this condition often develop constipation or partial or total obstruction of the bowels, leading to discomfort and pain, says Jeremy Fisher, MD, pediatric surgeon with University Surgical Associates. […] The only treatment for Hirschsprung Disease is surgery, where the section of the rectum or colon that isnt functioning properly is removed. […] Typical recovery for this surgery is a few days in the hospital, usually less than a week depending on other factors. […] Parents are also educated about how to look out for enterocolitis or infection that can develop in the digestive tract after surgery. […] Dr. Fisher notes that Hirschsprung Disease is complex and multifaceted and can negatively impact a persons quality of life. […] Pediatric surgeons are trained to treat this disease and it is an essential pediatric surgical condition.

• #5 Hirschsprung Disease | Texas Children’s

  https://www.texaschildrens.org/content/conditions/hirschsprung-disease

  Hirschsprung disease is a condition children are born with that stops or slows the movement of food and waste through the intestine. It affects about 1 in 5,000 children. It is caused by a problem with the nerve cells in the rectum or colon (the lower part of the intestine). Normally these nerves help the muscles in the wall of the bowel squeeze and relax, pushing food and waste along the way. When these nerves are missing, the bowel cannot push the digested food through, and children cannot have normal bowel movements or have fewer bowel movements than normal. […] Even after surgery, children with Hirschsprung disease need specialized care. There are several problems that can occur: Constipation, Diarrhea, Fecal soiling, Frequent episodes of gastrointestinal infection (Hirschsprung-associated enterocolitis). […] Whether the initial surgery was done at Texas Children’s Hospital or elsewhere, we are able to evaluate and care for children with each of these problems with our multidisciplinary team.

• #6 Hirschsprung’s Disease – Noninflammatory Intestinal Disorders for Nursing RN

  https://www.picmonic.com/pathways/nursing/courses/standard/medical-surgical-nursing-pathophysiology-296/noninflammatory-intestinal-disorders-1420/hirschsprungs-disease_2210

  Hirschsprung’s disease is a congenital disorder wherein there is limited or absent intestinal motility due to a lack of ganglion cells in the intestine. […] The deficiency of ganglion cells present in Hirschsprung’s disease prevents the intestine from receiving appropriate signals from the nervous system, resulting in constant sympathetic stimulation; and, therefore, increased bowel tone. Because the intestine is in a contracted state, peristalsis does not occur. […] A newborns first stool is called meconium. In a healthy newborn, meconium should be passed within the first 24 hours after birth. An infant that does not pass meconium within 24 to 48 hours after birth, or one that requires digital stimulation to pass meconium, should be evaluated for Hirschsprungs disease. […] Neonates with Hirschsprung’s disease may exhibit a feeding intolerance, or refusal to feed, with bilious vomiting.

• #7 Care of client with hirschsprung’s disease | PPT

  https://www.slideshare.net/slideshow/care-of-client-with-hirschsprungs-disease/73780104

  1. Care of client with hirschsprungs disease […] 2. Hirschsprung disease, congenital aganglionic megacolon is a developmental disorder of the enteric nervous system and is characterized by an absence of ganglion cells in the distal colon resulting in a functional obstruction. […] 3. Approximately 90% of patients with Hirschsprung disease are diagnosed in the newborn period. […] 4. Hirschsprung disease has been found to be associated with Down syndrome. […] 5. The definitive diagnosis of Hirschsprung disease is confirmed by rectal biopsy, i.e., findings that indicate an absence of ganglion cells. […] 6. The general goals of medical care are 3-fold: 1. To treat the complications of unrecognized or untreated Hirschsprung disease, 2. To institute temporary measures until definitive reconstructive surgery can take place, 3. To manage bowel function after reconstructive surgery.

• #8 Hirschsprung Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care

  https://emedicine.medscape.com/article/178493-treatment

  If Hirschsprung disease is suspected, neonates and children should be assigned to a center where pediatric specialists are available to make the diagnosis and to provide definitive care. […] Consult with pediatric surgeons and pediatric gastroenterologists. Genetic consultation may be indicated (if a heritable or chromosomal anomaly is suspected). […] It is important that conversations between patients and/or caregivers and clinicians take place regarding transitions in care from pediatric providers to adult providers, and that plans for transitional care be implemented early. […] The general goals of medical care are three-fold: (1) to treat the manifestations and complications of untreated Hirschsprung disease, (2) to institute temporizing measures until definitive reconstructive surgery, and (3) to manage the postoperative bowel function.

• #9 Hirschsprung Disease (HD): Symptoms, Diagnosis and Treatment Options | Nationwide Children’s Hospital

  https://www.nationwidechildrens.org/conditions/hirschsprung-disease

  Children with Hirschsprung disease will always need surgery to remove the non-functional segment of the intestine, and to restore the ability to push stool through the digestive tract and out of the body. With proper surgical intervention, the long-term outcomes for children with Hirschsprung disease are excellent, and most will go on to recover normal bowel control and function. […] Treatment for Hirschsprung disease depends on the severity of the disease and your child’s overall health. All children with the condition undergo procedures that help eliminate static stool, and ultimately, surgery to remove the portion of the intestine that is malfunctioning. […] Most children who have treatment and surgery do very well and go on to have normal, healthy bowel function. […] As a first step, doctors will irrigate the bowel to remove trapped waste and gas. The irrigation is done by inserting a tube through the anus to flush out the colon. In most cases, this successfully clears the intestines and provides immediate relief to the child. These irrigations are continued until surgery can be done.

• #10 Total Colonic Hirschsprung Disease | Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/t/total-colonic-hirschsprung-disease

  Hirschsprungs disease can be more complicated as some patients develop an infection in their colon (called enterocolitis). Children suffer from foul smelling diarrhea and crampy abdominal pain. Enterocolitis can be mild or extremely severe, even life threatening. […] All children with Hirschsprung disease require surgical treatment. […] In cases of total colonic Hirschsprung’s disease children need to have the affected non-functioning intestine removed. […] Due to the complexity of the condition, the corrective treatment must be performed in stages. […] Children with total colonic Hirschsprungs disease will require rectal irrigations at the time of initial diagnosis. Performing these irrigations will relieve the obstructed non-functioning bowel by removing the trapped stool and gas. Rectal irrigations are something your healthcare team will teach you how to perform at the time of TCHD diagnosis.

• #11 Hirschsprung Disease: The Role of the Clinical Nurse Specialist

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11120011/

  The clinical nurse specialist (CNS) has the role of supporting and reassuring the family. In very practical terms, this can be in supporting the parents learning of how to undertake the process of rectal washout to support adequate decompression and in facilitating their ability to feed the child orally. […] The CNS will discuss with the family the red flags of concern that would necessitate immediate return for review and provide the family with an array of contact details (both telephone and email) to ensure that family has as a safety net. […] The time leading up to surgery can be a period of stress and concern for families, and many will seek reassurance and further guidance from the CNS at this time. […] One of the most important roles of the CNS is in initiating and supporting effective bowel management for children following reconstruction.

• #12 Hirschsprung Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK562142/

  Hirschsprung disease (HD) is a congenital disorder characterized by the absence of ganglion cells (GC) at the Meissner’s plexus (submucosa) and Auerbach’s plexus (muscularis) of the terminal rectum that extends in a variable distance proximally. […] This activity reviews the evaluation and treatment of Hirschsprung disease and highlights the role of the interprofessional team in the care of patients with this condition. […] The diagnosis of Hirschsprung disease (HD) almost exclusively demands surgical intervention. Pediatric healthcare providers should possess a comprehensive understanding of the most popular surgical procedures to assist in the bridging referral phase between the surgeon and the patient’s family. Rectal irrigation before the surgery and in the management of HAEC is highly recommended. It might have a couple of crucial advantages, including colon size decompression and preventing the most devastating complication, enterocolitis.

• #13 Hirschsprung Disease

  https://www.saralmind.com/nursing/pcl-nursing/pcl-3-rd-year/child-health-nursing/childhood-morbidity-condition-and-their-nursing-management/hirschsprung-disease

  Assess and treat any respiratory problems caused by abdominal distention. […] Observe and measure belly circumference on a regular basis. […] Provide assistance to the child and his or her parents. […] Assist with symptom management prior to surgery, including a low fiber, high calorie, high protein diet. […] Preoperative care includes assessment of child’s health condition, written consent from parents, physical preparations, and regular measurement of abdominal girth. […] Postoperative care includes assessment of child’s general condition, monitoring vital signs, maintaining airway patency, and preventing aspiration. […] Family education on in-home care includes instructions for colostomy dietary management and explaining ostomy care.

• #14 Hirschsprung Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care

  https://emedicine.medscape.com/article/178493-treatment

  The objectives of medical care are to maintain normal fluid and electrolyte balance, to minimize bowel distention and prevent perforation, and to manage complications. […] Postoperatively, routine colonic irrigation and prophylactic antibiotic therapy may decrease the risk of developing enterocolitis. […] For patients who do develop enterocolitis, nasogastric decompression, intravenous fluids, antibiotics, and colonic lavage may be necessary. […] Sodium cromoglycate, a mast cell stabilizer, has also been reported to benefit these patients. […] The patient should have nothing by mouth for 6-8 hours prior to operation. […] Postoperatively, the patient will receive intravenous fluids and antibiotics; however, nothing may be administered by mouth until the passage of flatus or stool signifies return of bowel function.

• #15 Hirschsprung Disease: The Role of the Clinical Nurse Specialist

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11120011/

  The clinical nurse specialist (CNS) has the role of supporting and reassuring the family. In very practical terms, this can be in supporting the parents learning of how to undertake the process of rectal washout to support adequate decompression and in facilitating their ability to feed the child orally. […] The CNS will discuss with the family the red flags of concern that would necessitate immediate return for review and provide the family with an array of contact details (both telephone and email) to ensure that family has as a safety net. […] The time leading up to surgery can be a period of stress and concern for families, and many will seek reassurance and further guidance from the CNS at this time. […] One of the most important roles of the CNS is in initiating and supporting effective bowel management for children following reconstruction.

• #16 Hirschsprung Disease (HD): Symptoms, Diagnosis and Treatment Options | Nationwide Children’s Hospital

  https://www.nationwidechildrens.org/conditions/hirschsprung-disease

  The goals of surgery are to remove the abnormal part of the intestine, and to reconnect a healthy part of the intestine to the anus so that the body can pass waste normally. In most cases, the rectum and the sigmoid colon (the last parts of the large intestine) will need to be removed. […] Surgery is typically performed on a healthy infant that has responded well to bowel irrigations. […] You can expect your child will remain in the hospital for about three to seven days after their procedure, but some children may need to stay longer, depending on the severity of their disease. Your child will be given IV fluids to help maintain hydration and pain medication as needed while they are in the hospital. […] The skin around your child’s anus will need special care once you get home. Your child is likely to pass several stools a day, which can cause the skin to become very irritated. Your healthcare team will talk to you about what types of topical medicine you can use to help protect your child’s bottom.

• #17 Total Colonic Hirschsprung Disease | Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/t/total-colonic-hirschsprung-disease

  Hirschsprungs disease can be more complicated as some patients develop an infection in their colon (called enterocolitis). Children suffer from foul smelling diarrhea and crampy abdominal pain. Enterocolitis can be mild or extremely severe, even life threatening. […] All children with Hirschsprung disease require surgical treatment. […] In cases of total colonic Hirschsprung’s disease children need to have the affected non-functioning intestine removed. […] Due to the complexity of the condition, the corrective treatment must be performed in stages. […] Children with total colonic Hirschsprungs disease will require rectal irrigations at the time of initial diagnosis. Performing these irrigations will relieve the obstructed non-functioning bowel by removing the trapped stool and gas. Rectal irrigations are something your healthcare team will teach you how to perform at the time of TCHD diagnosis.

• #18 Hirschsprung’s Disease: What It Is, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/9844-hirschsprung-disease

  Theres no cure for Hirschsprungs disease, but surgical treatment often results in a positive outcome. There are two types of surgeries to treat Hirschsprungs disease: a pull-through procedure and an ostomy. […] The pull-through procedure is the most common surgery for Hirschsprungs disease and has the best outcome for recovery. […] Your baby may need a colostomy (large intestine) or ileostomy (small intestine) before, or at the same time as, a pull-through procedure. […] Some nonsurgical treatments work well in addition to surgery. They include: Bowel management: A routine involving medicines and/or enemas to make sure your childs pooping habits are healthy. […] Children with Hirschsprungs disease often feel much better soon after pull-through surgery. But some children may still have problems after they heal, including: Accidental pooping (fecal incontinence).

• #19 Hirschsprung’s Disease: Causes, Symptoms, Diagnosis, Treatment

  https://www.webmd.com/children/what-is-hirschsprungs-disease

  Hirschsprung’s disease is a very serious condition. But if it’s found and treated quickly, your child can live a normal life. […] Doctors will usually do one of two types of surgery: […] Pull-through procedure. This surgery removes the part of the large intestine that lacks nerve cells. Then, the surgeon connects the rest of the intestine to the anus. […] Ostomy surgery. This surgery connects the intestine to an opening in the belly. The doctor then attaches an ostomy bag to the outside of the opening to hold waste from the intestine. Ostomy surgery is usually a temporary measure until the child is ready for the pull-through procedure. […] Surgery can help kids with Hirschsprung’s disease poop normally and have a better quality of life. But after the procedure, some children may have problems such as constipation, diarrhea, or incontinence (lack of control over bowel movements or urination).

• #20 Treatment Options for Children with Hirschsprung’s Disease | Children’s National Hospital

  https://www.childrensnational.org/get-care/departments/colorectal/provider-resources/treatment-options-for-hirschsprung-disease

  Surgery is performed using the latest laparoscopic techniques to reduce the risk of complications, allow for shorter hospital stays and result in a faster recovery. Prior to surgery, newborns with intestinal obstruction and children with enterocolitis should have a nasogastric tube placed and receive intravenous fluids with broad-spectrum antibiotics. […] Many children thrive after surgery for Hirschsprung’s disease and experience normal bowel function and an improved quality of life. […] It is imperative that family members and primary care physicians stay alert to the symptoms of HAEC. HAEC can come on suddenly and result in sepsis and possible death if left untreated. […] An organized and methodical approach to diagnosing the issue is key to successfully managing symptoms and developing an appropriate treatment plan.

• #21 Hirschsprung’s Disease Treatment & Care | Dayton Children’s Hospital

  https://www.childrensdayton.org/patients-visitors/services/pediatric-surgery/conditions-we-treat/hirschsprung-disease

  Surgery is the most effective treatment for Hirschsprung disease. The most common surgery to correct Hirschsprung disease involves removing the section of the colon without nerves and reattaching the remaining portion of the colon to the rectum. Often, this can be done through minimally invasive (laparoscopic) surgery immediately after the condition is diagnosed. […] In some cases, the repair may require two steps. In the first step, the pediatric surgeon will remove the unhealthy portion of the colon and then perform a procedure called an ostomy. In an ostomy, the surgeon creates a small opening, or stoma, in the child’s abdomen and attaches the upper, healthy portion of the colon to the opening. The child’s stool passes through the stoma into a bag that is connected to it and needs to be emptied several times a day. This allows the lower part of the colon to heal prior to the second surgery. In the second surgery, the surgeon closes up the opening and attaches the normal portion of the colon to the rectum.

• #22 Hirschsprung Disease in Children | Phoenix Children’s Hospital

  https://phoenixchildrens.org/specialties-conditions/hirschsprung-disease-children

  Hirschsprung disease is treated with surgery called a pull-through procedure. A surgeon removes the part of the large intestine that lacks nerve cells. When possible, the healthy part that is left is connected to the anal opening. Often, the surgery can be done through small incisions (laparoscopy), and only 1 surgery may be needed. […] In some cases, surgery may be done in 2 stages. A child who is very sick from Hirschsprung disease may first need ostomy surgery. This can help the child heal before the pull-through surgery. With ostomy surgery, the diseased part of the large intestine is removed. The end of the healthy intestine is moved to an opening made in the belly. This opening is called a stoma. Stool passes through the stoma and into a bag worn outside the body. The bag must be emptied several times a day.

• #23 Hirschsprung Disease: What It Is and How It’s Treated

  https://www.healthline.com/health/hirschsprungs-disease

  For most people diagnosed with Hirschsprung disease, surgery is the standard treatment. Depending on the type of Hirschsprung disease, different procedures might be used. […] There are two main types of Hirschsprung disease surgeries: pull-through procedure (corrective surgery) or ostomy surgery. […] During a pull-through procedure, a surgeon removes the part of the large intestines that’s missing nerve cells. Then the remaining intestines are surgically connected to the rectum. This surgery is done either laparoscopically or via open surgery. […] This surgery is usually recommended for people who have more serious complications like perforations, megacolon, or Hirschsprung-associated enterocolitis. An ostomy essentially allows the intestines time to heal. The procedure may sometimes be recommended before performing a pull-through procedure.

• #24 Hirschsprung Disease (HD): Symptoms, Diagnosis and Treatment Options | Nationwide Children’s Hospital

  https://www.nationwidechildrens.org/conditions/hirschsprung-disease

  The goals of surgery are to remove the abnormal part of the intestine, and to reconnect a healthy part of the intestine to the anus so that the body can pass waste normally. In most cases, the rectum and the sigmoid colon (the last parts of the large intestine) will need to be removed. […] Surgery is typically performed on a healthy infant that has responded well to bowel irrigations. […] You can expect your child will remain in the hospital for about three to seven days after their procedure, but some children may need to stay longer, depending on the severity of their disease. Your child will be given IV fluids to help maintain hydration and pain medication as needed while they are in the hospital. […] The skin around your child’s anus will need special care once you get home. Your child is likely to pass several stools a day, which can cause the skin to become very irritated. Your healthcare team will talk to you about what types of topical medicine you can use to help protect your child’s bottom.

• #25 Hirschsprung Disease

  https://www.saralmind.com/nursing/pcl-nursing/pcl-3-rd-year/child-health-nursing/childhood-morbidity-condition-and-their-nursing-management/hirschsprung-disease

  Assess and treat any respiratory problems caused by abdominal distention. […] Observe and measure belly circumference on a regular basis. […] Provide assistance to the child and his or her parents. […] Assist with symptom management prior to surgery, including a low fiber, high calorie, high protein diet. […] Preoperative care includes assessment of child’s health condition, written consent from parents, physical preparations, and regular measurement of abdominal girth. […] Postoperative care includes assessment of child’s general condition, monitoring vital signs, maintaining airway patency, and preventing aspiration. […] Family education on in-home care includes instructions for colostomy dietary management and explaining ostomy care.

• #26 Hirschsprung Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care

  https://emedicine.medscape.com/article/178493-treatment

  The objectives of medical care are to maintain normal fluid and electrolyte balance, to minimize bowel distention and prevent perforation, and to manage complications. […] Postoperatively, routine colonic irrigation and prophylactic antibiotic therapy may decrease the risk of developing enterocolitis. […] For patients who do develop enterocolitis, nasogastric decompression, intravenous fluids, antibiotics, and colonic lavage may be necessary. […] Sodium cromoglycate, a mast cell stabilizer, has also been reported to benefit these patients. […] The patient should have nothing by mouth for 6-8 hours prior to operation. […] Postoperatively, the patient will receive intravenous fluids and antibiotics; however, nothing may be administered by mouth until the passage of flatus or stool signifies return of bowel function.

• #27 Hirschsprung Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care

  https://emedicine.medscape.com/article/178493-treatment

  Upon resumption of bowel function, tube feeding or formula/breast milk may resume. […] Clear liquids are delivered by mouth, and the diet may be advanced until the feeding goals are met. […] Diets consisting of fresh fruits, vegetables, and high-fiber articles may improve postoperative bowel function. […] With regard to activity, limit physical activity for about 6 weeks to allow the incisions to heal properly (applies more to older children). […] After a definitive pull-through procedure is performed, the patient should achieve normal growth and development. […] Patients should be monitored for their bowel habit. […] Patients who retain stool despite laxative therapy may require enemas.

• #28 Hirschsprung Disease (HD): Symptoms, Diagnosis and Treatment Options | Nationwide Children’s Hospital

  https://www.nationwidechildrens.org/conditions/hirschsprung-disease

  The goals of surgery are to remove the abnormal part of the intestine, and to reconnect a healthy part of the intestine to the anus so that the body can pass waste normally. In most cases, the rectum and the sigmoid colon (the last parts of the large intestine) will need to be removed. […] Surgery is typically performed on a healthy infant that has responded well to bowel irrigations. […] You can expect your child will remain in the hospital for about three to seven days after their procedure, but some children may need to stay longer, depending on the severity of their disease. Your child will be given IV fluids to help maintain hydration and pain medication as needed while they are in the hospital. […] The skin around your child’s anus will need special care once you get home. Your child is likely to pass several stools a day, which can cause the skin to become very irritated. Your healthcare team will talk to you about what types of topical medicine you can use to help protect your child’s bottom.

• #29 Hirschsprung Disease in Children | Phoenix Children’s Hospital

  https://phoenixchildrens.org/specialties-conditions/hirschsprung-disease-children

  While older children will feel better as well, they must adjust to living with a permanent ostomy. This means learning how to take care of the stoma and how to change the ostomy pouch. Living with an ostomy can be very hard for the child and the caretaker. A special nurse, called an ostomy nurse, can help you and your child learn how to care for the ostomy. The nurse can also provide education, demonstrations on ostomy care, problem-solving help, community resources, emotional support, and encouragement. […] Your child’s bowel function may be affected after surgery. The most common long-term problems include bowel control and leaking stool, constipation, and infections. […] Talk with your child’s healthcare provider about your child’s specific situation. […] Caring for a child with Hirschsprung disease can be emotionally and physically exhausting. If you are struggling, ask your healthcare provider about home care support or community-based support groups or resources.

• #30 Hirschsprung Disease in Children

  https://healthlibrary.vidanthealth.com/Library/Wellness/TodaysMedicine/90,P01999

  While older children will feel better as well, they must adjust to living with a permanent ostomy. This means learning how to take care of the stoma and how to change the ostomy pouch. Living with an ostomy can be very hard for the child and the caretaker. A special nurse, called an ostomy nurse, can help you and your child learn how to care for the ostomy. The nurse can also provide education, demonstrations on ostomy care, problem-solving help, community resources, emotional support, and encouragement. […] Caring for a child with Hirschsprung disease can be emotionally and physically exhausting. If you are struggling, ask your healthcare provider about home care support or community-based support groups or resources.

• #31 Hirschsprung’s Disease – familydoctor.org

  https://familydoctor.org/condition/hirschsprungs-disease/

  Children may need to make some lifestyle changes to help manage their condition. For instance, eating foods that are high in fiber can help reduce constipation. The large intestine absorbs water and salt that the body needs. If your child has a large portion of the intestine removed, it will absorb less. Make sure your child gets plenty of fluids and salt to make up for this. […] Your baby and older children will feel better after ostomy surgery because they will be able to pass stool easily. Adjusting to life with an ostomy pouch will take time and may make your child feel different. Your child will need to learn how to care for the stoma and how to change the ostomy pouch. With a few lifestyle changes, children with ostomies can lead normal lives. An ostomy nurse can answer questions and show your child how to care for an ostomy.

• #32 Hirschsprung’s Disease: Post-Surgery Care | Patient Education | UCSF Benioff Children’s Hospitals

  https://www.ucsfbenioffchildrens.org/education/hirschsprungs-disease-post-surgery-care

  After the operation for Hirschsprung’s disease, children who do not have a stoma will have a lot of diarrhea. It is important to protect the skin around the anus at all times to prevent irritation, using a cream called „butt balm.” Your child’s nurse will teach you how to mix and apply the cream. […] Do not stop using the skin protective products until your child is having fewer bowel movements, usually after many weeks. If your child develops a rash that does not get better, please call Pediatric Surgery. […] If your child has a stoma, you will need to make an appointment to be seen one after discharge. If your child has a primary surgery or stoma closure, he or she will need to be seen in the Pediatric Surgery office two weeks after the operation. At this visit, your child’s anus will be checked to make sure the opening is large enough for stool to pass easily. Sometimes the anus has to be dilated, or stretched, for several weeks or months following surgery. If this is necessary, your child’s surgeon or surgical nurse will teach you how to do dilations at home.

• #33 Hirschsprung’s disease

  https://www.nhs.uk/conditions/hirschsprungs-disease/

  It’s important they drink plenty of fluids as they recover. […] At first they’ll probably have a sore bottom when they poo. It can help to: leave their bottom open to the air whenever possible […] use nappy cream after each change. […] Call 999 or go to your nearest AE if your child develops problems such as a swollen belly, new tummy pain, a fever or foul-smelling diarrhoea.

• #34 Hirschsprung Disease: What You Need to Know

  https://www.massgeneral.org/children/hirschsprung-disease

  Your child’s pediatric surgeon will help you decide when they can perform pull-through surgery. In this surgery, the aganglionic part of your child’s intestine is removed and the healthy section of the intestine (the part with normal nerve cells) is attached to the rectum. Most babies with Hirschsprungs disease have surgery within the first several months of age. […] After surgery, your child’s care team can help with pain control and monitor for stool output (how much stool your child produces and passes). They can also help with feeding once your child’s digestive tract starts to work again. […] The pediatric surgeon may ask you to continue to dilate your child’s anus every day to keep it from scarring. Caring for the skin around the anus is very important. It is common for children to have diarrhea after pull-through surgery. Diarrhea can irritate the skin around the anus. Apply barrier creams immediately after surgery to help prevent severe diaper rash.

• #35 Hirschsprung’s Disease: Post-Surgery Care | Patient Education | UCSF Benioff Children’s Hospitals

  https://www.ucsfbenioffchildrens.org/education/hirschsprungs-disease-post-surgery-care

  After the operation for Hirschsprung’s disease, children who do not have a stoma will have a lot of diarrhea. It is important to protect the skin around the anus at all times to prevent irritation, using a cream called „butt balm.” Your child’s nurse will teach you how to mix and apply the cream. […] Do not stop using the skin protective products until your child is having fewer bowel movements, usually after many weeks. If your child develops a rash that does not get better, please call Pediatric Surgery. […] If your child has a stoma, you will need to make an appointment to be seen one after discharge. If your child has a primary surgery or stoma closure, he or she will need to be seen in the Pediatric Surgery office two weeks after the operation. At this visit, your child’s anus will be checked to make sure the opening is large enough for stool to pass easily. Sometimes the anus has to be dilated, or stretched, for several weeks or months following surgery. If this is necessary, your child’s surgeon or surgical nurse will teach you how to do dilations at home.

• #36 Hirschsprung’s Disease: Post-Surgery Care | Patient Education | UCSF Benioff Children’s Hospitals

  https://www.ucsfbenioffchildrens.org/education/hirschsprungs-disease-post-surgery-care

  After the operation for Hirschsprung’s disease, children who do not have a stoma will have a lot of diarrhea. It is important to protect the skin around the anus at all times to prevent irritation, using a cream called „butt balm.” Your child’s nurse will teach you how to mix and apply the cream. […] Do not stop using the skin protective products until your child is having fewer bowel movements, usually after many weeks. If your child develops a rash that does not get better, please call Pediatric Surgery. […] If your child has a stoma, you will need to make an appointment to be seen one after discharge. If your child has a primary surgery or stoma closure, he or she will need to be seen in the Pediatric Surgery office two weeks after the operation. At this visit, your child’s anus will be checked to make sure the opening is large enough for stool to pass easily. Sometimes the anus has to be dilated, or stretched, for several weeks or months following surgery. If this is necessary, your child’s surgeon or surgical nurse will teach you how to do dilations at home.

• #37 The Outpatient Management of Hirschsprung’s Disease – Department of Surgery

  https://www.surgery.wisc.edu/2018/03/02/the-outpatient-management-of-hirschsprungs-disease/

  Hirschsprungs is a congenital disease often diagnosed within the first week of life. […] individual follow-up and care is a delicate balance that illustrates the need for strong communication between surgeon and primary care physician to advance a successful care plan for each patient. […] Surgery is required for Hirschsprungs disease. […] Post-surgery, children will typically undergo rectal dilations to prevent narrowing, or stricture formation, of the anus for a period of up to one year. […] In the long-term, experience has taught us that even after surgery, patients with Hirschsprungs disease often have significant bowel dysfunction. […] In fact, stool leakage or incontinence after surgery is most often a sign of underlying constipation and can typically be controlled with appropriate bowel management.

• #38 Hirschsprung’s Disease Treatment & Care | Dayton Children’s Hospital

  https://www.childrensdayton.org/patients-visitors/services/pediatric-surgery/conditions-we-treat/hirschsprung-disease

  The general outlook for children who have been treated surgically for Hirschsprung disease is excellent. After surgery, most can pass stool normally and have no lasting complications. However, a few kids might continue to have symptoms, including constipation and bowel control problems. Some children benefit from daily stretching of the anal opening for several months after the surgery. […] Children with Hirschsprung disease also continue to be at risk of developing bowel infection (enterocolitis) after surgery, especially in the first year. Signs and symptoms of enterocolitis can include: bleeding from the rectum, diarrhea, fever, swollen abdomen, and vomiting. You should call your child’s doctor immediately if any of these signs and symptoms occur. Your child’s doctor may recommend home rectal irrigations (fluid placed into the colon via a small tube) to prevent or treat enterocolitis.

• #39 Treatment Options for Children with Hirschsprung’s Disease | Children’s National Hospital

  https://www.childrensnational.org/get-care/departments/colorectal/provider-resources/treatment-options-for-hirschsprung-disease

  Hirschsprung’s disease is a heterogeneous disease that is caused by a variety of genetic mutations and is characterized by the absence of ganglion cells that are present in the distal intestine. The treatment of Hirschsprung’s disease is primarily surgical, and involves resection of the aganglionic bowel and reconstruction using one of several techniques: the Swenson, Soave and Duhamel procedures. […] Although there have been many advances in the diagnostic and surgical approaches for Hirschsprung’s disease, many of these children continue to have ongoing functional problems even after surgical correction. The most common postoperative complications are persistent obstructive symptoms, soiling and Hirschsprung’s associated enterocolitis. […] To best treat these children and accurately identify the problem, the latest diagnostic techniques are implemented, including contrast enema and anorectal manometry. A definitive diagnosis is based on a rectal biopsy.

• #40 Hirschsprung Disease in Children – Stanford Medicine Children’s Health

  https://www.stanfordchildrens.org/en/topic/default?id=hirschsprung-disease-in-children-90-P01999

  Hirschsprung disease is treated with surgery called a pull-through procedure. A surgeon removes the part of the large intestine that lacks nerve cells. When possible, the healthy part that is left is connected to the anal opening. […] Treatment will depend on your child’s symptoms, age, and general health. It will also depend on how severe the condition is. […] Your child’s bowel function may be affected after surgery. The most common long-term problems include bowel control and leaking stool, constipation, and infections. […] Problems that may occur after surgery depend on the amount of intestine that lacked nerve cells, and how much intestine was removed. […] Talk with your child’s healthcare provider about your child’s specific situation.

• #41 Hirschsprung’s disease – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hirschsprungs-disease/diagnosis-treatment/drc-20351561

  For most people, Hirschsprung’s disease is treated with surgery to bypass or remove the part of the colon that’s lacking nerve cells. There are two ways this can be done: a pull-through surgery or an ostomy surgery. […] After surgery, most children are able to pass stool through the anus. […] If your child has constipation after surgery for Hirschsprung’s disease, discuss with your doctor whether to try any of the following: Serve high-fiber foods. If your child eats solid foods, include high-fiber foods. […] Encourage physical activity. Daily aerobic activity helps promote regular bowel movements. […] Laxatives (only as directed by your child’s doctor). If your child doesn’t respond to or can’t tolerate increased fiber, water or physical activity, certain laxatives medications to encourage bowel movements might help relieve constipation.

• #42 Hirschsprung’s Disease Treatment & Care | Dayton Children’s Hospital

  https://www.childrensdayton.org/patients-visitors/services/pediatric-surgery/conditions-we-treat/hirschsprung-disease

  Some children continue to experience constipation after their surgery and may need to make modifications to their lifestyle and diet to encourage more comfortable and regular bowel movements. If your child continues to experience constipation after surgery, discuss with your pediatric gastroenterologist or pediatric surgeon whether to try any of the following: Serve high-fiber foods, Increase fluids, Encourage physical activity, Laxatives (only as directed by your child’s doctor).

• #43 The Outpatient Management of Hirschsprung’s Disease – Department of Surgery

  https://www.surgery.wisc.edu/2018/03/02/the-outpatient-management-of-hirschsprungs-disease/

  Hirschsprungs is a congenital disease often diagnosed within the first week of life. […] individual follow-up and care is a delicate balance that illustrates the need for strong communication between surgeon and primary care physician to advance a successful care plan for each patient. […] Surgery is required for Hirschsprungs disease. […] Post-surgery, children will typically undergo rectal dilations to prevent narrowing, or stricture formation, of the anus for a period of up to one year. […] In the long-term, experience has taught us that even after surgery, patients with Hirschsprungs disease often have significant bowel dysfunction. […] In fact, stool leakage or incontinence after surgery is most often a sign of underlying constipation and can typically be controlled with appropriate bowel management.

• #44 Total Colonic Hirschsprung Disease | Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/t/total-colonic-hirschsprung-disease

  Hirschsprungs disease can be more complicated as some patients develop an infection in their colon (called enterocolitis). Children suffer from foul smelling diarrhea and crampy abdominal pain. Enterocolitis can be mild or extremely severe, even life threatening. […] All children with Hirschsprung disease require surgical treatment. […] In cases of total colonic Hirschsprung’s disease children need to have the affected non-functioning intestine removed. […] Due to the complexity of the condition, the corrective treatment must be performed in stages. […] Children with total colonic Hirschsprungs disease will require rectal irrigations at the time of initial diagnosis. Performing these irrigations will relieve the obstructed non-functioning bowel by removing the trapped stool and gas. Rectal irrigations are something your healthcare team will teach you how to perform at the time of TCHD diagnosis.

• #45 Hirschsprung’s Disease – familydoctor.org

  https://familydoctor.org/condition/hirschsprungs-disease/

  Hirschsprung disease is a rare birth defect that affects the large intestine (bowel). This condition occurs while a baby is in the womb. It is caused by missing nerve cells in the intestine. These nerve cells tell your intestine to push stools through your bowel. Cells may be missing from a small part of the intestine near the bottom (anus). Or cells may be missing from a large part of the intestine. This causes stools to back up in the intestine. It causes severe constipation, blockage, and colon infections. […] Surgery is the only way to treat Hirschsprung’s disease. It should be done as soon as it is diagnosed. The type of surgery your child has depends on the severity of the disease. […] After surgery, most children lead normal lives. They may have minor health problems because of the disease. These include diarrhea, constipation, or other problems passing stool. Some children develop an infection called enterocolitis (digestive tract inflammation). This can be serious, so call your doctor right away if your child has the following symptoms: Vomiting, Fever, Swollen stomach, Abnormal fatigue, Blood in the stool.

• #46 Hirschsprung’s Disease | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/hirschsprung-s-disease

  Once a child recovered after a couple of months, then we do frequent follow-ups so that we can monitor their patterns of bowel movements and eating and make sure they thrive. […] The surgery’s only the beginning of a very long journey, and I’m glad that we have a program that’s willing to spend the time with these families that it takes. […] They know that they can call us no matter what time of day or night it is. There’s always either one of the nurse practitioners or surgeon that can answer their phone calls. […] The Pediatric Colorectal Program will continue to follow your child’s progress closely after surgery. […] If your child has any of the following symptoms, call us right away or take your child to an emergency room: Explosive diarrhea, Unusually foul-smelling diarrhea, Gray or pale-colored stool, Vomiting, Abdominal distension (swollen stomach area), Fever (with any of the other symptoms listed above), Lethargy (unusual sleepiness). […] Your child will have access to expert pediatric colorectal surgeons, gastroenterologists, urologists and other specialists who understand the unique challenges faced by children with colorectal conditions and their families.

• #47 Hirschsprung’s Disease: Nursing Management and Evidence-Based Care | NursingAnswers.net

  https://nursinganswers.net/essays/hirschsprungs-disease-nursing-management-and-evidence-based-care.php

  Hirschsprungs disease also known as congenital megacolon or aganglionic megacolon, is a rare birth defect of the colon in which the colon is missing vital nerve cells that are required to initiate peristalsis. […] There is currently no education or preventative measures that can be taken to prevent a child from developing or getting Hirschsprungs disease. However, one of the most common complications associated with Hirschsprungs disease is enterocolitis and is responsible for more than half of the deaths associated with Hirschsprungs disease. The best way to prevent Hirschsprung-Associated Enterocolitis is early diagnosis and treatment. […] Due to this a nurse must be aware of the signs and symptoms of Hirschsprung-Associated Enterocolitis which include fever, abdominal distention, explosive watery diarrhea, foul-smelling stools, pain, rectal bleeding, and poor appetite and can occur prior to or after treatment for Hirschsprungs disease.

• #48 Hirschsprung Disease: What You Need to Know

  https://www.massgeneral.org/children/hirschsprung-disease

  Most children do very well after their pull-through surgery. Some may develop problems with how their digestive tract works. This can include obstructive symptoms, fecal incontinence and enterocolitis. […] Enterocolitis must be treated promptly. If the enterocolitis is mild, treatment includes antibiotics and rectal irrigation. More severe cases require admission to the hospital for rectal irrigations, IV antibiotics and IV fluids.

• #49 Hirschsprung Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care

  https://emedicine.medscape.com/article/178493-treatment

  The objectives of medical care are to maintain normal fluid and electrolyte balance, to minimize bowel distention and prevent perforation, and to manage complications. […] Postoperatively, routine colonic irrigation and prophylactic antibiotic therapy may decrease the risk of developing enterocolitis. […] For patients who do develop enterocolitis, nasogastric decompression, intravenous fluids, antibiotics, and colonic lavage may be necessary. […] Sodium cromoglycate, a mast cell stabilizer, has also been reported to benefit these patients. […] The patient should have nothing by mouth for 6-8 hours prior to operation. […] Postoperatively, the patient will receive intravenous fluids and antibiotics; however, nothing may be administered by mouth until the passage of flatus or stool signifies return of bowel function.

• #50 Hirschsprung Disease: The Role of the Clinical Nurse Specialist

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11120011/

  Hirschsprung disease is a life-long condition that can have a significant impact on both children and their families. This article explores the role of the clinical nurse specialist and the support they can provide from initial diagnosis through the patients surgical journey and right through to transition into adult services. Through the provision of education, training, signposting of social and psychological support, and linking in with community-based services, the clinical nurse specialist can help the child and family to limit that impact of the disease. […] They can provide a vital interface between the family and their needs and requirements while outside of hospital and the in-hospital medical team. They are vital in the support of the family as they deal with the trauma of understanding the new diagnosis and its implications for their child.

• #51 Hirschsprung Disease: The Role of the Clinical Nurse Specialist

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11120011/

  The clinical nurse specialist (CNS) has the role of supporting and reassuring the family. In very practical terms, this can be in supporting the parents learning of how to undertake the process of rectal washout to support adequate decompression and in facilitating their ability to feed the child orally. […] The CNS will discuss with the family the red flags of concern that would necessitate immediate return for review and provide the family with an array of contact details (both telephone and email) to ensure that family has as a safety net. […] The time leading up to surgery can be a period of stress and concern for families, and many will seek reassurance and further guidance from the CNS at this time. […] One of the most important roles of the CNS is in initiating and supporting effective bowel management for children following reconstruction.

• #52 Hirschsprung’s Disease: Nursing Management and Evidence-Based Care | NursingAnswers.net

  https://nursinganswers.net/essays/hirschsprungs-disease-nursing-management-and-evidence-based-care.php

  Infants and young children require more frequent assessments as they have a higher risk of developing enterocolitis due to their weakened immune systems. […] Nursing care for Hirschsprungs disease is primarily depends on the age of the child and treatment required. During the neonatal period, diagnosis support is the most immediate concern. A nurse may help a parent adjust to the new diagnosis, assist with parent-child bonding, prepare and support the parents during the medical-surgical decision making process, and teach the parents about how to perform post-operative care. […] A nurse is also responsible for preparing the child for surgery. This includes making sure the patient is stable enough for surgery and if not initiating systematic treatments which may include; enemas, and/or low fiber, high calorie, high-protein diets.

• #53 Hirschsprung Disease: The Role of the Clinical Nurse Specialist

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11120011/

  The clinical nurse specialist (CNS) has the role of supporting and reassuring the family. In very practical terms, this can be in supporting the parents learning of how to undertake the process of rectal washout to support adequate decompression and in facilitating their ability to feed the child orally. […] The CNS will discuss with the family the red flags of concern that would necessitate immediate return for review and provide the family with an array of contact details (both telephone and email) to ensure that family has as a safety net. […] The time leading up to surgery can be a period of stress and concern for families, and many will seek reassurance and further guidance from the CNS at this time. […] One of the most important roles of the CNS is in initiating and supporting effective bowel management for children following reconstruction.

• #54 Hirschsprung’s Disease | Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/h/hirschsprung

  Hirschsprungs disease (aganglionic megacolon) is a benign congenital (present at birth) condition that affects how the lower intestine works. […] Each child with Hirschsprungs disease has unique needs. The care team will make a treatment plan for your child’s condition and overall health. Based on your child’s needs and stage of treatment, the care team may include: Specialized pediatric colorectal surgeon and nurse, Pediatric gastroenterologist specializing in motility (motion of the digestive system), Registered dietician, Pelvic floor physical therapist, Psychologist, Social worker, Child life specialist, Other experts as needed. […] Almost all children with Hirschsprungs disease need a surgery called a pull-through procedure. This involves taking out the diseased part of colon and rectum. Then the end of the colon above the diseased part is pulled down and connected to the anus.

• #55 ERNICA guidelines for the management of rectosigmoid Hirschsprung’s disease | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-01362-3

  Structured follow-up to adulthood, including transition of care is indicated in HSCR. […] The availability of specialist nurses improves communication and access to care, and may reduce hospitalizations/admissions and health care costs. […] Achieving optimal outcomes requires collaboration between medical specialists, nurses and auxiliary resources, including psychologists and sexual therapists, physical and nutritional therapists. […] Planning transition of care should commence sufficiently early, around 13-14 years of age to allow sufficient time for adjustment and should have sufficient flexibility to allow overlap and contact between the existing and future practitioners until patients are satisfactorily established in adult care.

• #56 Hirschsprung Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK562142/

  An interprofessional team approach to treatment based on a bio-psychosocial view can significantly reduce the postoperative chronic bowel problems of the majority of children with operated HD. […] Experienced nurses are also crucial members of the group as they will not only monitor the patient’s vital signs and danger signs of possible devastating complications, including HAEC but also assist in educating the patient and family.

• #57 Hirschsprung Disease: The Role of the Clinical Nurse Specialist

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11120011/

  The CNS can support families through establishing simple routines for potty training and utilising natural physiological phenomena such as the gastrocolic reflex. […] Ensuring that the educational environment is aware of a childs needs from a continence perspective is important and is a key role of the CNS within the MDT. […] The involvement of clinical psychology at this stage is also important for support and therapy to ensure a smooth transition. […] A clinical nurse specialist has the unique ability to share in this journey from birth to transition and provide an effective interface between the clinical in-hospital environment and the day-to-day life of home and school.

• #58 ERNICA guidelines for the management of rectosigmoid Hirschsprung’s disease | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-01362-3

  Structured follow-up to adulthood, including transition of care is indicated in HSCR. […] The availability of specialist nurses improves communication and access to care, and may reduce hospitalizations/admissions and health care costs. […] Achieving optimal outcomes requires collaboration between medical specialists, nurses and auxiliary resources, including psychologists and sexual therapists, physical and nutritional therapists. […] Planning transition of care should commence sufficiently early, around 13-14 years of age to allow sufficient time for adjustment and should have sufficient flexibility to allow overlap and contact between the existing and future practitioners until patients are satisfactorily established in adult care.

• #59 Hirschsprung Disease: The Role of the Clinical Nurse Specialist

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11120011/

  The CNS can support families through establishing simple routines for potty training and utilising natural physiological phenomena such as the gastrocolic reflex. […] Ensuring that the educational environment is aware of a childs needs from a continence perspective is important and is a key role of the CNS within the MDT. […] The involvement of clinical psychology at this stage is also important for support and therapy to ensure a smooth transition. […] A clinical nurse specialist has the unique ability to share in this journey from birth to transition and provide an effective interface between the clinical in-hospital environment and the day-to-day life of home and school.

• #60 Hirschsprung Disease: The Role of the Clinical Nurse Specialist

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11120011/

  Hirschsprung disease is a life-long condition that can have a significant impact on both children and their families. This article explores the role of the clinical nurse specialist and the support they can provide from initial diagnosis through the patients surgical journey and right through to transition into adult services. Through the provision of education, training, signposting of social and psychological support, and linking in with community-based services, the clinical nurse specialist can help the child and family to limit that impact of the disease. […] They can provide a vital interface between the family and their needs and requirements while outside of hospital and the in-hospital medical team. They are vital in the support of the family as they deal with the trauma of understanding the new diagnosis and its implications for their child.

• #61 Hirschsprung’s Disease: Nursing Management and Evidence-Based Care | NursingAnswers.net

  https://nursinganswers.net/essays/hirschsprungs-disease-nursing-management-and-evidence-based-care.php

  Immediate postoperative care includes monitoring of vital signs, maintenance of fluid and electrolyte balance, and pain management. During the postoperative period, the nurse should include the child and their parents in care. This includes how to monitor for signs of wound infection and irregular bowel movement passage. […] While Hirschsprungs disease is curable, if left untreated it can result in death. However, many of the procedures used to treat Hirschsprungs disease often lead to postoperative complications. Long-term these complications can lead to a poor quality of life and severely affect the child psychological health. […] Until then, it is important that healthcare providers remain vigilant to the signs and symptoms related to these complications and intervene when appropriate. Supportive care may also be beneficial for these children, especially when self-esteem is affected.

• #62 Hirschsprung Disease in Children | Phoenix Children’s Hospital

  https://phoenixchildrens.org/specialties-conditions/hirschsprung-disease-children

  While older children will feel better as well, they must adjust to living with a permanent ostomy. This means learning how to take care of the stoma and how to change the ostomy pouch. Living with an ostomy can be very hard for the child and the caretaker. A special nurse, called an ostomy nurse, can help you and your child learn how to care for the ostomy. The nurse can also provide education, demonstrations on ostomy care, problem-solving help, community resources, emotional support, and encouragement. […] Your child’s bowel function may be affected after surgery. The most common long-term problems include bowel control and leaking stool, constipation, and infections. […] Talk with your child’s healthcare provider about your child’s specific situation. […] Caring for a child with Hirschsprung disease can be emotionally and physically exhausting. If you are struggling, ask your healthcare provider about home care support or community-based support groups or resources.

• #63 Hirschsprung Disease in Children

  https://healthlibrary.vidanthealth.com/Library/Wellness/TodaysMedicine/90,P01999

  While older children will feel better as well, they must adjust to living with a permanent ostomy. This means learning how to take care of the stoma and how to change the ostomy pouch. Living with an ostomy can be very hard for the child and the caretaker. A special nurse, called an ostomy nurse, can help you and your child learn how to care for the ostomy. The nurse can also provide education, demonstrations on ostomy care, problem-solving help, community resources, emotional support, and encouragement. […] Caring for a child with Hirschsprung disease can be emotionally and physically exhausting. If you are struggling, ask your healthcare provider about home care support or community-based support groups or resources.

• #64 Hirschsprung Disease: The Role of the Clinical Nurse Specialist

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11120011/

  The CNS can support families through establishing simple routines for potty training and utilising natural physiological phenomena such as the gastrocolic reflex. […] Ensuring that the educational environment is aware of a childs needs from a continence perspective is important and is a key role of the CNS within the MDT. […] The involvement of clinical psychology at this stage is also important for support and therapy to ensure a smooth transition. […] A clinical nurse specialist has the unique ability to share in this journey from birth to transition and provide an effective interface between the clinical in-hospital environment and the day-to-day life of home and school.

• #65 ERNICA guidelines for the management of rectosigmoid Hirschsprung’s disease | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-01362-3

  Structured follow-up to adulthood, including transition of care is indicated in HSCR. […] The availability of specialist nurses improves communication and access to care, and may reduce hospitalizations/admissions and health care costs. […] Achieving optimal outcomes requires collaboration between medical specialists, nurses and auxiliary resources, including psychologists and sexual therapists, physical and nutritional therapists. […] Planning transition of care should commence sufficiently early, around 13-14 years of age to allow sufficient time for adjustment and should have sufficient flexibility to allow overlap and contact between the existing and future practitioners until patients are satisfactorily established in adult care.

• #66 Hirschsprung Disease: The Role of the Clinical Nurse Specialist

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11120011/

  The CNS can support families through establishing simple routines for potty training and utilising natural physiological phenomena such as the gastrocolic reflex. […] Ensuring that the educational environment is aware of a childs needs from a continence perspective is important and is a key role of the CNS within the MDT. […] The involvement of clinical psychology at this stage is also important for support and therapy to ensure a smooth transition. […] A clinical nurse specialist has the unique ability to share in this journey from birth to transition and provide an effective interface between the clinical in-hospital environment and the day-to-day life of home and school.

• #67 ERNICA guidelines for the management of rectosigmoid Hirschsprung’s disease | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-01362-3

  Structured follow-up to adulthood, including transition of care is indicated in HSCR. […] The availability of specialist nurses improves communication and access to care, and may reduce hospitalizations/admissions and health care costs. […] Achieving optimal outcomes requires collaboration between medical specialists, nurses and auxiliary resources, including psychologists and sexual therapists, physical and nutritional therapists. […] Planning transition of care should commence sufficiently early, around 13-14 years of age to allow sufficient time for adjustment and should have sufficient flexibility to allow overlap and contact between the existing and future practitioners until patients are satisfactorily established in adult care.

• #68 Hirschsprung Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care

  https://emedicine.medscape.com/article/178493-treatment

  If Hirschsprung disease is suspected, neonates and children should be assigned to a center where pediatric specialists are available to make the diagnosis and to provide definitive care. […] Consult with pediatric surgeons and pediatric gastroenterologists. Genetic consultation may be indicated (if a heritable or chromosomal anomaly is suspected). […] It is important that conversations between patients and/or caregivers and clinicians take place regarding transitions in care from pediatric providers to adult providers, and that plans for transitional care be implemented early. […] The general goals of medical care are three-fold: (1) to treat the manifestations and complications of untreated Hirschsprung disease, (2) to institute temporizing measures until definitive reconstructive surgery, and (3) to manage the postoperative bowel function.

• #69 Hirschsprung Disease (for Parents) | Nemours KidsHealth

  https://kidshealth.org/en/parents/hirschsprung.html

  Surgery is thought to be the most effective treatment for Hirschsprung disease. This is done in one step or two, depending on how severe it is. Children who are very sick at the time of surgery (from an inflamed intestine or poor nutrition) may need to undergo surgery in two steps. […] After surgery, kids often get constipated. Laxatives can offer some relief, but check with your doctor about which would be best for your child. […] For children old enough to eat solid foods, a high-fiber diet can ease and prevent constipation. Drinking plenty of water is also important, and helps prevent dehydration. The large intestine helps absorb water from food, so dehydration can be a concern for children who have had part of their intestine removed. […] Kids who still have symptoms or get new ones after surgery (such as explosive and watery diarrhea, fever, a swollen belly, or bleeding from the rectum) should have medical attention right away. These can be signs of enterocolitis, an inflammation of the intestines. […] Most children treated surgically for Hirschsprung disease have an excellent outcome. Most can pass stool normally and have no lasting problems. A few kids might continue to have symptoms, including constipation and bowel control problems.

• #70 Hirschsprung’s Disease: Causes, Symptoms, Diagnosis, Treatment

  https://www.webmd.com/children/what-is-hirschsprungs-disease

  If your child has symptoms such as rectal bleeding, a fever, vomiting, or a swollen belly, take them to the hospital right away. […] About 40% of children who have a pull-through procedure lose control over their bowel movements, which is called fecal incontinence. […] After the pull-through procedure, up to 30% of children get a blockage in their intestines. […] After surgery, most children with Hirschsprung’s disease can poop normally and regain control over their bowels. […] The main treatment is surgery to remove the part of the intestine with the missing nerve cells. After surgery, many children with Hirschsprung’s are able to have normal bowel movements.

• #71 Hirschsprung’s Disease | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/hirschsprung-s-disease

  Hirschsprungs disease is a birth defect of the intestines in which nerves in the wall of the intestine do not form properly. Children with Hirschsprungs disease are unable to defecate or pass stool, which can cause bowel obstruction. It can be life threatening if left untreated. […] Newborns with Hirschsprungs disease will often have abdominal distension (an enlarged abdomen), while older children may suffer from chronic constipation. […] The cause is the absence of those specialized ganglion cells. Parents might observe their baby’s belly’s to become swollen, either very few or no poop at all. […] After a child has a pull-through procedure for Hirschsprungs disease, the first few months there’s very close follow-up. Children who’ve had a pull-through procedure are at risk for some infectious complications called Hirschsprungs-related enterocolitis.

• #72 Hirschsprung Disease in Children – Stanford Medicine Children’s Health

  https://www.stanfordchildrens.org/en/topic/default?id=hirschsprung-disease-in-children-90-P01999

  Hirschsprung disease is treated with surgery called a pull-through procedure. A surgeon removes the part of the large intestine that lacks nerve cells. When possible, the healthy part that is left is connected to the anal opening. […] Treatment will depend on your child’s symptoms, age, and general health. It will also depend on how severe the condition is. […] Your child’s bowel function may be affected after surgery. The most common long-term problems include bowel control and leaking stool, constipation, and infections. […] Problems that may occur after surgery depend on the amount of intestine that lacked nerve cells, and how much intestine was removed. […] Talk with your child’s healthcare provider about your child’s specific situation.

• #73 Treatment Options for Children with Hirschsprung’s Disease | Children’s National Hospital

  https://www.childrensnational.org/get-care/departments/colorectal/provider-resources/treatment-options-for-hirschsprung-disease

  Even though post-surgical problems are fairly common, most children with Hirschsprung’s disease eventually overcome these issues (usually in the first five years of life). Studies show that teenagers and adults with Hirschsprung’s disease have normal rates of sexual function, social satisfaction and a good quality of life despite their past challenges. […] Through our continued research, education and implementation of the latest advanced technology in diagnostic and treatment techniques, pediatricians and primary care physicians will have all of the knowledge and tools necessary to successfully manage and treat children with Hirschsprung’s disease.

• #74 Hirschsprung’s Disease | Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/h/hirschsprung

  Children with Hirschsprungs disease will need rectal irrigations when they are first diagnosed to help prevent enterocolitis. Irrigations will remove trapped stool (poop) and gas. Parents and caregivers will be taught to do rectal irrigations, as they may be needed after surgery to treat enterocolitis. […] After surgery, children are still at risk for enterocolitis. But, with successful surgery and long-term specialized follow-up care, most children can achieve normal bowel habits and an excellent quality of life.

• #75 Hirschsprung Disease in Children | Children’s Mercy Kansas City

  https://www.childrensmercy.org/departments-and-clinics/colorectal-center/hirschsprung-disease/

  While rectal irrigation may provide temporary relief, the primary treatment for Hirschsprung disease is surgery to remove the portion of the colon without any nerve cells. This is called a pull-through procedure, or the Swenson procedure. […] As with any surgical procedure, there are risks to pull-through surgery. These include bleeding, infection, blockage of the bowel, constipation, incontinence and healing issues at the site of the pull-through. […] Constipation is another common problem that many children have after pull-through surgery. Constipation usually can be managed with medicines and laxatives. […] Bowel management is a clinical experience in which we work to empty the patient’s bowels and colon. This experience may be managed in one of three ways: outpatient clinic visits, inpatient stay, or one-week intensive outpatient experience called Bowel Management Bootcamp.

• #76 Hirschsprung Disease: The Role of the Clinical Nurse Specialist

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11120011/

  Hirschsprung disease is a life-long condition that can have a significant impact on both children and their families. This article explores the role of the clinical nurse specialist and the support they can provide from initial diagnosis through the patients surgical journey and right through to transition into adult services. Through the provision of education, training, signposting of social and psychological support, and linking in with community-based services, the clinical nurse specialist can help the child and family to limit that impact of the disease. […] They can provide a vital interface between the family and their needs and requirements while outside of hospital and the in-hospital medical team. They are vital in the support of the family as they deal with the trauma of understanding the new diagnosis and its implications for their child.

• #77 Hirschsprung Disease: The Role of the Clinical Nurse Specialist

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11120011/

  The clinical nurse specialist (CNS) has the role of supporting and reassuring the family. In very practical terms, this can be in supporting the parents learning of how to undertake the process of rectal washout to support adequate decompression and in facilitating their ability to feed the child orally. […] The CNS will discuss with the family the red flags of concern that would necessitate immediate return for review and provide the family with an array of contact details (both telephone and email) to ensure that family has as a safety net. […] The time leading up to surgery can be a period of stress and concern for families, and many will seek reassurance and further guidance from the CNS at this time. […] One of the most important roles of the CNS is in initiating and supporting effective bowel management for children following reconstruction.

• #78 Hirschsprung Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK562142/

  An interprofessional team approach to treatment based on a bio-psychosocial view can significantly reduce the postoperative chronic bowel problems of the majority of children with operated HD. […] Experienced nurses are also crucial members of the group as they will not only monitor the patient’s vital signs and danger signs of possible devastating complications, including HAEC but also assist in educating the patient and family.

• #79 Hirschsprung Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK562142/

  An interprofessional team approach to treatment based on a bio-psychosocial view can significantly reduce the postoperative chronic bowel problems of the majority of children with operated HD. […] Experienced nurses are also crucial members of the group as they will not only monitor the patient’s vital signs and danger signs of possible devastating complications, including HAEC but also assist in educating the patient and family.

• #80 ERNICA guidelines for the management of rectosigmoid Hirschsprung’s disease | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-01362-3

  Structured follow-up to adulthood, including transition of care is indicated in HSCR. […] The availability of specialist nurses improves communication and access to care, and may reduce hospitalizations/admissions and health care costs. […] Achieving optimal outcomes requires collaboration between medical specialists, nurses and auxiliary resources, including psychologists and sexual therapists, physical and nutritional therapists. […] Planning transition of care should commence sufficiently early, around 13-14 years of age to allow sufficient time for adjustment and should have sufficient flexibility to allow overlap and contact between the existing and future practitioners until patients are satisfactorily established in adult care.

• #81 Hirschsprung Disease: The Role of the Clinical Nurse Specialist

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11120011/

  Hirschsprung disease is a life-long condition that can have a significant impact on both children and their families. This article explores the role of the clinical nurse specialist and the support they can provide from initial diagnosis through the patients surgical journey and right through to transition into adult services. Through the provision of education, training, signposting of social and psychological support, and linking in with community-based services, the clinical nurse specialist can help the child and family to limit that impact of the disease. […] They can provide a vital interface between the family and their needs and requirements while outside of hospital and the in-hospital medical team. They are vital in the support of the family as they deal with the trauma of understanding the new diagnosis and its implications for their child.

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