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Ongoing care for the patient with an anorectal malfromation; transitioning to adulthood
Journal Pre-proof
Ongoing care for the patient with an anorectal malfromation; transitioning to adulthood Alessandra Gasior , Paola Midrio , Dalia Aminoff , Michael Stanton PII: DOI: Reference:
S1055-8586(20)30111-6 https://doi.org/10.1016/j.sempedsurg.2020.150991 YSPSU 150991
To appear in:
Seminars in Pediatric Surgery
Please cite this article as: Alessandra Gasior , Paola Midrio , Dalia Aminoff , Michael Stanton , Ongoing care for the patient with an anorectal malfromation; transitioning to adulthood, Seminars in Pediatric Surgery (2020), doi: https://doi.org/10.1016/j.sempedsurg.2020.150991
This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. © 2020 Published by Elsevier Inc.
Ongoing care for the patient with an anorectal malfromation; transitioning to adulthood Authors: Alessandra Gasior1, Paola Midrio2, Dalia Aminoff3, Michael Stanton4
1
Pediatric and adult colorectal surgery
Nationwide Children’s Hospital, The Ohio State University, Columbus, Ohio, USA 2
Pediatric Surgery
Cà Foncello Hospital Treviso, ITALY 3 President Italian Association for Anorectal Malformation AIMAR Via Tripolitania, 211 00199 Rome - Italy 4
Consultant Paediatric Surgeon
Southampton Children's Hospital, Tremona Road, Southampton, UK Corresponding author: [email protected] Abstract: Planned health care transition can improve the ability of young adults to manage their own health care to effecively use health services and ultimately maximize life-long functioning and wellbeing. Transitional care is a purposeful, planned process that addresses the medical, psychosocial and educational needs of adolescents and young adults with chronic physical and medical conditions as they move from child-centered to adult-oriented healthcare systems. Unsuccessful surgical transtion may result in physical and mental health implications for young patients, negative long-term outcomes and suboptimal use of health care resources. Anorectal malformation and Hirschsprung patients are an especially vulnerable patient population with ongoing surgical, physiologic and pyschosocial challenges.
Case 1: A 14-year-old female with a history of an anorectal malformation presents to your clinic for her annual follow-up. She denies any current complaints. She takes senna daily and has good control of her bowel movements. Her abdominal x-ray is clear of stool today. She denies any history of urinary tract infections. She has started her period and is able to insert a tampon without difficulty. The patient’s mother asks you what her medical follow up will look like in 10 years. Will she continue to be seen by you, her pediatric surgeon? a) She no longer needs to be seen by a physician and can be discharged from colorectal care related to her anorectal malformation b) She can continue to be seen by her pediatric surgeon indefinitely c) She will need to be transitioned to an adult colorectal surgeon who specializes in the needs of adults living with anorectal malformations Answer: c Transitional care for pediatric patients with chronic diseases is a topic that has been recognized as a priority in our health care system. However, the practice of transitioning patients, particularly congenital colorectal patients, has not been implemented. Only 22% of adolescents and young adults receive planning for health care transition.1We know that the complexity of the original malformation and associated anomalies will affect the patients fecal and urinary continence throughout adolescents and adulthood.2At present, there is no international consensus for long-term follow-up or transition of care to adulthood. The psychosocial needs of a patient will also need to be addressed by health care providers as well. 3 The process for transitional care should begin around 12-14 years of age.4 A general health knowledge tool should be used to assess the patient’s own understanding of their medical condition including their surgical history, medications, and recent imaging. An annual readiness to transition worksheet should be completed by the caregiver and patient starting at age 12 years old. (Figure 1) The readiness to
transition annual assessments can be used as a guide to determine when the patient is ready to transition, as this may not be at the same age for all patients. Generally, this may occur between the ages of 16 and 21, depending how the patient scores on the readiness assessment. The most common reason that a patient does not transition from a pediatric surgeon is the fear (on the part of the patient and the pediatric surgeon) that the adult surgeon will not understand the treatment and specific needs of the anorectal malformation patient. 5 An appropriate adult surgical provider should be identified early in the process. The patient should transfer to an adult surgical provider when they are at a stable period in their medical care and not during an acute intervention if at all possible. A multi-disciplinary approach should be used for transition. The stakeholders include urology, gynecology, fertility specialist, colorectal surgery, gastroenterology, social work and psychiatry. Additionally, some pediatric nurse practioners or pediatric surgeons may not have credentials to care for adult patients. Case 2: A 32-year-old male presents with a history of having had a pull-through for Hirschsprung disease (HD) when he was a baby. He complains of fecal incontinence. Unfortunately, the patient does not have any medical records from his childhood. He does not recall episodes of enterocolitis during his postsurgical childhood or during adolescence. He denies a history of abdominal distension, fevers, or emesis. He does not take any enemas, or pro- or anti-motility agents. On abdominal examination he has a left lower quadrant transverse scar, presumably from a colostomy site. What studies would you like to obtain? a. Water-soluble contrast enema b. Anorectal manometry c. Rectal biopsy d. Exam under anesthesia
e. All of the above Answer: e Hirschsprung associated enterocolitis (HAEC) occurs in 5% of adult patients and has the potential to be life threatening if unrecognized by adult providers, therefore this should be a consideration in any HD patient presenting with distension or obstructive symptoms, even for adults that are post-surgical repair. 6,7 The majority of HD patients have life-long GI complications with constipation occurring in up to 44% of patients.8,9 Fecal incontinence occurs in up to 76% of patients due to damage to the sphincters during dissection which results in fecal incontinence.10-12 A Hirschsprung’s patient who is not doing well usually falls into one of two categories – soiling or obstruction. Evaluation of the Hirschsprung patient that is not doing well should include: an exam under anesthesia to examine the anatomy which includes evaluating the dentate line, inspect for type of surgery performed which may include an obstructing Soave cuff, Duhamel spur, twist, or stricture.13 Figure 2. Patients with obstructive symptoms will benefit from a full-thickness rectal biopsy to evaluate for a transition zone pull-through or aganglionic pull-through.14 A contrast enema will allow the clinician to infer whether the patient has hypermotility or hypomotility, which will require different treatments. Hypermotile bowel will require antidiarrheal medications to slow transit that may be contributing to fecal incontinence. Hypomotility occurs more commonly with HD patients affected with constipation requiring a bowel regimen with laxatives that may require
periodic adjustments. The contrast enema will also identify a possible retained distal segment as well as a kink or twist of the pull-through.13 Patients with soiling can be assessed with anorectal manometry to further characterize the sphincter pressures. Patients with tight sphincters and absent RAIR may persist into adulthood resembling internal sphincter achalasia with the benefit of botox to help relax the anal sphincters and allow for proper evacuation. (Figure 3) Case 3: A 19-year-old female with a history of an imperforate anus (unknown type) who underwent repair in the first year of life presents to your clinic for evaluation of fecal incontinence. She does not have any records from her previous surgeries. You are able to see a left sided scar that appears to be a colostomy closure site. She has a history of urinary tract infections and urinary retention. She performs urinary catheterization 4-6 times per day. She does not take any medications for a bowel regimen, although she remembers doing enemas when she was younger, but she has stopped now because she did not like them. She also says she would like to have children of her own one day and asks you what her chances of conceiving are. What is your next step? a. Water soluble contrast enema b. Rectal exam either under anesthesia or in the office c. Pelvic exam d. MRI spine and MRI pelvis e. All of the above Answer: e
Learning points: 73% of anorectal malformation patients are followed by a multidisciplinary team from birth.15 Additionally, during adolescence, 89% of these patients develop significant new problems that require a multidisciplinary team including fecal incontinence, chronic constipation, urinary incontinence, ejaculatory dysfunction, erectile dysfunction, psychosocial issues and quality of life. 15 However, 33% of pediatric surgeons discharge patients from their care before 10 years old.15 It is unknown if these patients go to their pediatrician or pediatric gastroenterology for further care. This occurs despite the patient having on-going unaddressed issues and underscores the importance of maintaining a multidisciplinary team for anorectal malformation patients through adulthood, as their needs evolve and change over time. Pelvic exam and rectal exam should be performed to evaluate the surgical site for stricture, prolapse, vaginal introital stenosis, quality of perineal body, and residual vaginal septum. This may have implications on menstrual hygiene, intercourse, obstetric indications as well as effects on the needed bowel regimen. A pelvic MRI may be necessary to determine the presence of Mullerian structures, and particularly if any are obstructed, such as hematometra or hematocolpos. The pelvic anatomy following a reconstruction can affect reproductive potential. Pre-conception counseling should occur with a high-risk obstetrician. Pregnancy is possible in patients with anorectal malformations and cloacal malformations and the mode of delivery must be individually addressed with a risk vs benefit analysis especially for increased risk of fecal and urinary incontinence with vaginal delivery.16 Other factors to take into account when determining mode of delivery include perineal body length and stability, presence of native vagina or short common channel cloaca. Labor and vaginal deliveries can lead to unpredictable perineal tears that may impair the patients already compromised fecal continence, therefore cesarean sections may be indicated. Additionally, patients with a
complicated abdomen with Malone, mitrofanoff or bladder augmentation must have a planned multidisciplinary surgical approach to the decisions regarding delivery. Case 4:
A 22-year-old male born with a recto-prostatic fistula, esophageal atresia type 3, and urethral stenosis due to PSARP, underwent a gastric pull-up for a persistent esophageal stricture and a two-step urethral reconstruction during adolescence (Bracka technique). He is currently on successful bowel management with transanal irrigation (TAI) and his main complaint is severe pain after masturbation and a subsequent urge to empty his bladder with a “burning feeling in the bladder” for the following 45 minutes. He visited an adult andrologist, with unsatisfactory results, who offered him sperm cryopreservation. The patient then called the Patients’ organization to ask for further help.
What is your next step? A
Refer him to an adult urologist
B
Refer him to his pediatric urologist
C
Suggest instrumental imaging in order to re-assess the urinary anatomy
D
Send him to an expert center for ARM where a multidisciplinary approach is used
Answer D: the patients’ organization sent the patient to his pediatric urologist who is working at a center of excellence. The surgeon puts patient on pain medication. A rectourethral prostatic fistula is defined as a defect in which the rectum is abnormally communicating with the middle portion of the posterior urethra known as the prostatic urethra. A rectoprostatic fistula represents a defect considered relatively complex, anatomically residing between a rectourethral bulbar
fistula and a recto-bladderneck fistula. It is important to differentiate patients affected by bulbar, prostatic or, rare, bladder-neck fistula as they have different therapeutic implications and functional prognosis. In addition, the frequency of associated malformations is significantly different among groups. [Molly’s new reference here] WHAT IS IT? Prostatic fistulas have a higher frequency of associated urological anomalies than recto-bulbar cases. Vesicoureteral reflux occurs in 26% of cases, absent kidney 10%, undescended testicles 8%, hypospadias 7.3%, and hydronephrosis 6%. It is important that such a patient has a proper follow up. From the urological point of view, the pediatric and adult urologist should be part of a multidisciplinary team together with an andrologist. This kind of complex patient needs a life-long follow up with appropriate transition of care. Namely, this patient would need assessment of his urinary tract by means of a VCUG to show the anatomy of the bladder and reconstructed urethra, in particular if a urethral diverticulum or stenosis are present. The urethral diverticulum, if present, develops at the site of the original recto-prostatic fistula that was ligated too proximal leaving behind a tract of fistula that filled with urine over time. The diverticulum can be removed thru an ASTRA (anterior sagittal trans-rectal approach). The severity of urethral stenosis should be assessed by means of an urofluxometry and VCUG and, if necessary, surgically treated.
Case 5: A 46-year-old-female with a history of an imperforate anus (unknown type), and having undergone a “redo for anal stenosis” and acro-oculo-renal/Okhiro syndrome (bilateral thumbs hypoplasia, StillingDuane anomaly, mitral valve prolapse) presents to your clinic for evaluation of asymmetrical mucosal prolapse, difficult evacuation of stool, and grade 1 bladder prolapse. She does not have any records
from her previous surgeries. You are able to see a left sided lower abdominal scar, but the patient cannot remember if it was the place of a previous colostomy. The patient is married, has one adopted child, has been in menopause for the past year, and is an active sports enthusiast. She feels that the prolapse impedes her sporting activity and evacuation of stool. Her anus looks widely patent (such as a “perineal colostomy”), without any muscle tone, and a voluminous mucosal prolapse obstructing the lumen is visible. What is your next step? a) Refer her to a proctologist b) Suggest starting with regular bowel management c) Refer her to a pelvic physiotherapist d) b+c Answer: d). The patient began a bowel management program, under the supervision of a stoma therapist, and pelvic training. Her prolapse improved as she was able to evacuate rapidly and completely, and she returned to her normal activity. The possibility of a rectal-pexy, performed laparoscopically together with the colorectal surgeon, was also anticipated. After 6 months, because of the prolapse’s relapse she underwent the Pelvic Organ Prolapse Suspension (POPS) performed by an adult coloproctologist she visited on her own. Ever since, she continues to complain of mucosal prolapse and painful insertion of the rectal probe to perform the transanal irrigation. Adults or, even, elderly people only rarely experience with complications related to their ARM reconstruction. When they do, even expert adult surgeons, may not know what an ARM is and therefore are unprepared to manage the surgical implications. Colorectal surgeons tend to propose solutions that are inadequate for the ARM population, such as the gracilis muscle sling or POPS with unpredictable consequences. These options are inadequate for ARM patient as their anatomy is different from the population with incontinence for which these types of interventions were created. In particular the anal canal of ARM patients maybe incompletely surrounded by muscle and the extensive previous surgery may impede to proceed with the standard techniques. The pelvic floor of an ARM patient, especially if she is a woman with previous deliveries, tend to weaken over time, specifically when she undergoes menopause. Vaginal delivery should be discouraged in any type of ARM as well as the standard uro-gynecological surgery for incontinence or prolapse. In ARM
patients, indeed, surgical procedures such as the retropubic suspension, midurethral synthetic slings, sacral neuromodulators, and posterior tibial nerve modulation may be ineffective, due to the congenital altered anatomy, or difficult to be performed due to scarring of previous ano-uro-genital surgery. The pediatric surgeon should offer her/his competency to interact with adult surgeons, including gynecologists at the time of C-section, in order to avoid mistakes (pregnant woman with Mitrofanoff, Csection on patients with a previous cloaca or, very rarely, cloacal exstrophy).
Case 6: You see an 18-year-old boy with a previous “high ano-rectal malformation” in clinic. He required a re-do surgery in infancy. He has a long-standing stoma due to fecal incontinence. He reports no concerns with urological or sexual function but does not want to discuss this in detail in front of his mother. He describes that he is happy having a stoma, but his mother is concerned that in the future he may want to explore whether he could live his life without one. He does not live near the tertiary center where he has been treated so far. What should be done to manage this patient? a) Transition to multidisciplinary adult colorectal and urology surgeons b) Referral to a large pediatric colorectal center c) Refer to an adult general surgeon d) Transfer care to an adult primary care physician Answer: a
Transition to adult services for a young person with a lifelong condition is best started at age 11-12 years. At this age, children will be moving to different schools and starting adolescence. It is important that they begin to feel empowered to start to manage their own condition. It would have been
preferable to have utilized a structured program for this patient, but this is a relatively new innovation and will not have been undertaken in every patient. It is importance to discuss with the patient his/her educational and/or vocational plans for the next few years. In the UK, transition to adult services is usually completed around the age of 18 years and patients start to be seen in adult clinics from this time. At this age, patients leave full-time education and may either start employment or continue studying, for example, at university. It is important to know where they will be living and where it will be best for them to be seen in the future. It is less likely that a patient with Hirschsprung disease or a previous anorectal malformation should be discharged to the care of their general practitioner. In some patients who have been asymptomatic for several years this may be appropriate, for example, those with an isolated previous low anorectal malformation with no sequelae in bowel or urinary function. Transition is not simply the transfer of a patient to an adult clinic. It is a gradual process of empowerment of a young person. This process should not only address the medical/surgical needs of the patient, but also consider their psychosocial needs and their educational/vocational plans. Although it is ideal that a joint clinic with an adult surgeon is undertaken, this is not essential, and may indeed not be practical depending on where the patient lives. In this example, it is important to identify an appropriate adult colorectal surgeon and/or urologist with an interest in pelvic floor function. It is essential to discuss with adult colleagues in advance, or there is a risk of non-attendance. It is also important to ensure that transition takes place to a center in the appropriate geographical location, which may not necessarily be the tertiary pediatric center where they have been treated so far. Although this patient may not be reporting any current symptoms or problems, these may well occur in the future. Adolescents may find it very difficult to discuss urological and/or sexual problems, in particular if their parents are present.
Average life expectancy in the UK is approximately 80 years and so it is important that appropriate long-term plans are in place. There is considerable evidence from other pediatric subspecialties that if suitable transition takes place for young persons with chronic conditions, then morbidity and mortality can be reduced. Complications such as ventriculo-peritoneal (VP) shunt failure and renal transplant failure can be avoided18,19. Rather than an increased risk of mortality, patients with Hirschsprung disease or anorectal malformations certainly experience morbidity, with constipation and soiling occurring at a higher rate than adult controls. There is also an increased risk of being diagnosed with a depressive disorder in adulthood20. In the UK, formal transition to adult services occurs in the majority (approximately 2/3) of centers treating children with inflammatory bowel disease. Following a recent survey study, it was found that only a minority of tertiary centers have a formal transition service for teenagers with Hirschsprung disease or anorectal malformations. The ‘Ready, Steady, Go’ and ‘Hello to adult services’ is an example of a generic transition program for young persons (www.uhs.nhs.uk/readysteadygo), which can be applied to any pediatric subspecialty21. Studies regarding successful transition programs often report qualitative data, and as such it is difficult to directly colorectal outcomes against these. However, it is clear that a lot of the difficulties faced by young people with a long-term condition are generic. For example, difficulties regarding sexual health, pregnancy and vocational possibilities are common in adolescents who have been treated for anorectal malformations and Hirschsprung disease 21,22.
References: 1. Chu PY, Maslow GR, von Isenburg M et al. Systematic Review of the impact of transition interventions for adolescents with chronic illness on transfer from pediatric to adult healthcare. J Pediatr Nurse. 2015;30:219-e27.
2. Cairo SB, Gasior A, Rollins MD, Rothstein DH, Delivery of Surgical Care Committee of the American Academy of Pediatrics Section on Surgery. Challenges in Transition of Care for Patients With Anorectal Malformations: A Systematic Review and Recommendations for Comprehensive Care. Dis Colon Rectum. 2018 Mar;61(3):390-399. 3. Schwartz LA, Brumley LD, Tuchman LK et al. Stakeholder validation of a model of readiness for transition to adult care. JAMA Pediatr 167 (10) 2013. 939-946. 4. Giuliani S, Grano C, Aminoff D et al. Transition of care in patients with anorectal malformations: consensus by the ARM-net consortium. J Pediatr Surg. 2017 Nov;52(11) 1866-1872. 5. Cairo SB, Chiu PP, Dasgupta R et al. Transitions in care from pediatric to adult general surgery: Evaluating an unmet need for patients with anorectal malformation and Hirschsprung disease. J Pediatr Surg. 2018 Aug;53(8):1566-1572. 6. Vorobyov et al. Clinical features diagnostics and treatment of Hirschsprung’s disease in adults. Colorectal Dis. 2010 Dec;12(12):1242-8 7. Sharma et al. Hirschsprung’s disease presenting beyond infancy: surgical options and postoperative outcome. Pediatr Surg Int. 2012 Jan; 28(1):5-8 8. Doodnath et al. A systematic review and meta-analysis of Hirschsprung’s disease presenting after childhood. Pediatr Surg Int. 2010 Nov; 26(11):1107-10 9. Hackam et al. Diagnosis and outcome of Hirshsprung’s disease: does age really matter? Pediatr Surg Int. 2004 May; 20(5):319-22. 10. Zaslavsky C, Loening-Baucke V. Anorectal manometric evaluation of children and adolescents postsurgery for Hirschsprung's disease J Pediatr Surg, 38 (2003), pp. 191-195 11. Yanchar NL, Soucy P. Long-term outcome after Hirschsprung's disease: patients' perspectives. J Pediatr Surg, 34 (1999), pp. 1152-1160
12. Scott AE, Jay JG. Long-term results of treatment of Hirschsprung's disease. Semin Pediatr Surg, 13 (2004), pp. 273-285 13. Levitt MA, Dickie B, Pena A. Evaluation and treatment of the patient with Hirschsprung disease who is not doing well after a pull-through procedure.Semin Pediatr Surg. 2010 May;19(2):146-53. doi: 10.1053/j.sempedsurg.2009.11.013. 14. Peña A, Elicevik M, Levitt MA. Reoperations in Hirschsprung's disease. J Pediatr Surg, 42 (2007), pp. 1008-1014 15. Giuliani S, Decker E, Leva E et al. Long term follow-up and transition of care in anorectal malformations: an international survey. J Pediatr Surg. 2016 Sep;51(9):1450-7. 16. Breech L. Sem Pediatr Surg. 2016 Apr;25(2):90-5. Gynecologic concerns in patients with cloacal anomaly. 17. Peña A, Bischoff A. – Surgical Treatment of Colorectal Problems in Children. 18. Complications with shunts in adults with spina bifida. P. Tomlinson, I. D. Sugarman. BMJ. 1995 Jul 29; 311(7000): 286–287 19. Weitz M, Heeringa S, Neuhaus TJ, et al. Standardized multilevel transition program: Does it affect renal transplant outcome? Pediatr Transplant. 2015;19(7):691–97. 20. Swedish national population-based study shows an increased risk of depression among patients with Hirschsprung disease. Amin L, Skoglund C, Wester T, Granström AL. Acta Paediatr. 2019 Oct;108(10):1867-1870. 21. Implementing transition: Ready Steady Go. Nagra A, McGinnity PM, Davis N, Salmon AP. Arch Dis ChildEduc Pract Ed. 2015 Dec;100(6):313-20. 22. Ready, Steady, Go - Achieving successful transition in cystic fibrosis. Connett GJ, Nagra A.Paediatr Respir Rev. 2018 Jun;27:13-15.
Figure 1: Patient Transition readiness assessment Date: ___________________ Name:__________________________Date of birth:_____________________ Transition Importance and Confidence: Please circle your response on a scale of 0-10 (0=not confident at all, 10=completely confident)
How important is it for you to transfer to adult doctors before age 22? 0
1
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How confident do you feel about your ability to transfer to an adult doctor? 0
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My Health: Please circle the box that applies to you today
I know my medical needs. Yes I know this
I need to work on this
Who can help with this?
I can explain my medical needs to others. Yes I know this
I need to work on this
Who can help with this?
I know my symptoms including those that require me to see a doctor quickly. Yes I know this
I need to work on this
I know what to do in case of an emergency.
Who can help with this?
Yes I know this
I need to work on this
Who can help with this?
I know my medicines, why I take them, when to take them. Yes I know this
I need to work on this
Who can help with this?
I know my allergies and what medicines I should not take. Yes I know this
I need to work on this
Who can help with this?
I have and carry important medical information daily (health insurance, emergency contact, allergies, medications, health summary). Yes I know this
I need to work on this
Who can help with this?
I know how consent and health care privacy change when I am 18 (legal adult). Yes I know this
I need to work on this
Who can help with this?
I can explain my customs and beliefs to others and how they affect my health care treatment and decisions. Yes I know this
I need to work on this
Who can help with this?
USING HEALTH CARE I can find my doctor’s number. Yes I know this
I need to work on this
Who can help with this?
I can make my own doctor appointments. Yes I know this
I need to work on this
Who can help with this?
Before my visit, I think of questions to ask. Yes I know this
I need to work on this
Who can help with this?
I have transportation to my doctor’s office. Yes I know this
I need to work on this
Who can help with this?
I know to show up 15 minutes before an appointment. Yes I know this
I need to work on this
Who can help with this?
I know where to get medical care when my doctor’s office is closed. Yes I know this
I need to work on this
Who can help with this?
I have a file at home with my medical information. Yes I know this
I need to work on this
Who can help with this?
I have a copy of my current plan of care. Yes I know this
I need to work on this
Who can help with this?
I need to work on this
Who can help with this?
I can fill out medical forms. Yes I know this
I know how to get referrals to other providers. Yes I know this
I need to work on this
Who can help with this?
I have a pharmacy and know how to refill my medications. Yes I know this
I need to work on this
Who can help with this?
I know where to obtain tests (x-rays/labs) if my doctor orders them. Yes I know this
I need to work on this
Who can help with this?
I have a plan to keep health insurance after I turn 18 or older. Yes I know this
I need to work on this
Who can help with this?
My family and I have discussed my ability to make my own health care decisions at age 18. Yes I know this
I need to work on this
Figure 2: Types of surgical pull-throughs in Hirschsprung disease
Who can help with this?
Figure 3:
Non-relaxing sphincters
Botox injection
Redo-pull-through
Ongoing care for the patient with an anorectal malfromation; transitioning to adulthood Alessandra Gasior , Paola Midrio , Dalia Aminoff , Michael Stanton PII: DOI: Reference:
S1055-8586(20)30111-6 https://doi.org/10.1016/j.sempedsurg.2020.150991 YSPSU 150991
To appear in:
Seminars in Pediatric Surgery
Please cite this article as: Alessandra Gasior , Paola Midrio , Dalia Aminoff , Michael Stanton , Ongoing care for the patient with an anorectal malfromation; transitioning to adulthood, Seminars in Pediatric Surgery (2020), doi: https://doi.org/10.1016/j.sempedsurg.2020.150991
This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. © 2020 Published by Elsevier Inc.
Ongoing care for the patient with an anorectal malfromation; transitioning to adulthood Authors: Alessandra Gasior1, Paola Midrio2, Dalia Aminoff3, Michael Stanton4
1
Pediatric and adult colorectal surgery
Nationwide Children’s Hospital, The Ohio State University, Columbus, Ohio, USA 2
Pediatric Surgery
Cà Foncello Hospital Treviso, ITALY 3 President Italian Association for Anorectal Malformation AIMAR Via Tripolitania, 211 00199 Rome - Italy 4
Consultant Paediatric Surgeon
Southampton Children's Hospital, Tremona Road, Southampton, UK Corresponding author: [email protected] Abstract: Planned health care transition can improve the ability of young adults to manage their own health care to effecively use health services and ultimately maximize life-long functioning and wellbeing. Transitional care is a purposeful, planned process that addresses the medical, psychosocial and educational needs of adolescents and young adults with chronic physical and medical conditions as they move from child-centered to adult-oriented healthcare systems. Unsuccessful surgical transtion may result in physical and mental health implications for young patients, negative long-term outcomes and suboptimal use of health care resources. Anorectal malformation and Hirschsprung patients are an especially vulnerable patient population with ongoing surgical, physiologic and pyschosocial challenges.
Case 1: A 14-year-old female with a history of an anorectal malformation presents to your clinic for her annual follow-up. She denies any current complaints. She takes senna daily and has good control of her bowel movements. Her abdominal x-ray is clear of stool today. She denies any history of urinary tract infections. She has started her period and is able to insert a tampon without difficulty. The patient’s mother asks you what her medical follow up will look like in 10 years. Will she continue to be seen by you, her pediatric surgeon? a) She no longer needs to be seen by a physician and can be discharged from colorectal care related to her anorectal malformation b) She can continue to be seen by her pediatric surgeon indefinitely c) She will need to be transitioned to an adult colorectal surgeon who specializes in the needs of adults living with anorectal malformations Answer: c Transitional care for pediatric patients with chronic diseases is a topic that has been recognized as a priority in our health care system. However, the practice of transitioning patients, particularly congenital colorectal patients, has not been implemented. Only 22% of adolescents and young adults receive planning for health care transition.1We know that the complexity of the original malformation and associated anomalies will affect the patients fecal and urinary continence throughout adolescents and adulthood.2At present, there is no international consensus for long-term follow-up or transition of care to adulthood. The psychosocial needs of a patient will also need to be addressed by health care providers as well. 3 The process for transitional care should begin around 12-14 years of age.4 A general health knowledge tool should be used to assess the patient’s own understanding of their medical condition including their surgical history, medications, and recent imaging. An annual readiness to transition worksheet should be completed by the caregiver and patient starting at age 12 years old. (Figure 1) The readiness to
transition annual assessments can be used as a guide to determine when the patient is ready to transition, as this may not be at the same age for all patients. Generally, this may occur between the ages of 16 and 21, depending how the patient scores on the readiness assessment. The most common reason that a patient does not transition from a pediatric surgeon is the fear (on the part of the patient and the pediatric surgeon) that the adult surgeon will not understand the treatment and specific needs of the anorectal malformation patient. 5 An appropriate adult surgical provider should be identified early in the process. The patient should transfer to an adult surgical provider when they are at a stable period in their medical care and not during an acute intervention if at all possible. A multi-disciplinary approach should be used for transition. The stakeholders include urology, gynecology, fertility specialist, colorectal surgery, gastroenterology, social work and psychiatry. Additionally, some pediatric nurse practioners or pediatric surgeons may not have credentials to care for adult patients. Case 2: A 32-year-old male presents with a history of having had a pull-through for Hirschsprung disease (HD) when he was a baby. He complains of fecal incontinence. Unfortunately, the patient does not have any medical records from his childhood. He does not recall episodes of enterocolitis during his postsurgical childhood or during adolescence. He denies a history of abdominal distension, fevers, or emesis. He does not take any enemas, or pro- or anti-motility agents. On abdominal examination he has a left lower quadrant transverse scar, presumably from a colostomy site. What studies would you like to obtain? a. Water-soluble contrast enema b. Anorectal manometry c. Rectal biopsy d. Exam under anesthesia
e. All of the above Answer: e Hirschsprung associated enterocolitis (HAEC) occurs in 5% of adult patients and has the potential to be life threatening if unrecognized by adult providers, therefore this should be a consideration in any HD patient presenting with distension or obstructive symptoms, even for adults that are post-surgical repair. 6,7 The majority of HD patients have life-long GI complications with constipation occurring in up to 44% of patients.8,9 Fecal incontinence occurs in up to 76% of patients due to damage to the sphincters during dissection which results in fecal incontinence.10-12 A Hirschsprung’s patient who is not doing well usually falls into one of two categories – soiling or obstruction. Evaluation of the Hirschsprung patient that is not doing well should include: an exam under anesthesia to examine the anatomy which includes evaluating the dentate line, inspect for type of surgery performed which may include an obstructing Soave cuff, Duhamel spur, twist, or stricture.13 Figure 2. Patients with obstructive symptoms will benefit from a full-thickness rectal biopsy to evaluate for a transition zone pull-through or aganglionic pull-through.14 A contrast enema will allow the clinician to infer whether the patient has hypermotility or hypomotility, which will require different treatments. Hypermotile bowel will require antidiarrheal medications to slow transit that may be contributing to fecal incontinence. Hypomotility occurs more commonly with HD patients affected with constipation requiring a bowel regimen with laxatives that may require
periodic adjustments. The contrast enema will also identify a possible retained distal segment as well as a kink or twist of the pull-through.13 Patients with soiling can be assessed with anorectal manometry to further characterize the sphincter pressures. Patients with tight sphincters and absent RAIR may persist into adulthood resembling internal sphincter achalasia with the benefit of botox to help relax the anal sphincters and allow for proper evacuation. (Figure 3) Case 3: A 19-year-old female with a history of an imperforate anus (unknown type) who underwent repair in the first year of life presents to your clinic for evaluation of fecal incontinence. She does not have any records from her previous surgeries. You are able to see a left sided scar that appears to be a colostomy closure site. She has a history of urinary tract infections and urinary retention. She performs urinary catheterization 4-6 times per day. She does not take any medications for a bowel regimen, although she remembers doing enemas when she was younger, but she has stopped now because she did not like them. She also says she would like to have children of her own one day and asks you what her chances of conceiving are. What is your next step? a. Water soluble contrast enema b. Rectal exam either under anesthesia or in the office c. Pelvic exam d. MRI spine and MRI pelvis e. All of the above Answer: e
Learning points: 73% of anorectal malformation patients are followed by a multidisciplinary team from birth.15 Additionally, during adolescence, 89% of these patients develop significant new problems that require a multidisciplinary team including fecal incontinence, chronic constipation, urinary incontinence, ejaculatory dysfunction, erectile dysfunction, psychosocial issues and quality of life. 15 However, 33% of pediatric surgeons discharge patients from their care before 10 years old.15 It is unknown if these patients go to their pediatrician or pediatric gastroenterology for further care. This occurs despite the patient having on-going unaddressed issues and underscores the importance of maintaining a multidisciplinary team for anorectal malformation patients through adulthood, as their needs evolve and change over time. Pelvic exam and rectal exam should be performed to evaluate the surgical site for stricture, prolapse, vaginal introital stenosis, quality of perineal body, and residual vaginal septum. This may have implications on menstrual hygiene, intercourse, obstetric indications as well as effects on the needed bowel regimen. A pelvic MRI may be necessary to determine the presence of Mullerian structures, and particularly if any are obstructed, such as hematometra or hematocolpos. The pelvic anatomy following a reconstruction can affect reproductive potential. Pre-conception counseling should occur with a high-risk obstetrician. Pregnancy is possible in patients with anorectal malformations and cloacal malformations and the mode of delivery must be individually addressed with a risk vs benefit analysis especially for increased risk of fecal and urinary incontinence with vaginal delivery.16 Other factors to take into account when determining mode of delivery include perineal body length and stability, presence of native vagina or short common channel cloaca. Labor and vaginal deliveries can lead to unpredictable perineal tears that may impair the patients already compromised fecal continence, therefore cesarean sections may be indicated. Additionally, patients with a
complicated abdomen with Malone, mitrofanoff or bladder augmentation must have a planned multidisciplinary surgical approach to the decisions regarding delivery. Case 4:
A 22-year-old male born with a recto-prostatic fistula, esophageal atresia type 3, and urethral stenosis due to PSARP, underwent a gastric pull-up for a persistent esophageal stricture and a two-step urethral reconstruction during adolescence (Bracka technique). He is currently on successful bowel management with transanal irrigation (TAI) and his main complaint is severe pain after masturbation and a subsequent urge to empty his bladder with a “burning feeling in the bladder” for the following 45 minutes. He visited an adult andrologist, with unsatisfactory results, who offered him sperm cryopreservation. The patient then called the Patients’ organization to ask for further help.
What is your next step? A
Refer him to an adult urologist
B
Refer him to his pediatric urologist
C
Suggest instrumental imaging in order to re-assess the urinary anatomy
D
Send him to an expert center for ARM where a multidisciplinary approach is used
Answer D: the patients’ organization sent the patient to his pediatric urologist who is working at a center of excellence. The surgeon puts patient on pain medication. A rectourethral prostatic fistula is defined as a defect in which the rectum is abnormally communicating with the middle portion of the posterior urethra known as the prostatic urethra. A rectoprostatic fistula represents a defect considered relatively complex, anatomically residing between a rectourethral bulbar
fistula and a recto-bladderneck fistula. It is important to differentiate patients affected by bulbar, prostatic or, rare, bladder-neck fistula as they have different therapeutic implications and functional prognosis. In addition, the frequency of associated malformations is significantly different among groups. [Molly’s new reference here] WHAT IS IT? Prostatic fistulas have a higher frequency of associated urological anomalies than recto-bulbar cases. Vesicoureteral reflux occurs in 26% of cases, absent kidney 10%, undescended testicles 8%, hypospadias 7.3%, and hydronephrosis 6%. It is important that such a patient has a proper follow up. From the urological point of view, the pediatric and adult urologist should be part of a multidisciplinary team together with an andrologist. This kind of complex patient needs a life-long follow up with appropriate transition of care. Namely, this patient would need assessment of his urinary tract by means of a VCUG to show the anatomy of the bladder and reconstructed urethra, in particular if a urethral diverticulum or stenosis are present. The urethral diverticulum, if present, develops at the site of the original recto-prostatic fistula that was ligated too proximal leaving behind a tract of fistula that filled with urine over time. The diverticulum can be removed thru an ASTRA (anterior sagittal trans-rectal approach). The severity of urethral stenosis should be assessed by means of an urofluxometry and VCUG and, if necessary, surgically treated.
Case 5: A 46-year-old-female with a history of an imperforate anus (unknown type), and having undergone a “redo for anal stenosis” and acro-oculo-renal/Okhiro syndrome (bilateral thumbs hypoplasia, StillingDuane anomaly, mitral valve prolapse) presents to your clinic for evaluation of asymmetrical mucosal prolapse, difficult evacuation of stool, and grade 1 bladder prolapse. She does not have any records
from her previous surgeries. You are able to see a left sided lower abdominal scar, but the patient cannot remember if it was the place of a previous colostomy. The patient is married, has one adopted child, has been in menopause for the past year, and is an active sports enthusiast. She feels that the prolapse impedes her sporting activity and evacuation of stool. Her anus looks widely patent (such as a “perineal colostomy”), without any muscle tone, and a voluminous mucosal prolapse obstructing the lumen is visible. What is your next step? a) Refer her to a proctologist b) Suggest starting with regular bowel management c) Refer her to a pelvic physiotherapist d) b+c Answer: d). The patient began a bowel management program, under the supervision of a stoma therapist, and pelvic training. Her prolapse improved as she was able to evacuate rapidly and completely, and she returned to her normal activity. The possibility of a rectal-pexy, performed laparoscopically together with the colorectal surgeon, was also anticipated. After 6 months, because of the prolapse’s relapse she underwent the Pelvic Organ Prolapse Suspension (POPS) performed by an adult coloproctologist she visited on her own. Ever since, she continues to complain of mucosal prolapse and painful insertion of the rectal probe to perform the transanal irrigation. Adults or, even, elderly people only rarely experience with complications related to their ARM reconstruction. When they do, even expert adult surgeons, may not know what an ARM is and therefore are unprepared to manage the surgical implications. Colorectal surgeons tend to propose solutions that are inadequate for the ARM population, such as the gracilis muscle sling or POPS with unpredictable consequences. These options are inadequate for ARM patient as their anatomy is different from the population with incontinence for which these types of interventions were created. In particular the anal canal of ARM patients maybe incompletely surrounded by muscle and the extensive previous surgery may impede to proceed with the standard techniques. The pelvic floor of an ARM patient, especially if she is a woman with previous deliveries, tend to weaken over time, specifically when she undergoes menopause. Vaginal delivery should be discouraged in any type of ARM as well as the standard uro-gynecological surgery for incontinence or prolapse. In ARM
patients, indeed, surgical procedures such as the retropubic suspension, midurethral synthetic slings, sacral neuromodulators, and posterior tibial nerve modulation may be ineffective, due to the congenital altered anatomy, or difficult to be performed due to scarring of previous ano-uro-genital surgery. The pediatric surgeon should offer her/his competency to interact with adult surgeons, including gynecologists at the time of C-section, in order to avoid mistakes (pregnant woman with Mitrofanoff, Csection on patients with a previous cloaca or, very rarely, cloacal exstrophy).
Case 6: You see an 18-year-old boy with a previous “high ano-rectal malformation” in clinic. He required a re-do surgery in infancy. He has a long-standing stoma due to fecal incontinence. He reports no concerns with urological or sexual function but does not want to discuss this in detail in front of his mother. He describes that he is happy having a stoma, but his mother is concerned that in the future he may want to explore whether he could live his life without one. He does not live near the tertiary center where he has been treated so far. What should be done to manage this patient? a) Transition to multidisciplinary adult colorectal and urology surgeons b) Referral to a large pediatric colorectal center c) Refer to an adult general surgeon d) Transfer care to an adult primary care physician Answer: a
Transition to adult services for a young person with a lifelong condition is best started at age 11-12 years. At this age, children will be moving to different schools and starting adolescence. It is important that they begin to feel empowered to start to manage their own condition. It would have been
preferable to have utilized a structured program for this patient, but this is a relatively new innovation and will not have been undertaken in every patient. It is importance to discuss with the patient his/her educational and/or vocational plans for the next few years. In the UK, transition to adult services is usually completed around the age of 18 years and patients start to be seen in adult clinics from this time. At this age, patients leave full-time education and may either start employment or continue studying, for example, at university. It is important to know where they will be living and where it will be best for them to be seen in the future. It is less likely that a patient with Hirschsprung disease or a previous anorectal malformation should be discharged to the care of their general practitioner. In some patients who have been asymptomatic for several years this may be appropriate, for example, those with an isolated previous low anorectal malformation with no sequelae in bowel or urinary function. Transition is not simply the transfer of a patient to an adult clinic. It is a gradual process of empowerment of a young person. This process should not only address the medical/surgical needs of the patient, but also consider their psychosocial needs and their educational/vocational plans. Although it is ideal that a joint clinic with an adult surgeon is undertaken, this is not essential, and may indeed not be practical depending on where the patient lives. In this example, it is important to identify an appropriate adult colorectal surgeon and/or urologist with an interest in pelvic floor function. It is essential to discuss with adult colleagues in advance, or there is a risk of non-attendance. It is also important to ensure that transition takes place to a center in the appropriate geographical location, which may not necessarily be the tertiary pediatric center where they have been treated so far. Although this patient may not be reporting any current symptoms or problems, these may well occur in the future. Adolescents may find it very difficult to discuss urological and/or sexual problems, in particular if their parents are present.
Average life expectancy in the UK is approximately 80 years and so it is important that appropriate long-term plans are in place. There is considerable evidence from other pediatric subspecialties that if suitable transition takes place for young persons with chronic conditions, then morbidity and mortality can be reduced. Complications such as ventriculo-peritoneal (VP) shunt failure and renal transplant failure can be avoided18,19. Rather than an increased risk of mortality, patients with Hirschsprung disease or anorectal malformations certainly experience morbidity, with constipation and soiling occurring at a higher rate than adult controls. There is also an increased risk of being diagnosed with a depressive disorder in adulthood20. In the UK, formal transition to adult services occurs in the majority (approximately 2/3) of centers treating children with inflammatory bowel disease. Following a recent survey study, it was found that only a minority of tertiary centers have a formal transition service for teenagers with Hirschsprung disease or anorectal malformations. The ‘Ready, Steady, Go’ and ‘Hello to adult services’ is an example of a generic transition program for young persons (www.uhs.nhs.uk/readysteadygo), which can be applied to any pediatric subspecialty21. Studies regarding successful transition programs often report qualitative data, and as such it is difficult to directly colorectal outcomes against these. However, it is clear that a lot of the difficulties faced by young people with a long-term condition are generic. For example, difficulties regarding sexual health, pregnancy and vocational possibilities are common in adolescents who have been treated for anorectal malformations and Hirschsprung disease 21,22.
References: 1. Chu PY, Maslow GR, von Isenburg M et al. Systematic Review of the impact of transition interventions for adolescents with chronic illness on transfer from pediatric to adult healthcare. J Pediatr Nurse. 2015;30:219-e27.
2. Cairo SB, Gasior A, Rollins MD, Rothstein DH, Delivery of Surgical Care Committee of the American Academy of Pediatrics Section on Surgery. Challenges in Transition of Care for Patients With Anorectal Malformations: A Systematic Review and Recommendations for Comprehensive Care. Dis Colon Rectum. 2018 Mar;61(3):390-399. 3. Schwartz LA, Brumley LD, Tuchman LK et al. Stakeholder validation of a model of readiness for transition to adult care. JAMA Pediatr 167 (10) 2013. 939-946. 4. Giuliani S, Grano C, Aminoff D et al. Transition of care in patients with anorectal malformations: consensus by the ARM-net consortium. J Pediatr Surg. 2017 Nov;52(11) 1866-1872. 5. Cairo SB, Chiu PP, Dasgupta R et al. Transitions in care from pediatric to adult general surgery: Evaluating an unmet need for patients with anorectal malformation and Hirschsprung disease. J Pediatr Surg. 2018 Aug;53(8):1566-1572. 6. Vorobyov et al. Clinical features diagnostics and treatment of Hirschsprung’s disease in adults. Colorectal Dis. 2010 Dec;12(12):1242-8 7. Sharma et al. Hirschsprung’s disease presenting beyond infancy: surgical options and postoperative outcome. Pediatr Surg Int. 2012 Jan; 28(1):5-8 8. Doodnath et al. A systematic review and meta-analysis of Hirschsprung’s disease presenting after childhood. Pediatr Surg Int. 2010 Nov; 26(11):1107-10 9. Hackam et al. Diagnosis and outcome of Hirshsprung’s disease: does age really matter? Pediatr Surg Int. 2004 May; 20(5):319-22. 10. Zaslavsky C, Loening-Baucke V. Anorectal manometric evaluation of children and adolescents postsurgery for Hirschsprung's disease J Pediatr Surg, 38 (2003), pp. 191-195 11. Yanchar NL, Soucy P. Long-term outcome after Hirschsprung's disease: patients' perspectives. J Pediatr Surg, 34 (1999), pp. 1152-1160
12. Scott AE, Jay JG. Long-term results of treatment of Hirschsprung's disease. Semin Pediatr Surg, 13 (2004), pp. 273-285 13. Levitt MA, Dickie B, Pena A. Evaluation and treatment of the patient with Hirschsprung disease who is not doing well after a pull-through procedure.Semin Pediatr Surg. 2010 May;19(2):146-53. doi: 10.1053/j.sempedsurg.2009.11.013. 14. Peña A, Elicevik M, Levitt MA. Reoperations in Hirschsprung's disease. J Pediatr Surg, 42 (2007), pp. 1008-1014 15. Giuliani S, Decker E, Leva E et al. Long term follow-up and transition of care in anorectal malformations: an international survey. J Pediatr Surg. 2016 Sep;51(9):1450-7. 16. Breech L. Sem Pediatr Surg. 2016 Apr;25(2):90-5. Gynecologic concerns in patients with cloacal anomaly. 17. Peña A, Bischoff A. – Surgical Treatment of Colorectal Problems in Children. 18. Complications with shunts in adults with spina bifida. P. Tomlinson, I. D. Sugarman. BMJ. 1995 Jul 29; 311(7000): 286–287 19. Weitz M, Heeringa S, Neuhaus TJ, et al. Standardized multilevel transition program: Does it affect renal transplant outcome? Pediatr Transplant. 2015;19(7):691–97. 20. Swedish national population-based study shows an increased risk of depression among patients with Hirschsprung disease. Amin L, Skoglund C, Wester T, Granström AL. Acta Paediatr. 2019 Oct;108(10):1867-1870. 21. Implementing transition: Ready Steady Go. Nagra A, McGinnity PM, Davis N, Salmon AP. Arch Dis ChildEduc Pract Ed. 2015 Dec;100(6):313-20. 22. Ready, Steady, Go - Achieving successful transition in cystic fibrosis. Connett GJ, Nagra A.Paediatr Respir Rev. 2018 Jun;27:13-15.
Figure 1: Patient Transition readiness assessment Date: ___________________ Name:__________________________Date of birth:_____________________ Transition Importance and Confidence: Please circle your response on a scale of 0-10 (0=not confident at all, 10=completely confident)
How important is it for you to transfer to adult doctors before age 22? 0
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How confident do you feel about your ability to transfer to an adult doctor? 0
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My Health: Please circle the box that applies to you today
I know my medical needs. Yes I know this
I need to work on this
Who can help with this?
I can explain my medical needs to others. Yes I know this
I need to work on this
Who can help with this?
I know my symptoms including those that require me to see a doctor quickly. Yes I know this
I need to work on this
I know what to do in case of an emergency.
Who can help with this?
Yes I know this
I need to work on this
Who can help with this?
I know my medicines, why I take them, when to take them. Yes I know this
I need to work on this
Who can help with this?
I know my allergies and what medicines I should not take. Yes I know this
I need to work on this
Who can help with this?
I have and carry important medical information daily (health insurance, emergency contact, allergies, medications, health summary). Yes I know this
I need to work on this
Who can help with this?
I know how consent and health care privacy change when I am 18 (legal adult). Yes I know this
I need to work on this
Who can help with this?
I can explain my customs and beliefs to others and how they affect my health care treatment and decisions. Yes I know this
I need to work on this
Who can help with this?
USING HEALTH CARE I can find my doctor’s number. Yes I know this
I need to work on this
Who can help with this?
I can make my own doctor appointments. Yes I know this
I need to work on this
Who can help with this?
Before my visit, I think of questions to ask. Yes I know this
I need to work on this
Who can help with this?
I have transportation to my doctor’s office. Yes I know this
I need to work on this
Who can help with this?
I know to show up 15 minutes before an appointment. Yes I know this
I need to work on this
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I know where to get medical care when my doctor’s office is closed. Yes I know this
I need to work on this
Who can help with this?
I have a file at home with my medical information. Yes I know this
I need to work on this
Who can help with this?
I have a copy of my current plan of care. Yes I know this
I need to work on this
Who can help with this?
I need to work on this
Who can help with this?
I can fill out medical forms. Yes I know this
I know how to get referrals to other providers. Yes I know this
I need to work on this
Who can help with this?
I have a pharmacy and know how to refill my medications. Yes I know this
I need to work on this
Who can help with this?
I know where to obtain tests (x-rays/labs) if my doctor orders them. Yes I know this
I need to work on this
Who can help with this?
I have a plan to keep health insurance after I turn 18 or older. Yes I know this
I need to work on this
Who can help with this?
My family and I have discussed my ability to make my own health care decisions at age 18. Yes I know this
I need to work on this
Figure 2: Types of surgical pull-throughs in Hirschsprung disease
Who can help with this?
Figure 3:
Non-relaxing sphincters
Botox injection
Redo-pull-through