PBLD Table #12. Moderators: Jennifer Aunspaugh, M.D. Institution: Arkansas Children s Hospital, Little Rock, Arkansas. Objectives:

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1 PBLD Table #12 A 12- year- old child with neuromuscular scoliosis for posterior spinal fusion with cardiac disease and an uncooperative Jehovah s Witness parent. Moderators: Jennifer Aunspaugh, M.D. Institution: Arkansas Children s Hospital, Little Rock, Arkansas Objectives: Delineate the anesthetic risks of posterior spinal fusion for a patient with severe neuromuscular scoliosis Be able to adequately manage a neuromuscular spine patient in the setting of cardiac disease, a surgeon who loses an extraordinary amount of blood with each case, and a family who has very strong religious beliefs against blood transfusion. Outline Jehovah s Witnesses current beliefs regarding blood transfusion and what is acceptable and non- acceptable. Review blood salvaging techniques and which techniques are acceptable to Jehovah s Witnesses. Be able to make ethically sound decisions and design an algorithm for the learner s institution to follow when faced with the altering of patient care as a result of religious beliefs. Case history: A 12- year- old child with severe scoliosis is scheduled for a posterior spinal fusion. Her medical history is complicated by severe cerebral palsy, static encephalopathy, and moderate cardiac disease. She is quadriplegic and wheelchair bound. Her parents are Jehovah s Witnesses. Is this considered a patient with idiopathic or neuromuscular scoliosis? How severe is the curvature of the spine? Does this information even matter or affect your anesthetic management? Case history and physical examination (continued): This child is unaware of her surroundings and is breathing with an upper airway snoring noise that is quite audible. She is quadriplegic, functions at less than a 6- month- old level and is wheelchair bound. Physical examination revealed a thin female sitting in her wheelchair with no signs of distress and unaware of her surroundings. Vital signs were within normal limits. Mouth opening and neck mobility were within normal limits but she did have protruding front teeth. Cardiac and lung examination were normal.

2 What are your concerns with this patient s pulmonary status? Would you order Pulmonary Function Tests on this patient? If yes, what would you expect to find? Is a consult from a pulmonologist required? Do you think this patient will require post- op mechanical ventilation? Preoperative studies: An echocardiogram with poor images revealed severe LV depression with a shortening fraction of 19%. What risks and possible complication should be disclosed to the patient (and his parents)? Would you mention death as a possible complication? How do you present these complications? Do you discuss advance directives for the patient? Is an ECHO typically sufficient cardiac evaluation in these patients? The student asks what a shortening fraction mean. How that relates to this minor procedure we are planning on doing? Do we have alternatives to poor ECHO images? If our ECHO showed adequate images with normal LV function are we safe to proceed? Would the same be true for a major operative procedure (posterior spine fusion)? Any other studies that might help us determine whether he would be safe for major surgery? Is there anything else needed to optimize cardiac status prior to surgery (or is that necessary?). Would you consider using a TEE probe intraoperatively? Case progression: There is a letter on the chart from the pulmonologist consult stating that this patient will progressively develop increasing respiratory insufficiency in spite of anything we do. However, the surgeon is insistent on the surgery and convinces the family that the child will benefit regardless of what the pulmonologist has stated. The parents state they are Jehovah s Witnesses and, therefore, do not want their child to receive any blood products whatsoever. So what is your anesthetic plan? Local vs. sedation (MAC) vs. general anesthesia? Do we have alternative ways of taking care of this patient? What is your plan for IV access? Do we need any special monitoring (what would you use?)? Are you happy with his airway? What airway would you use and how would you obtain it? What would you use for analgesia? What do Jehovah s Witnesses believe in regards to blood transfusions? Which blood products are acceptable and not acceptable? What kind of alternative to transfusions can you offer this patient? Are Jehovah s Witnesses allowed to store their blood preoperatively? Would you start erythropoietin tonight for surgery tomorrow? Iron replacement infusions? Albumin? You arrange a meeting with the parents, a social worker, the surgeon and yourself. The parents still refuse blood products despite your presenting the risks to them. What is the next step? If this surgery was emergent, how would you proceed?

3 Intraoperative care: The patient s starting hematocrit is 29. Would you consider normovolemic hemodilution in the patient? Why or why not? Should you use an antifibrinolytic agent? The patient becomes hypotensive just after the surgeon exposes the entire spine. How would you manage this? What pressor agents would you consider? Would you treat with crystalloids or immediately turn to blood products? Postoperative care: Your patient is extubated the following day in the ICU. She was hemodynamically stable and comfortable. Do you discuss the intra- operative problems with the patient and his family? Do you discuss the amount of blood products required? Discussion: Scoliosis is a lateral deviation of the normal vertical line of the spine, which when measured by x- ray, is greater than 10 degrees. There is a lateral curvature of the spine with rotation of the vertebrae with rotation of the vertebrae within the curve. The etiology of scoliosis remains unknown, and the term idiopathic remains appropriate when otherwise healthy children develop this condition. Children with neuromuscular diseases, such as cerebral palsy and muscular dystrophies, develop scoliosis as a result of deteriorating muscle function in addition to mechanical distortion. Children presenting for scoliosis surgery represent a spectrum from the uncomplicated adolescent to severely compromised children. The primary reason for repairing the spines of these debilitated children is to allow them to sit upright without assistance, thus decreasing the incidence of chronic pulmonary aspiration and extending life expectancy. The most important preoperative medical concern in these patients is determination of the severity of restrictive lung disease. Preoperative pulmonary function testing (PFT) is indicated for curves >80 degrees, and a vital capacity less than 30% of predicted may indicate the need for postoperative mechanical ventilation. There is a direct correlation between pulmonary impairment and the magnitude of the thoracic curve. Severely affected patients may also suffer from cardiovascular dysfunction and will require echocardiography and cardiology consultation. These patients are also at risk of peripheral nerve injury during positioning as well as postoperative visual loss caused by ischemic optic neuropathy. Parents should be aware that the face of their child may appear swollen immediately postoperatively and this will reside over the first two postoperative days. Spinal fusion surgery in children and adolescents is often associated with major blood loss. It is primarily from disruption of internal vertebral veins and decortication of large areas of bone. Increased blood loss has been shown in a patients with preexisting neuromuscular disease, losing greater than 50% of their estimated blood volume, due to osteopenic bone, malnourishment, decrease factor VII activity, prolonged prothrombin time, and decreased venous tone. In addition, some seizure medications commonly prescribed to these patients can affect coagulation. Neuromuscular scoliosis patients typically have many more vertebrae fused; up to 15 levels with posterior procedures. Blood loss has been shown to increase with the number of vertebral levels fused

4 at approximately ml per vertebrae. Total blood loss in Duchenne s muscular dystrophy patients can be as high as ml. The following techniques are used to minimize blood loss during spinal fusion surgery: Positioning with the abdomen free to avoid venous compression. Controlled hypotension target MAP of 55-60mmHg has been recommended and various opioid and pressor agents have been used. Antifibrinolytic agents for an antifibrinolytic to be most effective an effective plasma concentration should be established before skin incision. Aminocaproic acid has been shown to decrease the EBL by 25% in comparison with tranexamic acid. In neuromuscular scoliosis, there is very high chance that a blood transfusion will be required regardless of techniques used to minimize blood loss. Some of the salvaging techniques available include hemodilution, autologous pre- donation, intraoperative salvage of shed blood (Cellsaver) and direct donation. Patients who are Jehovah s Witnesses present quite a challenge to the surgeon as well as the anesthesiologist when considering spinal fusion surgery. Jehovah s Witnesses have long been known for their rejection of blood and blood- component transfusion, even when necessary to save life. Witnesses believe that blood removed from the body should be disposed of, and to receive it back is against God s will, so they do not accept autotransfusion of predeposited blood. If a blood transfusion is required for a minor patient and the parents/guardians refuse blood products, a physician, social worker, and hospital legal counsel should be notified. An explanation should be provided regarding the hospital s policy or a parent/guardian s refusal of blood products to a minor and the process of obtaining a court order so that the child may receive blood transfusions as a life saving measure. Being respectful of the parent s religious beliefs and having an established protocol to follow, gave us the ability to handle this situation in a systematic fashion and to easily educate the parents about the risks involved and the steps that would be taken as life saving measures for their child. References: 1. Lin, Eric S., Kaye, Alan D., Baluch, Amir R., Preanesthetic Assessment of the Jehovah s Witness Patient. The Ochsner Journal (2012). 12: Shapiro, F., Sethna, N., Blood loss in pediatric spine surgery. Eur Spine J (2004). 13 (Supp1): S6-S Elder, Lee., Jehovah s Witnesses Accepting Blood Transfusion. Associated Jehovah s Witnesses for Reform on Blood, Wilton, N., Anderson, B. (2009). Orthopedic and Spine Surgery. A Practice of Anesthesia for Infants and Children. Cote, Lerman, Todres (Fourth Edition). Philadelphia, PA: Saunders Elsevier. 5. Do Jehovah s Witnesses Really Abstain From Blood? The Associated Jehovah Witnesses for Reform on Blood (AJWRB). Last addendum, 2000.

5 6. Kannan, S. et al. Bleeding and coagulation changes during spinal fusion surgery: A comparison of neuromuscular and idiopathic scoliosis patients. Pediatric Critical Care Medicine, vol.3 no Religious objections to medical care. American Academy of Pediatrics Committee on Bioethics. Pediatrics Feb; 99(2):