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What are the Benefits and Risks of Long Term use of Intrathecal Baclofen Pump Therapy in Pediatrics?

Cindy Staszak

Introduction Children with cerebral palsy (CP) and other neurological conditions often have severe spasticity, which leads to discomfort and deformity, impedes their ability to perform functional skills, and increases the burden of care for their families. Management of severe spasticity is difficult. Oral Baclofen is used to treat generalized spasticity; however, its effectiveness is often limited by the inability of the drug to cross into the central nervous system. (1). Baclofen is a Gamma-Aminobutyric acid (GABA) agonist that acts at the spinal cord level to impede the release of excitatory neurotransmitters. Since the 1960s, it has been used orally as a treatment against spasticity, but the benefits with this administration form is limited by sideeffects at higher doses (2 Ramstad). Unfortunately, Baclofen crosses the blood-brain barrier poorly, so oral administration cannot readily achieve therapeutic concentration in the cerebrospinal fluid. So, oftentimes, oral Baclofen administration isnt adequate to help these individuals and there are many unwelcome side effects. Administration of Intrathecal Baclofen (ITB) using doses in the order of one-hundredth of those required by the oral route may reduce spasticity while lowering the risk of dose-related adverse effects. Continuous intrathecal baclofen infusion (CIBI) [a.k.a. ITB therapy] via a subcutaneously implanted pump was approved by the US Food and Drug Administration for the treatment of spasticity of cerebral origin in 1996. Acting on spinal GABA receptors, CIBI causes fewer systemic side effects than orally administered baclofen. Theoretically, by reducing hypertonia, facilitation of daily care and symptomatic relief from painful spasm is achieved (3). A programmable ITB pump can be surgically implanted in a subcutaneous or subfascial pocket in the abdominal wall. The pump will then deliver continuous Baclofen through a catheter which runs under the skin and is inserted into an intrathecal space in the spinal column. The patient will then be monitored by a Baclofen Pump manager (doctor, nurse, etc.) to slowly increase the dosage over several visits to figure out the most beneficial continuous dosage. Before implanting the baclofen pump, care providers typically ask how it will benefit their child (1). Over the past two decades, ITB therapy has become widely acknowledged to be clinically effective in the relief of intractable spasticity of both spinal and cerebral origin (2). The purpose of this case study report is to find current research regarding the benefits and long term effects of ITB therapy in pediatrics and compare those findings with a pediatric student with an implanted ITB pump in school based physical therapy.

Case Description Patient (Student) The student was a 10.8 year-old male who had Cerebral Palsy, a seizure disorder (grand mal and petite mal), who was a 5th grade student at Hartvigsen, a school for special needs children in the Granite School District. His past medical history included that he had been born at 34 weeks gestation, had gastroschisis repaired at birth, a bacterial infection and meningitis at 2 months of age causing cerebral infarcts, and had an ITB pump placed into his abdomen in 2004 for which he has been receiving ITB therapy since then. He had demonstrated significant limitations in gross motor skills and mobility, which adversely affected his ability to access his school environment. This required school-based Physical Therapy services and involvement in a positioning program to increase opportunities for mobility throughout the school day to help develop his skills and increase his overall independence. He would also benefit from continuing to work on trunk control and sitting balance to help enable him to self-feed, an Individualized Education Program (IEP)1 goal, and complete fine motor tasks. He should also work on increasing independence with transfers to decrease the load on caregivers. The students medications are as follows: Medication:
Baclofen Keppra Vimpat Zoloft Risperidone Diastat/as needed 10 mg

Used for:
Spasticity Seizures Convulsions Depression, panic disorders, social anxiety disorder, eating disorders Schizophrenia, bipolar, mania, autism Anxiety, muscle spasm, status epilepticus Occasional constipation

Side effects:
Dizziness, drowsiness, insomnia Dizziness, somnolence, headache Dizziness, nausea, vomiting, ataxia Weight loss, insomnia, somnolence, fatigue, tremor, etc Orthostatic hypotension, sedation, dystonias, constipation Sedation, amnesia, decreased heart rate, decreased blood pressure, decreased respiration rate Upset stomach, bloating, cramping, gas, severe dehydration, hives

Miralax daily

The Individualized Education Program (IEP) is a written document required for each child who is eligible to receive special education services. It is provided to a student who has been determined first to have a disability and, second, to need special education services because of that disability. The IEP, the team that develops it, and what it must contain are governed by Part B of the Individuals with Disabilities Education Act (IDEA) and amendments to it. The IEP provides information on children's current levels of performance and directs the special services and supports that are provided to students who have IEPs. It includes provisions for defining annual goals, evaluating progress, and formalizing what is to be a free and appropriate public education (FAPE) for the student with the disability (4).

Discussion The following examination was performed by the physical therapist upon three year reevaluation for his Individual Education Plan (IEP). Range of Motion Measurements: Muscle Tone/ROM: Student demonstrates increased muscle tone in his left upper and lower extremities. His left upper extremity exhibits a flexion synergy pattern with voluntary movement. He often uses total body extension when standing or walking. Clonus was elicited in bilateral ankles. There were no muscle contractures noted in the lower extremities, though bilateral hamstrings, the left quadriceps, and left hip adductors demonstrated some tightness. Manual Muscle Test (MMT): Not tested Behavior/Communication Student is able to verbalize his wants and needs. He follows directions inconsistently. Posture/Alignment Student tends to hold his head/neck side-bent to the left. He demonstrates a flexible scoliosis, with a very mild left lumbar convexity noted in certain positions. His right lower extremity is longer than the left, and he displays excessive external rotation of the right hip and right lower knee valgus. His feet/ankles collapse into planovalgus in weight-bearing and are supported by Cascade Dynamic Ankle Foot Orthoses (DAFOs). Neuromotor - There were no abnormal reflexes noted. Head righting is intact. Equilibrium responses are present but weak in sitting. Protective reactions are inconsistent. Gross Motor/Mobility Student is able to lift and turn his head freely. He can maintain prone prop, roll from prone or supine, and transition supine to side-sitting independently. Preferred floor sit positions tend to be side-sitting with posterior pelvic tilt. Students modes of floor mobility are rolling, scooting in supine and occasionally commando-crawling very short distances. He is unable to attain quadruped, but will bear weight on his elbows and knees with assistance. Student cannot assume tall-kneeling, but can maintain when placed given maximal assistance or leaning heavily on a support surface. He is able to maintain bench sitting short periods with only close cuing, sit-up, and minimal assistance, but requires maximal assistance to maintain standing balance. Student walks with pony walker at school, using reciprocal steps. He is able to progress the walker independently but requires occasional assistance for direction. Student also participates in a standing program at school, utilizing a Rifton prone stander. Bed Mobility (Move up and down in bed, supine to edge of bed, etc): He can maintain prone prop, roll from prone or supine. Floor mobility Students modes are: rolling, scooting in supine, and occasionally commando crawling very short distances. Transfers (Sit to stand, bed to wheelchair, wheelchair to mat, etc): Supine to Sitting independently. Sitting: Preferred floor sit positions tend to be side-sitting with posterior pelvic tilt. He is able to maintain bench sitting short periods with only close cuing, sit-up, and minimal assistance.

Standing: Requires maximal assistance to maintain standing balance. Student also participates in a standing program at school, utilizing a Rifton prone stander. Ambulation/Stairs: Student walks with pony walker at school, using reciprocal steps. He is able to progress the walker independently but requires occasional assistance for direction. Interventions After examining the student, the physical therapist, parents and school staff discussed therapy and patient goals. The following year long term goals were set and agreed upon by the therapist, patients family and school staff. Long Term Goals: Goals for this Evaluation to be met in one year, then new goals will be established. 1. Student will maintain upright sitting position without support for a 5 minute long tabletop activity. 2. Student will perform a stand-pivot transfer with only set-up, verbal cues, and minimal assistance. I.E.P. Date Re-Evaluation Physical Therapist (PT) Frequency Physical Therapist Assistant (PTA) Frequency 3/28/2013 3/13/2016 1x/month 2x/week Initiation Date End Date PT Length of Session PTA Length of Session 3/28/2013 3/27/2014 At least 15 minutes 30 minutes

At the time of the IEP, the student was attending another school within this school district as a fourth grader within a contained classroom for special needs children and was then transferred to the Hartvigsen school when it opened in August 2013. The initial plan of treatment was set forth by the physical therapist.

Learning Environment School Related Self-Help Skills Functional Mobility Gross Motor Adaptive Equipment Learning Environment Bench sitting for a table-top activity. School Related Self-Help Skills

Areas of Intervention Yes Yes Yes Yes Yes

Assists with sit to stand transfer from wheelchair. Assists with transfer back into wheelchair by scooting back in seat. Stands at a table or support surface with assistance to facilitate weight bearing through lower extremities to increase strength, endurance and facilitate ability to perform activities of daily living. Functional Mobility Pony or Bronco Strider/walker to encourage and facilitate ambulation. Gross Motor Prone over a ball to facilitate wt bearing on outstretched arms. Tall-kneeling at a support surface with assistance to facilitate balance, endurance and improved posture. Adaptive Equipment Use of Standing frame with tray 30 minutes/day, 5 days/week in classroom to facilitate weight bearing while doing school table top activities.

Key: + -R A ~

Activity performed Student tolerated activity or position Student did not tolerate activity or position Student refused to participate Student Absent from school Approximately

Motor Activities

2013

Date: 10/7 10/9 10/14 A A A A A A A A A A A A + Assist + + + +

10/21 10/23 10/28

10/30

Student assists with sit to stand transfer from wheelchair Stands at a table or support surface with assistance Bench sitting for a table-top activity Prone over a ball-weight bearing on outstretched arms Tall kneeling at a support surface with assistance Student assists with transfer back into wheelchair by scooting back in seat

A A A A A A

+ + + + + +

+
In Pony Walker

+ + +

Prone Prop on Elbows

Maximum

+ + +

Motor Activities

2013

Date:

11/4 +

11/6 +

11/11 11/13
Mobile Fitter Sitter Danced to Music!

Student assists with sit to stand transfer from wheelchair Stands at a table or support surface with assistance Bench sitting for a table-top activity Prone over a ball-wt bearing on outstretched arms Tall kneeling at a support surface with assistance Student assists with transfer back into wheelchair by scooting back in seat Walk in Pony Walker back to classroom after completing his other Activities initiated 11/6/2013

A A A

+ + + + ~ 540
feet

A A

+ ~ 75
feet

A A

Patient Family Education The student, family and school staff was educated on safety with wheelchair, prone stander, transfers, use of Pony walker and other exercises performed in the motor room.

Outcomes The following table outlines the students current status at time of this assignment (note: strength was not formerly assessed due to students inability to comprehend requests and due to this students diagnosis of Cerebral Palsy). The student is continuing with ambulation, transfers and standing activities at home as well as at school.

Range of Motion Measurements: Muscle Tone/ROM: Student demonstrates increased muscle tone in his left upper and lower extremities. His left upper extremity exhibits a flexion synergy pattern with voluntary movement. He often uses total body extension when standing or walking. Clonus was elicited in bilateral ankles. There were no muscle contractures noted in the lower extremities, though bilateral hamstrings, the left quadriceps, and left hip adductors demonstrated some tightness. Manual Muscle Test (MMT): Not tested. Behavior/Communication Student is able to verbalize his wants and needs. He follows directions inconsistently, but on his good days he is more consistent. Posture/Alignment Student tends to hold his head/neck side-bent to the left. He demonstrates a flexible scoliosis, with a very mild left lumbar convexity noted in certain positions. His right lower extremity is longer than the left, and he displays excessive external rotation of the right hip and right lower knee valgus. His feet/ankles collapse into planovalgus in weight-bearing and are supported by Cascade DAFOs. Neuromotor - There were no abnormal reflexes noted. Head righting is intact. Equilibrium responses are present but weak in sitting. Protective reactions are inconsistent. Gross Motor/Mobility Student is able to lift and turn his head freely. He cannot maintain prone prop. Student cannot assume tall-kneeling, but can maintain when placed given maximal assistance or leaning heavily on a support surface. He has great difficulty maintaining bench sitting for short periods with only close cuing, sit-up, and now requires moderate assistance, and continues to require maximal assistance to maintain standing balance. Student walks with pony walker at school, using reciprocal steps. He is able to progress the walker independently but requires occasional assistance for direction. Student also participates in a standing program at school, utilizing a Rifton prone stander. Student was not re-tested on: Ability to roll from prone or supine, and transition supine to side-sitting independently. Preferred floor sit positions tend to be side-sitting with posterior pelvic tilt. Students modes of floor mobility are rolling, scooting in supine, and occasionally commando-crawling very short distances. He is unable to attain quadruped, but will bear weight on his elbows and knees with assistance.

Bed Mobility (Move up and down in bed, supine<>EOB, etc): Student was not re-tested on: Ability to maintain prone prop, roll from prone or supine. Floor mobility: Student was not re-tested on: Students modes are: rolling, scooting in supine, and occasionally commando-crawling very short distances.

Transfers (Sit to stand, bed to wheelchair, wheelchair to mat, etc): Supine with sitting with contact guard assist. Sitting: Student is having difficulty with maintaining bench sitting short periods with only close cuing, sit-up, and moderate assistance. Student was not re-tested on: Preferred floor sit positions tend to be side-sitting with posterior pelvic tilt. Standing: Requires maximal assistance to maintain standing balance. Student participates in a standing program at school, utilizing a Rifton prone stander 30 minutes daily. Ambulation/Stairs: Student walks with pony walker at school, using reciprocal steps. He is able to progress the walker independently but requires occasional assistance for direction. Student was absent from school on 11/13/2013, when the remainder of the re-testing was to be performed, for a scheduled surgery that we had not be informed about however, the surgery had nothing to do with the ITB pump or therapy. I sent a questionnaire to the mother of this student asking the following:
1. Did you notice a change in your childs tone following the ITB treatment initiation? 2. Did your child experience any problem(s) after the ITB pump implant surgery? 3. Does your child experience any side effects from the ITB treatment? 4. Do you ever notice a change in your child just prior to or after refilling his pump? 5. Has your childs pump ever been replaced? 6. Do you feel that your child, you and your family has benefitted from the ITB treatment? 7. Do you have any other information that you feel is important for me to know?

Unfortunately, as of the time of the final revisions to this paper were made, and turned in, the students mother had still not responded to the original questionnaire and follow-up that she had been sent.

Discussion An important factor that should be mentioned is that students with special needs can and often do have high absentee rates. This can have an adverse effect on the positive and hopeful outcomes that we would like to see because it is difficult to continue to increase their therapeutic exercise and activities due to their ongoing medical conditions. Students other prescribed medications can also play a role in their day to day abilities. In a study by Murphy etal (5) they discuss that in general, the systemic medications currently available to manage spasticity and dystonia in children with CP, including benzodiazepines, baclofen, and 2-adrenergic agonists, may suboptimally control the movement disorder while potentially exacerbating other functional limitations. This case study is a prime example. If you refer back to this students medication list and the possible side effects, it is a wonder that this student is ever able to stay awake during class, let alone have the ability to perform in a motor session or ambulate down the hall in a Pony Walker. There are many days that modifications need to be made to accommodate a students physical and emotional status. So, improvements are often times very subtle and may take a very long time to occur. The goal of ITB therapy is to improve quality of life for not only the child, but for their caregivers as well. ITB improves both spasticity and spasms. As a result of reduced spasticity and spasms, patients will be able to sleep better, become more independent with mobility, and their ability to do self-care helps improve urinary function. A decrease in muscle pain and fatigue that accompany spasm may also be seen. Thus effective Baclofen therapy can be delivered using [an] ITB pump, where effects of baclofen are maximized, while its side effects are minimized (6). According to a controlled study by Morton etal (7) significant improvements were found in comfort and ease of care. Few Improvements were found in active functioning or social participation. ITB can be a major benefit to the quality of life of the children and their carers [caregivers]. A main measure of the quality of life (QoL) used was the Caregiver Questionnaire, which showed marked improvements in overall score over 18 months of treatment, involving comfort, positioning, transfers, and personal care. This had been previously found by Gooch etal (1) in an open study. Comfort is very important for children with severe disabilities, who sit in the same position for long periods. Improvements in transfers and personal care are highly beneficial both to children and their carers [caregivers] (7). Some of the side effects of ITB pump implantation and therapy that have been reported are: Device related malfunction, decreased function due to decreased tone, seizures, constipation, pancreatitis, decubitus ulcers (8), implant infections (6), scoliosis (9), urinary retention, lethargy, wt gain, epilepsy, jumpy legs, hypothermia, drooling, reflux, discomfort with bending, nausea, worse swallowing (10) somnolence, headache, dizziness, hypotonia, etc. (11). Some of these side effects only affect a small percentage of patients, but they are possible side effects nonetheless.

In a study by Burn et al (9), the authors feel that the Baclofen induced hypotonia increases the instability of any, even mild, pre-existing spinal deformities. When the ITB pumps are inserted and treatment is ongoing in these young people during their growing years (at ~ 10 -15 years of age), the spine, in a relaxed, poor seated postural muscular position, may have the propensity to grow into a scoliotic curve according to the sitting forces put upon it. When deprived from its muscular control, the collapse of the spine can accelerate the progression toward kyphoscoliotic deformities. In a study by Gray etal (10), these authors found that even when significant problems arose, such as scoliosis, caregivers would often remain pleased because this was outbalanced by the benefits. The authors pre-set goals consisted of: ease of nursing care, improved sitting and reduction of spasm. These goals were chosen by the families and therapists and were achieved by 80% of the children. In a study by Tsoi etal (12) the researchers stated that, despite significant results found in several studies [e.g. children receiving exercise training, strength training, intrathecal baclofen therapy and diazepam had improved QoL (effect sizes measured using standardized difference ranged from 3.8 to 9.1)], were not able to identify a single intervention that was most useful in terms of improving the overall well-being of children with CP. Also important to note is the Important Safety Information for ITB Therapy that Medtronic, a Baclofen pump manufacturer, has published: Intrathecal Baclofen Withdrawal: Abrupt discontinuation of intrathecal baclofen, regardless of the cause, has resulted in sequelae that include high fever, altered mental status, exaggerated rebound spasticity, and muscle rigidity, that in rare cases has advanced to rhabdomyolysis, multiple organ-system failure, and death (11). Conclusion There are mixed reviews of the benefits, side effects and risks of ITB therapy, but research has shown it to be safe and effective in children in most cases. As with any invasive procedure and ongoing treatment there are inherent risks and possible side effects. The parents need to be fully aware of these risks and possible side effects before allowing an ITB pump to be surgically implanted in their child. On the opposite side of the side effect and risk spectrum are the benefits that this treatment can have. Decreased spasticity and spasms will decrease muscle pain as well as help the individual with ease of sitting and assist with the ability to help with transfers. But, sometimes decreased spasticity can make transferring a bit more difficult as some level of spasticity can help with transferring due to the muscles being more hypertonic (more rigid) as opposed to hypotonic (limp). Other added benefits are increased independence with activities of daily living and improved ability to sleep, which should help the overall health and well being of the child. With all of the comforts that may be achieved by the child, the caregivers will also reap some of those benefits with ease of care. Although there are benefits that can occur with ITB therapy, the parents also need to be educated on the realistic goals for this ongoing treatment, as oftentimes the child may not gain more function, but will hopefully maintain what function they do have.

Although there has been a plethora of research already done on ITB therapy, I would like to see more studies done with the addition of physical therapy treatments that could be initiated immediately after the ITB pump is inserted, and the children monitored more closely, so that these children, along with future ITB therapy candidates, could be given the best chance of possibly preventing some, if not all of the adverse side effects. We as physical therapy professionals and paraprofessionals, can help by educating the child, if old enough to comprehend, the childrens families, school staff, other support personnel, etc., on the importance of proper positioning in and out of the wheelchair as well as the need for exercise outside the wheelchair. If we could all critically think, outside the wheelchair, to create and further develop beneficial physical therapy programs that would address the individual needs of these children then that should assist with decreasing the side effects and enhance the positive benefits of ITB therapy.

References 1. Gooch, J. L., Oberg, W. A., Grams B., Ward, L. A. and Walker, M. L. (2004), Care provider assessment of intrathecal baclofen in children. Developmental Medicine & Child Neurology, 46: 548552. doi: 10.1111/j.1469-8749.2004.tb01013.x 2. Ramstad, K., Jahnsen R., Lofterod, B., Skjeldal, OH. (2010). Continuous Intrathecal Baclofen Therapy in Children with Cerebral Palsy When Does Improvement Emerge? Acta Paediatrica, 99:1661-1665. doi: 10.1111/j.1651-2227.2009.01596.x 3. RUSSMAN, B. S. (2010), Intrathecal baclofen. Developmental Medicine & Child Neurology, 52: 601602. doi: 10.1111/j.1469-8749.2009.03515.x 4. Individual Education Program (IEP) information retrieved 11/22/2013. http://www.education.com/reference/article/individualized-education-program-iep1/ 5. Murphy, N.A., Nicole Irwin M.C., Hoff, C. (2002), Intrathecal baclofen therapy in children with cerebral palsy: efficacy and complications. Arch Phys Med Rehabil 2002;83:1721-5. 6. Yasser Awaad, Tamer Rizk, Iram Siddiqui, Norbert Roosen, Kelly Mcintosh, and G. Michael Waines, Complications of Intrathecal Baclofen Pump: Prevention and Cure, ISRN Neurology, vol. 2012, Article ID 575168, 6 pages, 2012. doi:10.5402/2012/575168 7. MORTON, R. E., GRAY, N. and VLOEBERGHS, M. (2011), Controlled study of the effects of continuous intrathecal baclofen infusion in non-ambulant children with cerebral palsy. Developmental Medicine & Child Neurology, 53: 736741. doi: 10.1111/j.14698749.2011.04009.x 8. Campbell, W. M., Ferrel, A., McLaughlin, J. F., Grant, G. A., Loeser, J. D., Graubert, C. and Bjornson, K. (2002), Long-term safety and efficacy of continuous intrathecal baclofen. Developmental Medicine & Child Neurology, 44: 660665. doi: 10.1111/j.14698749.2002.tb00267.x 9. Burn, Sasha C., Zeller, Reinhard, and Drake, James M. (2010), Do baclofen pumps influence the development of scoliosis in children? Journal of Neurosurgery: Pediatrics, Feb; 5(2):195-9. doi: 10.3171/2009.9.PEDS08460. 10. Gray N, Morton RE, Brimlow K, Keetley R, Vloeberghs M. (2012), Goals and outcomes for non ambulant children receiving continuous infusion of intrathecal baclofen. European Journal of Paediatric Neurology, 2012 Sep;16(5):443-8. doi: 10.1016/j.ejpn.2012.01.003. Epub 2012 Jan 25.

11. Medtronic Literature Review. Intrathecal Baclofen Therapy for the Management of Severe Spasticity. Retrieved October 25, 2013, from http://professional.medtronic.com/pt/neuro/itb/eff/journalarticles/index.htm#.UoP9Sk7n_IU. 12. Tsoi, W.SE., Zhang, L.A., Wang, W.Y., Tsang, K.L., Lo, S.K. (2011), Improving quality of life of children with cerebral palsy: a systematic review of clinical trials. Child Care Health
Dev. 2012 Jan;38(1):21-31. doi: 10.1111/j.1365-2214.2011.01255.x. Epub 2011 Jun 15.