The Management of Congenital Muscular Dystrophy ... - Cure CMD

More documents

Recommendations

Info

8 Palliative CareIndividual and Family Emotional Well-beingPalliative care aims to provide comfort byintegrating the emotional, spiritual, developmental,and physical dimensions of life into thecare of individuals with life-threatening diseases.Incorporating palliative care from the time ofdiagnosis can benefit you, your child, and themedical team as you anticipate and makedecisions regarding interventions that affectyour child’s quality of life.Although palliative care may seem to offer abroad range of services, the goals of palliativetreatment are concrete: relief from suffering,treatment of pain and other distressingsymptoms, psychological and spiritual care,a support system to help your child live asactively as possible, and a support system tosustain the entire family. Many peopleassociate palliative care with “giving up” orwhen end of life is near. However, palliativecare is much more than that: it is a holisticapproach to treating symptoms caused byserious diseases.Pain/FatiguePain may be a significant and under-recognizedproblem that can be due to various conditionsin different systems of the body. For example,the pain arising from progressive muscleweakness, scoliosis, and contractures mayrequire adjustments with seating and splinting.Emotional and psychological aspects, includinganxiety, depression, and fear, may also contributeto pain and fatigue. The interrelationshipbetween these areas may be considerable andneeds to be explored.Effective management for your child’s pain beginswith a comprehensive assessment of acuteand chronic symptoms. Determining the presence,frequency, and duration of painful episodeswill help to identify contributing factors andthose that help provide relief.Fatigue is commonly reported by children withCMD. Activity level, respiratory status, sleephabits, and various medications may cause orworsen fatigue.27Mental HealthBecause CMD can be difficult to diagnose, withmany uncertainties about the course of thedisease, you, your child, and other familymembers are naturally at an increased risk foremotional distress; among these are feelings ofdepression, anxiety, fear, and grief.It is important to monitor the emotionalwell-being of your child. Signs of concern maybe either direct, such as sadness, or indirect,like anger or restlessness. If you have anyconcerns about your child’s mental well-being,speak with your child’s medical team aboutobtaining a supportive psychologicalconsultation and discussing helpful resourcesfor coping. It is also important to monitor youremotional well-being as a parent or caregiver.Everyone has different ways of coping withstress and emotions; it is very common forparents to have difficulty dealing with theirfeelings when it comes to pediatric chronicillnesses such as CMD. When parents andfamily members are stressed, children willalso be stressed. Oftentimes family counselingis helpful.Such consultations should help bring aboutopen discussion, relationship building, and theacknowledgement of fears, tension, and sadness.Other resources for support may include:• Internet lists and groups (for example,Facebook groups such as Merosin PositiveMums for Merosin Negative Kids andYahoo support groups for LMNA-RD,SEPN1-RM, Ullrich CMD, Bethlemmyopathy, Walker-Warburg syndrome,lissencephaly and αDG-RD)• Cure CMD website (curecmd.org),information and message boards• in-person support groups at hospitals orother agencies• Support from your faith tradition ifrelevant.These supports can help to enable you and your
family to plan meaningfully and effectivelyanticipate and participate in care decisions forthe future when things may feel confusing andunclear.End-of-Life CareFamily members and health care providersunderstandably often find it difficult to discussthe possibility of death, but CMD can be apotentially life-limiting disease and sodiscussion of end-of-life care is appropriate.It is important that your child’s health careproviders help to guide you through potentialend-of-life concerns. Ideally this would happenbefore the occurrence of a major life-threateningevent, allowing you as a family time to clearlyexplore options and gather information beforedecisions need to be made.The need and timing for such discussion variesdepending on the diagnosis and course of thedisease, and is often more urgent when thediagnosis is more severe or is unknown. Thegoal is always for your child’s health care teamand your family to work together throughthese painful issues.The last decade has offered tremendousprogress to those with CMD. The draftingof care guidelines, development of aninternational registry and growingmomentum in research to identify possibletreatments have contributed to hope for thefuture. This building of infrastructureand raising awareness to support improvedhealth care and science will lead to newdiscoveries and continue to prolong andimprove the quality of life for your children.The journey of life with CMD is not a straight path,but rather spirals from issue to issue and back and forward again.Along the way, ongoing attention, care, and patience in the areas ofmedical, emotional, practical, and spiritual needs are mostimportant to support and enrich the lives of the affected individualand those most closely involved with his or her care.While reaching in for strength, be sure to also reach out.In addition to the CMD medical community, there is also agrowing community of families that may be able to share informationand insight as you continue along the journey of life with CMD,in all of its complexities.28
Page 6 and 7: Box 1CMD-LGMD Subtypes(abbreviation
Page 11 and 12: Hospital CareYour child may require
Page 13 and 14: 4 Respiratory ManagementCare of Bre
Page 15 and 16: Types of PulmonaryFunction Testing
Page 17 and 18: • fast heart rate or breathing ra
Page 22 and 23: 6 Cardiac ManagementTaking Care of
Page 24: IMPORTANT FACTS TO REMEMBERBe aware
Page 27 and 28: Table 5Age of onset of orthopedic c
Page 29: Box 4ConsiderationsforSpinalSurgery
Page 33 and 34: Children with LAMA2-RD are born wit
Page 35 and 36: Neurologist:A physician who has spe
Page 37 and 38: Advocate:To work with your child’
Page 39 and 40: Genetic mutation:A change in a pers
Page 41 and 42: Positive airway pressure (PAP):A ty
Page 43: of CMD. It is important for people

The Management of Congenital Muscular Dystrophy ... - Cure CMD

You also want an ePaper? Increase the reach of your titles

Delete template?

Save as template?