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Duchenne and Becker Muscular Dystrophy
OverviewDuchenne (DMD), Becker (BMD), and intermediate (IMD) muscular dystrophies represent varying clinical presentations of an X-linked, progressive symmetric muscle weakness caused by a relative or absolute absence of dystrophin, a muscle protein. The weakness, greater proximally than distally, occurs because muscles are gradually replaced by fatty, fibrotic connective tissue. DMD refers to the earliest, most common, and most severe form of the disease. It occurs primarily in males, although occasionally, female carriers demonstrate increasingly recognized manifestations, such as cardiac and motor issues, especially later in adulthood.
Boys with DMD will present in the preschool years with muscle weakness, difficulty walking, and large calves. Over time, muscle weakness worsens, resulting in loss of ambulation by 8 to 13 years of age. Orthopedic manifestations of DMD, such as toe-walking, contractures of the ankles, knees, and hips, and scoliosis, are common. Bowel and bladder function are often mildly affected with symptoms of constipation and urinary urgency. Mild to moderate developmental delay is common, but not universal. Treatment with oral corticosteroids can prolong the ability to walk by several years. After loss of ambulation, muscle weakness continues to progress and respiratory weakness leads to difficulty breathing. Susceptibility to respiratory infections and progressive deterioration of pulmonary function generally lead to premature death, usually in the twenties. Cardiomyopathy is common and generally progressive.
Boys with BMD present with weakness later in childhood, adolescence, or even young adulthood. Progression is much slower and life expectancy is longer. Cardiac problems are common in boys and adults with BMD.
IMD refers to cases of intermediate severity between DMD and BMD
Other Names & Coding
Pseudohypertrophic muscular dystrophy
G71.01, Duchenne or Becker muscular dystrophy
G71.0, Muscular dystrophy
ICD-10 for Duchenne and Becker Muscular Dystrophy (icd10data.com) and ICD-10 for Muscular Dystrophy (icd10data.com) provide further coding details.
PrevalenceThe pooled prevalence of DMD and BMD is estimated to be 1:20,000 and 1:50,000 in males (respectively). [Mah: 2014] Worldwide prevalence estimates of Duchenne and Becker muscular dystrophies vary, though, likely due to differences in diagnostic criteria, ascertainment, and survival. To date, no population-based prevalence data for DBMD by race/ethnicity have been published in the United States. [Romitti: 2015]
GeneticsDMD, BMD, and IMD are X-linked and caused by mutations in the DMD gene, which codes for the dystrophin protein. Dystrophin is large and has many possible deletions, duplications, and point mutations; 70% of mutations are deletions of one or more exons. Some areas of the gene are more prone to mutation than others. Although most mutations are known to be associated with either a milder (Becker-like) or more severe (Duchenne-like) phenotype, this is not true of all the known mutations. [Flanigan: 2009] Because of the complex genetics of the disease and one third of cases being due to new mutations, there is often no family history of DMD. Emerging therapies such as exon skipping and nonsense mutation readthrough are dependent on the mutation, emphasizing the need for a confirmed genetic diagnosis. For more information, see Duchenne Muscular Dystrophy: Genetics, Dystrophinopathies (GeneReviews), and Muscular Dystrophy, Duchenne (OMIM).
PrognosisBoys with DMD usually become non-ambulatory by age 12 and die in their mid-20s of respiratory complications. Oral corticosteroid use and proactive care of heart and respiratory issues, including early surgery for scoliosis and early use of non-invasive ventilation, has enabled affected boys to walk longer, live to adulthood, and make meaningful contributions to the community. Individuals with BMD often have a life expectancy into the 40s, and walk into their 30s. Up to 50% of individuals with BMD die of cardiac complications. Cardiac death is less common in individuals with DMD (20%), but likely to become more common as pulmonary complications are increasingly prevented and life expectancy is longer. [Darras: 2008]
Practice Guidelines
Roles of the Medical HomeA key role of the medical home is to coordinate care with a multi-disciplinary neuromuscular clinic; if none are available, the medical home will need to ensure proper surveillance. The medical home will also be responsible for collaborating with subspecialists and monitoring for side effects from steroids (e.g., weight gain, behavior changes, osteoporosis, and hypertension) and problems that indicate need for further intervention, such as worsening shortness of breath, increasing difficulty walking, and developmental delay. The medical home may assist with an Individualized Education Plan (IEP) or 504 plan, often needed for physical education and toileting; paperwork for a handicapped license plate (often downloadable from the state DMV web site); and suggestions for parent support networks or local, state, and national resources. Continuing family education would include information about disease progression and emerging treatments. As respiratory complications arise, the medical home should help the family understand the risks and benefits of potential interventions. Quality of life issues and end-of-life directives are important topics to discuss, though some families may be uncomfortable doing so in their son's presence. Pain can also become a significant issue as affected children reach adolescence. The medical home can support the family regardless of their choice to intervene minimally or provide full ventilatory support (physicians often underestimate quality of life on a ventilator). [Gibson: 2001] Referral to a hospice and/or wish-granting organizations may be appropriate. See (Other Resources) for wish-granting organizations, family resources, clinical tools, and educational materials.
Clinical Assessment
OverviewDMD is the most common childhood muscular dystrophy. Preschool-aged males with DMD presentations should have a creatine kinase (CK) measured with referral as needed.
In addition to regular well-child checks and acute care visits, boys with DMD, BMD, and IMD need ongoing assessment in the following areas:
- Strength and mobility
- Cardiac
- Pulmonary
- GI/nutrition
- Orthopedics
- Bone density
- Dental
- Psychosocial
Pearls & Alerts for AssessmentNegative genetic testing
When clinical presentation is consistent with DMD, but negative genetic testing exists, a muscle biopsy with dystrophin staining should be performed. Some genetic testing laboratories are able to detect up to 95% of mutations in the dystrophin gene. (See University of Utah DMD Deletion and Mutation Screening.) Even with the best available technology, 5% of genetic diagnoses remain elusive due to genetic anomalies that are difficult to detect under routine testing paradigms. In these cases, absence of dystrophin staining on muscle biopsy confirms the diagnosis. Additional testing may be available in some centers on a research basis.Females with DMD symptoms
Non-random inactivation of the X chromosome is likely responsible for the 8-10% of female manifesting carriers who have mild symptoms of DMD. [Soltanzadeh: 2010] In the early embryo stage of females, one X chromosome is inactivated in each cell, resulting in half of the embryo's X chromosomes being from the mother and half from the father. Occasionally, there is disproportionate inactivation of the X chromosomes from one parent. When this happens to the normal X chromosomes in a DMD carrier, sufficient reduction in dystrophin production results in symptoms including proximal muscle weakness, tea-colored urine (myoglobinuria) on exertion, and high creatine kinases (generally in the thousands).Mosaic carriers
During initial evaluation of a boy with DMD, maternal genetic testing helps determine if the mother is a carrier. Approximately 9 to 14% of mothers who are carriers may be missed by standard genetic testing because of germline mosaicism, where the maternal germ cells carry the mutation but somatic cells (including blood cells) do not. [Helderman-van: 2009] [Bakker: 1989] Mothers testing negative for carrier status should be warned about this possibility.Mothers of boys with DMD
Even asymptomatic carrier mothers of boys with DMD may present with cardiac problems later in life. Mothers of boys with DMD, particularly those known to be carriers, should be evaluated by their physicians for early heart disease. Echocardiograms and EKGs are recommended every 5 years, and more frequently if abnormalities are found.
For the ConditionEfforts for approval of newborn screening for DMD are underway. [Mendell: 2013]
Of Family MembersMothers of boys with DMD who have a known mutation, and female siblings over age 18, should be screened for carrier status and counseled accordingly. Symptomatic cardiomyopathy and mild motor symptoms are increasingly recognized in carrier females; screen for cardiomyopathy with a baseline echocardiogram in adolescence, then every 5 years starting at 25-30 years of age.
If carrier status is confirmed, prenatal testing and preimplantation diagnosis for subsequent pregnancies are available. For families who have not yet identified a mutation, have not had genetic testing, or, are not interested in prenatal testing, check the CK of male infants several weeks after birth (it may be falsely high right after birth) to determine likelihood of DMD. Follow by genetic testing if the CK is abnormal.
For ComplicationsSee the practice guidelines DMD-Part 1: Diagnosis, Pharmacological & Psychosocial Management (HHS) and DMD-Part 2: Implementation of Multidisciplinary Care (HHS) for screening of the many associated complications/comorbidities.
PresentationsPatients typically present with proximal muscle weakness, manifested as difficulty getting up from the floor, and large calves. The mean age of diagnosis of DMD, without a family history, is 4 to 5 years old. Affected boys may also have developmental delay and toe walking. Symptoms are often identifiable by age 2, but diagnosis is delayed an average of 2.5 years. [Ciafaloni: 2009] For an excellent review of presenting symptoms in patients with neuromuscular disease including photographs and video of boys with DMD demonstrating hypertrophied calf muscles, gait abnormalities, and the Gowers' sign, see Muscle Weakness Video Library (childmuscleweakness.org).
Diagnostic CriteriaDMD/BMD/IMD may be diagnosed based on typical clinical presentation, lack of or decrease in dystrophin staining on a muscle biopsy, and/or the demonstration of a mutation in the DMD gene.
Clinical ClassificationThe diagnosis of Duchenne, Becker, and intermediate muscular dystrophy may depend on the examiner's judgment, particularly before age 12. Some boys fit easily into a category: The boy who presents at age 2 and uses a wheelchair exclusively by age 10 has Duchenne, whereas the boy who presents at age 11 and is still walking at age 17 has Becker. But there are many in-between cases, and the nomenclature is not as important as understanding that they all are due to a mutation in the dystrophin gene and that they all will have progressive muscle weakness and, in many cases, cardiac problems. Literature is increasingly evaluating the links between genotype and phenotype in these disorders.
Differential DiagnosisGiven the high prevalence of DMD, a diagnosis of DMD is likely when, for example, there is a 4- to 5-year-old boy with an unusual gait, Gowers' sign, and a marked elevation in CK. Still, other neuromuscular disorders should be considered. Before genetic testing and/or dystrophin protein staining on biopsy were available, DMD/BMD/IMD could be confused with spinal muscular atrophy (SMA), congenital myopathies, other muscular dystrophies (especially limb girdle), polymyositis, and other muscle diseases. Using current technology, genetic diagnosis provides definitive diagnosis in >95% of patients.
History & Examination
In earlier stages, monitor for muscle strength and endurance and progression of contractures in ankle and hamstrings. Does the child fall frequently, have muscle pain after exertion, or get so tired the family does not bring him along? Later on, look carefully for complications of muscle weakness, including muscle contractures, scoliosis, decubitus ulcers, cardiomyopathy, respiratory muscle weakness (cough strength), nocturnal hypoventilation, dysphagia, malnutrition/obesity, and constipation.
Current & Past Medical HistoryGI/nutrition: Ask about weight gain and loss, calcium and vitamin D intake, activity level, constipation and toileting at school, water intake, and any difficulty with choking or swallowing liquids or solids. Treatment with corticosteroids complicates nutritional issues and may result in significant weight gain, particularly as boys become less active. Weight loss issues become more significant in later stages when mechanical aspects of eating limit intake.
Muscle strength and mobility: Ask about progression of weakness and fatigue. If walking, do frequent falls or difficulty with short treks signal the need for a scooter or wheelchair? Can the family go to the grocery store, or other places, without having to use a stroller? Can the child get up from the floor by himself? Is there difficulty negotiating stairs? Is the home wheelchair accessible? Does the family have a handicapped placard or license plate?
Cardiac/pulmonary: Any history of palpitations, shortness of breath, or dizzy spells? A sleep history (awakening, snoring, refreshed upon awakening, sleep position, AM headaches) can provide clues to possible nocturnal hypoventilation. The decreased activity of children in wheelchairs with endstage disease can make cardiovascular symptoms difficult to notice.
Immune: With steroidal use, ask about problems with frequent or severe infections; and, for annual flu shots, use the injected killed virus variety, rather than the intranasal live virus.
Orthopedic manifestations: Any fractures? Contractures? Scoliosis? Recent visits to orthopedics? Last X-rays. Dexa scan results, if any?
Dental: Last dental exam/treatment?
Family HistoryMaternal uncles may have the disease or, more likely, will already have died of DMD. Older brothers may also be affected, in which case children may be genetically tested before birth or have a creatine kinase (CK) done several weeks after birth. CK should not be performed at birth as delivery may raise the value even in boys without DMD. Families of children with DMD should have genetic counseling before future pregnancies. See Duchenne Muscular Dystrophy: Genetics. Boys with BMD may have uncles or brothers with BMD as well; families do not have both BMD and DMD clinical presentations except in very rare circumstances.
Pregnancy/Perinatal HistoryPregnancies with, and deliveries of, children with DMD/BMD/IMD are not distinctive.
Developmental & Educational ProgressAsk about gross motor and language delays. Cognitive delays are common in boys with DMD with full scale IQ's averaging 88. [Snow: 2013] School difficulties and/or learning problems should be inquired into in school age children.
Maturational ProgressPuberty changes? Chronic treatment of oral corticosteroids can delay puberty. Toileting concerns with adolescence?
Social & Family FunctioningExploring strengths and needs of the family of a child with newly diagnosed DMD/BMD/IMD will help the Medical Home anticipate family functioning in the future. Emotional status, depression? Behavior changes or attention problems if the boy is on steroids. Leisure activities? Family coping and resources? Autism spectrum symptoms?
Physical Exam
GeneralBoys with DMD are often quiet and rely on family members to answer questions. Look for Cushingoid facies if on steroid treatment. Look for signs of the wheelchair needing fitting or repair. (See Wheelchairs and Adapted Strollers.) Look for excessive hair growth if on steroids. Look for a withdrawn demeanor in adolescents that may signal depression and social isolation.
Vital SignsBP | HR for changes secondary to steroid treatment. See Blood Pressure Percentiles for Boys (Medcalc3000).
Growth ParametersHT | WT | BMI: Young children with DMD may exhibit decreased weight due to a decreased muscle mass; older boys with DMD, especially those on steroids, may be obese. Triceps skin fold measurements may be helpful. See the BMI Calculator (KidsHealth).
HEENT/OralDeflazacort and prednisone are associated with early cataracts. Check for a robust red reflex bilaterally.
HeartCardiac and pulmonary exams are initially normal. With progression, signs of cardiac dysfunction such as murmurs, irregular beats, and unusual heart sounds may appear. The cardiac examination may, however, remain relatively normal even with advanced disease. Symptoms of weak cardiac muscle include shortness of breath and easy fatigue. As weakness progresses, lung volume decreases. Check cough strength; although subjective, a weak cough in the office should prompt further evaluation such as peak expiratory flow or other PFTs and a referral to pulmonology. Severe scoliosis can affect cardiopulmonary function.
Extremities/MusculoskeletalLook for large calves, heel cord, and hip abduction contractures; a waddling lordotic walk, with increased difficulty when trying to run; and, difficulty getting up from the floor. See Gowers' Sign Video (YouTube). Observe for difficulty going up and down stairs. Muscles, especially those with pseudohypertrophy, may feel "woody." Circulation is often severely impaired in the feet and lower legs (as they are often in a dependent position for long periods of time). Contractures appear – early on, ankle tendons are tight and, later, contractures are also noted at the knee (hamstring) and at the hips (hip flexors). Scoliosis is common.
Neurologic ExamAt diagnosis, look for proximal muscle weakness, decreased reflexes (although normal reflexes do not exclude diagnosis), normal sensory and cranial nerve exams. A waddling, wide-based, lordotic gait is typical, as the pelvis drops to the side of the leg being raised. There is often a forward curvature of the lumbar spine. Toe walking may also be noted, due to contractures of the heel cord. Boys may not be able to run, or the waddling might become more prominent when they try. With progression, boys will have increased difficulty with stairs and a more prominent Gowers' sign. Over time, boys will demonstrate decreased, symmetrical proximal muscle strength. Hip girdle muscles are affected first, followed closely by shoulder girdle muscle weakness. Muscles feel 'woody.' Muscles become atrophied and muscle mass is decreased. Walking becomes increasingly difficult; most children become nonambulatory around 10-12 years of age. See excellent videos at Muscle Weakness Video Library (childmuscleweakness.org).
Boys with BMD may present later, but with a similar distribution of weakness in proximal muscles, particularly the hip girdle muscles. Progression is slower and walking continues into the late teens, sometimes into the thirties or more. Neck flexion strength in individuals with BMD is often preserved.
Laboratory TestingA very elevated creatine kinase (CK) is not specific for DMD, but almost always present; values from boys with DMD are in the thousands (20,000 or higher is not unusual). A CK is useful for diagnosis only; its level is not followed once diagnosis is established. The degree of elevation is not significant at diagnosis; for instance, a value of 20,000 in one patient does not indicate a more severe form of DMD than a value of 12,000 in another patient. Liver enzymes (AST and ALT) are often abnormally high as well, reflecting leaky muscle fiber membranes rather than liver disease (unless other signs of liver disease, such as jaundice or abnormal clotting, are present). Elevated liver enzymes sometimes bring a child to the attention of a gastroenterologist before muscle disease is diagnosed. If so, measurement of the liver-specific enzyme, gamma glutamyl transferase (GGT) should be normal, as should bilirubin and alkaline phosphatase measures.
CK is not as reliably elevated in boys with BMD or IMD, or in boys with advanced DMD and very low muscle mass.
Urinary or blood monitoring for impaired glucose tolerance should be performed periodically for boys on steroids.
Genetic TestingMolecular genetic testing should be performed to confirm the diagnosis and guide genetic counseling for family members. The type of mutation may determine the boy's eligibility for clinical trials, e.g. nonsense readthrough and exon skipping therapies now in clinical trials. See Research in DMD (Parent Project Muscular Dystrophy). Because not all mutations can be identified, a negative genetic test does not rule out DMD. Some labs offer limited testing while others are able to detect the mutation in >95% of the cases. For the most comprehensive genetic testing, see University of Utah DMD Deletion and Mutation Screening and Genetic Testing Labs - Dystrophinopathies (GeneTests). If the genetic testing is negative but the clinical situation is highly suggestive of DMD, a muscle biopsy is recommended.
Other TestingMuscle biopsy: Preferred by some professionals for diagnosis of DMD, muscle biopsy may also be useful with negative genetic testing. In addition to non-specific signs of muscular dystrophy, including muscle fiber degeneration and regeneration, central nuclei, and replacement of muscle fibers by fat and connective tissue, dystrophin staining will be 0-5% in boys with DMD and 20-50% in boys with BMD. [Darras: 2008]
EKG and echocardiogram: At diagnosis, biannually until age 10, and then yearly, and preoperatively if undergoing general anesthesia.
Cardiac MRI: Some cardiologists use an MRI to assess cardiac structure and function in boys with DMD. In older boys, or those with significant chest wall deformities, echocardiographic images may be limited and a cardiac MRI will provide better functional assessment. In addition, using contrast agents with cardiac MRI allows assessment of scarring of the heart muscle.
Spine x-rays: To look for scoliosis or compression fractures, as clinically indicated.
PFTs and overnight oximetry: 1-2 times per year, starting when boys become non-ambulatory or as clinically indicated.
Ophthalmologic exam: Yearly, if the patient is on steroids.
Dexa scan: Consider if risk factors are high (e.g., nonmobility, poor calcium/vitamin D intake, previous fractures, on steroids) or if there have been fractures.
Specialty Collaborations & Other Services
Neuromuscular Clinics (see NM providers [2])
A multidisciplinary approach to care of boys with DMD is preferred. MDA Clinics, which are sponsored by the Muscular Dystrophy Association, are found in many locations nationally and often have various subspecialists available. Visits to the MDA Clinic are free for the uninsured. These clinics also may be involved in research protocols for treatment of children with DMD. See Find Your MDA Clinics (MDA.org) for clinic locations and local details.
Pediatric Neurology (see NM providers [31])
If a multidisciplinary clinic is not available, consider a referral to pediatric neurology with expertise in muscle disease for confirmation, genetic testing, counseling, and the initiation of treatment. Collaboration with the Medical Home should assure visits at six-month intervals to monitor the disease and order specific screening (e.g., ECHO and pulmonary function testing).
Pediatric Genetics (see NM providers [4])
Geneticists or genetic counselors help educate the family and determine the risk for subsequent children. Several visits may be needed to handle the large amount of information and to address new questions. Genetic expertise may be available in a multidisciplinary clinic for muscle disease.
Pediatric Orthopedics (see NM providers [13])
Consider referral to orthopedics for baseline evaluation and management of contractures, gait problems, and the need for equipment for ambulation, such as walkers. Initially these visits may be every year, but, as the disease progresses, the child may need to be seen at 6-month intervals.
Pediatric Physical Medicine & Rehabilitation (see NM providers [4])
A referral to physical medicine and rehabilitation may help in the evaluation of contractures, gait problems, and obtaining aids for ambulation. PM&R may be available at MDA Clinics.
Social Workers (see NM providers [35])
Families may need social work at diagnosis for counseling and finding available financial and community resources. Should be available on an ongoing, as needed basis for questions regarding the emotional impact of the disease on the family and child, for help negotiating available services (e.g., funding for a ramp to the home), and for school issues.
Physical Therapy (see NM providers [321])
Periodic visits with PT can help to evaluate and maintain abilities. Frequency of visits should be based on many factors (need, financial resources, availability, and access) and should be balanced with the goals of treatment (ranging from post-surgical PT to a home-therapy program taught to the parents).
Pediatric Cardiology (see NM providers [31])
Boys with DMD and BMD should receive cardiac evaluation with echocardiogram (or MRI) and EKG at diagnosis, every two years until age 10, and then annually. Additional visits before general anesthesia are also recommended.
Pediatric Pulmonology (see NM providers [7])
Boys with DMD should initially see a pulmonary specialist for a baseline evaluation and then visit regularly after loss of ambulation. Periodic screening may include pulmonary function testing and/or overnight oximetry. If overnight oximetry is abnormal, an overnight sleep study determines if NIPPV (noninvasive positive pressure ventilation) is needed. If needed, a specialist will fit the child with NIPPV equipment and determine settings. Cough strength should also be evaluated.
Nutrition Assessment Services (see NM providers [84])
Early referral should be made for patients who become overweight (which makes it more difficult for already weak muscles to move the body) or underweight (no reserve, risk of pressure ulcers).
Bone Densitometry/DEXA (see NM providers [2])
Bone densitometry can guide dietary and physical therapy interventions. Children who are on steroids, non-ambulatory, have low bone density on x-ray, have low calcium and/or vitamin D intake, or who have had fractures, are particularly at risk.
Pediatric Ophthalmology (see NM providers [9])
Children taking steroids should have periodic eye exams for cataracts.
Treatment & Management
Pearls & Alerts for Treatment & ManagementSteps before starting steroids
Before starting steroids, ensure immunity to chicken pox and, if warranted, test for tuberculosis. Make sure all immunizations are up to date, especially pneumococcus. While on steroids, annual influenza shots should be injectable, killed-virus type.Depolarizing muscle agents and anassthesia risk
Depolarizing muscle agents should be avoided during anesthesia due to risk for malignant hyperthermia. See [Birnkrant: 2007] and [Hayes: 2008].Monitor nocternal hypoventilation
Nocturnal hypoventilation can be monitored for by taking a sleep history (awakening, snoring, refreshed upon awakening, AM headaches).Assess cough strength
Cough strength assessment should not be forgotten during appointments. Although subjective, if cough strength is weak, consider a pulmonology referral and/or a prescription for a cough-assist device.
Before starting steroids, immunizations should be up to date and immunity to varicella and pneumococcus confirmed. Also, because steroid use in DMD is associated with an increased risk for osteoporosis, 25-OH vitamin D levels should be checked and referral made to a dietician to determine typical calcium and vitamin D intake. If necessary, recommend dietary calcium and vitamin D supplements. A baseline DEXA scan to assess bone mineral density at the commencement of steroid treatment is also recommended. [Quinlivan: 2005] See also Duchenne Muscular Dystrophy: Osteoporosis and Calcium and Vitamin D.
Little evidence exists on optimum time to start prednisone or how long to continue it. Some investigators believe it should be started as early as possible and others believe that treatment should start at age 5. [Manzur: 2008] [Merlini: 2003] The recommended dosage of oral prednisone/prednisolone is 0.75 mg/kg/day (although doses as low as 0.3 mg/kg/day have some beneficial effect). Some experts will dose prednisone daily, while others prefer alternate regimens that may decrease side effects, particularly during the school week. Two alternate regimens are:
- On alternate days, averaging at 0.75 mg/kg/day
- A week's dose of prednisone (5.25 mg/kg/week) given in 2 divided doses on weekend days.
- Hypertension
- Behavioral problems
- Weight gain, with Cushingoid appearance and failure to gain expected height
- Osteoporosis
- Impaired immune and adrenal function
- Impaired glucose tolerance
- Gastrointestinal symptoms, including discomfort and gastric ulcers
- Cataracts
- Excessive hair growth
- Acne and striae
Deflazacort, (not available in the US), is thought to have the same efficacy as prednisone but with a different side effect profile, including a greater incidence of cataracts but less weight gain. Parents may ask about this drug and some may wish to order it from abroad via the Internet. The dose is 0.9 mg/kg/day. See [Houde: 2008].
All boys on steroids should be monitored for efficacy and side effects, including:
- Height, weight, and BMI monitoring, check face for Cushingoid features
- Blood pressure surveillance
- Fasting or random blood glucose surveillance
- Mood and behavior changes
- Skin changes
- GI symptoms
- History of fractures
- History of frequent infections
- Eye changes
Stopping Steroids
Some experts continue to give steroids after boys with DMD become non-mobile since pulmonary and cardiac function may still benefit. [Balaban: 2005] Steroids should be tapered over time and not stopped abruptly due to the risk of adrenal suppression. Common practice is to go to 1/2 of the original dose for a week, then 1/2 of that dose for a week, and then 1/2 of that dose for a week before stopping.
Specialty Collaborations & Other Services
Neuromuscular Clinics (see NM providers [2])
Boys with DMD and BMD should be referred periodically to a multidisciplinary neuromuscular clinic, such as an MDA clinic, when available.
Pediatric Neurology (see NM providers [31])
When a multidisciplinary clinic is not available, consider a referral to pediatric neurology for treatment or co-management.
Pediatric Physical Medicine & Rehabilitation (see NM providers [4])
If not available at the multidisciplinary clinic, consider periodic referrals to physiatry to follow mobility issues and activities of daily living.
Physical Therapy (see NM providers [321])
Consider a baseline referral to physical therapy to evaluate and prevent contractures, maintain conditioning, and perform wheelchair fitting as needed. The frequency of visits should be guided by need, financial resources, availability, and access. Visits should be balanced with treatment goals (e.g., post-surgical PT or a home-therapy program taught to the parents).
Especially with advanced cardiomyopathy, arrhythmias are common, and periodic Holter monitoring should be considered. Thromboembolic events may occur in boys with significant cardiomyopathy and anticoagulant therapy may be warranted.
See also Duchenne Muscular Dystrophy: Cardiomyopathy.
Specialty Collaborations & Other Services
Pediatric Cardiology (see NM providers [31])
Baseline and subsequent periodic visits to pediatric cardiologists familiar with DMD are recommended for evaluation and treatment of cardiac complications
Boys with DMD usually die from complications related to the respiratory system, particularly respiratory failure during an infection. Symptoms of pneumonia should prompt evaluation by the Medical Home. Pneumovax and influenza vaccines should be kept up to date. Death from pneumonia/respiratory failure occurs in approximately 75% of older boys with DMD, often occurring within approximately one year of the appearance of daytime hypercapnia; this may be delayed with appropriate intervention. [Ishikawa: 1999] [Simonds: 1998] See [Birnkrant: 2010] for the newest respiratory guidelines for the care of children with DMD.
Early in the course of DMD, progressive muscle weakness leads to nocturnal hypoventilation and hypoxia, but the process is gradual and patients may not be aware of the problem. Begin monitoring for signs and symptoms of respiratory problems once the child is non-ambulatory. Inquire about symptoms of nighttime hypoventilation:
- Morning headache
- Daytime sleepiness
- Increased restlessness during sleep, increased need for turning during sleep
Assisted Nighttime Ventilation
Several studies have suggested that assisted night-time ventilation (non-invasive intermittent positive pressure ventilation or IPPV) can improve general health and life expectancy; boys with DMD may notice less frequency of chest infections, more energy, and better sleep. [Bach: 1995] However, respiratory muscle weakness will continue to progress and assisted daytime ventilation will become necessary during illnesses and then on a more regular basis. Intervention may be by non-invasive techniques or by tracheostomy, depending on local experience and patient/family preferences. See DMD Respiratory Care (Consensus Statement, ATS) ( 102 KB), [Finder: 2004] and [Birnkrant: 2010].
Some patients and their families choose not to pursue assisted ventilation. Families should be counseled about options and supported in their choices.
Specialty Collaborations & Other Services
Pediatric Pulmonology (see NM providers [7])
Evaluate for baseline lung function testing when approaching full-time wheelchair use. Annual, or bi-annual visits may be helpful after age 12, after full-time wheelchair use, or if vital capacity is below 80% predicted.
When first diagnosed, a majority of patients have tight heel cords and a history of toe-walking. An orthopedic surgeon, experienced in the care of children with DMD, should be consulted to follow this problem and initiate treatment. Treatment might include ankle-foot orthoses (AFOs), heel cord stretching exercises, night splinting, and/or serial casting. At some point, surgery may be necessary to lengthen heel cords, despite other treatments. Some investigators suggest waiting until the fixed deformity is greater than 20 degrees, whereas other investigators suggest early prophylactic muscle/tendon lengthening procedures. In boys who have significant weakness the risk of losing ambulation due to a prolonged non-weight bearing period after surgery exists. Surgery should be considered carefully in light of this risk, and if performed, early mobilization is imperative. Reasons to consider surgery include:
- Heel cord lengthening may prolong the ability to walk
- Preventing foot deformities after wheelchair confinement may help with positioning and allow continued shoe wearing
As the disease progresses, contractures may also develop at the knees and hips leading to difficulty with wheelchair seating, discomfort while sleeping, and skin ulcers. Stretching exercises may be useful in delaying these contractures. Occasionally, contractures may require surgical correction (i.e., hip flexor-abductor releases, distal hamstring releases).
Older boys with DMD exhibit scoliosis 90-95% of the time; the age of onset is variable. Trunk muscles progressively weaken, leading to collapse of the spine into a long C-shaped curve. In addition to seating problems and discomfort, scoliosis can lead to respiratory compromise due to decreased lung volume. A spine exam and spine X-rays (sitting anteroposterior spine) should be performed on a routine basis after the age of 10 years or when patients become non-ambulatory. Scoliosis surgery is recommended when the curvature measures 20 to 30 degrees; bracing is not thought to be helpful and is poorly tolerated. Scoliosis correction is major surgery and should be performed before pulmonary or cardiac function is too compromised.
Specialty Collaborations & Other Services
Pediatric Orthopedics (see NM providers [13])
Baseline evaluations and periodic visits with pediatric orthopedics are recommended for the management of contractures and scoliosis.
Pediatric Physical Medicine & Rehabilitation (see NM providers [4])
Periodic referrals to physiatry help maximize mobility and activities of daily living.
Hospitals (see NM providers [102])
Shriners Hospitals, where available, provide free orthopedic care to children with DMD.
In adolescence and beyond, families will often need additional equipment, including bath chairs, slides, power lifts, and commode systems. Physiatrists and physical and occupational therapists can help determine needs and the best equipment for the family, as well as helping with insurance preauthorization. Sometimes organizations such as the MDA Clinic and Shriners Hospitals will have donated equipment for families without financial resources.
Specialty Collaborations & Other Services
Pediatric Physical Medicine & Rehabilitation (see NM providers [4])
If not provided in the neuromuscular clinic, refer to physiatrist and physical and occupational therapists for periodic evaluation of mobility and daily living needs and help with equipment ordering and insurance preauthorization.
Gastro-Intestinal & Bowel Function
Specialty Collaborations & Other Services
Pediatric Physical Medicine & Rehabilitation (see NM providers [4])
Helpful to evaluate and treat toileting issues in youths and young adults with DMD.
Physical Therapy (see NM providers [321])
For evaluating equipment needs, such as adapted toilets and lifts.
Occupational Therapy (see NM providers [298])
May be helpful for evaluating needs of daily living such as toileting.
Nutrition and the prevention of obesity should be discussed early and often. As the child grows and develops, dietary and caloric needs should be routinely evaluated. Obesity, often independent of steroid use, may be seen as early as age 7; its prevalence seems to peak in the early teens at about 54%. [Willig: 1993] Obesity makes movement and activity even more difficult. Undernutrition may also be seen after the age of 14 years due to weakness and incoordination of the muscles used in chewing and swallowing. When possible, involve a nutritionist for underweight or overweight boys.
Boys should be instructed to maintain cardiovascular conditioning through activities such as walking and swimming and to begin a program of stretching. Boys with DMD should avoid body-building type exercises, such as weight lifting. It might be necessary to write a note or communicate directly with physical education teachers regarding limitations and exercising.
Steroidal use increases risk of osteoporosis. Provide guidelines regarding nutrient intake, especially for Calcium and Vitamin D. Daily use of an oral calcium supplement (like calcium carbonate antacids) is an inexpensive way to provide calcium supplements, although excess calcium may lead to kidney stones. If fractures occur in boys with osteopenia, IV or oral bisphosphonate treatment is sometimes recommended by endocrinologists. [Bushby: 2005]
With a history of choking or dysphagia, address swallowing problems and consider silent aspiration, which may further compromise respiratory ability. If adequate nutrition cannot be safely accomplished with oral feedings, gastrostomy tube placement and enteral feedings under guidance of a nutritionist is strongly recommended. See Feeding Tubes and Gastrostomies
Specialty Collaborations & Other Services
Pediatric Endocrinology (see NM providers [29])
Consider a referral if osteoporosis and fractures are present for consideration of bisphosphonate treatment.
Nutrition Assessment Services (see NM providers [84])
Periodic visits with a nutritionist can help to avoid/manage nutritional deficits, overweight, and underweight, and to manage calcium intake.
Development (general)
Specialty Collaborations & Other Services
Early Intervention for Children with Disabilities/Delays (see NM providers [156])
Can provide developmental evaluation and intervention in the preschool years.
Developmental - Behavioral Pediatrics (see NM providers [2])
May assist in evaluating and monitoring development, and with tapping helpful community resources and coordinating their interventions.
- Special transportation needs
- Toileting issues
- Class placement (e.g., mainstreaming vs. a special education class)
- Responsibility in case of a school emergency
- Therapy needs
- Physical education restrictions
- Developing an IEP for Boys with DMD (Parent Project MD)
- Educational Issues for Boys with DMD (Parent Project MD)
- Inclusion Activities for Boys with DMD (Parent Project MD)
Specialty Collaborations & Other Services
General Counseling Services (see NM providers [95])
This category includes all types of counselors/counseling for children. Once on the page, the search can be narrowed by city or using the Search within this Category field.
Neuropsychiatry/Neuropsychology (see NM providers [0])
To evaluate and suggest interventions for boys with DMD who are experiencing learning problems.
Specialty Collaborations & Other Services
Pediatric Dentistry (see NM providers [70])
Dentists who have received training in caring for children with special health care needs as part of their pediatric dentistry fellowship are preferred.
General Dentistry (see NM providers [13])
Dentists who have interest or experience in caring for children with special health care needs are preferred.
In response to increasing awareness of transitional issues, the MDA has initiated a transitions program targeted at adolescent and young adult patients with neuromuscular disease. Goals of the program include empowering patients and families through access to information and services. See the MDA Young Adult Programs, Transition Issues.
Specialty Collaborations & Other Services
Hospice & Palliative Care (see NM providers [65])
Programs can be very helpful for patients and families as debility encroaches on life span; can assist with making key choices regarding interventions and help family members cope.
Wish Foundations (see NM providers [17])
Provide children with serious illness and their families with trips, visits, gifts, and other services, usually on a one-time basis.
Issues Related to Duchenne and Becker Muscular Dystrophy
Clinical AssessmentDuchenne Muscular Dystrophy: Cardiomyopathy
Funding & Access to CareWriting Letters of Medical Necessity
Gastro-Intestinal & Bowel FunctionDuchenne Muscular Dystrophy: Bowel and Bladder Problems
Mobility/Function/ADLs/AdaptiveDuchenne Muscular Dystrophy: Orthopedic Concerns
Nutrition/Growth/BoneDuchenne Muscular Dystrophy: Osteoporosis
Ask the Specialist
How can we tell our son about his DMD in a truthful, but not frightening way?
Discussions depth will depend on the age and cognitive level of the boy with DMD and family preferences. Often, discussions need to continue over the years as the boy gains understanding or hears new information. Sometimes, the boy with DMD understands a fair amount without being told; the first step, therefore, is to learn his level of understanding. Attending an MDA camp, where the boy can meet other children with DMD and see boys in various stages, is often helpful. Research in DMD is progressing quickly, discussions should hold hope for the future.
My son is 19 and will be attending community college nearby. He doesn't want to wear diapers and won't/can't get anyone to help him go to the bathroom while he is at school. What can we do?
There are various types of external catheters, used primarily by men with spinal cord injuries. Although many providers worry about the risk of infection and skin irritation/breakdown with these systems, many users are quite satisfied. When seeking a local rehabilitation facility, locate one with expertise in spinal cord injury and ask to set up an appointment with a provider who is familiar with catheter use and side effects.
Resources for Clinicians
On the Web
DMD Respiratory Care (Consensus Statement, ATS) ( 102 KB)
New approaches for therapies of respiratory complications in DMD. Covers sleep evaluation, nutrition, cardiac involvement, scoliosis, and corticosteroids. Discusses end-of-life care and the decision to use or not use a respirator; American Thoracic Society.
Dystrophinopathies (GeneReviews)
An expert-authored, peer-reviewed, current disease description that applies genetic testing to diagnosis and management information; sponsored by the U.S. National Center for Biotechnology Information, U.S. National Library of Medicine.
Muscular Dystrophy, Duchenne (OMIM)
Extensive review of literature that provides technical information on genetic disorders; Online Mendelian Inheritance in Man site, hosted by Johns Hopkins University.
Neuromuscular Disease Center
Comprehensive website on neuromuscular diseases; Washington University, authored by Alan Pestronk, MD.
Helpful Articles
PubMed search for Duchenne or Becker muscular dystrophies in children, last 2 years
Bushby K, Bourke J, Bullock R, Eagle M, Gibson M, and Quinby J.
The multidisciplinary management of Duchenne muscular dystrophy.
Current Paediatrics. 2005;15:292-300. / Full Text
Flanigan KM, Dunn DM, von Niederhausern A, Soltanzadeh P, Gappmaier E, Howard MT, Sampson JB, Mendell JR, Wall C, King WM,
Pestronk A, Florence JM, Connolly AM, Mathews KD, Stephan CM, Laubenthal KS, Wong BL, Morehart PJ, Meyer A, Finkel RS, Bonnemann
CG, Medne L, Day JW, Dalton JC, Margolis MK, Hinton VJ, Weiss RB.
Mutational spectrum of DMD mutations in dystrophinopathy patients: application of modern diagnostic techniques to a large cohort.
Hum Mutat. 2009;30(12):1657-66. PubMed abstract
Clinical Tools
Growth/BMI Charts
BMI Calculator (KidsHealth)
BMI calculator with instructions and interpretations of BMI scores.
Patient Education & Instructions
Diagnosis & Management of DMD - Guide for Families ( 1.8 MB)
Care standards based on the DMD published in the Lancet Neurology in 2010; contains many images and graphics, uses much ink if printed.
FAQs about Wheelchairs (Parent Project, MD)
Answers to frequently asked questions about wheelchairs.
Resources for Patients & Families
Information on the Web
Duchenne Muscular Dystrophy (MedlinePlus)
A brief description for patients and their families of DMD, diagnosis, and management; from the National Library of Medicine.
Muscular Dystrophy (MedlinePlus)
Health Topic page with numerous links to reliable information; National Library of Medicine.
Duchenne and Becker Muscular Dystrophy (Genetics Home Reference)
Detailed review of 2 major types of muscular dystrophy; sponsored by the National Library of Medicine.
Learning about DMD (National Human Genome Research Institute)
Information for families with a focus on genetics.
Muscular Dystrophy (CDC)
General information about MD for families that includes references to recent publications, extensive web resources, sites for kids, and genetic research for parents and families. Also includes current surveillance and research sponsored by the CDC to track incidence and treatment; Centers for Disease Control and Prevention.
Muscular Dystrophy (NINDS)
Information about muscular dystrophy, treatment, prognosis, research, and links to other organizations; National Institute of Neurological Disorders and Stroke.
Consumer Guide to Hospice (WashingtonPost)
A consumer guide to hospices that participate in Medicare, searchable by state and county.
National & Local Support
Parent Project Muscular Dystrophy
Comprehensive site that covers the latest research, treatments, and related issues; site founded by family members of children with DMD.
Muscular Dystrophy Association
In-depth information about care, research, and support.
Research in DMD (Parent Project Muscular Dystrophy)
Listing and explanation of clinical trials for DMD.
Clinical Trials in DMD (clinicaltrials.gov)
A list of trials registered with the National Institutes of Health; some may be of interest and offer the opportunity to participate in research.
Services for Patients & Families in New Mexico (NM)
|Service Categories
|# of providers* in:
|Other states (5) (show)
|Assistive Technology Equipment
|Bone Densitometry/DEXA
|Developmental - Behavioral Pediatrics
|Disability Awareness Programs
|Disability Related Employment Programs
|Early Intervention for Children with Disabilities/Delays
|General Counseling Services
|General Dentistry
|Hospice & Palliative Care
|Neuromuscular Clinics
|Nutrition Assessment Services
|Occupational Therapy
|Pediatric Cardiology
|Pediatric Dentistry
|Pediatric Endocrinology
|Pediatric Genetics
|Pediatric Neurology
|Pediatric Ophthalmology
|Pediatric Orthopedics
|Pediatric Physical Medicine & Rehabilitation
|Pediatric Pulmonology
|Physical Therapy
|Social Workers
|Speech - Language Pathologists
|Student Disability Services
|Supportive Housing & Residential Care Homes
|Wish Foundations
For services not listed above, browse our Services categories or search our database.
* number of provider listings may vary by how states categorize services, whether providers are listed by organization or individual, how services are organized in the state, and other factors; Nationwide (NW) providers are generally limited to web-based services, provider locator services, and organizations that serve children from across the nation.
Anderson JL, Head SI, Rae C, Morley JW.
Brain function in Duchenne muscular dystrophy.
Brain. 2002;125(Pt 1):4-13. PubMed abstract
Bach JR.
Respiratory muscle aids for the prevention of pulmonary morbidity and mortality.
Semin Neurol. 1995;15(1):72-83. PubMed abstract
Bakker E, Veenema H, Den Dunnen JT, van Broeckhoven C, Grootscholten PM, Bonten EJ, van Ommen GJ, Pearson PL.
Germinal mosaicism increases the recurrence risk for 'new' Duchenne muscular dystrophy mutations.
J Med Genet. 1989;26(9):553-9. PubMed abstract / Full Text
Balaban B, Matthews DJ, Clayton GH, Carry T.
Corticosteroid treatment and functional improvement in Duchenne muscular dystrophy: long-term effect.
Am J Phys Med Rehabil. 2005;84(11):843-50. PubMed abstract
Birnkrant DJ, Bushby KM, Amin RS, Bach JR, Benditt JO, Eagle M, Finder JD, Kalra MS, Kissel JT, Koumbourlis AC, Kravitz RM.
Respiratory management of patients with DMD: A DMD care considerations working group specialty article.
Pediatr Pulmonol. 2010. PubMed abstract
Birnkrant DJ, Panitch HB, Benditt JO, Boitano LJ, Carter ER, Cwik VA, Finder JD, Iannaccone ST, Jacobson LE, Kohn GL, Motoyama
EK, Moxley RT, Schroth MK, Sharma GD, Sussman MD.
American College of Chest Physicians consensus statement on the respiratory and related management of patients with Duchenne muscular dystrophy undergoing anesthesia or sedation.
Chest. 2007;132(6):1977-86. PubMed abstract
Bushby K, Bourke J, Bullock R, Eagle M, Gibson M, and Quinby J.
The multidisciplinary management of Duchenne muscular dystrophy.
Current Paediatrics. 2005;15:292-300. / Full Text
Ciafaloni E, Fox DJ, Pandya S, Westfield CP, Puzhankara S, Romitti PA, Mathews KD, Miller TM, Matthews DJ, Miller LA, Cunniff
C, Druschel CM, Moxley RT.
Delayed diagnosis in DMD: data from the Muscular Dystrophy Surveillance, Tracking, and Research Network (MD STARnet).
J Pediatr. 2009;155(3):380-5. PubMed abstract
Cotton SM, Voudouris NJ, Greenwood KM.
Association between intellectual functioning and age in children and young adults with Duchenne muscular dystrophy: further results from a meta-analysis.
Dev Med Child Neurol. 2005;47(4):257-65. PubMed abstract
Darras, BT, Korf, BR, and Urion, DK.
GeneReviews (NIH); (2008) http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=gene&part=dbmd.
Finder JD,Birnkrant D,Carl J,Farber HJ,Gozal D,Iannaccone ST,Kovesi T,Kravitz RM,Panitch H,Schramm C,Schroth M,Scharma G,Sievers
L,Silvestri J,Sterni L.
Respiratory Care of the Patient with Duchenne Muscular Dystrophy.
The official statement of the American Thoracic Society; (2004) http://www.parentprojectmd.org/site/DocServer/filename?docID=123.
This statement is designed to educate the practitioner about new approaches to therapies available for the management of the respiratory complications of DMD. In addition to respiratory care, it covers sleep evaluation, nutrition, cardiac involvement, scoliosis and corticosteriods. It also discusses end-of-life care and the decision to use or not use a respirator.
Flanigan KM, Dunn DM, von Niederhausern A, Soltanzadeh P, Gappmaier E, Howard MT, Sampson JB, Mendell JR, Wall C, King WM,
Pestronk A, Florence JM, Connolly AM, Mathews KD, Stephan CM, Laubenthal KS, Wong BL, Morehart PJ, Meyer A, Finkel RS, Bonnemann
CG, Medne L, Day JW, Dalton JC, Margolis MK, Hinton VJ, Weiss RB.
Mutational spectrum of DMD mutations in dystrophinopathy patients: application of modern diagnostic techniques to a large cohort.
Hum Mutat. 2009;30(12):1657-66. PubMed abstract
Gibson B.
Long-term ventilation for patients with Duchenne muscular dystrophy : physicians' beliefs and practices.
Chest. 2001;119(3):940-6. PubMed abstract
Hayes J, Veyckemans F, Bissonnette B.
Duchenne muscular dystrophy: an old anesthesia problem revisited.
Paediatr Anaesth. 2008;18(2):100-6. PubMed abstract
Helderman-van den Enden AT, de Jong R, den Dunnen JT, Houwing-Duistermaat JJ, Kneppers AL, Ginjaar HB, Breuning MH, Bakker
Recurrence risk due to germ line mosaicism: Duchenne and Becker muscular dystrophy.
Clin Genet. 2009;75(5):465-72. PubMed abstract
Hinton VJ, De Vivo DC, Nereo NE, Goldstein E, Stern Y.
Selective deficits in verbal working memory associated with a known genetic etiology: neuropsychological profile of DMD.
J Int Neuropsychol Soc. 2001;7(1):45-54. PubMed abstract / Full Text
Houde S, Filiatrault M, Fournier A, Dubé J, D'Arcy S, Bérubé D, Brousseau Y, Lapierre G, Vanasse M.
Deflazacort use in Duchenne muscular dystrophy: an 8-year follow-up.
Pediatr Neurol. 2008;38(3):200-6. PubMed abstract
Ishikawa Y, Bach JR.
Duchenne muscular dystrophy.
Thorax. 1999;54(6):564. PubMed abstract
Mah JK, Korngut L, Dykeman J, Day L, Pringsheim T, Jette N.
A systematic review and meta-analysis on the epidemiology of Duchenne and Becker muscular dystrophy.
Neuromuscul Disord. 2014;24(6):482-91. PubMed abstract
Manzur AY, Kuntzer T, Pike M, Swan A.
Glucocorticoid corticosteroids for Duchenne muscular dystrophy.
Cochrane Database Syst Rev. 2008(1):CD003725. PubMed abstract
Markham LW, Kinnett K, Wong BL, Woodrow Benson D, Cripe LH.
Corticosteroid treatment retards development of ventricular dysfunction in Duchenne muscular dystrophy.
Neuromuscul Disord. 2008;18(5):365-70. PubMed abstract
Mendell JR, Lloyd-Puryear M.
Report of MDA muscle disease symposium on newborn screening for Duchenne muscular dystrophy.
Muscle Nerve. 2013;48(1):21-6. PubMed abstract
Merlini L, Cicognani A, Malaspina E, Gennari M, Gnudi S, Talim B, Franzoni E.
Early prednisone treatment in Duchenne muscular dystrophy.
Muscle Nerve. 2003;27(2):222-7. PubMed abstract
Moxley RT 3rd, Ashwal S, Pandya S, Connolly A, Florence J, Mathews K, Baumbach L, McDonald C, Sussman M, Wade C.
Practice parameter: corticosteroid treatment of Duchenne dystrophy: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society.
Neurology. 2005;64(1):13-20. PubMed abstract
Quinlivan R, Roper H, Davie M, Shaw NJ, McDonagh J, Bushby K.
Report of a Muscular Dystrophy Campaign funded workshop Birmingham, UK, January 16th 2004. Osteoporosis in Duchenne muscular dystrophy; its prevalence, treatment and prevention.
Neuromuscul Disord. 2005;15(1):72-9. PubMed abstract
Romitti PA, Zhu Y, Puzhankara S, James KA, Nabukera SK, Zamba GK, Ciafaloni E, Cunniff C, Druschel CM, Mathews KD, Matthews
DJ, Meaney FJ, Andrews JG, Conway KM, Fox DJ, Street N, Adams MM, Bolen J.
Prevalence of Duchenne and Becker muscular dystrophies in the United States.
Pediatrics. 2015;135(3):513-21. PubMed abstract / Full Text
Schram G, Fournier A, Leduc H, Dahdah N, Therien J, Vanasse M, Khairy P.
All-cause mortality and cardiovascular outcomes with prophylactic steroid therapy in Duchenne muscular dystrophy.
J Am Coll Cardiol. 2013;61(9):948-54. PubMed abstract
Simonds AK, Muntoni F, Heather S, Fielding S.
Impact of nasal ventilation on survival in hypercapnic Duchenne muscular dystrophy.
Thorax. 1998;53(11):949-52. PubMed abstract / Full Text
Snow WM, Anderson JE, Jakobson LS.
Neuropsychological and neurobehavioral functioning in Duchenne muscular dystrophy: a review.
Neurosci Biobehav Rev. 2013;37(5):743-52. PubMed abstract
Soltanzadeh P, Friez MJ, Dunn D, von Niederhausern A, Gurvich OL, Swoboda KJ, Sampson JB, Pestronk A, Connolly AM, Florence
JM, Finkel RS, Bönnemann CG, Medne L, Mendell JR, Mathews KD, Wong BL, Sussman MD, Zonana J, Kovak K, Gospe SM Jr, Gappmaier
E, Taylor LE, Howard MT, Weiss RB, Flanigan KM.
Clinical and genetic characterization of manifesting carriers of DMD mutations.
Neuromuscul Disord. 2010;20(8):499-504. PubMed abstract / Full Text
Willig TN, Carlier L, Legrand M, Riviere H, Navarro J.
Nutritional assessment in Duchenne muscular dystrophy.
Dev Med Child Neurol. 1993;35(12):1074-82. PubMed abstract


text:
Spotting unsound science in Pakistan
Astonishing science claims abound in the media. A self-taught engineer in Swat claims he can "fix Pakistan's energy problem in three years" by splitting water to produce electricity.
In 2011, a Fellow of the Royal Society from Pakistan published an article saying that the High Frequency Active Auroral Research Programme experiment in Alaska caused floods and earthquakes in Pakistan.
A recent PhD thesis, titled 'A quantitative study on chromotherapy', guided by a 'meritorious professor' from the Higher Education Commission (HEC) and publication paid for by the HEC, elicited criticism from two Nobel prize winners for showing lack of understanding of the fundamentals of physics
Two top Pakistani scientists, Samar Mubarakmand and A. Q. Khan, neither of who is a mining engineer, meanwhile, have locked horns on whether coal from the Thar desert can be gasified underground in a commercially viable way
Mubarakmand believes the gasification project will turn Pakistan into a coal superpower.
But Khan says But Khan says the project is doomed to fail, and advses giving the Thar coal project to Shenhua Group of China.
This controversy cannot be resolved without transparency, data, and an independent evaluation by experts familiar with mining, chemistry, soil mechanics, and hydrology. Because none exist in Pakistan, perforce such experts should be sought in Australia, China, or elsewhere.
Common sense says that a detailed, independent feasibility plan is the very first step in a mega project.
Link to full article in the Express Tribune

subset: commoncrawl


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by Charles Poliquin Iron Man Magazine
Q; I'm a personal trainer, and I get a lot of resistance from potential female clients who think that increasing their muscle mass will have no real benefits. How can I answer them?
A: Increasing your muscle and bone mass offers myriad health benefits. The very extreme muscular women you may have seen have had hormone "support" to build their overblown muscles—better known as "East German acupuncture."
Here's what you should tell those who frown on gaining a few kilos of muscle mass:
1) The more muscle and bone mass you have, the greater the acid-buffering power you create; that is, you now have more protein, potassium, magnesium and calcium to buffer the acidity in your body. The more alkaline you are, the greater the endurance potential of your body and the greater your immune power, thus the better your chances of surviving cancer or the swine flu.
2) According to research out of Tufts University, the greater your muscle mass, the greater your longevity potential. It is, in fact, the number-one biomarker of longevity, a far better predictor than total cholesterol or blood pressure.
3) The more muscle you have, the more insulin receptor sites you have, and the more sensitive they are. Increased muscle prevents diabetes and metabolic syndrome. Translation: The more muscle you have, the easier it is to have low bodyfat.
4) The more muscle you have, the more you burn calories at rest. It's estimated that for every pound of muscle you gain, you burn an extra 50 calories a day.
5) The more muscle you have, the more strength you have, which is the number-two predictor of longevity, according to the same researchers from Tufts University. Strength is always empowering. You never know when that new level of strength may come in handy.
6) In my 30 years of experience as a strength coach, for every pound of lean tissue gained, there was an equal amount of bodyfat loss. In other words, the body composition changed dramatically. For example, a female executive weighing in at 132 pounds with 20 percent bodyfat has about 25 pounds of fat. So if she gains about eight pounds of muscle mass and loses eight pounds of fat at the same time in 10 weeks, her bodyfat will be 13 percent at the end of the 10 weeks. Her body will look tremendously better.
Source: http://www.ironmanmagazine.com/6-ben…ing-for-women/


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Why do some readers read the ayat... qul huwallahu ahadunillahussamad? He continues reading instead of pausing on it...
Why do some readers read the ayat... qul huwallahu ahadunillahussamad? He continues reading instead of pausing it(ahad)...Is there any name for this rules in tajweed? The same thing with ayat... lumazatinillalladhee...he continues reading instead of pausing..(lumaza )....
It is sunnah to stop at the end of an aayah and take a breath before reading the next aayah, but it is not forbidden to join two aayaat together. When we do that, we must pronounce the vowel on the end of the first aayah and join it with the first pronounced letter of the next sequential aayah.
In the two aayaat exampled in the question there are tanweens at the end of each of both aayaat followed by a sukoon as the first pronounced letter of the next aayah. In Arabic there is a forbiddance of two saakin letters meeting between two words, and the tanween has a noon saakinah as the last pronounced part of it.
When there are two saakin letters meeting, the first letter changes so that there will no longer be two saakin letters meeting. In the case of the tanween for the recitation of Hafs, the noon saakinah of the tanween will always acquire a kasrah. This is why you hear a kasrah on the noon saakinah of the tanween in both aayaat in the question.
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Daniel Borenstein of the Contra Costa Times, a leading voice on state and local pension problems in California, doesn't think very much of Gov. Jerry Brown's proposals, which had received a lukewarm to positive reception when they were unveiled last week.
Borenstein's main beef: even Brown's worthy reforms would apply only to new hires, rather than current hires.


text:
George M. Marsden
The best man at my parents' wedding on June 25, 1935, was Harold J. Ockenga. Carl McIntire officiated. They and my father, Robert S. Marsden (1905-1960), had left Princeton Theological Seminary with J. Gresham Machen in 1929 to be in the first class at Westminster Theological Seminary. Machen himself had been asked to officiate, but could not, because he had been suspended from the ministry of the Presbyterian Church in the U.S.A.
Due to that crisis, his three protégés, then ministers in that denomination, would soon go their separate ways. My mother, Bertha Mish Marsden, later recalled that they argued church politics as they were waiting for the wedding to begin.
The wedding picture on the lawn of my grandmother's spacious home in Middletown, Pennsylvania, juxtaposes two worlds that I would be born into four years later. The one world was shaped by the intense theological concerns of the old Princeton tradition that was about to fragment into several pieces. Ockenga would remain in mainline churches and lead the "new evangelical" movement associated with Billy Graham. McIntire and my father would leave the Presbyterian Church in the U.S.A. in 1936, but after Machen's death at the beginning of 1937, McIntire would found his own Bible Presbyterian Church and later become widely known as a fundamentalist radio preacher and organizer. My father would remain loyal to Machen and the OPC and soon become the tiny church's general secretary for Home Missions, a position he held until 1948, when he became executive secretary for Westminster Seminary.
The other world represented in the picture is that of conventional small-town, post-Victorian America. My father, who had worked hard to make good, came to Middletown to pastor its Presbyterian church in 1930. A year after having married the daughter of one of the town's respected families in 1935, he did something almost unthinkable in polite society. He split the staid Presbyterian church, leading a group of mostly younger or poorer members to form what would become the local Orthodox Presbyterian church.
Since our family always lived with my grandmother in our large old home, even when my father commuted a couple of days a week to his jobs in Philadelphia, there was no escaping the contrasts between these two worlds. In the OPC, we were set apart and stood against the modernism of the mainline churches and, by implication, the townspeople who attended them. Yet my grandmother, who was a model of gracious goodwill, while a member of the OP church, maintained her other social ties.
My father was the dominant figure in the home and in shaping our religious training, even if my mother and grandmother taught by modest, loving example. Dad took over whenever he entered a scene. He was that way outside the home as well. He exuded self-confidence and was quick to let people know that he knew more on a subject than they did. He was a whiz with numbers and could awe supermarket checkout clerks by announcing the total of the bill before they rang it up. He was also the model of the educated clergyman. He read avidly and subscribed to the Book-of-the-Month Club on the grounds that there were too many preachers who knew a lot about theology, but not much about life. He was a fine preacher himself, with a dramatic flair for oratory. He was not known to be self-effacing. Privately he claimed that his super-confident demeanor was a cover for his inferiority complex. If that was so, he put on a good front. He once announced that he could name "the ten best preachers in the OPC." Mary Carson Kuschke, who was as smart and quick as he was, retorted, "So, Bob, tell us who the other nine are."
This sense of confidence carried over into religious training. Growing up OP meant learning from an early age that we were correct in doctrine, based on solid biblical and intellectual grounds. We were not like the modernists, who had both abandoned the authority of the Bible and shaped their theology by modern fads. We were also not like the more revivalistic fundamentalists, who substituted enthusiasm for substance. Correct doctrine was, of course, to be accompanied by genuine piety. In my father's case, that probably showed through best in his love for the great hymns of the faith, a love that he shared with others through untold hours of work as the chairman of the committee that produced Trinity Hymnal.
Our family's sense of being set apart against the rest of our town was reinforced by the founding, under my father's leadership, of the Middletown Christian Day School in 1944, the year I entered first grade. The Rev. Edward Kellogg, our pastor at that time, was cofounder. He was a descendant of Wheaton College founder Jonathan Blanchard, and he and his wife Eleanor were Wheaton graduates. They added a strong evangelical conversionist sensibility to the somewhat more intellectualized Old School Princetonian tone that my father cultivated. Even as a young child, I was deeply impressed by the fervor and energy of Mr. Kellogg's preaching, which was the more effective because of personal warmth.
The golden age for Calvary OPC in Middletown was right after World War II. The great practical problem for the fledgling denomination was that it did not have a social base. As in Middletown, most families that were simply loyal to Presbyterianism remained in the old church, so that OP members had to be recruited from somewhere else. In postwar America, these recruits were to be found among younger people who had strongly Protestant, but not necessarily Presbyterian, backgrounds, and who had recently moved and hence were open to change. Postwar Middletown was next to a U.S. Air Force maintenance base that hired many young men, who moved with their families to housing projects and suburbs. Vigorous recruiting brought in a core of families who contributed much to the church during the next decade.
Although Ed Kellogg began this work, it was effectively carried on from 1947 to 1952 by the Rev. Robert Atwell, who was a very different type of person. If Kellogg represented the warm evangelical side of the Presbyterian heritage, and my father represented the Princeton Old School, Atwell was a product of a more rugged Western Pennsylvania, Scotch-Irish, no-compromise piety that was used to working on rocky soil. Theologically, these men all agreed on the necessity of orthodoxy plus heart commitment. But they went about it in different ways. Atwell was a disciplinarian and had a short fuse, and so most of us children tended to lie low when he was in the vicinity. Nonetheless, his intense passion could be effective, and it included a passion for the souls of young people.
From the point of view of a boy growing up in this environment, the church's ministry to young people seemed most effective when it moved out of town to summer Bible conferences. One problem during the other fifty-one weeks of the year was that the proclaimed doctrines of grace often seemed to be obscured by the strictures of the law. With more or less the same people in charge of discipline in the home, the church, and the school, kids learned how to live in alternative universes. Neighborhood friends, sports, games, and the radio (with its American entertainment and commercial values) all competed for our loyaltiesand that list does not include intentionally subversive institutions, such as our "Luckies Club." I report this as someone who was more positively responsive to the church training than were most of my peers. At camp, by contrast, there was a more controlled environment that mixed teaching, sports, and fun with a quest for commitment in a way that rarely happened during the rest of the year.
Robert Atwell has to be given a lot of credit for establishing, along with Louis Grotenhuis, the French Creek Bible Conferences, which began in 1950 and became legendary among many generations of OP young people. There a lot of pastors and laypeople spent much time and energy that displayed their love and concern for the young. Perhaps it helped that most of them were not the people who disciplined and catechized us the rest of the year. They could also step out of their formal roles, get a hearing for the gospel teachings, and simply demonstrate how much they cared. These qualities were surely there the rest of the year, not only from pastors, but also from caring teachers and many other wonderful laypeople. These people surely created the loving foundation that was the basis for any effective ministry. Nonetheless, for me it was in the camp environment that this combination of factors emerged with greatest clarity.
God's grace works through many unpredictable means. One lesson of parenting (and in ministry also) is that there is not a one-to-one correlation between what one intends and how a child responds. We receive our spiritual gifts through earthen vessels that have strengths and weaknesses. In my case, I can see the wonder of God's covenantal work coming through these imperfect circumstances, and I praise God's grace and mercy alone for it. There was certainly nothing that I did to deserve it.
The author, a member of the Christian Reformed Church, teaches history at the University of Notre Dame. Reprinted from New Horizons, June 2006.
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Surging Grizzlies top Clippers for 14th win in 15 games
- The Grizzlies had five score in double figures for their sixth straight win and 14th in 15 games
- Memphis improved to 15-4 since trading star Rudy Gay to the Toronto Raptors on Jan. 30
- Beset by minor injuries recently%2C the Clippers lost for the third time in their last five games
LOS ANGELES (AP) — Exactly six weeks after the Memphis Grizzlies traded their leading scorer for spare parts in what was widely seen as a cost-cutting move, they dominated the Pacific Division-leading Los Angeles Clippers for their 14th victory in 15 games.
So what's got into the Grizz left behind by Rudy Gay?
They say it's nothing fancy: Just hard work, determination and that signature Memphis toughness.
Marc Gasol scored 21 points, Tayshaun Prince added 18 and the surging Grizzlies beat the Clippers 96-85 on Wednesday night for their sixth straight win.
Mike Conley had 17 points and 11 assists, and Zach Randolph scored 13 points as Memphis hung on in the fourth quarter of back-to-back road victories. Shutting down the Clippers' lob game and scoring with their own unflashy offense, the Grizzlies followed up last night's win in Portland with a tenacious effort against the Clippers, who have lost three of five.
It might have been a statement victory, but nobody was making a statement in the upbeat visitors' locker room.
"We don't get caught up in the gravity of the situation too much," Conley said. "We just go out there as a hardworking team every night. This is a huge win, obviously, second game of a back-to-back against a good team. We just did a good job defensively, and playing at our pace."
The Grizzlies realize they're on a 15-4 surge since trading Gay to Toronto on Jan. 30, but they're not getting too excited about their achievements in his absence — although they took particular satisfaction in beating the Clippers, who eliminated them from the first round of the postseason last spring and beat the Grizzlies in their first two meetings this year.
"We've seen this team before, and we have some good battles against this team," Gasol said. "It feels good to get one against them."
What's more, the Grizzlies are excelling during a particularly brutal stretch in their schedule with help from newcomer Prince, the Los Angeles native acquired in the deal for Gay. They're late in a five-week stretch in which they never play consecutive games in the same city, with 10 of 14 games on the road.
Memphis (44-19) is percentage points ahead of Los Angeles (45-21) for third place in the Western Conference, although the Clippers are division leaders.
"We just keep playing good basketball," Prince said. "It's very surprising I'm able to build that bond with them so quickly, without having a training camp. We trust each other, and that's the most important thing."
Chris Paul had 24 points and nine assists, and Blake Griffin added 22 points for the Clippers, who won the clubs' first two meetings this season.
Matt Barnes scored 14 points as a fill-in starter for Los Angeles in a physical meeting of two Western Conference contenders whose bruising seven-game playoff series is still fresh in most players' minds.
"They're pretty physical," Paul said. "We obviously saw that in seven games last year and we were able to adjust to it, but we've got to figure some things out. ... I could care less what (playoff) seed we are. We just need to be playing the right way when the time comes, and we're not there yet."
One game after scoring a season-high 129 points, the Clippers struggled offensively, including a 6-for-25 effort on three-pointers while scoring just 33 points after halftime. Although the Clippers seem capable of coasting to the first division title in the long-suffering franchise's history, the Pacific leaders might be in danger of entering another slump in their mostly stellar season.
"We want to be playing better," Griffin said. "Concern, I don't know if that's the right word. Frustration, yeah. We're not scared of any team we have to face in the playoffs, but we do need to be executing better to be able to impose our will and our game on other teams. ... It's disappointing we didn't play as well as we should have, but this game meant more to them, and that's the way they played. They played hungrier."
The Clippers' impressive depth has been thinned by minor injuries recently. Los Angeles played without starting forward Caron Butler, who's out with a strained left elbow, and backup point guard Eric Bledsoe, who has a strained calf. Although Jamal Crawford returned from a two-game absence with a sore left ankle, he scored just two points against Memphis on 1-for-10 shooting.
Memphis jumped to an 11-point lead in the third quarter with a rally led by Gasol, who had 11 points and three steals in the period. Los Angeles got within three points with 7 minutes to play, but Quincy Pondexter's three-pointer kick-started Memphis' final rally.
NOTES: After a day off, Memphis finishes its four-game trip in Utah and Denver. ... Along with his playmaking prowess, Paul scored his 10,000th career point on a jumper early in the second quarter, getting a standing ovation from the sellout crowd when the milestone was announced. But the All-Star point guard managed just six points in the second half. ... Unbeaten WBO welterweight champion Timothy Bradley watched the game in a Clippers jersey. The Palm Springs-area native defends his belt Saturday night in nearby Carson against Ruslan Provodnikov.


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My daughter has a stuffy nose. Every. Minute. Of. Every. Day. Sometimes she is even lucky enough to have some combination of stuffy and runny. And, last year, for a girl in the first grade, that meant she was up getting a tissue every 10 minutes. I kid you not. Her teacher even questioned me on it. Well, crap, of course I know about it! We are trying to figure out how to fix it. If she would only let me do a sinus rinse! I've been doing them on myself for years and have sold so many of my friends and family on it, I should make a commission. Alas, asking a little girl to squeeze water into one nostril in order to make "boogies" shoot out the other nostril was an exceptionally hard sell.
So we dealt with the recurring sinus infections, the red nose, the lack of smell, or the ability to chew with her mouth closed — because she literally couldn't breathe!
I took her to our pediatrician and our pediatric ENT and both suggested allergy medicine. Yep, I'd tried that ... doesn't seem to help. So the ENT put a scope up her nose (she got a nice toy after that visit) in order to get a good look at her tonsils and adenoids. Her tonsils were fine but her adenoids were enlarged, though only slightly. We tried a different allergy medicine and a nose spray. I hate nose sprays but took the mild one he gave us out of desperation.
Back at the ENT two weeks later, her stuffy nose seemed to get worse and her adenoids got bigger.
We scheduled surgery. The adenoids came out. It was a simple procedure, but seeing your child in a hospital gown is never something you would elect to do if you felt like you had another option. Six full weeks after surgery, we went back to the ENT and my girl could breathe! And she could smell! "Holy cow," I thought. "Success!"
Nope. Not so fast. The next effing day. The next day ... her nose went right back to where it had been before the surgery.
So that day I gave my 7-year-old a choice. She's always been good when given two options, even if she doesn't particularly like either one, she just likes knowing she's the one deciding. Hmmm ... that doesn't sound like anyone I know.
Her choice was this: Try a new allergy medicine for a week or do the dreaded sinus rinse.
You see, even though I thought the sinus rinse might help her, I simply couldn't force her to put a squeeze bottle to her nostril. It isn't the kind of treatment I could (or would want to) force upon someone. They have to be a willing participant. And this time she bought what I was selling. It helped that there is nothing she despises more than taking medicine.
The nasal rinse won!
When I finally squeezed that bottle of water up her left nostril, what shot out of the right nostril could possibly have been entered in the Guinness Book of World Records, if they recorded such things. Seriously, the amount of "boogies" that fell into the sink were unreal and couldn't have possibly come out of her small head. She was grossed out. I was secretly grossed out (but ecstatic!). And then it happened ... she took a deep breath in ... through her nose. Since that day in April we have done the sinus rinse well over 100 times, using it whenever she starts to get stuffy — and amazingly, not one sinus infection!
In fact, she now tells me when she needs it! It is a fantastic feeling to go from being completely stuffed to being clear — all in a matter of 60 seconds.
Mommy guilt did set in: Why didn't I push her more to try the rinse? Maybe she didn't need the the surgery after all? Could we have avoided many of those bottles of medicine we needed over the years?
But I try not to think about it too much because I can't change the past, and it felt better to direct some disappointment at my doctors who, while being wonderful and supportive, never once pushed me to try the rinse. Which turned out to be an amazing natural alternative to everything they threw my way.
In the meantime, I found this video of an adorable 5-year-old girl using the same sinus rinse squeeze bottle my daughter uses. I wish I'd found this a year ago!
Have you ever tried a sinus rinse, on either you or your child?
Image via YouTube


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Amal and George Clooney enjoyed a romantic evening out on Saturday night in Porto San Paolo in Sardinia.
Amal Clooney wore a white and black dress with laced-up pumps.
Style Files
Amal Clooney wore
An MOSCHINO dress from the fall 2017 collection designed by Jeremy Scott with trash theme
With ALTUZZARA pumps


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Local government officials aren't the only ones who have to worry about meeting the stormwater pollution reduction targets set by the Environmental Protection Agency as part of its Chespaeake Bay pollution diet. The State Highway Administration also has a massive infrastructure of outmoded stormwater management systems that need upgrading, not to mention thousands of lane-miles of impervious surfaces that contribute to the flow of polluted water into the bay. Acheiving that is going to cost hundreds of millions of dollars during the next five years.
The General Assembly was conscious of that fact when it enacted a major transportation funding bill last year. It required the governor to include either capital or operating funds in the budget for the next five years to cover an anticipated $395 million in costs for the SHA's Watershed Implementation Plan. But lawmakers were also cognizant of the fact that the gas tax increase they were passing as part of that bill was hugely unpopular and would be more so if the public thought any part of the money was being diverted to a purpose other than increasing the capacity of the state's highways and mass transit systems. Legislators decided the governor would have to find some way besides the new gas tax revenues to pay for the watershed improvements.
Gov. Martin O'Malley opted for the simplest solution. His administration proposed that the state increase its self-imposed borrowing limit by $75 million a year for the next five years to cover most of those costs. The plan was approved by the state's Capital Debt Affordability Committee 4-1, with Comptroller Peter Franchot the lone dissenting vote. Now it's up to members of the legislature to decide whether to accept the committee's recommendation, and their chief budget analyst is reccommending that they say no out of concern for the state's rising debt service payments and uncertain fiscal future.
The question is not completely clear-cut. The state has to find the money somewhere, and it's not exactly flush with cash. Funding the watershed improvements within existing resources would mean cutting programs or eliminating capital spending for things like schools and other infrastructure projects that would help shore up a weak economy. And raising taxes again is (and should be) a political non-starter. Meanwhile, increasing the debt ceiling by $375 million over the next five years is not expected to cause the state to run afoul of its self-imposed debt limit tests — that debt not exceed 4 percent of total state personal income and that debt service payments not exceed 8 percent of government revenues.
On the other hand, the level of state debt has increased markedly under the O'Malley administration, and with it, debt service payments are projected to take up a big chunk of his successor's budgets. Part of the reason for the increase in debt is the governor's tactic of using bonds to pay for Program Open Space and diverting the transfer tax revenues that are supposed to fund it into covering operating expenses. This shell game is legal but not ideal, and it is projected to continue into the forseeable future, albeit at a lower level than during the worst years of the recession.
The upshot is that debt service payments are expected to be, by far, the state's fastest growing expenditure during the next five years. State property taxes are supposed to cover debt service, but because of the growth in borrowing and the weak real estate market, they will fall short. The Board of Public Works could increase the property tax rate to make up the difference, but that is both unlikely and undesireable. Instead, the state is expected to have to start kicking in general funds to make up the difference, starting with a nominal amount this fiscal year but growing by an average of more than 24 percent annually until the payments reach $557 million in fiscal 2019, the last year of the next governor's term.
The $375 million in borrowing at issue now would contribute only a small portion of that — $43 million annually when it is fully phased in — but there is good reason at the moment to approach the matter with caution. Governor O'Malley estimated the cost of the government shutdown at $5 million a day (Mr. Franchot's office will have more precise numbers in December), and that came on top of the effects of sequestration. Given the continued uncertainty around the federal budget, Maryland needs to be prepared for more fiscal shocks in the future. In addition, the state is due for another study on the adequacy of education funding — a follow-up to the Thornton Commission's work of a decade ago — and the chances that it will conclude that everything is rosy are slim. Finally, all of the candidates for governor are making proposals that will significantly affect the bottom line through new spending, tax cuts or both.
If the legislature accepts the committee's recommendation now, it could change course later if circumstances warrant, but it would be better to approach this and all other fiscal decisions in the months ahead with due caution. Lawmakers should defer the decision about adding to the state's debt limit until all those uncertainties become clearer. The cost of the state's Watershed Implementation Plan in the next fiscal year is $45 million. The General Assembly should find ways to absorb that cost within existing resources and leave the decision on whether to cover the remaining tab through additional debt for the new governor and legislators who will take office in 2015.
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Downstream from Unidentified Bridge,
Dimensions (Image): 160 x 234 mm (6.3 x 9.2 in.)
Inscribed on protective tissue: title
Accession number: 1918.104.22.168
Gift of Michael M. Senft, 1983
Bibliography: J. Geddie, The Royal Palaces, Historic Castles and Stately Homes of Great Britain (Charlecote by James Valentine); note (223) (Falcon Tavern).
Work Type: photograph
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For Couples
Eva's Boutique offers a wide variety of toys for you and your partner to choose from. So if you want to heighten playtime there are plenty of options designed specifically for couples to ensure that you both experience maximum please. So embark on a journey of shared sexual discovery.


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Rats could grow to the size of sheep or even bigger as they evolve to fill vacant ecological niches, it is claimed.
The terrifying scenario could become a reality as super-adaptable rats take advantage of larger mammals becoming extinct, an expert predicts.
"Animals will evolve, over time, into whatever designs will enable them to survive and to produce offspring," said geologist Dr Jan Zalasiewicz, from the University of Leicester.
"For instance, in the Cretaceous Period, when the dinosaurs lived, there were mammals, but these were very small, rat and mouse-sized, because dinosaurs occupied the larger ecological niches.
"Only once the dinosaurs were out of the way did these tiny mammals evolve into many different forms, including some very large and impressive ones: brontotheriums, horses, mastodons, mammoths, rhinoceri and more.
"Given enough time, rats could probably grow to be at least as large as the capybara, the world's largest rodent, that lives today, that can reach 80 kilos (176 pounds).
"If the ecospace was sufficiently empty, then they could get larger still."
The largest extinct rodent known, Josephoartegasia monesi, which lived three million years ago, was larger than a bull and weighed over a ton.
Like its modern-day relative, the sheep-sized capybara, it lived in South America.
A hint of the nightmare to come can be seen on "rat islands" - isolated regions where rats introduced by humans have quickly risen to become the dominant species.
Rat islands act as a "laboratory" for studying the future evolution of the rodents, according to Dr Zalasiewicz.
"Rats are one of the best examples of a species that we have helped spread around the world, and that have successfully adapted to many of the new environments that they found themselves in," he said.
"They are now on many, if not most, islands around the world - and once there, have proved extraordinarily hard to eradicate. They're often there for good, essentially. Once there, they have out-competed many native species and at times have driven them to extinction.
"As a result, ecospace is being emptied, and rats are in a good position to re-fill a significant chunk of it, in the mid to far geological future."
Gigantism is a well known evolutionary response that occurs when a small creature steps into an ecological niche left by a larger species.
Fifty million years ago, a distant ancestor of the blue whale was the size of a wolf, Dr Zalasiewicz pointed out.
He expected rats to adapt in a host of other ways, besides some of them growing to a large size.
"Animals can evolve to smaller as well as larger sizes," he said. "This will depend on what particular circumstances they find themselves in and what the selective pressures on them are.
"Each island that rats are now present on is in effect a laboratory of future evolution, and each will produce different results.
"So there will be future thin rats, future fat rats, slow and heavy rats, fast and ferocious rats, probably future aquatic rats - the list goes on. Other animals will likely follow the same pattern, such as domestic cats, rabbits, goats and more."
He suspected that rats will have a major influence on the geological future of the Earth and over time were likely to produce "some remarkable descendants".


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Utilities Management
facilities services
The Boston College Utilities Management group has direct responsibility for the University's utility budget, utility consumption, and utility conservation programs. The group supports the management of the University's Energy Management Control System (EMCS), develops utility budgets for all of BC's properties, and oversees payments of utility bills.
Utility Conservation Programs
Fuel Oils
Boston College has converted the heating plant that serves Claver, Loyola, and Xavier Residence Halls with state-of-the-art dual-fuel boilers. This conversion directly reduces the amount of emissions to the environment by decreasing fuel consumption and allowing the use of cleaner-burning natural gas.
Other heating plants, such as the one located in St. Mary's Hall, have been upgraded and emissions lowered through the use of low-sulfur fuel oil.
Ongoing preventive maintenance efforts to steam distribution infrastructure, such as a steam trap program, save fuel oil and reduce emissions.
BC strives to reduce electric use to save dollars and, in turn, to reduce the amount of emissions the electric utility companies emit from their generating plants. Electric conservation has taken place at BC in the following areas:
- Retrofitting buildings with efficient lighting: Stuart Hall, 66 Commonwealth Ave., McElroy, the Bookstore, Lower Campus Dining Facilities, the Central Heating Plant, and Edmonds Hall have been recently retrofitted, saving BC over 400,000 kilowatt hours of electricity per year.
- Installing high-efficiency motors to run building equipment
- Controlling efficient motor operation with variable-frequency drives
- Implementing a computerized energy management system to operate the heating, ventilation, and air conditioning in buildings
- Installing motion sensors to turn lights off when areas are not being occupied
- Participating in Utility-Sponsored Demand Side Management and Response Programs
Natural Gas
The Central Heating Plant at Boston College provides steam to the majority of buildings on the Main Campus. The Plant has the capability to use #6 oil or natural gas. Knowing that natural gas is an environmentally cleaner burning fuel, the Plant uses natural gas from March through November.
Water & Sewer
Water conservation is important to guarantee adequate supplies for future needs. In addition, water and sewer costs have risen over 500% in the last 10 years and are continuing to rise. To address these challenges, Boston College has taken the following water conservation measures:
- Installation of low-flow toilets
- Installation of low-flow showerheads and faucet aerators
- Utilization of underground sprinkler systems that provide for an efficient irrigation process