What is the patient population for the SPS project?
This definition clearly defines the patient population for the SPS project for inpatient/observational status patients. The objective is to provide guidance for designing initial data collection and to consistently report data using the same methods each month. Other methods are acceptable. II. Population Definition
What is the average daily dose of SPS ® suspension?
The average daily adult dose is 15 g (60 mL) to 60 g (240 mL) of suspension. This is best provided by administering 15 g (60 mL) of SPS ® Suspension one to four times daily.
What is the best treatment for SPS?
People with SPS respond to high doses of diazepam and several anti-convulsants, gabapentin and tiagabine. A recent study funded by the NINDS demonstrated the effectiveness of intravenous immunoglobulin (IVIg) treatment in reducing stiffness and lowering sensitivity to noise, touch, and stress in people with SPS.
What are the signs and symptoms of SPS?
Abnormal postures, often hunched over and stiffened, are characteristic of the disorder. People with SPS can be too disabled to walk or move, or they are afraid to leave the house because street noises, such as the sound of a horn, can trigger spasms and falls.
Is SPS a terminal illness?
Stiff person syndrome (SPS) is considered dangerous in babies because they generally meet unfortunate and untimely death within a few months. The normal lifespan of a grown adult can be stated as 50 years (if symptoms start early).
Is there a cure for SPS?
Treatment with IVIg, anti-anxiety drugs, muscle relaxants, anti-convulsants, and pain relievers will improve the symptoms of SPS, but will not cure the disorder. Most individuals with SPS have frequent falls and because they lack the normal defensive reflexes; injuries can be severe.
Is Stiff Person Syndrome life expectancy?
What is the life expectancy of a patient with stiff person syndrome? The life expectancy for people with SPS ranges from 6 to 28 years from the onset of the condition.
Can Stiff Person Syndrome go into remission?
Two female patients achieved clinical remission from the rare, debilitating neurological disease called stiff person syndrome (SPS, which can be marked by a "tin soldier" gait) after an autologous (from your own body) stem cell transplant that eventually allowed them to return to work and regain their previous ...
Do all patients with SPS have diabetes?
It has been shown that ~45% of patients with SPS have type 1 diabetes (T1D), which is an autoimmune disease characterized by the immune-mediated loss of the insulin-producing pancreatic beta cells (1, 5, 8, 9).
Does Stiff Person Syndrome get worse?
General Discussion. Stiff-person syndrome (SPS) is a rare acquired neurological disorder characterized by progressive muscle stiffness (rigidity) and repeated episodes of painful muscle spasms. Muscular rigidity often fluctuates (i.e., grows worse and then improves) and usually occurs along with the muscle spasms.
Is Stiff Man Syndrome progressive?
Stiff person syndrome (SPS) is a rare progressive and often underdiagnosed immune-mediated disorder of the central nervous system characterized by progressive rigidity and triggered painful spasms of predominantly axial and proximal limb muscles.
How common is Stiff Person Syndrome?
Stiff person syndrome (SPS) affects only about one or two in a million people. Being diagnosed with this very rare condition can leave patients and families with many questions and few answers.
Does Stiff Person Syndrome affect the brain?
Stiff person syndrome (SPS) is a rare, progressive syndrome that affects the nervous system, specifically the brain and spinal cord. Symptoms may include extreme muscle stiffness, rigidity and painful spasms in the trunk and limbs, severely impairing mobility.
How does IVIg help stiff person syndrome?
Patients with Stiff-man Syndrome may produce antibodies that attack enzymes required for the normal function of the nervous system. Steroids, plasmapheresis, and intravenous immunoglobulin (IVIg) have been given to relieve some of the symptoms of Stiff-man Syndrome.
What is similar to stiff person syndrome?
Symptoms of stiff person syndrome are similar to other conditions such as tetanus, multiple sclerosis and muscular dystrophies. Your healthcare provider may use several tests to rule out these conditions and look for signs of stiff person syndrome.
Why is my body always so stiff?
Muscle stiffness typically occurs after exercise, hard physical work, or lifting weights. You may also feel stiffness after periods of inactivity, like when you get out of bed in the morning or get out of a chair after sitting for a long time. Sprains and strains are the most common reasons for muscle stiffness.
Q: When did you first start having symptoms, and what did you do?
A: I started having symptoms 10 years ago when I was 32. I was working as a physical therapist and teaching ballet, and I started feeling numbness in my foot, along with extreme back pain.
Q: How did you finally get on the path to a correct diagnosis?
A: A former patient of mine referred me to an immunologist named Arthur Lubitz. I went to him with a shopping bag full of scans. He took one look at me –barely able to breathe, crooked shoulders and spine—and said, “I don’t know what you have, but can see that you’re re really sick.
Q: What insurance problems did you encounter?
A: At one point I ended up at Johns Hopkins for another opinion, and they didn’t think I had a nervous system disorder and didn’t think I should have IVIG. This ended up causing a lot of problems with insurance. They wanted to cut me down to one IVIG treatment a month, but my doctors in New York wanted me on it twice a week.
Q: What treatment are you receiving now for SPS?
A: I’m not on IVIG anymore. I plateaued on the treatment, and my rheumatologist was able to find an oncologist to prescribe a chemotherapy drug rituximab, which has been helping me. Most oncologists would not prescribe rituximab because it is not FDA-approved for SPS.
Q: What advice do you have for other patients with rare diseases like SPS?
A: It’s really important to find a doctor who will listen to you and take you seriously.
What is SPS in pediatrics?
The Children’s Hospitals’ Solutions for Patient Safety Network (SPS Network) works to improve pediatric patient safety and represents the first and one of the most significant efforts by children’s hospitals to eliminate harm to hospitalized children. Initially started by the Ohio Children’s Hospital Association in 2009, Ohio State officials, the Ohio Department of Health, and eight Ohio children’s hospitals joined together to establish the Ohio Children’s Hospitals Solutions for Patient Safety Network to focus on specific pediatric quality improvement projects. In 2011, following success in Ohio, 25 additional hospitals from outside Ohio joined this initial group to launch the SPS Network and implement quality improvement and patient safety strategies across the country. In 2015, the Network grew to encompass more than 100 children’s hospitals in total and expanded into Canada.
How does SPS work?
SPS Network implements proven strategies and approaches to improve pediatric quality care and patient safety, including adapting each hospital’s organizational culture into one that prioritizes patient safety and quality, employing High-Reliability Organization practices, involving key leaders across children’s hospitals to increase administrative and clinical staff participation and engagement in patient safety improvement , establishing Patient Safety Committees, educating and training staff and providers, developing and adopting safe protocols and procedures, and using technology to share best practices that aid hospitals and health care facilities in reducing medical errors and improving patient safety.
Is SPS suspension contraindicated?
SPS ® Suspension is contraindicated in the following conditions: patients with hypokalemia, patients with a history of hypersensitivity to polystyrene sulfonate resins, obstructive bowel disease, oral or rectal administration in neonates (See PRECAUTIONS ).
Can sodium polystyrene sulfonate cause potassium deficiency?
Serious potassium deficiency can occur from sodium polystyrene sulfonate therapy. The effect must be carefully controlled by frequent serum potassium determinations within each 24 hour period. Since intracellular potassium deficiency is not always reflected by serum potassium levels, the level at which treatment with sodium polystyrene sulfonate should be discontinued must be determined individually for each patient. Important aids in making this determination are the patient's clinical condition and electrocardiogram. Early clinical signs of severe hypokalemia include a pattern of irritable confusion and delayed thought processes.
Is sodium polystyrene sulfonate selective?
Like all cation-exchange resins, sodium polystyrene sulfonate is not totally selective (for potassium) in its actions, and small amounts of other cations such as magnesium and calcium can also be lost during treatment. Accordingly, patients receiving sodium polystyrene sulfonate should be monitored for all applicable electrolyte disturbances.
Can you give sodium polystyrene sulfonate to nursing mothers?
Nursing Mothers. It is not known whether this drug is excreted in human milk. Because many drugs are excreted in human milk, caution should be exercised when sodium polystyrene sulfonate is administered to a nursing woman.
Issue Background
About Sps Network
- The Children’s Hospitals’ Solutions for Patient Safety Network (SPS Network) works to improve pediatric patient safety and represents the first and one of the most significant efforts by children’s hospitals to eliminate harm to hospitalized children. Initially started by the Ohio Children’s Hospital Association in 2009, Ohio State officials, the Ohio Department of Health, and …
Overview of Activities
- SPS Network implements proven strategies and approaches to improve pediatric quality care and patient safety, including adapting each hospital’s organizational culture into one that prioritizes patient safety and quality, employing High-Reliability Organization practices, involving key leaders across children’s hospitals to increase administrative and clinical staff participation and engage…
Results
- SPS Network’s efforts have resulted in significant improvement in pediatric care in its Network hospitals, including improved quality and patient safety across targeted HACs. Since 2012, this national effort has led to an estimated savings of more than $130 million.ix These reductions have saved an estimated 6,944 children from serious medical harm...
Alignment to The National Quality Strategy
- SPS Network promotes: 1. Making care safer. 2. Person- and family-centered care through the levers of learning and technical assistance; certification, accreditation, and regulation; and innovation and diffusion.