RSVP Home Care provides home medical equipment for patients with Pompe Disease. Pompe disease is a rare disorder that is often overlooked because it mimics other disorders in infants and children. It is very debilitating, progressive and affects many systems in the body.
Synonyms for Pompe Disease
Pompe Disease is broken down into various classifications depending upon how the disease is presented and when the disease occurs. The result is various synonyms for Pompe Disease including:
- Acid Maltase Deficiency (AMD)
- Glycogen Storage Disease (GSD) type II
- Acid Alpha-glucosidase (GAA) deficiency
- Lysosomal alpha-glucosidase deficiency
- Glycogenosis type II
- Infantile onset Pompe Disease
- Late Onset Pompe Disease
What is Pompe Disease?
Pompe Disease is a multisystem genetic disorder that is characterized by a deficiency in lysosomal enzyme alphaglucosidase (GAA). GAA is required to breakdown glycogen (a complex carbohydrate) and convert it into glucose. Massive accumulation of glycogen in the cells, particularly in cardiac, smooth and skeletal muscle cells results in muscle weakness and diminished muscle tone.
Infantile Onset Pompe Disease
Pompe Disease in Infants is characterized by severe muscle weakness and abnormally diminished muscle tone within the first few months of life. There may also be other abnormalities present including enlargement of the heart, the liver and/or the tongue. Without treatment, progressive cardiac failure usually occurs causing complications early in life.
Late Onset Pompe Disease
As the name suggests, late onset Pompe Disease occurs later in life: in childhood, adolescence or adulthood. The extent of the organ involvement varies; however skeletal muscle weakness is usually present with less cardiac involvement. Pompe Disease may go unrecognized for years.
Home Medical Equipment for Patients with Pompe Disease
Infants and children with Pompe Disease require expert medical treatment often including a team of medical professionals including: physician, cardiologist, neurologist and respiratory therapist. RSVP Home Care offers all the medical equipment necessary to provide you with the right breathing and feeding equipment you need to care for your child with Pompe Disease. Your respiratory therapist from RSVP home care can educate you on how to use the medical equipment your doctor prescribes to ensure you feel comfortable caring for your infant or child at home. We work closely with your medical team to ensure your child is getting the best care and treatments needed.
RSVP Home Care
RSVP Home Care is a full service DME (Durable Medical Equipment) provider supplying home medical equipment for patients with Pompe Disease in Northern Kentucky, Lexington, Louisville and Cincinnati. We maintain a large inventory of home medical equipment offering the latest technologies. RSVP Home Care accepts most major insurances including Medicaid. We accept insurance assignments and bill both your primary and secondary insurances for you. For all your home medical equipment needs call RSVP Home Care today.
Equipment Listing
RESPIRATORY EQUIPMENT
Ventilators – LTV 950 and 1150 Trilogy
Ventilator supplies; circuits, humidifiers
CPAP/NIPPV/BI-Level
Aerosol compressors (50 PSI) & supplies
Nebulizers & supplies
Suction machines & supplies (oral and tracheal)
Trach. Tubes (Shiley, Bivona, Smiths) standard & custom sizes
Tracheostomy supplies
Cough-assist devices
OXYGEN EQUIPMENT
Oxygen concentrators
High pressure systems
Oximeters and Probes
ENTERALS
Enteral pumps & poles
Nutrients & feeding kits
G-tubes, N/G tubes & supplies
MIC-KEY buttons & supplies
OTHER SUPPLIES
Complete line of dressings & gauze supplies
Incontinent supplies & Uri logical supplies