Fighting for Joshua

Georgia Medical Care Foundation

TEFRA/Katie Beckett Review Nurse

PO Box 105406

Atlanta, GA 30348

To Whom It May Concern:

I am writing this letter in support of Joshua Folden’s renewal application for the Katie Beckett Deeming Waiver.  Joshua’s application has been denied because the criteria for Nursing Facility Level of Care (LOC) has supposedly not been met. 

Joshua most definitely requires and receives daily skilled nursing and/or rehabilitation care.  It just so happens that I, his mother, am the trained professional providing his care on a daily basis.  If I were not willing, not properly trained, or physically incapable of providing Joshua’s daily medical care due to illness or injury, he would require immediate placement in a nursing facility or multiple visits from a skilled nurse per day.

Following Joshua’s birth in July 2012, I lived in the Children’s Healthcare of Atlanta (CHOA) NICU with Joshua for 18 weeks, working side by side with his team of nurses learning how to provide care for his various conditions.  I learned CHOA protocol for maintaining his Central Venous Line (CVL) including changing the sterile dressing, changing the end cap, performing a Heparin lock, performing an Ethanol lock, and emergency procedures in case of a line breakage.  I learned how to prepare Joshua’s IV fluids and connect/disconnect him from the fluids following CHOA protocol.  I learned how to inject the proper additives, operate and troubleshoot the pumps, and set up and prime the tubing.  I learned how to administer his many medications, prepare his specialty allergen-free diet, and interpret the signs of diarrhea, malabsorption, and dehydration.  I learned how to speak the language of the hospital, navigate the different departments, and push doctors for more information.  And I continue to use these learned skills at home to care for Joshua’s chronic medical condition 24 hours a day, 7 days a week.

Joshua’s daily care can be broken down into the following categories:  TPN & Lipid preparation, Connect/Disconnect CVL including Heparin Lock, Sterile Dressing& End Cap Management, Ethanol Lock Administration, Medicine Preparation & Administration, Allergen-free Food Preparation, Diapering & Skin Care, Supply Management & Storage.  Each section will be explored in more detail with notations for tasks that are performed by a skilled nurse in a hospital or nursing facility setting.

*TPN & Lipid Preparation

On a daily basis, all necessary supplies are gathered and the work surface thoroughly cleaned.  Following standard CHOA protocol, including being gloved and masked, I inject three medications (Infuvite, Addamel, & Ranitidine) into Joshua’s TPN bag after thorough cleaning of the injection port, connect four sets of tubing, spike both bags of TPN and lipids, and prime tubing to remove all air bubbles from the line.

*In a hospital or nursing facility setting, this task is performed by a skilled nurse.

*Connect/Disconnect CVL

Joshua requires connection and disconnection from his IV fluids on a daily basis.  The pumps are turned off and all lines are clamped.  Following standard CHOA protocol, including being gloved and masked, the CVL end cap is thoroughly cleaned.  For disconnection, 2 mL of saline are flushed through the line, then a Heparin Lock is performed to prevent clotting in the line.  For connection, an empty syringe is used to draw back until blood is returned, then 2 mL of saline are flushed through the line, and the IV line is connected.  The TPN and lipids pumps are turned on, the programs started, and all lines are unclamped.  Extremely special care now has to be taken when handling and supervising Joshua to guard against line breaks which can lead to Central Line-Associated Bloodstream Infections (CLABSI’s) known to be life-limiting, which can lead to sepsis, coma, shock, embolism, endocarditis, losing catheter access for life-sustenance, up to and including death.

*In a hospital or nursing facility setting, these tasks are performed by a skilled nurse.

*Sterile Dressing & End Cap Management

Joshua’s CVL must be clean and free of bacteria and protected by a sterile dressing and Biopatch.  The dressing and Biopatch must be changed at least once per week and more often in cases when the integrity of the dressing has been compromised.  Following standard CHOA protocol, including sterile technique, I perform the dressing change in times of need.  Although our home nurse performs this task on a routine basis, I have been required to change the dressing myself when the dressing has come off inadvertently and during inclement weather when the nurse is unable to travel.  In addition to the dressing, the CVL end cap also must be changed on a weekly basis or more often in the case of soiling (this happens frequently since the end cap naturally falls around the top of the diaper area).  Improper cleaning and maintenance of the CVL end cap can lead to potentially life-threatening Central Line-Associated Bloodstream Infections (CLABSI).

*In a hospital or nursing facility setting, these tasks are performed by a skilled nurse.

*Ethanol Lock Administration

Twice a week Joshua requires an Ethanol lock in the CVL to help prevent potentially life-threatening Central Line-Associated Bloodstream Infections (CLABSI).  Following standard CHOA protocol, the CVL end cap is thoroughly cleaned, blood return is confirmed, 2 mL of saline are flushed through the line, then 1 mL of 45% Ethanol solution is allowed to dwell in the line for 4 hours.  Following this 4 hour period, the Ethanol must be withdrawn from (not flushed into) the CVL and a Heparin lock performed.

*In a hospital or nursing facility setting, these tasks are performed by a skilled nurse.

*Medication Ordering, Preparation, & Administration

Joshua’s medication schedule requires multiple medicines a day on a rotating weekly schedule that must be carefully followed.  Care and planning must go into obtaining the right compounded formulation of his medications with no excess sugars, artificial sweeteners, and dyes due to his GI system’s sensitivities.  Joshua receives medications throughout the day and night which must be specially prepared and administered.  His medication schedule is as follows:

Loperamide, 1 mg, 4x a day:  to combat chronic diarrhea caused by Short Bowel Syndrome; capsule is twisted apart releasing 2 mg of Loperamide powder, this is suspended in 2 mL of water and 1 mL is placed in his bottles of Pediasure Peptide at 9 am, 3 pm, 9 pm, & 3 am.

Simethicone, 0.6 mL, 3x a day: to help with gas and cramping, administered in bottles

Metronidazole, 85 mg, 3x a day on Days 1-7 and 14-21 of each month:  to treat ongoing Small Bowel Bacterial Overgrowth (SBBO) caused by loss of the Ileocecal Valve.  1.6 mL of specially compounded Metronidazole suspension is administered by mouth.  Medication requires cold storage.

Nystatin, 100,000 units/mL, 2.5 mL, 4x a day on Days 8-14 of each month:  to treat yeast and fungus overgrowth often caused by long term antibiotic use.

Lactobacillus GG, 1.5 billion cells, once a day on Days 21-28 of each month:  to reintroduce healthy bacteria to the gut after extensive antibiotic use

Ranitidine, 20.8 mg/0.83 mL syringe, injected into TPN once per day: to treat acid reflux

Infuvite, 5 mL syringe, add contents of 1 mL syringe to 4 mL syringe then inject combination into TPN once per day: vitamins

Addamel N, 1.6 mL syringe, injected into TPN once per day: trace elements

*In a hospital or nursing facility setting, these tasks are performed by a skilled nurse.

Allergen-Free Food Preparation

Joshua requires a special hypoallergenic diet due to food allergies and sensitivities.  His current formula regimen includes 7 three ounce bottles per day of Pediasure Peptide 1.0 at12 am, 3 am, 9 am, 12 pm, 3 pm, 6 pm, and 9 pm.  He receives a ratio of 2/3 Unflavored and 1/3 Vanilla flavored Pediasure Peptide to limit excess sugar.  Pediasure Peptide is a specialty formula that must be custom ordered and costs between $6-9 per bottle.  Pediasure Peptide requires cold storage after opening and use within 48 hours.  Joshua also eats limited solid foods.  Special care must be taken to offer only the foods which his nutritionist has approved.  Joshua has been diagnosed with a dairy allergy and wheat sensitivity.  He is also not allowed nuts, soy, eggs, shellfish, most fruits (except bananas), beans, and anything with excess sugar.  Failure to follow these guidelines could result in a serious allergic reaction including bloody stools.  Joshua’s food and formula intake must be closely monitored and immediately adjusted in the event of diarrhea, vomiting, or other symptom of GI distress.  If left untreated, excess diarrhea and vomiting will lead to dehydration and potential acidosis requiring an extended hospital stay.

Diapering & Skin Care

Short Bowel Syndrome results in an excessive number of stools per day, for Joshua this can be as many as 14-16 stools per day in times of illness.  These stools are usually loose or even watery in consistency and can quickly cause serious diaper rash that affect skin patency and increase risk of infection.  Swift diaper changing and a strict skin care regimen, including a specially ordered hospital-strength diaper cream, are adhered to throughout the day and night to protect the integrity of Joshua’s skin.  Loose stools often escape from the diaper and can expose the CVL to bacterial contamination potentially leading to Central Line-Associated Bloodstream Infections (CLABSI’s).  Excessive cleaning of surfaces, clothing, and anything else the CVL comes in contact with is necessary to help prevent potentially life-threatening infection. 

*Supply Management & Storage

Treating Joshua’s condition from home requires a significant amount of equipment and supplies.  I must be ever vigilant in planning, ordering, storing, and maintaining Joshua’s medical equipment.  The TPN & Lipids pumps and chargers must be routinely calibrated and verified.  The pumps must be charged on a daily basis to insure they are ready to use each night.  The TPN, lipids, Zantac, and Infuvite require cold storage, but also must be allowed to reach room temperature before infusion to prevent significant cooling of Joshua’s core body temperature.  I must always have enough gloves, masks, alcohol wipes, saline syringes, heparin syringes, empty syringes, three different types of tubing, needles, and dressing change supplies.  A sharps container must be maintained and hazardous materials properly disposed of.

*In a hospital or nursing facility setting, these tasks are performed by a skilled nurse.

Emergency CVL Procedures

CVL’s are prone to breakage and the formation of small holes at junctures in the line.  Line breaks and holes in the line have serious consequences.  A line break must be immediately clamped to prevent blood loss and minimize exposure to harmful bacteria that cause CLABSI’s.  Failure to clamp off the line can lead to potentially life-threatening excessive blood loss.  Holes in the line form slowly and can be difficult to identify until an infusion is running which leads to fluid and blood loss until the hole is discovered and the line clamped.  Joshua has had two holes form in the line.  One was discovered when our home health nurse flushed the line during a lab draw.  The other hole formed in the middle of the night and I found Joshua lying in a pool of blood and fluids during a routine nightly check.  Joshua has had his CVL’s break three times.  Each time I have had to rip his clothes off and pinch the line off with my fingernails to prevent blood spurting out while I located the regular line clamp or a pair of hemostats.  Every time this happens requires an ER visit, placement of a peripheral IV while the CVL is being repaired, and close monitoring at home for development of a CLABSI in the two weeks after a line break.  Joshua must be supervised at all times by an individual trained in emergency CVL procedures.  A CVL can become compromised at any time of the day or night.  My nightly routine includes checking on Joshua every 1-2 hours at minimum for confirmation the CVL is infusing properly.  I am also always on alert for pump alarms to clear air bubbles from the line in the middle of the night.  Since Joshua may require emergency attention anywhere we go, an emergency kit that includes hemostats, alcohol wipes, an end cap, saline syringes, heparin, gloves, masks, and a dressing change kit is always packed with us. 

After reading about Joshua’s daily care regimen, surely anyone can agree he requires daily skilled nursing care.  How fortunate that he has a mother concerned and capable enough to provide the around the clock care and supervision Joshua requires.  I have been at the bedside every day and night he has spent in the hospital, have attended every GI clinic, every Developmental Progress clinic, every pediatrician’s appointment, every nurse’s visit, every Physical Therapy, Occupational Therapy, Speech Therapy and Music Therapy session.  There is no one better equipped to provide the continuity of care Joshua’s chronic GI condition and developmental delays necessitate. 

Please allow me the opportunity to continue caring for my son in the way he deserves by renewing his application for the Katie Beckett Deeming Waiver as soon as possible. 

Sincerely,

Kristi L. Folden

Mother, Legal Guardian, & Skilled Nurse to Joshua Folden