The last 5 years...

(Originally published on 1/31/14 on cotaforjensh.com)

The last 5 years…

Jens spent the afternoon New Year’s Eve (2008) in Nashville cheering on the Vanderbilt Commodores to their first bowl victory in 53 years at the Music City Bowl. However, that bitterly cold day marked a significant turn in Jens’s health when he spiked a 103 fever. At that time, his lung function was around 30%. His doctors at Vanderbilt (VUMC) decided to perform a bronchoscopy to determine why he was declining. Jens is a fighter, though, and so he eventually battled his way back to his baseline after those events.

Later that year, Jens would experience more health difficulties. In mid-August of 2009, he began to experience gut-wrenching pain while at work. He went to the hospital, was diagnosed with pancreatitis, and spent about a week there without being able to eat. After he was discharged, he was put on a no-fat diet for a while. Those of you who know Jens understand how awful that is, since he is typically on a high-fat, high-calorie diet to keep his weight stable. Pancreatitis has been said to “bring the toughest of men to their knees,” so you can imagine the pain he experienced.

September 3, 2009 is a memorable date in Jens’s life. In the early afternoon, he went down to the post office in his office building and quickly realized he was coughing up blood while discussing stamps with Sam, the mailman. This continued as he went back to his office, so he drove himself to the ER and it was decided that he needed to have an embolization to stop the bleeding. They placed a PORT during the same procedure, since he had needed IV antibiotics on a more regular basis. He spent three weeks at VUMC recovering while developing several complications, including:

• extended headaches and fevers 
• swelling of the legs/feet (put on diuretics to help with fluid) 
• pancreatitis (again) 
• major weight loss 
• bad reaction to an antibiotic, leading to kidney trouble 
• severe lung function drop 

Basically, it seemed that everything that could have gone wrong went wrong. Jens was determined, however, and he worked extremely hard with respiratory therapists to regain some of the lost lung function.

Once he was stable and well enough to travel, he went with his mom to Philly so that he could have her around to better support him during this time. He logged a weeklong stay at the UPenn hospital to continue to recover. The doctors there discovered that his PORT had been placed too close to his heart, so they had to redo the procedure at that time, as well.

In October, Jens went to the University of Pittsburgh Medical Center (UPMC) for his first lung transplant evaluation. This was a scary time for Jens, since this was the first time he went through this evaluation to hear about the transplant process. By the end of the evaluation, it was determined that he had made enough progress since his difficult month of September, and so he left Pittsburgh without being listed. He returned to Philadelphia to continue his recovery for the remainder of 2009.

Jens returned to Nashville in January of 2010. He was on IV antibiotics, and only able to work part-time for a few weeks to try to regain strength. An avid hockey fan, Jens took his roommate up on an offer to go with a friend who had extra tickets to the Preds game. It was that night, January 30, that Jens and Abby met for the first time. They went on their first date (to a Vanderbilt-Tennessee basketball game) on February 9. Jens traveled with his brother to Pittsburgh that summer to check in with UPMC. Jens again had made great progress and was not listed as a candidate for transplant at that time. Minus a few stints on IV antibiotics, he had a relatively uneventful (health-wise) 2010.

In the summer of 2011, Jens’s PFT’s actually went up a bit. He had been able to spend the summer travelling and being active. However, in August, he started to feel bad again. He battled for August and September, and on September 23, he went in for a chest x-ray. The doctors noticed that Jens had a collapsed lung. He was admitted to the hospital for X days to “reinflate” the lung and recover. This was the first time Abby had ever seen Jens in the hospital; it would not be the last that fall. One Sunday, Jens began to cough up blood again. He went to the ER, but the doctors could not determine where the blood was coming from. After spending a few days in the hospital, he was released. But, about a week later on October 20, he was able to get to the hospital in time after coughing up blood so that the doctors could tell where the blood was coming from. He had an embolization performed that Friday on his Mom’s birthday and spent the next 11 days in the hospital. During his time there, he developed pancreatitis again, and was again forced to battle through the pain while on a no-fat diet. No fat= no fun. Because of all of the difficulties of this year, Jens went to Pittsburgh after Thanksgiving to be evaluated, for the fourth time, by the transplant team. However, the doctors felt that Jens had made good progress during his recovery and decided to continue to monitor his health, without listing him on the active transplant list. 

Jens continued his good progress and he felt (relatively) well enough that he did not feel the need to travel to Pittsburgh for an evaluation in 2012.Thankfully, this year’s biggest/only health scare was some severe back pain Jens had around his birthday in August, requiring a weekend ER trip for some pain medicine. Coughing, which is hard to avoid during his treatments only aggravated his pain, so the doctor’s prescribed some muscle relaxers.

In January and February of 2013, Jens struggled again to be healthy enough to get off IV antibiotics. The doctors at VUMC recommended that he go to UPMC for another evaluation, his fifth to date. Jens’s mom and Abby accompanied him on this trip in late March. He went into this knowing that he probably wouldn’t be listed, as he showed some improvement once he stopped IVs in early March. The doctors there stressed that he should continue to monitor his “quality of life,” and that he should weigh the risks of the transplant against the “quality of his life” outside of his health regimen. The discussions Jens and his caregivers had post-evaluation were hopeful: everyone thought that he was maintaining good progress and that the transplant adventure was still a few years away.

2013’s most anticipated event, however was celebrating Jens’s 30th birthday! As a child, the life expectancy for people with CF was not even 30 years old. (The average life expectancy is around 38 right now.) Every birthday is a special one, but this one was a magical milestone. Many friends and family members contributed to a 65-minute birthday video, with their memories and best wishes for Jens. It was truly a day to remember! However, as the year progressed and the temperatures dropped, Jens struggled. Winter is always tough, as the “extreme” temperatures tend to aggravate him more. He went on IVs and did not see much difference.

We learned at the beginning of 2014 that his lung function had dropped to about 16%. He currently spends about 12 hours a day hooked up to IV antibiotics, in addition to continuing his 4+ hours of breathing treatments each day. The extreme cold weather of this winter has not helped him, either. He feels it is too risky to be off of IV antibiotics right now, and so his overall quality of life has decreased drastically. Jens and his caregivers are discussing the best plan of action in regards to when to go for an evaluation at UPMC, knowing that a double lung transplant is in the nearer future than anticipated.

COTA- FAQ

(Originally published on 1/25/14 on cotaforjensh.com)

Children's Organ Transplant Association (COTA)

FREQUENTLY ASKED QUESTIONS

Do Children’s Organ Transplant Association (COTA) parents typically have insurance? 
Yes, COTA parents typically have individual (private, Medicaid or Medicare) and family coverage (private only), and most COTA transplant-needy kids and young adults have been insured since birth.

If COTA families have insurance, why do they need help? 
No insurance plan covers all transplant-related expenses, which are significant. Transplant costs vary tremendously based on a number of factors, but generally transplants can cost anywhere between $250,000 and $800,000. These costs do not include family needs including lodging, food and transportation during the transplant stay. Additionally, post-transplant medications and medical care costs often add up to more than $10,000 annually. 

Most insurance providers require a family to pay an annual deductible that can exceed $10,000 and require a co-payment for each visit to the doctor, clinic or for individual medical tests. No plan pays all transplant-related expenses and even a ‘solid’ insurance plan may only cover 80% of the ‘normal and customary’ expenses incurred within a pre-determined network or at a specific hospital.

Even with insurance coverage, very few transplant families have the resources to meet these tremendous financial demands. When faced with the burden of a child’s potentially fatal diagnosis, most families need help.

Why do families choose to raise funds through an organization like COTA? 
When most parents first hear of a medical diagnosis where a transplant is the only chance at survival, the medical team typically tells them they are going to need a strong and supportive social network of family and friends to help them not only emotionally, but also financially. Several transplant fundraising organizations exist, and COTA encourages transplant families to research all of the options before making their decision regarding which one to partner with for assistance. Fundraising for transplant-related expenses eliminates a significant stress for families and allows them to focus on their patient’s medical and emotional needs.

While contributors’ natural instinct is to provide financial support directly to the transplant family, there are several reasons it is recommended that a qualified not-for-profit, such as COTA, is utilized for fundraising:

• Money received directly from friends and family could be considered taxable income to a transplant family, whereas the support received from COTA is not.  
• Money received directly from friends and family could jeopardize government-based aid (Medicare, Medicaid and others) for a transplant family, whereas the support received from COTA does not. 
• Gifts made to COTA are tax-deductible to the fullest extent of the law. Contributions made directly to a transplant family are not tax-deductible to the contributor. 
• Contributors who give to COTA can do so with the reassurance that all monies given to transplant families are used solely for transplant-related expenses, such as annual co-payments, deductibles and medications. 

The Internal Revenue Service (IRS) does not allow tax-deductible gifts to be made to individuals. Therefore, COTA works with community volunteers and ensures that 100% of the funds raised by a COTA community campaign in honor of a child or young adult will be used for transplant-related expenses. Once a family chooses to work with COTA, that family is eligible to submit reimbursement requests to COTA throughout their patient’s lifetime. 

Does COTA take a percentage of what is raised in honor of a child or young adult? 
No, COTA does not charge for its services or take a percentage of funds raised. 100% of funds raised in honor of patients are used for transplant-related expenses. Each COTA family (at no cost to the family) is provided a website for fundraising and communications through their community campaign volunteer team.

How does COTA invest the funds it collects? 
COTA’s Board of Directors has approved an investment policy statement, which the Board believes is a conservative approach to minimize risk, preserve investment capital and meet the transplant-related expenses of patients as well as day-to-day operational needs of COTA.

How do COTA families access the funds contributed? 
COTA has established internal procedures to ensure funds granted to families are used for appropriate transplant-related expenses. Each family must follow COTA guidelines, which includes providing appropriate documentation for all transplant-related expense requests. COTA reviews the documentation, and distributes funds to patient families or service providers.

Is COTA considered a reputable charity? 
Most definitely; Guidestar, considered by those in philanthropy to be the gold standard for evaluating charitable organizations, has given COTA an "Exchange Seal” that is comparable to the Good Housekeeping Seal of Approval and demonstrates COTA’s ‘commitment to transparency.’ Also, COTA is an Accredited Charity with the Better Business Bureau and its Wise Giving Alliance.

In June 2013, COTA received Charity Navigator’s 4-star rating for the third year in a row. The 4-star rating (Charity Navigator’s highest) is given to not-for-profit organizations that practice exceptional fiscal responsibility and governance in fulfilling their mission. According to Charity Navigator CEO Ken Berger, “Only 10% of the charities we rate have received at least three consecutive 4-star evaluations indicating that the Children’s Organ Transplant Association outperforms most other charities in America. This ‘exceptional’ designation from Charity Navigator differentiates COTA from its peers, and demonstrates to the public that COTA is worthy of their trust.”

What if COTA is not around in 10 years?
COTA was founded in 1986 and COTA’s Board of Directors and supporters are committed to ensuring its sustainability long into the future. In the unlikely event that COTA would cease business operations, COTA’s corporate and legal structure ensures that remaining funds would be disbursed for qualified transplant-related services to the patients on its roster.

What is Cystic Fibrosis?

(Originally published on 1/22/14 on cotaforjensh.com)

What is Cystic Fibrosis?

Jens was born with Cystic Fibrosis (CF). He was diagnosed when he was about 1 year old. CF is an inherited disease that affects about 30,000 people in the United States. A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that: 

• clogs the lungs and leads to life-threatening lung infections; and 
• obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food. (http://www.cff.org/AboutCF/) 

Each case of CF is different. Jens has a tougher case than most because he also has a bacteria called B. Cepacia. Because it is dangerous for CF patients with this bacteria complex to be around each other, Jens cannot be in physical proximity to anyone with CF. Jens spends over 4 hours a day doing breathing treatments of inhaled medicines, including time using an airway clearance device called “The Vest.” He must also take enzymes before every meal and snack to help his body digest his food. (For more info, stay tuned for an upcoming blog, “A Day in the Life of Jens”)

There is no known cure for CF at this time. A lung transplant will not cure CF because the defective gene that causes the disease is in all of the cells in the body, not just in the lungs. At this time, scientists are not able to “fix” genes permanently (see gene therapy). While a transplant does give a person with CF a new set of lungs, the rest of the cells in the body still have CF and may already be damaged by the disease. Further, organ rejection is always possible and drugs that help prevent organ rejection can cause other health problems. (http://www.cff.org/AboutCF/Faqs/ )

Why Transplant?

(Originally posted on 1/22/14 at cotaforjensh.com)

When Jens tells people about a transplant, he hears many reactions. One reaction is, “Why don’t you just go get one now?” Jens has fought hard to delay having a transplant because it does not solve all of his problems. A transplant exchanges one set of issues to deal with for another.

Why have a transplant?

• to extend his life 
• better quality of life 
• the ability to breathe normally 
• the risk of death without is greater than the risk of death due to surgery 

Why he’s waited…

• a lung transplant does not cure CF 
• he felt that he had more “mileage” left in his current lungs 

Jens has mostly felt that he wants to get as much mileage out of his current lungs as possible. But, he also does not want to wait too long so that he is not healthy to endure a transplant. He needs to be “sick enough to have one, but healthy enough to get one.” This is why he has worked so hard to stay as strong as possible.

When the time comes, Jens will undergo his transplant at the University of Pittsburgh Medical Center (UPMC). Jens and his family have chosen this facility due to its close proximity to his home base (Philadelphia) and the center’s acceptance of CF patients with B. Cepecia. They have been impressed with the doctors there throughout the transplant evaluations they have attended there over the past 5 years. http://www.upmc.com/Services/transplant/heart-lung-transplants/lung/Pages/default.aspx