~My Cystic Fibrosis~

By Franklin D. Heintz, Jr.

There is no way for me to know when you read this article weather or not I will have had my transplant or still on the waiting list. There are many sources of information on Cystic Fibrosis (CF) available. This article is for those who wish to know what it is like for me to live and have an active lifestyle with "End Stage CF" and why I chose to have a transplant. Please, remember that each case of CF is different with it's own set of unique problems. I am planning, in the near future, to have a Bilateral (double) Lung Transplant at Ochsner Foundation Hospital. I have become very optimistic with the love and support of my friends and family. I have a wonderful life.

My name is Franklin "Frankie" Delano Heintz, Jr. I live in New Orleans, Louisiana, where I have been followed by the Cystic Fibrosis Center at Tulane University Hospital and Clinic since 1973, when I was first presented at the age of seven weeks. A diagnosis of Cystic Fibrosis (CF) was officially made at that time. I was then followed at periodic intervals in the pediatric CF Clinic until 1994. During much of my childhood and teenage years, I had a relatively sever case of Cystic Fibrosis with several hospitalizations and numerous surgeries.

In June of 1994 I had an acute exacerbation requiring emergent hospitalization and emergency intubation of ventilatory support and was hospitalized in the Intensive Care Unit at Tulane University Hospital. I then improved gradually during that hospitalization and after several weeks was removed from ventilatory support and discharged home on continued IV antibiotics. An aggressive airway clearance therapy program was instituted at that time and since then I have been followed at close intervals subsequently in the adult CF Clinic.

I did reasonably well with the aggressive airway clearance therapy program consisting of Pulmozyme twice a day and combined nebulizer therapies with Albuterol and Ipratropium four times a day and chest-physical therapy (CPT) three to four times a day for the balance of 1994, 1995 and 1996. In 1997, it became apparent that there was a drastic decline in my lung function studies since the peak values that were obtained following my aggressive therapy and hospitalization in mid 1994.

Because of my decline in lung function and increase in clinical symptoms with fatigability, increased sputum production and low-grade fever, I was hospitalized and given a course of IV antibiotic therapy in late June of 1997. I clinically improved but without any improvement in lung function studies. Because of the decline in my lung function and overall functional status, it had become apparent to my doctor in the latter part of the summer of 1997 that I had now entered a phase of my Cystic Fibrosis (CF) that made lung transplantation my only consideration.

The last Pulmonary Function Test (PFT) I performed on August 25, 1998, revealed a FVC of 1.74 liters (36% of predicted) and an FEV1 of 0.80 liters (20% of predicted) with an FEV1/FVC ratio of 0.46. My referral for evaluation for lung transplantation was hampered by the fact that several sputum cultures taken had isolated an organism that was initially identified as Burkholderia Cepacia. Subsequently those specimens were then referred to a reference laboratory at Allegheny University in Philadelphia, and it now has been identified as Burkholderia Gladioli.

Other problems that complicated my referral for evaluation for lung transplantation in the past several months were persistent, and sever abdominal pain. That was diagnosed with an extensive GI Evaluation Study and in March of 1998 my Gallbladder was surgically removed and significant depression. My depression has responded rather dramatically to Zoloft.

I am a young adult that has Cystic Fibrosis (CF) with severe pulmonary disease consisting of Bronchiectasis with sever Obstructive Ventilatory Impairment. In terms of progression of the disease and lung function status. My doctor has assured me; "I am viable candidate for a Bilateral (double) Lung Transplantation."

I have always known I had CF. I was relatively healthy and had a completely "normal childhood." I did not start going into the hospital for the regular scheduled "Tune Up." Which is a two-week stay in hospital designed specifically for the administration of Intravenous (I.V.) medications, until I was 21 years old. I attended college, went to work for WRNO Worldwide as Operations Manager and was engaged to be married.

The one question I am most frequently asked is "How long do you have to live?" I know this, because I ask the same question of my doctors. Seriously, probably a year. I am just guessing of course, no one knows for sure, but you are not recommended for an organ transplant of any kind unless you have only 1 and 1/2 to 2 years left to live. I was told in August of 1997 that a Bilateral (double) Lung Transplant was the only option left available to me.

No one warned me it could happen that fast!

Drastically my health declined...

I really felt perfectly "normal" until just last year. I realized it was getting "Near the end." When gradually, it started becoming difficult for me to walk up stairs and maintain my weight. Still thinking that I had at least 3 years left. I began initiating my plans to move back home and begin saving money. This was a shock to my parents. They never thought I would be sensible enough to move back home even in the case of a medical emergency. As I moved home unfortunately, I became seriously ill. My health was in a total downward spiral. I ended up in the hospital and when I was released, I was on 3.0 liters of oxygen full time and could no longer work. I lost my apartment, my independence, my livelihood, and my fiancé. Most importantly, I lost the ability to breathe on my own overnight, literally.

Now What?

Physically...

Well, it has been a struggle all my life. Most recently, I cannot do much of anything; even changing my clothes leaves me breathless, literally. I can only walk a few feet without having to rest. I had to have a "Port-A-Cath" surgically inserted in my upper chest. This is used for the frequent and numerous treatments of I.V. medications that are prescribed for me. I get tangled up at times walking with my oxygen, which I must wear even while taking a bath. All of this is necessary for my survival. To say that I am thin is an understatement. I spend approx. 5 to 6 hours a day doing aerosol treatments and chest-physical therapy (CPT) designed specifically so I can cough up the infected mucus that is clogging my Lungs. That is only the time consuming part of CF. There is more, Sinusitis, Kidney and Liver disease, and Gallbladder infections but these are not time-consuming.

Mentally...

I lost my entire sense of self worth. I went from having purpose as a "Operations Manager" to, nothing. I went from being a reasonably attractive gentleman to this person who is just skin and bones. I realized my life was already over. The life I had grown accustom to anyway. So, sometimes I just cry. You teeter on the edge of a suicide. It is dark and frightening and you feel you can topple over at any moment. I have occasional anxiety attacks; out of the blue. I get fearful and depressed. My doctors tell me it is normal. I stress over the unknown, the surgeries and hospitalizations that are in my near future. I fear the suddenness in which my life will be one day snatched away.

There is an upside!

However...

I always knew I had this disease, and subsequently I make the most of my life. I have the photo albums and the memories to prove it. I appreciate the simplest things in life, more then most I believe. Spending time with my friends. Taking a shower without an "accessed" I.V. Not wearing an oxygen mask and enjoying hours of restful sleep undisturbed by coughing. I have few regrets. I am grateful for the love my friends show me. The resurgence of hope I feel after being listed for a Bilateral (double) Lung Transplant. Friends and hope are essential ingredients to surviving I discovered. For me at least they are.

I am thankful for my greatest ability. The ability to deny. Even as I type this I know that I am dying. There is a difference between knowing and admitting to yourself that you are dying. Knowing that you are dying, you are still surprised at the very idea you are dying. Here is the greatest gift of all. Thinking it will happen tomorrow, and I know that there will always be a tomorrow thanks to my chance at a new beginning with the gift of a transplant.

The End