Personal patient story – Cystic Fibrosis
Hello everyone, my name is R. Klein, I am 31 years old, married, and the mother of two charming girls.
I’ll start my personal story at the beginning. We are five children in my family. I’m one of twins. We were both born at the same weight, 2 kg, but we are dissimilar in both looks and character. From the moment I was born, I was a restless, crying baby whereas my sister was calm. I often suffered from ear infections, and for two years, I was constantly taking antibiotics. I also suffered from diarrhoea that left unresolvable stains on my clothes. After a while, a prolapse appeared in the intestine, so that every time I pooped the rectum would protrude outwards. Out of despair, my parents had found an exceptional gastro specialist who immediately identified the problem.
Thus, at the age of 2.5 and weighing only 6 kg, I was awarded the label of Cystic Fibrosis (CF) patient. The specialist explained to my parents that I am not absorbing the food and that is why I’m suffering from such severe diarrhoea. He advised that I take prior to every meal pancreas enzymes that would help with food absorption, a total of five capsules a day. Of course, a 2.5-year-old girl can’t swallow pills and so the capsule content was poured onto a spoon with water, and that’s how I took the enzymes. It took a long time until the dosage of enzymes right for me was found. I was living a nightmare, they checked the dosage through faecal monitoring every three days. During that time, I had to gain weight and catch up on my deficiency. I remember the pressure placed on my parents to increase my weight. The more pressure they applied, the more I refused to cooperate. It started with me being required to eat a slice of yellow cheese daily and continued with the pressure to eat sour cream daily, and later on it was also, ‘drink a cup of grape juice, too’ (who thought about diabetes back then?).
The rigidness of my parents made me an objector. I remember that when my parents weren’t paying attention, I would quickly throw the plate with the food, and I’d strike deals with my younger brother to drink the Scandishake (today it is Ensure). I don’t recall it was so awful but the pressure placed on me just made me not drink. I’m also not angry at my parents who felt pressurized to have me gain weight and also under pressure of the clinic.
Today, I’m certain that they know there are psychological consequences to this and that there are other ways to make a child eat. I promised myself that when I’ll have children of my own, I won’t run after them with food. When the girls were born prematurely and I was told to feed them every three hours, I couldn’t wake them up to feed. I told myself, a hungry baby would wake up on her own, and indeed that’s how it was. I never woke them up to eat, and furthermore, I wouldn’t breastfeed them because I didn’t want them to develop dependency.
My parents placed full responsibility on me, and I learned to take the enzymes with every meal. When I was 9 years old, I remember being shocked when a close friend told me that her mother was going to the funeral of someone who had CF. I always knew I had CF, but I didn’t realize it was a disease, and I certainly didn’t think you could die from it. That day, when I returned home, my parents explained what I had and that’s when I understood what I suffered. When I was 11 years old, my parents brought a physiotherapist to our home. Until then my siblings and I had physiotherapy with music so I wouldn’t feel different.
At the age of 14, I started coughing and a bronchoscopy test revealed a very rare bacteria, a first cousin of tuberculosis. Antibiotics were administered to me intravenously, and it took me seven years to abolish this bacteria. During this time, I had two PICC lines and a Hickman tear and replaced. Today, I have a port. From that time (age 14), I started coping with the disease by myself. I would use inhalations several times a day and do physiotherapy for an hour a day to remove phlegm and continue my life as usual. I conducted a very lively social life at high school.
My twin, who was in the same class as I, supported and helped me with my studies when I was absent. Classmates helped me inject medicine intravenously in a sterile manner and frequently visited me at the hospital. Some of my friends started different “weight loss diets”. I found it difficult to tell them I had to gain weight and I was ashamed that I had to do the opposite, to gain weight and have some reserves.
My mother was a salaried employee and had difficulties missing workdays so I started going to the clinic with my twin sister. The bacteria that had attacked my lungs developed resistance and the doctors had to seek new antibiotics, leading to lengthy hospitalizations. At the same time, my physician, Professor Kerem, found a new antibiotic that was sensitive to the bacteria that I had, but it was manufactured in the US and was very expensive.
Fortunately, a childhood friend of my mother’s in France, who works for a pharmaceutical company, had found the medication in Belgium at low costs. The Ministry of Health helped import the medication which had helped contain the bacteria for two years. Then it developed further resistance to the antibiotics. Infectious disease specialists had found a new medication that was still being researched.
I received this medication, which had very harsh side effects, nausea, vomiting, and drastic weight loss to the point of damage to liver function and jaundice. At that point, the pharmaceutical treatment was ceased, and I was administered TPN (total parenteral nutrition) to allow the body to restore itself. The antibiotic treatment failed, and after a few months, they found an orally administered antibiotic that cost 500 ILS per pill. Within three months, by the grace of the heavens, the bacteria were eradicated.
I finished high school and three years of preschool teaching. I worked in educational noon settings and quickly realized that it wasn’t for me. It was hard physical work. I then tried working as a receptionist but the disease took its toll. Clinic visits, hospitalizations, and the heavy therapy burden made it difficult to persist in the workplace.
At the age of 19, I married an amazing guy who learned to know me and the disease.
At the age of 21, diabetes burst. For me, this was just another piece of luggage appended to the large luggage I was already carrying. It was not pleasant to start counting sugars and measuring sugars. I was confused about my diet – could I eat sugar and inject? Or refrain from sugar altogether? Was it regular diabetes or was it related to the CF?
After two years of marriage, my medical condition stabilized so the doctors allowed me to get pregnant, provided I achieve high lung functionality and stable weight. After trying for a year, I was finally pregnant. I can’t describe the joy and happiness. Quickly, however, difficulties arose. Nausea and vomiting like any pregnant woman, but for me, there was a substantial weight loss. At week 14, the nausea subsided but I was unable to gain weight.
At week 24, strong pains emerged and the CF specialists claimed these were early labor contractions, which fortunately were not observed by the gynaecologist. After another week of pain and vomiting, they realized it was bowel obstruction. From that moment on, I was hospitalized until week 34. They tried to increase my weight with Ensure, which worsened the obstruction so they reverted to TPN alone. At week 36, I gave birth (it was an amazing labour) to a daughter weighing 1.720 kg who was released from NCIU two weeks later weighing 1.990 kg. Breastfeeding attempts failed. I started the pregnancy at 50 kg and finished it at 52 kg.
Life went back to its regular course and I had to manoeuvre between the demands of treatment and the demands of the baby but my family helped me a lot. After four years, I gave birth to another daughter. I had to face a lack of weight gain and the occasional hospitalization to take antibiotics intravenously. My girl was born at week 37, weighing 1.980. I was very pleased with myself.
I am happily raising my girls, studying marital counselling at the YNR college. I manage a neighbourhood charity organization, Mutsafim Bintina (Overwhelmed by Giving) named after the street on which I live – Mutsafi St. I volunteer in the medical equipment borrowing centre of Ezer MeZion (Help from Zion), and I’m also a member of the Help to the Individual Committee in the CF organization in Israel. My desire and dream is to help patients and their families as they cope with the disease and family.
Coping with the disease is not easy – every day a private physiotherapist comes to my home. I take antibiotics intravenously every three months for two weeks. In times of setbacks, I am orally fed by PEG to balance my weight.
I was nevertheless blessed by the Lord with a supporting family, two sisters who live in the same city as I, a highly collaborating husband. My daughters have learned to manage when I am hospitalized or feeling unwell. I also have friends who help me, caring for hot meals, helping with ironing, and hosting my little one during hospitalizations.
I pray and thank the Lord to continue to give me the strength to help myself and my entire family. Amen!!!
Written by R Klein
Israel
