CF is not contagious:

Due to the frequent coughing of children with CF, it is often thought to be contagious. The fact is every child that has CF is born with it. It is an inherited disorder.

CF is not caused by anything the parents did:

Parents often feel responsible for everything that happens to their children. It must be made clear that nothing parents do before or during pregnancy can cause CF.

CF does not impair intellectual ability:

People sometimes confuse CF with cerebral palsy (CP). Cystic Fibrosis does not involve the brain and therefore does not impair intellectual ability at all.

CF is not curable, at this time:

There is no cure for CF at this stage, although the drugs and methods to treat CF improve everyday. This helps people with CF to grow up and lead active, productive lives.

The sweat test remains the most important and frequently used clinical test for the diagnosis of Cystic Fibrosis in South Africa.

What is a sweat test?

CF affects the exocrine glands. The sweat gland is a type of exocrine gland. CF causes an abnormal amount of salt to be lost in the sweat. The basis of the test is measuring the salt content in the sweat. If the child has CF, the analysis will show a high salt (sodium and chloride) level. This is a simple, painless and inexpensive test. However to be accurate it must be performed and interpreted correctly.

It is important to keep in mind that the test does not give an indication of the severity of the disease. This test cannot be used to identify a carrier since they do not have CF and therefore have normal sweat glands.

Individuals with cystic fibrosis can be diagnosed prior to birth by genetic testing and in overseas countries newborn screening tests are increasingly common. Due to the low numbers of affected people in South Africa, newborn screening tests are not performed unless there is a high risk of the child having CF.

There is currently no conclusive database of CF patients in South Africa, however information from the three regional CF Associations indicate that there are approximately 700 people with Cystic Fibrosis in South Africa presently.

In addition to this, due to the complex nature of the disease an unknown number of people with CF from the outlying/rural areas are misdiagnosed and do not have access to proper treatment. These people (more specifically children) sadly often die from malnutrition or pneumonia.

SACFA hopes to reduce the incidence of misdiagnosis by raising awareness of the disease in all parts of South Africa.

Where is CF treated?

Persons with CF are treated in specialized clinics at State Hospitals around the country (click here to find details on the clinic in your area). With primary health care and the treatment of other diseases like HIV/AIDS increasingly enjoying priority, specialized health care is suffering and the regional CF Associations are having to fund Doctors, support staff and equipment.

What is the treatment for Cystic Fibrosis?

Individualized treatment programs, involving mainly home treatment, generally include:

• physiotherapy two or three times daily to loosen mucus;
• inhaling of medications to alleviate congestion;
  • intravenous antibiotics for a two week period every 3 to 12 months to ward off or combat particularly nasty germs which persons with CF are susceptible to, for example pseudomonas;
  • pancreatic enzyme capsules to improve food absorption (often as many as 40-50 capsules a day);
• nutritional supplements and vitamins to promote good nutritional status;
• exercise programs and sporting activities.

The possibility of more effective therapy approaching a cure within the next few years brings a new urgency to diagnosing CF before lung damage occurs. For CF babies born between now and the time when new treatment becomes available, it would be tragic if their condition deteriorated to the extent that they were not able to benefit from any new treatment.

CF Specialists and support staff at regional CF clinics will establish individual treatment plans for CF patients.

Research demonstrates a clear link between regular exercise, weight gain, body mass, lung function and survival in CF.

Exercise is an important part of the daily treatment routine for people with CF as it assists with airway clearance and builds up muscle mass and strength.

Coughing is a natural part of having CF. People with CF are encouraged to cough and should not suppress this important airway clearance mechanism.

During sporting activities people with CF may experience coughing, wheezing and/or breathlessness.

If any of these symptoms are experienced by a person with CF, it does not necessarily mean that they need to give up their sporting activity. If in doubt individuals should consult their doctor and physiotherapist. Some may need to use a bronchodilator before exercising.

People with CF are encouraged to drink plenty of water during and after exercise to avoid dehydration especially in hot weather.

Those who live in conditions of high environmental temperature should take salt replacement tablets (normally 1 – 3 per day) may be needed during periods of exercise as people with CF lose excessive amounts of salt in their sweat. Speak to your dietitian or doctor.

CF specialist clinics should be consulted to advise gym instructors and physical education teachers if they have any concerns regarding the participation of a person with CF in specific physical activities.

PLEASE NOTE : Exercise does not replace regular physiotherapy.

Medication for Cystic Fibrosis patients can cost up to R30 000 a month depending on the severity of the disease. Please note the below mentioned is purely an indication of what medication is used generally for CF patients - it is for information purposes only. Always ensure that you discuss your treatment plan with your CF specialist in your area.

Cystic Fibrosis affects mainly the lungs and the digestive tract - these areas are likely to require medication.

Lungs

Medication can be administered in various ways: inhaled into the lungs using nebulisers, taken orally, or taken intravenously.

These drugs treat the lungs in the following ways:

• Bronchodilator drugs open the airways by relaxing the surrounding muscle. They relieve tightness and shortness of breath.
• Antibiotics help to treat or control persistent infection.
• Hypertonic saline has been evaluated and benefits most patients. It can be helpful where secretions are particularly thick.
• Steroids can help reduce inflammation in the airways.

Digestive Tract

Cystic Fibrosis affects the pancreas, so enzyme replacement capsules should be taken with meals and snacks to replace pancreatic enzymes and enable people with CF to gain more energy from the food they eat.

Pancreatic Enzyme Insufficiency

Pancreatic enzyme insufficiency is one of the common symptoms of CF. The CF gene defect results in a thickening of the pancreatic secretions. The accumulation of thick secretions causes obstruction and eventually leads to damage of the pancreas. Pancreatic enzyme insufficiency prevents digestion and absorption of nutrients. Large, bulky, smelly stools containing undigested nutrients are a direct result of a lack of pancreatic enzymes.

Pancreatic enzyme insufficiency is treated with pancreatic enzyme replacement capsules, which should be taken with every meal or snack. Dosing can be based on the fat content of the food items. Foods containing only carbohydrate, such as fruit, juices and boiled lollies will not require pancreatic enzyme replacement capsules.

Pancreatic Enzyme Replacement Therapy (PERT)

Specially coated enzyme beads (microspheres) have been a treatment breakthrough for people with CF. Effective and acceptable enzyme replacement therapy has been a significant factor in maintaining health for those with CF.

For infants and younger children, who cannot swallow capsules whole, the capsules can be opened and the enteric coated microspheres mixed with fruit gel. This is then fed to the infant before and during their feed. Older children can have the microspheres mixed with yoghurt or pureed fruit.

Dosage Of Enzymes

The physician and dietitian will monitor pancreatic enzyme therapy dosing. The dietitian will provide information on the correct dosage of enzymes for different foods.

Enzyme Storage

Certain areas in South Africa have unique climate which is not suited to preserving the potency of enteric coated microspheres. Parents and adults with Cystic Fibrosis need to be aware that this is costly medication and needs to be handled carefully. The Pancreatic Enzyme Replacement Capsules need to be stored below 25C, and kept in the dark and dry. They do not like to be left in the car. During summer, the preparations can go off quickly.

Your dietitian or doctor can advise you on the appropriate type/dosage of enzyme supplement.

Vitamin supplements

All CF individuals should receive supplements of the fat soluble vitamins A,D & E. Please refer to the South African Cystic Fibrosis Consensus Document for guidelines on daily recommended intake for nutritional supplements.

Chest Percussion

This technique aids clearance of secretions up and out of the lungs and increasing the amount of air entering the lungs. A cupped hand is used to clap the chest firmly (it is more comfortable through clothing or a towel). Chest percussion is often combined with postural drainage positions. Parents or carers will be taught the chest percussion and other friends can be taught as the person with CF becomes an adult.

Why is chest percussion important?

Chest percussion is important because helps to prevent the thick, sticky lung secretions from blocking the air tubes. This helps to reduce infection and prevent lung damage.

How much chest percussion is needed?

• The length of treatment sessions varies according to need. If there are few or no secretions, treatment sessions may only need to last 10-15 minutes. However, it could take as long as 45-60 minutes if there are many secretions to be cleared.
• The number of treatment sessions should be varied. Most people do two a day when all is well, increasing to four a day when necessary. If no secretions are present, some people with CF only need treatment once a day.
• Your physiotherapist can advise you on how much chest percussion is appropriate.

Who will do chest percussion?

• At first the adults that care for the child should do it. Later on, relatives or friends should learn so that no one person becomes indispensable.
• Breathing exercises can be introduced in the form of a game from the age of two or three. From around the age of nine, most children can start doing part of their physiotherapy for themselves.
• Most teenagers become completely independent and only require help if they have increased secretions.

Breathing Techniques (The Active Cycle of Breathing Techniques)

A physiotherapist can teach a variety of breathing techniques. These techniques use controlled deep and shallow breathing to move mucus up through the airways. Breathing techniques are also used to help re-inflate any areas of the lungs which may have been deflated because of mucus blockage of the airways.

Flutter Therapy/Bronch-u-Vibe

This technique uses a handheld oscillating positive pressure device through which you breathe out against an alternating resistance. This causes back pressure which results in expansion of the small airways, keeping them open for longer, whilst mini bursts of air flow move secretions out of the small airways and into larger airways where they can be cleared with a huff of cough in combination.

Positive Expiration Pressure (PEP)

PEP therapy involves breathing out though a mask or mouthpiece where a backpressure is created in the lungs by only allowing air out through a small hole. This back pressure expands the small airways and opens side channels (collateral ventilation) to get behind secretions, moving them to larger airways where they can be cleared.

Vibrations

These are usually done in a drainage position in alternation with percussion. "Vibes" are a gentle shaking or vibrating of the chest whilst breathing out. This creates mini bursts of air flow which dislodge the secretions, moving them up and out of the airways. They are usually followed by a huff or cough in combination, to clear secretions from the lungs.

Autogenic Drainage

This technique was developed in Belgium and is a controlled method of breathing that requires no equipment. It involves training to breathe at three lung volumes:

• low-lung volume to 'unstick' mucus
• mid-lung volume to 'collect' mucus
• high-volume maneuvers to 'expel' mucus

This technique requires extensive training and frequent practice.

The Vest™ system

The Vest™ system is an easy-to-use airway clearance device for both children and adults. It was originally introduced in 1988. The Vest™ System is currently used widely mainly in the U.S.A.. In South Africa, due to the costs (approximately R135 000 per vest), this system is currently not used by many CF patients. The device consists of an inflatable vest connected by two tubes to a small air-pulse generator. The current patented version is quiet and easy to transport, enabling people to receive effective airway clearance therapy at home, work, or on the road.

Speak to the physiotherapist at your clinic if you have any queries about your individual physio treatment. Always ask your doctor before considering any change in your treatment.

Transplantation remains the best option for prolonging life for many patients with CF who are nearing death. In South Africa there is a dedicated transplant team based in Johannesburg, Gauteng.

The following general information has been provided as a basic guide to people with CF. For information that relates to their personal health and situation people are encouraged to discuss the issue with their CF specialist.

Lung transplantation is an option for people with advanced lung disease resulting from CF.

Making the decision

Your CF specialist may discuss the option of a lung transplant with you when you have developed severe lung disease. This may be when your lung function levels have fallen below a certain point, when you have had an increased frequency of hospital admissions or you have an increasing resistance of bacteria to antibiotics.

Referral

When you have made the decision to have a lung transplant your doctor will refer you to the transplant centre for assessment. This will involve a series of clinical and laboratory tests both physical and psycho-social to determine suitability.

Lung transplantation may not be appropriate for some people with CF as there are some factors that increase the risk of complications developing after transplant. Your CF specialist will be able to discuss these with you.

Does CF “go away” after transplant?

The transplanted lungs come from people who do not have CF, so the new lungs do not have and will not develop CF. However, after transplant the person still has CF in the pancreas, sweat glands, sinuses and reproductive tract. The person still has to take enzymes with food and to help absorb the anti-rejection medications.

Challenges

Transplanted lungs are susceptible to infection, rejection and other complications that require treatment. Following transplantation the immune system is suppressed to help prevent organ rejection and this will decrease the ability to fight infections. Therefore people must become vigilant about their drug treatment in that they need to take their drugs at the same time every day. This ensures that the most constant levels of immunosuppressive drugs is maintained. At first, this, along with the number of drugs, can be quite overwhelming. However, people with CF tend to adjust easily as they are used to taking daily medication. They are swapping one lot of treatment for another. They are also used to attending outpatient clinics and generally taking an active role in the management of their treatment. In addition their carers and families are well rehearsed at providing much needed support.

Benefits

After recovery from the surgery many people with CF find they have more energy, can gain weight more easily and are more active than they were before.

Cystic Fibrosis is a disease of many disguises. The exact symptoms can vary greatly from person to person. The early symptoms of CF are also often very similar to other childhood problems. This can make it difficult to diagnose and as result may go undiagnosed or even misdiagnosed. Here is a list of some common symptoms of CF:-

Early symptoms (infancy and early childhood):

• 15–20 % of children born with CF are diagnosed at birth because they present with an intestinal blockage known as meconium ileus.
• persistent cough which expels thick mucus;
• recurrent pneumonia
• difficulty breathing
• excessive appetite, with weight loss;
• bowel disturbances, i.e. fatty / grey coloured stools;
• extremely salty tasting skin;
• excessive sweating in a very high salt content
• small salt crystals forming on the scalp
• failure to thrive

Later symptoms (later childchood/adolescents/adults):

• clubbing (enlargement) of the fingertips
• persistent chest symptoms
• uncontrolled “asthma”
• bronchiectasis
• male infertility/azoospermia
• sinusitis / nasal polyposis

Some of the common diseases that CF can sometimes be confused with is asthma, chronic bronchitis or pneumonia.

People are born with Cystic Fibrosis; it is a genetic disorder. CF is caused by a mutation in a gene called the cystic fibrosis transmembrane conductance regulator (CFTR) which helps create sweat, digestive juices, and mucus. Although most people without CF have twoworking copies of the CFTR gene, only one gene is needed to prevent cystic fibrosis. CF develops when neither gene works normally. The CFTR gene is therefore a recessive gene and, because both men and women can develop cystic fibrosis, CF is known as an autosomal recessive disease. The name cystic fibrosis comes from the characteristic scarring (fibrosis) and cyst formation within the pancreas, first recognized in the 1930s.

When two people who carry a defective version of the gene responsible for CF have a child, there is:

• a 25% chance that the child will be born with cystic fibrosis; 
• a 50% chance that the child will not have CF, but will be a carrier;
• a 25% chance that the child will not have CF, and will not be a carrier.

CF is one of the most common inherited disorders of Caucasians (whites). In South Africa 1 in 27 individuals in the White population, 1 in 50 in the Coloured population and at least 1 in 90 in the Black population carriers a CF mutation. The total is increasing as more children are correctly diagnosed, treated earlier and living much longer.

Cystic Fibrosis, (CF) is an inherited genetic disease that affects a number of organs in the body, primarily the lungs and pancreas) by clogging them with thick, sticky mucus. Repeated infections and blockages can cause irreversible lung damage and death. Mucus blocks the tiny ducts of the pancreas which supply enzymes required for digestion, and consequently food is not properly digested and nutritional value is lost in the process.

The sweat glands are also affected and the body may lose an excessive amount of salt during exercise or hot weather.

In early childhood, prominent symptoms include growth problems or frequent infections, especially of the lungs. As the disease progresses, frequent lung infections (pneumonia) often lead to problems breathing, lung damage, prolonged courses of antibiotics, and respiratory failure requiring support by a ventilator. CF can also lead to frequent sinus infections, diabetes mellitus, difficulty with digestion, and infertility.

The most consistent aspect of therapy in cystic fibrosis is limiting and treating the lung damage caused by thick mucus and infection with the goal of maintaining quality of life. Intravenous, inhaled, and oral antibiotics are used to treat chronic and acute infection.

There is no cure for CF, and most individuals with cystic fibrosis die young: many in their 20s and 30s from lung failure. However, with the continuous introduction of many new treatments, the life expectancy of a person with CF is increasing to ages as high as 40 or 50 in some CF individuals.

TESTIMONY BY CAROL BRYANT

SURFING THE WAVES OF LIFE – WITH A PURPOSE

Have you every sat on the beach, or in a high rise building, and watched the waves as they roll in and break on the beach, or further out to sea. Some humongous and frightening in size and challenge, and others small enough to ride with caution, but still have fun.

I’m not a surfer, but if you’ve ever watched surfers go out to “catch” the waves, you’ll notice they go out there with a purpose, not just to drift in the calm waters, but to challenge the big waves with respect and endurance, and then enjoy the exhilaration of their success.

The pattern of our every day lives is very similar, with highs, lows, and sometimes even breaking through to calm waters. All measured by the way in which we live our lives. The choices we make, the things we say and do, the way we react or deal with different situations and circumstances.

I would like to share some of the waves in my life, and how by the grace of God, I have managed to ride them, and find purpose.

I was born and raised in the good old southern suburbs of Johannesburg. Blessed with wonderful parents and family. I was however ill from the day I was born, and eventually at the age of two, I was diagnosed with the disorder which was then known as Fibrocystic disease of the Pancreas – now more commonly known as Cystic Fibrosis.

Basically, C.F. is a genetically inherited disorder, affecting mainly the pancreas /digestive system, lungs and sweat glands. Usually diagnosed in infancy, it is life threatening, and to date there is not cure. Many children diagnosed with CF do not reach adulthood. My parents were told my prognosis was “uncertain”

Now at age 49, and some change, I am believed to be ONE, of the oldest surviving patients still living with the disorder in S.A. Whilst this by no means makes me special, it does highlight just how blessed I am, and how much I have to be thankful for.

Having said that, it has not been an easy journey, and my life has been a series of highs and lows health wise, and more so, as an adult.

I have been in and out of hospitals, more times that I care to remember. In fact I should have shares in Entabeni and Westville Hospitals by now, so next time you go there, mention my name, you may get a good bed.

I am on first name terms with many Drs. and nurses at both hospitals. At this point I usually insert my own IV lines, administer my own meds, and monitor my diabetes, during hospital admissions.

Living with CF can be, emotionally, physically and financially draining

- On going infections weaken immune system and resistance, resulting in low energy levels
- Medical Bills even with Medical Aid – well I won’t even go there.
- Treatment is both costly and time consuming
- R12000 to R15000 p/m, p/patient (conservative estimate)
- Daily physio, nebulising, breathing on a daily basis.

At this point in my life, no two days are the same. Some days I can spring out of bed and be full of the joys of life, other days, I can hardly get out of bed, but it is on those days that I make sure I do.

I choose not to dwell on the negatives. God never promised us a problem free life. We all have a cross to bear, mine just happens to be CF. CF however is not the be all and end all of my life. I prefer to see it as a means to learn, grow and help others. THEREIN LIES PART OF MY PURPOSE!

I truly believe that it was part of God’s plan for my life, although it took me too many years to realize it. Before I did, I thought I could do it on my own. WOW! Was I wrong.

Reading “Purpose Driven Life” by Rick Warren, confirms so clearly that God has a special purpose for each of our lives. We just have to be open to recognize and make the best of it.

In 1985, I was offered a transfer from JHB to Durban. I was working as a sales rep at the time, and gladly accepted the opportunity. I was in an unhealthy relationship, and, I was in total denial of C.F.

Little did I realize, that by accepting the transfer, my next blessing was staring me in the face. About a year or so after moving to Durban, I moved into a commune. There I met my knight in shining armour, and now, my husband and best friend Mark. I was also blessed with an amazing extended family of in-laws.

Our love for each other, and decision to marry, were unquestionable, but we still had to deal with the reality, the facts on hand, bearing in mind that at this point we were still trying to do things in our own strength.

- probability of shortened life span
- possibility of no children of our own – we both love children
- financial impact
- the fact that CF is not an individual journey, but very much shared.

By the grace of God, and against all odds, we were married on June 1st 1990. A truly memorable occasion.

Two and a bit years later, we were further blessed with a little boy to raise as our own. A little boy who at 2 yrs, 8 months, came along with his own set of challenges. It was through Josh, now 18, that Mark and I found our way back to God, and for the right reasons. In hindsight, I believe it was God’s way of saying – it was about time.

God has thrown many a curved ball at us over the years, C.F. and other, but He has also always been there for us when we have fallen short of figuring them out.

Living with a disorder, and a child with special needs is challenging, and definitely builds character.

Part of that character building, is to be able to laugh, to see the funny side of difficult situations.

Mark one Sunday was reading the papers, when he noticed an advert for a dog. We were keen on getting a dog, so he phoned. The owners were re-locating. The lady explained that whoever took the dog, needed to understand that it had a pancreas problem. “No problem” said Mark, so does my wife, they can share medication”

Acceptance and knowledge help to understand and deal with the difficulties of C.F. I have tried wherever possible to keep up to date with medical advances. I am also actively involved in both the KZN and S.A. Associations.

A few years ago, I decided to put my knowledge in writing, and what started out as a therapeutic exercise, turned into what I call my “God Inspired book”. Three weeks before going to print, I had no title, no ending and no funds. I prayed about this “book” and before I knew it, I had a title, I had an ending, and what’s more, I had a sponsor.

My book – “Behind the Smiles” Our journey through life with Cystic Fibrosis has sold many more copies than anticipated. The glory however is not in the number of copies sold, but in the amazing people I have met along the way. Many of who, have influenced my life. As a result of my book, I have been able to fulfill a part of my dream, .. to become a Motivational/Inspirational speaker. This came about as a result of my being “re-trenched” on medical grounds, after holding the position of Regional Branch Manager for a Top Brand Hardware Manufacturer, for 12 years.

Support is imperative, and I am blessed with support in abundance, in my life with C.F., and in general.

Living with CF has enriched my life. It has opened doors of opportunity, built true friendships, boosted my confidence, memorable travel journeys, ability to council others in need, respect life and be humble, but more importantly, by God’s grace I have found Purpose in my life.
I give thanks daily, on the good days and the not so good.

God and I have a great relationship. Because he made me human, I do get anxious, frustrated, even scared sometimes, and I let him know too, but I can honestly say that I have never been angry, at having CF.

God has spared me for many extra years, and given me a great life. A Life with a Purpose.

Reality tells me that my health is and will continue to deteriorate, and I accept that, but it does not mean I’m ready to give up. In fact, I have my foot firmly on the brakes, but I am also not afraid to die. I know that when my times comes, I will be with God, and He with me. Until then, I will continue to surf the waves.

At the end of the day, in the big sea of life, C.F. is a small wave to those who don’t know any better, and a big wave to those of us who do.

MY PRAYER FOR EACH AND EVERYONE READING MY TESTIMONY,,IS THAT YOU SURF THE BIG WAVES WITH ALL YOUR MIGHT, AND ENJOY THE CALMNESS OF THE SMALLER ONES, AND FIND PURPOSE IN ALL.
AMEN!

My motto, you may want to share:

Learn and grow from yesterday, live, love, laugh and be thankful for today, plan for tomorrow, but never take it for granted.

GOD BLESS
CAROL BRYANT
carolbryant@vodamail.co.za

Hi, my name is Grant Desmond. I was born with an obstruction and when I was only one day old, I had to have major surgery - this is when I was diagnosed with Cystic Fibrosis. My parents were told that I probably would not survive and should I do so, the average life expectancy of a CF patient was only 12 years! My mom made sure that doctors and staff worked around the clock as she was not planning to leave the hospital without me. That was 29 years ago. CF is not just a "condition" you are born with - it is a way of life.

When I was a child my mom was responsible for the management of my health and made sure my nebulising and physio was done in the mornings with a second round of nebulising and PEP mask breathing at night. As I got older the PEP mask breathing was replaced with The Flutter. As a family we had a well balanced and healthy diet and I had all the necessary vitamin supplements which goes hand in hand with CF. It was absolutely ingrained in me to follow the daily routine of my health care regime.

After I matriculated I was fortunate enough to get a job in a video shop which enabled me to study through UNISA. Once I graduated with a B-Com degree, I was employed by an accounting company and currently work there as the office manager.

Three years ago I went on an overseas holiday and became really ill. On my return I discovered that the infection had lead to pneumonia. As a result, when I recovered, I took up cycling to get fit. Some close friends of mine rode with me and we entered the Amashova race in 2006...The Amashova is 108km long and we finished it! What an achievement! In 2007 we entered again but one of our healthy riders held us back and we unfortunately did not complete the race. We are hoping to enter again in 2009...watch this space!

I now live on my own (closer to work) and completely manage my own health.

My faith in God, a healthy lifestyle and my positive mindset carries me forward every day. What I have learnt from CF is that what you put into life is what you get out - CF is no exception.

The story of Noo

By Ashleigh Robertson

On November 10th 1999 my husband Alex and I we welcomed our first child into the world, Courtney Jade Robertson. She was born in the UK and was a very healthy and strong baby. The first year of her life she grew well and by nine months was walking everywhere. By the age of one, this had become running.

On December 5th 2000, Courtney was diagnosed with Type 1 diabetes. We thought our world had come to an end, as our darling little girl was so sick. The months that followed were very hard for all of us, but we had to try and learn as much as we could about Type 1 Diabetes and get on with life. The first couple of years Courtney spent a lot of time in and out of hospital, and her sugar levels were erratic. Fortunately, as she has got older, it has become a little easier for all of us. When I look back at how hard it was trying to keep Courtney healthy and well, I did not realise at the time it was preparing me for what was to come with Amy, when she was diagnosed with Cystic Fibrosis.

Amy Jessica, fondly known to all of us as "Noo", was born on September 9th 2004. Courtney was over the moon to have a sister and we were all very relieved that Amy was healthy and did not have diabetes. From day one she was a very hungry baby and would practically eat us out of house and home if given the chance. When she was six weeks old I had to add cereal into her formula milk to satisfy her hunger. Amy did well her first year and every time we went to the baby clinic they could not believe how much weight she was putting on, never mind the amount of times she went to the toilet or the smelly stools she produced after every meal. Nevertheless, she was still gaining weight and we nicknamed her our “Little Buddha”.

When Amy was a year old we sent her off to a daycare centre, but the months that followed were not good for her and she contracted one chest infection after another. Each time we took her to the doctor and after many tests and x-rays we were told she could have allergies to certain foods and we needed to pay extra special attention to her diet. We were even told she could have asthma.

When Amy was two, she had a rectal prolapse, and we went back to the doctor. More tests, and we were told there was nothing wrong with her. In February 2007 we took Amy to Courtney’s endocrinologist, where he ordered more tests including a Trypsin test. I duly took Amy’s stool off to the laboratory for testing and to my surprise discovered they did not know what the test was for. I tried to contact our doctor but he was already on a plane, heading to the USA for a conference. I decided to “Google” and discovered the test was used to diagnose cystic fibrosis.

It was something we had never heard of or knew anything about. However, the Internet is a wealth of information and soon I started to get a better understanding of the disease. We waited about three weeks before results came back from the Trypsin test. When they came back we were called in to our doctor, who requested a “sweat test”. By now I was convinced Amy had cystic fibrosis and had prepared myself for the outcome. We headed off to the lab for the test, and a few hours later the results came through. The nurse read the results and told us to wait for a doctor. He confirmed what we had been dreading. We had been expecting it, so were already a bit more mentally prepared. A further blood test confirmed she has the DF508 mutation.

The weeks that followed her diagnosis was crazy. Not many people could give us information on medication, physiotherapy or the treatment of cystic fibrosis. Courtney’s doctor was happy to treat her but said it would be better to see a specialist CF doctor. Some months later, I was at our local clinic seeing our GP about Courtney and got talking to a lady I had worked with a few years before. She was now working at the clinic and I told her about Amy. She told me the lady she was working for had a niece with CF and she would put me in contact with her. That is how I meet Marie Allen and our saving grace at that time.

Marie told us all about the monthly CF clinic at Durban's Addington Hospital, and Dr Egner. On the first Tuesday of May 2008 we headed off to the hospital to attend our first CF clinic. Two years later, all I can say is a huge thank you to Dr Egner and to our wonderful physiotherapist Mary Rudd. Presently Amy is doing very well, and although we have our moments when she gets sick, we all work hard to get her better again. Our lives have changed drastically again, and if we are not busy nebulising Amy, issuing her with Creon, or giving her all of her vitamins and antibiotics, then we are busy sorting Courtney out with her Diabetes.

I don’t think anybody that has healthy children will be able to appreciate what we live with on a daily basis: how much hard work goes into trying to keep them healthy; sleepless nights we have; the amount of medication we have to administer and the financial burden that it puts on us, as a family. There are times when I get frustrated with people who ask me “How are you coping ?”, “How do you live a normal life ?” and even “I could never do what you do”.

The bottom line is that we have no choice. For the wellbeing of our children, and to give them the best possible care in the world and as normal a life as possible, we do it and just get on with it! Alex Robertson adds: "I would like to thank my wonderful wife who gives so effortlessly to Courtney and Amy and who really takes such good care of them. From me, your husband a huge thank you, and from Courtney and Amy, thank you mummy for taking such good care of us. We all really appreciate everything you do."